Health CommitteeWritten evidence from Dr Karen Lowton, King’s College London (LTC 49)

1. This submission focuses on one of the issues the Committee wishes to examine: the implications of an ageing population for the prevalence and type of long term conditions, together with evidence about the extent to which existing services will have the capacity to meet future demand. The submission gives evidence on:

The rise of “new” ageing populations (NAPs) and associated long term conditions.

Psychosocial needs of these populations.

Professional careers and service provision for NAPs.

Prescription charges.

Research and recommendations.


2. The notion that population is ageing is leading to an increase in long term conditions (LTCs) which are commonly experienced by older adults has been well rehearsed. However, there are “new” ageing populations (NAPs) whose growth is mirroring that found in general population ageing: those born with serious health conditions who did not previously survive to adulthood and those developing previously life-limiting conditions in early or mid-life who are now more likely to reach mid- to late-adult life. These NAPs include adults with cystic fibrosis (CF), congenital heart disease (CHD), childhood liver disease (CLD), cerebral palsy (CP), human immunodeficiency virus (HIV), and Down syndrome. These populations are on average younger than those we traditionally conceptualise as the ageing population, yet as they age they are experiencing many of the LTCs found among the older population, but at a much earlier age.

3. The Department of Health’s definition of LTCs acknowledged that the life of an individual with a LTC “is forever altered—there is no return to ‘normal’”.1 Here the emphasis is on the more common conditions that are acquired in later life such as diabetes, coronary heart disease and stroke, and assume that there has been a period of “normality” in early life. For many NAPs, there is no earlier period of “normality”.

4. In 2010, the Chief Medical Officer emphasised that rare conditions (which underlie the health problems of many “new” ageing individuals) are an important cause of illness and death, yet do not currently receive the attention they need and deserve2. However, this terminology leads us to consider people with rare conditions separately from those with LTCs, even though the former are likely to experience both their underlying rare condition and acquired LTCs as they grow older.

The Rise of NAPs

5. Better understanding of disease processes and progress in medical and surgical interventions has enabled NAPs to live longer lives. We are moving towards a society that contains more adults than children living with traditional diseases of childhood. For example, for the first time in history the number of adults with CF now exceeds the number of children with the condition. In 1938 the average survival age for those with CF was under one year;3 now 8% of patients are aged over 40.4 Furthermore, those born with CF in 2000 are predicted to live over 50 years.5 Similar growth is seen in adults with severe CHD;6 before paediatric cardiac surgery programmes began in 1938 very few of these children survived7 yet in 2000 there were nearly equal numbers of adults and children.8 A US study death found that for those with Down Syndrome median age at death increased from 25 years in 1983 to 49 years in 1997, an average increase of 1.7 years per year over this period. By comparison, median age at death in the general population increased by only three years during this entire period.9 Similarly, life expectancy for those with cerebral palsy is now approaching that of the general population.10

6. The epidemiology of many traditional conditions of childhood into mid-late adulthood is largely unknown.11 One exception is the European Cystic Fibrosis Society (ECFS) Patient Registry, which follows individuals through their lifetime.12 Many young people do not attend specialist clinics or are lost to follow-up after leaving paediatric care, leading to difficulties in understanding the size, diagnosis, treatment requirements, medical problems, complexity and speed of growth of these groups.13

7. Although numbers of individuals per condition are relatively small when compared to those with more typical acquisition of LTCs in later life, NAPs are growing significantly in terms of numbers of individuals and groups involved. For example, those with CLD also commonly died in early life.14 Following experimental childhood liver transplant, which began in the UK in the mid-1980s, ten-year survival is now at least 80%, with clinicians predicting that “a significant learning curve” will fuel even longer survival.15 Today, around 100 children undergo liver transplant in the UK annually.16 It is likely therefore that these children will also eventually be outnumbered by adults with “childhood” liver disease, many of whom will experience LTCs at an early age.

8. These NAPs will potentially increase further through increasing survival of premature babies at lighter weights and earlier weeks. By analysing routine NHS datasets, Fraser et al17 report an increase in prevalence of children aged 16–19 years with life-limiting conditions of 45% over the decade to 2009–10; the authors attribute this to increasing survival times rather than an increasing incidence of conditions.

NAPs and Long Term Conditions

9. Successful early intervention, ongoing treatment, and ageing processes for those with previously life-limiting conditions, has resulted in complex conditions, co-morbidities, and LTCs in NAPs. One prominent example of this phenomenon is highly active antiretroviral therapy (HAART) for those with HIV infection. This has turned a previously acute, fatal illness into a LTC, and as those with HIV age they are likely to experience more HIV-associated LTCs such as dementia; it is suggested that by 2030 the number of people of all ages living with HIV-related cognitive disorders will increase 5–10 fold.18 Another example is thalidomide impairment, where problems of ageing and the need for appropriate social care are now being recognised for a population that has yet to reach a traditional “old age”. There are many more examples of LTCs being found in NAPs as they grow into adulthood. For example, surgical repair is possible for many underlying congenital heart conditions, although few people have been “cured”, and for some the effects of their underlying genetic condition continue. Many adults with CHD have myocardial dysfunction, are exercise intolerant, and some have heart failure. As a group, these adults have neurohormonal activation levels similar to those found in a much older adult heart failure population with acquired heart disease, with the prevalence of acquired heart disease increasing as the CHD population ages.19 For people with Down syndrome, the risk of dementia is significantly higher, and is developed around 30–40 years earlier than for the general population.20 These individuals also develop LTCs including obesity and diabetes in early adult life,21 as do those with CP.22

Psychosocial Needs

10. Psychosocial needs including mental health needs are many and diverse for NAPs. Many individuals, for example adults with CHD, experience being different in terms of body image, effects of surgery, limitations and restrictions in the physical environment and in education and employment.23 Additionally, there is a psychological challenge of becoming an adult who is at higher risk of particular health problems than the general population, for example cancer (young women who have had a paediatric liver transplant undergo annual cervical smears due to the increased risk of cervical cancer). Many possess a heightened sense of their own mortality, for example young people with CF question regularly what their life expectancy will be;24 others with CHD live with the knowledge of heart problems and a limited life span.25 The prevalence of cognitive or intellectual impairment, primarily due to underlying genetic or chromosomal syndromes, is also increasing in frequency due to a more active approach to treatment in childhood.26 , 27 , 28

11. Additional psychosocial needs relate to missed education due to time spent in ill health or in hospital, and non-continuous employment records; many of these NAPs are not able to save for a pension to support them in later life. Many cannot obtain life, health or travel insurance, and experience difficulties in obtaining a mortgage. There may also be an ongoing need for education and counselling relevant to the underlying condition and its wider implications. Although some psychosocial issues around living with an underlying condition and its comorbidities have begun to be rehearsed, for example for survivors of childhood cancer, many needs still need to be defined before they can be met.29

Professional Careers and Service Provision for NAPs

12. Adult specialist and general physicians are not currently equipped for the range and complexity of adults growing older with traditional diseases of childhood and associated LTCs. Clinical expertise for NAPs has developed in an unplanned fashion,30 with training assuming relatively rigid transitions for individuals from child to adult, and from adult to old age.31 For example, in one Canadian survey half of adult neurologists sampled reported that they had not received enough child neurology training to treat the growing number of adults with child-onset neurological disorders.32 In cardiology, most general clinicians have very little understanding or experience of the management of adults with CHD.33 Similarly, paediatric specialists cannot manage within a paediatric environment the many adult conditions that these populations acquire. In one study, 60% of Canadian paediatric neurologists reporting difficulty finding an adult physician to take over care.34

13. Health services have been developed around professional clinical career structures. For many NAPs, for example those with CHD, both specialist and generalist cardiology input are required as this “new” population of adults no longer fit within the traditional service divisions which split “adult and paediatric cardiology”.35 Most adult services for NAPs are based exclusively within secondary care, where multi-disciplinary teams have been developed to enable holistic care. Commonly, models are hierarchical, in a tertiary or specialist centre, with shared or outreach care delivered at district general hospitals; few receive care at local hospitals alone. One exception to secondary care provision is CP, where adults, largely an outpatient population, give hospital neurologists little exposure to this group in their training.36 Furthermore, many community-based clinicians, especially general practitioners (GPs), would not expect to see more than one patient with, for example, CF, during their career. When they do, there is a risk of their attributing any new symptom to the underlying condition, with patients acknowledging that a “specialist” patient may not always help educate their GP about their condition through their preference to contact specialist services.37 Although these adults have specialist needs that are met by specialist services in hospital, other needs of this ageing group mirror closely those with LTCs who are typically seen in adult or elderly care services. For example, urinary incontinence in women with CF and heart failure in those with CHD could potentially be treated in the community, although it is not clear how or when the relationship with community-based services should begin for NAPs who acquire LTCs at a relatively early age. Some examples exist of how specialist care can be moved out of hospitals into the community, for example hospital at home schemes for adults with CF with chest infection, yet studies suggest these may not be as effective as hospital-based care.38

14. Arising from professional career and service structures, transition services act as a bridge to move children from paediatric to adult services. Established services, for example CF, demonstrate good outcomes39 although many others are less established,40 with many patients having to travel significant distances to fit service provision. Transition services’ focus is on developing an “independent” adult able to manage their condition; transition clinics assume a linear progression through health services, although in practice many individuals find this difficult to achieve. There is little time to respond to wider health needs, for example the sexual health of those with CF.41 Additionally, outcomes rarely take into account ongoing family support or the strain on families who provide this. Despite ongoing improvements in life expectancy for NAPs, the emphasis remains on transition rather than adult health and social care services. Many young adults need supportive and palliative care services; these are not always addressed through the End of Life care strategy.42

15. The absence of datasets for many rare and complex conditions makes planning difficult (both for precise numbers and size of growth) for future NAPs who will require health and social care services. Based on just one UK health region Wren predicted the need for adult follow up of CHD of over 1,600 extra cases every year in the UK.43 Adult services are playing “catch up” as many of these NAPs are pioneers; they are the first cohort to survive to adult age (for example adults living with paediatric liver transplant) and clinicians cannot easily predict what their future may hold.

16. An ageing society is not ageing solely at the “geriatric” end but there is no advocacy for adults in their 20s and 30s who may have lived with LTCs for as long as a 70 year old may have done, but with a wholly different set of challenges. Charities, interest groups and health services see NAPs as having separate, disease specific conditions rather than acknowledging the common challenges of providing long-term care for these groups, and do not usually share their learning about training and organisational ways of working. In doing this, the voluntary sector is still to become effective advocates for NAPs navigating adult health and social care. Similarly, age-related voluntary sector advocacy on LTCs also misses NAPs.

Prescription Charges

17. The case has previously been made for exemption from prescription charges for people living with LTCs in England.44 The list of exemptions from prescription charges was drawn up when many individuals with childhood conditions did not survive to adulthood; these individuals were exempt from payment by virtue of their being children. People with traditional childhood conditions who are now surviving into adulthood must pay for their prescriptions; prepayment certificates are costly, especially for those who may not be able to work due to their condition. Anecdotally, some people with CF feel one benefit of the onset of CF-related diabetes is that it allows them to claim exemption from all their prescription charges. Despite not personally consenting to their surgery, adult survivors of childhood transplants also have to pay an adult lifetime’s charges for prescriptions that include the immunosuppressants that keep them alive. At the time the exemption list was compiled immunosuppressants had not been developed, yet they are now life saving for the vast majority of people who rely on them post transplant. This is at odds with people with diabetes who receive free prescriptions because insulin keeps them alive.

In Conclusion

18. In summary, NAPs are a significant growing part of our society for whom the experience, treatment and care of LTCs is becoming paramount; only a handful of examples have been given here. There is little evidence to clarify the extent to which existing services will have capacity to meet future demand. These NAPs are not included in long term care strategies, which situate health and social care issues within populations who typically acquire LTC at older ages, serving to further marginalise treatment and care for their younger counterparts.

Research and Recommendations

19. Extending lifespans has been one of the UK’s greatest achievements, but we must develop the epidemiology of traditional conditions of childhood into old age, and address workforce and service delivery issues to enhance the lives of “new” ageing individuals and their families as they grow older. To do this, we must reinterpret the “success” of medical and surgical interventions within a timeframe that incorporates the amelioration of underlying and acquired LTCs as NAPs age. Longitudinal studies that examine care delivery across the lifespan would allow us to understand what approaches and providers can deliver the best outcomes for these groups. Condition-specific research may miss the bigger picture of the challenges that NAPs face in respect of ageing with LTCs. Exempting individuals from prescription charges for immunosuppressants and other life-preserving drugs would meet one need felt by many of these “new” ageing individuals.

9 May 2013

1 Department of Health, 2008.

2 Chief Medical Officer (2010). 2009 Annual Report of the Chief Medical Officer. Department of Health, London. (Rare is Common, Pages 38–45).

3 Andersen D. 1938 Cystic fibrosis of the pancreas and its relation to celiac disease: A clinical and pathologic study. American Journal of Diseases of Children, 56 (2), 344–399.

4 Simmonds, N J. Ageing in Cystic Fibrosis and Long-term Survival. Paediatric Respiratory Reviews, early online

5 Dodge J A, et al (2007). Cystic fibrosis mortality and survival in the UK: 1947–2003. European Respiratory Journal, 29, 522–526

6 Wren, C & O’Sullivan, J J (2001). Survival with congenital heart disease and need for follow up in adult life. Heart 85, 438–443.

7 Castañeda, A (2005). Congenital heart disease: a surgical-historical perspective. The Annals of Thoracic Surgery, 79, S2217-20.

8 Marelli, A J, et al (2007). Congenital heart disease in the general population: changing prevalence and age distribution. Circulation, 115 (2), 163–172.

9 Yang Q, et al (2002). Mortality associated with Down’s syndrome in the USA from 1983 to 1997: a population-based study. Lancet, 359, 1019–25.

10 Aisen M L, et al (2011). Cerebral Palsy: clinical care and neurological rehabilitation. Lancet Neurology, 10, 844–52.

11 See 8


13 The Task Force on the Management of Grown Up Congenital Heart Disease of the European Society of Cardiology. (2003) Management of Grown Up Heart Disease. European Heart Journal, 24, 1035–1084.

14 Ohi, R (2001). Surgery for biliary atresia. Liver, 21, 175–182.

15 Otte J B (2002). History of pediatric liver transplantation. Where are we coming from? Where do we stand? Pediatric Transplantation, 6, 378–387

16 NHS Blood and Transplant (2010). Transplant activity in the UK: Activity Report 2009–10.

17 Fraser L K, et al (2012). Rising national prevalence of life-limiting conditions in children in England. Pediatrics, 129 (4), e923–9.

18 Cysique L A, et al (2011). The burden of HIV-associated neurocognitive impairment in Australia and its estimates for the future. Sexual Health, 8 (4), 541–550.

19 Shaddy R E & Webb G (2008). Applying heart failure guidelines to adult congenital heart disease patients. Expert Review of Cardiovascular Therapy, 6 (2), 165–74.

20 Glasson E J, et al (2013). The triple challenges associated with age-related comorbidities in Down syndrome. Journal of Intellectual Disability Research. Article first published online: DOI: 10.1111/jir.12026

21 Urbano R C (2010). International Review of Research in Mental Retardation. Volume 39. Health Issues in Down Syndrome. Academic Press, New York.

22 Bauman W A (2009). The potential metabolic consequences of cerebral palsy: inferences from the general population and persons with spinal cord injury. Developmental Medicine & Child Neurology, 51 (suppl 4), 64–78.

23 Lowton K (2012). Reconceptualising later life: Using qualitative methods to refine understanding of new ageing populations. In Leontowitsch M (ed) Researching Later Life and Ageing—Expanding Qualitative Research Agendas and Methods. London, Palgrave.

24 Iles N J, & Lowton K (2008). Young people with cystic fibrosis’ concerns for their future: when and how should concerns be addressed, and by whom? Journal of Interprofessional Care 22 (4), 436–438.

25 See 13.

26 See 13.

27 Lowton K & Higgs P (2010). New ageing populations. Nursing Older People 22 (7), 10.

28 Liptak G S (2008). Health and well being of adults with cerebral palsy. Current Opinion in Neurology, 21, 136–142.

29 Schor N F (2013). Life at the interface: Adults with “pediatric” disorders of the nervous system. Annals of Neurology, DOI: 10.1002/ana.23910

30 See 13

31 See 30

32 Oskoui M & Wolfson C (2012). Treatment comfort of adult neurologists in childhood onset conditions. Canadian Journal of Nuerological Sciences, 39 (2), 202–205.

33 See 13

34 See 33

35 See 13

36 See 30

37 Lowton K & Ballard K D (2006). Adult cystic fibrosis patients’ experiences of primary care consultations: a qualitative study. British Journal of General Practice 56 (528), 518–525.

38 Thornton J (2004). Long term clinical outcome of home and hospital intravenous antibiotic treatment in adults with cystic fibrosis. Thorax, 59:242–246.

39 Kerem E, et al (2005). Standards of care for patients with cystic fibrosis: a European consensus. Journal of Cystic Fibrosis, 4, 7–26.

40 See 30

41 Sawyer S M, et al (2001). Reproductive and sexual health in males with cystic fibrosis: a case for health professional education and training. Journal of Adolescent Health, 28 (1), 36–40.

42 Lowton K (2009). ‘A bed in the middle of nowhere’: parents’ meanings of place of death for young adults with cystic fibrosis. Social Science & Medicine 69 (7), 1056–1062.

43 Wren C & O’Sullivan J J (2001). Survival with congenital heart disease and need for follow up in adult life. Heart 85, 438–443.

44 2009. Prescription Charges Review: Implementing Exemption from Prescription Charges for People with Long Term Conditions. A report for the Secretary of State for Health by Professor Ian Gilmore.

Prepared 3rd July 2014