Patients with sickle cell disease (SCD) require comprehensive care to manage the symptoms and complications of their disease. In addition to managing your disease with actions to remain healthy and pain management, there may be times where more treatment is necessary.
Some sickle cell patients need blood transfusions (simple or exchange) because of severe anemia, before surgery or to help control pain. Emergency blood transfusion is used to treat stroke and acute chest syndrome. Patients who have had a stroke may receive chronic transfusion therapy (blood transfusions every 4 to 6 weeks) to prevent a second stroke and other SCD complications.
Blood transfusions have potential complications including too much iron over time or transfusion reactions like hives and other symptoms of an allergic response. A person’s immune system can also develop antibodies that can make finding matching blood difficult. Blood transfusions also carry a very low risk for infections such as HIV and hepatitis. Any possible complications are checked closely and will be addressed as needed.
If your provider suggests a blood transfusion, you will need blood drawn. You will also need to sign a consent. In the outpatient setting, a new consent is needed each year. Transfusions are given in the Infusion Clinic on the first floor of the Specialty Clinics building at Froedtert Hospital.
Medications to Treat Sickle Cell Disease
Hydroxyurea (Hydrea) is a long-time standard drug that works by several mechanisms, primarily by increasing the fetal hemoglobin in your blood which in turn results in less sickle cell hemoglobin. Studies have shown having fewer sickle cells can help decrease pain, and patients who take hydroxyurea live longer.
If a woman becomes pregnant while taking hydroxyurea, the clinic staff should be contacted. Hydroxyurea is usually stopped during pregnancy.
While taking hydroxyurea, patients need to have monthly blood tests to monitor the medication. This medication can change the blood cell values, and the dose may need to be stopped temporarily.
Other medications include:
- Endari, which is a naturally occurring amino acid called L-glutamine. Taken orally, it helps prevent damage to red blood cells and reduce complications of sickle cell disease.
- Crizanlizumab-tmca (Adakveo®), which is a targeted biologic medication that works by attaching to P-selectin, a protein related to stickiness in your blood. The drug works by making certain blood cells and blood vessels less sticky. It improves blood flow and reduces the number of pain crises you have.
- Voxtlotor (Oxbryta®) is a drug that can reduce sickling and breakdown of red blood cells, allowing mmore healthy red blood cells to carry oxygen throughout the body. This reduces anemia, pain crises and other damage caused by sickle cells in your blood.
Additional Medications to Treat Sickle Cell Disease Symptoms
The following is a list of common medications that are used to help treat and manage SCD and its symptoms.
- Acetaminophen (Tylenol): For fevers and/or mild pain
- Deferasirox (Exjade): Treatment for iron overload due to blood transfusion
- Deferoxamine (Desferal): If unable to tolerate exjade. This medication is given subcutaneous under the skin over 8 hours, 5-7 days a week.
- Gabapentin (Neurontin): For the nerve pain. Works well for numbness and tingling
- Hydromorphone (Dilaudid): For severe pain
- Ibuprofen (Advil, Motrin): Anti-inflammatory for mild pain and headaches
- Ketorolac (Toradol): For mild pain
- Morphine: For severe pain. Remember, narcotics cause constipation.
- Oxycodone (Percocet/Endocet): For moderate pain
- Polyethylene Glycol (Miralax): To prevent and/or treat constipation
- Senna (Senokot): Stool softener
- Tramadol (Ultram): For mild-moderate pain
Sickle cell disease can affect many organ systems. Therefore, it can cause a variety of symptoms and complications. Many times, a patient will need different types of therapies to treat these symptoms and complications. Sometimes, a patient will need to work with other medical specialists.