Journal of the Royal Society of Medicine Volume 85 February 1992
63
Editorials
Familial adenomatous polyposis It has been considered that surgery for familial adenomatous polyposis (FAP) presents one of the major opportunities for the prevention of bowel cancer. Excision of all, or most of the at-risk mucosa, together with regular surveillance should reduce the incidence and thereby the mortality from colorectal cancer in FAP. While this is indeed still true as far as the large bowel is concerned, there is increasing realization that, FAP is -a systemic disease. Until recently it was felt that there was a separate entity, that of Gardner's syndrome, where intestinal polyposis was associated with extra colonic manifestations/ osteomata, desmoids, epidermal cysts, etc. Again the evidence is accumulating that this is indeed a variant of FAP and that many patients with FAP have extra-colonic manifestations. As can be seen from the two papers published in the Journal of the Royal Society of Medicinel"2, there is a high incidence of duodenal adenomas and carcinomas and a somewhat lower incidence of gastric adenomas. Most patients with FAP show changes in the retinal epithelium, the characteristic congenital hypertrophy of the retinal pigmentation epithelium (CHRPE)3 and if looked for carefully, osteomata are present4. Perhaps it is to be expected that with a defect in the constitutional genome one would expect to see a diffuse disorder within the organism. Because of its mode of inheritance (autosomal dominant) and the relative ease by which the phenotypic changes can be detected ie by endoscopy, FAP has become a particularly interesting disorder to study. The aspects in which special interest have been shown are the clinical management, screening and prevention and as a model for genesis ofcolorectal cancer. Whether this latter change is the same as happens with the sporadic form of colorectal cancer has yet to be established. There are a number of areas in clinical management and research in FAP where there are now changes occurring and in which controversy exists. As far as screening for the disease is concerned, more polyposis registries are being established throughout the United Kingdom. The careful collection offamily trees gives a mechanism for more comprehensive screening of the offspring in those families. Traditionally, the mainstay of screening has been rigid sigmoidoscopy, since it is said that presence or lack of polyps within the rectum will reflect the presence or absence of polyps throughout the colon. Some workers feel that this should be supplemented either with flexible sigmoidoscopy or a total colonoscopy early in the screening process since proximal polyps may occur in the absence of rectal polyps5. It is now recognized that the eye signs (CHRPES) are present in most affected individuals in a FAP3 kindred and therefore it is now possible to assign risk to an individual using a risk factor calculated from a combination of the
endoscopic and retinal findings, since if both are negative then risk is lower. These risk scores as yet need to be verified in prospective studies but if validated they may have important implications for planning screening - allowing less frequent examinations in low risk individuals and in counselling members of FAP families. With the realization of the frequency of the upper gut abnormalities in FAP6, the screening of the upper GI tract by endoscopy in patients with established FAP and surveillance of those with duodenal adenomas (over 90%) will become more important. With regard to treatment of FAP the standard for the prevention and/or treatment of large bowel cancer has been excision of the large bowel, either in total or with preservation of the rectum and an ileorectal anastomosis. This of course leaves at risk rectal mucosa which requires long term surveillance. However, there is evidence to suggest that rectal adenomas regress after sub-total colectomy and ileorectal anastomosis7 which indicates that even in FAP, a strongly genetic condition, there is some effect of the environmint, ie the luminal contents on the production or regression of adenomas. This gives encouragement to the concept of dietary intervention to alter the course of the progression of FAP. The development of the ileo-anal pouch procedure, has enabled surgeons to remove all of the rectal mucosa, replacing the rectum with an ileal reservoir, while retaining anal evacuation. A report from St Mark's Hospital indicates that the function between an ileo-rectal anastomosis and pouch is fairly similar and that the benefit of the pouch procedure is that all of the at risk mucosa is removed8. It is however a larger operation with a higher postoperative complication rate and particularly in young and asymptomatic individuals, many surgeons still prefer rectal preservation and surveillance. A further major problem in the clinical management for FAP is that of desmoid tumours. These affect only a relatively small proportion of patients with FAP and in some cases may be a relatively small clinical problem. On the other hand, when they occur intra-abdominally and particularly in the small bowel mesentery, they can present considerable management problems. The choice lies between observation, surgical removal or some form of medical treatment. Observation of the patient runs the risk of 'missing the boat' with respect to surgical excision, however, operation can be extremely hazardous and haemorrhagic and can result in the loss of considerable quantities of intestine. Since the tumours often reoccur after excision, many surgeons will adopt a conservative approach9. Various non-operative measures have been tried including non-steroidal anti-inflammatory drugs, tamoxifen and others, with occasional reports of successful regression of desmoids'0. However, there is no consensus in this area and the complications of desmoid tumour remain a major problem.
0141-0768/92/ 020063-03/$02.00/0 © 1992 The Royal Society of Medicine
64
Journal of the Royal Society of Medicine Volume 85 February 1992
Perhaps more encouraging are the prospects for intervention therapy in FAP. As previously mentioned, it is known that rectal polyps can regress after ileorectal anastomosis7. A study performed by Bussey et aL showed that the adenoma mass could be reduced by administration of vitamin C to patients with FAP". A recent long term study in patients with FAP after colectomy and ileorectal anastomosis randomized to receive vitamins C and E, wheat fibre or controls suggested a benefit for patients given wheat fibre'2. Spigelman et al. have shown that white blood cell levels of ascorbic acid were lower in post colectomy FAP patients than in healthy controls and were similar to levels found in chronic illness - rheumatoid arthritis, inflammatory bowel disease, etc suggesting either reduced dietary intake or absorption'3. Friedman has shown that abnormally high proliferation of colon epithelial cells in biopsies from asymptomatic individuals in FAP kindreds were reduced in vitro by increasing the concentration of calcium as were two of seven adenoma cells although carcinomas (non polyposis) were unaffected14. However, a report from Toronto'5 showed that although dietary intervention with calcium in patients with FAP showed a reduction in mucosal proliferation at 6 months this effect was not present at 9 months. Longer term studies will be needed to evaluate any effect on the risk of progression of adenomas. An interesting report of the regression of colon polyps in 10 patients with FAP/Gardner's syndrome on the administration of sulindac - a non-steroidal antiinflammatory agent'6 and suggests a role for such agents possibly by prostaglandin inhibition and/or the inhibition of ornithine decarboxylase in the control of cell cycle abnormalities in FAP patients. If we accept that FAP is a systemic disorder and recognition ofthe genotypic abnormality can be made before the production of adenomas in the colon, intervention may prevent the colonic manifestations and the possibility for perhaps influencing the extra colonic manifestations. Perhaps the most exciting developments in FAP have been in the area of genetics. Since Herrera reported the deletion of part of chromosome 5 in a patient with a polyposis syndrome'7, intense interest has centred on this chromosome. Bodmer reported a DNA marker, CllP1l which showed close linkage to the FAP locus and has shown to be present on the long arm of chromosome 518. Using this marker and five other markers which 'flank' the FAP gene, Dunlop reported a series of 41 asymptomatic individuals from FAP families who were genetically screened19. Twenty-eight were informative for the probes and in these 14 of 15 individuals who were screened by colonic examination or retinal examination and radiology were found to have probe-derived risks of 0.93 or greater. Of these, four had been previously discharged from follow up. A further 14 individuals were assigned risk estimations of less than 0.01 and 16 risk of less than 0.1. Although yet to be validated by such observations, follow up taken together with clinical data should prove to be very useful in counselling members of FAP families and in determining the frequency and duration of screening. One very interesting observation was made by Sasazukri et al. who introduced a single human chromosome 5 into a murine hepatoma cell line (7R.1) which suppressed the tumnorogenicity ofthat cell line in nude mice and suppressed the in vitro growth of a human
carcinoma cell line which showed partial deletion of chromosome 5q20. Such observations indicate that there may be a possibility of correcting the genetic defect in FAP although further work is necessary to show this. Other genetic abnormalities have been shown in tumours in affected individuals with FAP which include frequent allele losses in chromosome 12q, 17p, and 2221. Such findings suggest that changes of more than one chromosome may be necessary for the initiation and progression of tumours in FAP. Thus, studies of the genetic changes in FAP may allow early identiflcation of affected individuals and by implication unaffected individuals. This allows a possibility of altering the genome in order to correct the abnormality or by intervention to delay or prevent the manifestations of the clinical condition. Above all however, important insights may be gained into the genetics of the sporadic type of colorectal cancer which may allow similar advances.
N C Armitage University Hospital, Queen's Medical Centre Nottingham NG7 2UH References 1 Spigelman AD, Granowska M, Phillips RKS. Bile reflux and gastric adenomas: A scintigraphic study in patients with familial adenomatous polyposis (FAP). JR Soc Med 1991;84:476-8 2 Spigelman AD, Williams CB, Phillips RKS. Rectal polyposis as a guide to duodenal polyposis in familial adenomatous polyposis. J R Soc Med 1992;85:77-79 3 Polkinghorne PJ, Ritchie S, Neale K, Schoeppner G, Thomson JPS, Jay BS. Pigmented lesions of the retinal epithelium and familial adenomatous polyposis. Eye 1990;4:216-21 4 Bulow S, Sondergaard JO, Witt I, Larsen E, Tetens G. Mandibular osteomas in familial polyposis coli. Dis Colon Rectum 1984:105-8 5 Perry RE, Christensen MA, Thorson AG, Williams T. Familial polyposis: colon cancer in the absence of rectal polyps. Br J Surg 1989;76:744 6 Spigelman AD, Williams CB, Talbot IC, Domizio P, Phillips RKS. Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet 1989;
i:783-5 7 Hubbard TB. Familial polyposis of the colon: the role ofthe retained rectum after colectomy in children. Am J Surg 1957;23:577-86 8 Madden MV, Neale KF, Nicholls RJ, et al. Comparison of morbidity and function after colectomy with ileo-rectal anastomosis or restorative proctocolectomy for familial adenomatous polyposis. Br J Surg 1991;78:788-92 9 Dozois RR. Surgical aspects offamilial. Int J Colorectal Dis 1988;3:1-16 10 Ramos R, Carrel A, Herrera L. Current treatment of desmoids. In: Herrera L, ed. Familial adenomatous polyposis. New York: Alan Liss, 1990:133-46 11 Bussey HJR, De Cosse JJ, Deschner EE, et al. A randomised trial of ascorbic acid in polyposis coli.
Cancer 1982;50:1434-9 12 De Cosse JJ, Miller HH, Lesser ML. Effect ofwheat fiber and vitamins C and E on rectal polyps in patients with familial adenomatous polyposis. J Natl Cancer Inst 1989;81:1290-7 13 Spigelman AD, Uff CR, Phillips RKS. Vitamin C levels with familial adenomatous polyposis. Br J Surg 1990;77:508-9 14 Fvriedman E, Lipkin M, Winawer S, Buset M, Newmark H. Heterogeneity in the response of familial polyposis epithelial cells and adenomas to increasing levels of calcium in vitro. Cancer 1989;63:2486-91
Journal of the Royal Society of Medicine Volume 85 February 1992 15 Stern HS, Gregoire RC, Kashtan H, Stadler J, Bruce WR. Long-term effects of dietary calcium on risk markers for colon cancer in patients with familial polyposis. Surgery 1990;108:528-33 16 Waddell WR, Ganser GF, Cerise EJ, Loughry RW. Sulindac for polyposis of the colon. Am J Surg 1989; 157:175-9 17 Herrera L, Kataki S, Gibas L, Pietrzak E, Sandberg AA. Gardner syndrome in a man with an interstitial deletion of 5q. Am J Med Genet 1986;25:473-6 18 Bodmer WF, Bailey CJ, Bodmer J, et aL Localisation ofthe gene for familial polyposis coli on chromosome 5. Nature 1987;328:614-16
How to avoid pitfalls in ethnic medical history, examination and diagnosis Transcultural Medicine means the science and art of dealing with patients from different cultures. It is defined as follows: 'Transcultural Medicine is the knowledge of medical and communication encounters between a doctor or health worker of one ethnic group and a patient of another. It embraces the physical, psychological, and social aspects of care as well as the scientific aspects of culture, religion and ethnicity without getting involved in the politics of segregation or integration.'I
This approach may be useful in a cross-cultural contact during a medical consultation as short as 10 minutes or a detailed medical examination lasting an hour. I recognize four realities - patriotism, neocolonialism, racism, and fundamentalism - as political issues of our times, but leave them to other experts who specialize in these fields. A British general practice list or a hospital ward may have patients from two cultures (Western or Eastern), four races (European or Caucasian, African or Negroid, Asian or Asiatic, and Chinese or Mongoloid) and six major religions (Hinduism,
Buddhism, Sikhism, Judaism, Christianity, and Islam). In addition, mnany patients will be: westernized Easterners; children of mixed marriages; and custodians of secular views. It is within a doctor's intellectual grasp to appreciate these categories (without stereotyping) and provide appropriate care. This Editorial deals with a doctor's role in the management of patients from cultures other than his or her own, and it highlights only a few out of many transcultural issues. Every doctor must ask each patient about his or her history of: presenting complaints; story of present illness; summar of past illnesses; menstrual, obstetric, and sexual difficulties; treatment used for present and past illnesses; family patterns of disease; social habits; and occupational hazards. If a doctor and the patient come from the same culture, race and religion, then standard pathological, psychological, and socioeconomical terms will probably be sufficient in making a diagnosis and routine questions should be asked. Where this is not the case, transcultural factors must be considered. In taking history, three cultural
65
19 Dunlop MG, Wyllie AH, Steel CM, Piris J, Evans HJ. Linked DNA markers for presymptomatic diagnosis of familial adenomatous polyposis. Lancet 1991;337: 313-16 20 Sasazuki T, Sasaki M, Sugio K, et al. Molecular and genetic analysis in familial adenomatous polyposis (FAP). Int J Colorectal 1990;5:57 21 Okamoto M, Sato C, Iwama T, Miyaki M. Loss of alleles in patients with familial adenomatous polyposis and colon cancers. In: Herrera L, ed. Familial adenomatous polyposis. New York: Alan Liss, 1990: 383-91
aspects are now described relating to language, the meaning of pain, and the privacy of age and religion. It is important to make sure that the doctor and the patient are talking about the same thing. It is traumatic for the doctor and impending disaster for the patient if an English patient were to be interviewed by a French doctor who could not speak a word of English. Where a language barrier exists, an interpreter or link-person (language and culture 'translater) should be used. Pain of some sort is the commonest complaint which brings a patient to the doctor. It is naive to think that the social education of patients from all ethnic groups is the same as that of the doctor. Pain means different things to different people2. An Oxbridge patient may describe pain as an ache, cramp, hurt, irritation, pang, soreness, spasm, tenderness, throb, trouble, twinge, agony, anguish, bitterness, disquiet, distress, grief, heartache, misery, suffering, torment, torture, and woe. A Navajo (North American Indians who live in Arizona) tourist may be at a loss when seeing an English doctor because the Navajo are the only people in the world who have no word for pain in their language3. The vocabulary about various degrees of pain varies with the age, social class, education and culture of a patient. It is not uncommon for an ethnic minority patient: to be unable to describe the type and severity of pain; to translate wrongly from the mother tongue into English; to repeat the same word due to lack of vocabulary; and to exaggerate the symptom by using a word that a doctor from another culture can understand, so as to attract his attention. The knowledge of medicine is science but its practice is an art. A doctor should remember 'cum Scientia Caritas' and spend more time when taking history from patients from other cultures. An accurate history is a prerequisite of correct diagnosis and appropriate management. English patients are shy of revealing their age and reluctant to discuss their religion or political persuasion because they consider this a part of their personal privacy. An ethnic minority doctor should be very tactful when asking about their ages or religion. However, the reverse is the case among the ethnic minorities. An English doctor should be bold in asking the age or religion when taking the history from an Asian, African, Chinese,, or other such patients because they feel proud to give this information. In general, they live by their religion. Age is an advantage in the East and with advancing years an
0141-0768/92/ 020065-02/$02.00/0 © 1992 The Royal Society of Medicine
63
Editorials
Familial adenomatous polyposis It has been considered that surgery for familial adenomatous polyposis (FAP) presents one of the major opportunities for the prevention of bowel cancer. Excision of all, or most of the at-risk mucosa, together with regular surveillance should reduce the incidence and thereby the mortality from colorectal cancer in FAP. While this is indeed still true as far as the large bowel is concerned, there is increasing realization that, FAP is -a systemic disease. Until recently it was felt that there was a separate entity, that of Gardner's syndrome, where intestinal polyposis was associated with extra colonic manifestations/ osteomata, desmoids, epidermal cysts, etc. Again the evidence is accumulating that this is indeed a variant of FAP and that many patients with FAP have extra-colonic manifestations. As can be seen from the two papers published in the Journal of the Royal Society of Medicinel"2, there is a high incidence of duodenal adenomas and carcinomas and a somewhat lower incidence of gastric adenomas. Most patients with FAP show changes in the retinal epithelium, the characteristic congenital hypertrophy of the retinal pigmentation epithelium (CHRPE)3 and if looked for carefully, osteomata are present4. Perhaps it is to be expected that with a defect in the constitutional genome one would expect to see a diffuse disorder within the organism. Because of its mode of inheritance (autosomal dominant) and the relative ease by which the phenotypic changes can be detected ie by endoscopy, FAP has become a particularly interesting disorder to study. The aspects in which special interest have been shown are the clinical management, screening and prevention and as a model for genesis ofcolorectal cancer. Whether this latter change is the same as happens with the sporadic form of colorectal cancer has yet to be established. There are a number of areas in clinical management and research in FAP where there are now changes occurring and in which controversy exists. As far as screening for the disease is concerned, more polyposis registries are being established throughout the United Kingdom. The careful collection offamily trees gives a mechanism for more comprehensive screening of the offspring in those families. Traditionally, the mainstay of screening has been rigid sigmoidoscopy, since it is said that presence or lack of polyps within the rectum will reflect the presence or absence of polyps throughout the colon. Some workers feel that this should be supplemented either with flexible sigmoidoscopy or a total colonoscopy early in the screening process since proximal polyps may occur in the absence of rectal polyps5. It is now recognized that the eye signs (CHRPES) are present in most affected individuals in a FAP3 kindred and therefore it is now possible to assign risk to an individual using a risk factor calculated from a combination of the
endoscopic and retinal findings, since if both are negative then risk is lower. These risk scores as yet need to be verified in prospective studies but if validated they may have important implications for planning screening - allowing less frequent examinations in low risk individuals and in counselling members of FAP families. With the realization of the frequency of the upper gut abnormalities in FAP6, the screening of the upper GI tract by endoscopy in patients with established FAP and surveillance of those with duodenal adenomas (over 90%) will become more important. With regard to treatment of FAP the standard for the prevention and/or treatment of large bowel cancer has been excision of the large bowel, either in total or with preservation of the rectum and an ileorectal anastomosis. This of course leaves at risk rectal mucosa which requires long term surveillance. However, there is evidence to suggest that rectal adenomas regress after sub-total colectomy and ileorectal anastomosis7 which indicates that even in FAP, a strongly genetic condition, there is some effect of the environmint, ie the luminal contents on the production or regression of adenomas. This gives encouragement to the concept of dietary intervention to alter the course of the progression of FAP. The development of the ileo-anal pouch procedure, has enabled surgeons to remove all of the rectal mucosa, replacing the rectum with an ileal reservoir, while retaining anal evacuation. A report from St Mark's Hospital indicates that the function between an ileo-rectal anastomosis and pouch is fairly similar and that the benefit of the pouch procedure is that all of the at risk mucosa is removed8. It is however a larger operation with a higher postoperative complication rate and particularly in young and asymptomatic individuals, many surgeons still prefer rectal preservation and surveillance. A further major problem in the clinical management for FAP is that of desmoid tumours. These affect only a relatively small proportion of patients with FAP and in some cases may be a relatively small clinical problem. On the other hand, when they occur intra-abdominally and particularly in the small bowel mesentery, they can present considerable management problems. The choice lies between observation, surgical removal or some form of medical treatment. Observation of the patient runs the risk of 'missing the boat' with respect to surgical excision, however, operation can be extremely hazardous and haemorrhagic and can result in the loss of considerable quantities of intestine. Since the tumours often reoccur after excision, many surgeons will adopt a conservative approach9. Various non-operative measures have been tried including non-steroidal anti-inflammatory drugs, tamoxifen and others, with occasional reports of successful regression of desmoids'0. However, there is no consensus in this area and the complications of desmoid tumour remain a major problem.
0141-0768/92/ 020063-03/$02.00/0 © 1992 The Royal Society of Medicine
64
Journal of the Royal Society of Medicine Volume 85 February 1992
Perhaps more encouraging are the prospects for intervention therapy in FAP. As previously mentioned, it is known that rectal polyps can regress after ileorectal anastomosis7. A study performed by Bussey et aL showed that the adenoma mass could be reduced by administration of vitamin C to patients with FAP". A recent long term study in patients with FAP after colectomy and ileorectal anastomosis randomized to receive vitamins C and E, wheat fibre or controls suggested a benefit for patients given wheat fibre'2. Spigelman et al. have shown that white blood cell levels of ascorbic acid were lower in post colectomy FAP patients than in healthy controls and were similar to levels found in chronic illness - rheumatoid arthritis, inflammatory bowel disease, etc suggesting either reduced dietary intake or absorption'3. Friedman has shown that abnormally high proliferation of colon epithelial cells in biopsies from asymptomatic individuals in FAP kindreds were reduced in vitro by increasing the concentration of calcium as were two of seven adenoma cells although carcinomas (non polyposis) were unaffected14. However, a report from Toronto'5 showed that although dietary intervention with calcium in patients with FAP showed a reduction in mucosal proliferation at 6 months this effect was not present at 9 months. Longer term studies will be needed to evaluate any effect on the risk of progression of adenomas. An interesting report of the regression of colon polyps in 10 patients with FAP/Gardner's syndrome on the administration of sulindac - a non-steroidal antiinflammatory agent'6 and suggests a role for such agents possibly by prostaglandin inhibition and/or the inhibition of ornithine decarboxylase in the control of cell cycle abnormalities in FAP patients. If we accept that FAP is a systemic disorder and recognition ofthe genotypic abnormality can be made before the production of adenomas in the colon, intervention may prevent the colonic manifestations and the possibility for perhaps influencing the extra colonic manifestations. Perhaps the most exciting developments in FAP have been in the area of genetics. Since Herrera reported the deletion of part of chromosome 5 in a patient with a polyposis syndrome'7, intense interest has centred on this chromosome. Bodmer reported a DNA marker, CllP1l which showed close linkage to the FAP locus and has shown to be present on the long arm of chromosome 518. Using this marker and five other markers which 'flank' the FAP gene, Dunlop reported a series of 41 asymptomatic individuals from FAP families who were genetically screened19. Twenty-eight were informative for the probes and in these 14 of 15 individuals who were screened by colonic examination or retinal examination and radiology were found to have probe-derived risks of 0.93 or greater. Of these, four had been previously discharged from follow up. A further 14 individuals were assigned risk estimations of less than 0.01 and 16 risk of less than 0.1. Although yet to be validated by such observations, follow up taken together with clinical data should prove to be very useful in counselling members of FAP families and in determining the frequency and duration of screening. One very interesting observation was made by Sasazukri et al. who introduced a single human chromosome 5 into a murine hepatoma cell line (7R.1) which suppressed the tumnorogenicity ofthat cell line in nude mice and suppressed the in vitro growth of a human
carcinoma cell line which showed partial deletion of chromosome 5q20. Such observations indicate that there may be a possibility of correcting the genetic defect in FAP although further work is necessary to show this. Other genetic abnormalities have been shown in tumours in affected individuals with FAP which include frequent allele losses in chromosome 12q, 17p, and 2221. Such findings suggest that changes of more than one chromosome may be necessary for the initiation and progression of tumours in FAP. Thus, studies of the genetic changes in FAP may allow early identiflcation of affected individuals and by implication unaffected individuals. This allows a possibility of altering the genome in order to correct the abnormality or by intervention to delay or prevent the manifestations of the clinical condition. Above all however, important insights may be gained into the genetics of the sporadic type of colorectal cancer which may allow similar advances.
N C Armitage University Hospital, Queen's Medical Centre Nottingham NG7 2UH References 1 Spigelman AD, Granowska M, Phillips RKS. Bile reflux and gastric adenomas: A scintigraphic study in patients with familial adenomatous polyposis (FAP). JR Soc Med 1991;84:476-8 2 Spigelman AD, Williams CB, Phillips RKS. Rectal polyposis as a guide to duodenal polyposis in familial adenomatous polyposis. J R Soc Med 1992;85:77-79 3 Polkinghorne PJ, Ritchie S, Neale K, Schoeppner G, Thomson JPS, Jay BS. Pigmented lesions of the retinal epithelium and familial adenomatous polyposis. Eye 1990;4:216-21 4 Bulow S, Sondergaard JO, Witt I, Larsen E, Tetens G. Mandibular osteomas in familial polyposis coli. Dis Colon Rectum 1984:105-8 5 Perry RE, Christensen MA, Thorson AG, Williams T. Familial polyposis: colon cancer in the absence of rectal polyps. Br J Surg 1989;76:744 6 Spigelman AD, Williams CB, Talbot IC, Domizio P, Phillips RKS. Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet 1989;
i:783-5 7 Hubbard TB. Familial polyposis of the colon: the role ofthe retained rectum after colectomy in children. Am J Surg 1957;23:577-86 8 Madden MV, Neale KF, Nicholls RJ, et al. Comparison of morbidity and function after colectomy with ileo-rectal anastomosis or restorative proctocolectomy for familial adenomatous polyposis. Br J Surg 1991;78:788-92 9 Dozois RR. Surgical aspects offamilial. Int J Colorectal Dis 1988;3:1-16 10 Ramos R, Carrel A, Herrera L. Current treatment of desmoids. In: Herrera L, ed. Familial adenomatous polyposis. New York: Alan Liss, 1990:133-46 11 Bussey HJR, De Cosse JJ, Deschner EE, et al. A randomised trial of ascorbic acid in polyposis coli.
Cancer 1982;50:1434-9 12 De Cosse JJ, Miller HH, Lesser ML. Effect ofwheat fiber and vitamins C and E on rectal polyps in patients with familial adenomatous polyposis. J Natl Cancer Inst 1989;81:1290-7 13 Spigelman AD, Uff CR, Phillips RKS. Vitamin C levels with familial adenomatous polyposis. Br J Surg 1990;77:508-9 14 Fvriedman E, Lipkin M, Winawer S, Buset M, Newmark H. Heterogeneity in the response of familial polyposis epithelial cells and adenomas to increasing levels of calcium in vitro. Cancer 1989;63:2486-91
Journal of the Royal Society of Medicine Volume 85 February 1992 15 Stern HS, Gregoire RC, Kashtan H, Stadler J, Bruce WR. Long-term effects of dietary calcium on risk markers for colon cancer in patients with familial polyposis. Surgery 1990;108:528-33 16 Waddell WR, Ganser GF, Cerise EJ, Loughry RW. Sulindac for polyposis of the colon. Am J Surg 1989; 157:175-9 17 Herrera L, Kataki S, Gibas L, Pietrzak E, Sandberg AA. Gardner syndrome in a man with an interstitial deletion of 5q. Am J Med Genet 1986;25:473-6 18 Bodmer WF, Bailey CJ, Bodmer J, et aL Localisation ofthe gene for familial polyposis coli on chromosome 5. Nature 1987;328:614-16
How to avoid pitfalls in ethnic medical history, examination and diagnosis Transcultural Medicine means the science and art of dealing with patients from different cultures. It is defined as follows: 'Transcultural Medicine is the knowledge of medical and communication encounters between a doctor or health worker of one ethnic group and a patient of another. It embraces the physical, psychological, and social aspects of care as well as the scientific aspects of culture, religion and ethnicity without getting involved in the politics of segregation or integration.'I
This approach may be useful in a cross-cultural contact during a medical consultation as short as 10 minutes or a detailed medical examination lasting an hour. I recognize four realities - patriotism, neocolonialism, racism, and fundamentalism - as political issues of our times, but leave them to other experts who specialize in these fields. A British general practice list or a hospital ward may have patients from two cultures (Western or Eastern), four races (European or Caucasian, African or Negroid, Asian or Asiatic, and Chinese or Mongoloid) and six major religions (Hinduism,
Buddhism, Sikhism, Judaism, Christianity, and Islam). In addition, mnany patients will be: westernized Easterners; children of mixed marriages; and custodians of secular views. It is within a doctor's intellectual grasp to appreciate these categories (without stereotyping) and provide appropriate care. This Editorial deals with a doctor's role in the management of patients from cultures other than his or her own, and it highlights only a few out of many transcultural issues. Every doctor must ask each patient about his or her history of: presenting complaints; story of present illness; summar of past illnesses; menstrual, obstetric, and sexual difficulties; treatment used for present and past illnesses; family patterns of disease; social habits; and occupational hazards. If a doctor and the patient come from the same culture, race and religion, then standard pathological, psychological, and socioeconomical terms will probably be sufficient in making a diagnosis and routine questions should be asked. Where this is not the case, transcultural factors must be considered. In taking history, three cultural
65
19 Dunlop MG, Wyllie AH, Steel CM, Piris J, Evans HJ. Linked DNA markers for presymptomatic diagnosis of familial adenomatous polyposis. Lancet 1991;337: 313-16 20 Sasazuki T, Sasaki M, Sugio K, et al. Molecular and genetic analysis in familial adenomatous polyposis (FAP). Int J Colorectal 1990;5:57 21 Okamoto M, Sato C, Iwama T, Miyaki M. Loss of alleles in patients with familial adenomatous polyposis and colon cancers. In: Herrera L, ed. Familial adenomatous polyposis. New York: Alan Liss, 1990: 383-91
aspects are now described relating to language, the meaning of pain, and the privacy of age and religion. It is important to make sure that the doctor and the patient are talking about the same thing. It is traumatic for the doctor and impending disaster for the patient if an English patient were to be interviewed by a French doctor who could not speak a word of English. Where a language barrier exists, an interpreter or link-person (language and culture 'translater) should be used. Pain of some sort is the commonest complaint which brings a patient to the doctor. It is naive to think that the social education of patients from all ethnic groups is the same as that of the doctor. Pain means different things to different people2. An Oxbridge patient may describe pain as an ache, cramp, hurt, irritation, pang, soreness, spasm, tenderness, throb, trouble, twinge, agony, anguish, bitterness, disquiet, distress, grief, heartache, misery, suffering, torment, torture, and woe. A Navajo (North American Indians who live in Arizona) tourist may be at a loss when seeing an English doctor because the Navajo are the only people in the world who have no word for pain in their language3. The vocabulary about various degrees of pain varies with the age, social class, education and culture of a patient. It is not uncommon for an ethnic minority patient: to be unable to describe the type and severity of pain; to translate wrongly from the mother tongue into English; to repeat the same word due to lack of vocabulary; and to exaggerate the symptom by using a word that a doctor from another culture can understand, so as to attract his attention. The knowledge of medicine is science but its practice is an art. A doctor should remember 'cum Scientia Caritas' and spend more time when taking history from patients from other cultures. An accurate history is a prerequisite of correct diagnosis and appropriate management. English patients are shy of revealing their age and reluctant to discuss their religion or political persuasion because they consider this a part of their personal privacy. An ethnic minority doctor should be very tactful when asking about their ages or religion. However, the reverse is the case among the ethnic minorities. An English doctor should be bold in asking the age or religion when taking the history from an Asian, African, Chinese,, or other such patients because they feel proud to give this information. In general, they live by their religion. Age is an advantage in the East and with advancing years an
0141-0768/92/ 020065-02/$02.00/0 © 1992 The Royal Society of Medicine
