early admission to hospital of mentally defective babies

1
267 them fell because he had lost that which the living religions of every age have given to their followers." Would people haunted by fear and insecurity in the present age not be likely to benefit more from the Christian faith than from the " cosmology " suggested by Dr. Manning ? Surely, the claims of Christian teaching should be eagerly appraised. But Dr. Manning does not give a moment’s consideration to religious faith. He says that our generation has thrown it over- board and prescribes for the world’s ills as if religion did not exist. If Dr. Manning and those who think like him have aban- doned the Christian view of 1,ife, I hope that before doing so they gave to its credentials the consideration they demand. However, if after doing so they fail to accept the Christian faith, they should remember that there are very many who are deeply convinced that it represents the truth. G. E. HEWSON. 1. Lancet, 1957, ii, 950. EARLY ADMISSION TO HOSPITAL OF MENTALLY DEFECTIVE BABIES CHARLES W. DUNN. SIR,-Miss Adams’ views 1 about the care of the mongol child are of interest; but we must remember that the life-span of a mongol can extend up to 72 years. Formerly, I thought that the mongol child’s best interests in early childhood were secured by keeping him at home. It is hard to realise that the infant mongol, with his healthy appearance and almost normal early development, will so deteriorate physically and mentally that at the age of 10 and afterwards he will have a mental age of 3-. The decision must be made on the exclusive fact that the infant mongol can never become an independent person, mentally or economically. The female mongol is better adapted to home care than the male, and exceptions may be made to the general rule of institutional care in her case. As long as the home is intact all is well, but inevitably the parents die. At this late date in life custodial care in some form must be provided, involving a difficult adjustment period to a mode of life which she is not adapted to but which must be endured henceforth. Her greatest task is coping with her memories, which in the mongol are maintained at a high level. The initial decision about the care of the mongol should not be influenced by emotional factors, theories, or personal pre- judices. The mongol infant should not be used to satisfy a temporary intense maternal instinct provoked by the birth of a child. My opinion is that the normal-minded mother can use her reason if she unfortunately gives birth to a mongol child. She should not permit the development of a selfish frustration. Her husband should not be censured because he arranges for his mentally deficient baby to be admitted to an institution which can give him, as he develops, a happiness, a state of wellbeing, and a life with opportunities and an environment which no amount of wealth can give. One of the major advantages which the mongol has in an institution is that he is living in an environment of equality of abilities; he has physical freedom and competitive opportunities in sports where handicaps are equalised, and is not frustrated by exhibitions of physical dexterity and mental normality. Individual and small-team competitive spirit is at a high level of enthusiasm. In a good institution he will not lose his family membership, because a competent social-service worker maintains and promotes family membership. This type of function by a social-service system is in direct contrast to the role which Miss Adams plays in the care of the mongol. I do not feel she is contributing anything constructive by increasing the frustration of the mother when she criticises the obstetrician and paediatrician and condemns the husband for acting on the wisdom and long-term outlook of his competent advisers. For over fifteen years I have treated in an endocrine clinic young mongol children from a hospital for the mentally retarded-but not on the premise that a hor- monal deficiency is a primary actiological factor. Thyroid is administered in the presence of clinical hypothyroidism; I find the mongol child more prone than the normal to the hypothyroid state. I gave stilboestrol to the males with sexual disturbances, and in a few patients who were strong-willed and would flee from protective observation, I insisted on protective institutional care. Later I began early stilboestrol therapy to inhibit gonad development. This prevented trouble when the patient found that the heavy dose of stilboestrol required to suppress erections also produced breast development and deep pigmentation of the areola. When I assumed my duties as consulting endocrinologist at the hospital for the mentally retarded in late 1955, I had my first opportunity of observing and studying 50 mongols whose ages ranged from infancy to 72 years, and some 25 patients who were receiving endocrine therapy at home. I had little doubt that the survey of 75 mongols showed that the endocrine- treated mongol from the institution was the happiest and best- established individual, because socially, mentally, physically, and occupationally he was the most advanced and accomplished; this anolied to both sexes. 1. Cecil, R. L., Loeb, R. F. Textbook of Medicine. Philadelphia, 1955. 2. Holt, L. E., McIntosh, R. Pediatrics. New York, 1953. 3. Kalckar, H. M., Anderson, E. P., Isselbacher, K. J. Biochim. biophys. Acta, 1956, 20, 262. 4. Anderson, E. P., Kalckar, H. M., Kurahashi, K., Isselbacher, K. J. J. Lab. clin. Med. 1957, 50, 469. 5. Maxwell, E. S., Kalckar, H. M., Bynum, E. ibid. p. 478. 6. Schwarz, V., Holyel, A., Komrower, G. M. Lancet, Jan. 4, 1958, p. 24. CONGENITAL GALACTOSÆMIA AND MENTAL HEALTH SIR,-Current textbooks of medicine and pxdiatrics discuss congenital galactosxmia only briefly. 1 2 The incidence of congenital galactosasmia has not been estab- lished for lack of simple screening methods, but there is reason to suspect that the disease is not as rare as hitherto assumed. Awareness of the disease is essential for early diagnosis and treatment so that serious consequences may be prevented. Two illustrative cases are here reported. A white boy, aged 13 weeks, was admitted to the Children’s Hospital of Los Angeles with a diagnosis of galactosaemia made in another hospital by history and by the presence of reducing substance and albumin in the urine. At the age of 14 days the formula had been changed to one containing no milk. Subse- quently, the infant ate well and gained steadily. The original diagnosis was confirmed by a galactose-tolerance test and by assay of red blood-cells for PGal uridyl transferase by newly developed specific lIBethods.4-6 An older brother had been in an institution for feeble- minded children for several years. His i.Q. at 12 years of age was estimated at 11, and he could neither talk nor understand words addressed to him. Galactossemia had never been sus- pected, though he had been examined at several clinics. His feeble-mindedness had been ascribed to erythroblastosis. It was known that he had bilateral cataracts. Blood was made available for study through the cooperation of the staff of the institution (Dixon State School, Dixon, 111.), and we are especi- ally grateful to Dr. Abraham Gelperin. It revealed a striking defect of PGal uridyl transferase. The galactose-tolerance curve was also abnormal. It seems highly probable that similar cases of unsus- pected galactosaemia are present in mental institutions. Dr. Robert Cooke, of Johns Hopkins University, has found three cases of galactosaemia in training-camps for retarded children (personal communication). To clarify the incidence of the disease and to detect unknown cases for possible therapeutic benefit to them and certainly to their newborn siblings, large-scale screening seems advisable in institutions for mentally retarded children. The clinical

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267

them fell because he had lost that which the living religions of everyage have given to their followers."Would people haunted by fear and insecurity in the present

age not be likely to benefit more from the Christian faith thanfrom the " cosmology

" suggested by Dr. Manning ? Surely,the claims of Christian teaching should be eagerly appraised.But Dr. Manning does not give a moment’s consideration toreligious faith. He says that our generation has thrown it over-board and prescribes for the world’s ills as if religion did notexist.

If Dr. Manning and those who think like him have aban-doned the Christian view of 1,ife, I hope that before doing sothey gave to its credentials the consideration they demand.However, if after doing so they fail to accept the Christian faith,they should remember that there are very many who are deeplyconvinced that it represents the truth.

G. E. HEWSON.

1. Lancet, 1957, ii, 950.

EARLY ADMISSION TO HOSPITAL OF MENTALLYDEFECTIVE BABIES

CHARLES W. DUNN.

SIR,-Miss Adams’ views 1 about the care of the

mongol child are of interest; but we must remember thatthe life-span of a mongol can extend up to 72 years.Formerly, I thought that the mongol child’s best interests

in early childhood were secured by keeping him at home. Itis hard to realise that the infant mongol, with his healthyappearance and almost normal early development, will so

deteriorate physically and mentally that at the age of 10 andafterwards he will have a mental age of 3-. The decisionmust be made on the exclusive fact that the infant mongolcan never become an independent person, mentally or

economically. The female mongol is better adapted to homecare than the male, and exceptions may be made to the

general rule of institutional care in her case. As long as thehome is intact all is well, but inevitably the parents die. Atthis late date in life custodial care in some form must be

provided, involving a difficult adjustment period to a mode oflife which she is not adapted to but which must be enduredhenceforth. Her greatest task is coping with her memories,which in the mongol are maintained at a high level.The initial decision about the care of the mongol should not

be influenced by emotional factors, theories, or personal pre-judices. The mongol infant should not be used to satisfy atemporary intense maternal instinct provoked by the birth ofa child. My opinion is that the normal-minded mother canuse her reason if she unfortunately gives birth to a mongolchild. She should not permit the development of a selfishfrustration. Her husband should not be censured because hearranges for his mentally deficient baby to be admitted to aninstitution which can give him, as he develops, a happiness, astate of wellbeing, and a life with opportunities and an

environment which no amount of wealth can give.One of the major advantages which the mongol has in an

institution is that he is living in an environment of equality ofabilities; he has physical freedom and competitive opportunitiesin sports where handicaps are equalised, and is not frustratedby exhibitions of physical dexterity and mental normality.Individual and small-team competitive spirit is at a high levelof enthusiasm. In a good institution he will not lose his familymembership, because a competent social-service workermaintains and promotes family membership. This type offunction by a social-service system is in direct contrast to therole which Miss Adams plays in the care of the mongol. I donot feel she is contributing anything constructive by increasingthe frustration of the mother when she criticises the obstetricianand paediatrician and condemns the husband for acting on thewisdom and long-term outlook of his competent advisers.For over fifteen years I have treated in an endocrine

clinic young mongol children from a hospital for thementally retarded-but not on the premise that a hor-monal deficiency is a primary actiological factor. Thyroid

is administered in the presence of clinical hypothyroidism;I find the mongol child more prone than the normal tothe hypothyroid state.

I gave stilboestrol to the males with sexual disturbances,and in a few patients who were strong-willed and wouldflee from protective observation, I insisted on protectiveinstitutional care.

Later I began early stilboestrol therapy to inhibit gonaddevelopment. This prevented trouble when the patient foundthat the heavy dose of stilboestrol required to suppress erectionsalso produced breast development and deep pigmentation ofthe areola.When I assumed my duties as consulting endocrinologist at

the hospital for the mentally retarded in late 1955, I had myfirst opportunity of observing and studying 50 mongols whoseages ranged from infancy to 72 years, and some 25 patientswho were receiving endocrine therapy at home. I had littledoubt that the survey of 75 mongols showed that the endocrine-treated mongol from the institution was the happiest and best-established individual, because socially, mentally, physically,and occupationally he was the most advanced and accomplished;this anolied to both sexes.

1. Cecil, R. L., Loeb, R. F. Textbook of Medicine. Philadelphia, 1955.2. Holt, L. E., McIntosh, R. Pediatrics. New York, 1953.3. Kalckar, H. M., Anderson, E. P., Isselbacher, K. J. Biochim. biophys.

Acta, 1956, 20, 262.4. Anderson, E. P., Kalckar, H. M., Kurahashi, K., Isselbacher, K. J.

J. Lab. clin. Med. 1957, 50, 469.5. Maxwell, E. S., Kalckar, H. M., Bynum, E. ibid. p. 478.6. Schwarz, V., Holyel, A., Komrower, G. M. Lancet, Jan. 4, 1958, p. 24.

CONGENITAL GALACTOSÆMIA AND MENTAL

HEALTH

SIR,-Current textbooks of medicine and pxdiatricsdiscuss congenital galactosxmia only briefly. 1 2 Theincidence of congenital galactosasmia has not been estab-lished for lack of simple screening methods, but there isreason to suspect that the disease is not as rare as hithertoassumed. Awareness of the disease is essential for earlydiagnosis and treatment so that serious consequences maybe prevented. Two illustrative cases are here reported.A white boy, aged 13 weeks, was admitted to the Children’s

Hospital of Los Angeles with a diagnosis of galactosaemia madein another hospital by history and by the presence of reducingsubstance and albumin in the urine. At the age of 14 days theformula had been changed to one containing no milk. Subse-

quently, the infant ate well and gained steadily. The originaldiagnosis was confirmed by a galactose-tolerance test and byassay of red blood-cells for PGal uridyl transferase by newlydeveloped specific lIBethods.4-6An older brother had been in an institution for feeble-

minded children for several years. His i.Q. at 12 years of agewas estimated at 11, and he could neither talk nor understandwords addressed to him. Galactossemia had never been sus-

pected, though he had been examined at several clinics. Hisfeeble-mindedness had been ascribed to erythroblastosis. Itwas known that he had bilateral cataracts. Blood was madeavailable for study through the cooperation of the staff of theinstitution (Dixon State School, Dixon, 111.), and we are especi-ally grateful to Dr. Abraham Gelperin. It revealed a strikingdefect of PGal uridyl transferase. The galactose-tolerancecurve was also abnormal.

It seems highly probable that similar cases of unsus-pected galactosaemia are present in mental institutions.Dr. Robert Cooke, of Johns Hopkins University, hasfound three cases of galactosaemia in training-camps forretarded children (personal communication).To clarify the incidence of the disease and to detect unknown

cases for possible therapeutic benefit to them and certainly totheir newborn siblings, large-scale screening seems advisablein institutions for mentally retarded children. The clinical