75,000 SEVERELY DISABLED CHILDREN

Jonaihan Bradshaw Dorothy Lawion

Since 1973 an agency called the Family Fund has distributed 28 million pounds to families with severely disabled children in the United Kingdom. The Fund was established by the British Government during the public furore over thalidomide- damaged children. It was a novel initiative in British social policy because the Government by-passed the existing statu- tory system of benefits and services and turned to an independent charitable trust, the Joseph Rowntree Memorial Trust, based in York, to administer the Fund (Bradshaw 1980). 70,000 families have applied to the Fund.

The Fund has tried to operate on flexible and largely discretionary principles. Families apply directly to the Fund for items which they think might help to relieve the stress of caring for a severely disabled child and which cannot be provided by other statutory agencies. On first applying, families are visited by a social worker who assesses eligibility. The child must be under 16 years of age and be very severely disabled according to criteria established by a medical panel. For example, an epileptic child must have frequent or severe fits not controlled by drugs, a deaf child must have severe impairment or complete loss of hearing with marked communication problems, and a mentally handicapped child must have an IQ of less than 50.

A family may apply for and receive help more than once, and what they receive depends on what they ask for and whether this is appropriate in the light of the child’s particular disability. There is no formal means test, and although the social and economic circumstances of families are considered, very few are rejected because their income is too high. The Family Fund is unusual in the spectrum of provision: it is a hybrid, part cash benefit, part social service, distributing such help as washing machines and grants for holidays, tele- phones, clothing and bedding.

Since the Fund was established in April 1973, a computerised record has been kept of the characteristics of families applying. About 7 per cent of the families registered with the Fund have more than one disabled child; therefore the record represents about 75,000 disabled children throughout the United Kingdom. This computer record is used routinely to provide administrative information for the man- agers of the Fund. It has also been used as a sampling frame for studies of the social and economic consequences of severe disability in children, and of the operation of services and benefits (Bradshaw and Lawton 1980, Parker 1982, Baldwin et a/. 1983, Hirst 1983a, Glendinning 1984, Baldwin 1985). The data have been used in an analysis of the interaction between disease, disability and handicap (Hirst

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TABLE I Data recorded in the Familv Fund register

Local-authority area Sex Date of birth Child order of disabled child Number of other disabled children Family size and composition Economic position of head of household Mother’s employment outside the home Social class (Registrar General’s classification) Tenure Whether child is known to the Social Services Attendance allowance Mobility allowance Family Fund help provided Disease (principal handicapping condition) Impairments:

walking-degree of difficulty use of hands/arms-degree of difficulty sight-degree of difficulty speech-degree of difficulty hearing-degree of difficulty continence-degree of difficulty mental capacity-IQ behaviour problems-degree of severity fits-yes/no pain-yes/no s~ec ia l difficulties-ves/no , -

(9.g.. breathing, breakages, bruising) Disabilities:

washing- yes/no dressing- yes/no feeding-yes/no toileting-yes/no

night attention-yes/no keeping occupied-yes/no parents’s social life-yesho parents’ earnings-yes/no indoor mobility-yesho

Handicaps (third party):

19836) and to check the comprehensiveness of some local handicap registers (Bradshaw and Lawton 1982).

Has the Family Fund record any epidemiological potential? I t has been used to help medical researchers trace local populations of children suffering from particular conditions and to put parents of children suffering from unusual conditions in touch with each other, and in the early days of the Fund some characteristics of registered children were investigated (Bradshaw and Lawton 1978). In order to assess whether the data have any further epidemiological potential it is necessary to know what information is recorded and how representative the register is.

Data recorded When families first apply they are visited by one of the Fund‘s social workers, who completes a report form which is returned

to the Family Fund offices at York and from which the information given in Table I is recorded.

If the diagnosis or severity of the condition is uncertain, the social worker obtains an opinion from the child’s family doctor or consultant. In other cases the diagnosis is not validated. Where there are two or more disabled children in a family, only data for one child are recorded, either the most severely disabled or the eldest. Families may apply more than once- nearly 60 per cent have done so-but only the record of help provided is updated for subsequent applications.

The disease classification used is an adaptation of the shorter version of the International Classification of Diseases; the unmodified version was found to be unsatisfactory as a classification of children’s diseases. Many of the children suffer from more than one condition but only the principal handicapping condition is recorded. This is supplemented by data on impairment, disability and handicap. The classification of principal handi- capping condition is given in Table 11, together with the number of children, the average age at application and an estimated UK prevalence rate for each condition. Some of the potential inherent in the data is evident from this table: for example we have data for about 7000 spina bifida children, 919 with cystic fibrosis and 182 with arthrogryposis.

Representativeness Applications to the Family Fund are from a self-selecting sample of families with very severely disabled children. There is no entirely reliable method of assessing how comprehensive the register is or what biases may exist. I t is a record of the children and their families at the time they apply. Families with a child suffering from an obvious physical defect such as spina bifida are likely to apply while the child is quite young; those with children suffering from conditions of later onset or diagnosis, such as muscular dystrophy or mental handicap, apply when the child is older. This varying application age has to be taken into account in analysis, particularly of such characteristics as birth order, family size and family type. The oldest people in the register are now 26. It is less

TABLE I1 Family Fund classification of principal handicapping condition

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N ar application prevalence*

I . 2. 3. 4. 5 . 6. 7. 8.

10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31. 32. 33. 34. 35. 36. 37. 38. 39. 40. 41.

43. 45. 46. 47. 48. 49.

Tumours, cancer Diabetes Phenylketonuria, metabolic or glandular disorders Cretinism, thyroid disease Haemophilia, Christmas disease Leukaemia Other disease of the blood/blood-forming organs Autism, maladjusted, behaviour disorder Mental handicap (non-specific) Down's syndrome Microcephalus Meningitis Epilepsy, convulsions Cerebral palsy, spastic, hemiplegia, paraplegia Friedreich's ataxia Polio Tuberous sclerosis Other central nervous system disease Head injury Heart disease Cystic fibrosis Severe asthma Other lung or respiratory disease Renal disease Digestive system disease Congenital defects bladder, bowel or sex organs Congenital defects alimentary tract Maternal rubella Blind, vision defect Deaf Severe speech defect Skin disorder Rheumatoid arthritis, Still's disease Art hrogryposis Muscular dystrophy Werdnig-Hoffmann disease, spinal muscular atrophy Osteogenesis imperfecta, brittle bones Other bone disease Absence of limbs Spina bif idd

Hydrocephalus Dwarfism, achondroplasia Other physical malformations Burns Suspected vaccine damage Named syndromes

Spina bifida and hydrocephalus

695 174 186 97

317 208 199

2005 16,347

6978 677 42 1

2368 10,151

65 46

103 1414 30 1

1687 919 537

76 475 198 533 328 276

1754 3616

29 180 268 182

1624 266 170

I346 192

3440 3367 I692

98 58 1 37

118 897

67,638

7 8 6 7 7 7 6 8 7 6 4 5 7 6

10 10 5 6 9 5 5 7 6 7 5 6 3 6 6 6 7 6 9 5 9 5 6 7 7 6 5 5 6 5 7 7 5

7

0.38 0.09 0.09 0.06 0.15 0.13 0.12 0.86 8.03 3.61 0.48 0.28 1.20 5.10 0.03 0 .02 0.07 0.79 0.15 1.06 0.57 0.33 0.04 0.25 0.14 0 .33 0.24 0.17 0.93 2.02 0.02 0.12 0.12 0.10 0.56 0.18 0.10 0.77 0.10 I .68 1.76 0 .91 0.06 0 .39 0 .02 0 .06 0 .63

35.56 ~ ~~ ~ ~~

'Number under 16 per 10,000 UK population under 16 (1981 census).

complete for those over the age of 20 because they were teenagers when the Fund began and families may not have applied before the children became ineligible at the age of 16. Very young children are also under-represented be- cause disability may not have been diagnosed or may not yet present additional burdens.

The class distribution of families applying to the Fund is different from that

for the population of all families. However, investigations using the General Household Survey (Weale and Bradshaw 1980) and the 1970 birth cohort (Cooke et al. 1983) indicate that the Family Fund class distribution is only marginally more biased towards the manual social classes than the population of all families with severely disabled children in the UK.

Various attempts have been made to assess the comprehensiveness of the 27

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TABLE 111 Principal handicapping condition by sex

%

Population proportion Significantly more males than population

5. Haemophilia 36. Muscular dystrophy 32. Speech defect 20. Head injury 23. Severe asthma 8. Autism, erc.

27. Congenital defects bladder, erc. 28. Congenital defects alimentary canal 43. Hydrocephalus

40. Absence of limbs 10. Mental handicap 25. Renal disease 30. Blind, vision defect 14. Epilepsy, convulsions 46. Other physical malformations 15. Cerebral palsy, etc. 49. Named syndromes 31. Deaf 39. Other bone disease 1 1 . Down’s syndrome Signijicantly less males than population 41. Spina bifida 12. Microcephalus 34. Rheumatoid arthritis

2. Diabetes

51.3

9 5 . 2 91 .9 79.3 69 .8 67 .5 66.1 64.1 63.9 62.1 62 .0 61.8 60.2 58.4 58.1 58.1 57 .5 56.6 56 .6 56 .4 5 6 . 4 56 .4

47.7 41.8 39.7

register, using take-up experiments, record linkages and sample surveys of applicants. From these it is estimated that over three- quarters of all families with severely physically disabled children under 16 and about one-half of all families with severely mentally disabled children who are eligible have applied (Bradshaw and Lawton 1982). 41 out of 54 children in the 1970 birth cohort who were assessed as being eligible for help from the Fund had applied, a take-up rate of 76 per cent (Cooke et al. 1983).

Clearly the fact that the record is not complete and that its possible biases are uncertain are serious disadvantages in using it for some epidemiological purposes. Nevertheless, we know of no other record of this size or type in the world and wish to encourage the widest possible use of this data base by epidemiologists.

Analysis In an attempt to explore the epidemio- logical potential of the Family Fund register, this article very briefly presents the results of an analysis of the relationship between the principal handicapping

condition and other characteristics. Where there are differences between a particular condition and either the population or the sample as a whole, these are noted. Only differences which are significant at the 99 per cent level are noted (Bailey 1959). It should be borne in mind that the diagnostic categories refer to only very severe examples of the disorder concerned.

Sex In the general population of children 5 1 3 per cent are males and in the Family Fund register 57.4 per cent are males. There is no obvious reason why applications to the Family Fund should be biased by sex, so this indicates an excess of males in the population of very severely disabled children.

It is known that haemophilia and muscular dystrophy are sex-linked conditions and that autism is more common among males and spina bifida more common among females. It is interesting that microcephalus and rheumatoid arthritis appear to be more prevalent among females and that the conditions shown in Table I11 appear to be more prevalent among males. The high incidence of males among the severely mentally handicapped (60 2 per cent) may be further evidence of their greater vulnerability to this disorder.

Year of birth In analysing prevalence by year of birth, it is safest to ignore both the youngest and the oldest children in the register and observe the variation between 1962 and 1979 only. There are a number of conditions with random but significant variations in prevalence. Three show an increase in prevalence over time: heart disease, microcephalus and congenital defects of the alimentary tract are all more prevalent among children born in the 1970s than the 1960s. As Figure 1 shows, the prevalence of spina bifida was highest between 1969 and 1972 but since 1975 there is evidence of a sharp decline. 1979 appears to be a particularly bad year for rubella births in the UK. When rubella and deaf births are combined (Fig. 1) there also appear to be peaks in 1969 and 1972173.

Season of births Compared with the general population,

slightly more Family Fund children are born in the winter and spring and fewer are born in the summer and autumn. However, only four individual conditions appear to vary with the season of birth (Fig. 2). Children damaged by maternal rubella are more likely to be born in the winter. Blind and deaf children are also more likely to be born in the winter and as some of these children may also be rubella- damaged this does indicate that rubella is more likely to occur and cause damage to fetuses in late spring and early summer. There are more spina bifida births in the winter and spring. This seasonal variation has been noted before and may be evidence of the impact of diet in early pregnancy. However, if winter diets are a cause the effect must be delayed, since it is spring and summer conceptions that appear to be most at risk.

Socia I class 75.5 per cent of the families applying to the Family Fund are from the manual social classes, compared with 61.6 per cent in the population. All but five of the conditions contain more families from the manual social classes than would be expected in the population. The five exceptions are Friedreich's ataxia, tuberous sclerosis, arthrogryposis, suspected vaccine damage and severe speech defects. I t is possible that there is some class bias in knowledge or ascription of these conditions.

If there is no class bias in applications to the Fund, then there is an association between disorder and social class for some conditions which has not been noted previously. In order to control for possible class bias in the sample, Table IV lists the conditions containing significantly more or significantly fewer manual families than the Family Fund register as a whole. Head injuries and severe asthma show a particularly high excess among manual families and tuberous sclerosis has fewest children from manual families.

Where a condition exhibits an excess of children from a particular social class this may be because the severity of that condition, rather than its prevalence, varies with socio-economic conditions. Thus, for example, severe asthma may be more common among the manual social classes but not asthma in general.

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rr 1963 65 67 69 71 73 75 77 7 9

Year of birth

Spina bifida Maternal rubellaideafness

Fig. 1. Rates of spina bifida and maternal rubella/ deafness per 1000 live births by year of birth.

% population [all children born in England and Wales I 9 6 4 - 1 9 7 1 )

30. % blind 29. 8 maternal rubella

@ 2 9 . 4 -53 25.7 1 3 . 1

31. 8 deaf 41. % spina bifida

A=Spring B=Summer C=Autumn DzWinler

Fig. 2. Principal handicapping conditions by season of birth.

Region Variations in prevalence by region of domicile have been reported by identifying conditions in which the rate per 1000 children in a region is significantly higher than for the UK as a whole andin which the proportion of children with that condition in a region is significantly higher than the proportion of children nationally with that condition.

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TABLE IV Principal handicapping condition by class (two-parent families only)

Family Fund proportion Significantly mare manual than the FF register 20. Head injury 23. Severe asthma 27. Congenital defects bladder, etc. 39. Other bone disease 22. Cystic fibrosis 25. Renal disease 14. Epilepsy, convulsions 10. Mental handicap Significantly less manual than the FF register I I . Down’s syndrome 8. Autism, etc.

15. Cerebral palsy, etc. 49. Named syndromes 18. Tuberous sclerosis

75.5

86.4 85.1 82.3 82.3 81.8 81 .5 79.8 78.9

73.5 71.9 71.8 66 .4 61 .2

TABLE V Principal handicapping condition by proportion of singleparent families

%

Population proportion 11.3 Signijicantly more single parents than population 47. Burns 36.4 23. Severe asthma 25.7 4. Cretinism, thyroid disease 23.9

29. Maternal rubella 21.9 26. Digestive system disease 19.9 34. Rheumatoid arthritis 19.8 7. Other disease of the blood 19.7 6. Leukaemia 18.3

20. Head injury 18.0 5. Haemophilia 17.7

27. Congenital defects bladder, etc. 17.4 31. Deal’ 17.4 46. Other physical malformations 16.9 14. Epilepsy, convulsions 16.6 25. Renal disease 16.2

1. Tumours, cancer 16.1 22. Cystic fibrosis 15.8 43. Hydrocephalus 15.7 19. Other central nervous system disease 15.5 8. Autism, etc. 15.4

10. Mental handicap 15.0 39. Other bone disease 14.6 30. Blind, vision defect 14.5 1 I . Down’s syndrome 13.8 21. Heart disease 13.6 15. Cerebral palsy, etc. 12.5 49. Named syndromes 11.8 All Family Fund 13.9

Northern Ireland has a higher prevalence of diabetes, phenylketonuria, mental handicap and severe asthma. (Northern Ireland also has a higher rate of spina bifida but does not have a higher proportion of its handicapped children with spina bifida.) Scotland has higher rates of cerebral palsy, renal disease and bladder defects. The Northern region has higher rates of haemophilia and mental handicap. Yorkshire and Humber- side have higher rates ofepilepsy and other bone disease. Wales has higher rates of spina bifida, cretinism, leukaemia, severe asthma, other bone disease and other physical malformations. The South-east has higher rates of maternal rubella and deafness. The West Midlands has higher rates of microcephalus and the North-west has a higher rate of spina bifida.

One-parent families A higher prevalence of one-parent families among the Family Fund sample may indicate that children with severe dis- abilities are more likely to be born to single parents or that marriages are more likely to break up if there is a disabled child or that single parents with a disabled child are less likely to marry or remarry or that one- parent families are more likely to apply to the Fund. The proportion of one-parent families in the Family Fund is 13.9 per cent, significantly higher than the 11 .3 per cent for the UK population as a whole. Table V lists the conditions in which families are more likely to be headed by a single parent. Only spina bifida children (10.3 per cent) are less likely to be in single- parent families than would be expected in a normal sample of the population. When the comparison is restricted to the Family Fund sample, children with cretinism, mental handicap, epilepsy, severe asthma, maternal rubella, deafness, rheumatoid arthritis and severe burns are more likely to be living in single-parent families.

Large families With the exception of tuberous sclerosis, all conditions show a higher proportion of families with four or more dependent children than would be expected in a sample of families in the general population. The conditions which are significantly more likely than the rest of the

Family Fund sample to occur in large families are head injuries, severe burns, polio, congenital defects of the bladder, severe asthma and mental handicap (but not Down's syndrome) (see Table VI).

More than one disabled child 7 . 4 per cent of families applying to the Family Fund have more than one disabled child. Table VII shows that as well as the known genetically linked conditions, children severely disabled by asthma, blindness, deafness and skin disorders are more likely to have a disabled sibling, although not necessarily with the same condition. It is also interesting that children with Down's syndrome, hydro- cephalus, cerebral palsy and spina bifida are less likely to have a disabled sibling.

Conclusion In this article we have sought to demonstrate the limitations and potential of the Family Fund register for epidemi- ological investigations. The Family Fund sample is undoubtedly biased but the size of the register enables analyses to be made which can attempt to control for possible biases. It is also possible to use the register to investigate the social and health consequences of handicapping conditions, including some very rare ones. We would welcome interest from epidemiologists who wish to pursue further analysis of any condition.

Acknowledgements Versions of this paper were presented at the annual meeting of the Society of Social Medicine, Sheffield, 1983, and at the annual meeting of the British Paediatric Association, York, 1984.

Authors' Appointments *Jonathan Bradshaw, Professor of Social Policy; Director of the Social Policy Research Unit; Dorothy Lawton, Research fellow, Social Policy Research Unit; University of York, Heslington, York YO1 5DD.

*Correspondence to first author.

TABLE VI Principal handicapping condition by proportion of large families

%

Family Fund proportion ~

18.5 Significantly more than the FF register 17. Polio 65.1 47. Burns 38.7 20. Head injuries 27.2 23. Severe asthma 25.9 27. Congenital defects bladder; etc. 23.5

10. Mental handicap 19.6 14. Epilepsy, convulsions 22.0

TABLE VII Principal handicapping condition by presence of disabled sibling

%

Family Fund proportion 7.4 Signflcanrly more than the Family Fund register 16. Friedreich's ataxia 29.2 2. Phenylketonuria, etc. 19.9

22. Cystic fibrosis 19.5 5. Haemophilia 19.0

23. Severe asthma 17.9 33. Skin disorders 16.9

15.5 38. Osteogenesis imperfecta 14.3 36. Muscular dystrophy 14.2 31. Deaf 13.4 30. Blind, vision defect 10.8 10. Mental handicap 8 . 5 Significantly less than the Family Fund register 15. Cerebral palsy, etc. 4 . 6

7. Other disease of the blood

43. Hydrocephalus 4 . 5 11. Down's syndrome 3 . 9

13. Meningitis 3 . 4 41. Spina bifida 3 . 8

SUMMARY This article explores the epidemiological potential of the computerised record of applications to the Family Fund, which contains details of 75,000 children with severe disabilities in the UK. It illustrates the strengths and weaknesses of the record, with an analysis of variation in the characteristics of the children and their families by principal handicapping conditions.

RESUME 75 mille enfants en incapacite' se'vire Cet article analyse le potentiel tpidtmiologique des dossiers analysts sur ordinateur provenant du Family

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Fund et qui contiennent des donntes se rapportant sbvtre. Cette analyse dtmontre les forces et les faiblesses du dossier type et permet une analyse des variations des caracttristiques des enfants et de leur famille en fonction des principales conditions d’incapacitt.

75 mille enfants du Royaume Uni, avec incapaciti

ZUSAMMENFASSUNG 75,000 schwer behinderte Kinder Dieser Artikel untersucht das epidemiologische Potential der komputermiIl3ig erfaDten Antrage an den Familienfond, die Einzelheiten iiber 75,000 Kinder mit schweren Behinderungen im Vereinigten Konigreich enthalten. Es werden die Starken und Schwachen der Komputererfassung dargestellt und es wird eine Analyse Uber die Veranderung der Charakteristika dieser Kinder und ihrer Familien durch besondere Behinderungsbedingungen gegeben.

RESUMEN 75,000 nitios con minusvalias graves Este articulo explora el potencial epidemioldgico de la gravacidn computarizada de instancias realizada en la Fundaci6n de la Familia, que contiene detalles de 75,000 niilos con minusvalencias graves en el Reino Unido. Ilustra sobre 10s puntos fuertes y 10s dtbiles de 10s archivos, con un anllisis de la variacidn de las caracteristicas de 10s niilos y de sus familias de acuerdo con sus principales minusvalencias.

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London: English Universities Press. Baldwin, S. (1985) The Costs of Caring. London:

Routledge & Kegan Paul. - Godfrey, C., Staden, F. (1983) ‘Childhood

disablement and family income.’ Journal of Epidemiology and Community Health, 31,187-195.

Bradshaw, J. R. (1980) The Family Fund An Initiative in Social Policy. London: Routledge & Kegan Paul.

- Lawton, D. (1980) ‘An examination of equity in the administration of the Attendance Allowance.’ Policy 81 Politics, 8, ( I ) , 39-54. - - (1982) ‘Utilization of the Family Fund.’

Child: Care, Health and Development, 8, 221-231. - - (1978) ‘Some ch;tracteristics of children with severe disabilities. Journal of Biosocial Science, 10, 107-120.

Cooke, K., Bradshaw, J. R., Lawton, D. (1983) ‘Take-up of benefits by families with disabled

children.’ Child Care. Health and Development,

Glendinning, C. (1984) ‘The resource worker project: evaluating a specialist work service for severely disabled children and their families.’ British Journal of Social Work. 14, 103-115.

Hirst, M. (1983~) ‘Young people with disabilities: what happens after 16.’ Child Care, Health and Development. 9,273-284. - (19836) ‘The representation of child disable-

ment.’ Social Policy Research Unit University of York. DHSS 147. 7/83.

Parker, G. (1982) ‘The case for integrate! incontinence services for disabled children. Community Medicine, 4, 119-124.

Weak, J., Bradshaw, J. R. (1980) ‘Prevalence and characteristics of disabled children: findings from the 1974 General Household Survey.’ Journal of Epidemiology and Community Health, 34, I 1 1-1 18.

9, 145-156.

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