title: Adults with X-linked agammaglobulinemia: impact of disease on daily lives, quality of life, educational and socioeconomic status, knowledge of inheritance, and reproductive attitudes.
abstract: 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.
authors.affiliation: 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.
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Winkelstein
authors.forename: Mary Ellen
authors.initials: ME
authors.lastname: Conley
authors.forename: Cynthia
authors.initials: C
authors.lastname: James
authors.forename: Vanessa
authors.initials: V
authors.lastname: Howard
authors.forename: John
authors.initials: J
authors.lastname: Boyle
mesh_terms: 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
references.citation: Clin Immunol. 2002 Sep;104(3):221-30
references.pmid: 12217331
references.citation: Clin Immunol. 1999 Dec;93(3):190-7
references.pmid: 10600329
references.citation: Medicine (Baltimore). 1996 Nov;75(6):287-99
references.pmid: 8982147
references.citation: Blood. 2002 Feb 1;99(3):872-8
references.pmid: 11806989
references.citation: Clin Immunol. 2006 Feb-Mar;118(2-3):201-8
references.pmid: 16377251
references.citation: Medicine (Baltimore). 1985 May;64(3):145-56
references.pmid: 2581110
references.citation: Qual Life Res. 2000 Feb;9(1):55-63
references.pmid: 10981206
references.citation: J Pediatr. 2002 Oct;141(4):566-71
references.pmid: 12378199
references.citation: Immunol Rev. 2005 Feb;203:200-15
references.pmid: 15661031
references.citation: Nature. 1993 Jan 21;361(6409):226-33
references.pmid: 8380905
references.citation: Am J Med Genet. 1987 Mar;26(3):591-8
references.pmid: 3565475
references.citation: Haemophilia. 2000 Jan;6(1):33-40
references.pmid: 10632739
references.citation: JAMA. 1998 Jan 7;279(1):58-61
references.pmid: 9424046
references.citation: J Allergy Clin Immunol. 2004 Apr;113(4):793-800
references.pmid: 15100688
references.citation: N Engl J Med. 1991 Jul 11;325(2):110-7
references.pmid: 2052044
references.citation: Genet Med. 2006 Apr;8(4):234-42
references.pmid: 16617244
references.citation: Cell. 1993 Jan 29;72(2):279-90
references.pmid: 8425221
references.citation: Q J Med. 1993 Jan;86(1):31-42
references.pmid: 8438047
references.citation: Clin Nephrol. 1994 Oct;42(4):211-20
references.pmid: 7834912
references.citation: J Pediatr. 1999 May;134(5):589-96
references.pmid: 10228295
references.citation: Clin Rev Allergy Immunol. 2000 Oct;19(2):183-204
references.pmid: 11107501
references.citation: Medicine (Baltimore). 2006 Jul;85(4):193-202
references.pmid: 16862044