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Child Health, test 3
Child Health, test 3
699
Medical
Undergraduate 4
05/14/2009

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Term

______________ is an inborn error of metabolism (IEM).  There is an absence of phenylalanine hydroxylase needed to metabolize the essential amine acid phenylalanine to TYROSINE.  

Definition
Phenylketonuria
Term

Where does PKU come from?  Does it last a lifetime?

Definition
PKU is an inborn trait, in other words.... it's congenital. PKU is a LIFE LONG DISEASE.
Term
Why do we need Tyrosine... what does it form?
Definition
Tyrosine is needed to form melanin, epinephrine, and thyroxine. So there is a malfunction in the metabolism of these with PKU.
Term
So, without tyrosine there is a decreased production of melanin.  What does this result in?
Definition
Blond Hair, blue eyes, and fair skinned childred.
Term
If phenylalanine is not metabolized in the blood stream and causes damage to the developing brain and CNS.  What will occur if this is not corrected?
Definition
mental retardation
Term
PKU is an autosomal ________ disease that happens rarely (1 in 4000-12000 births) - controllable by diet when diagnosed.  The highest incidence is in US and Northern Europe.  It primarily effects _________ children.
Definition
PKU is autosomal RECESSIVE. It mostly effects CAUCASIAN CHILDREN.
Term
What are some clinical s/s of PKU?
Definition
FTT (failure to thrive), vomiting, irritable, developmental delays, unpredictable & erratic behaviors, mental retardation, mousy or musty smelling urine (the urine smells abnormal with PKU).
Term
The _______ blood test is used for PKU.  The Normal amount is 0.5-1 mg/dl.  A diagnosis of PKU is when there is more than ____ mg/dL.  Is Screening optional for parents?
Definition
Guthrie Blood Test. PKU > 4 mg/dL. Screening for PKU is MANDATORY in all 50 states. Usually done just before discharge - after child has ingested proteins.
Term
Managing PKU involves meeting the child's nutritional needs for growth & maintaining phenylalanine levels within a safe range.  It is necessary to restrict ________ from the diet.  However, some is allowed - small amount.
Definition
PROTEIN
Term
How much protein (phenylalanine) is allowed in a child's diet?  
Definition
The diet is allowed 20-30 mg/kg/day of phenylalanine. A level of 2-10 mg/dL is recommended for children.
Term
Management includes giving what kind of milk to kids?
Definition
NO COWS MILK. PKU needs Specially Prepared Milk Sustitutes/Special Formulas. (Some names - don't memorize tho - are Phenyl Free 1, Phenyl Free 2, Phenex, Phenex, Phenyl Free).
Term
Is breast feeding allowed for PKU children?
Definition
Yes, breastfeeding is allowed with careful monitoring as it has low phenylalanine content.
Term
Children with PKU should not be given any artificial _________ .  why?
Definition
Sweeteners (like Aspartame) b/c it is converted to penylalanine in the GI tract. NO ARTIFICAL SWEETENERS.
Term
Children with PKU really need assistance by a registered dietician... it's essential.  They will show child & family how important ____________ size is.
Definition
portion. They need to stay on their diets for life!
Term
It is very imp for PKU kids to monitor their _________ levels.
Definition
blood
Term
Women who have PKU need what?
Definition
genetic counseling for pregnancy. It's really important to know potential to pass PKU on b/c it is autosomal recessive.
Term
The inability to synthesize adequate amounts of Thyroid hormone (which does a lot of metabolism) is ____________ _____________.
Definition
congenital hypothyroidism.
Term
Is congenital hypothyroidism usually seen alone or with other abnormalities?
Definition
Often associated with other abnormalities & genetic defects. (Like PKU and others...)
Term
Congenital Hypothyroidism is an autosomal _________ trait.
Definition
recessive
Term
S/S of Congenital Hypothyroidism are:
Definition
poor feeding, lethargy, prolonged neonatal jaundice, poor breathing & bradycardia, constipation, hoarse cry, large fontanels (do not close by the time we expect). Essentially, everything is massively slowed down.
Term
Other S/S of Congenital Hypothyroidism that occur after 6 WEEKS OF AGE are:
Definition
Depressed nasal bridge, Short forehead, puffy eyelids, large tongue, coarse dry lusterless hair, large fontanels and wide cranial sutures, umbilical hernia, abdominal distention, hypothermia. DEPRESSED NASAL BRIDGE, LARGE TONGUE, LARGE FONTANELS, PUFFY EYELIDS
Term
Why is early dx of Congenital Hypothyroidism so imp?
Definition
Early Dx helps prevent progressive deterioration of the nervous system and mental handicaps.
Term
Is screening for congenital hypothyroidism mandatory?
Definition
Yes, mandatory in all 50 states.
Term
What screen is done for Congenital Hypothyroidism?
Definition
Simple Blood Test - measures T4 blood levels. Ok to take in first 24-48 hours, prior to discharge.
Term
What is the prognosis of Congenital Hypothyroidism like?
Definition
Normal physical growth & intelligence is possible if medication is started shortly after birth.
Term
What is the treatment given after Congenital Hypothyroidism is diagnosed?
Definition
Lifelong Hormone Replacement of SYNTHROID (synthetic levothyroxine sodium). The med is "LIFE LONG" and will stop/prevent any mental or physical problems.
Term
Parents must be taught about S/S of overdosing on thyroid medication... what are they?
Definition
Child is IRRITABLE, NERVOUS, TREMORS, INSOMNIA, LOOSE STOOLS, OR WEIGHT LOSS, INCREASE IN APPETITE, FEVER, SWEATING, TACHYCARDIA, PALPITATIONS. The infant is often "very high strung & inconsolable. It's very important to know everything is essentially ramped up. Imp to be able to check the child's pulse.
Term
What are the S/S the child is receiving an UNDER dosage of Synthroid (thyroid supplement)?
Definition
Under dosage = return to early manifestations. SLEEPY BABY, FATIGUE, DECREASED APPETITE, CONSTIPATED.
Term
How is Synthroid given?
Definition
Med is crushed & placed in formula or food.
Term
Blood levels & Thyroid function tests are done as recommended.  Imp to know later to get what kind of counseling?
Definition
genetic counseling d/t autosomal recessive. Be aware they could possibly pass this on.
Term
Growth Hormone Deficiency is controversial & can have many causes.  What is "hypopituitarism" cause?  What about "panhypopituitarism?"
Definition
Hypopituitarism = dysfunction of the pituitary causes decreased/absent hormone production, normally the cause. Panhypopituitarism = a total lack of ALL hormones. There is usually many other abnormalities with this.
Term
Is Growth Hormone Deficiency congenital or acquired?
Definition
BOTH. Can be acquired d/t damage at birth or after, as a result of a serious infection, head injury or tumor. A head injury can effect growth plates. We will see it at growth spurt (11-12) if not obvious at birth.
Term
What are other causes of short stature?
Definition
Constitutional delay of growth, familial or genetic short stature, nutritional issues, renal disease, chromosomal abnormalities, syndromes, small for gestational age, idiopathic short stature
Term
There are psychosocial aspects of having short stature...  
Definition
THE MOST IMPORTANT PROBLEM IS LOWER SELF-ESTEEM R/T HEIGHT DISCREPANCY, social skills and anxiety, academic issues, appearing younger than chronological age, parental guilt
Term
What's the diagnosis for GH deficiency?
Definition
Child who is only as tall as kids 2 or more years younger (ex/ a 6 yr old shorter than avg. 4 yr old) OR who falls away from a previously normal growth curve. They should both be evaluated by a ped endocrinologist.
Term
What is the purpose of evaluating for GH deficiency?
Definition
to verify whether or not there is an absence of GH and/or other organic causes.
Term
When evaluating GH deficiency, what else needs to be evaluated?
Definition
A detailed fam history r/t heights of relatives, siblings & parents. Info on child's general health and nutritional state, past illness and injuries. Growth patterns since birth are evaluated. Radiographic surveys including EPIPHYSEAL MATURATION & bone age. (epiphyseal - is growth plates... once they close - you can't add any height at the end of therapy)
Term
Drugs used to stimulate GH production are:
Definition
insulin, arginine, glucagon, & levodopa.
Term
When are blood samples taken for GH deficiency?
Definition
overnight during sleep. They will also monitor for 24 hrs & see if they are getting the hormone release amy when they're supposed to.
Term
Replacement of GH is effective in __% of children.
Definition
80
Term
How much synthetic hormone is given for treatment of GH deficiency?  What are the results in 1st year?  following years?
Definition
25-50 mcg/kg daily injections (subq or IM). Growth is 4-4.8 inches in the first year & 2.7-3.5 in following year.
Term
What is the criterion to stop giving synthetic growth hormone?
Definition
If the plates are closed (Epiphyseal Closure) means the end of height increase.
Term
The FDA has approved using GH in children who have what conditions?
Definition
GH deficiency, Chronic Renal Failure, Turner Syndrome, Prader-Willi Syndrome, SGA, Idiopathic short stature.
Term
GH treatments are very costly - $20-$30,000 per year! Why is GH treatment so controversial?
Definition
some think GH should not be given to otherwise healthy children. Others feel that short stature (below the 3rd percentile) should be treated.
Term
Precocious puberty used to be sexual development before 9 in boys & 8 in girls. What are the current ages?
Definition
Same for boys - before age 9. Puberty in girls is occuring earlier though. For caucasians, it's puberty before 7 yrs old. For African Americans, it's puberty before 6 years old. oh my GOSH!
Term
What is the cause of precocious puberty?
Definition
THE MAJORITY OF CASES, IT IS UNKNOWN. Pituitary gland may signal the ovaries and testicles to make hormones at an earlier time than usual. Or an abnormality may exist in the ovary, testicles, or adrenal glands causing the change.
Term
Signs of precocious puberty are -- Puberty is developing earlier.. what for females & males?
Definition
girls: breast development, pubic hair or underarm hair, a "growth spurt" in height, onset of menstruation, acne, body odor. boys: enlargement of testicles or penis, pubic, underarm & facial hair, deepening of voice, rapid height growth, acne
Term
What will the medications do for precocious puberty pt's?
Definition
The meds will cause females to decrease in breast size/allow for NO more development. meds in males will cause penis & testicles to shrink back to age appropriate development. Height will also slow down to the expected rate before puberty begins. The meds will halt development at that point until kids really should be going through these changes. Treatment is discontinued at a chronological appropriate time allowing puberty changes to resume.
Term
What psychological support is necessary for a precocious puberty pt?
Definition
Dress & activities should be age appropriate for child (a 6 yo child is a 6 yo even with breasts... follow correct age, not just b/c body is ahead).
Term
Is sexual interest usually advanced before the child's chronological age?
Definition
No, usually sexual interest is not advanced
Term
Are precocious puberty children fertile?  should they be on contraception?
Definition
Yes, they are fertile, but no contraception is recommended unless the child is sexually active.
Term
What are the most difficult years for precocious puberty kids?
Definition
school age years before puberty. After puberty begins for the other kids, the physical differences are not apparent.
Term
What is the definition of Congenital Adrenal Hyperplasia (CAH)?
Definition
It is deficiency of an ENZYMES used by ADRENAL CORTEX to produce CORTISOL from CHOLESTEROL.
Term
CAH -
Definition
Is the decrease in production of cortisol from cholesterol. Lack of enzyme needed to produce the cortisol from the cortex.
Term
What makes CAH occur?
Definition
CAH is an INHERITED DEFECT. It is an AUTOSOMAL RECESSIVE DISORDER. CAH occurs in 6 different enzymes - 90% of cases involve the ENZYME 21-HYDROXYLASE.
Term
The adrenal glands normally make steroids from cholesterol to secrete 3 hormones:
Definition
1. Mineralcorticoids to produce Aldosterone. 2. Glucocorticoids to produce Cortisol 3. Androgens to produce Testosterone
Term
In utero the developing fetus' pituitary senses a lack of cortisol.  It therefore attempts to FORCE THE ADRENAL TO PRODUCE CORTISOL by stimulating the gland.  Since there is no cortisol being made by the adrenal gland - what happens?
Definition
Androgens are produced in excess (they produce Testosterone). EXCESSIVE PRODUCTION OF ANDROGENS.
Term
CAH leads to in utero virilization of the uterus.  In other words...  CAH causes - 
Definition
Females to be born with AMBIGUOUS GENITALIA. (the clitoris is hypertrophied & resembles a penis with hypospadius, labia is fused to a variable degree and appears as a scrotum, urogenital sinus opens behind the clitoris, but the INTERNAL ORGANS ARE THOSE OF A FEMALE). If a male has CAH - their external genitalia looks normal. The genitalia & skin creases may be SLIGHTLY DARKER d/t increased ACTH. Immediate diagnosis with male is not as likely as with a female.
Term
How common is CAH?
Definition
very rare, 1 in 12,000-15,000 births.
Term
There are 3 kinds of CAH.  What is the classic kind?
Definition
Classic salt wasting form. These kids are the most severely effected. Females present with ambiguous genitalia at birth. Both males & females will present with a salt-losing adrenal crisis in the 1st few weeks of life d/t the absence of both the mineralcorticoids and cortisol. ABNORMAL ALDOSTERONE & CORTISOL in SALT WASTING FORM of CAH.
Term
What other symptoms happen to kids who have the classic salt wasting form of CAH?
Definition
Symptoms of Hypovolemic Shock d/t excessive Sodium losses in urine, dehydration, and elevated Potassium will occur.
Term
The 2nd kind of CAH is the Classical NON-salt wasting.  What is this?
Definition
Females present with a varying degree of genital virilization. Both males and females do not manifest the salt-losing problems as they have normal aldosterone.
Term
The NONCLASSICAL form of CAH is
Definition
the less severely affected. There are variable manifestations. Girls do not have ambiguous genitalia.
Term
How are children with CAH diagnosed?
Definition
The females at birth may be misdiagnosed as a male with undescended testicles and hypospadius or a small penis. All cases of ambiguous genitalia need to be followed up early to prevent problems with mistaken gender. (they need FOLLOW-UP!) Need an ultrasound or x-ray to see internal organs present b/c they will tell immediately. The chromosomes will be that of a normal female - it is not a chromosomal genetic disorder.
Term
Children suspected of CAH need a 24 hour urine to test presence of what?  
Definition
17-ketosteroid levels (testosterone)
Term
When is the appearance of acute adrenal insufficiency apparent?
Definition
2nd through the 7th week of life
Term
Children who have type 2 or 3 may go undiagnosed for years though.  When might they be diagnosed?  What triggers adrenal insufficiency episodes for them?
Definition
They can't adjust to stressful situations. STRESS triggers adrenal insufficiency episodes.
Term
What replacement is necessary for CAH & how long?
Definition
Hydrocortisone (Cortisol) replacement tablets. Just replace what's missing - pretty easy, but it is FOR LIFE. All of these are for life, except GH.
Term
What does replacement of hydrocortisone (Cortisol) do?
Definition
causes suppression of ACTH by the pituitary, Androgen production is decreased, virilization is halted.
Term
We know that lifelong replacement of hydrocortisone (Cortisol) is necessary for CAH, but do they need to increase their dose sometimes?  when?
Definition
Yes, increase dose during periods of stress, surgery, infection or injury.
Term
Salt wasting type of CAH needs __________ replaced also.  what is the med?  Also needs an injectable form of cortisone (ie Solu-Cortef) on hand if child is vomiting and can't keep med down or when experiencing severe stress.  
Definition
aldosterone. Florinef Acetate - orally once a day.
Term
Children with salt wasting CAH....  do they need their salt restricted, increased, what?
Definition
INCREASE in dietary salt (b/c they are wasting it away). Also recommend a MEDIC ALERT BRACELET.
Term
If no replacement medications are given then _____________ of the female will continue.
Definition
masculinization (like pubic hair, deeper voice, enlarging clitoris, facial hair, temporal baldness, no breasts, no menstruation, no fertility.) BOTH males & females have PREMATURE EPIPHYSEAL CLOSURE - DWARFED ADULTS ABOUT 3'6 in height.
Term
What happens to males if they are not treated for CAH?
Definition
Penis hypertrophies between 18 months & 5 years and secondary sex characteristics will appear. Rapid growth, voice deepening, acne, pubic & auxilliary hair and muscle development will occur. Gonads will fail to develop and child will be STERILE as an adult if no treatment.
Term
One nursing intervention is gender assignment.  What sex is usually chosen?
Definition
usually raised as females d/t the fact their internal structures are intact. The parents should be fully advised of situation if there is a question of gender at birth. Nurses can help them understand the diagnosis & explain to family members. They will need continued support & guidance, as you would expect...
Term
Surgical treatment involves -->
Definition
recession (size reduction) of the clitoris, separation of the labia, creation of vaginal orifice - plastic surgery pretty much to make girls more classic in genitalia. Surgery is best done before 17 months of age. Often done in stages & will need several surgeries to correct & revise.
Term
The parents of a CAH child need... 
Definition
genetic counseling. The affected child is going to need counseling too.
Term
What is the most common endocrine disorder in children?
Definition
Diabetes
Term
When is the peak incidence of diabetes discovery in children?
Definition
10-15 years old, but can occur at any age usually before 20 years old.
Term
Diabetes is most common to what ethnicity?
Definition
caucasians
Term
How is diabetes Type 1 characterized from Type 2?
Definition
Type 1 diabetes has an absolute insulin deficiency
Term
Type 1 diabetes is an AUTOIMMUNE disorder that causes beta cell ___________.
Definition
destruction
Term
What are the 2 main factors behind Type 1 diabetes?
Definition
1. Genetic predisposition 2. Environmental factors such as viral illness, toxin, cows milk can trigger the autoimmune response
Term
Type 1 diabetes is associated with ___ antigens.
Definition
HLA
Term
Is Type 2 increasing in children?
Definition
Yes it increasing in children - especially adolescents
Term
The majority of children who have type 2 diabetes have a ________ with the disease. 
Definition
1st or 2nd degree RELATIVE. It is GENETIC
Term
What ethnicities are presdisposed to Type 2 diabetes?
Definition
African Americans #1, American Indians, Eskimos
Term
What environmental  factors contribute to Type 2 obesity?
Definition
inactivity and diets high in fat and refined carbohydrates contribute to the problem
Term
What are the clinical S/S of Type 1 diabetes?
Definition
Polyuria (excessive urination), Polydipsia (excessive thirst), Polyphagia (excessive hunger). For children, big signs are: ENURESIS (BED WETTING) & NOCTURIA (WAKING IN THE NIGHT TO PASS URINE)
Term
What are other s/s of type 1 diabetes?
Definition
weight loss, ketonuria (there is not enough carbs to break down so fat is broken down to get glucose), hyperglycemia, fatigue, irritability
Term
What are the s/s of Type 2 diabetes?
Definition
insidious (SLOW) onset, overweight, may have 3 P's, Weakness, Fatigue, May have FREQUENT INFECTIONS d/t yeast (VAGINAL and UTI).
Term
Type 2 diabetes is characterized by Insulin ___________.
Definition
resistance
Term
Type 2 diabetes is insulin resistant and often occurs with:
Definition
Hypertension, Elevated LDL & triglycerides, acanthosis Nigricans (darkening at the folds like back of knees & arms only happens w/ Type 2), Polycystic Ovary Syndrome (PCOS) is very common with Type 2.
Term
Treatment for Type 2 diabetes focuses on:
Definition
nutrition & increased physical activity. Treatment plan should address the life style and risk factors of the ENTIRE family to be effective, blood sugar monitoring.
Term
_________ is the only oral diabetic agent approved for youths.  Insulin may also be used, but are phased off once lose wt & diet control happens.  
Definition
METFORMIN
Term
To diagnose both Type 1 & 2, a BG greater than or equal to ____ mg/dl on 2 occasions indicates DM.
Definition
126. (This is the preferred method to diagnose)
Term
Other methods to diagnose both types of diabetes are:
Definition
1. casual (random) plasma glucose 200 mg/dl+ w/ symptoms (3 P's & weight loss). 2. Oral Glucose Tolerance Test (OGTT). Remember, preferred method is a BG of 126 mg/dl+ on 2 occasions indicates DM
Term
If you lower your BG, you lower your risk of _____________.
Definition
complications
Term
What health professionals should be involved with a Diabetic peds pt?  (recommended by Amer Diabetes Assoc)
Definition
MULTIDISCIPLINARY TEAM APPROACH: Ped endocrinologist, diabetic nurse educator, dietitian, mental health professional, exercise physiologist, client & family who are an integral part of the team.
Term
Infants desired BG levels:
Definition
100-200 levels are allowed
Term
What should a toddlers BG be before meals?  at bedtime?
Definition
100-180 before meals. 110-200 at bedtime.
Term
HgBA1c of a toddler/preschooler should be between:
Definition
7.5%-8.5% (it's checked every 3 months & tells average of BG over last 90 days)
Term
School age child should have what BG before meals?  Bedtime?
Definition
before meal=90-180. Bedtime=100-180. A1c less than 7.5%.
Term
Adolescents & young adults should have a BG of what before meals?  at bedtime?  what about A1c?
Definition
before=90-130. Bedtime=90-150. A1c=less than 7.5%
Term
Why do children have higher rates for BG levels?
Definition
Kids have HIGHER BG RATES D/T THEIR VULNERABILITY TO HYPOGLYCEMIA.
Term
How often should kids be checking BG?
Definition
at least 4 times a day & often more frequently.
Term
What is the Honeymoon Period experienced often by Type 1 diabetics after the initiation of insulin therapy?
Definition
80-90% of beta cells are destroyed & the other 10% go on hiatus. When given insulin, the remaining cells work HARD. Honeymoon lasts 2 weeks to 2 years. This is VERY confusing for patients. Nurses must educate!!! Unfortunately it does NOT mean their diabetes is going away. Just getting a little extra help & eventually they'll be back to 4-5 injections/day probably.
Term
During the honeymoon period, how much insulin is given?  Is beta cell destruction continuing?
Definition
Continue to give low doses of insulin. The remaining beta cells are releasing insulin for a variable period of time, but the Beta cell destruction is continuing.
Term
Rapid acting Insulins used for children (lispro, aspart, glulisine) - are the most common insulins.  Tell about these..
Definition
BOLUS or MEALTIME INSULINS. Onset of action is 5-15 mins. Peak is 30-90 mins & the duration is 4-5 hours. Don't have to wait to eat. Brand names are: Humalog, Novolog, Apidra.
Term
What are the short acting insulins given to children?
Definition
REGULAR insulin. Onset 30-90 minutes. Peak 2-4 hours. Duration 5-7 hours. Brand names = Humulin R & Novolin R. (This one you take the shot & wait 30 mins to eat - why we've gotten away from using it as much..)
Term
Intermediate acting (basal insulin) is:
Definition
NPH. (not used as much d/t night time drop). Onset 1-2 hours. Peak action: 6-14 hours. Duration: 16-20 hours. Brand name= Humulin N
Term
Long Action (basal insulin) is
Definition
Insulin glargine, determir. Onset 90 mins, Peakless, Duration 24 hours. Brand name: Lantus and Levemir (bedtime injection). we DO NOT MIX this with other insulins!!!
Term
insulin may be given in many methods.  One is:
Definition
Subq at least 2x/day w/ 2 types of insulin Regular and NPH.
Term
Having tight control involves  MULTIPLE DOSE INJECTIONS of 3 or more a day to cover food intake.  Often it is:
Definition
Lispro to cover meals and Lantus at bedtime for basal control for 24 hours.
Term
To decrease the number of injections, an ________ _______ is available
Definition
insulin pump
Term
How does an insulin pump work?
Definition
allows for more physiologic insulin delivery than injections. It releases small amount of insulin 24 hrs/day. When eat? Bolus in/calculate the amount of insulin necessary for a meal.
Term
What are some advantages of the insulin pump?
Definition
More flexible meal & snack times, decreased amount of injections required, improved A1c levels.
Term
Insulin pumps allow for a continuous baseline infusion with intermittent boluses as determined by the user.  Boluses are determined by:
Definition
blood sugar, activity and diet. Blood sugar must be monitored 5-6 times a day still. Insulin pump is the size of a pager and worn on a belt. Medication is delivered by a small catheter in subcutaneous tissue.
Term
How often should the insulin pump insertion sites be changed?  It can't be removed for how long?  how much does an insulin pump cost?
Definition
change site every 48-72 hours, It can't be removed for more than 1 hour a day, expensive =$6000/yr or $120/month. Luckily, most insurance companies will cover now.
Term
A complication that occurs with a persons weight when starting on an insulin pump is:
Definition
can see a huge weight gain b/c all of a sudden they can eat... something HC worries about. Generally, though, therapy is managed much better for most who are in control.
Term
What is a key component to educate parents about diabetes?
Definition
There must be a coordination of food intake with insulin action. <-- key component
Term
There should be a consistent number of calories to meet exogenous ______ requirements.  
Definition
insulin - (to not become hypoglycemic)
Term
___________ counting is necessary for diabetics bc they are the main foods that raise blood sugar no matter what the source is.
Definition
carbohydrate
Term
So is 15 Grams of carbs from one source (fruits) the same as 15 Grams of carbs from another (potatoes)?
Definition
Yes - a dietician will help establish total grams of carbs to eat at meal's and snacks.
Term
One serving of starch, fruit or milk is equal to __-__ grams of carbs.  Are veggies counted?
Definition
12-15. No, vegetables are not counted (except potatoes, corn & peas are counted)
Term
Are fats & proteins recommended in a diabetic diet?
Definition
Yes, a reasonable amount of both are recommended. No foods are off limits anymore for Type 1.
Term
What is recommended about concentrated sweets for diabetics?
Definition
They are discouraged, but not prohibited.
Term
15 grams of carbohydrates = __ carb.
Definition
ONE. (some diabetics use carbs and some use grams of carbohydrates). Calculating the insulin/carb ratio to figure out the insulin needed is done next.
Term
what does exercise do for diabetics?
Definition
exercise & being physically active helps the cells utilize BG more effectively.
Term
Exercise _______ blood sugar & helps insulin to enter the cell more readily.  Therefore the insulin requirement is _________.
Definition
lowers, reduced
Term
Someone who is going to exercise, should do what?
Definition
they should always cary a carb source to treat hypoglycemia or take before exercise to prevent it from happening. MUST HAVE A RAPID ACTING GLUCOSE ON BODY AT ALL TIMES!
Term
When is exercise NOT recommended for a diabetic?
Definition
When BG is greater than 240 & ketones are present. When diabetes is that poorly controlled - do not exercise until control is achieved.
Term
Where should insulin injection go if you are going running?  what about lifting weights?
Definition
The injection should not be put in the limb that is being exercised. For example, do not give in leg if going for a run b/c it will metabolize much faster.
Term
After activity has taken place, when might delayed hypoglycemia happen?  what should be done?
Definition
Glucose may drop 6-12 hours after exercise. So check blood glucose at bedtime and/or during the night. Need to check these kids in the middle of the night b/c of DELAYED HYPOGLYCEMIA... it might not really drop for awhile after the activity.
Term
What are the causes of hypoglycemia?
Definition
Overdose of insulin, omitting or eating less food,overexertion without food on board, nutritional and fluid imbalance (N/V), alcohol intake. <-- recommend to eat whenever drinking alcohol b/c hypoglycemia occurs.
Term
Some mild s/s of hypoglycemia?
Definition
sweating, pallor, nervous, palpitations, tremulousness, hunger, cold, clammy
Term
some moderate s/s of hypoglyemia...
Definition
weakness, dizziness, headache, drowsiness, irritable, loss of coordination, disorientation, slurring words (classic signs of a drunk person - wear a med alert bracelet b/c cops have thought they were drunk!)
Term
severe s/s of hypoglycemia...
Definition
seizures, semi comatose or coma, slurred speech, staggering gait, combative, confused. An EMERGENCY INJECTION OF GLUCAGON is necessary! They need an IM INJECTION by someone else!
Term
How should mild to moderate symptoms of hypoglycemia be treated?
Definition
1. 10-15 grams of simple carbohydrates (Give them orange juice, soda, milk, gummi bears. not chocolate b/c it takes too long.) 2. Follow with a complex carbohydrate (give more fat & protein to maintain BG until a meal. 3. retest blood sugar in 15-30 minutes.
Term
How should a severe hypoglycemic rxn be treated?  
Definition
Administer Glucagon asap & call 911. 911 must be called so person can get 50% IV glucose to keep their BG up.
Term
Why might HYPERglycemia occur?
Definition
Too little insulin given or dose missed, too much food eaten, stress and stressful events (an illness/surgery, emotional upsets**), increase in growth hormone with puberty. (Natural increase of insulin dosage with puberty).
Term
Emotional stress causes ____glycemia.
Definition
hyper
Term
long term diabetes will cause the s/s of hyperglycemia to...
Definition
be decreased. Having diabetes long term will mask the s/s of hyperglycemia.
Term
Classic signs of Hyperglycemia with diagnosis are:
Definition
gradual onset of: lethargy,weakness, the 3 P's, N/V, abd pain, dehydration, warm & flushed
Term
When might children need a major readjustment of insulin dosages?
Definition
puberty d/t all the hormones being released and causing insulin resistance.
Term
What is diabetic ketoacidosis?
Definition
blood sugars above 240, pH less than 7.3. Can be fatal & is very serious. Often the first indication of diabetes is when this occurs & child ends up at hospital in full blown ketoacidosis. A person can go back into Diabetic Ketoacidosis at anytime.
Term
What are s/s of diabetic ketoacidosis?
Definition
ketosis, ketonuria, impaired consciousness, fruity odor to breath, kussmaul's respirations (rapid & shallow), dehydration, electrolyte imbalances
Term
Stress influences blood sugar!!!  what should a parent do if diabetic child is sick?
Definition
monitor BG every 2-4 hours, do NOT STOP taking insulin doses during sickness, monitor urine for ketones (with strip) every 2-4 hours, Maintain hydration: if able to tolerate have them take soft bland foods and 8 oz of fluid per hour. Notify HC practitioner if vomiting and BS is above 240.
Term
Infancy (0-12 months) is a very difficult time for diabetes diagnosis.  
Definition
The s/s of hypoglycemia can't be picked up easily & the parents need a ton of emotional support b/c giving 5-7 injections per day to your infant is really hard. Child needs support from parents. leads to TRUST ISSUES.
Term
Toddlerhood (12-36 months) is also a tough time for a diabetes diagnosis.  
Definition
Period of AUTONOMY vs. SHAME & DOUBT. Diagnosis is very elusive in this age group too (might not notice any increase in urinary out put). The child will fear the pain of injections. They already have finicky eating patterns & appetites. Toilet training issues & early symptoms can be confused. It is really hard to correctly identify and treat hypoglycemic episodes. Child will have tantrums and discipline is hard. Recommend that parents have normal rules/discipline.
Term
Preschoolers (3-6 years) diagnosed with diabetes:
Definition
INITIATIVE vs. GUILT - may think they're guilty. Say, "what'd I do wrong mommy?" Child may interpret diagnosis as punishment for real or imagined offenses. May suffer a set-back in self-esteem that needs positive reinforcement. They fear injections (which are usually after meals), parents will need support, they have unpredictable appetites & food intake and hypoglyemic episodes. It's hard to find child care willing to control BG, but it is necessary the babysitter knows how to effectively manage when parent is unable to.
Term
During preschool they are gaining confidence in their ability to master tasks but still do not have the coordination and cognitive development to perform most diabetic tasks.  Parents should:
Definition
involve diabetic child in activities as much as possible... give the young child the power/control!
Term
School Age (7-11)
Definition
INDUSTRY VS. INFERIORITY. The school age group is capable of taking an active role in his own care with adult supervision. They still need the parental support, but should share responsibilities. This age group still has dietary issues. The diabetes regiment needs to be FLEXIBLE to allow participation in school, sport, and peer activities.
Term
School age kids need to know they are kids first & diabetics second.  Term used is:
Definition
Person with Diabetes. (we don't say "diabetic". should not be defined by the disease.)
Term
Adolescence (12-15)
Definition
IDENTITY VS. IDENTITY DIFFUSION. There are rapid biological changes (that change insulin needs). They have body image concerns & do not want to wear an insulin pump. Behavioral experimentation happens in adolescence, loss of control & coping skills.
Term
The cornerstone with diabetes is ____________.
Definition
EDUCATION. It is most important! we must turn the power & control to child & family... to manage themselves.
Term
Late Adolescence (16-19)
Definition
Begin to Integrate diabetes into new lifestyle... need help transitioning into adult world, assure insurance. Need family support to transition to independence. Learn coping skills to enhance his ability for self-management. (Want to help avoid risky behaviors... alcohol, drug use, pregnancy - really need to control d/t many being born early or still birth.)
Term
What is a protrusion of the bowel into the groin region that causes an OBESRVABLE & PALPABLE bulge in that area?
Definition
INGUINAL HERNIA
Term
Inguinal hernias make up ___ of hernias & are more common in ____.
Definition
80%, males
Term
How are inguinal hernias managed?
Definition
prompt elective surgery to avoid incarceration (intestinal obstruction, strangulation, GANGRENE). Usually there is bilateral involvement. This is an outpatient surgery usually.
Term
Before surgery, nursing care for inguinal hernias involves...
Definition
Encourage the parents to watch the child for reddness, swelling, inability to reduce the hernia (going to basically be pushing it back in until surgery can happen). Want to monitor the infant & reduce crying or straining.
Term
Monitor the older child and reduce:
Definition
straining, coughing, standing for a long time. Want to keep all kids with inguinal hernias as calm as possible. No straining during BM's. Want to decrease pressure on the abdominal area.
Term
After operation on inguinal hernia, the goal is to...
Definition
PREVENT INFECTION. Going to sponge bathe for 2-5 days, do frequent diaper changes on infants, and promote comfort (give Acetaminophen (Tylenol).
Term
What are post op activity restrictions for inguinal hernia surgery?
Definition
INFANTS & TODDLERS = no restrictions. OLDER CHILDREN = restrict heavy lifting, pushing, wrestling, fighting, bike riding and athletics for 3 weeks. SCHOOL CHILDREN = go to school when can, but NO PE for 3 weeks.
Term
what is a fluctuant mass of fluid within the processus vaginalis that presents as scrotal swelling?
Definition
Hydrocele (if you back light it: can see fluid through & it's clear fluid, not a mass)
Term
What is a NONCOMMUNICATING hydrocele?
Definition
peritoneal fluid is trapped in the testicular tunica vaginalis during closure of the processus vaginalis. The fluid is gradually reabsorbed. Usually NO treatment is necessary.
Term
What is a COMMUNICATING hydrocele?
Definition
The processus vaginalis remains OPEN! So you're getting different amt's of fluid at diff times... very variable amt of fluid down there. because it did NOT close.
Term
A _____________ hydrocele is going to have changes in intra-abdominal pressure & gravity.  There will be force of peritoneal fluid into processus vaginalis & scrotal swelling of variable size.  
Definition
communicating
Term
A hydrocele will have transillumination.  whats that?
Definition
shows the fluid if back lit. lights up like a light bulb.
Term
Communicating hydroceles predispose the child to a _____.
Definition
hernia
Term
Surgical repair of the hydrocele is necessary if the following present:
Definition
a large, symptomatic hydrocele. remains after 3 months of age. hernia.
Term
Surgery for a hydrocele is best done by what age?
Definition
best done by 1 year of age (sooner if hernia is present). Surgery is usually an outpatient basis.
Term
What is normal to expect after surgery for hydrocele?
Definition
normal to have swelling & discoloration, will resolve spontaneously within 10 days.
Term
What is failure of one or both testes to descend into the scrotal sac?
Definition
Cryptochidism
Term
Who has much higher rates of cryptochidism?
Definition
Premature infants. Because the tests normally descend during the 8th month of gestation.
Term
What is Retractile Testes?
Definition
They have normally descended testes, but a hyperactive "cremasteric" reflex pulls the testes back into the inguinal canal. If you stroke the inside of thigh it is sensitive to stimulation/touch & cold, usually bilaterally. The testes normally retract, but this should go away at about 6 months. They can be "milked and brought back down into scrotum.
Term
What is done first for Cryptorchidism?
Definition
hormone therapy - to stimulate testicular descent
Term
What other methods are done for Cryptorchidism?
Definition
Luteneizing Hormone (LH) Nasal Spray or Human Chorionic Gonadotropin (hCG) Injection
Term
What is done if hormone therapy does not work for Cryptorchidism?
Definition
Sugery
Term
What is the surgery for Cryptorchidism called?
Definition
Orchiopexy. It surgically places testes in scrotum. Should be done if testes have not descended by one year of age. It is usually an outpatient surgery.
Term
When is the best time for Orchiopexy (surgery for Cryptorchidism) ? 
Definition
best time psychologically is between 1-2 years of age. Earlier if hernia is present.
Term
There are physical risks if the surgery is not done early... like what?
Definition
1. REDUCED FERTILITY (higher degree of body heat in abd damages the testes) 2. TESTICULAR MALIGNANCY (increased 20-40%, usually occurs at 30-40 years old. If untreated by 2 yrs of age? risks increase the longer the testes are in that area. Greatest risk if untreated or surgery is not done during or after puberty.
Term
After a pt has surgery to descend the testicles (orchiopexy), what nursing care is necessary?
Definition
1. PREVENT INFECTION - keep operative site free of stool & urine 2. PROMOTE COMFORT - pain med as needed 3. TEACH SELF EXAM OF TESTES to older child 4. TEACH IMPORTANCE OF LONG TERM FOLLOW UPS - for tumors & infertility.
Term
What is the congenital anomaly in which the urethral opening is located along the ventral surface (underside) of the penile shaft?
Definition
HYPOSPADIAS
Term
There is an increased risk for hypospadius if...
Definition
father or sibling has hypospadius (it's congenital)
Term
should boys born with hypospadius be circumcised as usual?
Definition
NO, they are NOT circumcised b/c the foreskin may be used in surgical reconstruction.
Term
When is the best time for surgery on hypospadius?  It may be a single or a multi stage surgery, just depends.
Definition
before 18 months of age
Term
What are potential areas of repair for hypospadius?
Definition
correction of chordee, straightening of penis, construction of new urethra.
Term
What is done for urination while hypospadius surgery heals?
Definition
urinary diversion - allows healing of the urethra. may have a suprapubic catheter.
Term
The goals of surgery for hypospadius are:
Definition
to make urinary & sexual function as normal as possible. To improve the cosmetic appearance of the genitalia.
Term
After surg for hypospadius nursing care involves caring for the drainage system... what's this mean?
Definition
Nurse may need to prevent disruption (use restraints possibly), tape catheter to prevent any tension, assure no clamping or kinking of the catheter tube & maintain patency.
Term
A UTI can change to sepsis in an infant.  Nursing care to reduce the risk of infection after hypospadius surg is very imp.  what must be done?
Definition
Give PROPHYLACTIC ORAL ANTIBIOTICS prior to the surgery, going to give urine acidifying agent (like ascorbic acid) b/c increasing acidity will decrease infection (want a low pH). Finally, put antibiotic ointment on the urethral meatus.
Term
Nursing care after hypospadius involves using a COMPRESSION DRESSING TO THE PENIS.  What for?
Definition
The compression dressing will Reduce Edema & Reduce Bruising.
Term
It is very important to CONTROL BLADDER SPASMS following hypospadius surgery.  what does nurse give for this?
Definition
Ditropan & Belladonna - an opium suppository.
Term
Older children who have hypospadius surgery need to have ...
Definition
their activity restricted post-op.
Term
What discharge planning is taught after hypospadius surgery?
Definition
care of the urinary drainage system. teach the parents how to maintain & allow child to be at home. ENCOURAGE FLUIDS. avoid activities like: rough play, swimming, contact sports, straddle type toys, sandboxes.... until the area is healed. Need to ensure the tubing doesn't get dirty or infected too.
Term
What is a congenital anomaly in which the urethra is located on the dorsal surface (topside) of the penile shaft?
Definition
EPISPADIAS (This is much more significant & doesn't happen in normal child... there is usually other issues happening)
Term
What is a MILD vs a SEVERE Epispadias?
Definition
Mild: meatus located in front of the glans penis. Severe: extends to exstrophy of the bladder. Surgical intervention depends on the degree of the involvement.
Term
What is bladder exstrophy? (occurs in severe epispadias)
Definition
a CONGENITAL anomaly characterized by the extrusion of the urinary bladder to the outside of the body through a defect in the lower abd wall. (there is a higher incidence of this in males)
Term
How is bladder exstrophy managed?
Definition
a series of staged reconstructions. Initially they close the abd defect within the first few hours/days of life (surg asap!). Subsequently, they will do an epispadias repair, bladder neck reconstruction, creation of urethral sphincter meachanism (usually there is not a sphincter), genitalia reconstruction. URINARY DIVERSION MAY BE PERMANENT.
Term
Parents who have a child with bladder exstrophy need help to promote their bonding.  What can a nurse do?
Definition
assist the parent to bathe, feed, diaper, etc... support the parents b/c they are grieving the loss of a "perfect child"
Term
What should a nurse do prior to surgery for a bladder exstrophy?
Definition
PREVENT INFECTION by covering the defect with a sterile nonadherent dressing. Need to protect the exposed bladder tissue. Practically covering the area with clear plastic wrap or thin film dressing without adhesive. Do NOT USE PETROLEUM JELLY!!! Keep the surrounding area clean & protect from dribbling urine.
Term
What does a nurse do after surgery?
Definition
routine post-surg repair, wound care, monitor urine output & appearance, comfort measures
Term
__________ Syndrome is a clinical state characterized by abnormal increase in the permeability of the glomerulus to plasma protein.  
Definition
NEPHROTIC.
Term
In nephrotic syndrome, the glomeruli is more permeable to plasma _________.
Definition
proteins
Term
The primary group of Nephrotic Syndrome includes the most common type in children (80%).  It is Idiopathic - no known cause.  What is it called?
Definition
Minimal Change Nephrotic Syndrome (MCNS)
Term
Why does MCNS happen?
Definition
It is idiopathic - no known cause - know that! Know it is Primary too!
Term
Secondary reasons for Nephrotic Syndrome:
Definition
Systemic Disease, Drugs, Toxins (3 causes)
Term
Nephrotic Syndrome can be congenital, but it is very RARE for it to be congenital.  
Definition
It is autosomal recessive, managed by dialysis & transplants, but death is common in the first 2 years of age.
Term
The glomerular membrane is normally impermeable to large proteins, but in Nephrotic Syndrome the glomerulus becomes permeable to protein, especially _________.
Definition
albumin
Term
Where are all the proteins found in Nephrotic Syndrome?
Definition
In the URINE (hyperalbuminuria!!!), but the serum albumin is really low (hypoalbuminemia). There are fluid shifts from plasma to interstitial spaces (causing HYPOVOLEMIA & ASCITES - fluid settles in the intraabdominal area).
Term
UA of nephrotic syndrome would show:
Definition
an increase in albumin in urine, but a decrease in serum albumin. also causes fluid shifts = ascites in abdomen.
Term
What are the clinical s/s of nephrotic syndrome?
Definition
Massive proteinuria, Hypoalbuminemia (low serum albumin), Edema (periorbital, ascites, labial or scrotal, lower extremities). swelling in groing & feet/ankles. There is HYPERLIPIDEMIA, weight gain, diarrhea, anorexia, difficulty breathing, skin breakdown on feet/ankles (wherever fluid sits), skin pallor, malnutrition, lethargy, irritability, depression, DARK/FROTHY URINE.
Term
There is often a recent history of an ______ with nephrotic syndrome.
Definition
Upper Respiratory Infection. (d/t increased fluid volume in lungs)
Term
Other common infections with nephrotic syndrome are:
Definition
cellulitis, pneumonia, peritonitis, sepsis. Pneumonia happens b/c there is a decease in kidney functioning, but an increase of fluid in lungs = pneumonia cycle.
Term
Causes of infection in nephrotic syndrome?
Definition
Edema, steroids, immunosuppressive therapy, loss of immunoglobulins, poor nutrition.
Term
What is the URINE like in Nephrotic Syndrome?
Definition
Decreased urine volume (amount), but the concentration of the urine is really increased (why it's dark & frothy). There is MASSIVE proteinuria. and MICROSCOPIC HEMATURIA (blood in urine is very common). The specific gravity is increased.
Term
The objectives of treatment for nephrotic syndrome are:
Definition
to decrease the urinary protein loss, balance nutrition, restore normal metabolic function, prevent or treat any function.
Term
What nursing care is important in Nephrotic Syndrome?
Definition
MEDICATIONS are #1. Rest is what is done in hosp anyway: monitor I & O, nutrition/fluid management, skin care, prevention of infection, activity, respiratory care, socialization/emotional support.
Term
What is the PRIMARY THERAPEUTIC AGENT used for Nephrotic Syndrome?
Definition
Prednisone (a steroid)
Term
The goals of prednisone use:
Definition
to reduce excretion of urinary proteins and promote excretion of edema fluid.
Term
Diuresis usually occurs between days __ & ___. 
Definition
7 & 21.
Term
 Continue diuresis until urine is free of protein & remains so - this takes.... 
Definition
10 days to 2 weeks.
Term
Should Prednisone be stopped right when diuresis is over?
Definition
No, The dosage needs to be TAPERED for weeks to months.
Term
What are the side effects of using Prednisone?
Definition
moon face, buffalo hump, hirsutism, increased appetite, gastric ulcer/bleeding, HTN, susceptible to infection, growth retardation.
Term
Relapse of Nephrotic Syndrome happens to 85% of kids with MCNS.  Though the tendency for relapse decreases with time (good news)...  How can parents prevent this?
Definition
Teach parent to monitor with urine dipstick (just do normal UA's, can get a target). If the child is having Proteinuria of 2+ or more, for 3 consecutive days = concern for relapse. Other s/s to watch are: increased edema, weight gain, fever.
Term
Nephrotic Syndrome kids need to be watched for FLUID MANAGEMENT!  how is this done?
Definition
REPORT LESS THAN 1 ml/kg/hr!!! monitor for edema, measure ABD GIRTH and WEIGH DAILY (same time & scale). A 'dry diaper' is a big sign if they are infants.
Term
BLOOD PRESSURE also needs to be monitored at least once per shift.  What is it usually?
Definition
NORMAL to SLIGHTLY DECREASED.
Term
Factors that promote HYPERTENSION in nephrotic syndrome kids are:
Definition
increased total body fluid volume & concurrent steroid therapy.
Term
What is the diet for nephrotic syndrome?
Definition
no added salt in diet & avoid high sodium foods (we are trying to get the fluids out) - no ramen noodles, no mcdonalds french fries... can go back to regular diet after awhile. Just NO SALT DURING RELAPSE or STEROID THERAPY. If they have severe edema? Will have further sodium restriction. They can go back to regular diet during remission.
Term
What is done to protect from skin breakdown in nephrotic syndrome?
Definition
change position every 1-2 hours, keep skin clean & dry, support edematous body parts.
Term
how is infection prevented for nephrotic syndrome?
Definition
meticulous handwashing, avoiding infectious persons, monitoring & reporting: fever, cough, sore throat, abd pain. Taking ANTIBIOTICS during the EDEMATOUS PHASE, Immunizations, reporting illness
Term
When does the Edematous stage happen?  What activity is allowed?
Definition
3-4 weeks after initial treatment. BEDREST
Term
What activity during remission is allowed?
Definition
no restrictions
Term
What is the prognosis for nephrotic syndrome?
Definition
it depends on response to steroid therapy, histology of the renal lesion. There is a VERY GOOD PROGNOSIS for most if MCNS. If the histologic pattern is not MCNS, there is a greater chance of progressive renal failure.
Term
What is acute glomerulonephritis?
Definition
an inflammation of the glomeruli which most commonly follows a GROUP A BETA HEMOLYTIC STREP INFECTION of the throat or skin.
Term
Acute Glomerulonephritis is typically due to:
Definition
STREP
Term
What are the clinical manifestations of acute glomerulonephritis?
Definition
they are variable, but GROSS HEMATURIA, HYPERTENSION, periorbital edema, anorexia, weight gain, decreased urine out.
Term
The S/S of acute glomerulonephritis are similar to nephrotic, but what is #1 with acute glomerulonephritis?
Definition
GROSS HEMATURIA & HYPERTENSION
Term
What complications happen with acute glomerulonephritis?
Definition
Hypertensive Encephalopathy is the primary one avoiding! (also there is acute cardiac decompensation & acute renal failure)
Term
what are the s/s thaHypertensive Encephalopathy is happening?
Definition
headache, vomiting, blurred vision, dizziness, restlessness, lethargy, disorientation, tachycardia, convulsions
Term
How long does the Edematous Phase in Acute Glomerulonephritis last?
Definition
usually 4-10 days
Term
What are the manifestations of the edematous phase?
Definition
listless, apathetic, anorexic, increased blood pressure, URINE IS CLOUDY, SMOKY BROWN, URINE OUTPUT IS DECREASED.
Term
The first signs of improvement in acute glomerulonephritis:
Definition
small increase in urine output& decrease in body weight.
Term
The ___________ phase usually starts 1-2 days after the first signs of improvement.  
Definition
Diuretic - causing copious diuresis
Term
Blood pressure management is #1 with acute glomerulonephritis.  What will happen to it during resolution ~2-3 weeks?  What else will decrease?
Definition
BP will decrease. Gross hematuria will disappear in a few weeks, but microscopic hematuria may persist for several months.
Term
Nursing care for acute glomerulonephritis involves:
Definition
fluid balance monitoring, monitoring for complications, preventing infection, medications, diet, activity.
Term
Meds given for acute glomerulonephritis are:
Definition
Diuretics, Antihypertensives, Antibiotics
Term
What is the diet for acute glomerulonephritis?
Definition
they have a POOR APPETITE affecting their intake. If child is uncomplicated: regular diet w/ no salt. If HTN or edema is starting to develop: moderate sodium restriction. Oliguria (when can't urinate? the cardiac compensation is increasing) it is necessary to RESTRICT POTASSIUM when no urine output. If severe Azotemia (nitrogen containing compounds like urea, creatinine) - then restrict protein.
Term
Uncomplicated child with acute glomerulonephritis' diet:
Definition
regular diet with no added salt
Term
Child with HTN or Edema's diet:
Definition
Moderate sodium restriction
Term
Child with Oliguria (when can't urinate, increase in cardiac decompensation):
Definition
restrict potassium
Term
Child with severe Azotemia (increased nitrogen compounds like urea or creatinine):
Definition
restrict protein
Term
What is the prognosis of APSGN (Acute poststreptococcal glomerulonephritis)?  reoccurence?
Definition
Usually runs its course in about one month. Reoccurence is unusual after the first 3 months. Most people completely recover & death is rare b/c it is easily treated. Give diuretics-> Antihypertensives-> increase fluids when in diuretic phase.
Term
What is a Urinary Tract Infection?
Definition
infection w/in the urinary tract affecting the bladder, urethra, ureters or collecting system of the kidneys.
Term
UTI's most commonly affect the _________.
Definition
urethra
Term
A UTI affecting the: urethritis, cystitis, or prostatitis.  is considered the _______ tract.
Definition
LOWER
Term
UTI affecting the: pyelonephitis or the VUR (vesicoureter reflex) is the ______ tract.
Definition
upper
Term
Common UTI's under 5 years old in girls? is often due to the..
Definition
VUR (vesicoureter reflex) so an UPPER tract infection
Term
Who is most at risk for UTI's?
Definition
Neonate/young infant boys, older infant to young adult (more common in females at this age) - peaks 2-6 yo if no anomaly. Also common in postmenopausal women & elderly.
Term
What is the most common cause of UTIs?
Definition
Gram Negative Bacilli -> E COLI!!! others are klebsiella pneumonia & proteus mirabilis. Also, Gram Positive cocci-> Staph.
Term
Hematogenous spread UTI's:
Definition
1. usually occur below 4 months of age. 2. Older-must have prior injury to urinary tract (obstruction of ureter, damage from stones, renal scars)
Term
Ascending Infection of UTI:
Definition
Usually only to those who are OVER 4 MONTHS OF AGE
Term
What are potential anatomic abnormalities that may cause UTI's?
Definition
anatomic or neurologic obstruction, nephrolithiasis (kidney stones), vesicouretral reflux (urine reflexes), noretractable foreskin, labial fusion
Term
What are S/S of a LOWER tract infection?
Definition
no fever, frequent urination (more than every 2 hours), suprapubic discomfort or pressure, uregency, dysuria (painful urination at the end of voiding often)
Term

What are S/S of a UPPER tract infection?

Definition
chills, FEVER, flank pain (even tap on area & you get pain
Term
What are the signs of a UTI in an infant/neonate?
Definition
VAGUE, NONSPECIFIC SYMPTOMS - (like FTT, difficulty feeding, irritability, diarrhea, persistent diaper rash). Fever without focus of infection? Suggests UTI. Need to get a urinary tract evaulation as part of sepsis work up.
Term
How do we screen for UTIs?  what would it show?
Definition
Simple UA. Urine dipstick would show positive leukocyte esterase, positive nitrite. Urine pH is alkaline (more than 7)
Term
a urine ________ is the GOLD STANDARD OF DIAGNOSIS FOR UTIs.
Definition
Culture (C&S is done to assure treating the correct bacteria as well)
Term
What is the most accurate way to get the urine for culture?
Definition
Catheterization or Suprapubic Aspiration. Small infants are catheteried often.
Term
Purpose of treating UTI?
Definition
eliminate the current infection, identify contributing factors to reduce the risk of recurrence, prevent systemic spread of infection, preserve renal function
Term
what is the purpose of treating UTIs?
Definition
to eliminate the current infection. To identify contributing factors to reduce the risk of recurrence (esp in young children b/c it's not real common), prevent systemic spread of infection, preserve renal function
Term
How do we treat complicated UTI's in children?
Definition
IV or IM antibiotics followed by oral
Term
How do we treat 'uncomplicated' UTI's in children?
Definition
TMP-SMX, (trimethaprim-sulfamethoxazole), Septra, Bactrim, nitrofurantoin, amoxicillin. These are OTC & effective in all age ranges. It elps pain asap & antibiotic will decrease ain in the next few days.
Term
What med relieves the pain of UTI?
Definition
Pyridium
Term
What follow-up is needed after meds?
Definition
need to get a monthly culture for 3 months, then --> every 3 months for 6 months.
Term
A 4 yo girl is seen in the outpt center w/ a  UTI.  She is started on Bactrim.  What teaching is important to discuss w/ the mom?
Definition
reporting the infection early is imp, proper hygiene (wipe front to back), avoid chemical irritants, decrease irritation from clothing (swimmers/divers need to watch), increase fluids & frequently void, if sexually active be sure to void after sex, avoid constipation.
Term
What is adequate fluid intake for a child?  what about an adult?
Definition
100 mL/kg for child. 2-3 L/day for an adult.
Term
What helps to acidify urine?
Definition
Vit C helps. A urine pH of 5 will decrease bacterial multiplication
Term
How does cranberry/blueberry juice help?
Definition
prevents bacteria from adhering to the bladder wall. Should have 2-3 glasses of juice per day. Need 4-6 weeks to work as a prevention strategy.
Term
Who is enuresis (bed wetting) more common in?
Definition
boys
Term
A mom comes to you & is very upset her 6 yo is still wetting the bed.  what do you say? 
Definition
Night time bed wetting until 6 years old is NOT a concern. (15% of five year olds do & 3% of 10 year olds)
Term
How can bed wetting be managed?
Definition
Enuresis alarm, Motivational Therapy, Bladder Stretching Techniques (increase fluids & teach to hold. Will expand the bladder & train child). Drug therapy is used if bad enough.
Term
What are some of the drugs given if enuresis gets bad enough?  A spasmodic bladder will cause it sometimes...
Definition
Imipramine, DDAVP, Ditropan
Term
What education is done for parents of a child struggling w/ bed wetting?
Definition
Limit Fluids, Discourage punitive approach (limit response to accidents and teach the child to participate). Observe for side effects of medications, Counseling if needed. esp if child is 10-12 years old, there are sleepover & they're not going type of thing.
Term
Iron Deficiency Anemia is a _________ __________ anemia.  It is brought about primarily by inadequate diet intake of iron necessary for normal RBC production.
Definition
HYPOCHROMIC, MICROCYTIC ANEMIA
Term
Children who are 6 mo's - 2 years old are diagnosed when their hemoglobin is less than ___ g/dL and hematocrit is less than ___%.
Definition
Hgb < ll g. Hct < 33%
Term
Children with Iron Deficiency anemia are diagnosed according to their labs ...
Definition
just know there are 3 levels of severity (mild, moderate & severe) of Iron Deficiency Anemia
Term
How does iron def anemia happen?
Definition
inadequate diet intake of iron (most common), iron malabsorption, low iron stores at birth, significant blood loss.
Term
What are the risk factors for iron def anemia in infants who are premature/low birth weight?
Definition
They have poor iron stores at birth. The maternal iron stores last approx 4 months, 50% of iron in maternal milk is absorbed, 10% of iron in cow's milk is absorbed (why we don't give milk until 1 year).
Term
What are the risk factors for iron def anemia in infants/children?
Definition
Rapid increase that is happening in their body size & blood volume has an effect. Usually insufficient iron rich formula & foods, they're having excessive intake of whole cow's milk which has less iron absorption. They may be breastfed without dietary iron. They're often very PICKY EATERS at this age. Eating a VEGETARIAN/VEGAN nonmeat diet in 2-5 year olds puts them at risk. Malnutrition.
Term
What is PICA?
Definition
eating disorder d/t anemia where they crave dirt, metal, nails d/t decreased iron intake.
Term
Why are adolescents at risk for iron def anemia?
Definition
rapid growth rate during puberty, increased muscle mass and circulating blood volume, unbalanced diet and poor eating habits, having menarche- esp if it is early and/or heavy menses.
Term
What factors put an adult at risk for iron def anemia?
Definition
premenopausal women, pregnant women, chronic blood loss, low socioeconomic background, older adult
Term
What are the S/S of Iron Deficiency Anemia?
Definition
Lethargy/fatigue, irritability, pallor, frequent infections, inability to concentrate, heavy menses
Term
Other iron def anemia findings:
Definition
conjunctiva in eyes may be pale, mucous membranes are pale, there are cardiac problems, devlopmental delays, weight is variable.
Term
Iron Def Anemia is ALWAYs checked between 9-12 mo's.  What else is checked if there is any concerns?
Definition
between 1-5 yo. Between 5-12 yo. Between 14-20 yo.
Term
Breast milk is highly recommended, but what should be done at 4-6 months?
Definition
Need to ADD iron supplement to breast milk. B/c breast milk has a low concentration of iron, the bioavailability is greater, and there is a progressive decline in iron content.
Term
Preterm infants need iron supplementation at __ months.
Definition
2
Term
Who needs iron supplementation by 6 months?
Definition
Infants that are EXCLUSIVELY BREASTFED
Term
Who else should receive iron supplements?
Definition
Infants getting formula that is not fortified with iron (it always should be!). and children with hemoglobin's below 11.
Term
MILK INTAKE RECOMMENDATIONS - Have formula + iron until 12 months.  WHOLE cow's milk after 12 mo's.  How many oz of milk should be given per day for 1-5 year olds.  This is sufficient to meet Ca++ needs...
Definition
24 oz/day
Term
Encouraging iron rich foods is always good... what is high & mod in iron?
Definition
high=red meat, oily fish. mod=beans, peas, fortified cereals, dark green vegetables, nuts.
Term
What foods decrease iron intake?
Definition
excess cows milk (more than 24 oz/day) and Tea
Term
All iron supplements are not the same.  Which ones are better absorbed?
Definition
ferrous sulfate
Term
What should NOT be taken with the iron supplement?
Definition
Do NOT consume with milk, antacids or tea
Term
What helps the absorption of Iron?
Definition
Ascorbic Acid (ORANGE JUICE HELPS!)
Term
What's recommended for taking liquid ferrous sulfate?  When should it be taken? 
Definition
Use a dropper or straw to avoid staining your teeth. Brush your teeth. Give between meals.
Term
When you start iron supplements, stools may normally be:
Definition
dark (greenish black) - warn families
Term
what are some sd effects of iron supplements?
Definition
n/v, diarrhea, constipation, anorexia. Can give iron with snack or small meal. ONLY give iron with meals if they have side effects.
Term
Iron therapy should return hemoglobin to normal within...
Definition
4-6 weeks. It's entirely possible to bring Hemoglobin up 1.0 g/dl or more in a month.
Term
Continue iron therapy for at least ___ months after Hgb levels return to normal
Definition
3
Term
However, iron therapy treatment should not exceed ___ months
Definition
five (except for older adults who may require life long supplementation of iron)
Term
What other ways can Iron be given?
Definition
parenteral iron. packed red blood cells.
Term
How does somebody get Sickle Cell Anemia?
Definition
Autosomal Recessive Inheritance
Term
With sickle cell anemia - there is an abnormal form of hemoglobin within the erythrocyte (RBC).  There is Hemoglobin ___ present when normally there is normal adult hemoglobin (Hgb A).
Definition
S
Term
Sickle cell causes - Chronic Hemolytic Anemia: RBC's only have a life span of __-__ days.  They normally live 90 days (3 months).  There is a chronic turnover of sickle Hgb & it is not lasting nearly as long as it should.  
Definition
10-20
Term
The genetic transmission of Sickle Cell Anemia is:
Definition
autosomal RECESSIVE. We know that both parents have a heterozygous trait. There is a 25% chance of having the disease, a 50% chance of being a carrier & a 25% change of having normal Hgb with each pregnancy.
Term
What nationality most commonly has sickle cell?
Definition
african american. (also in caribbean/mediterranean, arabian, india)
Term
In the American Black Population the disease is in 1 in 400 or 500 births.  The trait is in:
Definition
1 in 12 is a carrier. VERY HIGH!
Term
What is the basic defect of sickle cell anemia?
Definition
Normal hemoglobin: GLUTAMIC ACID in the 6th position of the beta chain Hgb A. Instead, VALINE replaces it -> Hgb S.
Term
what are sickle cell disease stressors?
Definition
Situations that promote Oxygen Deprivation. Like: hypoxia and acidosis. Vasooclusive crisis are the biggest problems with sickle cell. having frequent URI's (more likely to get hypoxic), there is an increased risk in winter. Environmental: low oxygen concentrations, weather. Stress: emotional & physical. It can be purely emotionally driven to have a crisis. STRESS AVOIDANCE is important for sickle cell. Although it can happen b/c of unknown causes too.
Term
What is the sickling process?
Definition
Hgb S + situations that cause oxygen deprivation (ie stressors). The sickled cells CLUMP TOGETHER (hard, sticky & can't flow through vessels). Small blood vessels become occluded -> Ischemia -> Necrosis -> Pain.
Term
Is Sickle Cell Anemia checked & apparent at birth?
Definition
No, sickle cell does not appear until 4-6 months of age b/c of the presence of FETAL HEMOGLOBIN. No problems until 6 mo's of age.
Term
One type of sickle crisis is: VASO-OCCLUSIVE crisis...
Definition
it is the MOST COMMON. There is a painful episode d/t clumping of RBC's, ischemia, infarction, pain. The biggest worry is CVA's... seen with repetitive crises.
Term
One type of sickle crisis is: SPLENIC SEQUESTRATION crisis...
Definition
The blood pools in the spleen. it's LIFE THREATENING. There is decreased intravascular blood volume --> resulting in shock. Going to need a Splenectomy if it's recurrent.
Term
One type of sickle crisis is: APLASTIC CRISIS...
Definition
a VIRAL INFECTION triggers it. RBC production stops -> profound anemia. This is usually self-limiting.
Term
Dactylitis is ---
Definition
pooling of sickle cells & inflammation in the fingers or toes. It is very painful.
Term
You are the nurse in an adult ER department.  A 24 year-old client with sickle cell disease comes in at least every 2 weeks c/o pain & requesting pain meds.  What do you think?  
Definition
Look at their hx... they're going to NEED a TON OF PAIN MEDS. Probably developed quite a bit of resistance. This is a situation where you need to trust your patient. Increase their dose of pain meds. Should talk to them about stress, dehydration (not taking drugs or excessively drinking).
Term
What are other s/s of sickle cell crisis?
Definition
Acute Chest Syndrome: sickling of small blood vessels of lungs, pain, temp is higher than 38.3 (101), cough, dyspnea, tachypnea, respiratory distress
Term
__________ is a major worry with sickle cell!  It can happen to an 8 yo w/ frequent crisis.  
Definition
Stroke (sickle cells block major cerebral blood vessels)
Term
What are S/S of Stroke to pay attn to in sickle cell child?
Definition
Severe headache, Slurred Speech, Unsteady Walk
Term
What is the leading cause of death for sickle cell kids (under 10 yrs old)?
Definition
INFECTION (Sepsis or Meningitis)
Term
Sickle cell disease causes an impairment of the spleen.  The spleen is replaced by fibrotic tissue.  This is /t infarcts in spleen (autosplenectomy).  There is a loss of the filtering system & this increases susceptibility of _________.  The spleen can even become nonfunctional by 5 years of age in kids.
Definition
infection
Term
What organisms cause infections under 5 yrs old?  what about over 5 yrs old?
Definition
<5=pneumococcal. >5=gram negative (E. COLI, Salmonella) are very dangerous. Remember, e. coli is the #1 cause of UTI's. Something as seemingly simple as a UTI in a menstruating female can cause a massive crisis. must be very careful!
Term
What are nursing interventions for sickle cell?
Definition
PAIN MGMT! Get it under control quick. Prevent/manage infection (need to start antibiotics asap), promote hydration, minimize tissue deoxygenation, minimize crises, drug therapy, patient/family education
Term
Sickle Cell Disease can cause SEVERE PAIN. (will give opioids parenterally, may be on PCA or continuous infusion).  However pain is very subjective & these pt's are used to pain.  What should the nurse get?
Definition
Their pain scales may be lower b/c of constant pain. a 4 may be high! It is important to get their tolerable level & achieve this before they go home.
Term
What is given for moderate & mild pain?
Definition
Acetaminophen & Ibuprofen for both (whatever works better for them)
Term
How else is pain managed?
Definition
assess pain Q 2-4 hrs (location, intensity, duration, character), Use pain intensity rating tool (monitor efectiveness of pain mgmt), use HEAT (not cold), REST (minimize O2 consumption), Hydration!!!
Term
What is done to prevent/manage infection in sickle cell pt's?
Definition
Need PROPHYLACTIC PENICILLIN (Pen VK) for pretty much any procedure. 3 mo's - 12 yrs of age. HANDWASHING is key. ISOLATION as appropriate. very imp to stay up to date on IMMUNIZATIONS (routine meningococcal, influenza, penumococcal (Prevnar, Pneumovax)
Term

Imp to teach the s/s of infection & to have them REPORT THEM EARLY.  What are the signs?

Definition
fever of 101 (38.3), lethargy, irritability, vomiting, diarrhea
Term
Sickle Cell Pt's need PROMPT TREATMENT with ...
Definition
parenteral antibiotics (e.g. ceftriaxone)
Term
It is SO important to teach & PROMOTE HYDRATION!!  Why?
Definition
Hemodilution is very imp to reduce blood viscosity & prevent sickling.
Term
IV + PO intake needs to be:
Definition
1 - 1.5 x maintenance
Term
Teach family:
Definition
Signs of Dehydration & to treat it promptly. Child unable to concentrate urine-low specific gravity leads to dehydration. Enuresis is common-do NOT decrease fluids for sickle cells kids!!!
Term
Teachers need to understand the child w/ sickle cell is going to need:
Definition
to be allowed to have water in class & permission to frequently use restroom.
Term
It is important to also MINIMIZE TISSUE DEOXYGENATION.  how?
Definition
Bed rest (very imp during acute crisis), avoiding stressors, monitor oxygen saturation (often go home with oxygen sats... if are hypoxic, <92%, give oxygen! Adminstering oxygen does not reverse sickling, but it may prevent more sickling from occuring.
Term
Need to MINIMIZE CRISES.  teach to:
Definition
avoid stressors, report s/s of crises immediately, frequent medical supervision, adequate nutrition, administer blood transfusion (real common especially with spleen problems). An acute spleen sequestration (corrects hypovolemia). In Aplastic crisis the packed RBCs will increase Hgb. Blood transfusions are also given Post CVa to reduce blood viscosity.
Term
Sickle Cell Disease kids must take prophylactic antibiotics, pain medications, like talked about.  Why are they also taking Folic Acid?
Definition
Increased Folate is required b/c of hemolysis. This prevents 'megaloblastic anemia'.
Term
A normal supplement that many sickle cell pt's take is ______________.  It is used with frequent pain episodes.  Elevates Hemoglobin F by decreasing sickling.
Definition
HYDROXYUREA
Term
Finally, people with sickle cell take ____________ to stimulate RBC production.
Definition
Erythropoietin
Term
What education is necessary for pt w/ sickle cell?
Definition
EDUCATE EARLY (12-13 yo) about getting pg & having child w/ sickle cell, encourage genetic counseling, educate about all aspects of the disease, encourage family to promote normal G & D, allow expression of feelings about the disease, Identify community and national resources.
Term
What is the prognosis for Sickle Cell pt's?
Definition
It's variable & depends on the severity. Men live until about 42 on average. Women live until about 48 on average. Improved survival is d/t: screening, follow-up & early intervention.
Term
What are the most common hereditary bleeding disorders?
Definition
Hemophilia, vonWillebrands's Disease
Term
Hemophilia & VonWillebrands's disease results from deficiencies abnormalities in specific _________ ___________.
Definition
coagulation proteins
Term
The coagulation cascade can be ridiculously confusing.  we just need to know... 
Definition
there can be deficiencies through out that cause problems
Term
Hemophilia A is a deficiency in Factor ____.
Definition
VIII (8)
Term
Hemophilia B is a deficiency in Factor ___
Definition
IX (9)
Term
VonWillbrands Disease is a deficiency of:
Definition
VonWillebrand's protein
Term
Hemophilia is genetically transmitted.  What chromosome is it on?
Definition
X-Linked Recessive Gene
Term
If the woman is a carrier & her partner does not have the disorder: what are the chances for children?
Definition
50% chance son will have the disorder. 50% chance daughter will be a carrier.
Term
The MILD form of hemophilia is the most common (5-40%).. what happens here & what needs to be restricted?
Definition
Have some restricted activities, still restricted, no contact sports. There is bleeding with severe trauma or surgery in mild.
Term

The MODERATE form of hemophilia affects 2-4.9%... what happens here & what needs to be restricted?

Definition
No Contact Sports (factors into activity), there is BLEEDING WITH TRAUMA
Term

The SEVERE form of hemophilia... what happens here & what needs to be restricted?

Definition
have very restricted activities. They get spontaneous bleeding without trauma. Just sit at home & get a nosebleed with nothing.
Term
Hemophilia pt's have the potential for bleeding:
Definition
anywhere in the body
Term
__________ is oozing of blood into the joint cavity.  The joints become sweollen, tender & painful.  Repeated hemorrhages can cause damage to synovial membrane, degeneration of cartilage & cysts, contractures, severe crippling is possible even.
Definition
Hemoarthrisis
Term
How can nurses help hemophilia?
Definition
administering the missing clotting factor, injury prevention, control bleeding, teaching
Term
If a person with Hemophilia ( no factor 8) gets an injury, what should be done asap?
Definition
administer the missing clotting factor asap w/ injury. There is a new recombinant factor that has no human product in it at all. The family should be taught how to admnister. The child should be taught to self adminster. An implanted venous access device may be necessary.
Term
What kind of injury prevention is done for infants w/ hemophilia?
Definition
pad the crib, use nonbreakable feeding utensils, wall-to-wall carpeting, pad edges of furniture, give safe toys, use approved car seat, wear a HELMET when learning to talk.
Term
What activities are OK for a child/adolescent with hemophilia?  What is NOT ok?
Definition
Mild cases need to use knee pads, elbow pads, helmets. If Mod/Severe no sports requiring padding. Swimming is OK for a young child. Swimming, bike riding, golfing, bowling (w/ protective equip) is all OK. NO Contact Sports, No sports that cause too much strain on the knees & ankles,
Term
Control bleeding for hemophiliacs w/ the RICE method.  What's that?
Definition
Rest, Ice, Compression, Elevation of joint
Term
Should pt's with hemophilia do active or passive exercises?
Definition
Do gentle ACTIVE ROM to their comfort. Do only after bleeding has stopped & within 24-48 hours. Allow client to control with own pain tolerance level. Do NOT do PASSIVE exercise, don't force b/c it could cause damage. Could stretch the joint capsule, could cause more bleeding.
Term
Home Infusion Therapy is something that families should be taught for hemophilia.  What are signs of major bleeding to know?
Definition
headache, blurred vision, vomiting, lethargy
Term
What are some family teaching done to prevent bleeding?
Definition
use a soft toothbrush, NO RECTAL TEMP, activity restrictions, environmental safety. Teach to control bleeding w/ RICE.
Term
What med should be taken for pain in hemophiliac?
Definition
Acetaminophen (Tylenol) - just NO aspirin b/c it's an antiplatelet
Term
Should a hemophiliac be taught basically that exercise is dangerous & not for them?
Definition
NO, regular exercise & physical therapy is good & strengthens muscles around joints. Support the family too.
Term
What is the prognosis/life expectancy of a hemophiliac?
Definition
approaches that of the general population
Term
What is the major cause of death for hemophiliacs?
Definition
intracranial hemorrhage
Term
What does ITP stand for?
Definition
Idiopathic (Immune) Thrombocytopenic Purpura
Term
Does ITP happen since birth?
Definition
NO, ITP is an ACQUIRED hemorrhagic disorder.
Term
Where may ITP come from?
Definition
autoimmune response to disease-related antigens. autoantibodies produced & attach to platelets. Phagocytosis of antibody coated platelets. Increased destruction of platelets (thrombocytopenia) in the spleen.
Term
The ACUTE form of ITP happens during what time frame?
Definition
lasts less than 6 months.
Term
The ACUTE form of ITP usually happens 1-4 weeks after...
Definition
1-4 weeks after a viral infection (e.g. URI, measles, mumps, rubella, chk pox, human parovirus)
Term
Who is the ACUTE form of ITP most common in?
Definition
children
Term
The CHRONIC form of ITP lasts..
Definition
longer than 6 months
Term
The CHRONIC form of ITP needs to rule out... what?
Definition
immunodeficiency disorder, autoimmune disease, neoplasms.
Term
The CHRONIC form of ITP is most common in who?
Definition
older children. women between 20-40 years old.
Term
What are S/S of ITP?
Definition
bleeding of the integument: usually a combination of petechiae, purpura, ecchymoses (getting bruising with no known cause). Mucous membranes are bleeding. There is potential internal blood loss. Intracranial hemorrhage risk, but less than 1%.
Term
ITP is characterized by a decreased ___________ count.
Definition
platelet - there is less than 150,000 platelets
Term
What happens in platelets are less than 50,000?
Definition
There is prolonged bleeding from trauma or injury
Term
What happens if platelets are less than 20,000?
Definition
can cause spontaneous life threatening hemorrhage
Term
what are the nursing interventions for ITP?
Definition
primarily supportive, prevent and/or minimize bleeding, administer medications.
Term
What should be done to prevent/minimize bleeding in ITP pt?
Definition
avoid Aspirin or aspirin containing products, avoid injections, use a soft toothbrush, Activity should be: no contact sports (football, ice hockey, wrestling, etc), no carnival rides, swimming all right, but NO diving.
Term
What meds are given for ITP?
Definition
PREDNISONE (most common, inexpensive, increased platelet survival-decrease production of antiplatelet antibodies), IVIG (2nd most common, expensive, blecks antibody that results in destruction of platelets), not as common -> ANTI-D ANTIBODY (WinRhoD)- (lessens phagocytosis of platelets), IMMUNOSUPPRESSIVE THERAPY (lessens antibody production).
Term
The prognosis of ITP is usually self-limited.  What are the indications of a splenectomy?
Definition
Unresponsive to Prednisone, High doses of Prensione are needed to maintain adequate platelet count, manifestations for 1 year or longer, can decrease the risk of hemorrhage w/ chronic form
Term
Lead Poisoning sources of lead are: 
Definition
lead-based paint (most common), soil, dust, air with lead particles (if live in industrial areas), water can contain lead, occupation & hobbies, dinnerware, cosmetics, folk remedies
Term
What are the effects of LEAD on body systems?
Definition
HEMATOPOIETIC (anemia), NEUROLOGIC (mental retardation, serizures, coma, death), RENAL (acute renal failure chronic renal failure
Term
Lead poisoning is defined as blood lead level (BLL) greater than or equal to ___ mcg/dl
Definition
10
Term
Children with BLL of greater than __ mcg/dl are considered lead exposed and worthy of intervention.
Definition
10
Term
Who is at high risk for lead poisoning?
Definition
child/sibling with elevated BLL, lives in/regularly visits home built before 1959, lives in/regularly visits house built before 1978 with rennovation or remodeling in the last 6 months. Lives in a building where other kids are being followed or treated for elevated lead levels, parents have occupational exposure, child lives near an active lead smelter, or other industry likely to release lead into the environment.
Term
What are red flags of lead poisoning?
Definition
foreign body ingestion/pica, unexplained seizures, neurologic symptoms, abd pain, growth failure, developmental delay, hyperactivity
Term
Children who have a BLL of 10 mcg/dl or above should have..
Definition
an environmental evaluation done... need to find the source: home, school, etc...
Term
What are strategies to reduce environmental lead hazards:
Definition
keep child away from chipping, peeling, flaking paint. temporarily cover above with contact paper, duct tape. want to WET MOP floors, windows. do NOT vacuum hard surfaced floors, window sills, it spreads the dust. Wash toys & pacifiers often.
Term
Should you vacuum or wet mop to prevent lead hazards?
Definition
WET MOP... vacuuming spreads dust in air more.
Term
Other ways to reduce lead hazards are: need to wash the child's face & hands often (esp before eating), hose down places where kids play (porch, driveway, sidewalk).  Should hot or cold water be used??  Food should not be stored in what?
Definition
Need to use only COLD water for drinking, cooking & food prep. No food storage in ceramic ware & lead crystal for food storage.
Term
Why are regular meals important to decrease lead?
Definition
more lead is absorbed on an empty stomach... so eating regularly decreases this
Term
Diet should be:
Definition
high iron, high calcium (to counteract loss of these minerals through lead exposure.)
Term
Calcium EDTA is given IM or IV...
Definition
it binds to lead & removes from system. giving it IM is very painful. need to give deep, mix with procaine, use warm compress. Monitor labs (e.g. BLL, electrolytes, liver function tests)
Term
Other meds given with Calcium EDTA for lead poisoning are...
Definition
British antilewisite (BAL) given IM. monitor labs & ECG. SUCCIMER (DMSO) given orally. May give as outpatient. Hydration is very important b/c it's excreted by the kidneys. These both have significant sd effects. Usually give Ca++ EDTA if it's going to be done. If they are over 25 mcg/dl it has to be done tho!
Term
Adequate _________ is VERY IMPORTANT since Chelates are excreted by the _________.
Definition
HYDRATION, kidneys
Term
The prognosis for lead poisoning is variable.  most of the major problems are r/t encephalopathy.  What can happen with an elevated lead level over time?
Definition
academic problems, delinquency, mental retardation, behavior changes, paralysis, seizures
Term
The bone growth in children is more porous & flexible.  When is bone growth activity high in children?
Definition
2-4 wks infant, 8-10 wks adolescent, 10-16 wks in adult. The poristeum is thicker & more active. The bone Remodeling is great. The Epiphyseal plate is present (growth plate) it is an area of weakness.
Term
_________ are the leading cause of death in 1-4 year olds.
Definition
injuries
Term
Playground deaths happen 15-17x/year.  How?
Definition
Usually strangulated somehow by clothes
Term
Parent tells you they are too scared to allow their child to participate in recreational sports... what do you know?
Definition
recreational injuries (playing in the yard) has more injuries than organized sports.
Term
Other big causes of injury are:
Definition
motor vehicle injuries. other: lawn mower, trampoline, fireworks, sledding & farm injuries.
Term
What is the #1 injury sport?
Definition
Basketball is #1 (not football!). 2) playground activities 3) biking 4) football
Term
Children spend more time & energy in sports than any other age group.  Every sport is going to have potential for injury.  There is an increased # of children involved in sports.  Why do injuries often occur?
Definition
often a physical mismatch, they don't realize their limitations
Term
Why should kids participate in sports?
Definition
contributes to GROWTH & DEVELOPMENT, education process and better health. Provides exercise, provides interaction with peers. Socially acceptable means enjoy stimulation and conflict. Helps process of self-appraisal, development of self-respect and concern for others.
Term
Some acute overload injuries from sports:
Definition
Dislocations (easier when loose, gymnastics, dancing, football is more tight), Sprains, Muscle Pulls are acute injuries.
Term
What are Chronic Overload sport injuries?
Definition
Stress Fractures, Tendonitis, bursitis, and fasciculitis (pain in arch or heel of foot)
Term
What is the #1 sports injury?
Definition
contusion (damage to soft tissue/bruising)
Term
If you're a first responder to a sports injury  what should you do?
Definition
#1 first thing is to IMMOBILIZE until treated. RICE for injury in extremity. Have someone else call 911 to get them there.
Term
When are extra calories & carbs needed?  increased fluids?
Definition
always increase fluids, don't need extra calories/carbs unless over one hour of continuous exercise.
Term
Fractures happen at high activity level with limited coordination.  What do you suspect if the story doesn't match the injury?
Definition
Child Abuse
Term
Where are the common locations for kids to fracture (break) bones?
Definition
forearm (distal, radius, ulna), Epiphyses (growth plate), elbow, leg, clavicle.
Term
If a child has a fracture in their Epiphyses (growth plate), ribs or skull fracturs... must rule out:
Definition
Abuse. Know red flags, suspicious fracture type or location, other injuries present.
Term
Common Types of Fractures in Children
Definition
BENDS (can bend 45 degrees until it breaks), BUCKLE (compression & bone looks like a bump. ex/ something falls on this to make a bump), GREENSTICK (snap.. leaves fragments, like a "tree snapping branch does this")
Term
What are the clinical s/s of fractures?
Definition
Swelling, Bruising/Redness, Pain/Tenderness, Diminished functional use of affected part, muscular rigidity, crepitus, vascular injury. The 5 S/S of vascular injury: pain pallor, pulselessness, paresthesia (tingling), paralysis. have wiggle fingers & toes b/c kids may not feel.
Term
What are some complications that may happen b/c of fractures?
Definition
Circulatory impairment (don't put cast on immediately b/c of swelling), Nerve Compression Syndromes (so much pressure from swelling it compresses the nerves), Epiphyseal Damage (if broken at growth plate could affect length of limb later), Nonunion/Malunion (movement after alignment in kids), Infection, Pulmonary Emboli (tho kids do have longer PTT than adults & it will take them longer to clot d/t longer bleeding studies)
Term
How are Fractures managed?
Definition
Diagnostic Studies (get x-ray, MRI-prob need sedation) Lab studies (don't really need to know, but some destruction of RBC's happens, Hgb & Hct may go down slightly), Closed/Open Reduction (open reduction means surgically put in screw, rods, while closed reduction means they do not break skin to realign), Plaster Immobilization, Traction, External/Internal Fixation (internal fixation is metal plates, rods, pins, screw. external is a cast)
Term
The orders for pin care will vary between doctors, but generally it's:
Definition
use HALF STRENGTH hydrogen peroxide on cutip around each pin. Press on tissue so it doesn't grow on. Use cutip with sterile water to wipe off. No ointment unless ordered.
Term
Types of casts for fracture:
Definition
plaster (better on upper ext), light weight, Spica (body cast that immobilizes hips, thighs. Going to do plaster 1st & then last layer is light weight.
Term
Nursing interventions for a cast:
Definition
Observe N/V status close, Turn every 2 hours, support wet cast with pillow & palm hand, elevate extremity, petal rough cast edges, no objects under cast, R.O.M. to extremity distal to cast. USE PALMS (or pillow) not fingers to move wet cast.
Term
What is Cast Petaling?
Definition
Cut several strips of petaling tape to about 4 inches, tuck one end of the tape under the edge of the cast and apply the tape to the cotton lining. Place the free end of the tape onto the cast's outside surface, continue to overlap the strips until a complete edge is formed, cover all edges of the cast, paying special attention to the diaper area of opening. Use the "cold" setting only with hairdryer.
Term
Is it ok to place stuff down in cast??
Definition
No, don't place anything into the cast
Term
Nurses should:
Definition
Restrict strenuous activity for just a few days, report foul odor & observe for drainage, clean soiled areas, prepare for cast removal (noise is frightening), provide positive diversion & therapeutic play
Term
What should a nurse do if she smells a foul odor coming from the cast?  what about drainage?
Definition
Foul odor is a sign of INFECTION & needs to be reported. Drainage should be marked & dated.
Term
Nursing interventions for a SPICA cast:
Definition
Takes 24-48 hours to dry, instruct movement of patient, protect from urine/stool, petal cast. (change diapers q hour. if holding stool? they may need a softener)
Term
Effects of immobilization:
Definition
circulatory/respiratory, muscle atrophy, joint contracture, metabolic, integument, GI/GU, Psychological, family maladaption.
Term
When immobilized it will be important to:
Definition
Promote as much normalcy as you can. If they are in traction for a long time they are going to need some diversional/distracting activities b/c they will go crazy after awhile.
Term
Why are children often placed in traction (instead of just having the extremity pinned)?
Definition
Pinning can disrupt growth. TRACTION is preferable to pinning in children.
Term
Traction might be intermittent or continuous.  They may just have skin traction.  Or may require skeletal traction.  Where is skeletal traction placed?
Definition
distal to bone to put pressure on.
Term
Bryant's traction is used for fractures of the femur or congenital anomalies of hip.  patient's limbs are suspended in the airvertically at a ninety degree angle from the hips and knees slightly flexed. Over a period of days, the legs hips are gradually moved outward from the body using a pulley system. The patient's body provides the countertraction.  This is used ONLY IF:
Definition
less than 2 yrs old & Less than 30 lbs. Otherwise tape will come off. The danger is pressure on the hip can cause necrosis. So it is not advised as much. The child should not be totally flat & you should be able to put hand under their bottom.
Term
You walk into a child who has tractions room.  They are conscious, breathing.  what do you always check first?
Definition
CHECK CIRCULATION. Assess 5 p's: pain, pallor, pulses, paresthesia (tingling), paralysis... wiggle/fingers & toes.
Term
What do you do if the pt does not have a pulse in extremity?
Definition
Call the Dr or have a friend call the Dr. Not going to cut off without the Dr there.
Term
What are the #2 & #3 things to assess in traction pt?
Definition
#2) get some pain medicine #3) assess if the weights are freestanding... get at the foot of the bed, look at the kid & get a clear straight angle with the traction. The foot of the bed & check if the weights are hanging freely.
Term
Can a child who is in traction be rolled?  how will you change the bed?
Definition
No. You will change bed head to bottom. scoot sheet & press mattress... get it under them.
Term
For a pt on bedrest in traction the nurse should:
Definition
observe their N/V status close, maintain traction apparatus, encourage diet of increased roughage & fluids, maintain skin integrity- should have special mattress like egg crate, sheep skin), ROM of unaffected parts, do Pin care, provide diversional activities & therapeutic play.
Term
What interaction/toys to promote fine motor development for child in traction?
Definition
Fine motor development? Puzzle, Play-doh. gross motor develoment? ball
Term
Developmental Dysplasia of the Hip has 3 different types.  what are they?
Definition
1. Preluxation (Dysplasia): is unstable hip that is "capable of" dislocation. 2. Subluxation: is "incomplete" dislocation. 3. Luxation (Dislocation): the hip IS dislocated
Term
Why does Dev Dysplasia of the Hip (DDH) happen?  risk factors for it?
Definition
ETIOLOGY IS UNKNOWN. 60% of the time there are no risk factors known. Potential risk factors are: caucasian, girls, positive family history, maternal hormone secretion, & intrauterine posture
Term
What are the clinical s/s of DDH?
Definition
Positive Ortolani & Barlow Tests (pt on back, bring hips to abd & open out to sides, hear clunk? positive), child has UNEQUAL SKIN FOLDS, Limitation of abduction on affected side, have unequal knee height, positive Trendelenburg sign (abnormal gait). X-RAYS are NOT helpful until 4 months of age.
Term
Explain the Ortolani Maneuver?
Definition
1. abduct each knee until the lateral aspects of knees touch the exam table 2. click or clunk is heard on abduction (confirms dislocated hip) 3. most reliable from birth to 2-3 months - only used then 4. confirms a dislocated hip (b/c an x-ray can't at this point)
Term
What is the Barlow Maneuver?
Definition
1. Flex & slightly adduct both hips while lifting the femur & applying pressure to the trochanter 2. Causes an unstable hip to dislocate 3. Confirms joint instability until 6 weeks
Term
May know DDH by:
Definition
Barlow & Ortolani tests, limited hip abduction, as seen in flexion at 6-10 weeks, apparent shortening of femur, as indicated by level of knees in flexion, Asymmetry of gluteal and thigh folds. Positive Trendelenburg (hopefully it's caught before chid is walking though. This is opposite what you would think: the lesion is on the contralateral side of the sagging hip. So, the healthy side is the downward side. The hurt side is upward.
Term
How is DDH managed in a newborn-6 months?
Definition
1. Cast (after aligned) 2. Abduction splint (pic holds babies hips out to side) 3. Pavlik Harness (allows for more movement, keeps in alignment they want. Successful about 89% of the time if done early though. Average is 2-3 months long for 24 hours.
Term
How is DDH handled in a 6-18 months?
Definition
1. gradual reduction by traction followed by a cast & then a brace 2. Closed reduction followed by cast & then a brace 3. If not successful an open reduction is required
Term
How is an OLDER Child managed for DDH?
Definition
Open Reduction then cast followed by rehabilitation
Term
Nurses need to put childs undershirt & diaper ________ harness or cast.  assure it's in proper position & it's not too flexed.
Definition
under
Term
How does a nurse maintain a reduction device?
Definition
they may or may not remove it for bathing, should wear undershirt & knee socks-place diaper UNDER The straps, assess skn beneath harness 2-3 times per day, do not powder or lotion baby, keep in proper positioning, prevent soiling, provide relief from boredom.
Term
What is Talipes?  who is it more common in?
Definition
Clubfoot. More common in males (2:1). Positive family history in 1/2 of cases.
Term
What are the 3 types of clubfoot?
Definition
1. POSITIONAL (able to get) 2. SYNDROMIC (unable to tun to normal position. Syndromic is the true congenital clubfoot (most common). 3. CONGENITAL (associated with another defect)
Term
What are the clinical manifestations of clubfoot?  
Definition
The deformity of the limb is characterized by: Smaller calf muscles & tightening or lax of foot, Adductus of the forefoot, increased longitudinal arch, heel turned in (Varus), heel turned out (valgus), plantar flexion, dorsiflexion. The most common type=Talipes Equinovarus
Term
If the pt has a 'Positional' clubfoot they need to do stretches & exercises to foot.  But if they have 'Syndromic' - True Clubfoot - they need:
Definition
serial casting... it is the treatment of choice. Position as much as can and cast. Remove cast & postition foot more. done every few days. Then every few weeks. Takes about 8 wks until to the position they want.
Term
The goal of managing clubfoot is:
Definition
a pain-free foot with good mobility, without calluses, and without the need to wear modified shoes & inserts. Functional abnormality? Passive Stretching exercises.
Term
Nursing Interventions & care of club foot involves:
Definition
assessment, teaching parents cast care, reinforce orthopedic surgeon's explanations & instructions, emotional support, support normal G & D
Term
What is Legg Calve Perthes Disease?
Definition
Aseptic necrosis of the femoral head. There is rubbing & a lot of pain! 2-12 yrs old: 20% bilateral. Mostly happens to caucasian males. The cause is UNKNOWN: there is trauma, inflammation, coagulation defects.
Term
What are the clinical manifestations of Legg Calve Perthes Disease?
Definition
The onset is insidious (slow), it's achy, stiff, referred pain, joint dysfunction, limited ROM. Diagnosis: radiographic examination.
Term
How is Legg-Calve-Perthes disease managed?
Definition
REST: non weight bearing, Nonsurgical containment: non-weight bearing devices help reduce pain. They are on bed rest for about 6 weeks. The Bucks traction will bring ball from hip joint. The parents are give the option of surgery or a non-surg treatment (wear brace). Surgery will go in, clean necrotic & transplant bone form somewhere else. takes 6 wks, cast, wheelchair, crutches, shorter length of time. 6 months vs. 2 years. Higher increased risk for developing arthritis as an adult.
Term
Nursing interventions for Legg Calve Perthes Disease?
Definition
Assessment, Education of corrective device (emphasize importance of compliance), post op cast care, promotion of normal G & D. They need to wear it to bed, but may take it off to bathe.
Term
How would you respond to a mom that says she needs to be ready for a gymnastic meet in 3 months?
Definition
Explain the process and the importance for the rest of her life. Even with surgical intervention, it is going to take 6 months. Very invasive & painful (transplanting bone)... cast, wheelchair, crutches.... she's not doing the meet!
Term
What is the disorder of the proximal femoral physis that results in malalignment of the femoral head relative to the femoral neck?  (the femoral head pulls out of the socket->displacing the hip)
Definition
SLIPPED FEMORAL CAPITAL EPIPHYSIS (SFCE)
Term
When doeSLIPPED FEMORAL CAPITAL EPIPHYSIS usually occur?
Definition
during growth spurts. There are HORMONAL & MECHANICAL factors that cause it. Endocrine disorders, GH therapy, radiation therapy & chemo agents.
Term
With SLIPPED FEMORAL CAPITAL EPIPHYSIS the toe is out & keeps the affected leg kind of out. Results in an "antalgic limp" Who is it common in?
Definition
more common in polynesians, african americans, males, can be bilateral
Term
The child with SLIPPED FEMORAL CAPITAL EPIPHYSIS often has a History of:
Definition
thigh or knee pain or maybe no pain. some limp, out-toeing or gait abnormality. less often, they're easily fatigued. May or may not related symptoms to an injury.
Term
What does the physical exam & diagnosis of SLIPPED FEMORAL CAPITAL EPIPHYSIS?
Definition
Antalgic Limp, Out-toeing, decreased internal rotation hips, hip flexion is limited & produces pain. Diagnosed by: radiography (x-ray), MRI or bone scan.
Term
How is SLIPPED FEMORAL CAPITAL EPIPHYSIS managed?
Definition
EARLY DIAGNOSIS IS CRUCIAL, a screw is placed to keep ball in hip spot, want NON-weight bearing, Internal Fixation, post surgery: non-weight bearing & crutch use until there is painless ROM achieved.
Term
Nurses can help SFCE management by:
Definition
assessment, maintain non-weight bearing status, pain control, teaching before surgery-need to be on BEDREST prior to surgery! This is rough, they're often athletic, adolescent boys. Then after surgery: teach wound care & crutch walking (going to be 2-6 months!), promote normal G & D
Term
When can a child following SFCE surgery expect to play sports again/recover?
Definition
if surgery during Fall? No Winter sports, but Spring sports will be OK.
Term
Scoliosis is a spinal deformity that usually involves the __________ curve, spinal rotation causing rib asymmetry, and thoracic hypokyphosis.  what's the cause?
Definition
lateral. cause may be unknown or r/t other disorders. It may be congenital or acquired.
Term
Scoliosis is most common beginning in:
Definition
the preadolescent growth spurt. Though they rarely experience discomfort & there is few outward signs. Why screening is so important
Term
Explain Scoliosis exam...
Definition
observe using Adam's Position (leaning way forward), use Scoliometer (highest position on back, take 3 different screening, higher than 5 degrees is red flag), radiographic exam, MRI is done if other pathology. Screening for scoliosis is controlversial. A large # get referred from school & it's costly & most turn out minor.
Term
There are exercises to do for scoliosis management & there are braces too.  Mild scoliosis is up to ____ degrees (just monitor the progression curve).  While moderate is ____-____ degrees (need bracing). 
Definition
mild: up to 15 degrees. moderate: 15-40 degrees.
Term
What are some teaching instructions for scoliosis brace kids & parents?
Definition
Emphasize the importance of compliance (is a real problem), going to take it off for showers, ok to take brace off for 1 hour per day & it won't be a problem, no contact sports with brace, tennis ok (some sports are)
Term
If child has more than 15 degree curve?  Need to consider surgery.  why does this benefit child?
Definition
If it's 15 degrees, they are having a 50% decrease in breathing/pulmonary. Surgery is done to help pulmonary, cosmetic, pain caused in older children, children with neuro difficulties have difficulty sitting & walking b/c of imbalance.
Term
Surgery for scoliosis works by:
Definition
realigning and straightening the spine with internal fixation and instrumentation combined with spinal fusion. Types: Harrington Rod, Luque segmental instrumentation, Cotrel-Dubousset approach
Term
Tell about the Harrington Rod surgery:
Definition
It can be put in anterior or posterior or both. have nuts & bolts to keep the rod straight. Will need a back brace. Should ambulate by day 3. Go to side of bed, put feet down & then stand up.
Term
Tell about the Cotrel-Dubousset Approach of surgery:
Definition
Rod & wire. Often need BLOOD during surgery. Often takes a couple of weeks for family to donate blood.
Term
what do nurses do for scoliosis?
Definition
assessment of the curve, bracing (teach care of brace, emphasize compliance, encourage normal G & D. Before surgeryL assist with teaching & work up.
Term
Care for pt who just had scoliosis back surgery involves:
Definition
Child usually doesn't realize what HUGE surgery this is. They will have an incision all the way down their back: ** monitor in ICU for 24-48 hours. ABC's! Careful assessment of N/V status. Place on special mattress or mattress pad. ** LOG ROLL every 2 hours. It is ok to log roll, just keep the back straight.
Term
Other care after scoliosis surgery involves:
Definition
frequent pain meds (PCA) after surg + FOley Cath, assess their bowel motility, skin integrity (incision), instruction on ambulation, instruct brace or cast care, promote normal G & D. They don't usually have an NG tube. The hospital stay is about 7 days: mostly for pain control.
Term
Osteogenesis Imperfecta is a type of defect in the ______________ gene.  The error results in faulty bone mineralization, abnormal bone architecture & increased susceptibility to fractures.
Definition
precollagen. Essentially, they're missing collagen (the structural component of the bone. It is also called brittle bone disease - their bones are really soft.
Term
Osteogenesis Imperfecta is an autosomal ______________ hereditary disorder.  
Definition
dominant (although she also said it can occasioally be autosomal recessive, but it's rare). It is heterogenous.
Term
There are 4 types of OI, but 2/3 of cases are Type I.  What are the symptoms of OI?
Definition
Mild bone fragility, BLUE SCLERA, normal teeth, joint laxity, have hearing loss or hear ringing in their ears during teen years & leads to deafness. Have vertigo by late 40's - 50's.
Term

OI may be confused with _____ _______

 

Definition
child abuse
Term
How is OI managed?
Definition
primarily supportive, medications to increase bone density, experimental: bone marrow transplants (they have had positive results, but it's still considered experimental). Lightweight braces & splints, physical therapy, may need surgery to correc the deformities later.
Term
Nurses help OI by:
Definition
being Supportive - parents need extensive teaching (how to care for the child, assessment of the fracture, 1st aid if fracture, discipline-the child needs to have discipline!). Should carefully support child when turned, positioned, or moved. Parents should stay in hospital to protect the child from HC workers. Parents are the ones who should move the child around. Do NOT do BP on these children! NO tourniquette before blood draws or IV.
Term
When you change a babies diaper with IO what is different?
Definition
you cannot lift by the ankles like normal, just sort of slide under.
Term
Need to help parents guide suitable activities to promote optimum ___________ , but need to protect the child from harm too.  Should refer the family to the OI foundation
Definition
development
Term
What is NOT done on OI kids?
Definition
no BP! no tourniquette before IV is put in! Parents need to stay in the hospital to protect the child from HC workers.
Term
What is Juvenile Idiopathic Arthritis (JIA)?  
Definition
It is aka "Juvenile RA."
Term
What sex & what 2 age groups does juvenile arthritis peak in?
Definition
Females. 1-3 yrs old. 8-10 yrs old.
Term
Juvenile Idiopathic Arthritis is a CHRONIC inflammation with joint effusion & eventual erosion.  Where does it come from?
Definition
unknown, some theories say it is caused when infectious agents create autoimmune inflammatory processes
Term
Juvenile Idiopathic Arthritis (JIA) clinical manifestations:
Definition
stiffness (often in morning), swelling, warm to touch, loss of motion, may be tender & painful to touch, growth disturbances (often d/t long term corticosteroids). RF is only in about 10% of cases
Term
3 major classifications of JIA?
Definition
systemic onset, pauciarticular, and polyarticular
Term
How is JIA diagnosed?
Definition
diagnosed by exclusion: age of onset, # of joints involved & exclusion of other causes. Antinuclear antibodies are common. Leukocytosis is frequently present during exacerbations. (ESR may or may not be present & RF is only present in about 10% of cases)
Term
Is there a cure for JIA?
Definition
NO
Term
What are the major goals of JIA?
Definition
control pain to a functional level (can't really take away all pain), preserve joint ROM, minimize effects of the inflammation, promote G & D. Use a multidisciplinary approach. Possibly: Surgery.
Term
What are the pharmacological treatments for JIA?
Definition
NSAIDs, slow acting antirheumatic drugs ,corticosteroids (like to give in bursts or every other day b/c they can stunt growth), etanercept, other cytotoxic agents.
Term
interventions for JIA:
Definition
assessment, PAIN RELIEF, diet & exercise, sleep & rest (when increased fatigue -> need more naps), encourage compliance, promote normal growth & development activities (school is encouraged though some have trouble. some maybe just go to afternoon classes), emotional support
Term
Duchenne Muscular Dystrophy (DMD) is caused by what?
Definition
it's unknown, but it's basically a defect in the muscle enzyme. it's a metabolic disturbance unrelated to the nervous system.
Term
Duchenne (DMD) is the most severe & most common muscular dystrophy.  who does it happen to?
Definition
Males. It's an X-LINKED RECESSIVE PATTERN (1/3 of cases is due to new gene mutations. There is an absence of Dystrophin in muscle. They're missing a protein in the muscle.
Term
Duchenne Muscular Dystrophy has an onset of what age group?  They usually have a history of delayed _________ development.
Definition
3-5 yrs old w/ a hx of delayed motor development.
Term
What are the 1st symptoms of DMD?  What about the later symptoms?
Definition
1st signs are often difficulty running, riding bicycle or climbing stairs. Later they have an abnormal gait, lordosis (protruding abdomen b/c of spinal curve), frequent falling and characteristic manner rising from squatting on the floor.
Term
What is Gower's Sign?
Definition
Manner of walking self up when on the floor w/ hands while legs are straight. They can't just stand up.
Term
What are some other manifestations of DMD?
Definition
calf muscle hypertrophy, loss of independent ambulation by 9-11 years old, mild mental retardation is common (does not occur with ALL, but on average a 20 pt lower IQ), slow progressive general weakness. The average age of death is early 20's. Death is usually due to respiratory or cardiac failure.
Term
What are some complications of Duchenne Musc Dystrophy?
Definition
Contractures, Scoliosis is VERY COMMON (need surgery just to sit up in chair, it's bad), disuse atrophy, infections (encourage cough & deep breathing), obesity is common (w/o active muscles they don't need as many calories, tend to be overfed), cardiac
Term
DMD kids often will wear ThAIRapy vests like CF pt's to break up secretions.  It is very important to teach DMD pt's to get a ________ vaccine.
Definition
pneumococcal & influenza
Term
DMD diagnosis looks at:
Definition
Genetics (defective dystrophin gene), serum enzymes (if checked 2 yrs prior to s/s, they would be elevated), biopsy (show muscle fibers), EMG (shock muscle & get decreased response with electrical stimulation).
Term
How is DMD therapeutically managed?
Definition
There is NO EFFECTIVE TREATMENT. Parents face long-term decisions like want a trach? want a vent?
Term
What is the GOAL of Duchenne Musc Dystrophy?
Definition
maintain ambulation as long as possible & prevent contractures.
Term
Children with DMD need nurses to:
Definition
promote as much independence as possible, they need a balance between limiting activity and allowing activity. Encourage avoiding respiratory infections. Going to need a lot of emotional support & have depression about their future. Will eventually need paliative care b/c die in early 20's.
Term
What is the DIET for Duchenne Muscular Dystrophy?
Definition
LOW CALORIE & HIGH VITAMIN C.
Term
a condition where the head is tilted to one side.  they have limited ROM in neck is called... 
Definition
Torticollis
Term
Metatarsus Adductus is a when the foot is turned ________ greater than 20 degrees.
Definition
outward
Term
Metatarsus Varus is a condition where the foot is turned ________ greater than 20 degrees.
Definition
inward
Term
Pes plantus is ______ feet
Definition
flat
Term
Genu Varum is ____ ________.
Definition
bow legged
Term
Genu Valgum is _____ ______.
Definition
knock knee
Term
Genu Recurvation is ...
Definition
hyperextension of the knee joint
Term
Osgood-Schlatter disease usually occurs during a growth spell.  What is seen?
Definition
Swelling beneath the knee. This will go away on its own. Parent will be scared by this. If it's painful? Rest.
Term
Cancer risk factors to children:
Definition
ionizing radiation, electromagnetic fields (not proven), parental exposure to Benzene petroleum agents, alcohol & smoking during pregnancy
Term
who has a genetic predisposition to Epstein-Barr Virus (EPV)? 
Definition
Chromosomal abnormalities (Down Syndrome, Fanconi Syndrome) & Diseases with specific genetic links (Retinoblastoma has the strongest genetic link to cancer, Wilm's Tumor (increased incidence of congenital anomalies), Neuroblastoma)
Term
How prevalent is cancer in children?
Definition
Cancer is the 2nd leading cause of death in children. There has been improved prognosis in the last 3 decades. The definition of "cure"
Term
__________ has the best prognosis.
Definition
Leukemia
Term
Bone Tumors (osteogenic & Ewing Sarcoma) happen more to _________.
Definition
caucasians
Term
Wilms Tumors (kidney, soft tissue) happen more to ___________ _______.
Definition
African Americans
Term
What is the definition of "cure"?
Definition
1) complete the therapy 2) continuous freedom of clinical care 3) cancer type & risk of relapse 4) period of time, usually 2-5 years
Term
LEUKEMIAS are the MOST COMMON form of childhood cancer.  It is more frequent in males.  The peak onset for leukemia what age group?  What is affected?
Definition
2-6 yrs old. Affects the bone marrow & lymphatic system.
Term
Leukemia is classified by the type of WBC affected by the disease.   What kind of leukemia is it if the WBC's being affected are: Lymphatic, Lymphoid, Lymphoblastic, and Lymphoblastoid (synonyms)?
Definition
It is ALL: Acute Lymphocytic Leukemia
Term
Leukemia is classified by the type of WBC affected by the disease.   What kind of leukemia is it if the WBC's being affected are: Myelocytic, Myelogenous, Monoblastic, acute NonLymphoid leukemia (ANLL)?
Definition
AML: Acute Myelogenous Leukemia. ANL=AML
Term
What is the most common form of leukemia in children?
Definition
ACUTE LYMPHOCYTIC LEUKEMIA (ALL). Is 80% of leukemias in children. This type rarely occurs in adults over 40.
Term
Within ACUTE LYMPHOCYTIC LEUKEMIA there is FAB subtypes (L1, L2, L3). Which one is most common & has best prognosis?
Definition
L1
Term
What are signs of a good prognosis?
Definition
FAB = L1. Cell surface markers = early Pre B cell & CALLA (common acute lymphocytic antigen). Leukocytes are LESS THAN 50,000. There are more than 50 chromosomes. You're a female. between the ages 2-10 yrs old. Have the FAB morphology L1.
Term
Acute Myelogenous Leukemia is most common leukemia in _________.
Definition
adults. AML is only 20% of leukemias in kids & is harder to treat. The chronic form is only 2% of childhood leukemias. The cell surface markers are not well defined with AML.
Term
What is the pathophysiology of Leukemia?
Definition
There is UNRESTRICTIVE PROLIFERATION OF IMMATURE WBCs IN THE BLOOD FORMING TISSUES OF THE BODY. Results in the REPLACEMENT OF NORMAL FUNCTIONING CELLS WITH NONFUNCTIONAL LEUKEMIC CELLS.
Term
The consequences of Leukemia are:
Definition
Decreased functioning eryhtrocytes (get ANEMIA), Decreased Platelet production (get BLEEDING), Decreased functioning WBCs (Neutropenia) (get Infection).
Term
The big 3 consequences of Leukemia are:
Definition
Anemia, Bleeding & Infection.
Term
Leukemia also causes:
Definition
infiltration, enlargement, and fibrosis of spleen, liver, and lymph glands. Insidious (slow) onset with a cold or minor symptoms, fever, pallor, fatigue, anorexia, petechiae, bone & joint pain, spleen, and liver enlargement, excessive bruising, lymphadenopathy, malaise, profound anemia, abnormal WBC, thrombocytopenia.
Term
Children are diagnosed with leukemia when:
Definition
usually when they're not sick, it's based on history & physical manifestations. Peripheral Blood Smears (show immature leukocytes, frequently low blood counts), Bone Marrow Aspiration is the MOST DEFINITIVE (will be a large number of BLAST cells), Lumbar Puncture shows bad significant positive CNS involvement.
Term
Goal of treating acute leukemia:
Definition
To achieve a complete remission & restoration of normal bone marrow function. Usually a combo of chemo is used with drugs according to the established protocol of the institution. Radiation is used sparingly in kids... only use radiation in very high risk leukemia situations. Kids normally just get chemo & drugs for leukemia.
Term
When is a bone marrow transplant considered for leukemia children?
Definition
ALL (most common type in kids) during 2nd remission period. AML (less common) during first remission period.
Term
The goal of acute leukemia treatment is:
Definition
complete remission and restoration of normal bone function
Term
Chemo treatments begin with Induction phase.  It is begun immediately and lasts 4-6 wks.  What is the goal?
Definition
Goal is to achieve a complete remission determined by absence of clinical signs of disease and presence of 5% blast cells in bone marrow.
Term
Induction uses 4-5 drugs combined.  There are different drugs used for ALL & AML types.  What are the types used in ALL?
Definition
corticosteroids, vincristine, L-asperaginase, and doxorubicin at various schedules.
Term

Induction uses 4-5 drugs combined.  There are different drugs used for ALL & AML types.  What are the types used in AML?

Definition
doxorubicin or daunomycin and cytosine arabinoside, and various other drugs
Term
The 2nd phase of chemo treatment is the 'Intensification' phase.  what happens during this phase?  how long does it last?
Definition
Pulses of several meds are given periodically during the first 6 months of treatment. The goal here is to eradicate residual leukemic cells and prevent resistant leukemic clones.
Term
When is CNS prophylactic Therapy used?
Definition
Continued during all phases with intrathecal METHOTREXATE sometimes in combination w/ other drugs to prevent CNS disease.
Term
The last phase of chemo is 'Maintenance' Therapy.  What is the goal here?
Definition
To preserve remission
Term
What is taken daily during the maintenance phase?
Definition
Daily 6-mercaptopurine, weekly methotrexate and pulses of steroids and vincristine. Patients will need to continue weekly or monthly blood counts to assess for effects of mylosuppression. Most relapses occur within the first year. A relapse would be the presence of leukemic cells in the bone marrow.
Term
how long does the maintenance phase last?
Definition
2.5-3 yrs
Term
What are the 3 phases & how long do they last again?
Definition
Induction (4-6 weeks), Intensification (6 months), Maintenance (2.5-3 yrs)
Term
What is the prognosis of Acute Leukemia?
Definition
The majority of children who receive multiagent chemo will survive. 80% achieve long term survival without serious health problems from the disease or treatment (ALL). Prognosis with bone marrow transplant varies, but long-term survival is between 25-50%. The prognosis for ALL (more common in kids) is better than prognosis of AML (more common in adults, but does occur in kids).
Term
What is (Lymphoma) Hodgkins Disease?
Definition
Neoplastic Disease originating in the lymphoid system
Term
What age group does Hodgkins affect in children?
Definition
It is rare under 5 yrs old. It is common between 15-19 yrs old.
Term
What is the etiology of Hodgkins Disease?
Definition
Unknown, but there is a possibly an association with the Epstein Barr Virus
Term
There is a systematic spread that occurs with Hodgkins Disease that helps to diagnose (as well as Reed Sternberg Cells).  Where is the predictable metastasis to?
Definition
spleen, liver, bone marrow, lungs & other tissues
Term
Hodgkins has enlarged lymph nodes that are painful or painless?
Definition
PAINLESS LARGE LYMPH NODES. They are firm, nontender, movable in the clavicular & cervical region.
Term
What lymph node is often involved first in a child?
Definition
Child usually has the "sentinel node" located near the lef of the clavicle often involved first. (Other S/S are: night sweats, nonproductive cough, wt loss, anorexia, pruritis, mediastinal mass (sentinal node), hepatosplenomegaly <- enlarged spleen)
Term
How is Hodgkins Disease diagnosed?
Definition
a CT scan of the neck, chest, abd and pelvis. Chest x-ray. bone marrow. Going to do a complete blood work up & urinalysis. In selected cases may do a laparotomy w/ multiple biopsies of organs and lymph nodes and a splenectomy.
Term
Hodgkins Disease diagnosis will do a lymph node biopsy.  Presence of ____________ cells is diagnostic.
Definition
Reed-Sternberg
Term
How is Hodgkins Disease treated?
Definition
Chemo & irradiation based on the clinical staging of the disease. Responds well to aggressive therapy, but increases chances of complications & serious late effects (risk for other cancer later in breast, thyroid). Follow up care is essential to monitor for relapses. Getting a Splenectomy requires prophylactic antibiotics for an undetermined length of time.
Term
Overall 10 year survival rate for kids with Hodgkins Disease is ___%.
Definition
90%. Prognosis is excellent with localized disease (stage 1 & 2). Late reocurences do occur both with the original disease process & 2nd malignancies. Risk for late reoccurences is higher in FEMALES than males.
Term
How many pediatric lymphomas are classified as NON-Hodgkins Lymphomas though?
Definition
60%
Term
Who does non-Hodgkins Lymphomas happen to?
Definition
Occurs most often under 15 yrs of age (peak ages are 7-11)
Term
Who is Non-Hodgkins Lymphoma seen the most in?
Definition
Patients with immunodeficiency syndromes or those who are immunosuppressed due to having a transplant.
Term
Non-Hodgkin Lymphoma (NHL) us a malignancy in the B & T lymphocytes.  Clones of the malignant cells infiltrate the lymph nodes, bone marrow, blood & other organs.  NHL includes many subtypes classified by cell size, structure, appearance, grade (how fast or slow they grow), etc...  What are the 3 NHL that are very fast, aggressive growing types?
Definition
Lymphoblastic, Burkitt or non-Burkitt and Large Cell Lymphoma. These 3 are fast growing, but respond well to chemo.
Term
What are the clinical s/s of NHL?
Definition
depends on the anatomic area involved & the extent of involvement. It can metastasize to bone marrow & resemble leukemia. Might have abd cramps, constipation, pain, anorexia, wt loss, ascites, vomiting. Painless and enlarged lymph nodes in cervical and auxilliary region, fever. Compression of tumor to organs & abd, mediastinum, head & neck cause symptoms of obstruction.
Term
Diagnosing Non-Hodgkins Lymphoma is done by:
Definition
surgical biopsy of lymph nodes, bone marrow aspiration, x-rays and CT of the chest, abd & pelvis. Lumbar puncture. Staging (how much has spread to other areas of the body). grading (how aggressive the cancer is)
Term
Non-Hodgins Lymphoma: Burkitt's Lymphoma.  What is the presentation of Burkitt's Lympomas?
Definition
Rapid growing tumor, in abdomen, jaw or eye orbit. Rarely seen enlarged lymph nodes or hepatosplenomegaly. NO LARGE LYMPH NODES. Tumor Lysis Syndrome happens b/c very fast growing. The tumor lyses & biproducts are dumped in system. may cause metabolic complications. give allopurinol.
Term
How is Non-Hodgkins Lymphoma treated?
Definition
aggressive approach w/ chemo. similar treatment for leukemia. Administer for ** 6-24 months ** Aggressive tumors are very responsive to treatment, thankfully.
Term
What are the most common solid tumors in children?
Definition
brain tumors. They are 20% of childhood CAs.
Term
Where are 60% of brain cancer tumors at?
Definition
infratentorial or below the tentorium cerebri (below the cerebellum and brain stem)
Term
About 40% brain tumors are:
Definition
in the anterior 2/3 of the brain (cerebrum)
Term
What's the patho of brain tumors?
Definition
they originate from abnormal DNA and uncontrolled cell division. There is NO CLEAR ETIOLOGIC FACTOR for them though.
Term
Brain tumors cause DEATH TO SURROUNDING TISSUE b/c infiltration & compression.  Cerebral edema leads to increased ICP causing...
Definition
focal neuro deficits, obstruction of CSF flow, pituitary dysfunction, and eventual herniation of the brain stem.
Term
Where do neoplasms come from?
Definition
any cell within the cranium. Tumors are named from the tissue of origin.
Term
what does high level vs low level tumor mean? 
Definition
high level means more aggressive
Term
What is the most common pediatric tumor?
Definition
Astrocytoma
Term
_____________ make up 20-25% of ped brain tumors.  Is highly malignant.
Definition
Medulloblastoma
Term
What are child brain tumors that are difficult to access?
Definition
Cerebellar Astrocytoma (15% brain tumors), Brainstem Glioma (15% of tumors, hard to remove), Ependymoma (4% tumors, invades ventricles)
Term
Signs of brain tumors are r/t anatomic loc, size & child's age.  What are some signs though?
Definition
headache upon wakening, vomiting that is not r/t food intake, neuromuscular changes (lose balance, clumsy, loss of developmental milestones), behavior changes (irritble, lethargic, acting bizzare)
Term
Clinical signs of cranial nerve neuropathy:
Definition
head tilt, face weakness, visual defects
Term
What are other VS disturbances & other clinical signs of brain tumors?
Definition
decreased pulse & respirations, increased BP, hypothermia & hyperthermia, seizures, nuchal rigidity, papilledema, cranial enlargement, bulging fontanel
Term
Diagnosis of brain tumors involves:
Definition
careful & thorough hx, physical & neuro exam to establish deficits, MRI, CT, skull films, EEG, LP, definitive diagnosis is usually based on TISSUE SPECIMENS taken during surgery.
Term
How are brain tumors treated?
Definition
surgery, radiotherapy, and chemo are used depending on type of tumor.
Term
Stereostatic surgery involves the use of CT & MRI and special computer techniques.  What does Radiotherapy do?
Definition
used to treat many tumors & to shrink tumors before surgery.
Term
In the last decade, Chemotherapy has come up with more effective drugs (b/c they can cross the ______ and treat tumors) all alone or with radiotherapy (shrinking) and surgery.  
Definition
BBB - blood brain barrier
Term
What are other drugs used for brain tumors?
Definition
corticosteroids to control cerebral edema, anticonvulsants for seizures, pain meds, antibiotics, antiemetic and histamine blockers
Term
Obviously, dx of brain tumor is a crisis event.  Family & child will need a lot of support.  What can the nurse do to prepare the family?
Definition
inform the child & family the head will be shaved, prepare them for the size of the dressing, inform them they will be in an ICU and the child will likely be asleep for a couple of days.
Term
Nursing care after brain tumor surgery.  What position should the bed be in?  
Definition
Place the HOB at 30 degree angle (if supratentorial tumor, in the upper 1/2 head) and flat on either side (if infratentorial tumor, lower 1/2 head by brainstem)
Term
What other nursing care post op?
Definition
monitor VS (potential for hypothermia/hyperthermia so do TEMP!), monitor for increased ICP and hemorrhage (neuro signs like decreased LOC & dressing), may need Restraints, Fluids
Term
__________________ are primarily found in the abdomen.  They are silent killers that usually metastasize before they are found.  have an overall poor prognosis.  50% cases occur in kids under 2 yrs old.
Definition
Neuroblastoma's
Term
What are the S/S of a neuroblastoma?
Definition
a firm not tender mass that CROSSES THE MIDLINE OF ABD, causes compression of adjacent structure creating symptoms, compression of ureter, kidney, bladder causing urinary problems, compression of spinal cord: bowel & bladder problems, paralysis. Chest problems are cough, breathing problems. Lymphadenopathy. Intracranial lesion-neuro impairment. May cause BLACK EYES and edema around the eye b/c of metastasis from original tumor.
Term
Diagnosis of Neuroblastoma tries to locate the primary site of metastasis.  Does the usual x-ray, CT scan, bone marrow aspirate & biopsies.  What else is analyzed?
Definition
24 hour urine for breakdown products of catecholamines (VMA & HVA, dopamine and norepinephrine)
Term
Neuroblastomas do have a bad survival rate :(  and the treatment is going to be based on clinical staging (how far it has spread).  If in stage 1 & 2 ...
Definition
surgical removal is ideal. Chemo is the main therapy for extensive disease. Recurrent disease in children over 2 yrs old is 10-20% survival rate (low!) with high dose chemo, BMT or peripheral stem cell rescue.
Term
The prognosis for neuroblastoma is better ...
Definition
the younger you are at diagnosis, the better the prognosis. This one has a genetic component & 15-20% are inheritable.
Term
__________________ is the most common intraabdominal and kidney tumor of children.  
Definition
Wilms Tumor: Nephroblastoma
Term
S/S of Wilms Tumor?
Definition
Most common sign is: swelling or mass w/in abdomen (but this does NOT cross the midline. Firm, nontender, confined to one side. Other s/s are hematuria, anemia, HTN, wt loss & fever, metastasis to lungs (pain, dyspnea, SOB, cough, etc)
Term
Wilms Tumor is diagnosed by:
Definition
abd ultrasond, CT, MRI, heme studies, kidney fx tests, UA, bone marrow, phys exam, tumors can be quite large & usually remain encapsulated for an extended period of time. Pre-op they should NOT BE PALPATED IN ABDOMEN. Do want to monitor BP frequently.
Term
Surg for Wilms Tumor involves chemo & surgery....
Definition
entire kidney & adrenal gland is removed. great care taken to avoid rupturing encapsulated tumor. other kidney & lymph nodes are inspected for disease, any involved structures are removed, if both kidney are involved a partial nephrectomy may be done on the least affected and a total on the most affected kidney.
Term
Radiotherapy is only done on really large Wilm's Tumors, ones that metastasized or reoccured.  All children need to receive _____________ after surgical removal of Wilm's Tumor for 6-15 months.
Definition
chemotherapy
Term
What nursing care is needed for Wilm's Tumor?
Definition
These kids have no symptoms of disease other than mass, parents feel guilty, have a swift intervention with surgery within 24-48 hours after being diagnosed. Prior to surgery the kid needs a DO NOT PALPATE ABD sign on bed. Carefully handle child. MONITOR BLOOD PRESSURE. Support child & family.
Term
After surgery for wilm's tumor, nursing care is to...
Definition
accurate I & O, monitor BP, monitor GI activity. Life long considerations with one kidney.... really need to avoid UTI's in girls and avoid contact sports. Protect the one kidney from injury!
Term
Rhabdomyosarcoma is the most common..
Definition
soft tissue sarcoma in children.
Term
Sarcomas derive name from tissue of origin too.  Where is Rabdo?  Where is Myo?  Where is Striated?
Definition
Rabdo=Striated. Myo=muscle. Striated=skeletal... found almost anywhere in the body...
Term
Where do Rhabdomyosarcoma occur?
Definition
Many sites... the most common are head, neck, and orbit of eye.
Term
Who are Rhabdomyosarcoma most common in?
Definition
children under 5, but can be seen in children of all age groups.
Term
What are clinical s/s of Rhabdomyosarcoma?
Definition
s/s depend on tumor location and compression of organs (soft to hard, non tender, immobile, mass). Perioorbital (eye): visual changes. Nasopharynx: stuffy nose, nasal obstruction, pain, sinus discharge, swelling. ear: otitis media, pain, drainage. Extremity: ROM is affected.
Term
What are the s/s of Rhabdomyosarcoma in the retroperitoneal & pelvic area?
Definition
function of organs affected, abd mass, pain, intestinal & GU obstruction. Tumors here are often widely metastasized before diagnosis. Many of the signs are vague & suggest childhood illness!
Term
___________ is imp to establish with Rhabdomyosarcoma because it is a tumor that is highly malignant with metastasis at diagnosis.  Aggressive multimodal therapy is recommended.
Definition
Staging
Term
What is the treatment of Rhabdomyosarcoma?
Definition
Now it's complete removal of primary tumor followed by radiation and chemo for 1-2 years
Term
What is the prognosis of Rhabdomyosarcoma?
Definition
5 yr survival rates are about 65%. If child is disease free for 2 years he is probably cured. If a relapse occurs though prognosis is poor.
Term
Osteosarcoma & Ewings Sarcoma account for 85% of primary malignant _________ tumors in children.
Definition
bone
Term
Bone Tumors (osteosarcoma & ewings sarcoma) are most common in males.  highest incidence growing in adolescents.  S/S are similar:     
Definition
LOCALIZED PAIN & SWELLING at tumor site is seen the most, palpable mass, LIMP or decreased ROM.
Term
To diagnose bone tumors:
Definition
need to r/o infection. definitive dx is (CT scans, bone scans, bone biopsy), MRI, LABS: ELEVATED ALKALINE PHOSPHATASE with some bone tumors, LUNG TOMORGRAPHY to r/o metastasis
Term
_____________ Sarcoma is the most frequent bone tumor in children.  It peaks at age 10-25 yrs old.  Where are the primary tumor sites?
Definition
Osteogenesis Sarcoma. Metaphyses of long bones adjacent to the epiphyseal growth plate. More than 505% occur in the distal femur. Other sites: humerus, tibia, pelvis, jaw. METASTASIS TO LUNG FREQUENTLY BY THE TIME DIAGNOSIS HAPPENS.
Term
_________ Sarcoma is the most common frequent malignant tumor in children & adolescents.  Occurs in under 30 yrs old, majority betw 4-25 yrs old.  
Definition
Ewing
Term
Ewing Sarcoma arises in the marrow especially in the femur, vertebre, tibia, ulna, umerus, pelvis, scapula, rubs & skull.  Is metastasis common?
Definition
No, metastasis is uncommon at diagnosis time for Ewing
Term
Treatment of Osteogenic Sarcoma is pretty traditional.  Going to need a pretty radical surgial resection or amputation of affected area.  Usually going to do a resection of bone with prosthetic replacement.  MUST get aggressive chemotherapy after surgery ... why?
Definition
to kill microscopic metastasis to lungs
Term
What is the most common first approach for Ewing Sarcoma?  what is not routinely recommended?
Definition
Radiation. Surgery is not routinely recommended, but considered if radiotherapy renders the extremity useless or if the tumor is resectable. follow radiation by chemo. prognosis is best if no metastasis at time of diagnosis.
Term
Pre-op prep is crucial for bone tumors...
Definition
need to support on concept of amputation, surgical resection. they def will have body image concerns - its already a major issue in an adolescent. Nurses should use an honest approach. Help them to verbalize their feelings/anticipate grieving, talk with someone who has been through it.
Term
Nurses should let people know about
Definition
pain management (phantom limb pain, stump care, prosthesis, rehab) Reactions of anger and depression are normal. Assist family in dealing with diagnosis of malignancy. Manage complications of radiation & chemo.
Term
What's a rotationoplasty?
Definition
remove the diseased area. Then take the lower leg & turn knee so it becomes the hip joint. and ankle joint becomes the knee joint.
Term
______________ is a CONGENITAL malignant tumor that arises from the retina.  may be bilateral or unilateral.
Definition
RETINOBLASTOMA
Term
All bilateral cases of retinoblastoma are ___________.
Definition
Inherited. Unilateral may be inhereited or may not be.
Term
The average age of diagnosis with retinoblastoma is ____ months.
Definition
17
Term
What's the patho of retinoblastoma?
Definition
tumor grows on the retina and grows inward. may be one or multiple tumor sites. Cells break off of the tumor & cause "seeding" throughout the vitreous. these are "Floaters in the eyes" Most cases are diagnosed before metastasis occurs. It rarely extends to the optic nerve to CNS.
Term
What's the most common sign of retinoblastoma?
Definition
Cat's reflex (Leukokoria): a whitish glow seen in the pupil. 2) strabismus - uncoordinated eye other s/s: red, pain in eye, often w/ glaucoma, nystagmus, orbital cellulitis, etc... blindness is a late sign.
Term
Diagnosis of retinoblastoma involves:
Definition
opthalmologist exam under anesthesia (get staging of tumor, tumor size, and location established). Metatastasis is not common at time of diagnosis, it is rare. Retinoblastoma is a disease that can even spontaneously regress..
Term
What's the goal of treatment with retinoblastoma?
Definition
to eradicate the tumor & preserve vision in affected eye. Radiotherapy is the standard of care (shrink it down!) Other approaches: surg implant of Iodine 125 applicator on sclera, photocoagulation w/ laser to destroy blood vessels to tumor, Cryotherapy (freezing the tumor)
Term
What if the optic nerve is involved... what is the treatment of choice?
Definition
Enucleation (eye removal) must be done & is the treatment of choice
Term
Is chemo used for retinoblastoma?
Definition
Only used if it metastasized & it has not been shown to improve survival
Term
What happens if there is bilateral involvement with retinoblastoma?
Definition
Q attempt to preserve useful vision in less affected eye with enucleation of the most severely affected eye.
Term
What is the overall survival rate for retinoblastoma?
Definition
nearly 90%, it's great
Term
What is the most common developing secondary tumor to Retinoblastoma?
Definition
Osteogenic Sarcoma is most common. Children with bilateral form are more likely to develop tumors.
Term
Tell about Enucleation...
Definition
support parent & child & dispel misconceptions, going to remove inside of eye, all external structures are untouched. After surg, a temporary prosthesis will be placed to retain the shape of the eye, observe for infection, hemorrhage and swelling. Need genetic counseling for parents, siblings & affected child.
Term
What are the CARDINAL SYMPTOMS OF CANCER IN CHILDREN??
Definition
Unusual mass or swelling, unexplained PALENESS and LOSS OF ENERGY, Sudden tendency to BRUISE, Persistent, localized pain or limping, prolonged unexplained fever or illness, frequent headaches, often with vomiting, sudden eye or vision changes, excessive, rapid weight loss
Term
How is CA managed in children?
Definition
surgery, radiation, chemotherapy, biologic response modifiers, bone marrow transplantation
Term
 How does radiation work?
Definition
destroys the cell's ability to reproduce by damaging the cell's DNA. rapidly growing & dividing cells are most vulnerable. Normal tissues are most sensitive to radiation: Hematopoietic, Epithelial (skin, mucus membranes, hair), Reproductive
Term
Radiation sd effects on hematopoietic system:
Definition
anemia & myelosuppression & fatigue
Term
Radiation sd effects on skin & hair
Definition
erythemia, desquamation, dryness, itching, alopecia. skin markings for radiation fields.
Term
Radiation sd effects on GI tract:
Definition
N/V, diarrhea, altered taste
Term
Radiation sd effects on Reproductive:
Definition
gonadal sterility
Term
Chemotherapy works by
Definition
all diff chemo agent cytotoxic actions (how they are classified) are going to effect DNA/RNA synthesis, interfere with cell division, and alter cell metabolism in some way. combining drugs allows for optimum cell destruction with minimum toxic effects and decreased resistance.
Term
Is Chemotherapy selective on killing only malignant cells?
Definition
no chemo is not selctive to kill only malignant. most harmful to the rapidly growing cells like: hair, skin, mucous membrane of GI tract, cells in the hematopoietic system, reproductive cells.
Term
Chemo agents require extra precaution of handling & administering...  
Definition
they are vesicants (sclerosing agents) that cause severe damage if it infiltrates into the tissue (extravasation). Venous access devices have greatly facilitated safe & effective drug administration. Risk for anaphylaxis. Careful handling of drugs by nurses is necessary to protect themselves.
Term
Chemo drugs are combined for...
Definition
optimal cell destruction & minimal sd effects. Drugs are cycled for a specific number of times (ie. every 3-4 weeks for 6-12 times according to an established protocol). Use drugs with different NADIRs. Clinical trials are important to investigate new drugs and optimum usage.
Term
What is NADIR?
Definition
the pt when WBCs are platelets are lowest & the pt is most vulnerable. Usually occurs within 7-14 days. Predicts when he client is at greatest risk for infection & bleeding. Recovery of WBCs and platelets is usually within 21-28 days. should monitor with a frequent blood count.
Term
What are Biologic Response Modifiers?
Definition
They're Colony Stimulating Factors. They help stimulate cell growth of specific cells and help reduce the hematologic toxicity of chemo (G-CSF). Neupogen-Neutrophils. Epogen-Neutrophils. Neumega-Platelets.
Term
an __________ bone marrow transplant is where the recipient donated to themself. This is most common.
Definition
autologous
Term
an __________ bone marrow transplant is where an identical twin donated.
Definition
syngeneic
Term
an __________ bone marrow transplant is where the marrow donor is a sibling or parent with similar HLA type.   May even be an unrelated donor through the bone marrow registry.
Definition
allogenic
Term
What do bone marrow transplants allow CA pt's to do?
Definition
receive lethal and potentially more effective doses of chemo & radiation in order to rid the body of all CA cells. BMT's are standard use for leukemia's. Also used with neuroblastoma, Hodgkin disease, non-Hodgkins, rhabdomyosarcoma, ewing & wills tumors.
Term
what happens during 'conditioning' phase of BMT?
Definition
eradicate malignant ells to create space in bone marrow for engraftment of new marrow. Total body irradiation and high doses of chemo are used to totally immunosuppress the pt.
Term
They will then harvest the bone marrow.  Next is pre-engraftment...
Definition
lasts 2-4 wks. marrow can't produce any cells. pt is totally immunosuppressed. High risk for bleeding & infection.
Term
Next is engraftment..
Definition
Where WBC, CBC & platelet counts start to rise
Term
Finally a Bone Marrow Transplant.  Where do they need to be?
Definition
Protective Isolation for several weeks or more. (Laminar air flow, HEPA filters, Neutropenic diet, limited visitors). Vigilant Assessment & monitoring is needed. They are at HIGH RISK FOR INFECTION, BLEEDING, ANEMIA. Can have GI, cardio, hepatic, and renal complications. Monitor for rejection (graft) failure and GVHD (graft vs host disease). Also monitor for infection.
Term
What is Neutropenia?
Definition
Absolute Neutrophil count is WBC x % neutrophils = ANC. IF ANC is below 1000 it is defined as neutropenia. ANC is below 500? Child is at GREAT RISK for INFECTION.
Term

Calculate ANC.  (Bands + Segs) x WBC = ANC.

 If there is 7% segs + 7% bands = 14% or 0.14.  The neutrophils WBC=1000.  try...

Definition
ANC=1000 x 0.14 = 140 (way lower than 500 even, very high risk for infection)
Term
Child with Fever + ANC below 500 merits ____________.
Definition
hospitalization. they're at great risk for sepsis, secondary infection, malaise, dehydration, seizures. Should have culture of every orifice and lines child has. Antibiotics, protective isolation, monitor temp & VS closely, monitor any wound site carefully.
Term
What precautions are necessary with neutropenia?
Definition
Laminar Air Flow Systems, HEPA filtration, Special masks may be used, Avoid using stagnant water in pitchers, no fresh flowers or potted plants in room, Neutropenic diet is: no fresh fruit, vegetables, no raw or uncooked foods.
Term
The administration of _________ to help raise WBCs after chemo more quickly.
Definition
Colony Stimulating Factors (Neupogen).
Term
When neutropenic should be given ________ or _________ 3 times a week for prophylaxis of Pneumocystitis carinni.
Definition
Bactrim or Septra
Term
____________ gel for alpha-strep prophylaxis to teeth & gums.
Definition
Vancomycin
Term
At home, a neutropenic client needs to..
Definition
avoid crowds of people with infection, avoid childre who have been recently vaccinated with live vaccines, If ANC is 500 or below? child should stay home from school. Check temp daily. Continue normal family contact and activities including school as long as ANC is above 500.
Term
Advise parents with neutropenic child that:
Definition
no antipyretics with a fever until the physician has been notified. No immunizations. Good handwashing for the entire family. Get adequate nutrition, fluids and rest.
Term
What is thrombocytopenia?
Definition
Platelets. Less than 100,000? avoid high risk activities. Less than 10,000? risk of hemorrhage
Term
With thrombocytopenia the nurse needs to:
Definition
assess for bleeding. do NOT give any IM injections, no aspirin. Control nose bleeds & blow nose gently. Mouth care (gingival bleeds). administer platelets.
Term
For anemia, blood transfusions are to approach: __ g/dL.
Definition
10
Term
What should be done for anemia?
Definition
Children are able to tolerate the low heme levels. Allow child to regulate activity with reasonable adult supervision. Inform school teachers of potential limitations to strenuous activity.
Term
What should be done for Nausea & Vomiting?
Definition
Administer the antiemetic prophylactically (about 30 minutes to an hour before chemo starts). Then regular administration every 2-4-6 hours for at least 24 hours after chemo. Some evidence beginning antimetic therapy up to 24 hours before is helpful. Avoid development of anticipatory symptoms. Give chemo at night w/ a mild sedative is helpful also.
Term
What should be done for altered nutrition?
Definition
Needs assessment of nutrition status. Should continue evaluating throughout treatment. Get ht, wt & head circumference. Look at labs: albumin, transferrin and albumin. Give Oral Supplements with high-protein and high-calorie foods. Calorie Counts.
Term
what is done for altered nutrition and anorexia?
Definition
increase calories (substitute crm for milk**, full fat yogurts & ice crms, cook with butter, use real sugar, high cal snacks, enteral feeds may be necessary, pareteral hyperalimentation is some cases)
Term
what is done for altered nutrition & anorexia?
Definition
bring fave foods from home or take out! Eat small frequent males. avoid any bad odors that would deter eating. Encourage parents to relax pressures placed on eating. Some kids don't want food during chemo... avoid offering their favorite food during this time. Otherwise allow child to eat any food tolerated at any time.
Term
What can be done for mucosal irritation (stomatitis or mucositis)?
Definition
ulcers are anywhere along the alimentary tract, bland soft moist diet, eat foods COLD or at ROOM TEMP, encourage fluids through a straw, avoid acidic foods, use sponge toothbrush or cotton tipped applicator to brush teeth after eating.
Term
What else can be done to reduce mucosal irritation?
Definition
rinse mouth with NORMAL SALINE rinses, for infant: swab gums with gauze soaked in saline, clean mouth both before & after eating and at night. Local anesthetics without alcohol. Systemic pain meds. Meticulous toilet hygiene, stool softeners.
Term
Some chemotherapy can cause neurotoxic effects such as constipation due to decreased bowel innervation.  We know that opioids given for pain can make this worse.  What can make it better?
Definition
physical activity, ambulation, stool softeners, and laxatives.
Term
After chemo, neuropathy can occur & cause a...
Definition
foot drop, weakness & numbness in feet so bad... starts with the tingles & can progress to where walking it very difficult.
Term
What is Post irradiation Somnolence (brain tumor)?
Definition
Sleeping for long period after brain tumor removal
Term
_________ ________ is  a side effect of chemical irritation of bladder & chemotherapy or radiation.  
Definition
Hemorrhagic Cystitis
Term
Hemorrhagic Cystitis can be prevented by giving...
Definition
liberal oral or parenteral fluid intake (1.5 times the recommended intake), frequent voiding, administer chemo early in the day, administer Mesna (inhibits urotoxicity)
Term
What skin care should be done?
Definition
assess skin for erythema, pain and dry or moist desquamation (peeling). keep area clea with tepid water & soft washcloth, use mild soap sparingly. Do not remove markings on skin for treatment purposes. Avoid sun exposure. Do not use powders, ointments, lotions, deodorants, etc...
Term
What should be done for alopecia?
Definition
warn children and fam that this is a strong possibility w/ chemo & radiation, warn that hair falls out in clumps, often choose to cut hair short or shave head, consider buying a wig, use mild shampoos & soft brushes, protect the scalp (use hat & sunscreen).
Term
what should be done for steroid effects?
Definition
INCREASE APPETITE, Promote a sense of well-being as much as possible, the will have alterations in body image (d/t the puffy face, wt gain, wide range of mood changes). Need to ASSURE THEM THE EFFECTS ARE TEMPORARY. Encourage them to share their feelings (parent & child).
Term
What is the long term after affects of disease & multimodal therapy are:
Definition
psychosocial, cognitive, emotional, and phys development may be effected by treatments and disease. G & D issues, reproductive dysfunction, neuro deficits, organ fibrosis, cardiomyophaty, 2nd malignancies.
Term
What needs to be considered before & after treatments?
Definition
the importance of follow-up after completion of therapy to monitor for long-term and late effects and 2ndary malignancies. Genetic counseling when child reaches adulthood. Reproductive concerns.
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