source: Hematology in the Adolescent Female

year: 2020

authors: Nelda Itzep, Vivien Sheehan

Sickle cell disease (SCD) is an inherited blood disorder that causes affected individuals to produce an abnormal hemoglobin that results in frequent pain events, organ damage, and early mortality. Disease- specific treatments include hydroxyurea, L-glutamine, voxelator, crizanlizumab, and blood transfusions. Hematopoietic stem cell transplant is curative but limited due to a requirement for a matched-related donor in most cases. Adolescence can be a difficult time for females affected by SCD. Pain events may increase in frequency, due to decline in protective fetal hemoglobin levels, medication noncompliance, hormone effects, or progression of the chronic illness. Some patients may develop chronic, daily pain in adolescence, from repeated pain crises, or opioid-induced hyperalgesia.

Transitioning from pediatric to adult providers may be a source of anxiety, and developing independence from parents is a challenge. Gynecological issues include a need to avoid estrogen containing birth control products due to the hypercoagulable state of SCD, difficulties in diagnosing dysfunctional uterine bleeding, and the high-risk nature of pregnancy in an individual with SCD. Individuals with SCD have high rates of psychological complications, including depression, anxiety, and body image dysfunction.

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