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Pediatr Rheumatol Online J. 2018 Dec 20;16(1):81. doi: 10.1186/s12969-018-0300-7.

Living with autoinflammatory diseases: identifying unmet needs of children, adolescents and adults.

Author information

1
Rheumatology, Department of Pediatrics and autoinflammation reference center Tuebingen, University Children's Hospital Tuebingen, Tuebingen, Germany.
2
Institute of Education, University of Tuebingen, Tuebingen, Germany.
3
Division of Oncological Surgery, Neurosurgery, Urology Gynaecologic Surgery District Clinics Reutlingen, Reutlingen, Germany.
4
Rheumatology, Department of Pediatrics and autoinflammation reference center Tuebingen, University Children's Hospital Tuebingen, Tuebingen, Germany. susanne.benseler@albertahealthservices.ca.
5
Rheumatology, Department of Paediatrics, Alberta Children's Hospital, Cumming School of Medicine, University of Calgary, Shaganappi Trail NW, Calgary, Alberta, T3B 6A8, Canada. susanne.benseler@albertahealthservices.ca.

Abstract

BACKGROUND:

Autoinflammatory diseases (AIDs) illnesses of the innate immunity resulting in clinical signs and symptoms of systemic inflammation and loss of organ functions. While pathophysiological mechanisms are heavily studied and increasingly well understood, psychosocial needs are much less explored. The disease impact on the everyday life of patients including school and work is poorly studied. The purpose of the study was to identify the spectrum of unmet needs of children, adolescents and adults living with autoinflammatory disease and their families, to define key unmet needs and strategies and to develop and evaluate a pilot intervention addressing the unmet need "school".

METHODS:

A single-center, mixed-method study of AID patients and their families was conducted. Consecutive patients ages ≥4 years and their families were included. Expert consulting, focus groups and questionnaires explored the patient perspective of "unmet needs in AID". Quantitative and qualitative content analyses were performed and informed the development of a framework of unmet needs. A targeted pilot multimodular intervention for the unmet need "school" was developed and tested. Health-related Quality of Life (HRQoL) was evaluated using DISABKIDS-questionnaires and psychosocial impact evaluations.

RESULTS:

The study included 83 patients and their families. These were 14 children, 9 adolescents and 25 adults with AID and 35 family members; patients' median age was 19 years (5-78). Expert consultations: 110 AID patients with 320 visits/year; 99 (90%) were children and adolescents. 78 patients and family members (94%) participated in 10 groups. Qualitative content analysis delineated 9 domains of unmet needs, the most relevant being school, health care system and public institutions. The pilot intervention"school" included 18 participants; median age was 9 years (7-16). HRQoL improved with the intervention including "understanding" by 53%, however improvement was not sustained over time.

CONCLUSION:

Unmet needs of AID patients and families affect all areas of life. Accessible networks increasing knowledge and empowering patients, strategies supporting academic and workplace environments to ensure successful participation and integrated concepts addressing psychosocial needs are urgently needed.

KEYWORDS:

Autoinflammatory disease; Health communication; Health-related quality of life; Intervention; Participation; Psychosocial impact; Rare disease; School bullying; School performance; Unmet needs

PMID:
30572912
PMCID:
PMC6302479
DOI:
10.1186/s12969-018-0300-7
[Indexed for MEDLINE]
Free PMC Article

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