Sickle Cell Disease: Population Impacts

Sickle cell disease (SCD) affects about 100,000 people in the United States. Most people who have SCD in the United States are Black. It is also common in Hispanic Americans.¹

Because SCD is a genetic disease, people must be born with it. Children begin showing symptoms around 5 months old. Symptoms and complications then tend to get worse with age. The transition from pediatric to adult care is also linked to more medical problems.¹

There is no difference in the rate of men with SCD and women with SCD because it is not a sex-linked disease. However, men and women experience gender-specific complications. As life expectancy increases, providing healthcare to older adults is becoming more important.¹

Infants and children

If someone has SCD, it must be present at birth. Most infants do not show symptoms until they are 5 or 6 months old. The first symptoms of SCD usually include:²

• Jaundice (yellowing of the skin or eyes)
• Fatigue
• Swelling of hands and feet

Universal newborn screening in the United States has improved care for people with SCD. Stroke and infection were major causes of death for infants and children with SCD. Measures have helped reduce childhood mortality, including:³

• Early diagnosis
• Stroke screening
• Prophylactic antibiotics (the use of antibiotics before surgeries or dental procedures)
• Vaccinations have reduced childhood mortality

Now, more than 90 percent of people with SCD in the United States survive to adulthood.³

Teens

Managing SCD in teens is challenging. Pain crises and other complications may negatively impact:⁴

• School attendance and functioning
• Sleep patterns
• Daily and social activities
• Health services use

Teens with SCD also transition from pediatric care to adult care. This period often leads to more hospital admissions and medical problems. This may be because of:²

• Increased organ damage and disability with age
• Difficulty of independent self-management
• Changes in emotional, social, and school lives happening at the same time
• Fewer adult SCD programs
• Difficulty of finding an experienced doctor

Families can prepare for this transition by searching early for doctors who have experience treating SCD in adults. Be prepared to educate doctors who may be less familiar with SCD. Support groups for young people can also be helpful.⁵

Many teens also have poor interactions with doctors while they are in the hospital. Their pain crises are often doubted, underestimated, and undertreated due to healthcare providers’ lack of specialized training and experience in treating SCD. Racial disparities and discrimination in healthcare also contribute to this problem.⁴

Delayed puberty and other physical symptoms (like jaundice and leg ulcers) may lead to low self-esteem in teens. Low self-esteem, self-consciousness, and a desire for a “normal” life can lead to depression and anxiety, poor treatment adherence, and risky behaviors.⁴

Students with SCD may have difficulty concentrating because of fatigue. They may have higher school absences because of severe pain and doctor appointments. Many students with SCD feel that teachers lack empathy and knowledge of SCD. Teachers often view their self-care practices as disruptive to the class. Steps to support students with SCD include:^4,6

• Ensuring access to water
• Allowing frequent bathroom breaks
• Allowing adjustments during extreme temperatures and during physical education
• Taking special care of injuries
• Watching for signs of stroke
• Being aware of their emotional well-being
• Maintaining open communication between teachers and parents

Women

SCD can cause problems specific to women. Women can experience the major complications of sickle cell disease, as well as:⁷

• Delayed puberty
• More pain crises before and during their period
• Increased SCD complications during pregnancy
• Problems during pregnancy for the mother and baby
• Problems getting pregnant
• Pain during sex

Women with SCD who want to have a child should talk to their doctor about how to prepare and reduce the risk of problems during pregnancy. Women with SCD may need special care during their pregnancy to help them and their baby stay healthy. This may include:⁷

• Blood transfusions
• Changes to treatment
• More frequent doctor’s visits
• Pain medicines
• Tests

Men

Some studies have found that men experience more pain crises and more severe complications than women. Men with SCD also tend to have a shorter life expectancy than women. More research must be done to understand why.⁸

Men with SCD often experience an inability to conceive a child (infertility). One common reason for this is priapism (prolonged, painful erections). This happens when blood flow out of the penis is blocked by the rigid sickle cells. Over time, this can cause permanent damage and lead to infertility. Some treatments may also cause infertility.^2,9

Sickle cell disease in older adults

As the life expectancy for people with SCD increases, more older adults need treatments and care. Providing care for older adults is difficult because there are not many doctors with experience treating SCD complications.¹⁰

Half of the people living with SCD over age 50 have suffered permanent damage to at least 1 organ. Pain crises and complications tend to be more serious in older adults. Stress, vision loss, bone health, pain, and other chronic complications must be regularly assessed.^10,11