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Chronic Illness in Young People
920
the physiological aspects of these problems in detail." The late Sir GEOFFREY JEFFERSON once remarked that this age was more Cartesian than was generally realised. Science, however, no longer has the kind of optimism which imagined that a comprehensive explanation of the universe might follow the next empirical discovery, and DESCARTES’ simple dualism is probably inadequate for modern purposes. The unknowable universe is abstracted in four-dimensional terms by the living brain, which maintains a close relationship with the other unknowable term, the mind. If a compendious scheme of philosophy can be compressed to a sentence, this represents the more complex and sophisticated dualism of KANT, who named the features of the unknowable universe and the mind, noumena. From these, the brain received phenomena, which it analysed, according to its capabilities, by categories. Nothing that has been discovered in physical, physiological, or psychological science has really improved on this view. True, it has become appreciated that the brain is capable of directing its own activity by feedback pathways forming multiple goal-seeking circuits. But apart from this, and the realisation that the continuous activity of the cerebral network makes the brain-mind relationship one between a constantly moving signal and a moment of time, the vast amount of work in neurophysiology has added nothing to the basic concept. In this respect therefore it might be even truer to state that this age is more Kantian than it itself realises. Nevertheless, on the level of empiricism, attempts to correlate the feelings and thoughts of human beings with operations on their brains will continue to lie at the core of the problem of the human condition. Correlation
ing
between epilepsy (particularly psychomotor epilepsy) and temperament is one area of study. Investigation of the remaining elements of the limbic lobe such as the anterior and the posterior cingulate gyri, is another; and analysis of the disturbances of sensory categorisation or of speech by naturally occurring lesions is another. It may well turn out that these first deliberate attempts to influence the mind by operations on the brain will have even greater significance for subsequent generations than they have for us who are living with them and see them for the first time.
Chronic Illness in Young People MEASURES to promote the health and wellbeing of
country’s young people have developed enormously during the present century, and the improvement in their health has been plain to see. But most of the activity has been piecemeal and divided, in relation to age-groups and to specific disabilities, some provided by statutory authorities, some by voluntary authorities. this
of no-man’s-land which can be frustrating handicapped young people, to their parents, and to those who have their welfare at heart. Recently the report1 of the working parties set up by the British Council for Rehabilitation of the Disabled drew attention to some of these defects: and now a report bv There
are
still
areas
to
Handicapped School-leaver. British Council for Rehabilitation of Disabled, Tavistock House, Tavistock Square, London, W.C.1. Pp. 172. 15s.
1. The the
a subcommittee of the Scottish. Health Services Council, under the chairmanship of Lord MACDONALD, on young chronic sick2 returns to the subject. Indeed,-this short report of less than 10,000 words points to the need for reappraisal of our child care services, and makes a pithy and stimulating contribution to the welfare of young people. The Council received information about the incidence of child defects: there are about 1000 new cases of congenital heart-disease per annum in Scotland, an incidence of cerebral palsy of 2-04 per 1000 in children of school age, and in the West of Scotland about 1000 people suffering from muscular dystrophy, two-thirds of them children. Such figures imply an enormous number of human problems, some of which might be solved by earlier and better ascertainment. Regular clinical examinations during early childhood would help, and parents must be educated to see the vital importance of these examinations. There ought clearly to be continuity of care between child welfare and school health services. The thoroughness of ascertainment, even among children of school age, varies from one education authority to another; it is often inadequate, and seems to be partly related to the availability of special schooling. The committee believe that the school health service is not fully exploiting its opportunity to ascertain at an early stage the presence of illness or disability which may, in the absence of early treatment, lead to ill health later. At post-school ages, they would like to see extension of the machinery for periodic medical examination and they hope that family doctors will, so far as practicable, encourage young people to have regular health checks if the nature of their employment does not ensure that this is done. They advocate the establishment of a register of young handicapped, kept by the medical officer of health, registration to be on a voluntary basis. Such a register would form a means of identifying the needs of individual patients; it would provide-for planning services-a body of information about the incidence of particular disabilities and their impact on the community; and it could be an administrative tool in the hands of a coordinating agency for the various services involved in the care of the young chronic sick. It is recognised that the success of any registration system of the kind would depend entirely on the zeal, tact, and imagination of those who operate it. Counselling centres should be established to guide parents in the management of chronic illness in their children and to give advice to young adults with chronic disability. Local authorities should be encouraged to give sympathetic consideration to the housing needs of the parents of a handicapped child and to take an enlightened view of their responsibilities for internal adaptation designed to make life easier for handicapped people. Residential accommodation may be required for severely handicapped young people, one unit in Scotland for chronic sick young people with multiple handicaps being regarded as essential; and there is scope for 2.
The Young Chronic Sick. Report of a Sub-Committee appointed by the Scottish Health Services Council, Scottish Home and Health Department, Edinburgh. H.M. Stationery Office. Pp. 180. 1s. 6d.
921
development (with the help of voluntary organisations) of day centres, to provide accommodation where children can be looked after while relatives are carrying out other necessary family duties, to provide recreational facilities as well as occupational facilities for those who are able to make use of them. The provision of suitable employment for young handicapped people is important-and especially the need to help the schoolleaver to find a job in which he is likely to be able to continue successfully. The committee emphasise the need for improved coordination of services for the young chronic sick, and they believe that responsibility for coordination should be placed on an officer, preferably a medical officer, appointed by the medical officer of health in each area. Even when a doctor, he would not, of course, have clinical responsibility for patients, but he would be expected to advise family doctors, hospital consultants, and almoners on the provision of domiciliary services; to advise relatives on the availability of services; to see that registered disabled were in touch with existing services; and to bring together statutory and voluntary agencies. He should not be merely a post office" but should play a useful and constructive part in the provision of services. the
"
Annotations MALFORMATION SEASONS
THERE
many ways of studying the causes of malbut so far clinical inquiry has proved the most formations, fruitful. The inquiry may take the form of an ad-hoc search for the cause of a malformation epidemic, or a longterm search for correlations between the appearance of malformations and an illimitable number of genetic and environmental variables. The first approach linked cataract with rubella and reduction deformities of the limbs with thalidomide. The second approach has revealed a number of pieces of an enormous jigsaw puzzle. One day they will fall into place and a pattern will be revealed. One of the most fascinating pieces of the puzzle is the apparent seasonal variation in the incidence of certain malformations. Anencephalic births were found to be about 50% more common in the winter months than in the summer in Birmingham and Scotland,I-3 but not in Rhode Island.4 A similar seasonal trend for spina bifida has been reported from Manchester5 (but not from dislocation Birmingham or Scotland1 2) and for congenital of the hip from Birmingham and Japan.6 The incidence of patent ductus arteriosus was found to be highest from October to January in Boston7and from May to August are
in Birmingham.8 A retrospective survey of congenital abnormalities carried out by the College of General Practitioners provided data for a recent preliminary report by Slater, Watson, and McDonald 9 on seasonal variations. Members and associates of the college in the United Kingdom and Eire 1. 2. 3. 4. 5. 6. 7.
8. 9.
McKeown, T., Record, R. G. Lancet, 1951, i, 192. Edwards, J. H. Brit. J. prev. soc. Med. 1958, 12, 115. Record, R. G. ibid. 1961, 15, 93. MacMahon, B., Pugh, T. F., Ingalls, T. H. ibid. 1953, 7, 211.
Guthkelch, A. H. ibid. 1962, 16, 159. Record, R. G., Edwards, J. H. ibid. 1958, 12, 8. Rutstein, D. D., Nickerson, R. J., Heald, F. P.
Amer. J. Dis. Child. 1952, 84, 199. Record, R. G., McKeown, T. Brit. Heart J. 1953, 15, 376. Slater, B. C. S., Watson, G. I., McDonald, J. C. Brit. J. prev. soc. Med. 1964, 18,1.
invited to supply details of infants with congenital defects born between 1954 and 1960. About a third of the 4000 practitioners responded, providing information on 12,900 defects in 9951 children. This represents about 7% of all malformed infants born in the time and place studied, but there is no reason to suppose that the sample size should influence any apparent seasonal variations. The total numbers of malformations in each -quarter of the year were comparable. The numbers of malformations in each of 83 diagnostic categories were examined by comparing the 1st and 2nd quarters with the 3rd and 4th, and the 2nd and 3rd with the 4th and lst. The authors point out that in 166 x2 tests, about 8 will reach 5% significance by chance (and 1 or 2 will reach 1 % significance). In fact, probabilities of 0.05 or less were found in 17 tests, and of 0.01 or less in a further 5 tests. 16 of the 22 significant results appeared in the summer/winter comparison. A striking winter excess was found in anencephaly, spina bifida, cataract, oesophageal atresia, and congenital dislocation of the hip. A summer excess was seen in abnormalities of the aorta and pulmonary artery and their valves, some categories of limb defects, and anorectal abnormalities. These observations are of great interest but require cautious interpretation. The winter excess for cesophageal atresia did not apply to oesophagotracheal fistula, and the relative numbers of cases of these two conditions (86 and 46 respectively) suggest that the terms may have been used rather loosely by the informants. The 5-to-3 summer excess for partial absence of limbs may be related to the fact that in Great Britain the " thalidomide epidemic covered roughly 3 summers and 2 winters. When evidence appears conflicting-as in the seasonal incidence of anencephaly and spina bifida-further research is clearly needed. As information on seasonal variations in malformations accumulates, it will become possible to compare one region with another and one year with another. It may well prove fruitful to ask: in what way do seasons vary in time and place ? were
THE SIMIAN CREASE
INTERPRETING the palms of the hands (and the soles of the feet) is now a respectable science. The shape of the hands and fingers, the form of the skin creases, and the pattern of the dermal ridges all contain information of diagnostic relevance. Of these three, the dermal ridge arrangements (dermatoglyphics) seem to contain the most information: they certainly discriminate with remarkable reliability between monozygotic and dizygotic twins, and between mongols and normals, and they show characteristic patterns in other karyotypic abnormalities. Further exploitation of the information in the dermal ridges is a
fascinating study. The internal structure of the ridges is another reason for giving them special attention. They seem to act as a lever mechanism for magnifying very small surface pressures and especially transverse movements. The ridges may be regarded as a special sense organ, an extension of the nervous system, and therefore likely to supply fundamental information on development. The widespread notion that the ridges are no more than a gripping surface, " a tread ", is a little implausible when given a second thought (perhaps this idea arises from tyre advertisements, though in fact the main function of the grooves in a tyre is to accommodate the water film squeezed from a wet
surface).
the physiological aspects of these problems in detail." The late Sir GEOFFREY JEFFERSON once remarked that this age was more Cartesian than was generally realised. Science, however, no longer has the kind of optimism which imagined that a comprehensive explanation of the universe might follow the next empirical discovery, and DESCARTES’ simple dualism is probably inadequate for modern purposes. The unknowable universe is abstracted in four-dimensional terms by the living brain, which maintains a close relationship with the other unknowable term, the mind. If a compendious scheme of philosophy can be compressed to a sentence, this represents the more complex and sophisticated dualism of KANT, who named the features of the unknowable universe and the mind, noumena. From these, the brain received phenomena, which it analysed, according to its capabilities, by categories. Nothing that has been discovered in physical, physiological, or psychological science has really improved on this view. True, it has become appreciated that the brain is capable of directing its own activity by feedback pathways forming multiple goal-seeking circuits. But apart from this, and the realisation that the continuous activity of the cerebral network makes the brain-mind relationship one between a constantly moving signal and a moment of time, the vast amount of work in neurophysiology has added nothing to the basic concept. In this respect therefore it might be even truer to state that this age is more Kantian than it itself realises. Nevertheless, on the level of empiricism, attempts to correlate the feelings and thoughts of human beings with operations on their brains will continue to lie at the core of the problem of the human condition. Correlation
ing
between epilepsy (particularly psychomotor epilepsy) and temperament is one area of study. Investigation of the remaining elements of the limbic lobe such as the anterior and the posterior cingulate gyri, is another; and analysis of the disturbances of sensory categorisation or of speech by naturally occurring lesions is another. It may well turn out that these first deliberate attempts to influence the mind by operations on the brain will have even greater significance for subsequent generations than they have for us who are living with them and see them for the first time.
Chronic Illness in Young People MEASURES to promote the health and wellbeing of
country’s young people have developed enormously during the present century, and the improvement in their health has been plain to see. But most of the activity has been piecemeal and divided, in relation to age-groups and to specific disabilities, some provided by statutory authorities, some by voluntary authorities. this
of no-man’s-land which can be frustrating handicapped young people, to their parents, and to those who have their welfare at heart. Recently the report1 of the working parties set up by the British Council for Rehabilitation of the Disabled drew attention to some of these defects: and now a report bv There
are
still
areas
to
Handicapped School-leaver. British Council for Rehabilitation of Disabled, Tavistock House, Tavistock Square, London, W.C.1. Pp. 172. 15s.
1. The the
a subcommittee of the Scottish. Health Services Council, under the chairmanship of Lord MACDONALD, on young chronic sick2 returns to the subject. Indeed,-this short report of less than 10,000 words points to the need for reappraisal of our child care services, and makes a pithy and stimulating contribution to the welfare of young people. The Council received information about the incidence of child defects: there are about 1000 new cases of congenital heart-disease per annum in Scotland, an incidence of cerebral palsy of 2-04 per 1000 in children of school age, and in the West of Scotland about 1000 people suffering from muscular dystrophy, two-thirds of them children. Such figures imply an enormous number of human problems, some of which might be solved by earlier and better ascertainment. Regular clinical examinations during early childhood would help, and parents must be educated to see the vital importance of these examinations. There ought clearly to be continuity of care between child welfare and school health services. The thoroughness of ascertainment, even among children of school age, varies from one education authority to another; it is often inadequate, and seems to be partly related to the availability of special schooling. The committee believe that the school health service is not fully exploiting its opportunity to ascertain at an early stage the presence of illness or disability which may, in the absence of early treatment, lead to ill health later. At post-school ages, they would like to see extension of the machinery for periodic medical examination and they hope that family doctors will, so far as practicable, encourage young people to have regular health checks if the nature of their employment does not ensure that this is done. They advocate the establishment of a register of young handicapped, kept by the medical officer of health, registration to be on a voluntary basis. Such a register would form a means of identifying the needs of individual patients; it would provide-for planning services-a body of information about the incidence of particular disabilities and their impact on the community; and it could be an administrative tool in the hands of a coordinating agency for the various services involved in the care of the young chronic sick. It is recognised that the success of any registration system of the kind would depend entirely on the zeal, tact, and imagination of those who operate it. Counselling centres should be established to guide parents in the management of chronic illness in their children and to give advice to young adults with chronic disability. Local authorities should be encouraged to give sympathetic consideration to the housing needs of the parents of a handicapped child and to take an enlightened view of their responsibilities for internal adaptation designed to make life easier for handicapped people. Residential accommodation may be required for severely handicapped young people, one unit in Scotland for chronic sick young people with multiple handicaps being regarded as essential; and there is scope for 2.
The Young Chronic Sick. Report of a Sub-Committee appointed by the Scottish Health Services Council, Scottish Home and Health Department, Edinburgh. H.M. Stationery Office. Pp. 180. 1s. 6d.
921
development (with the help of voluntary organisations) of day centres, to provide accommodation where children can be looked after while relatives are carrying out other necessary family duties, to provide recreational facilities as well as occupational facilities for those who are able to make use of them. The provision of suitable employment for young handicapped people is important-and especially the need to help the schoolleaver to find a job in which he is likely to be able to continue successfully. The committee emphasise the need for improved coordination of services for the young chronic sick, and they believe that responsibility for coordination should be placed on an officer, preferably a medical officer, appointed by the medical officer of health in each area. Even when a doctor, he would not, of course, have clinical responsibility for patients, but he would be expected to advise family doctors, hospital consultants, and almoners on the provision of domiciliary services; to advise relatives on the availability of services; to see that registered disabled were in touch with existing services; and to bring together statutory and voluntary agencies. He should not be merely a post office" but should play a useful and constructive part in the provision of services. the
"
Annotations MALFORMATION SEASONS
THERE
many ways of studying the causes of malbut so far clinical inquiry has proved the most formations, fruitful. The inquiry may take the form of an ad-hoc search for the cause of a malformation epidemic, or a longterm search for correlations between the appearance of malformations and an illimitable number of genetic and environmental variables. The first approach linked cataract with rubella and reduction deformities of the limbs with thalidomide. The second approach has revealed a number of pieces of an enormous jigsaw puzzle. One day they will fall into place and a pattern will be revealed. One of the most fascinating pieces of the puzzle is the apparent seasonal variation in the incidence of certain malformations. Anencephalic births were found to be about 50% more common in the winter months than in the summer in Birmingham and Scotland,I-3 but not in Rhode Island.4 A similar seasonal trend for spina bifida has been reported from Manchester5 (but not from dislocation Birmingham or Scotland1 2) and for congenital of the hip from Birmingham and Japan.6 The incidence of patent ductus arteriosus was found to be highest from October to January in Boston7and from May to August are
in Birmingham.8 A retrospective survey of congenital abnormalities carried out by the College of General Practitioners provided data for a recent preliminary report by Slater, Watson, and McDonald 9 on seasonal variations. Members and associates of the college in the United Kingdom and Eire 1. 2. 3. 4. 5. 6. 7.
8. 9.
McKeown, T., Record, R. G. Lancet, 1951, i, 192. Edwards, J. H. Brit. J. prev. soc. Med. 1958, 12, 115. Record, R. G. ibid. 1961, 15, 93. MacMahon, B., Pugh, T. F., Ingalls, T. H. ibid. 1953, 7, 211.
Guthkelch, A. H. ibid. 1962, 16, 159. Record, R. G., Edwards, J. H. ibid. 1958, 12, 8. Rutstein, D. D., Nickerson, R. J., Heald, F. P.
Amer. J. Dis. Child. 1952, 84, 199. Record, R. G., McKeown, T. Brit. Heart J. 1953, 15, 376. Slater, B. C. S., Watson, G. I., McDonald, J. C. Brit. J. prev. soc. Med. 1964, 18,1.
invited to supply details of infants with congenital defects born between 1954 and 1960. About a third of the 4000 practitioners responded, providing information on 12,900 defects in 9951 children. This represents about 7% of all malformed infants born in the time and place studied, but there is no reason to suppose that the sample size should influence any apparent seasonal variations. The total numbers of malformations in each -quarter of the year were comparable. The numbers of malformations in each of 83 diagnostic categories were examined by comparing the 1st and 2nd quarters with the 3rd and 4th, and the 2nd and 3rd with the 4th and lst. The authors point out that in 166 x2 tests, about 8 will reach 5% significance by chance (and 1 or 2 will reach 1 % significance). In fact, probabilities of 0.05 or less were found in 17 tests, and of 0.01 or less in a further 5 tests. 16 of the 22 significant results appeared in the summer/winter comparison. A striking winter excess was found in anencephaly, spina bifida, cataract, oesophageal atresia, and congenital dislocation of the hip. A summer excess was seen in abnormalities of the aorta and pulmonary artery and their valves, some categories of limb defects, and anorectal abnormalities. These observations are of great interest but require cautious interpretation. The winter excess for cesophageal atresia did not apply to oesophagotracheal fistula, and the relative numbers of cases of these two conditions (86 and 46 respectively) suggest that the terms may have been used rather loosely by the informants. The 5-to-3 summer excess for partial absence of limbs may be related to the fact that in Great Britain the " thalidomide epidemic covered roughly 3 summers and 2 winters. When evidence appears conflicting-as in the seasonal incidence of anencephaly and spina bifida-further research is clearly needed. As information on seasonal variations in malformations accumulates, it will become possible to compare one region with another and one year with another. It may well prove fruitful to ask: in what way do seasons vary in time and place ? were
THE SIMIAN CREASE
INTERPRETING the palms of the hands (and the soles of the feet) is now a respectable science. The shape of the hands and fingers, the form of the skin creases, and the pattern of the dermal ridges all contain information of diagnostic relevance. Of these three, the dermal ridge arrangements (dermatoglyphics) seem to contain the most information: they certainly discriminate with remarkable reliability between monozygotic and dizygotic twins, and between mongols and normals, and they show characteristic patterns in other karyotypic abnormalities. Further exploitation of the information in the dermal ridges is a
fascinating study. The internal structure of the ridges is another reason for giving them special attention. They seem to act as a lever mechanism for magnifying very small surface pressures and especially transverse movements. The ridges may be regarded as a special sense organ, an extension of the nervous system, and therefore likely to supply fundamental information on development. The widespread notion that the ridges are no more than a gripping surface, " a tread ", is a little implausible when given a second thought (perhaps this idea arises from tyre advertisements, though in fact the main function of the grooves in a tyre is to accommodate the water film squeezed from a wet
surface).