Term
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Definition
common genetic disorder in US
affects approximately 1 in every 350 African American newborns
characterized by predominance of hemoglobin S
most common type is hemoglobin SS
other sickle hemoglobinopathies:
hemoglobin SC - another recessive trait; SC is the second most common cause of sickle cell disease; less severe than SS
beta thalassemia - decreased production of hemoglobin; patients can inherit both this and hemoglobin S
sickle cell trait - carriers of sickle cell; no signs or complications of the disease |
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Term
| pathophysiology of sickle cell disease |
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Definition
hemoglobin A consists of 2 globin chains: alpha and beta
substitution of valine for glutamic acid in 6th position of beta chain results in hemoglobin S or sickle hemoglobin
various conditions may cause hemoglobin S to change shape or sickle
sickling of hemoglobin S leads to deformed red blood cells that are rigid and inflexible; the red cell membrane is altered causing adhesion to endothelial cells
under normal conditions, it is not problematic, but under low oxygen conditions (too much exercise, cold, extreme changes in temperature) can cause cells to become sickle shape
sickled hemoglobin results in:
red blood cell trapping
impedance of blood flow - obstruction, hypoxia, infarction
break down of damaged, rigid cells resulting in anemia |
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Term
| signs ans symptoms of sickle cell disease |
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Definition
pain
anemia
fatigue
pallor
abdominal pain
hematuria |
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Term
| diagnosis of sickle cell disease |
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Definition
newborn screening: all newborns in MO are screened at birth
hemoglobin electrophoresis: definitive test used to diagnose sickle cell syndromes |
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Term
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Definition
most common manifestation of sickle cell anemia
onset correlates with fall in Hgb F (fetal hemoglobin)
pain is repetitive and unpredictable
leading cause of hospitalization
precipitating factors include infection, fever, dehydration, exposure to cold temperatures, and stress |
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Term
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Definition
stroke
second leading cause of death
occurs in about 10% of patients with Hgb SS disease
high recurrence rate
hemiparesis, dysphasia, and seizures
acute management
chronic transfusion therapy to prevent another stroke from occurring |
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Term
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Definition
presence of pulmonary infiltrate on chest x-ray
cause may be infection or infarction
presentation includes fever, cough, tachypnea, chest pain, and hypoxia
treatment includes oxygen, IVFs, narcotics, antibiotics, transfusion |
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Term
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Definition
painful swelling of the back of hands and feet
usually the first manifestation of the disease
treatment includes fluids and analgesics |
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Term
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Definition
painful erection of penis lasting a few hours to days
causes extreme pain which may lead to urinary retention and impotence
treatment includes analgesics and transfusion |
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Term
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Definition
considered a medical emergency
most acutely dangerous crisis in young patients
< 5 years of age at highest risk
characterized by severe anemia, splenomegaly, and hypovolemic shock
treatment includes IVFs and transfusion
signs and symptoms:
irritability
unusual sleepiness
looks pale
weakness
fast heart beat
big spleen
pain on the left side of the abdomen
if a patient has an episode of splenic sequestration there is a very high likelihood that they will have another episode
spenectomy is the treatment to avoid it from happening again |
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Term
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Definition
occurs when bone marrow slows or stops new red blood cell production
often occurs following a viral illness
presentation includes fatigue, severe anemia with few reticulocytes, and dyspnea
therapy is supportive and may include a transfusion
signs and symptoms:
paleness
lethargy
"not feeling good"
headache
fever
low blood count (anemia)
recent upper respiratory infection
passing out (fainting) |
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Term
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Definition
major cause of morbidity and mortality
organs most frequently affected by vaso-occlusion are frequent sites of infection - lungs, bone (osteomyelitis)
presentation includes fever, chills, and leukocytosis
treatment includes prompt administration of antibiotics and vigilant monitoring |
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Term
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Definition
chronic anemia causes delay in growth and development
bone changes secondary to vaso-occlusion may lead to osteoporosis
decreased blood flow and occlusion of small capillaries causes retinopathy in as many as 90% of patients |
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Term
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Definition
decrease number of sickle cell crises
decrease complications
improve quality of life |
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Term
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Definition
thorough assessment
hydration
non-pharmacologic therapies:
heating pad
whirlpool
relaxation techniques
opiate analgesics:
MORPHINE IS THE GOLD STANDARD TREATMENT!
IV morphine q2-3h OR IV morphine continuous infusion
IV hydromorphone q3-4h
IV fentanyl continuous infusion (fentanyl can only be given as a continuous infusion b/c of its short t1/2)
common ADRs of morphine: constipation
itching (more histamine release with morphine than the other opiates; #1 reason to chose another product); use diphenhydramine or buprenorphine (opioid agonist/antagonist) in low dose which antagonizes the side effect of itching, but not the analgesia
PO codeine q4-6h (used in infants)
PO morphine q4-6h
PO oxycodone q4-6h (PO product that is first line)
MS Contin - peak analgesia in 2-4 hours; duration of action 8-12 hours
oxycontin - peak analgesia in 2-3 hours; duration of action 12 hours (works immediately so no need for overlap of another immediate acting opiate as there is with MS Contin)
NSAIDs:
ibuprofen - good for bone pain; used in combination with morphine
bowel regimens:
docusate
senna or bisacodyl - stimulant laxatives are the drug of choice
miralax |
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Term
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Definition
MOST LIKELY PATHOGENS:
Streptococcus pneumoniae
Haemophilus influenzae
Salmonella
prompt administration of antibiotics necessary
chest x-ray, blood, urine, and throat cultures should be obtained
antibiotic therapy:
cephalosporins - CEFOTAXIME, cefuroxime, ceftriaxone is NOT RECOMMENDED due to risk of hemolytic anemia
vancomycin - if the patient is potentially septic (blood pressure instability)
clindamycin - great penetration into the lung
prophylaxis:
PROPS (penicillin prophylaxis showed significant decrease in deaths from strep pneumo < 5 years) and PROPS II (no benefit after 5 years to give prophylaxis)
penicillin BID starting at age 6 months until 5 years old
alternate therapies - erythromycin, trimethoprim/sulfamethoxazole |
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Term
| management - immunizations |
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Definition
pneumococcal vaccines:
Pneumovax - given to older patients and high risk patients (patients with no spleen); given to SCD patients at age 2 and booster at 5; covers 23 other streps
influenza vaccine yearly
Prevnar - given to young children as part of the routine vaccination series; covers 13 of the most common strains of pneumococcus in the US |
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Term
| transfusion therapy: simple transfusion |
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Definition
transfusion of RBCs - give them blood to dilute their blood volume and decrease adhesion of sickle cells
the body will sense that there is enough hemoglobin A and stop producing hemoglobin S
used for:
aplastic crisis
sequestration crisis
priapism
acute or suspected CVA
acute chest syndrome
severe symptomatic anemia |
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Term
| transfusion therapy: chronic transfusion |
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Definition
prevention of recurrent CVA
recurrent episodes of acute chest syndrome
chronic congestive heart failure
complications of blood transfusions: infections, iron toxicity (patients will also take a chelating agent to prevent iron overload) |
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Term
| transfusion therapy: exchange transfusion |
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Definition
used to alter Hgb level rapidly and to replace sickled cells with normal erythrocytes
used for:
acute impending CVA
acute chest syndrome
refractory priapism
only used during extremely urgent situations |
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Term
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Definition
hydroxyurea - stimulates production of Hgb F
THE ONLY DISEASE MODIFYING AGENT FOR SICKLE CELL DISEASE |
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Term
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Definition
| supplementation needed b/c of accelerated erythropoiesis |
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Term
| bone marrow transplantation |
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Definition
high rate of morbidity and mortality
only curative treatment available to date
unclear of risk vs. benefit - not routinely performed |
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Term
| this strategy is employed in patients with VOC to decrease blood viscosity and dilute blood |
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Definition
| hydration therapy/IV fluids |
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Term
| this agonist/antagonist is used in low doses to decrease the itching associated with IV opioid therapy |
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Definition
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Term
| this opiate of choice is given IV for management of severe pain associated with VOC |
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Definition
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Term
| this is the oral agent of choice for the management of pain associated with sickle cell disease |
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Definition
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Term
| this pain medication is given in addition to opiates and specifically targets bone pain |
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Definition
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Term
| this vaccine should be administered yearly to all patients with SCD |
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Definition
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Term
| this vaccine covers the 13 most common strains of pneumococcus |
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Definition
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Term
| this complication is the major cause of morbidity and mortality in patients with SCD |
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Definition
| infection (stroke is the second leading cause of death) |
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Term
| this procedure is completed after a life threatening episode of splenic sequestration |
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Definition
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Term
| thses pathogens are the most likely cause of infection in patients with SCD |
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Definition
H. influenza
Strep pneumo
salmonella |
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Term
| this IV antibiotic is the initial drug of choice in febrile patients with SCD |
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Definition
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Term
| this simulant laxative is commonly prescribed to prevent opioid induced constipation |
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Definition
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Term
| this is considered the only current cure for SCD but is role in therapy is controversial |
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Definition
| bone marrow transplantation |
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Term
| this is a necessary supplement given to support the accelerated erythropoesis in patients with SCD |
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Definition
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Term
| this medication is given prophylactically to significantly reduce the incidence of acute chest syndrome |
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Definition
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Term
| this is the fastest way to lower Hgb S levels in emergent situations like in acute chest syndrome and impending CVA |
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Definition
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Term
| this IV antibiotic may be given as the initial drug of choice in febrile patients with SCD who are allergic to cephalosporins |
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Definition
| clindamycin or vancomycin |
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Term
| this medication is utilized to decrease the frequency of VOC by increasing the production of Hgb F |
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Definition
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