Cerebral palsy in adults: summary of NICE guidanceBMJ 2019; 364 doi: https://doi.org/10.1136/bmj.l806 (Published 19 March 2019) Cite this as: BMJ 2019;364:l806
All rapid responses
With respect to feeding problems, the NICE guidance on Cerebral palsy (CP) in adults (1,2) lacks the detail of a previous guideline (3) that covered patients with CP under the age of 25. However, neither guideline recognizes the increasing cohort of young people with very severe CP and other complex co-morbidities who have such severe feeding problems that they are entirely dependent on enteral tube feeding as they approach transition to adult services.
Many of these unfortunate young people have co-existing swallowing problems and are unable to protect their airway. They may have had fundoplication surgery as children and increasingly may have progressed from continuous pump gastrostomy feeds to feeding via a gastro-jejunal device. With time, their underlying gut dysmotility will deteriorate and this often occurs at the same time as their kyphoscoliosis has evolved. This combination can make effective enteral feeding increasingly challenging and in some patients leads to intestinal failure.
This new problem is covered briefly in the NICE guideline on Gastro-oesophageal reflux disease in children (4) but I am concerned that insufficient attention is being directed by clinicians, commissioners and the NHS toward this increasing clinical and ethical dilemma.
1 BMJ 2019;364:l806
2 Cerebral palsy in adults. NICE guideline NG119 (2019)
3 Cerebral palsy in under 25s: assessment and management. NICE guideline NG62 (2017)
4 Gastro-oesophageal reflux disease in children and young people:diagnosis and management. NICE NG1 (2015)
Competing interests: I chaired NICE NG1 2015
Re: Cerebral palsy in adults: summary of NICE guidance - importance of assistive technology underestimated
The NICE guidance on cerebral palsy (CP) is very welcome and the importance of an annual review by a suitably qualified health professional is vital . For those with severe impairments, the multidisciplinary team is invaluable but what it does is not spelled out in the guidance. This probably reflects the lack of research into assistive technology (AT) and into the problems associated with CP in adults. AT is usually provided after assessment by therapists who may be speech and language therapists, physiotherapists or occupational therapists.
For those with severe CP (e.g. with Gross Motor Function Classification system levels lV and V) AT assists individuals with communication impairments (with communication aids), immobility (with powered mobility) and inability to control simple switches (with environmental control units) which can greatly improve quality of life and independence . The therapeutic qualities of AT are often neglected. Specialised seating  can accommodate the subluxed hips, contractures, kyphoscoliosis and other deformities and facilitate the management of spasticity thus reducing the pain and discomfort correctly noted by Bromham . The electric powered indoor outdoor wheelchairs (EPIOCs for those eligible for them ) can incorporate tilt-in-space which can itself minimise pain and pressure issues . Indeed standard technology can control communication and the environment from one control system often mounted on a wheelchair tray .
The guidance also made no reference to the need to enable those with CP to work. Employment can be greatly assisted with the use of AT. Although neglected by the NHS in recent years , government policy now encourages all professionals to facilitate the employment of disadvantaged individuals . The techniques available to support those with CP commence with support during childhood, adolescence and early adult life facilitating the development of confidence and social skills as well as education [7-8]. Placement into work has been transformed by training disadvantaged individuals in the work place rather than training them prior to employment (place then train rather than train then place ). For those in work but having problems (which may or may not be associated with changes in their CP), job modifications (accommodations) may be very helpful, whilst those who are out of work can be assisted into work through a variety of ways .
Another area not mentioned in the NICE review  is the assistance often available from the voluntary sector. AbilityNet offers advice for those with physical impairments using computers. Peer support can be invaluable in supporting disadvantaged individuals during difficult periods .
Supporting those with CP transcends the medical model. Rehabilitation professionals recognise the need for all bio-psycho-social methods in providing support for disadvantaged individuals – but need to evaluate their work more effectively so that all can benefit from their holistic approach.
(1) Bromham N, Dworzynski K, Eunson P, Fairhurst C. Cerebral palsy in adults: summary of NICE guidance. BMJ (Clinical research ed ) 2019; 364:l806.
(2) Frank AO, De Souza LH. Problematic clinical features of children and adults with cerebral palsy who use electric powered indoor/outdoor wheelchairs: a cross-sectional study. Assistive Technology 2016; 29(2):68-75.
(3) British Society of Rehabilitation Medicine. Specialised wheelchair seating national clinical guidelines. Report of a multidisciplinary expert group (Chair: Marks, LJ). 1-39. 2004. London, British Society of Rehabilitation Medicine.
(4) Frank AO, Ward JH, Orwell NJ, McCullagh C, Belcher M. Introduction of the new NHS Electric Powered Indoor/outdoor Chair (EPIOC) service: benefits, risks and implications for prescribers. Clin Rehabil 2000; 14(December):665-673.
(5) British Society of Rehabilitation Medicine. Vocational Rehabilitation - the way forward: Report of a Working Party (Chair Frank AO). First ed. London: British Society of Rehabilitation Medicine; 2000.
(6) Department for Work and Pensions, Department of Health. Improving Lives: the Future of Work, Health and Disability. 1 ed. London: Department for Work and Pensions; Department of Health; 2017. https://whitehall-admin.publishing.service.gov.uk/government/uploads/sys...
(7) Frank AO. Vocational rehabilitation: supporting ill or disabled individuals in(to) work: a UK perspective. Healthcare 2016; 4(46).
(8) Connolly P, Stevens T. Get back to where we do belong. Disability Rights UK, London, 2016.
(9) Grove B. International employment schemes for people with mental health problems. BJPsych International 2016; 12(Research Supplement):97-99.
Competing interests: No competing interests
The National Institute for Health and Care Excellence (NICE) provided an important recommendation that we should assess osteoporotic fracture risk in adults with cerebral palsy and risk factors (1). Here I would like to point out that the risk factors mentioned by NICE (1) could need to be amended. In addition to skeletal fragility caused by physical disability (1), for example, joint contracture is another key risk factor that we need to know (2). In contrast to the NICE suggestion (1), however, current evidence is unlikely to support the concern that proton pump inhibitor use is a causal risk factor that adversely affects bone health (3-5).
Among individuals with cerebral palsy, fracture is one of the serious health problems that impair their quality of life, but no effective therapy has been established for the management of bone fragility resulting from physical disability (2). Although they cannot perform dynamic exercise that results in bone gain, inhibiting the activity of sclerostin secreted from bones would have the effect of such vigorous exercise (6-8). Consequently, treatment with romosozumab, a monoclonal antibody that inhibits the sclerostin activity, recently approved in Japan and under review in the USA and the EU, is highly expected to reduce fragility fractures in individuals with physical disability.
(1) Bromham N, Dworzynski K, Eunson P, Fairhurst C on behalf of the Guideline Committee. Cerebral palsy in adults: summary of NICE guidance. BMJ 2019;364:l806.
(2) Sugiyama T, Taguchi T, Kawai S. Spontaneous fractures and quality of life in cerebral palsy. Lancet 2004;364:28.
(3) Sugiyama T. Proton pump inhibitor use and fracture risk. Aliment Pharmacol Ther 2018;47:449-50.
(4) Sugiyama T. Association between proton pump inhibitor use and fracture risk - causality or confounding? Aliment Pharmacol Ther 2018;47:1569-70.
(5) Sugiyama T. Proton pump inhibitor use and fracture risk: an update of drug safety communication needed? Am J Gastroenterol 2019;114:360-1.
(6) Sugiyama T, Kim YT, Oda H. Osteoporosis therapy: a novel insight from natural homeostatic system in the skeleton. Osteoporos Int 2015;26:443-7.
(7) Sugiyama T, Torio T, Miyajima T, Kim YT, Oda H. Romosozumab and blosozumab: alternative drugs of mechanical strain-related stimulus toward a cure for osteoporosis. Front Endocrinol 2015;6:54.
(8) Sugiyama T, Oda H. Osteoporosis therapy: bone modeling during growth and aging. Front Endocrinol 2017;8:46.
Competing interests: No competing interests