2
and papers and in this field the recent review by Rever and Dunbar (Revere and Dunbar, 2001) provides a more academic and systematic review of the different types of tailoring. Nevertheless, this is a good book and well worth getting for anyone working in the field. It would be particu- larly useful for students or new researchers starting out in this interesting and developing field. R. B. Jones, D. Bental A. Cawsey, J. Pearson and J. Marsden Department of Public Health, University of Glasgow, Glasgow, Scotland, UK REFERENCES Kreuter M, Farrell D, Olevitch L, Brennan L. Tailoring Health Messages. Customizing Communication With Computer Technology. Lawrence Erlbaum Associates: New Jersey, London. Pearson JD, Jones RB, Cawsey A, Barrett A. 2001. Do clinicians ‘tailor’ information for patients with cancer and if so do their methods provide guidance for computer-based personalisation? (Abstract). Health- care Computing. BJHC Books: Weybridge, Surrey; 229–230. Revere D, Dunbar PJ. Review of computer-generated health behavior interventions. J Am Med Inform Assoc 2001; 8: 62–79. Beyond a Shadow of Doubt: Assessing the Psycho- logical Impact of Predictive Genetic Testing for Multiple Endocrine Neoplasia Type 2. Edited by Grosfield, F., University Medical Center, Utrecht Multiple endocrine neoplasia, type 2 (MEN2) is one of the first hereditary cancer syndromes for which genetic testing is available and it is one in which the medical benefits of testing are most clearly apparent. In 1993, direct mutation testing was introduced for the RET gene, an autosomal dominant gene responsible for the development of MEN2. MEN2 is characterized by increased risk of medullary thyroid cancer and by over-stimula- tion of the adrenal glands due to the presence of tumors called pheochromocytomas. Prior to this time, it had been recommended that at-risk individuals undergo repeated biochemical screen- ing for calcitonin and pentagastrin to detect changes suggestive of the presence of medullary thyroid cancer. This screening was both unplea- sant and, at times, inaccurate, since micro-carci- nomas were frequently undetectable. Because the age of onset of MEN2 is typically before age 30, biochemical screening was typically offered to individuals from age 5 to 35 in the Netherlands. Medullary thyroid cancer does not cause symp- toms in the early stages. Hence, thyroid cancer was not always found by these methods before it had metastisized, portending a significantly shortened lifespan. When medullary thyroid cancer is de- tected prior to metastasis, surgical removal of the thyroid is recommended. The physical conse- quences of such surgery are considered relatively minor. Surgery, followed by taking synthetic thyroid hormone and continuing to watch for symptoms of adrenal over-stimulation, often allows for normal survival. Genetic testing for mutations in the RET gene allows for earlier and more accurate determination of who is at risk for MEN2. Presumably, early identification of those chil- dren and adults who would be likely to develop thyroid cancer can lead to surgery prior to meta- stasis or possibly even before cancerous changes occur in the thyroid. MEN2, then represents a rather unusual genetic cancer syndrome in that (a) children are at risk for developing malignancy and thus are likely to undergo genetic testing, and (b) there is a surgical treatment which is recommended if the tested child or adult is found to be a mutation carrier. As such, despite the rarity of the syndrome (there are 1000 affected individuals in the Netherlands), study of the psychological consequences of RET genetic testing for children or adults undergoing testing stands to contribute significantly to our knowledge about the impact of this new technology. This occurs in the nearly ideal circumstance where testing stands to eliminate unpleasant screening for the 50% who are not gene carriers and to offer immediate, potentially life-saving, treatment to those who are found to be carriers. In this compilation of six papers which comprise this book, Frans Grosfeld and his colleagues at the University of Utrecht offer reports on the psycho- logical status of 83 adults or adolescents (16 or over) applying for RET testing and 47 parents applying for the testing of one or more of their children. Psychological functioning at the time of application for testing, 2 weeks after genetic testing results disclosure and 1 year after disclo- BOOK REVIEWS 543 Copyright # 2002 John Wiley & Sons, Ltd. Psycho-Oncology 11: 542–545 (2002)

Beyond a Shadow of Doubt: Assessing the Psycho-logical Impact of Predictive Genetic Testing for Multiple Endocrine Neoplasia Type 2. Edited by Grosfield, F., University Medical Center,

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Page 1: Beyond a Shadow of Doubt: Assessing the Psycho-logical Impact of Predictive Genetic Testing for Multiple Endocrine Neoplasia Type 2. Edited by Grosfield, F., University Medical Center,

and papers and in this field the recent review byRever and Dunbar (Revere and Dunbar, 2001)provides a more academic and systematic reviewof the different types of tailoring. Nevertheless,this is a good book and well worth getting foranyone working in the field. It would be particu-larly useful for students or new researchers startingout in this interesting and developing field.

R. B. Jones, D. BentalA. Cawsey, J. Pearson

and J. MarsdenDepartment of Public Health,

University of Glasgow, Glasgow, Scotland, UK

REFERENCES

Kreuter M, Farrell D, Olevitch L, Brennan L. TailoringHealth Messages. Customizing Communication WithComputer Technology. Lawrence Erlbaum Associates:New Jersey, London.

Pearson JD, Jones RB, Cawsey A, Barrett A. 2001. Doclinicians ‘tailor’ information for patients with cancerand if so do their methods provide guidance forcomputer-based personalisation? (Abstract). Health-care Computing. BJHC Books: Weybridge, Surrey;229–230.

Revere D, Dunbar PJ. Review of computer-generatedhealth behavior interventions. J Am Med InformAssoc 2001; 8: 62–79.

Beyond a Shadow of Doubt: Assessing the Psycho-logical Impact of Predictive Genetic Testing forMultiple Endocrine Neoplasia Type 2. Edited byGrosfield, F., University Medical Center, Utrecht

Multiple endocrine neoplasia, type 2 (MEN2) isone of the first hereditary cancer syndromes forwhich genetic testing is available and it is one inwhich the medical benefits of testing are mostclearly apparent. In 1993, direct mutation testingwas introduced for the RET gene, an autosomaldominant gene responsible for the development ofMEN2. MEN2 is characterized by increased riskof medullary thyroid cancer and by over-stimula-tion of the adrenal glands due to the presence oftumors called pheochromocytomas. Prior to thistime, it had been recommended that at-riskindividuals undergo repeated biochemical screen-ing for calcitonin and pentagastrin to detectchanges suggestive of the presence of medullarythyroid cancer. This screening was both unplea-sant and, at times, inaccurate, since micro-carci-nomas were frequently undetectable. Because theage of onset of MEN2 is typically before age 30,biochemical screening was typically offered toindividuals from age 5 to 35 in the Netherlands.Medullary thyroid cancer does not cause symp-toms in the early stages. Hence, thyroid cancer wasnot always found by these methods before it hadmetastisized, portending a significantly shortenedlifespan. When medullary thyroid cancer is de-tected prior to metastasis, surgical removal of thethyroid is recommended. The physical conse-quences of such surgery are considered relativelyminor. Surgery, followed by taking syntheticthyroid hormone and continuing to watch for

symptoms of adrenal over-stimulation, oftenallows for normal survival. Genetic testing formutations in the RET gene allows for earlier andmore accurate determination of who is at risk forMEN2.

Presumably, early identification of those chil-dren and adults who would be likely to developthyroid cancer can lead to surgery prior to meta-stasis or possibly even before cancerous changesoccur in the thyroid. MEN2, then represents arather unusual genetic cancer syndrome in that (a)children are at risk for developing malignancy andthus are likely to undergo genetic testing, and (b)there is a surgical treatment which is recommendedif the tested child or adult is found to be amutation carrier. As such, despite the rarity of thesyndrome (there are 1000 affected individuals inthe Netherlands), study of the psychologicalconsequences of RET genetic testing for childrenor adults undergoing testing stands to contributesignificantly to our knowledge about the impact ofthis new technology. This occurs in the nearly idealcircumstance where testing stands to eliminateunpleasant screening for the 50% who are notgene carriers and to offer immediate, potentiallylife-saving, treatment to those who are found to becarriers.

In this compilation of six papers which comprisethis book, Frans Grosfeld and his colleagues at theUniversity of Utrecht offer reports on the psycho-logical status of 83 adults or adolescents (16 orover) applying for RET testing and 47 parentsapplying for the testing of one or more of theirchildren. Psychological functioning at the time ofapplication for testing, 2 weeks after genetictesting results disclosure and 1 year after disclo-

BOOK REVIEWS 543

Copyright # 2002 John Wiley & Sons, Ltd. Psycho-Oncology 11: 542–545 (2002)

Page 2: Beyond a Shadow of Doubt: Assessing the Psycho-logical Impact of Predictive Genetic Testing for Multiple Endocrine Neoplasia Type 2. Edited by Grosfield, F., University Medical Center,

sure was measured using a number of standardpsychological measures and in-person interviews.Additional subjects for the research were 66 adults(56 spouses and 10 parents) accompanying thetested adult/adolescent.

A very intelligent, organized sense pervadesthese reports of outcomes. The questions to beanswered are clearly stated, the literature review isthorough and relevant, and the measures utilizedare justified by an attempt to reveal both obviousand more subtle levels of emotional and familialimpact. The writing is generally very clear and attimes, poetic (as in the observation that genetictesting is perceived by some at-risk individuals andparents as an ‘inescapable offer’). Some additionalEnglish editing might have eliminated the in-clusion of a few, clearly mis-translated words.Limitations to the study are clearly stated, includ-ing the small absolute number of participants ineach group (the authors believe, however,that their study included one-third of healthyindividuals at risk for MEN2 in the Netherlands).While not unduly ‘‘psychological’’, some psycho-dynamic interpretation is offered of findings which,at first, appear illogical. For example, in attempt-ing to explain why 20% of non-carriers continueendocrinological testing after genetic testing, theauthors suggest that their supposed mistrust of thegenetic test result could be a way of undoing thefamily separation effected by the receipt of positivetest results by some relatives and negative resultsby others. The book describes well the complexityof the possible outcomes, as in the finding thatparents who have several children tested one ormore of whom is found to be a carrier and one ormore of whom is not, are more distressed.

The authors emphasize the need for moreextensive counseling of all parents, adolescentpatients, and accompanying persons who havespecial concerns. They recognize that new ap-proaches to the treatment of disease result inconfusion, discomfort, and distress for thosetrying to make sense of the information provided.They found that parents were hesitant to act on

the results of a positive genetic test; 30% ofchildren testing positive had not had their thyroidremoved 1 year following disclosure of theirgenetic status. This series of articles does well toreport not only both the large trends (e.g. a trendtowards reduced distress between 2 weeks and 1year following disclosure), but also to illustrate therange of responses, including the fact that 52% ofadult and adolescent carriers had a high score onone or more measures of distress 2 weeks afterdisclosure. Quite specific recommendations aboutthe nature of counseling to reduce distress areoffered. In addition, a brochure which the authorsprepared for use with at-risk families is reprintedin translation in the appendix. Thoughtful, cogentobservations are offered about some topics whichhave been overlooked in much previous work inthis area. For example, the impact of time oncurrent findings is highlighted in this commentwhich follows a listing of risk factors for emotionaldistress following disclosure, ‘Today’s declinersmay be tomorrow’s applicants. Whether thesesame risk factors apply to persons who are moreambivalent about their desire to be tested needs tobe clarified.’

This book can serve as a valuable introductionto the psychosocial aspects of genetic testing forMEN2 as well as offering a thoughtful templatefor many considerations regarding the genetictesting of children. It is somewhat regrettablethat the chapters are the direct reprints ofpublished articles, as this mandates the repeatedreading of the experimental design. This is,however, a relatively small price to pay for thepsychological incisiveness which the authors con-vey in their close inspection of individual andfamilial outcomes of genetic testing for predis-position to MEN2.

Andrea Farkas PatenaudeDana-Farber Cancer Institute,

Boston, USA

DOI: 10.1002/pon.610

Understanding Clinical Papers. Edited by D.Bowers, A. House and D. Owens. Wiley, Chiche-ster, 2001. £19.99, 202pp. ISBN 047148976X

The title of this book is immediately appealing,given that it is something that all of us will have

tried to do at some time in our careers (withvarying degrees of success!). The appeal of thebook is the highly readable style and structure andthe carefully chosen content. It systematicallycovers the main components that are to be foundin clinical papers in a clear and succinct manner.

BOOK REVIEWS544

Copyright # 2002 John Wiley & Sons, Ltd. Psycho-Oncology 11: 542–545 (2002)