title: Adults with congenital limb deficiency in Norway: demographic and clinical features, pain and the use of health care and welfare services. A cross-sectional study.
abstract: OBJECTIVE
  To describe an adult population with congenital limb deficiency (CLD) recruited through the National Resource Centre for Rare Disorders (TRS) in Norway: (1) demographic factors, (2) clinical features, (3) pain and (4) use of health care and welfare services.
  
  
  METHODS
  Cross-sectional study. In 2012, a postal questionnaire was sent to 186 eligible persons with CLD, age 20 years and older.
  
  
  RESULTS
  Ninety-seven respondents, median-age 39 years (range: 20-82); 71% were women. The population was divided into two subgroups: (1) unilateral upper-limb deficiency (UULD) n = 77, (2) multiple and/or lower-limb deficiency (MLD/LLD). About 40% worked full-time, 18% received disability pensions and 64% reported chronic pain, mostly bilateral pain. Grip-improving devices were used more often than prostheses; 23% were previous prosthesis users. Use of health care and welfare services are described. No significant differences were found between the subgroups regarding pain or employment status.
  
  
  CONCLUSIONS
  Persons with CLD reported increased prevalence of chronic pain, mostly bilateral, and increased prevalence of early retirement. A greater focus on the benefits of the use of assistive devices, the consequences of overuse and vocational guidance may moderate pain and prevent early retirement. Further studies of more representative samples should be conducted to confirm our findings.
  
  
  CONCLUSIONS
  Most adults with congenital limb deficiency (CLD) live ordinary lives and experience normal life events. However, several report chronic pain and retire before normal retirement age. In spite of free and accessible prosthetic services, a large fraction chooses not to use prosthesis, more use grip-improving devices for specific activities. These preferences should be acknowledged by rehabilitation specialists. Focus on individually adapted environments, more information about the consequences of overuse, and vocational guidance may moderate pain and prevent early retirement.
authors.affiliation: a TRS, National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital , Nesodden , Norway .
authors.forename: Heidi
authors.initials: H
authors.lastname: Johansen
authors.affiliation: b Department of Physical Medicine and Rehabilitation , Innlandet Hospital Trust , Ottestad , Norway , and.
authors.forename: Kristin
authors.initials: K
authors.lastname: Østlie
authors.affiliation: a TRS, National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital , Nesodden , Norway .
authors.forename: Liv Øinæs
authors.initials: LØ
authors.lastname: Andersen
authors.affiliation: a TRS, National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital , Nesodden , Norway .|c Faculty of Medicine , Institute of Clinical Medicine, University of Oslo , Oslo , Norway.
authors.forename: Svend
authors.initials: S
authors.lastname: Rand-Hendriksen
mesh_terms: D000203:Activities of Daily Living; D000328:Adult; D000368:Aged; D059350:Chronic Pain / Q000453:epidemiology; D003430:Cross-Sectional Studies; D003695:Delivery of Health Care / Q000706:statistics & numerical data*; D006233:Persons with Disabilities / Q000534:rehabilitation / Q000706:statistics & numerical data*; D004651:Employment; D005260:Female; D006801:Humans; D017880:Limb Deformities, Congenital / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D009664:Norway; D010147:Pain Measurement; D012656:Self-Help Devices / Q000706:statistics & numerical data*; D012946:Social Welfare; D011795:Surveys and Questionnaires; D014830:Vocational Guidance; D055815:Young Adult
keywords: Adults; chronic pain; congenital limb deficiency; multiple and lower limb deficiency; unilateral upper limb deficiency; work participation