Chronic Illness Research Paper

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  • Topic: Immune system, Hematopoietic stem cell transplantation, Chemotherapy
  • Pages : 3 (927 words )
  • Download(s) : 301
  • Published : April 18, 2013
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Wiskott-Aldrich syndrome is an X-linked recessive immunodeficiency disorder usually inherited by males from their mother. This syndrome involves both T- and B-lymphocytes characterized in one third of patients by the triad of recurrent bacterial sinopulmonary infections, eczema (atopiclike dermatitis), and a bleeding diathesis caused by thrombocytopenia and platelet dysfunction. The characteristic triad of bleeding, eczema, and recurrent infections in Wiskott-Aldrich syndrome generally become evident during the first year of life, with petechiae and ecchymoses of the skin and oral mucosa and bloody diarrhea being the first clinical signs. Bone marrow transplant improves prognosis of Wiskott-Aldrich syndrome. Investigators reported that before hematopoietic stem cell transplantation, cutaneous manifestations occurred frequently, with the most common lesion being eczema similar to atopic dermatitis (71%), followed by petechiae and/or ecchymosis (58%) and cutaneous infections (17%). The classic triad of symptoms was seen in 46% of the boys, who were later treated with hematopoietic stem cell transplantation. The chronic underlying medical needs, the unpredictable nature of the disease, with its share of life threatening emergencies, all contribute to a stressful life for the family. These families need support from physicians, family, friends, teachers, clergy, neighbors and sometimes professional counselors to help them cope with the disease. Families living with Wiskott-Aldrich Syndrome are challenged by many stressors and often struggle with the imbalance it causes. Some of the stressors of WAS include variability of the disease's presentation, sudden and unexpected onset of the symptoms (infection, bleeds, malignancies and autoimmunity), the lack of consensus in the medical community on how best to treat WAS, and the incurable nature of the syndrome without bone marrow transplantation that in itself carries significant risk of serious side effects including...
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