281
allowed to develop if there is impairment of both cellular and humoral immune responses to hepatitis-B virus. Our finding indicates that this patient was infected twice with hepatitis-B virus. Patients who have severe reactions to y-globulin therapy and therefore require infusions of fresh frozen plasma may be at particular risk of becoming HBAg carriers. J. W. M. LAWTON South-East Scotland R. HOPKINS Regional Blood Transfusion Service, A. B. KAY Royal Infirmary, P. C. DAS. Edinburgh EH3 9HB. Respiratory Unit, Northern General Hospital,
Edinburgh.
METIAMIDE
I. C. PATERSON I. W. B. GRANT.
IN ZOLLINGER-ELLISON SYNDROME
SiR,—The report by Mr Thompson and his colleagues (Jan. 4, p. 35) on the use of metiamide in the therapy of a
marginal ulcer in a post-gastrectomy patient with proven Zollinger-Ellison syndrome prompts us to report our experience. We have also used metiamide, a histamine-H2 antagonist, to treat a patient with Zollinger-Ellison syndrome. Our indication for its use was a severe, progressive, systemic metabolic alkalosis which was refractory to intravenous therapy and greatly increased the hazard of major surgery. We would seriously question the rationale of chronic metiamide therapy in a patient with ZollingerEllison syndrome-a relentless, progressive disorderwhen surgery has produced such excellent results in experienced hands. The rationale for the use of metiamide in the case described below seems clearer, and the therapeutic result was remarkable.
patient, a 43-year-old Black man, was admitted to the College of Virginia Hospitals in June, 1974, with a perforated, gastrojejunocolic fistula which followed truncal vagotomy, antrectomy, and gastrojejunostomy for chronic The
Medical
duodenal ulceration.
The transverse- colon
neogastrojejunostomy was constructed. course was complicated by the appearance
was
resected and
a
His
postoperative of a high-output (4-6
litres per 24 hours) enterocutaneous fistula at the anastomosis. Serum-gastrin levels of 900-1100 pg. per 100 ml. confirmed the
diagnosis of Zollinger-Ellison syndrome. Despite massive conventional intravenous therapy, the patient became markedly alkalotic. Serum sodium was 134, chloride 74, potassium 4,2, and carbon dioxide 41 meq. per litre. The arterial blood pH was 7-56 and urea nitrogen 126 mg. per 100 ml. Urgent total gastrectomy was contemplated to stop the massive hydrogen-ion leak. However, the Food and Drug Administration approved the emergency use of metiamide in the hope of pharmacologically plugging this leak. Intravenous metiamide was begun at a dose of 20 mg. per kg. per 24,hours without any markedly apparent change in fistula output, although the patient’s blood chemistry immediately improved. After 12 hours, the dose was increased to 125 mg. per hour, with a decrease in fistula output and increase in fistula pH. After 36 hours of therapy serum sodium was 131, chloride 105, potassium 4-9, and carbon dioxide 20 meq. per litre. The arterial blood pH was 7.40 and the urea nitrogen 18 mg. per 100 ml. During metiamide administration, gastric hydrogenion-secretory rates as low as 0-05 meq. per 15 minutes were measured. After his severe acid-base disorder had been corrected, successful total gastrectomy was performed. Thus in our patient metiamide successfully corrected a
serious hypochlorsemic metabolic alkalosis secondary to gastric hydrogen-ion loss by virtually abolishing gastricacid secretion in a case of Zollinger-Ellison syndrome complicated by a high-output enterocutaneous fistula.
Smith, Kline and French Laboratories, the metiamide used in this patient. Department of Surgery, Medical College of Virginia,
Richmond, Virginia 23298, U.S.A.
Philadelphia, provided
L. GREGG HALLORAN MICHAEL SWANK B. W. HAYNES.
PHÆOCHROMOCYTOMA
AND MYOCARDIAL INFARCTION
SIR,-In your editorial on coronary bypass surgery1 you said: " We do not know the natural history of ischaemic disease with pharmacologically demonstrable long-term pblockade. It has been suggested that clofibrate may lessen mortality, possibly an anti-arrhythmic action. &bgr;-blockade, with well-known anti-arrhythmic action, might therefore have a more powerful beneficial influence-not necessarily dependent on the degree to which angina is controlled." Dr Wilhelmsson and others (Nov. 16, p. 1157), reporting beneficial effects of such therapy, discuss the possibility that " chronic P-blockade might prevent myocardial infarction ..." They also refer to earlier workers who did not find similar results. Inevitably the controversy is going to grow, with some workers finding results in favour and others failing to confirm benefits. To settle the issue on basic pharmacological reasoning, I asked myself the question-if p-blockade is going to prevent acute myocardial infarction, it should be possible to show that excessive p-receptor stimulation (as in phaeochromocytoma) results in increased incidence of myocardial infarction. I was frankly sceptical about finding anything to support the idea, but I have been surprised at the number of myocardial infarctions in case-reports of phaeochromocytoma either during the course of illness, or immediately after operative removal (it is well known that massive amounts of adrenaline [epinephrine] and noradrenaline [norepinephrine] may be released into the circulation during operative handling of phxochromocytoma). Particularly impressive are reports of cases where persistent hypertension was not a feature of the illness, and thus by inference these patients would secrete adrenaline and/or norSince the occurrence of adrenaline only at intervals. infarctions in painless myocardial phaeochromocytoma is well documented 2,3 the actual number of such incidents may be considerably greater than is now realised. We are now doing a survey of the incidence of myocardial infarction in phaeochromocytoma in this country, which, with 3 such cases observed at this hospital, will form a detailed report later. I wish to cite a few (of many) relevant case-reports collected from the world literature. Case J.*—A 55-year-old male business executive admitted for carotid-sinus denervation. Hypertension never observed before admission to hospital. Blood-pressure (B.P.) on admission was 130/80 mm. Hg. During left carotid-sinus denervation, B.P. rose suddenly and fluctuated widely. Patient developed acute pulmonary oedema, and later hemiparesis. He died 36 hours after the operation. Necropsy showed an unsuspected phseochrcmocytoma of the right adrenal, and also an old myocardial infarct. (Eight years earlier he was in hospital twice in the same year for " heart-attacks " but without hypertension and without E.c.G. confirmation of myocardial infarction.) Case 2.,-A 53-year-old male White physician had a right suprarenal phseochromocytoma removed in 1959. He had episodic symptoms since the age of 28. He had an acute anterior myocardial infarction at the age of 37, several hours after an episode of palpitation, pallor, and sweating. In 1953 his B.P. was first determined during an attack, and it was 200-210/100 settling to 130/85 five minutes later. He was not hypertensive before operation, and had remained normotensive since. Case 3.,-A 49-year-old female was operated on in 1958 when a She left adrenal benign pha:ochromocytoma was removed. collapsed and died 30 hours later. Necropsy showed old healed myocardial infarct, mild coronary atherosclerosis, fresh cerebral infarct, and shock nephrosis. She had a six-year history of episodes of generalised body heat and sweating which were 1.
Lancet, 1973, 1, 137. Boldt, M. H., Flexner, M., Ortner, A. B. Ann. intern. Med. 1957, 46, 1165. 3. Mattman, P. E. Am. J. Cardiol. 1961, 8, 426. 4. Cushman, P. Am. J. Med. 1962, 32, 352. 5. Bell, M. A., Blakemore, W. S., Rose, E. Ann. intern. Med. 1962, 57, 406.
2.
282 unrelated to effort or emotion. For three years before operation she was treated for moderate hypertension. Case 4.6-A 40-year-old woman admitted to hospital with ten " years’ history of migraine ". B.P. noted to be high only once; at several other observations B.P. was normal. On the day of hospital admission she complained of substernal pressure and nausea. Shock, B.P. 75; 60, and pulmonary redema developed. E.c.G. showed anteroseptal myocardial infarction. At necropsy, the coronary arteries were patent, no atherosclerotic changes. She also had right adrenal phaeochromocytoma. Case —A 22-year-old man was seen because of excessive sweating episodes. He had spent four years in the U.S. Army. B.P. was normal on admission to the Army. Two years earlier he was admitted to hospital with " headache ", and B.P. then was 180/150. On Feb. 24, 1948, he was operated on and a right adrenal phaeochromocytoma was removed. Two hours postoperatively he had an acute posterior myocardial infarction. Case 6.8-A 22-year-old man had a fatal acute myocardial infarction. He was known to have had headaches for the previous two years. B.p. then was 160-240/120-160 and cardiomegaly was also found. Necropsy showed a recent posterior myocardial infarction and a left adrenal phxochromocytoma.
The early results of our survey point in the same direction as these case-reports. The fact that the frequency of acute myocardial infarction is sizeable in patients with phseochromocytoma who do not have persistent hypertension seems to support the hypothesis that excessive sudden stimulation of p-receptors in the heart could lead to myocardial infarction in man. The results of trials on larger numbers of patients and longer follow-up along lines similar to those of Wilhelmsson et al., if confirmatory of their findings, may really prove to be the turning-point in our thinking about the possible preventive use of drugs in patients who have had a myocardial infarction, and, maybe later, in those patients thought to be prone to it-e.g., those with hypertension, diabetes, or a strong family history. University Department of Medicine, Martin Wing, General Infirmary, Leeds LS1 3EX.
K. K. GUPTA.
A TIME TO BE BORN
SiR,—Ihave been prompted by your leader on induction of labour (Nov. 16, p. 1183) to relate my own experience of two very different births. My first child was born naturally in 1967 while I was suffering from toxaemia. The first stage of labour lasted 15 hours, the second stage 1 hour, and the third stage 10 minutes. During the actual birth I required no anaesthetics and my husband was present; it was a very rewarding experience. My second child was born in October last year. I again had the same symptoms during pregnancy, and was admitted to hospital to be induced. I approached the birth in a state of happy expectancy and confidence, since the procedure had been explained to me. However, I received a severe shock. The membranes were ruptured, causing extreme pain and great mental distress. Then the oxytocin drip was set up, and about 10 minutes later contractions started, roughly 40 minutes apart at first. Later they were coming every 2 minutes and were very painful; at no time ride it ". Injections had could I control the pain and no effect, and I was thankful to be given gas-and-air, but the pain was so great and so regular that I could not use it without assistance. Finally, I was roused and told to push. After the third push I was told to stop, and the baby was delivered by forceps. My immediate reaction was relief that it was over, instead of a feeling of achievement. "
6. 7.
8.
Pelkonen, R., Pitkanen, E. Acta med. scand. 1963, 173, 41. Wilkins, R. W., Greer, W. E. R., Culbertson, J. W., Halperin, M. H., Litter, J., Burnett, C. H., Smithwick, R. H. Archs intern. Med. 1952, 86. 51. Priest, W. M. Br. med. J. 1952, ii 860.
a great deal of pain, and required 3 weeks to recover, compared with 11 days on the previous occasion. The whole birth was a very distressing experience and one I will not repeat. I realise that, in my case, the life of my baby and myself could not be risked by allowing the pregnancy to continue, but if I, understanding this and having confidence in my doctors, could experience such distress and pain, what must the effect be on women who are being induced to suit the convenience of the hospital authorities ? I appeal to all hospital staff to remember that women in labour. are human beings, and to allow them to have their babies with pleasure and dignity.
Afterwards, I had
6 Parc Hen
Bias, Llanfairfechan, Gwynedd LL33 0RW, North Wales.
LESLEY J. STRAIN.
EMERGENCY DOMICILIARY TREATMENT FOR HÆMOPHILIA SIR,-Le Quesne et al.1 reported a home treatment programme for haemophiliacs in which close relatives of patients learnt to prepare and administer plasma fractions. We would like to report the findings of a small pilot study in which a State-registered nurse, trained specifically in giving plasma fractions to haemophiliacs, provided a limited domiciliary service. There are several advantages of this modification to the usual home-treatment programmes; documentation is easier to control, samples for assays and screening for HBAg can be carried out more easily, and venepuncture tends to be of,a higher standard. It is also possible in such a scheme to include patients whose relatives would not be suitable for a routine home-treatment programme of the type described by Le Quesne et al. A disadvantage is that a doctor is not involved, unless the nurse feels that the nature or extent of the bleeding episode warrants further medical opinion; the director of the haemophilia centre has, of course, to agree to be responsible for the nurse. The pilot study ran for six months (July, 1974, to December, 1974). 6 severe haemophiliacs were selected for the study,
ranging from 10 to 33 years. All had a factor vm of less than 1%. 4 took part in the study, and a total of 27 emergency calls were made. At first, patients were diffident about using the service but towards the end of the study more confidence was gained by patients and their relatives; eventually up to 4 ’
calls a week were carried out. The system involved the use of an’Air-Call’ radio system switchboard. Patients would call this number, the nurse would be located, and the visit made within an hour. We felt that the services of a routine hospital switchboard might just as easily have been used; it so happened that one of the patient’s parents had a radio-telephone system at his disposal.’The nurse was on call from 12 noon-11.00 P.M. on Saturdays, 9.00 A.M.-l1.00 P.M. on Sundays and 6.00 p.M.-ll.OO P.M. on weekdays, and was paid the routine rates for on-call services. Only spontaneous and minor bleeding episodes were treated, with either 6 packs of cryoprecipitate cr 500 units of freeze-dried factor vin concentrate (’Hemofil’) as a standard treatment regime. Possible bleeding episodes into chest, abdomen, or skull, and all traumatic bleeding episodes were excluded from the domiciliary service, and treated at the hospital centre.
The
of
"
"
nurse-practitioners in the treatment of haemophilia may be an improvement on some of the existing schemes. Professor Ingram (St. Thomas’s Hospital Haemophilia Centre) has employed a sister for treating haemophilia during working hours. We would be interested use
hear of any other centres using similar schemes for the " after emergency treatment of haemophilia, particularly
to
hours ". Quesne, B., Britten, M. I., Maragaki, C., Dormandy, K. M. Lancet, 1974, ii, 507.
1. Le
allowed to develop if there is impairment of both cellular and humoral immune responses to hepatitis-B virus. Our finding indicates that this patient was infected twice with hepatitis-B virus. Patients who have severe reactions to y-globulin therapy and therefore require infusions of fresh frozen plasma may be at particular risk of becoming HBAg carriers. J. W. M. LAWTON South-East Scotland R. HOPKINS Regional Blood Transfusion Service, A. B. KAY Royal Infirmary, P. C. DAS. Edinburgh EH3 9HB. Respiratory Unit, Northern General Hospital,
Edinburgh.
METIAMIDE
I. C. PATERSON I. W. B. GRANT.
IN ZOLLINGER-ELLISON SYNDROME
SiR,—The report by Mr Thompson and his colleagues (Jan. 4, p. 35) on the use of metiamide in the therapy of a
marginal ulcer in a post-gastrectomy patient with proven Zollinger-Ellison syndrome prompts us to report our experience. We have also used metiamide, a histamine-H2 antagonist, to treat a patient with Zollinger-Ellison syndrome. Our indication for its use was a severe, progressive, systemic metabolic alkalosis which was refractory to intravenous therapy and greatly increased the hazard of major surgery. We would seriously question the rationale of chronic metiamide therapy in a patient with ZollingerEllison syndrome-a relentless, progressive disorderwhen surgery has produced such excellent results in experienced hands. The rationale for the use of metiamide in the case described below seems clearer, and the therapeutic result was remarkable.
patient, a 43-year-old Black man, was admitted to the College of Virginia Hospitals in June, 1974, with a perforated, gastrojejunocolic fistula which followed truncal vagotomy, antrectomy, and gastrojejunostomy for chronic The
Medical
duodenal ulceration.
The transverse- colon
neogastrojejunostomy was constructed. course was complicated by the appearance
was
resected and
a
His
postoperative of a high-output (4-6
litres per 24 hours) enterocutaneous fistula at the anastomosis. Serum-gastrin levels of 900-1100 pg. per 100 ml. confirmed the
diagnosis of Zollinger-Ellison syndrome. Despite massive conventional intravenous therapy, the patient became markedly alkalotic. Serum sodium was 134, chloride 74, potassium 4,2, and carbon dioxide 41 meq. per litre. The arterial blood pH was 7-56 and urea nitrogen 126 mg. per 100 ml. Urgent total gastrectomy was contemplated to stop the massive hydrogen-ion leak. However, the Food and Drug Administration approved the emergency use of metiamide in the hope of pharmacologically plugging this leak. Intravenous metiamide was begun at a dose of 20 mg. per kg. per 24,hours without any markedly apparent change in fistula output, although the patient’s blood chemistry immediately improved. After 12 hours, the dose was increased to 125 mg. per hour, with a decrease in fistula output and increase in fistula pH. After 36 hours of therapy serum sodium was 131, chloride 105, potassium 4-9, and carbon dioxide 20 meq. per litre. The arterial blood pH was 7.40 and the urea nitrogen 18 mg. per 100 ml. During metiamide administration, gastric hydrogenion-secretory rates as low as 0-05 meq. per 15 minutes were measured. After his severe acid-base disorder had been corrected, successful total gastrectomy was performed. Thus in our patient metiamide successfully corrected a
serious hypochlorsemic metabolic alkalosis secondary to gastric hydrogen-ion loss by virtually abolishing gastricacid secretion in a case of Zollinger-Ellison syndrome complicated by a high-output enterocutaneous fistula.
Smith, Kline and French Laboratories, the metiamide used in this patient. Department of Surgery, Medical College of Virginia,
Richmond, Virginia 23298, U.S.A.
Philadelphia, provided
L. GREGG HALLORAN MICHAEL SWANK B. W. HAYNES.
PHÆOCHROMOCYTOMA
AND MYOCARDIAL INFARCTION
SIR,-In your editorial on coronary bypass surgery1 you said: " We do not know the natural history of ischaemic disease with pharmacologically demonstrable long-term pblockade. It has been suggested that clofibrate may lessen mortality, possibly an anti-arrhythmic action. &bgr;-blockade, with well-known anti-arrhythmic action, might therefore have a more powerful beneficial influence-not necessarily dependent on the degree to which angina is controlled." Dr Wilhelmsson and others (Nov. 16, p. 1157), reporting beneficial effects of such therapy, discuss the possibility that " chronic P-blockade might prevent myocardial infarction ..." They also refer to earlier workers who did not find similar results. Inevitably the controversy is going to grow, with some workers finding results in favour and others failing to confirm benefits. To settle the issue on basic pharmacological reasoning, I asked myself the question-if p-blockade is going to prevent acute myocardial infarction, it should be possible to show that excessive p-receptor stimulation (as in phaeochromocytoma) results in increased incidence of myocardial infarction. I was frankly sceptical about finding anything to support the idea, but I have been surprised at the number of myocardial infarctions in case-reports of phaeochromocytoma either during the course of illness, or immediately after operative removal (it is well known that massive amounts of adrenaline [epinephrine] and noradrenaline [norepinephrine] may be released into the circulation during operative handling of phxochromocytoma). Particularly impressive are reports of cases where persistent hypertension was not a feature of the illness, and thus by inference these patients would secrete adrenaline and/or norSince the occurrence of adrenaline only at intervals. infarctions in painless myocardial phaeochromocytoma is well documented 2,3 the actual number of such incidents may be considerably greater than is now realised. We are now doing a survey of the incidence of myocardial infarction in phaeochromocytoma in this country, which, with 3 such cases observed at this hospital, will form a detailed report later. I wish to cite a few (of many) relevant case-reports collected from the world literature. Case J.*—A 55-year-old male business executive admitted for carotid-sinus denervation. Hypertension never observed before admission to hospital. Blood-pressure (B.P.) on admission was 130/80 mm. Hg. During left carotid-sinus denervation, B.P. rose suddenly and fluctuated widely. Patient developed acute pulmonary oedema, and later hemiparesis. He died 36 hours after the operation. Necropsy showed an unsuspected phseochrcmocytoma of the right adrenal, and also an old myocardial infarct. (Eight years earlier he was in hospital twice in the same year for " heart-attacks " but without hypertension and without E.c.G. confirmation of myocardial infarction.) Case 2.,-A 53-year-old male White physician had a right suprarenal phseochromocytoma removed in 1959. He had episodic symptoms since the age of 28. He had an acute anterior myocardial infarction at the age of 37, several hours after an episode of palpitation, pallor, and sweating. In 1953 his B.P. was first determined during an attack, and it was 200-210/100 settling to 130/85 five minutes later. He was not hypertensive before operation, and had remained normotensive since. Case 3.,-A 49-year-old female was operated on in 1958 when a She left adrenal benign pha:ochromocytoma was removed. collapsed and died 30 hours later. Necropsy showed old healed myocardial infarct, mild coronary atherosclerosis, fresh cerebral infarct, and shock nephrosis. She had a six-year history of episodes of generalised body heat and sweating which were 1.
Lancet, 1973, 1, 137. Boldt, M. H., Flexner, M., Ortner, A. B. Ann. intern. Med. 1957, 46, 1165. 3. Mattman, P. E. Am. J. Cardiol. 1961, 8, 426. 4. Cushman, P. Am. J. Med. 1962, 32, 352. 5. Bell, M. A., Blakemore, W. S., Rose, E. Ann. intern. Med. 1962, 57, 406.
2.
282 unrelated to effort or emotion. For three years before operation she was treated for moderate hypertension. Case 4.6-A 40-year-old woman admitted to hospital with ten " years’ history of migraine ". B.P. noted to be high only once; at several other observations B.P. was normal. On the day of hospital admission she complained of substernal pressure and nausea. Shock, B.P. 75; 60, and pulmonary redema developed. E.c.G. showed anteroseptal myocardial infarction. At necropsy, the coronary arteries were patent, no atherosclerotic changes. She also had right adrenal phaeochromocytoma. Case —A 22-year-old man was seen because of excessive sweating episodes. He had spent four years in the U.S. Army. B.P. was normal on admission to the Army. Two years earlier he was admitted to hospital with " headache ", and B.P. then was 180/150. On Feb. 24, 1948, he was operated on and a right adrenal phaeochromocytoma was removed. Two hours postoperatively he had an acute posterior myocardial infarction. Case 6.8-A 22-year-old man had a fatal acute myocardial infarction. He was known to have had headaches for the previous two years. B.p. then was 160-240/120-160 and cardiomegaly was also found. Necropsy showed a recent posterior myocardial infarction and a left adrenal phxochromocytoma.
The early results of our survey point in the same direction as these case-reports. The fact that the frequency of acute myocardial infarction is sizeable in patients with phseochromocytoma who do not have persistent hypertension seems to support the hypothesis that excessive sudden stimulation of p-receptors in the heart could lead to myocardial infarction in man. The results of trials on larger numbers of patients and longer follow-up along lines similar to those of Wilhelmsson et al., if confirmatory of their findings, may really prove to be the turning-point in our thinking about the possible preventive use of drugs in patients who have had a myocardial infarction, and, maybe later, in those patients thought to be prone to it-e.g., those with hypertension, diabetes, or a strong family history. University Department of Medicine, Martin Wing, General Infirmary, Leeds LS1 3EX.
K. K. GUPTA.
A TIME TO BE BORN
SiR,—Ihave been prompted by your leader on induction of labour (Nov. 16, p. 1183) to relate my own experience of two very different births. My first child was born naturally in 1967 while I was suffering from toxaemia. The first stage of labour lasted 15 hours, the second stage 1 hour, and the third stage 10 minutes. During the actual birth I required no anaesthetics and my husband was present; it was a very rewarding experience. My second child was born in October last year. I again had the same symptoms during pregnancy, and was admitted to hospital to be induced. I approached the birth in a state of happy expectancy and confidence, since the procedure had been explained to me. However, I received a severe shock. The membranes were ruptured, causing extreme pain and great mental distress. Then the oxytocin drip was set up, and about 10 minutes later contractions started, roughly 40 minutes apart at first. Later they were coming every 2 minutes and were very painful; at no time ride it ". Injections had could I control the pain and no effect, and I was thankful to be given gas-and-air, but the pain was so great and so regular that I could not use it without assistance. Finally, I was roused and told to push. After the third push I was told to stop, and the baby was delivered by forceps. My immediate reaction was relief that it was over, instead of a feeling of achievement. "
6. 7.
8.
Pelkonen, R., Pitkanen, E. Acta med. scand. 1963, 173, 41. Wilkins, R. W., Greer, W. E. R., Culbertson, J. W., Halperin, M. H., Litter, J., Burnett, C. H., Smithwick, R. H. Archs intern. Med. 1952, 86. 51. Priest, W. M. Br. med. J. 1952, ii 860.
a great deal of pain, and required 3 weeks to recover, compared with 11 days on the previous occasion. The whole birth was a very distressing experience and one I will not repeat. I realise that, in my case, the life of my baby and myself could not be risked by allowing the pregnancy to continue, but if I, understanding this and having confidence in my doctors, could experience such distress and pain, what must the effect be on women who are being induced to suit the convenience of the hospital authorities ? I appeal to all hospital staff to remember that women in labour. are human beings, and to allow them to have their babies with pleasure and dignity.
Afterwards, I had
6 Parc Hen
Bias, Llanfairfechan, Gwynedd LL33 0RW, North Wales.
LESLEY J. STRAIN.
EMERGENCY DOMICILIARY TREATMENT FOR HÆMOPHILIA SIR,-Le Quesne et al.1 reported a home treatment programme for haemophiliacs in which close relatives of patients learnt to prepare and administer plasma fractions. We would like to report the findings of a small pilot study in which a State-registered nurse, trained specifically in giving plasma fractions to haemophiliacs, provided a limited domiciliary service. There are several advantages of this modification to the usual home-treatment programmes; documentation is easier to control, samples for assays and screening for HBAg can be carried out more easily, and venepuncture tends to be of,a higher standard. It is also possible in such a scheme to include patients whose relatives would not be suitable for a routine home-treatment programme of the type described by Le Quesne et al. A disadvantage is that a doctor is not involved, unless the nurse feels that the nature or extent of the bleeding episode warrants further medical opinion; the director of the haemophilia centre has, of course, to agree to be responsible for the nurse. The pilot study ran for six months (July, 1974, to December, 1974). 6 severe haemophiliacs were selected for the study,
ranging from 10 to 33 years. All had a factor vm of less than 1%. 4 took part in the study, and a total of 27 emergency calls were made. At first, patients were diffident about using the service but towards the end of the study more confidence was gained by patients and their relatives; eventually up to 4 ’
calls a week were carried out. The system involved the use of an’Air-Call’ radio system switchboard. Patients would call this number, the nurse would be located, and the visit made within an hour. We felt that the services of a routine hospital switchboard might just as easily have been used; it so happened that one of the patient’s parents had a radio-telephone system at his disposal.’The nurse was on call from 12 noon-11.00 P.M. on Saturdays, 9.00 A.M.-l1.00 P.M. on Sundays and 6.00 p.M.-ll.OO P.M. on weekdays, and was paid the routine rates for on-call services. Only spontaneous and minor bleeding episodes were treated, with either 6 packs of cryoprecipitate cr 500 units of freeze-dried factor vin concentrate (’Hemofil’) as a standard treatment regime. Possible bleeding episodes into chest, abdomen, or skull, and all traumatic bleeding episodes were excluded from the domiciliary service, and treated at the hospital centre.
The
of
"
"
nurse-practitioners in the treatment of haemophilia may be an improvement on some of the existing schemes. Professor Ingram (St. Thomas’s Hospital Haemophilia Centre) has employed a sister for treating haemophilia during working hours. We would be interested use
hear of any other centres using similar schemes for the " after emergency treatment of haemophilia, particularly
to
hours ". Quesne, B., Britten, M. I., Maragaki, C., Dormandy, K. M. Lancet, 1974, ii, 507.
1. Le
