Sickle Cell Disease

What it is

Sickle cell disease is an inherited condition in which red blood cells stiffen into a curved shape, block blood flow and break down early. The blockages cause sudden episodes of strong pain, called pain crises, that often need medical attention [1].

Why this guide is written for Black patients

Sickle cell disease affects more than 100,000 people in the United States, and more than 90 percent of them are non-Hispanic Black or African American, with an estimated 3 to 9 percent Hispanic or Latino [1]. About 1 in every 365 Black babies is born with sickle cell disease, and about 1 in 13 Black babies is born with sickle cell trait [1].

The part of this that is not biology is what happens in an emergency department. In a study comparing adults arriving with a sickle cell pain crisis against adults arriving with kidney stone pain, the median wait for the first dose of pain medicine was 80 minutes for sickle cell patients and 50 minutes for kidney stone patients [4]. The sickle cell patients reported higher pain on arrival, an average of 8.6 against 7.9 [4]. Worse pain, longer wait, same building.

What to ask a clinician

• What type of sickle cell disease do I have, and what does that mean for how often crises come?
• Am I a candidate for hydroxyurea, and if not, why not [2]?
• What is my written pain plan for a crisis, and can I have a copy to take to the emergency department?
• Which hospital near me has a sickle cell day hospital or an infusion clinic, so I do not have to start at triage [3]?
• Am I a candidate for a transplant or for one of the gene therapies [2]?

What good care looks like

Ongoing care is scheduled, not reactive. Most people with sickle cell disease should see a clinician every 3 to 12 months, depending on age and the type of disease they have [3]. Vaccines matter more here than for most people, including an influenza shot every year and COVID-19 vaccination as recommended [3].

Treatment has more in it than it did ten years ago. Hydroxyurea is an oral medicine that can reduce sickling and help prevent serious symptoms, including pain crises [2]. Red blood cell transfusions raise the number of normal, flexible red cells [2]. A blood and bone marrow transplant is a potentially curative therapy for some people [2]. In December 2023 the Food and Drug Administration approved two gene therapies for sickle cell disease, one that adds a modified gene and one that edits existing genes [2].

Day to day, extreme heat or cold and sudden changes in temperature are common triggers, so ease into water rather than jumping in [3]. Over-the-counter acetaminophen or ibuprofen can handle mild to moderate pain at home [2], and a crisis that outruns that is a reason to go to a sickle cell day hospital, clinic or emergency department for stronger medicine [3].

When it is urgent

Go now for pain that home medicine will not touch [3]. Go now for fever, since infection in sickle cell disease moves fast. Go now for chest pain, difficulty breathing, sudden weakness or numbness, trouble speaking, a change in vision, or a painful erection that will not settle. Take your written pain plan and your medicine list, and if you are able, ask for the plan to be read before the first dose is decided.

What to do next

Care belongs with a hematologist, and continuity matters more here than almost anywhere else. Find Black hematologists and ask, at the first visit, for a written crisis plan you can hand to a stranger at 2am.

If you or your partner has sickle cell trait, ask about testing and counseling before a pregnancy rather than during one [1].