NAPLEX Sickle Cell Disease

What is the normal lifespan of red blood cells?

120 days

What population is most commonly affected by sickle cell disease?

African americans

What happens to cells in sickle cell disease?

The cells are sickle shaped, hemolyze and die after 10-20 days, block small blood vessels, cannot transport oxygen effectively

When do symptoms of sickle cell develop?

2-3 months after birth

What is acute chest syndrome?

Pain in the chest with evidence of a lung infection

Can be life-threatening!

What are some complications of sickle cell disease?

Severe anemia, cholecystitis, infection, multiorgan failure, priapism, stroke, vaso-occlusive crisis, pain

How is pain managed in sickle cell patients?

IV opioids or PCA

How does a healthy spleen aid in sickle cell management?

Removes old red blood cells, aids immune function, clears bacteria (esp. s.pneumoniae, h.flu, and n.meningitidis)

What can happen to the spleen in patients with sickle cell?

Becomes fibrotic and shrinks which causes loss of function (asplenia)

Patients are considered immunocompromised and are at increased risk for serious infections

What prophylactic antibiotic is essential to prevent infection in young children?

Penicillin twice daily until age 5

What vaccines are essential in sickle cell patients?

Haemophilus influenza B

Pneumococcal

Meningococcal

What treatment is typically used to help prevent vaso-occlusive complications?

Blood transfusion

*Monitor for iron overload

What therapy is used to remove excess iron?

Iron chelation therapy - deferasirox, deferiprone

What is the primary drug treatment in sickle cell disease?

Hydroxyurea

What does hydroxyurea do?

Stimulates HbF production

Warnings for hydroxyurea

Myelosuppression, fetal toxicity, avoid live vaccines, macrocytosis

Toxicity with hydroxyurea

ANC below 2k, platelets < 80k

What is required during and 6-12 months after treatment with hydroxyurea?

Contraception

What mineral should be supplemented with hydroxyurea?

Folic acid