Delayed Puberty and Sickle Cell Disease
Many teenagers with sickle cell disease (SCD) experience developmental delays because of anemia. Boys and girls with sickle cell anemia may see a puberty delay of about 2 years. Teenagers who have milder types of SCD usually experience a shorter delay.
Boys and girls with SCD also tend to be shorter and weigh less than their peers. Chronic blood transfusions may help children with SCD reach average height and weight.
What developmental complications do people with sickle cell disease experience?
Children with SCD usually develop slower than children without SCD. They will eventually go through puberty, but it may be delayed. People with sickle cell anemia (HbSS) and sickle beta zero thalassemia tend to develop slower than people with sickle hemoglobin C (HbSC) and sickle beta plus thalassemia.1,2
Infants with SCD are usually an average size. But by 2 to 5 years old, slow growth becomes apparent. For boys, the growth delay may continue beyond the teen years. Girls may experience some recovery of height and weight as they go through puberty.3
Girls with HbSS experience menarche (first menstruation) about 2.5 years later than girls without SCD. Girls with HbSC have a delay of about 6 months. Delayed puberty in girls with SCD is linked to lower weight.4
Boys with HbSS reach puberty about 2 years later than their peers. They grow body and facial hair later than expected and may have less body hair as adults. Boys and men with HbSS are also shorter than their peers and weigh less.5
Because of delayed growth and other visible symptoms, many teenagers with SCD have lower self-esteem. They also have a higher risk for:6
- Social withdrawal
- Attention deficit
Why does SCD cause delayed growth and puberty?
People with SCD develop slower because of anemia (low red blood cell count). Sickle cells are unstable and burst prematurely. The body cannot make enough red blood cells to keep up with this destruction. This causes many people with SCD experience complications of anemia, such as delayed growth.2,4
Anemia affects the cardiovascular system and forces the heart to work harder to maintain energy demands. This means people with SCD may need more calories than people without SCD. Poor nutrition may contribute to delayed growth.3,4,7
Many aspects of teenage development can be affected by SCD. Children with SCD can experience:8
- Lower height
- Lower weight
- Lower body mass index (BMI)
- Less facial and body hair
- Delayed stages of puberty
What are treatment options?
Children receiving long-term blood transfusions may reach average height and weight. We do not yet know if transfusions can reduce delays in puberty. We also do not know if hydroxyurea treatment affects growth and puberty.9
Some studies have also shown malnutrition in children with SCD. For example, children with SCD tend to have lower levels of vitamins B6, D, and E, and zinc. However, there are few nutrition guidelines geared toward improving growth and development in children with SCD.3