A ski and adventure camp for young patients with severe forms of epidermolysis bullosa

Nading, Mary Alice; Lahmar, J. J.; Frew, John W.; Ghionis, Nicholas; Hanley, M J; Welch, Anna Kemble; Murrell, Dédée F.

doi:10.1016/j.jaad.2008.12.004

Cited by 8 publications

(6 citation statements)

References 2 publications

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“…Having access to peer relationships with other families and people living with EB may help provide a sense of being understood, alongside education being available to those around the person with EB and their family; though not all individuals or families may wish to access this resource [38, 39]. DEBRA events and online social media support networks could also be beneficial [35, 36].…”

Section: A Discussion Of Psychosocial Care For Individuals Living Wimentioning

confidence: 99%

“…Psychosocial care for individuals living with EB We strongly recommend easy access to psychosocial support to improve QoLB✓2++[28–32] We strongly recommend psychosocial support to improve well-beingC2+[32, 33–36] We strongly recommend gaining access to psychosocial support for the whole familyC✓2-[37–39] α We recommend psychosocial support to help with painC2-[3, 7, 17, 30, 37, 40–44] We strongly recommend psychosocial support to help coping with EBC✓2-[34, 37, 45, 46] We strongly recommend psychosocial support from a multidisciplinary Health Care TeamC✓2-[17, 29, 38, 40, 46–51]B. Psychosocial care for family and care givers of people with EB We strongly recommend access to psychosocial family support to improve the family QoLB2+[29, 30, 38] We strongly recommend psychosocial support to improve family well-beingC2-[30, 36, 50, 52–54] We strongly recommend family counselling in order to prevent family breakdownC✓2-[16, 17, 30, 38, 50, 52, 55] We strongly recommend psychosocial support to help the whole family to cope with living with EBC2-[16, 17, 30, 51] We recommend psychosocial support to reduce emotional burden during daily painful proceduresC2-[35, 37, ...…”

Section: Recommendationsmentioning

confidence: 99%

“…We strongly recommend psychosocial support to improve well-beingTo promote self-efficacy and support around body image to aid psychological well-being • Having access to knowledge and resources about EB can help people have a greater role in managing their EB. This self-management can help improve well-being. • Improved self-efficacy and locus of control, as well as support around body-image could help to develop a more positive sense of well-being.For support during transition periods in life (school transitions, transition into adulthood) • Communication and education about EB to improve people’s understanding. • Support from families, EB healthcare professionals and DEBRA.Review of inherited &autoimmune blistering diseasesAdults ( n = 87) RDEB, DEB, EBSChildren 10–14 years old ( n = 11) EBS (autosomal recessive)Young male adults (aged 21–35 years) with RDEB ( n = 5) and EBS ( n = 2)Observational reportC2+1-2+2+44[32][33][34][35][36]iii. We strongly recommend gaining access to psychosocial support for the whole familyPeople diagnosed with EB should be referred for psychosocial support as early as possible in childhood or in adulthood, if the person with EB wishes• To support the family unit.Encourage supportive network for the family, for example: • Education about EB for others • Provide access to DEBRA (or other EB support groups)✓Children ( n = 11/82) EBS; JEB; DDEB; RDEBChildren ( n = 16) JEB Adult ( n = 1) RDEBC2-2-2-4[37][38][39] α iv.…”

Section: Recommendationsmentioning

confidence: 99%

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Psychosocial recommendations for the care of children and adults with epidermolysis bullosa and their family: evidence based guidelines

Martin

Geuens

Asche³

et al. 2019

Orphanet J Rare Dis

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Epidermolysis Bullosa (EB) is a group of rare genetic disorders resulting in skin fragility and other symptoms. Commissioned by DEBRA International and funded by DEBRA Norway, this evidence-bases guideline provides recommendations to optimise psychosocial wellbeing in EB. An international multidisciplinary panel of social and health care professionals (HCP) and people living with EB was formed. A systematic international literature review was conducted by the panel following the Scottish Intercollegiate Guidelines Network (SIGN) methodology. The resulting papers underwent systematic selection and critique processes. Included papers were allocated to 6 different outcome groups to allow data synthesis and exploration: quality of life, coping, family, wellbeing, access to HCP and pain. Based on the evidence in those papers, recommendations were made for individuals living with EB, family and caregivers and HCP working in the field. Few studies have investigated interventions and which factors lead to better outcomes, but general recommendations can be made. EB is a complex disease impacting enormously on every aspect of psychosocial life. People and families living with EB need access to multidisciplinary support, including psychological guidance, in order to improve quality of life and psychosocial wellbeing. Interventions should stimulate social participation to prevent isolation. People with EB and their families should be able to access a supportive network. HCP should be well supported and educated about the complexity of EB. They should work collaboratively with those around the individual with EB (e.g. schools, employers etc.) to provide psychosocial opportunity and care. Attention should be paid to the psychosocial impact of EB as well as physical needs. Directions for research are indicated. Electronic supplementary material The online version of this article (10.1186/s13023-019-1086-5) contains supplementary material, which is available to authorized users.

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Section: A Discussion Of Psychosocial Care For Individuals Living Wimentioning

confidence: 99%

Section: Recommendationsmentioning

confidence: 99%

Section: Recommendationsmentioning

confidence: 99%

See 1 more Smart Citation

Psychosocial recommendations for the care of children and adults with epidermolysis bullosa and their family: evidence based guidelines

Martin

Geuens

Asche³

et al. 2019

Orphanet J Rare Dis

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“…5 To satisfy the demands of these elite athletes, significant new technological developments in wheelchair design and prostheses have occurred. 6 The somewhat radical equipment designs of the sledge or sit-ski, which is a specially built chair that can be attached to a pair of modified skis for winter sports, 7 have revolutionized sport medicine thinking. A similar theme of innovation has occurred in summer sports with the developments of the J-Leg, 8 seated throwing chairs, 9 and racing wheelchairs.…”

Section: Introductionmentioning

confidence: 99%

Paralympic Sports Medicine—Current Evidence in Winter Sport

Burkett

2012

Clinical Journal of Sport Medicine

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Assistive equipment is fundamental for a person with a disability to participate and compete in winter sport activities. Although there have been improvements in the mechanical function of some assistive devices, the key issue is matching the residual function of the person with the assistive equipment. Equitable access to this technology will also ensure that the fundamental spirit of fair play that underpins the Paralympic Games is maintained.

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“…In 2004, an inaugural adventure camp for Australasian patients with RDEB was held by a non‐profit charitable trust, the DEB Research Association New Zealand (DEBRA NZ), followed by two more camps in 2006 and 2007 2 . The many positive responses to these encouraged DEBRA NZ to organise another adventure camp in 2010.…”

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confidence: 99%