DELAYED PUBERTY IN SICKLE CELL ANAEMIA – By Dr. Motunrayo Adekunle

Introduction
Puberty is the transition from childhood to adulthood. It is accompanied by physical,
emotional, and behavioural changes. It is characterised by rapid body growth, maturity of
the reproductive organs, and development of the secondary sexual characteristics such as
breasts in girls, testicular enlargement, increased height and voice deepening in boys.
Pubertal development begins between 8–13 years in girls and 9–14 years in boys.
However, in children living with sickle cell anaemia, this process is often delayed.

Reasons for Pubertal Delay in Sickle Cell Anaemia

Puberty begins when the brain releases signals that trigger hormones from the pituitary
gland, an organ in the brain. These hormones stimulate the testes, in boys, to produce
testosterone and the ovaries, in girls, to produce oestrogen. The produced sex hormones
cause the body changes seen in puberty. On the average, puberty in sickle cell patients is
delayed by 1–2 years, and this delay is usually more pronounced in boys.

Several factors contribute to this delay:

• Chronic anaemia: Persistent low haemoglobin levels reduce oxygen supply to
  tissues, limiting the energy available for growth and development.
• Malnutrition: Children with sickle cell disorder often struggle with poor appetite or
  nutritional deficiencies, which slow down normal growth.
• High energy demand: The body uses much of its energy to cope with illness, leaving
  less energy for growth and maturation.

Effects of Delayed Puberty
Delayed puberty affects more than just physical growth. It can also impact a child’s
emotional and social wellbeing. Adolescents may feel different from their peers, leading to
low selfesteem, embarrassment, anxiety or social isolation. These challenges can affect
school performance and also lead to parental stress.

Treatment and Support
Managing delayed puberty in sickle cell anaemia requires a combination of medical care,
nutritional support, and emotional reassurance.

• Reassurance: Families should be informed that although puberty may be delayed, most
  children with sickle cell anaemia eventually reach normal development. This helps reduce unnecessary anxiety.
• Hydroxyurea therapy: This medication improves blood health by reducing sickling of red
  blood cells. Studies show it can support growth and help reduce delays in puberty.
• Nutritional support: A balanced diet rich in proteins, vitamins, and minerals is essential.
  Good nutrition strengthens the body, supports growth, and boosts immunity.
• Growth monitoring: Regular followup visits are crucial to track height, weight, and
  pubertal milestones. If puberty has not started by 13 years in girls or 14 years in boys,
  doctors may investigate further to rule out other causes.

Conclusion
Delayed puberty is a common challenge in children with sickle cell anaemia. While the
onset of puberty may be later than in healthy peers, most affected adolescents eventually
achieve normal development. Affected individuals and their families should be reassured
that there is no need for panic. With proper nutrition, medical care such as hydroxyurea,
and regular growth monitoring, children with sickle cell can progress into adulthood in a
healthy way.

Reference:
1. Sickle Cell Society. Sickle cell disease in childhood: Standards and recommendations for
clinical care. Sickle cell society. 3 rd Edition. 2019. SCD-in-Childhood_Final-version-1.pdf
3. Philippe Backeljauw, George A. Tomlinson, et al. Growth and Puberty in African Children
with Sickle Cell Anemia Treated with Hydroxyurea. Blood, Volume 144, Supplement 1,2024,
Page 544, ISSN 0006-4971, https://doi.org/10.1182/blood-2024-205848.