guidebook. - Fanconi Anemia Research Fund

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Chapter 8: Hearing and Ear Abnormalities in FA 177 National Institute on Deafness and Other Communication Disorders Information Clearinghouse 1 Communication Avenue Bethesda, MD 20892-3456 800-241-1044; 800-241-1055 (TTY) nidcdinfo@nidcd.nih.gov Self Help for Hard of Hearing People (SHHH) 7910 Woodmont Avenue, Suite 1200 Bethesda, MD 20814 301-657-2248; 301-657-2249 (TTY) info@hearingloss.org References 1. Giampietro PF, Adler-Brecher B, Verlander PC, et al. The need for more accurate and timely diagnosis in Fanconi anemia. Pediatrics 1993; 91: 1116-1120. 2. Kim HJ, Brewer CC, Zalewski C. Otologic manifestations in Fanconi anemia. The Triologic Society Meeting. Combined Otolaryngology Spring Meeting. Chicago, IL, 2006. 3. Roberts JE, Wallace IF, Brackett D. Pediatric Otology and Neurotology. Lalwani AK, Grundfast KM, eds, Philadelphia, PA, Lippincott-Raven Publishers; 1998. 4. The Individuals with Disabilities Education Act (IDEA) 1997. http://www.ed.gov/offices/OSERS/Policy/IDEA/the_law.html 5. Krueger WW, Feghali JG, Shelton C, et al. Preliminary ossiculoplasty results using the Kurz titanium prosthesis. Otology and Neurotology 2002; 23: 836-839. 6. Tjellstrom A, Hankansson B, Granstrom G. Bone-anchored hearing aids: current status in adults and children. Otolaryngolic Clinics of North America 2001; 34: 337-364. 7. Soulier J, Leblanc TL, Larghero J, et al. Detection of somatic mosaicism and classification of Fanconi anemia patients by analysis of the FA/BRCA pathway. Blood 2005; 105: 1329-1336.
Chapter 9 Matched Sibling Donor Hematopoietic Stem Cell Transplantation Farid Boulad, MD Matched sibling donor (MSD) transplantation for Fanconi anemia is currently the best therapy available to cure the FA patient of marrow aplasia, to prevent progression to myelodysplasia or leukemia or cure the myelodysplasia or leukemia if they are already present. Overview In the early 1980s, the use of high-dose cyclophosphamide and radiation in preparative stem cell transplant regimens for FA patients from matched sibling donors often resulted in excessive organ toxicity and death in the early post-transplant period. Eliane Gluckman, MD, Hôpital St. Louis in Paris, showed that the extreme hypersensitivity of FA patients to high dose alkylator therapy or irradiation was an inherent aspect of the disease. 1 The use of low doses of cyclophosphamide (20-40 mg/kg) combined with 400-600 cGy of thoracoabdominal or total body irradiation resulted in reduced toxicity, substantially improved the outcome for the FA patients transplanted from HLA-matched donors, and became the standard of care cytoreductive regimen for FA patients transplanted from matched sibling donors. In the 20 years since, and through collaborations sponsored by the Fanconi Anemia Research Fund and the IBMTR/ABMTR, 2 physicians from a number of hospitals with expertise in FA transplants have been working
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Chapter 4 Gastrointestinal, Hepatic
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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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