Term
| How many people does cystic fibrosis affect? |
|
Definition
|
|
Term
| What kind of disease is cystic fibrosis? |
|
Definition
| Autosomal recessive genetic disorder. |
|
|
Term
| What is your chance of getting cystic fibrosis if both parents have the trait |
|
Definition
| 1:4 chance with each pregnancy |
|
|
Term
| Definition of cystic fibrosis |
|
Definition
| dysfucntion of exocrine glands which increases viscosity of mucus gland secretions. |
|
|
Term
| 6 places cystic fibrosis affects |
|
Definition
sweat glands
salivary galnds
respiratory system
GI track
pancreas
Reproductive track |
|
|
Term
| What happens at cellular level with cystic fibrosis |
|
Definition
| Transmembrane conductance regular is an enzyme that usually controls the opening and closing of gated ion channels in the transmembrane area. in cystic fibrosis patient, it keeps this channel closed which trapps sodium and chloride inside the cell. |
|
|
Term
|
Definition
sweat test
genetic testing |
|
|
Term
|
Definition
| done with chorionic villi and amniocentisis can be done prenatally. |
|
|
Term
| sweat test (to diagnosis) |
|
Definition
requires 100mg of sweat for two site on body. (simple, painless, inexpensive test)
You are considered positive if NACL count is greater than 60 meQl/L |
|
|
Term
| When do clinical manifestations occur |
|
Definition
| can occur at birth or develop over time. |
|
|
Term
| seven clinical manifestation of disease |
|
Definition
-failure to thrive
-recurring lung infections (pneumonia)
-pancreatic insufficiency
-gastrointestinal symptoms
-sinusitis
-loss of pulmonary function
-cystic fibrosis related diabetes |
|
|
Term
| What is failure to thrive? |
|
Definition
chronic diarrhea
from nutritional deficiency
commonly treated by supplementing meals with pancreatic enzymes. |
|
|
Term
| What causes lung infections (three bacteria) |
|
Definition
staphylococcus, pseudomonas, Bcepacia
also scarring leads to respiratory insufficiency |
|
|
Term
| how are recurrent lung infections controlled (two things) |
|
Definition
|
|
Term
| What happens with pancreatic insufficiency |
|
Definition
| impaires digestion of fats and protein. Leads to malabsorption of nutrients. |
|
|
Term
| How is pancreatic insufficiency treated (2 ways) |
|
Definition
| enzyme supplementation orally and fat soluble bvitamines |
|
|
Term
| 6 Common gastrointestinal symptoms that can develope |
|
Definition
rectal prolapse
weight loss
steatorrhea
abdominal distention
fatus
meconium ileus |
|
|
Term
| 2 things sinusitis effect |
|
Definition
|
|
Term
|
Definition
| irreversible dilation and destruction of bronchial walls. |
|
|
Term
| CFRD (cystic fibrosis related diabetes) |
|
Definition
| occurs in 1-2% of patients combination of type 1 and type II diabetes. |
|
|
Term
|
Definition
|
|
Term
|
Definition
| result of biliary obstruciton from thick mucus |
|
|
Term
| If portal hypertension develops what is only therapy? |
|
Definition
|
|
Term
| How does CF affect reproductive status |
|
Definition
delays puberty
98% of men are sterile
20% of women cannot get pregnant |
|
|
Term
| (5 approaches) multi-pronged approach to manage pulmonary |
|
Definition
airway clearance
Medications :mucolytics,
antibiotics
anti-inflammatory
Nutrition: high calorie diet and taking panreatic enzymes |
|
|
Term
| Three things to remember when preforming airway clearance test |
|
Definition
pretreat with beta 2 agonist (albuterold)
preform on empty stomach
CPT protect skin with shirt |
|
|
Term
| What is chest physio therapy |
|
Definition
| postural drainage- technique used for loosening mucus in airway so that it can be tapped off. |
|
|
Term
| Hypertonic saline as CF treatment |
|
Definition
| new therpay ajunct to pulmozyme |
|
|
Term
| Dornase Alpha (pulmozme) treatment for CF |
|
Definition
Mucolytic that is nebulized
well tolerated, increases FEV1 by 15%
expesnsive. |
|
|
Term
| When do you discuss transplant options ( two things) |
|
Definition
When FEV 1 is at 30%
patient has stable weight |
|
|
Term
| What might disqualify someone from a lung transplant (1 thing) |
|
Definition
| multiple resistant organisms in sputum |
|
|
Term
| Double lung cadaver transplants |
|
Definition
| hard to find donor and costly procedure long wiat |
|
|
Term
| Living donor of lung transplant |
|
Definition
300% possible mortality
not practiced |
|
|
Term
| Two common bacteria seen in early childhood cases |
|
Definition
|
|
Term
| 1 common bacteria seen in adolescence |
|
Definition
|
|
Term
| When are antibiotics given ( 2 times) |
|
Definition
| for acture illness and prophylaxis (prophylaxis: preventative measure taken to decrease the chance of getting an acute illness) |
|
|
Term
| When are high dose ibuprofen PO given to P/T |
|
Definition
| As an anti-inflammatory, given to slow rate of decline in lung fucntion. this does cause long term consequence like renal failure |
|
|
Term
| Why is arithromycin given to p/t"s and how often is it taken? |
|
Definition
As an anti inflammatory
This is taken three times a week per oral
proven to decrease exacerations/hospitalizations |
|
|
Term
| What does nutritional therapy do? |
|
Definition
| good nutrition = growth improvement = stable pulmonary fucntion |
|
|
Term
| 4 things diagnosis of CF based on... |
|
Definition
family hisotry
increased sweat electrolyte content
absent pancreatic enzymes
chronic pulmonary involvement |
|
|
