Supportive Medications for Sickle Cell Disease

Many different medicines can help manage or prevent complications of sickle cell disease (SCD). SCD increases vulnerability to infections, especially during childhood. These can be deadly or cause other complications of SCD. Preventing infections with antibiotics and immunizations has greatly improved childhood survival rates.

Medicines to manage pain are also common in SCD. This includes pain medicines used at home and in a hospital. Other medicines may also be necessary, depending on your specific complications.

Penicillin and other antibiotics

Until the 1990s, up to 30 percent of young children with SCD in the United States died from infections. The most common causes of infection are certain types of bacteria, such as pneumococcus. This is because SCD damages the immune system’s response to these types of bacteria, especially if there are spleen problems.^1,2

It is now common for children with SCD to take prophylactic (preventive) penicillin twice a day from 2 months old to 5 years old. This has reduced the incidence of pneumococcal infections in children with SCD by 85 percent.^2-4

The success of preventive penicillin is the main reason why every newborn is screened for SCD. Screening followed by prophylactic penicillin has reduced deaths in the first 5 years of life from 25 percent to less than 3 percent.^2,5

Upon diagnosis, newborns can begin taking a liquid form of penicillin. Older children will then take tablets. Many children stop taking penicillin after the age of 5. Some people continue into adulthood, especially those who have had a pneumococcus infection or had their spleen removed. Long-term penicillin can cause resistance to the antibiotic, so the decision to continue must balance the risks and benefits.^1,4

Immunizations

Routine vaccinations and universal pneumococcal vaccinations have also decreased childhood mortality from infections. The first pneumococcal vaccine decreased the rate of infections in children under 3 years old by over 90 percent.^1,6

There are 2 types of pneumococcal vaccines that are regularly used:^7,8

• Pneumococcal conjugate vaccines, including Prevnar (PCV13) and Vaxneuvance (PCV15)
• Pneumococcal polysaccharide vaccines, including Pneumovax (PPSV23)

Your doctor will determine when to give which vaccines. Usually, infants receive a series of Prevnar or Vaxneuvance vaccines starting soon after birth, and a Pneumovax vaccine should be given around the age of 2 (at least 8 weeks after the last Prevnar dose).⁸

Another Pneumovax dose should be administered 5 years after the first Pneumovax vaccine. Children and adults should also get all routine vaccinations, including the annual flu shot. People with SCD may also have special vaccination schedules for meningococcus and haemophilus influenzae type b (Hib).⁸

Pain medicines

Pain is the most common symptom of SCD. It is most often caused by an acute sickle cell crisis (also called a vaso-occlusive crisis) but can be caused by other complications. If the pain is caused by an acute sickle cell crisis, pain medicines should be given right away.⁹

Most episodes of acute pain are treated at home with non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen and diclofenac. When home management does not relieve pain, treatment in an ER is necessary. The goal is to begin relieving pain within 30 minutes of arriving at the hospital. Unfortunately, many people with SCD see delays in treatment because of racial bias and stigma surrounding the disease.^10-12

The dose and type of pain medicine used in the ER depends on:⁹

• Your recent pain medication use
• Location and intensity of pain
• Other symptoms
• What has worked well in the past

Doctors usually treat moderate-to-severe pain with opioids. Codeine and oxycodone are oral (by mouth) drugs that may be used for moderate pain, while morphine and dilaudid are intravenous (IV) and may be used for more severe pain. Opioids may be given as an intravenous injection (in the vein) or subcutaneously (under the skin).^10,13

A continuous infusion can be recommended to avoid repeated injections in the hospital. This lets you control your own pain, which is why it is called patient-controlled analgesia (PCA). PCA pumps allow you to add single doses of pain medicines during the infusion, as needed.⁹

As the pain subsides, opioids will gradually be reduced. After leaving the hospital, you may be given oral opioids to manage pain at home and prevent withdrawal symptoms. You may also try drug-free approaches to manage pain, such as heat application, massage, relaxation, and deep-breathing.^3,11

Other medicines

Sickle cell disease is much more than a pain condition. People with SCD experience chronic and acute complications in many parts of the body. These complications vary in severity for each person. Depending on your specific complications and procedures, your doctor may recommend other medicines. These may include:¹⁰

• Asthma drugs
• Iron chelation therapy to prevent iron overload from blood transfusions
• Skin medicines and ointments to treat leg ulcers
• ACE inhibitors to manage or prevent kidney disease
• Antidepressants

Other things to know

Remember that all medicines have potential side effects. Side effects may very depending on the drug you are taking. Talk to your doctor about what to expect when taking medicines for SCD. You also should call your doctor if you have any changes that concern you when taking one of these drugs.

Before beginning treatment for SCD, tell your doctor about all your health conditions and any other drugs, vitamins, or supplements you take. This includes over-the-counter drugs.