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Sickle Cell Disease in the Adolescent Female

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Hematology in the Adolescent Female

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Abstract

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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Authors and Affiliations

  1. Department of Pediatrics, University of Texas MD Anderson Cancer Center, Department of Pediatric Hematology Oncology, Houston, TX, USA

    Nelda Itzep

  2. Department of Pediatrics, Baylor College of Medicine, Division of Hematology/Oncology, Houston, TX, USA

    Vivien Sheehan

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  1. Nelda Itzep
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  2. Vivien Sheehan
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Correspondence to Vivien Sheehan .

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  1. Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA

    Lakshmi V. Srivaths

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Itzep, N., Sheehan, V. (2020). Sickle Cell Disease in the Adolescent Female. In: Srivaths, L. (eds) Hematology in the Adolescent Female. Springer, Cham. https://doi.org/10.1007/978-3-030-48446-0_20

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  • DOI: https://doi.org/10.1007/978-3-030-48446-0_20

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