Treatments for SCD

Long Term treatments for SCD

1. Hydroxyurea:
   • All patients should take it
   • It increases the levels of Hb.F in the RBCs which reduces sickling
2. Folic acid tablets: “LAAZIM” for all patients; bone marrow hyperactive needing nutrients such as folic acid which depleted in body.
3. L-Glutamine (oral):
   • Approved by U.S. FDA
   • Reduces the frequency of vaso-occlusive crises

ALL THE ABOVE TREATMENTS SHOULD BE TAKEN LONG TERM

4. Stem cell transplant: Can “cure” the disease BUT

   • Very costly
   • Limited donor availability
   • Toxicity issues

5. Gene therapy: Still awaiting approval by FDA

   • The abnormal Hb. gene is replaced by a normal gene. Can be a curative treatment

PREVENTIVE MEASURES TO BE DONE

• Avoid dehydration, hypoxia
• Folic acid, hydroxyurea, glutamine
• Vaccinations for Pneumococci, meningococci, H. Flu (why?; spleen preventive measure)
• Annual eye exam, to check for retinal damage
• Due to asplenia → high risk of many infections → so give antibiotic prophylaxis (Oral PCN till the age of 5 years)

TRANSFUSION

INDICATIONS FOR TRANSFUSION IN SCD

• Hemolytic crisis
• Aplastic crisis
• Stroke
• Acute Chest syndrome
• Splenic sequestration crisis

INDICATIONS FOR TRANSFUSION IN SCD (Mnemonic) Hamoud And Saleh Cry Soon

• H = Hemolytic crisis
• A = Aplastic crisis
• S = Splenic sequestration crisis
• C = Chest syndrome
• S = Stroke

Treatment of pain crisis:

a) Analgesia, usually narcotics (morphine) b) Good hydration c) Check for any infection (e.g., UTI, chest infec. & treat) d) No transfusion e) O2 (if hypoxic)