Questions to ask your doctor about managing sickle cell disease

Empower yourself: Questions to ask your doctor about managing sickle cell disease

(BPT) – Being an informed patient with sickle cell disease has become an important part of health care today. Physicians are increasingly looking upon their patients (and the parents of their young patients) as active partners. In light of this, consider preparing a list of specific questions before visiting your doctor to help start the vital conversation about your health.

For patients with sickle cell disease — a rare but debilitating blood disorder — one focus of the questions might include the creation of a plan for overall health maintenance to help prevent the occurrence of additional illnesses, which can quickly become dangerous to people with sickle cell disease.

“The best defense is prevention,” said Joylene John-Sowah, MD, of the National Heart, Lung, and Blood Institute (NHLBI), part of the National Institutes of Health. “Common illnesses can affect individuals with sickle cell disease much more severely than they affect others. If their physicians do not already have them on a health maintenance plan, there are a few simple questions patients can use to start the conversation with their doctors and potentially help set their care on a better path.”

NHLBI offers these questions as examples of topics patients might discuss with the specialist managing their care:

* I’d like to develop a health maintenance plan for my sickle cell disease. How do I get started? This plan should involve having the routine immunizations and screenings recommended by the Advisory Committee on Immunization Practices (ACIP). It also should include preventive measures such as hydration and avoiding exposures to extreme weather conditions.

* What can I do to prevent infections? Talk to your doctor about the use of vaccines to prevent infections. An expert group brought together by the NHLBI recommends three important steps that should be taken for children with sickle cell disease to help guard against potentially life-threatening illnesses like pneumonia and meningitis: 1) Administer penicillin to children beginning in early infancy and continuing through at least 5 years of age. 2) Vaccinate children against such organisms as pneumococcal and meningococcal bacteria. 3) In the event of a fever, take children to a doctor immediately due to the risk of severe bacterial infections.

In addition, everyone should have the typical childhood vaccines such as for measles, mumps, and rubella and flu vaccinations per the ACIP recommendations. Adults should also receive booster shots of the tetanus and diphtheria vaccine every 10 years.

* Is hydroxyurea right for me? Hydroxyurea is an oral medication that comes in the form of a capsule. It is used to treat a variety of disorders. In sickle cell disease, it helps reduce the frequency of pain episodes (or “crises”) and acute chest syndrome. The expert group that NHLBI brought together recommends wide adoption of hydroxyurea for the care of individuals with sickle cell disease due to its potential to improve their quality of life.

Use of hydroxyurea requires frequent testing of a patient’s blood to be effective. For the first two months, a patient might need to come in for testing every two weeks, and after an effective and tolerated dose is reached, once every two to three weeks. During these visits, the patient’s dose might be altered. If your doctor decides that hydroxyurea might be good for you, develop a plan and schedule for regular visits to get the most out of the therapy.

* Should blood transfusions be a part of my care? Blood transfusions can be an effective therapy for individuals with sickle cell disease. The expert group recommends physicians give regular blood transfusions to children with sickle cell disease who have had a stroke or have an increased risk of stroke as determined by special testing to help reduce their risk and establish for the first time a stroke-free generation. Blood transfusions are also used as therapy for symptomatic anemia, acute chest syndrome, and some acute spleen disorders. Those receiving frequent transfusions should be monitored closely for side effects like iron buildup.

Bone marrow transplants performed in children can cure sickle cell disease, but only a small percentage of these young people are eligible for the procedure. Currently, there is no widely available cure for children or adults. Talk to your doctor about taking some preventive care steps through a health maintenance plan to potentially improve your health and quality of life, and see if you can help advance sickle cell disease research through participation in a clinical trial (

To learn more about sickle cell disease, visit NHLBI’s website: NHLBI’s “Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014” can be found at: The ACIP immunization recommendations are located at