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
