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.
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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 death.
They also struggle with the potentiality that their child's symptoms may increase or worsen with age, and, because of the rarity of the syndrome, families are often dealing with a disease that is not well understood and specialists that are often hard to find. These types of acute and prolonged stressors naturally challenge the safety and predictability within the family system and also the emotional stability of all members within including the child affected with WAS as he ages. Although each family is unique and each family member is different in the ways in which he or she esponds to the stress and trauma associated with WAS, many experience moderate to profound grief, anxiety and or depression. Some experience hyper-vigilance, avoidance of situations or places reminiscent of traumatic events associated with WAS, have nightmares or recurring intrusive traumatic thoughts or images associated with the trauma and or isolation. While others may experience panic attacks, sleep disturbances, eating disorders, ADHD-like symptoms, under performance at work or school and or symptoms of oppositional defiance.
Management of infection includes antibiotics and possibly intravenous immunoglobulin G (IVIG). The decision to use prophylactic antibiotics and/or IVIG is made case-by-case, based on incidence and severity of infection in the individual patient. Postsplenectomy, prophylactic antibiotics are mandatory, although the patients who undergo splenectomy remain at considerable risk for overwhelming sepsis despite of prophylaxis. The nurse must teach that immunization is mandatory.
Varicella-zoster immune globulin is administered within 48 hours if possible, although it may be effective until 96 hours post exposure. To manage acute bleeding the nurse’s role is to administer platelet transfusions and packed erythrocytes. The nurse must also insure that she and the healthcare team are minimizing exposure to allogeneic cells in the patient for whom stem cell reconstitution is planned is important because such exposure increases graft rejection rates.
The nurse must teach the parents how to manage symptoms of eczema at home along with an oral antiviral that the child will be receiving. Parents are taught to treat eczema with conventional topical moisturizing creams and topical steroids. Nurses also advise that milk and other potential food allergens may be eliminated from the diet on a trial basis to observe for improvement. Clinical services provided for the family and child coping with Wiskott Aldrich Syndrome include, groups such as the Wiskott Adlrich Foundation.
This foundation is awarded grants to help unraveling the mysteries of WAS, explore avenues for improved cures and find ways to improve the quality of life for those who are impacted by WAS. Resources provided in the U. S. include financial assistance, fundraising assistance, housing-travel and medication, wish granting organizations, and educational scholarships. These assistive programs provide the family with detailed information about their disease, and financial supports to cope with the stresses that come with treating and the management of WAS.
When dealing with WAS the family is taught that nothing is more important to the health of a child with WAS than warding off potential infections. Basic precautions including: keep the child away from crowds, dirty environments and sick people; use protective face masks at the recommendation of the child’s doctor; following a strict hand washing regimen for the child, family, and visitors. These actions are taken because the child’s body does not have healthy B cells that produce antibodies against infection, they may also need regular infusions of the antibody immunoglobin.
Works Cited Boztug, K. (n. d. ). Stem-cell gene therapy for the wiscott-aldrich syndrome. (2010). The NewEngland Journal of Medicine, Retrieved from http://www. nejm. org Schwartz, R. (n. d. ). Pediatric wiskott-adrich syndrome. (2013). MedScape Reference, Retrieved from http://emedicine. medscape. com/article/ Brickwall, P. , & Katz, D. (n. d. ). Wiskott-aldrich syndrome: current research concepts. (2001). Wiley Online Library, 101(4), 603-608. Retrieved from http://onlinelibrary. wiley. com
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