Adults with X-linked agammaglobulinemia: impact of disease on daily lives, quality of life, educational and socioeconomic status, knowledge of inheritance, and reproductive attitudes.
Since many children with X-linked agammaglobulinemia (XLA) can now be expected to reach adulthood, knowledge of the status of adults with XLA would be of importance to the patients, their families, and the physicians caring for these patients. We performed the current study in adults with XLA to examine the impact of XLA on their daily lives and quality of life, their educational and socioeconomic status, their knowledge of the inheritance of their disorder, and their reproductive attitudes. Physicians who had entered adult patients with XLA in a national registry were asked to pass on a survey instrument to their patients. The patients then filled out the survey instrument and returned it directly to the investigators. Adults with XLA were hospitalized more frequently and missed more work and/or school than did the general United States population. However, their quality of life was comparable to that of the general United States population. They achieved a higher level of education and had a higher income than did the general United States population. Their knowledge of the inheritance of their disease was excellent. Sixty percent of them would not exercise any reproductive planning options as a result of their disease. The results of the current study suggest that although the disease impacts the daily lives of adults with XLA, they still become productive members of society and excel in many areas.
Winkelstein
Jerry A
JA
From Department of Pediatrics (JAW, CJ), Johns Hopkins University School of Medicine, Baltimore, MD; Department of Immunology (MEC, VH), St. Jude Children's Research Hospital, Memphis, TN; and Abt SRBI Inc (JB), Silver Spring, MD.
Conley
Mary Ellen
ME
James
Cynthia
C
Howard
Vanessa
V
Boyle
John
J
D000223:Adaptation, Psychological; D000328:Adult; D000361:Agammaglobulinemia / Q000235:genetics / Q000523:psychology; D004522:Educational Status; D040181:Genetic Diseases, X-Linked / Q000235:genetics / Q000523:psychology; D007722:Health Knowledge, Attitudes, Practice; D006306:Health Surveys; D006801:Humans; D008297:Male; D010865:Pilot Projects; D011594:Psychometrics; D011788:Quality of Life / Q000523:psychology; D012042:Registries; D012098:Reproduction; D012307:Risk Factors; D012923:Social Class; D011795:Surveys and Questionnaires
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