guidebook. - Fanconi Anemia Research Fund

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Chapter 1: Clinical Management Checklist 23 and a high risk of squamous cell carcinoma of the lower genital tract, which includes cervical, vaginal, vulvar, and anal cancers. • Gynecologic Examinations: ▫▫ Beginning at age 13, obtain annual examinations by a gynecologist for visual inspection of the external genitalia. ▫▫ Comprehensive annual gynecologic exams with cervical cytology testing (Pap smears) should begin at age 18 and include discussion of STDs and contra- ▫▫ ception. Colposcopy and biopsy should be done if lesions are noted on inspection or if the cervical cytology test is abnormal. • HPV vaccination: Obtain an HPV vaccination series beginning at age nine for prevention of HPV-associated cancers. The safety and immunogenicity of HPV vaccination in FA men and women has yet to be determined. • Reproductive tract anomalies: Assess for reproductive tract anomalies if patient is known to have kidney anomalies. • Breast cancer: Breast cancer surveillance should begin by the early 20s and include annual breast exams. Screening mammograms should be initiated by age 25 or if a mass is detected. • Pregnancy: ▫▫ ▫▫ Discuss childbearing options before transplant, since the transplant may affect future fertility. The patient should not take androgens during pregnancy.
24 Fanconi Anemia: Guidelines for Diagnosis and Management ▫▫ While pregnancy for women with FA is not life-threatening, the pregnancy should be considered high risk and be co-managed by a maternal/fetal medicine specialist and a hematologist. • Menopause: FA patients usually go through premature menopause. Thus, the physician should consider the post-menopausal health risks of osteoporosis, cardiovascular disease, breast cancer, and the management of hot flashes. Squamous Cell Cancer of the Head and Neck Fanconi anemia patients are at extremely high risk of acquiring squamous cell carcinoma of the head and neck (HNSCC). Proper prevention, surveillance, and treatment of HNSCC are essential. If the patient with HNSCC has not been assessed for a possible diagnosis of FA: • Testing for FA should be considered in younger SCC patients (
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Chapter 4 Gastrointestinal, Hepatic
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Chapter 7 Endocrine Disorders in Fa
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Chapter 9 Matched Sibling Donor Hem
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Chapter 12 Novel Treatment Options
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Chapter 13 Head and Neck Squamous C
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Chapter 14 The Adult Fanconi Anemia
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