A Guide to medication used in sickle cell disease

Sickle Cell Disease (SCD) is an inherited blood disorder characterized by sickling of red blood cells. Red blood cells contain hemoglobin, which is responsible for delivering oxygen to all body parts. Sickling of red blood cells in SCD results in blockage of blood flow resulting in recurrent pain crises, infections, anemia and fatigue.

SCD can be managed with the right medications and support. Unfortunately, many patients and caregivers struggle with their medications as they do not understand what they do or how they should be used safely and effectively. When used correctly, medications help to manage symptoms, prevent complications, and improve the quality of life.

Here is a guide on how to effectively use common key medications prescribed for SCD:

1. Hydroxyurea

This is the backbone of SCD therapy for children and adults with frequent crises, severe anemia, or organ complications. It works by increasing the production of fetal hemoglobin (HbF) which prevents sickling of red blood cells as it keeps them round and flexible. Through this, it reduces pain crises, lowers the risk of acute chest syndrome, and decreases the need for blood transfusions.

Pharmacist’s tips:

• Take each dose at the same time everyday.
• Do regular blood tests to monitor safety and the efficacy of the prescribed dosage.
• Common side effects include nausea and low blood count.

2. Folic acid

The purpose of folic acid in sickle cell management is to build healthy red blood cells because SCD causes increased breakdown of red blood cells.

Pharmacist’s tips:

• Take each dose at the same time every day, preferably in the morning.
• Combine with a balanced diet rich in iron and vitamins.
• In case of a missed dose, do not double up doses.

3. Pain relievers (analgesics)

Analgesics are often prescribed to manage pain resulting from vaso-occlussive crises.
Paracetamol is often prescribed for mild pain. NSAIDS e.g. diclofenac and ibuprofen are often used to manage moderate pain, whereas opioids such as morphine are used to manage severe pain during crises.

Pharmacist’s tips:

• Do not ignore early signs of pain, but treat early to prevent it from worsening.
• Avoid overusing NSAIDS as they can cause stomach issues and damage kidneys over time.
• Opioids should be used cautiously due to the risk of dependence, and they should be stored carefully and used under supervision.
• In addition to medication, heat pads, hydration and rest are recommended.

4. Antibiotics

Penicillin V or amoxicillin are commonly used to prevent infections especially in children from 2 months to at least 5 years. SCD patients are prone to common infections such as pneumonia due to spleen damage.

Pharmacist’s tips:

• Never skip doses.
• Watch for signs of allergy such as rash or swelling.
• Store as instructed by your pharmacist or the medication label.

5. Vaccines

Pneumococcal, meningococcal, Hemophilus influenzae (Hib), hepatitis B and a yearly flu shot are recommended for SCD patients in order to prevent infections.

Pharmacist’s tips:

• Keep an updated vaccination card.
• Adult patients are encouraged to get boosters.
• Report any side effects to your healthcare provider.

6. Other supportive medications

Depending on individual patient cases, the doctor may prescribe:

• Antimalarials if in a malaria endemic zone.
• Laxatives if opioids are used and constipation occurs.
• Zinc supplements to boost immunity.

Final dose of clarity

SCD remains to be a public health concern in Kenya and many parts of Africa. Managing SCD requires consistent and informed care that includes a combination of medications depending on individual needs. These medications can help to manage the symptoms if used correctly and monitored regularly. While hydroxyurea remains a cornerstone therapy, pain management and infection prevention play critical roles in disease management.