Sickle Cell Disease Flashcards
How many days do sickle cell RBC have before they hemolyze?
10-20 days
When do symptoms of sickle cell disease present?
2-3 months after birth
What is vaso-occlusive crisis?
Acute pain crisis caused by sickle cell disease Most commonly occurs in the lower back, legs, hips, abdomen and chest
What is acute chest syndrome?
vaso-occlusive crisis/acute pain crisis that occurs in the chest and is the leading cause of death in sickle cell disease
What types of contraception should be used in sickle cell disease and why?
Progestin-only, levonorgestrel intrauterine devices (IUDs) and barrier methods are preferred for contraception
NOT ESTROGEN
What organ becomes fibrotic and shrinks in size in sickle cell disease?
spleen
called functional asplenia
What is functional asplenia and what does it cause?
decreased or absent spleen function
increases risk of serious infections
What are acute sickle cell disease complications?
Acute chest syndrome Anemia Cholecystitis Infection Multiorgan failure Priapism Spleen sequestration Stroke Vaso-occlusive crisis
What are the chronic sickle cell disease complications?
Avascular necrosis Leg ulcers Gallstones Pain Pregnancy complications Pulmonary hypertension Renal impairment Retinopathy Recurrent priapism
When to give blood transfusions for sickle cell anemia
After stroke, acute chest syndrome, and severe anemia
Do not push Hgb >10
What is one adverse outcome with monthly blood transfusions for sickle cell anemia
Iron overload
What is the only cure for sickle cell disease?
bone marrow transplantation
What drug classes are used in sickle cell disease?
Immunizations, antibiotics, analgesics, hydroxyurea or L-glutamine, and chelation therapy
What vaccinations should patients with sickle cell disease get?
Haemophilus influenzae type B Pneumococcal conjugate (PCV13, prevnar) Meningococcal conjugate plus boosters Meningococcal serogroup B Pneumococcal polysaccharide (PPSV23, Pneumovax23)
Infants with sickle cell disease should be treated with what antibiotic and for how long?
Penicillin BID until the age of 5 years old
If spleen is removed continue PCN indefinitely
What analgesics are used for patients with pain?
NSAIDs, APAP, can be opioids or PCA
Hydroxyurea MOA
stimulates production of HgbF
How does hydroxyurea benefit patients with sickle cell disease?
reduces frequency of acute pain crises, episodes of acute chest syndrome and the need for blood transfusions
When to use hydroxyurea in patients with sickle cell disease
Adults with 3 or more severe pain crises in one year or with severe or recurrent acute chest syndrome, chronic symptomatic anemia
Hydroxyurea BBW, warning, SE
BBW: myelosuppression, malignancy (leukemia, skin cancer)
Warnings: fetal toxicity, avoid live vaccines
SE: increased LFTs, uric acid, BUN and SCr, mouth ulcers, N/V/D, alopecia, hyperpigmentation
What needs to be supplemented during hydroxyurea use?
Folic acid to prevent macrocytosis
How long to use contraception after using hydroxyurea?
6 months for females
12 months for males
L-glutamine MOA
Amino acid that reduces acute complications of SCD
MOA not fully known but might decrease oxidative stress
L-glutamine side effects
constipation, flatulence, nausea, HA, pain, cough
Oral powder
