Treatment Overview

Successful treatment of sickle cell disease requires:

• The latest treatment and home care information for families affected by sickle cell disease.
• A partnership between the family, a doctor experienced in sickle cell disease treatment, and other health professionals, as needed, to provide full care for symptoms that involve different body systems.
• Immediate treatment for sudden, serious problems and complications.

Initial treatment

When parents learn that their baby has sickle cell disease, it's the beginning of a lifelong education process. Knowing as much as possible about the disease can help you control symptoms as they arise and know what to do in emergency situations. Initial treatment includes:

• Routine childhood immunizations and other vaccines.
• Daily antibiotics from 2 months to 5 years of age.
• Multivitamin supplements with iron during infancy.
• Folic acid supplements daily.
• Protein supplements if there is a lag in weight gain.

Starting at age 2 years, your child should get screened every now and then with a transcranial ultrasound.⁶ This test measures blood flow in the arteries of the head and neck. If test results show a high chance for stroke, your child may get blood transfusions to lower the risk.²

Ongoing treatment

Children age 1 to 5 with sickle cell disease often receive daily antibiotics, such as penicillin, to prevent life-threatening infections. This practice stops at age 5 because older children don't have as many severe infections.

Routine lab tests to monitor health include:

• Complete blood count (CBC).
• Urine test.
• Tests to monitor the functioning of organs.

Pain is sometimes a chronic problem for people with sickle cell disease. Your doctor or a pain treatment specialist can help you develop pain management skills. These skills include distraction, guided imagery, deep breathing, relaxation, and positive self-talk.

You can sometimes treat mild pain with pain medicines while at home. But call your doctor or go to the hospital if your pain is not controlled.

Severe episodes of prolonged erection of the penis (priapism) need evaluation by your doctor. Treatment may include fluids (hydration), pain medicines, treatment by a urologist, and blood transfusions.

If your child with sickle cell disease is at high risk for a stroke, your doctor may recommend blood transfusions, which may reduce this risk.

Acute chest syndrome may develop after a painful event or another illness and can be life-threatening. Early treatment is very important and may include oxygen, pain medicines, antibiotics, and transfusions.

You may not notice vision problems until damage has begun. Have your child's eyes checked when he or she is a newborn and again at all routine well-child visits.⁷ And get routine eye exams as an adult. Try to go to a doctor who specializes in eye problems (ophthalmologist).

People with sickle cell disease should avoid contact with anyone suspected of having fifth disease, which is caused by parvovirus. Parvovirus can cause the body to temporarily stop making blood cells, a severe life-threatening problem in someone with sickle cell disease. Aplastic anemia can develop as a result of a shortage of red blood cells. It can come on suddenly and is life-threatening if not treated.

Find more home treatment information for sickle cell disease at:

• Home treatment for pain.
• Home treatment for priapism.
• General health tips.

Treatment if the condition gets worse

Treatment for severe cases of sickle cell disease may include:

• Hydroxyurea medicine.
• Blood transfusions.

Organ failure can affect any part of the body in a person who has sickle cell disease. Treatment may include transfusions, medicines, and surgery. Surgery options include:

• Removal of the spleen (splenectomy), to prevent the trapping of too many red blood cells in the spleen (splenic sequestration).
• Removal of the gallbladder (cholecystectomy), to prevent problems caused by gallstones.
• Fluid drainage from the penis in cases of severe priapism.
• Hip replacement, if the tissue in the hip breaks down and dies because it doesn't get enough blood (osteonecrosis).

Identifying children at risk and treating them with blood transfusions may prevent strokes and other nervous system problems. A child or adult with sickle cell disease who has a stroke needs immediate medical help including medicines and transfusions.

What To Think About

Pregnant women who have sickle cell disease need specialized medical care.

A series of blood transfusions is the treatment of choice to prevent strokes and treat other aspects of this disease. But transfusions can cost a lot. And if you get a lot of them, you could develop antibodies that attack and destroy the donor blood. This is called alloimmunization. It occurs in about 1 out of 4 people with sickle cell disease who get frequent transfusions.³

People with sickle cell disease and their families face ongoing stress. A support network (such as doctors, extended family, friends, church, and counselors) can help ease stress and worry.

Painful events and serious complications of the disease can happen suddenly and unpredictably and can become life-threatening. Bouts of severe pain can last for hours to days and are difficult to treat. They're exhausting for caregivers as well as for the person in pain. For more information, see the topic Chronic Pain.