BRITISH MEDICAL JOURNAL
18 JANUARY 1975
Small Bowel Tumours Cancer of the small intestine is rare; it accounts for only 3-6% of all gastrointestinal neoplasms and less than 1% of all malignant tumours.1 The diagnosis is difficult to make preoperatively because the condition is so uncommon in clinical practice and because the symptoms are vague and often non-specific.2 The reason for the rarity of the tumours is not known; explanations include the fluidity and relative sterility of small bowel contents and the rapid transit time, which reduces any exposure to potential carcinogens. More recently it has been suggested that local immune responses are able to suppress the development of tumours.3 Small bowel neoplasms occur usually between the ages of 40 and 70 years and men and women are equally affected.4 More malignant than benign lesions are seen,5 and more than 90% of the malignant lesions are symptomatic.2 The two most common symptoms of malignant lesions are loss of weight and abdominal pain,6 which is often colicky, suggesting obstruction. Nausea and vomiting can occur, sometimes associated with the pain, the site of which is of little help in the diagnosis.5 Gastrointestinal bleeding and altered bowel habits are other common symptoms, but there is no characteristic clinical pattern. Benign tumours are much more likely to be asymptomatic, often being found incidentally at operation or necropsy. If symptoms do present pain is the most common, but insidious loss of blood leading to anaemia is another frequent manifestation. The length of history of small bowel tumours is surprisingly long, and asymptomatic intervals lasting weeks are frequently described.7 More than half the patients with a malignant lesion have symptoms present for longer than six months and in a recent series of cases symptoms had been present for longer than one year in 30% of patients.2 Diagnosis is either made by a barium meal and follow-through examination (50%) or by laparotomy; palpable masses are found in less than 30% of cases, though this figure is variable. Special syndromes exist which may help in the diagnosis. A carcinoid tumour associated with liver metastases produces the characteristic flush, diarrhoea, and heart disease of the carcinoid syndrome.8 In the absence of metastases and the carcinoid syndrome the majority of small intestinal carcinoids remain clinically silent. 9 The Peutz-Jeghers's syndrome,10 in which multiple polypoid hamartomas are found, predominantly in the small bowel, is inherited as a single dominant trait. The melanin pigmentation seen in the lips and the buccal and nasal mucosa gives the diagnosis. In Gardner's syndrome1' polyps are present in the small and large bowel. Multiple cutaneous soft tissue tumours and osteomas are sometimes seen in this condition, which is probably inherited as an autosomal dominant. Finally in the Cronkhite and Canada syndrome'2 diffuse gastrointestinal polyposis is associated with alopecia, nail dystrophy, and hyperpigmentation. In what sort of patients should there be a high suspicion of a small intestinal neoplasm ? Recent reports suggest an association between chronic regional enteritis (Crohn's disease) and small bowel adenocarcinoma." The association, though rare, seems convincing and may occur in patients who have had a bypass operation for Crohn's disease. Small bowel villous adenomas appear to have the same relationship to carcinoma as in colonic lesions, and there is also a high incidence of additional primary tumours in patients in whom one small bowel neoplasm has been found.'4 Lastly, there is a strong association between adult coeliac disease (gluten
115
enteropathy) and malignant lesions of the small bowel.15 Indeed if a treated patient with coeliac disease relapses then either he is not sticking to his diet or a malignant lesion has developed. Both lymphoma and carcinoma occur, but the incidence of carcinoma is reduced if the patient is treated with a gluten-free diet; this is as yet unproved for lymphomas.16 These findings justify the continued use of a gluten-free diet for the whole of the patient's life. Treatment for neoplasms of the small bowel is usually resection, with occasional radiotherapy for a malignant lesion. Resection of a benign tumour gives a cure, but the outlook for malignant lesions is gloomy. The five-year survival after diagnosis of an adenocarcinoma varies from 16-4 to 32%, 25% being inoperable at the time of laparotomy.2 Figures for lymphoma range from 31 to 40% five-year survival, while over half the patients with the carcinoid syndrome will survive five years. If susceptible patients are to be recognized awareness of the condition must improve-but the diagnosis is not always obvious, and the treatment is inadequate at present. Archives of Surgery, 1965, 91, 452. 'Ebert, P. A., and Zuidema,R.G.W.,D.,and Caplan, H. S., Annals of Surgery,
2
Silberman, H., Crichlow,
1974, 180, 157. Calman, K. C., Gut, 1974, 15, 552. Richlin, D. B., and Longmire, W. P., Jr., Surgery, 1961, 50, 586. Southam, J. A., Annals of the Royal College of Surgeons of England, 1974, 55, 129. 6 Wilson, J. M., et al., Annals of Surgery, 1974, 180, 175. 7Ostermiller, W., Joergenson, E. J., and Weilbel, L., American Journal of Surgery, 1966, 111, 403. 8 Sjoersdma, A., and Melmon, K. L., Gastroenterology, 1964, 47, 104. 9 Kuiper, D. H., Gracie, W. A., and Pollard, H. M., Cancer, 1970, 25, 1424. 10 Bussy, H. J. R., Gut, 1970, 11, 970. 1 Sachatello, C. R., Pickren, J. W., and Grace, J. T., Gastroenterology, 1970, 58, 699. 12 Cronkhite, L. W., and Canada, W. J., New England Journal of Medicine, 1955, 252, 1011. 13 Tyers, G. F. O., Steiger, E., and Dudrick, S. J., Annals of Surgery, 1969, 169, 510. 14 Reyes, E. L., and Talley, R. W., American Journal of Gastroenterology, 1970, 54, 30. 15 Barry, R. E., and Read, A. E., Quarterly3Journal ofMedicine, 1973,42, 665. 16 Holmes, G. K. T., et al., Gut, 1974, 15, 339. 17 Naqvi, M. S., Burrows, M. D., and Kark, A. E., Annals of Surgery, 1969, 170, 221. 3 4 5
Neonatal Nurse Practitioners Doctors are beginning to realize that responsibility which has traditionally been in their domain can be delegated safely to others. If they do not realize this they should visit the U.S.S.R.' or China,2 where physicians' assistants are responsible for much of the primary care. Alternatively, they can look to the U.S.A., where several types of specialist nurse have evolved.3 The obstetric and paediatric fields are particularly suitable for nurse practitioners. Many areas of America have in addition to midwives other registered nurses working either independently or in multidisciplinary practices or hospitals as paediatric nurse associates, family nurse practitioners, and well-baby nurse practitioners. A new species has recently entered the scene in Arizona: the neonatal nurse practitioner.4 The job of the neonatal nurse practitioner includes much of what a neonatal house officer does in Britain. She examines newborn infants and identifies anatomical abnormalities and physical signs. She his the knowledge and skill to order investigations such as a chest x-ray or an estimation of the blood sugar and is able to start therapy while waiting for the paediatrician. She discusses the condition of the baby with the mother, including giving her information on infant care and feeding.
BRITISH MEDICAL JOURNAL
116
Apparently 300 hours practical experience and teaching over a two-month period produces competent neonatal practitioners.4 Such a brief course must demand a good general knowledge of biological and behavioural sciences as well as considerable previous clinical experience. The objections to new roles for nurses sometimes seem to be based more on prejudice and ancient history than on modern realities. The nursing profession rightly claims the "caring role" to be its unique contribution to the welfare of the sick. However, good caring has always included skillful observation, decision making, empathy, and the ability to communicate with worried people. The training for neonatal nurse practitioners is one way of legitimizing and increasing the value of these skills. The experienced nurse has always had a hunch that there is something wrong with an ill noenate; with suitable training she can be more precise and more useful. Those who question the advisability of nurses being empowered to request radiological or pathological investigations are shutting their eyes to a practice which occurs in many departments of most British hospitals. There is no difficulty in suggesting new roles for able nurses, but when nurses are in short supply such suggestions must be considered with caution. Some paediatricians would welcome neonatal nurse practitioners. In the last decade the appointment of resident neonatal house physicians has done much to improve the standard of neonatal care, but the rule that they must be on duty only one night in three, and the impracticability of appointing three residents to a small or medium-sized unit, means that alternative solutions have to be considered. Neonatal nurse practitioners might be one such a solution-provided that once trained they stayed in the job for a reasonable length of time. For the nurse the job would be a rewarding one. Nurse dissatisfaction is not confined to low pay; their career structure, status, and job satisfaction are also important. Nurse practitioners would have considerable status and job satisfaction; it would be (and indeed is) up to the nursing profession to provide the necessary career structure for the clinical nurse. Sidel, V. W., Annals New York Academy of Science, 1969, 166, 957. Sidel, V. W., New England Journal of Medicine, 1972, 286. 1292. 3 U.S. Department of Health, Education and Welfare. Health Resources News Release, 16 August, 1973. 4 Slovis, T. L., and Comerci, G. P., American_Journal of Diseases of Children, 1974, 128, 310. 2
Protecting Her Flanks The Secretary of State underestimated the widespread opposition among consultants to her contract proposals. So last week saw Mrs. Castle protecting her flanks by trying to placate G.P.s over their pay prospects (p. 166) and negotiating the principles of a new contract for junior staff to start in October (p. 159). This at least gives the Government a chance to think again about its stand on the consultants' new contracts. It would be a grave mistake for the Elephant and Castle to conclude that the consultants are now nicely isolated: they are not. General practitioners and junior doctors have already voiced their continuing support for their colleagues. Indeed, the parliamentary statement Mrs. Castle made as we went to press (p. 159) suggests that she may at last
18 JANUARY 1975
be recognizing the force of the consultants' views. The Centra Committee for Hospital Medical Services were due to assess on 16 January her latest comments on the situation. It is often difficult to separate the conduct of politics from public relations-and Mrs. Castle is an experienced user of the media. So doctors must look through the shadow of what she says in public and search for the substance beyond. Admittedly, the joint statement issued after the B.M.A.'s junior staff representatives had seen her seems straightforward enough. The young doctors have by determined negotiating tactics obtained her promise of contract proposals that fit the mood of the 1970s. Nevertheless, no sooner had Mrs. Castle given her word than the Chancellor of the Exchequer was warning high wage earners of possible cuts in their living standards.' The starting date of October 1975 is later than the H.J.S. Group Council wanted. But it gives enough time for the details to be tidied up in the Joint Negotiating Committee, and for evidence on the contract's pricing to be presented to the Review Body. Young doctors are as aware as are their seniors that the N.H.S. cannot be insulated from the country's worsening economic state. So they may question whether the Government, when actually faced in October with the cost of "overtime" above the basic 40-hour standard working week, will not find it necessary to postpone payment-for "compelling reasons."2 Certainly the General Medical Services Committee had no illusions last week about the credibility gap between Westminster's words and Whitehall's execution. But eschewing calls for immediate resignation-though taking the precaution to ask all N.H.S. family doctors for their undated resignations -the G.M.S. Committee did accept the Secretary of State's assurances, which included the important undertaking to meet a deadline of 1 April for the 1975 award. But as the Government's public sector borrowing for 1974-5 spirals towards £7000m. where will the money come from to pay the doctors ? And it is not only doctors' pay. Nurses are reported to be seeking further large increases, and Lord Halsbury has proposed welcome-and overdue-rises for the paramedical professions that will put top physiotherapists, radiographers, and chiropodists into the £5000-a-year bracket, (p. 160). His report also endorses realistic on-call payments -an item specifically excluded from the Government's December proposals for senior medical staff. The cost of this award is a mere £13m., some of it already paid on account. Double this sum and more could be needed when a new consultants' contract has been priced. In face of the mounting cost of staff and equipment, together with a virtual standstill on capital expenditure, is the Government still determined to refuse an independent inquiry into N.H.S. financing ? This should consider not just how to raise moneythough we all manage to spend £2000m. a year or more on cigarettes and alcohol-but also priorities in N.H.S. spending. The health professions' leaders are to follow up their meeting with the Prime Minister by visiting the Health Ministers to discuss the underfinancing of the Health Service.3 Could they not urge them to forget party politics and in the interests of the nation set up such an inquiry ? Apart from opening the public's eyes to the vast resources needed to run a first class comprehensive Health Service it would do a lot for staff morale. They are fed up with years of trying to run the Service on a hand-to-mouth basis. 1
The Guardian, 11 January 1975, p. 1. British Medical Journal, 1970, 3, 359. 3British Medical Journal, 1974, 4, 297. 2
18 JANUARY 1975
Small Bowel Tumours Cancer of the small intestine is rare; it accounts for only 3-6% of all gastrointestinal neoplasms and less than 1% of all malignant tumours.1 The diagnosis is difficult to make preoperatively because the condition is so uncommon in clinical practice and because the symptoms are vague and often non-specific.2 The reason for the rarity of the tumours is not known; explanations include the fluidity and relative sterility of small bowel contents and the rapid transit time, which reduces any exposure to potential carcinogens. More recently it has been suggested that local immune responses are able to suppress the development of tumours.3 Small bowel neoplasms occur usually between the ages of 40 and 70 years and men and women are equally affected.4 More malignant than benign lesions are seen,5 and more than 90% of the malignant lesions are symptomatic.2 The two most common symptoms of malignant lesions are loss of weight and abdominal pain,6 which is often colicky, suggesting obstruction. Nausea and vomiting can occur, sometimes associated with the pain, the site of which is of little help in the diagnosis.5 Gastrointestinal bleeding and altered bowel habits are other common symptoms, but there is no characteristic clinical pattern. Benign tumours are much more likely to be asymptomatic, often being found incidentally at operation or necropsy. If symptoms do present pain is the most common, but insidious loss of blood leading to anaemia is another frequent manifestation. The length of history of small bowel tumours is surprisingly long, and asymptomatic intervals lasting weeks are frequently described.7 More than half the patients with a malignant lesion have symptoms present for longer than six months and in a recent series of cases symptoms had been present for longer than one year in 30% of patients.2 Diagnosis is either made by a barium meal and follow-through examination (50%) or by laparotomy; palpable masses are found in less than 30% of cases, though this figure is variable. Special syndromes exist which may help in the diagnosis. A carcinoid tumour associated with liver metastases produces the characteristic flush, diarrhoea, and heart disease of the carcinoid syndrome.8 In the absence of metastases and the carcinoid syndrome the majority of small intestinal carcinoids remain clinically silent. 9 The Peutz-Jeghers's syndrome,10 in which multiple polypoid hamartomas are found, predominantly in the small bowel, is inherited as a single dominant trait. The melanin pigmentation seen in the lips and the buccal and nasal mucosa gives the diagnosis. In Gardner's syndrome1' polyps are present in the small and large bowel. Multiple cutaneous soft tissue tumours and osteomas are sometimes seen in this condition, which is probably inherited as an autosomal dominant. Finally in the Cronkhite and Canada syndrome'2 diffuse gastrointestinal polyposis is associated with alopecia, nail dystrophy, and hyperpigmentation. In what sort of patients should there be a high suspicion of a small intestinal neoplasm ? Recent reports suggest an association between chronic regional enteritis (Crohn's disease) and small bowel adenocarcinoma." The association, though rare, seems convincing and may occur in patients who have had a bypass operation for Crohn's disease. Small bowel villous adenomas appear to have the same relationship to carcinoma as in colonic lesions, and there is also a high incidence of additional primary tumours in patients in whom one small bowel neoplasm has been found.'4 Lastly, there is a strong association between adult coeliac disease (gluten
115
enteropathy) and malignant lesions of the small bowel.15 Indeed if a treated patient with coeliac disease relapses then either he is not sticking to his diet or a malignant lesion has developed. Both lymphoma and carcinoma occur, but the incidence of carcinoma is reduced if the patient is treated with a gluten-free diet; this is as yet unproved for lymphomas.16 These findings justify the continued use of a gluten-free diet for the whole of the patient's life. Treatment for neoplasms of the small bowel is usually resection, with occasional radiotherapy for a malignant lesion. Resection of a benign tumour gives a cure, but the outlook for malignant lesions is gloomy. The five-year survival after diagnosis of an adenocarcinoma varies from 16-4 to 32%, 25% being inoperable at the time of laparotomy.2 Figures for lymphoma range from 31 to 40% five-year survival, while over half the patients with the carcinoid syndrome will survive five years. If susceptible patients are to be recognized awareness of the condition must improve-but the diagnosis is not always obvious, and the treatment is inadequate at present. Archives of Surgery, 1965, 91, 452. 'Ebert, P. A., and Zuidema,R.G.W.,D.,and Caplan, H. S., Annals of Surgery,
2
Silberman, H., Crichlow,
1974, 180, 157. Calman, K. C., Gut, 1974, 15, 552. Richlin, D. B., and Longmire, W. P., Jr., Surgery, 1961, 50, 586. Southam, J. A., Annals of the Royal College of Surgeons of England, 1974, 55, 129. 6 Wilson, J. M., et al., Annals of Surgery, 1974, 180, 175. 7Ostermiller, W., Joergenson, E. J., and Weilbel, L., American Journal of Surgery, 1966, 111, 403. 8 Sjoersdma, A., and Melmon, K. L., Gastroenterology, 1964, 47, 104. 9 Kuiper, D. H., Gracie, W. A., and Pollard, H. M., Cancer, 1970, 25, 1424. 10 Bussy, H. J. R., Gut, 1970, 11, 970. 1 Sachatello, C. R., Pickren, J. W., and Grace, J. T., Gastroenterology, 1970, 58, 699. 12 Cronkhite, L. W., and Canada, W. J., New England Journal of Medicine, 1955, 252, 1011. 13 Tyers, G. F. O., Steiger, E., and Dudrick, S. J., Annals of Surgery, 1969, 169, 510. 14 Reyes, E. L., and Talley, R. W., American Journal of Gastroenterology, 1970, 54, 30. 15 Barry, R. E., and Read, A. E., Quarterly3Journal ofMedicine, 1973,42, 665. 16 Holmes, G. K. T., et al., Gut, 1974, 15, 339. 17 Naqvi, M. S., Burrows, M. D., and Kark, A. E., Annals of Surgery, 1969, 170, 221. 3 4 5
Neonatal Nurse Practitioners Doctors are beginning to realize that responsibility which has traditionally been in their domain can be delegated safely to others. If they do not realize this they should visit the U.S.S.R.' or China,2 where physicians' assistants are responsible for much of the primary care. Alternatively, they can look to the U.S.A., where several types of specialist nurse have evolved.3 The obstetric and paediatric fields are particularly suitable for nurse practitioners. Many areas of America have in addition to midwives other registered nurses working either independently or in multidisciplinary practices or hospitals as paediatric nurse associates, family nurse practitioners, and well-baby nurse practitioners. A new species has recently entered the scene in Arizona: the neonatal nurse practitioner.4 The job of the neonatal nurse practitioner includes much of what a neonatal house officer does in Britain. She examines newborn infants and identifies anatomical abnormalities and physical signs. She his the knowledge and skill to order investigations such as a chest x-ray or an estimation of the blood sugar and is able to start therapy while waiting for the paediatrician. She discusses the condition of the baby with the mother, including giving her information on infant care and feeding.
BRITISH MEDICAL JOURNAL
116
Apparently 300 hours practical experience and teaching over a two-month period produces competent neonatal practitioners.4 Such a brief course must demand a good general knowledge of biological and behavioural sciences as well as considerable previous clinical experience. The objections to new roles for nurses sometimes seem to be based more on prejudice and ancient history than on modern realities. The nursing profession rightly claims the "caring role" to be its unique contribution to the welfare of the sick. However, good caring has always included skillful observation, decision making, empathy, and the ability to communicate with worried people. The training for neonatal nurse practitioners is one way of legitimizing and increasing the value of these skills. The experienced nurse has always had a hunch that there is something wrong with an ill noenate; with suitable training she can be more precise and more useful. Those who question the advisability of nurses being empowered to request radiological or pathological investigations are shutting their eyes to a practice which occurs in many departments of most British hospitals. There is no difficulty in suggesting new roles for able nurses, but when nurses are in short supply such suggestions must be considered with caution. Some paediatricians would welcome neonatal nurse practitioners. In the last decade the appointment of resident neonatal house physicians has done much to improve the standard of neonatal care, but the rule that they must be on duty only one night in three, and the impracticability of appointing three residents to a small or medium-sized unit, means that alternative solutions have to be considered. Neonatal nurse practitioners might be one such a solution-provided that once trained they stayed in the job for a reasonable length of time. For the nurse the job would be a rewarding one. Nurse dissatisfaction is not confined to low pay; their career structure, status, and job satisfaction are also important. Nurse practitioners would have considerable status and job satisfaction; it would be (and indeed is) up to the nursing profession to provide the necessary career structure for the clinical nurse. Sidel, V. W., Annals New York Academy of Science, 1969, 166, 957. Sidel, V. W., New England Journal of Medicine, 1972, 286. 1292. 3 U.S. Department of Health, Education and Welfare. Health Resources News Release, 16 August, 1973. 4 Slovis, T. L., and Comerci, G. P., American_Journal of Diseases of Children, 1974, 128, 310. 2
Protecting Her Flanks The Secretary of State underestimated the widespread opposition among consultants to her contract proposals. So last week saw Mrs. Castle protecting her flanks by trying to placate G.P.s over their pay prospects (p. 166) and negotiating the principles of a new contract for junior staff to start in October (p. 159). This at least gives the Government a chance to think again about its stand on the consultants' new contracts. It would be a grave mistake for the Elephant and Castle to conclude that the consultants are now nicely isolated: they are not. General practitioners and junior doctors have already voiced their continuing support for their colleagues. Indeed, the parliamentary statement Mrs. Castle made as we went to press (p. 159) suggests that she may at last
18 JANUARY 1975
be recognizing the force of the consultants' views. The Centra Committee for Hospital Medical Services were due to assess on 16 January her latest comments on the situation. It is often difficult to separate the conduct of politics from public relations-and Mrs. Castle is an experienced user of the media. So doctors must look through the shadow of what she says in public and search for the substance beyond. Admittedly, the joint statement issued after the B.M.A.'s junior staff representatives had seen her seems straightforward enough. The young doctors have by determined negotiating tactics obtained her promise of contract proposals that fit the mood of the 1970s. Nevertheless, no sooner had Mrs. Castle given her word than the Chancellor of the Exchequer was warning high wage earners of possible cuts in their living standards.' The starting date of October 1975 is later than the H.J.S. Group Council wanted. But it gives enough time for the details to be tidied up in the Joint Negotiating Committee, and for evidence on the contract's pricing to be presented to the Review Body. Young doctors are as aware as are their seniors that the N.H.S. cannot be insulated from the country's worsening economic state. So they may question whether the Government, when actually faced in October with the cost of "overtime" above the basic 40-hour standard working week, will not find it necessary to postpone payment-for "compelling reasons."2 Certainly the General Medical Services Committee had no illusions last week about the credibility gap between Westminster's words and Whitehall's execution. But eschewing calls for immediate resignation-though taking the precaution to ask all N.H.S. family doctors for their undated resignations -the G.M.S. Committee did accept the Secretary of State's assurances, which included the important undertaking to meet a deadline of 1 April for the 1975 award. But as the Government's public sector borrowing for 1974-5 spirals towards £7000m. where will the money come from to pay the doctors ? And it is not only doctors' pay. Nurses are reported to be seeking further large increases, and Lord Halsbury has proposed welcome-and overdue-rises for the paramedical professions that will put top physiotherapists, radiographers, and chiropodists into the £5000-a-year bracket, (p. 160). His report also endorses realistic on-call payments -an item specifically excluded from the Government's December proposals for senior medical staff. The cost of this award is a mere £13m., some of it already paid on account. Double this sum and more could be needed when a new consultants' contract has been priced. In face of the mounting cost of staff and equipment, together with a virtual standstill on capital expenditure, is the Government still determined to refuse an independent inquiry into N.H.S. financing ? This should consider not just how to raise moneythough we all manage to spend £2000m. a year or more on cigarettes and alcohol-but also priorities in N.H.S. spending. The health professions' leaders are to follow up their meeting with the Prime Minister by visiting the Health Ministers to discuss the underfinancing of the Health Service.3 Could they not urge them to forget party politics and in the interests of the nation set up such an inquiry ? Apart from opening the public's eyes to the vast resources needed to run a first class comprehensive Health Service it would do a lot for staff morale. They are fed up with years of trying to run the Service on a hand-to-mouth basis. 1
The Guardian, 11 January 1975, p. 1. British Medical Journal, 1970, 3, 359. 3British Medical Journal, 1974, 4, 297. 2
