title: Influence of learning disabilities on the tumour predisposition syndrome NF1--survey from adult patients' perspective.
abstract: OBJECTIVE
  To analyze psychosocial burdens associated with neurofibromatosis type-1 (NF1) phenotype--visible symptoms, medical complications, learning disabilities (LD)--from patients' perspective with focus on LD.
  
  
  METHODS
  A survey of 228 adult patients with NF1 was carried-out. Symptoms to estimate disease severity and visibility, and learning disability were assessed. Outcome parameters were social situation and psychosocial aspects.
  
  
  RESULTS
  Social situation and psychosocial aspects differed depending on NF1 phenotype. Patients with LD (n=55) were less frequently in a partnership (p=0.005) or had children (p=0.015) than those without (n=132). They also reported a higher frequency of depression (p=0.019) and sensitivity to stress (p<0.001) and more uncertainty regarding NF1-associated symptoms. These differences were significant when adjusting for disease severity and self-perceived disease visibility.
  
  
  CONCLUSIONS
  Beside the psychosocial needs of patients with LD with NF1, medical management of this sub-group should include doctor-patient communication in easy language to compensate for patients' lack of knowledge about symptoms associated with cancer.
authors: Department of Neurology, Hamburg, Germany s.granstroem@uke.de.
authors: Sofia
authors: S
authors: Granström
authors: Neurofibromatosis Laboratory, Hamburg, Germany Oral and Cranio-Maxillofacial Surgery, Institute for Health Services Research in Dermatology and Nursing, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors: Reinhard E
authors: RE
authors: Friedrich
authors: German Center for Health Services Research in Dermatology (CVderm), Institute for Health Services Research in Dermatology and Nursing, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors: Anna Katharina
authors: AK
authors: Langenbruch
authors: German Center for Health Services Research in Dermatology (CVderm), Institute for Health Services Research in Dermatology and Nursing, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors: Matthias
authors: M
authors: Augustin
authors: Department of Neurology, Hamburg, Germany.
authors: Victor-Felix
authors: VF
authors: Mautner
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007859:Learning Disabilities / Q000523:psychology*; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000523:psychology*; D011584:Psychology; D057566:Self Report; D055815:Young Adult
keywords: Neurofibromatosis type-1; adult; cancer; disease severity; learning disability; phenotype; psychosocial aspects; survey; tumour predisposition syndrome; visibility