What is Sickle Cell Disease?

Test Your Knowledge

Sickle Cell Disease is an inherited red blood cell disorder characterized by abnormal hemoglobin. Pain is a major symptom of the disease.

Take this quiz from the National Institutes of Health.
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1 Sickle cell disease is considered a pediatric disease, because few adolescents survive into adulthood

That is correct.

The correct answer is FALSE.

In the 1970s few children survived into adulthood, but with interventions such as newborn screening, penicillin prophylaxis and hydroxyurea, over 95% of children with SCD reach their 18th birthday.

2Sickle cell patients only experience acute painful episodes called "crises"

That is correct.

The correct answer is FALSE.

Many adults with SCD suffer from chronic pain in addition to acute painful episodes.

3Hydroxyurea effectively alleviates the chronic pain in adult patients

That is correct.

The correct answer is FALSE.

While hydroxyurea is the frontline treatment for vascular aspects of sickle cell disease, it does little if anything to alleviate the chronic pain.

4Hematopoietic Stem Cell Transplant, though rare, is considered a cure for sickle cell disease

That is correct.

The correct answer is TRUE.

Most transplants are performed in children who have had major complications. HSCT is considered more risky in adults.

5Acute and Chronic Pain is easily managed with opioid medications

That is correct.

The correct answer is FALSE.

Patients with sickle cell disease struggle with the same side effects of opioid medications except that the effects are worse because higher doses of opioids are often needed due to higher opioid clearance.

6Renal failure is the leading cause of death in adults with SCD

That is correct.

The correct answer is FALSE.

Cardiopulmonary complications are the leading cause of death in adults. However, renal dysfunction in SCD is very common with chronic kidney disease affecting about one-fifth of the population.

7Children with SCD are not likely to suffer from strokes

That is correct.

The correct answer is FALSE.

Children can suffer from silent cerebral infarcts that may go unnoticed and can impact their cognitive function.

8Sickle Cell Disease can affect all races and ethnicities

That is correct.

The correct answer is TRUE.

While SCD prevalence is highest in African-Americans, Hispanics are also affected at a higher rate than people with non-Hispanic and African American ancestry. Further, Mediterranean, Indian and Caribbean and Middle Eastern populations are also affected.

9The transition from pediatric to adult care, in patients with sickle cell disease is considered a risky time for high morbidity and mortality

That is correct.

The correct answer is TRUE.

Many young adults with SCD use the emergency department as their primary method of treatment. Studies have shown young adults with SCD are subject to discrimination and bias that reduces their quality of life and affects their health outcomes.

10The majority of physicians know how to most effectively treat patients with sickle cell disease

That is correct.

The correct answer is FALSE.

Physicians who have not had experience with this patient population may not know how to treat them. This is particularly true in certain rural areas of the US.

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