BMJ

Sterilisation Of Mentally Retarded MinorsSource: The British Medical Journal, Vol. 281, No. 6247 (Oct. 18, 1980), pp. 1025-1026Published by: BMJStable URL: http://www.jstor.org/stable/25441731 .

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BRITISH MEDICAL JOURNAL VOLUME 281 18 OCTOBER 1980 1025

5 Hypertension Detection and Follow-up Program Cooperative Group.

Five-year findings of the hypertension detection and follow-up program. II. Mortality by race, sex and age. JAMA 1979;242:2572-7.

6 Management Committee. Initial results of the Australian Therapeutic

Trial in Mild Hypertension. Clinical Science 1979;57:449-52s. 7 Management Committee. The Australian Therapeutic Trial in Mild

Hypertension. Lancet 1980 ;i: 1261-7. 8

Breckenridge A, Dollery CT, Parry EHO. Prognosis of treated hyper tension. Changes in life expectancy and causes of death between 1952 and 1967. QJMed 1970;39:411-29.

9 Beevers DG, Fairman MJ, Hamilton M, Harpur JE. The influence of

antihypertensive treatment over the incidence of cerebral vascular disease. Postgrad Med J 1973;49:905-7.

10 Berglund G, Wilhelmsen L, Sannerstedt R, et al. Coronary heart-disease

after treatment of hypertension. Lancet 1978 ;i: 1-5. 11 Medical Research Council Working Party on Mild to Moderate Hyper

tension. Randomised controlled trial of treatment for mild hyper tension : design and pilot trial. Br MedJ 1977 ;i : 1437-40.

12 Barlow DH, Beevers DG, Hawthorne VM, Watt HD, Young GAR. Blood pressure measurement at screening and in general practice. Br

Heart J 1977;39:7-12. 13 Hart JT. Semicontinuous screening of a whole community for hyper

tension. Lancet 1970;ii:223-6. 14

Coope J. A screening clinic for hypertension in general practice. J R Coll Gen Pract 1974;24:161-6.

Uraemic pruritus

Pruritus is not a feature of acute renal failure but is common

in severe chronic renal failure, the reported1-3 incidence

being as high as 86%. Many factors have been incriminated. Patients commonly have a dry skin (xerosis), and this may contribute to the pruritus.2 The xerosis may be related to the

atrophy of the sebaceous glands4 and the eccrine sweat

glands that occurs in uraemia.5 The disturbances of calcium

and phosphorus metabolism in chronic renal failure have also been implicated,16~9 while other possible factors include the

proliferation of mast cells in the skin of some patients with

uraemia,10 the high serum concentrations of magnesium,11 and

an association with uraemic neuropathy.12 With so many

possibilities the only certainty is that the mechanism of uraemic pruritus remains unknown.

Regular and intensive haemodialysis is said to cure or

improve pruritus in many patients,1314 but some reports1 have

put the proportion relieved of their symptoms as low as 14%. Sometimes the pruritus may worsen or appear for the first time after starting maintenance haemodialysis. In some (but not all) patients with severe secondary hyperparathyroidism subtotal parathyroidectomy may dramatically improve or cure intractable pruritus.6-8 Not all patients with severe secondary

hyperparathyroidism have pruritus, however,6 and many who have intractable pruritus do not have severe secondary

hyperparathyroidism. Uraemic pruritus may be helped by simple measures such as skin emollients, systemic anti

histamine preparations, and minor tranquillisers.15 Very low

protein diets may help,16 and so may sauna baths.17 Recently a whole variety of treatments have been tried in patients being treated with maintenance haemodialysis with intractable

pruritus; but the best answer is renal transplantation with a

good functioning graft. Among the empirical treatments that have been commended

is regular intravenous heparin for several weeks; good results were claimed but these were uncontrolled observations.18 A

double-blind trial comparing intravenous lignocaine with

placebo saline during haemodialysis showed an improvement in the patients given the drug.3 Oral cholestyramine gave good results in one controlled trial19 but not in another20? and cholestyramine carries a possible risk of inducing or

aggravating a metabolic acidosis in uraemic patients.21 Ultra

violet phototherapy has also been tried22 23 and was effective in a controlled trial.22

Pruritus is a notoriously difficult symptom to assess, and in all these trials with lignocaine, cholestyramine, and ultra violet phototherapy the numbers were small; but these treatments may be worth trying in patients with intractable

itching. A final note of caution : patients with severe uraemia are not immune from other causes of pruritus, such as scabies.

1 Young AW, Sweeney EW, David DS, et al. Dermatologie evaluation of

pruritus in patients on hemodialysis. NY State J Med 1973;173: 2670-4.

2 Rosen T. Uremic pruritus: a review. Cutis 1979;23:790-2. 3 Tapia L, Cheigh JS, David DS, et al. Pruritus in dialysis patients treated

with parenteral lidocaine. N EnglJ Med 1977;296:261-2. 4 Rosenthal SR. Uremic dermatitis. Archives of Dermatology and Syphilology

1931;23:934-45. 5

Cawley EP, Hoch-Ligeti C, Bond GM. The eccrine sweat glands of

patients in uremia. Arch Dermatol 1961;84:889-97. 6 Hampers CL, Katz AI, Wilson RE, Merrill JP. Disappearance of

"uremic" itching after subtotal parathyroidectomy. N Engl J Med

1968;279:695-7. 7 Kleeman CR, Massry SG, Popovtzer MM, Makoff DL, Maxwell MH,

Coburn JW. The disappearance of intractable pruritus after para thyroidectomy in uremic patients with secondary hyperparathyroidism. Trans Assoc Am Physicians 1968;81:203-12.

8 Massry SG, Popovtzer MM, Coburn JW, Makoff DL, Maxwell MH,

Kleeman CR. Intractable pruritus as a manifestation of secondary hyperparathyroidism in uremia. Disappearance of itching after subtotal

parathyroidectomy. N EnglJ Med 1968;279:697-700. 9 Massry SG, Coburn JW, Hartenbower DL, et al. Calcium and magnesium

content in skin of patients with uremia and calc?mie disorders. Clinical Research 1971;19:188.

10 Neiman RS, Bischel MD, Lukes RJ. Uraemia and mast-cell proliferation. Lancet 1972;i:959.

11 Graf H, Kovarik J, Stummvoll HK, Wolf A. Disappearance of uraemic

pruritus after lowering dialysate magnesium concentration. Br Med J 1979 ;ii: 1478-9.

12 Raskin NH, Fishman RA. Neurologic disorders in renal failure (second of two parts). N EnglJ Med 1976;294:204-10.

13 Hampers CL, Schupak E, Lowrie EG, Lazarus JM. Long-term hemo

dialysis: the management of the patient with chronic renal failure. 2nd ed. New York: Grune and Stratton, 1973.

14 Curtis JR, Williams GB. Clinical management of chronic renal failure. London: Blackwell, 1975.

15 Tapia L. Pruritus on hemodialysis. Int J Dermatol 1979;18:217-8.

16 Boulton-Jones JM, Sissons JGP, Harrison ER. Itching in renal failure.

Lancet 1974;i:355. 17

Snyder D, Merrill JP. Sauna baths in the treatment of chronic renal failure. Trans Am Soc Art if Intern Organs 1966;12:188-92.

18 Yatzidis H, Digenis P, Tountas C. Heparin treatment of uremic itching. JAMA 1972;222:1183.

19 Silverberg DS, Iaina A, Reisin E, Rotzak R, Eliahou HE. Cholestyramine

in uraemic pruritus. Br MedJ 1977'?:1'52-3. 20 Van Leusen R, Lojenga JCK, Ruben ATh. Is cholestyramine helpful in

uraemic pruritus ? Br MedJ 1978 ;i:918-9. 21

Wrong OM. Cholestyramine in uraemic pruritus. Br MedJ 1977;i: 1662. 22 Gilchrest BA. Ultraviolet phototherapy of uremic pruritus. Int J Dermatol

1979;18:741-8. 23 Shultz BC, Roenigk HH. Uremic pruritus treated with ultraviolet light.

JAMA 1980;243:1836-7.

Sterilisation of mentally retarded minors

Severe mental handicap in a child is always a heavy burden for parents, but the circumstances are especially distressing

when a girl is approaching the reproductive years. The risk of

pregnancy is greater now than in the past, when more of these

girls were cared for in single-sex institutions. There is an

understandable concern to protect the youngster from

pregnancy, and the parents may well seek medical advice and

help. Some form of contraception may be offered, but at this

age and in these circumstances none is really satisfactory. The two obvious choices?an intrauterine device and an injectable

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1026 BRITISH MEDICAL JOURNAL VOLUME 281 18 OCTOBER 1980

steroid contraceptive?have clear limitations. Neither is well suited to the needs of these young, backward girls. There is

growing concern over the problem of pelvic infection resulting from the use of intrauterine contraception, particularly among,

young, nulligravid girls.1 2 Moreover, the risk of expulsion and

hence of pregnancy is significantly higher among nulligravidae in the youngest age groups.3

4 Again, these girls are ill equipped

to cope with the menstrual irregularities and cramp-like pains that may accompany the use of an intrauterine device. In

Britain the Committee on Safety of Medicines has not yet given general approval to the use of long-acting progestogen injections for contraceptive purposes, reports of abnormal

patterns of bleeding and of troublesome mood changes, breast

discomfort, and weight gain being causes of concern.5

Understandably, some parents raise the question of sterilisa

tion and their request is not unreasonable, though it does raise

profound moral and ethical issues to which there are no easy answers. This was frankly acknowledged by Dr David Owen as Secretary of State for Health and Social Security when the issue was discussed in the House of Commons in June 1975.6 In his statement to Parliament Dr Owen agreed that regret tably there were circumstances when a child under the age of 16 might best be sterilised. "What we have a duty to do," he

added, "is to ensure that those decisions, when they are

made, are made in a manner which will be acceptable to

public opinion as a whole." In a later statement in the House7

he referred to the case of a young Sheffield girl who had been made a ward of court by Mrs Justice Heilbron as a means of

blocking a proposal to sterilise her. In the light of this case, after conferring with its expert advisers the Department of

Health in October 1975 issued to all area and regional health authorities a discussion paper setting out proposals that it was

hoped would result in an agreed code of practice to be followed

by doctors faced with a request to sterilise a minor for non

therapeutic reasons. In effect, this document proposed that no

single doctor should assume responsibility for sterilising a

minor. All those able to provide information about the medical,

psychological, educational, and social problems of the child should be consulted as well as the parents, without whose consent the matter could not proceed. Where doubt or argu

ment arose the paper suggested that the case should be referred to a local, independent ethical committee with lay as well as

professional members. And there the matter rests. The

department has offered no firm guidance and no code of

practice has been agreed.

These cases must be managed in conformity with the

present law. Before any operation is undertaken informed consent is essential, and the most important aspect of any consent procedure is the duty to explain to the patient the nature and purpose of the proposed operation and to obtain

fully informed consent. Persons of unsound mind cannot

give a valid and fully informed consent, and if these young

girls are incapable of appreciating fully the nature and conse

quences of a sterilising procedure then it would be unlawful to subject them to the operation. Arranging for a committee to

make a decision about such an operation might look like an

attempt to shed some of the responsibility on to others, whereas the legal responsibility lies clearly with the surgeon who undertakes the operation. The law at present is not permissive in regard to the sterilisation of mentally retarded minors, and

any doctor approached by parents with a request for such an

operation would be well advised to seek advice from his defence society. Each case will differ in some respect from the

next, and all the circumstances would have to be weighed carefully before any surgeon took it on himself to challenge

the law in the way that Bourne did over therapeutic abortion over 40 years ago. But the law, representing society, is likely to take a more serious view of a sterilising operation, which is in

large measure an irreversible procedure. We cannot assess accurately how many families would like

such an operation carried out, for some parents, aware of the

present difficulties, will not approach their doctor. Were it to be established openly that medicine and the law are prepared to give a sympathetic and understanding hearing to their

problem more families might seek help. But there is no

possibility of sterilising even the most seriously affected children within the framework of the law as it stands?from

every viewpoint a most unsatisfactory state of affairs.

1 United States Department of Health, Education and Welfare, Food and

Drug Administration. Medical Device and Drug Advisory Committee on Obstetrics and Gynecology. Second report on intrauterine contra

ceptive devices. Washington, DC: US Government Printing Office,1978. 2 Guillebaud J. Pelvic inflammatory disease and IUCDs. British Journal of

Family Planning 1978;4:25, 29-30. 3 Bernard RR. IUD performance patterns : a 1970 world view, hit J Gynaecol

Obstet 1970;8:926-40. 4 Guillebaud J. The safety of intrauterine devices. Stud F am Plann 1979 ;10:

174-7. 5

Savage W. The use of Depo-Provira in East London. Fertility and

Contraception 1978;2:41-7. 6 House of Commons Official Report {Hansard) 1975 June 25;894:cols 633-8. 7 House of Commons Official Report (Hansard) 1975 July 21;896:col 248.

Arrhythmia in hypertrophie cardiomyopathy Patients who die from hypertrophie cardiomyopathy most often do so suddenly.1 Before the fourth decade of life symptoms tend to be mild and often respond to treatment with beta

adrenergic-blocking drugs; the patient usually becomes ill as

his haemodynamic condition deteriorates and he develops atrial fibrillation and heart failure.2 Patients who are in atrial fibrillation with grossly raised venous pressures and a low cardiac output might be expected to die suddenly, presumably from ventricular fibrillation. What is disturbing is that sudden

death strikes down not only patients with advanced disease but also younger, often asymptomatic patients. In one series of

220 patients with hypertrophie cardiomyopathy followed up for a mean of six years, 27 patients died suddenly : most were

younger than 40 years and were asymptomatic before death.3 Continuous electrocardiographic monitoring of patients

outside hospital has shown that asymptomatic arrhythmia is

surprisingly common in patients with hypertrophie cardiomyo pathy4: an investigation at the Royal Postgraduate Medical School showed that 50% of such patients had serious ven

tricular arrhythmias and half of these had ventricular tachy cardia. Similar results have been reported by other groups.5

Treatment with beta-adrenergic-blocking drugs (mean dose of

propranolol 280 mg/day) did not reduce the number of attacks of supraventricular

or ventricular arrhythmias.4 Recently the

calcium antagonist verapamil has been advocated as an

alternative treatment,6 7

but in short-term studies (two

months) it was not shown to reduce the number of episodes of arrhythmia in hypertrophie cardiomyopathy.8

The questions that need to be answered are whether

arrhythmia is the cause of sudden death, and if so whether

successful treatment of arrhythmia is possible.9 A prospective study using ambulatory electrocardiographic monitoring showed that patients found to have episodes of ventricular

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