- Email: [email protected]
CARE OF DISABLED—A CACOPHONY
613 VT and
SVT, and this in turn will improve their diagnostic
approach is of course clinical and electrocardiographic
accuracy. The cornerstone of this
familiarity with the
criteria. Algorithms may be a useful aide-memoire.8 At the end of the day there are bound to be some patients whose tachycardia does not fall neatly into either group, SVT or VT. One should not be ashamed to call this "broad-complex tachycardia" until such time as a firm diagnosis can be made. If urgent treatment is needed for haemodynamic reasons, then an agent that is active at both atrial and ventricular levels should be chosen. But if the patient’s condition allows, the opportunity for recording an oesophageal or intracardiac ECG should be taken. Verapamil can be dangerous in VT, and should be reserved for patients in whom the diagnosis of SVT is unequivocal.
CARE OF DISABLED—A
CACOPHONY
IN one of those endless dreams we all have, the more musical among us may have been afflicted by a conductorless orchestra. It is not too difficult to imagine, with violins playing tunefully out of step with the rest of the orchestra,
with drums beating their own rhythm and the beautiful sounds of woodwind lost in the melee of noise. Such a nightmare might be considered a far-fetched parallel to current provision for the long-term ill and disabled in the UK. But it is not. The Royal College of Physicians reportl of July, 1986 (see Lancet, July 19, p 171) draws attention to the fact that for many years there has been professional and public concern about the care given to disabled people. Numerous surveys of various medical disorders (eg,
multiple sclerosis, strokes, amputation, myocardial infarction, ileostomy, colostomy, and incontinence) have revealed a uniformity of themes. There are great deficiencies in specialist medical staffing (eg, rheumatologists and neurologists); patients often receive inadequate information and see a succession of junior doctors. There are also major problems in provision with respect to housing, employment, and care allowances. In this situation, and lacking information, the disabled person cannot take appropriate decisions to retain autonomy, and is only too aware that spouses’ and children’s jobs and other activities are curtailed, often drastically, because of his disability. How has this sad state of affairs come about? Why have doctors practising in the NHS failed to take note of the demographic changes that have taken place in the past 20-30 years? As tuberculosis and poliomyelitis and other infectious diseases have receded, and as surgery has become more adventurous and successful, how have we failed to see what is left, uncured and almost uncared for? The Royal College of Physicians did notice some time ago; in 1978, it published its first report in which it was recommended that all physicians and surgeons dealt with their own patients’ disabilities. But little has changed since then and, with hindsight, perhaps it was unrealistic to expect it to do so. Physicians and surgeons find themselves too busy to make links with social services or the housing department to ensure that their disabled patients’ needs are understood, especially necessary at a time of financial constraint when the budgets of these departments are not expanding and not coping with the needs of the very elderly. Nor do they have
time to run clinics for physically handicapped school leavers, set up disabled living centres, run wheelchair clinics, or investigate the advantages which good or even purpose-built seating would bring some of their patients (eg, in reducing pressure sores). So, for the majority of patients, in most districts, these facilities either do not exist or have developed in isolation. In a few centres, notably where chairs of rehabilitation have been established or where highly motivated consultants work with considerable interest in the specialty, there have been pioneer developments and expert knowledge has begun to accumulate. Things have evolved differently in geriatrics and paediatrics. There are no longer general physicians with a minor interest in children. Provision for children is cohesive, yet at 16 it fragments, so that the shock of emerging to adulthood in a competitive, hard world is all the greater and is not eased by proper transfer of care within the Health Service. At the other end of life, the lot of many elderly persons has been greatly improved with effective geriatric care. Although some very elderly people require much hospital care, most remain at home, often alone and often leading energetic and fulfilled lives to the end. In contrast, adults of the same age as the doctors who serve them are a deprived population; it is not fortuitious that there are few doctors with any formal professional commitment to the provision of a medical disability service for this age group. The Royal College proposes that in each district there should be some ten sessions of consultant time devoted to disability, divided as appropriate between relevant medical specialties, but allowing each consultant sufficient time to become both a catalyst for change and a focus for help for colleagues. At a regional level, regional units for severely disabled patients, especially those with multiple disability, are proposed; links with artificial limb and appliance centres, disabled living centres, and bioengineering departments are necessary, and teaching, research, and evaluation should be part of their brief. In addition, the report strongly recommends a system of audit, with timetabling. Such improved provision might be thought expensive, but there is evidence that prolonged disability is ’also expensive, even with little or no management. Many will be familiar with the "bed-blocker" who merely needs support or suitable accommodation, and with the round of "social admissions" to medical wards who thereby occupy expensive and inappropriate accommodation. The appointment of physicians with a commitment to medical disability is vital; this itself depends on the foresight of doctors and administrators, both in the districts and at regional level, who are in a position to ensure the change occurs. Publication of a College document is always of importance and frequently signals benefits for patients. Let us hope that this one is heeded without delay, so that the cacophony of a conductorless orchestra is stilled.
**A second report3 on disability from the Royal College of Physicians was published last week. It is based on a survey of opinions, resources, and innovations (largely in England) which was conducted in 1984 and 1985 by a research fellow of the College, Dr J. F. Harrison, a consultant geriatrician in Birmingham. Supported financially by the Development Trust for the Young Disabled, the survey examined the 2.
8
Dancy M, Ward D. Diagnosis of ventricular tachycardia: a clinical algorithm. Br Med
J 1985, 291: 1036. 1 Physical disability in 1986 and beyond. A report of the Royal College of Physicians. JR Coll Phys Lond 1986; 20: 161-94.
Develop Med Child Neurol 27 (suppl 50). London: Spastics International Medical Publications, 1985. 3. The Young Disabled Adult: The use of residential homes and hospital units for the age group 16-64. September, 1986.Royal College of Physicians, 11 St Andrew’s Place, London NW1 4LE. £4.95.
614
support and care available to severely disabled adults who below retirement age, when the resources which their family and friends can supply are inadequate to meet the need. It concentrates on the services provided by residential homes and by hospital units, setting them in the context of all the other services which are or should be available. We shall return to this new report in a later editorial.
are
FAMILIAL MOLES AND MALIGNANT MELANOMA IN THE NETHERLANDS EIGHT
years
ago
two
groups
described
almost
syndrome of multiple clinically and histologically atypical cutaneous melanocytic naevi in families who were clearly at significantly increased risk of cutaneous malignant melanoma.1,2 These observations have not only stimulated interest, research, and argument but also simultaneously
a
familial
formed part of the basis for a National Institutes of Health consensus development conference on precursors of malignant melanoma.3 Bergman and her colleagues in the Netherlands now report the results of a detailed clinical, pathological, and genetic study of six families with this syndrome.4 Four of the six families come from a small fishing village near Leiden, although there is no proof of a common ancestor. 314 family members have been traced and 243 of these have been examined by one of the Dutch investigators. To date, malignant melanoma has developed in 33 members of the families (10-5%). The Netherlands has an overall annual incidence of new cases of melanoma of between 6 and 7 per 100 000, therefore this percentage clearly represents a figure several orders of magnitude higher than that which would be expected in the general Dutch population. One of the groups responsible for first recognising the syndrome has previously suggested that affected families may also have an increased frequency of non-melanoma cancers.5 It is therefore interesting that two of the Dutch families appear to have a high frequency of other malignancies. 22 primary tumours other than malignant melanoma have been diagnosed in 19 members of these kindred-these include 6 pancreatic, 3 gastrointestinal, 4 laryngeal, and 2 pulmonary malignancies. None of the other tumours has developed in patients who already have malignant melanoma. Without further information on the age at which the tumours developed and on the incidence of these types of malignancy in the Netherlands, it is difficult to establish the significance of these observations. However, the non-melanoma malignancies appear to be concentrated in only two of the six kindred. Clinical examination of the 22 patients with melanoma who remained alive showed that 12 (54%) had both clinical and histological evidence of atypical or dysplastic melanocytic naevi. A further 28 members of the families had ’
naevi but did not have malignant melanoma. An additional 36 family members had naevi believed by the investigators to be clinically unusual but "quiet", and were not thought to merit biopsy and histological examination. Thus, of 243 family members
histologically atypical
1. 2.
Lynch HT, Fnchot BC, Lynch JF. Familial atypical mole malignant melanoma syndrome. JMed Genet 1978; 15: 352-56. Clark WH, Reimer RR, Greene M, et al. Origin of familial malignant melarioma from hentable melanocytic lesions. The BK mole syndrome. Arch Dermatol 1978; 114:
examined 76 (31 %) had atypical naevi; malignant melanoma had developed in 12 (17% or 1 in 6). Nevertheless, it is important to note that 10 of the family members who had malignant melanoma did not have clinical or histological evidence of any atypical naevi. Clearly further work is required relating age at development of malignant melanoma to total lifetime risk of the tumour in members of these families, with and without atypical naevi. Pedigree analysis of the affected families shows vertical transmission through three consecutive generations with equal sex distribution; this pattern clearly suggests an autosomal dominant mode of inheritance. 91 obligate gene carriers have been identified in the families, 15 of whom do not have an atypical naevus pattern. The non-penetrance rate on the basis of the figures quoted in the Dutch study is estimated at 0-45, but there are considerable variations between the six families. An important feature of families with this particular constellation of problems is that multiple primary melanomas are not uncommon. Patients who have already had one primary melanoma must be shown how to recognise a second primary at an early stage. In the Dutch series, 8 of the 22 (36%) melanoma patients had multiple primaries, while the frequency in the general melanoma population is quoted at between 2 % and 3 % .6 The Dutch study has obviously involved much painstaking clinical follow-up. Further study of these familes should provide the answers to several additional clinical points. There is much controversy about the age at which the majority of atypical or dysplastic naevi first develop; most normal naevi appear around the time of puberty,’ but it has been suggested that a large number of atypical naevi develop in early childhood. The Dutch kindred could help to settle this issue. Similarly, careful examination of children in the Dutch families could reveal whether the incidence of congenital naevi in family members who subsequently get melanoma is higher than the figure of 1 % quoted in the general population. Other outstanding questions concern the effect of exogenous hormones such as the contraceptive pill or pregnancy on future melanoma risk. Moreover, it is unclear whether the site of malignant melanoma in individuals with the syndrome is the same body site occupied by maximum numbers of naevi. In addition to the clinical studies suggested above, familial cancer presents an excellent opportunity for study of activated oncogenes in the aetiology of various types of malignancy; it is perhaps in this respect that the large group of patients in The Netherlands offers the most exciting prospect. Tissue from more than one primary malignant melanoma, from secondary tumour deposits in lymph nodes and other sites, and from benign melanocytic naevi may all be available from the one patient, along with normal tissue. Comparative analysis of these specimens should clearly establish the association of activated oncogene expression with primary tumours, secondary tumours, and possible precursor lesions by comparison with normal control tissue, all taken from the same source. Comparison of oncogene activation in several members of one family, and between affected families, could also help to establish whether observations on oncogene activation can be extended to all melanomas in these kindreds.
732-38. 3 National Institutes of Health. Precursors
to
malignant melanoma. JAMA 1984; 251:
864-66. 4. Bergman W, Palan A, Went LN. Clinical and genetic studies in six Dutch kindreds with the dysplasuc naevus syndrome. Ann Hum Genet 1986; 50: 249-58. 5. Lynch HT, Fusaro RM, Pester J, et al. Tumour spectrum in the FAMMM syndrome.
Br J Cancer 1981; 44: 553-60.
6. Greene MH, Fraumeni JF. The hereditary variant of malignant melanoma. In: Clark WH, ed. Human malignant melanoma. New York: Grune and Stratton, 1979. 7. Mackie RM, English J, Aitchison T, Fitzsimons CP, Wilson P. The number and distribution of melanocytic naevi in a British population. Br J Dermatol 1985; 113: 167-74.
SVT, and this in turn will improve their diagnostic
approach is of course clinical and electrocardiographic
accuracy. The cornerstone of this
familiarity with the
criteria. Algorithms may be a useful aide-memoire.8 At the end of the day there are bound to be some patients whose tachycardia does not fall neatly into either group, SVT or VT. One should not be ashamed to call this "broad-complex tachycardia" until such time as a firm diagnosis can be made. If urgent treatment is needed for haemodynamic reasons, then an agent that is active at both atrial and ventricular levels should be chosen. But if the patient’s condition allows, the opportunity for recording an oesophageal or intracardiac ECG should be taken. Verapamil can be dangerous in VT, and should be reserved for patients in whom the diagnosis of SVT is unequivocal.
CARE OF DISABLED—A
CACOPHONY
IN one of those endless dreams we all have, the more musical among us may have been afflicted by a conductorless orchestra. It is not too difficult to imagine, with violins playing tunefully out of step with the rest of the orchestra,
with drums beating their own rhythm and the beautiful sounds of woodwind lost in the melee of noise. Such a nightmare might be considered a far-fetched parallel to current provision for the long-term ill and disabled in the UK. But it is not. The Royal College of Physicians reportl of July, 1986 (see Lancet, July 19, p 171) draws attention to the fact that for many years there has been professional and public concern about the care given to disabled people. Numerous surveys of various medical disorders (eg,
multiple sclerosis, strokes, amputation, myocardial infarction, ileostomy, colostomy, and incontinence) have revealed a uniformity of themes. There are great deficiencies in specialist medical staffing (eg, rheumatologists and neurologists); patients often receive inadequate information and see a succession of junior doctors. There are also major problems in provision with respect to housing, employment, and care allowances. In this situation, and lacking information, the disabled person cannot take appropriate decisions to retain autonomy, and is only too aware that spouses’ and children’s jobs and other activities are curtailed, often drastically, because of his disability. How has this sad state of affairs come about? Why have doctors practising in the NHS failed to take note of the demographic changes that have taken place in the past 20-30 years? As tuberculosis and poliomyelitis and other infectious diseases have receded, and as surgery has become more adventurous and successful, how have we failed to see what is left, uncured and almost uncared for? The Royal College of Physicians did notice some time ago; in 1978, it published its first report in which it was recommended that all physicians and surgeons dealt with their own patients’ disabilities. But little has changed since then and, with hindsight, perhaps it was unrealistic to expect it to do so. Physicians and surgeons find themselves too busy to make links with social services or the housing department to ensure that their disabled patients’ needs are understood, especially necessary at a time of financial constraint when the budgets of these departments are not expanding and not coping with the needs of the very elderly. Nor do they have
time to run clinics for physically handicapped school leavers, set up disabled living centres, run wheelchair clinics, or investigate the advantages which good or even purpose-built seating would bring some of their patients (eg, in reducing pressure sores). So, for the majority of patients, in most districts, these facilities either do not exist or have developed in isolation. In a few centres, notably where chairs of rehabilitation have been established or where highly motivated consultants work with considerable interest in the specialty, there have been pioneer developments and expert knowledge has begun to accumulate. Things have evolved differently in geriatrics and paediatrics. There are no longer general physicians with a minor interest in children. Provision for children is cohesive, yet at 16 it fragments, so that the shock of emerging to adulthood in a competitive, hard world is all the greater and is not eased by proper transfer of care within the Health Service. At the other end of life, the lot of many elderly persons has been greatly improved with effective geriatric care. Although some very elderly people require much hospital care, most remain at home, often alone and often leading energetic and fulfilled lives to the end. In contrast, adults of the same age as the doctors who serve them are a deprived population; it is not fortuitious that there are few doctors with any formal professional commitment to the provision of a medical disability service for this age group. The Royal College proposes that in each district there should be some ten sessions of consultant time devoted to disability, divided as appropriate between relevant medical specialties, but allowing each consultant sufficient time to become both a catalyst for change and a focus for help for colleagues. At a regional level, regional units for severely disabled patients, especially those with multiple disability, are proposed; links with artificial limb and appliance centres, disabled living centres, and bioengineering departments are necessary, and teaching, research, and evaluation should be part of their brief. In addition, the report strongly recommends a system of audit, with timetabling. Such improved provision might be thought expensive, but there is evidence that prolonged disability is ’also expensive, even with little or no management. Many will be familiar with the "bed-blocker" who merely needs support or suitable accommodation, and with the round of "social admissions" to medical wards who thereby occupy expensive and inappropriate accommodation. The appointment of physicians with a commitment to medical disability is vital; this itself depends on the foresight of doctors and administrators, both in the districts and at regional level, who are in a position to ensure the change occurs. Publication of a College document is always of importance and frequently signals benefits for patients. Let us hope that this one is heeded without delay, so that the cacophony of a conductorless orchestra is stilled.
**A second report3 on disability from the Royal College of Physicians was published last week. It is based on a survey of opinions, resources, and innovations (largely in England) which was conducted in 1984 and 1985 by a research fellow of the College, Dr J. F. Harrison, a consultant geriatrician in Birmingham. Supported financially by the Development Trust for the Young Disabled, the survey examined the 2.
8
Dancy M, Ward D. Diagnosis of ventricular tachycardia: a clinical algorithm. Br Med
J 1985, 291: 1036. 1 Physical disability in 1986 and beyond. A report of the Royal College of Physicians. JR Coll Phys Lond 1986; 20: 161-94.
Develop Med Child Neurol 27 (suppl 50). London: Spastics International Medical Publications, 1985. 3. The Young Disabled Adult: The use of residential homes and hospital units for the age group 16-64. September, 1986.Royal College of Physicians, 11 St Andrew’s Place, London NW1 4LE. £4.95.
614
support and care available to severely disabled adults who below retirement age, when the resources which their family and friends can supply are inadequate to meet the need. It concentrates on the services provided by residential homes and by hospital units, setting them in the context of all the other services which are or should be available. We shall return to this new report in a later editorial.
are
FAMILIAL MOLES AND MALIGNANT MELANOMA IN THE NETHERLANDS EIGHT
years
ago
two
groups
described
almost
syndrome of multiple clinically and histologically atypical cutaneous melanocytic naevi in families who were clearly at significantly increased risk of cutaneous malignant melanoma.1,2 These observations have not only stimulated interest, research, and argument but also simultaneously
a
familial
formed part of the basis for a National Institutes of Health consensus development conference on precursors of malignant melanoma.3 Bergman and her colleagues in the Netherlands now report the results of a detailed clinical, pathological, and genetic study of six families with this syndrome.4 Four of the six families come from a small fishing village near Leiden, although there is no proof of a common ancestor. 314 family members have been traced and 243 of these have been examined by one of the Dutch investigators. To date, malignant melanoma has developed in 33 members of the families (10-5%). The Netherlands has an overall annual incidence of new cases of melanoma of between 6 and 7 per 100 000, therefore this percentage clearly represents a figure several orders of magnitude higher than that which would be expected in the general Dutch population. One of the groups responsible for first recognising the syndrome has previously suggested that affected families may also have an increased frequency of non-melanoma cancers.5 It is therefore interesting that two of the Dutch families appear to have a high frequency of other malignancies. 22 primary tumours other than malignant melanoma have been diagnosed in 19 members of these kindred-these include 6 pancreatic, 3 gastrointestinal, 4 laryngeal, and 2 pulmonary malignancies. None of the other tumours has developed in patients who already have malignant melanoma. Without further information on the age at which the tumours developed and on the incidence of these types of malignancy in the Netherlands, it is difficult to establish the significance of these observations. However, the non-melanoma malignancies appear to be concentrated in only two of the six kindred. Clinical examination of the 22 patients with melanoma who remained alive showed that 12 (54%) had both clinical and histological evidence of atypical or dysplastic melanocytic naevi. A further 28 members of the families had ’
naevi but did not have malignant melanoma. An additional 36 family members had naevi believed by the investigators to be clinically unusual but "quiet", and were not thought to merit biopsy and histological examination. Thus, of 243 family members
histologically atypical
1. 2.
Lynch HT, Fnchot BC, Lynch JF. Familial atypical mole malignant melanoma syndrome. JMed Genet 1978; 15: 352-56. Clark WH, Reimer RR, Greene M, et al. Origin of familial malignant melarioma from hentable melanocytic lesions. The BK mole syndrome. Arch Dermatol 1978; 114:
examined 76 (31 %) had atypical naevi; malignant melanoma had developed in 12 (17% or 1 in 6). Nevertheless, it is important to note that 10 of the family members who had malignant melanoma did not have clinical or histological evidence of any atypical naevi. Clearly further work is required relating age at development of malignant melanoma to total lifetime risk of the tumour in members of these families, with and without atypical naevi. Pedigree analysis of the affected families shows vertical transmission through three consecutive generations with equal sex distribution; this pattern clearly suggests an autosomal dominant mode of inheritance. 91 obligate gene carriers have been identified in the families, 15 of whom do not have an atypical naevus pattern. The non-penetrance rate on the basis of the figures quoted in the Dutch study is estimated at 0-45, but there are considerable variations between the six families. An important feature of families with this particular constellation of problems is that multiple primary melanomas are not uncommon. Patients who have already had one primary melanoma must be shown how to recognise a second primary at an early stage. In the Dutch series, 8 of the 22 (36%) melanoma patients had multiple primaries, while the frequency in the general melanoma population is quoted at between 2 % and 3 % .6 The Dutch study has obviously involved much painstaking clinical follow-up. Further study of these familes should provide the answers to several additional clinical points. There is much controversy about the age at which the majority of atypical or dysplastic naevi first develop; most normal naevi appear around the time of puberty,’ but it has been suggested that a large number of atypical naevi develop in early childhood. The Dutch kindred could help to settle this issue. Similarly, careful examination of children in the Dutch families could reveal whether the incidence of congenital naevi in family members who subsequently get melanoma is higher than the figure of 1 % quoted in the general population. Other outstanding questions concern the effect of exogenous hormones such as the contraceptive pill or pregnancy on future melanoma risk. Moreover, it is unclear whether the site of malignant melanoma in individuals with the syndrome is the same body site occupied by maximum numbers of naevi. In addition to the clinical studies suggested above, familial cancer presents an excellent opportunity for study of activated oncogenes in the aetiology of various types of malignancy; it is perhaps in this respect that the large group of patients in The Netherlands offers the most exciting prospect. Tissue from more than one primary malignant melanoma, from secondary tumour deposits in lymph nodes and other sites, and from benign melanocytic naevi may all be available from the one patient, along with normal tissue. Comparative analysis of these specimens should clearly establish the association of activated oncogene expression with primary tumours, secondary tumours, and possible precursor lesions by comparison with normal control tissue, all taken from the same source. Comparison of oncogene activation in several members of one family, and between affected families, could also help to establish whether observations on oncogene activation can be extended to all melanomas in these kindreds.
732-38. 3 National Institutes of Health. Precursors
to
malignant melanoma. JAMA 1984; 251:
864-66. 4. Bergman W, Palan A, Went LN. Clinical and genetic studies in six Dutch kindreds with the dysplasuc naevus syndrome. Ann Hum Genet 1986; 50: 249-58. 5. Lynch HT, Fusaro RM, Pester J, et al. Tumour spectrum in the FAMMM syndrome.
Br J Cancer 1981; 44: 553-60.
6. Greene MH, Fraumeni JF. The hereditary variant of malignant melanoma. In: Clark WH, ed. Human malignant melanoma. New York: Grune and Stratton, 1979. 7. Mackie RM, English J, Aitchison T, Fitzsimons CP, Wilson P. The number and distribution of melanocytic naevi in a British population. Br J Dermatol 1985; 113: 167-74.