Helping a child to understand her own testicular feminisation

33

11. Clark J, Saxinger C, Gibbs WN, et al. Seroepidemiologic studies of human T-cell leukemia/lymphoma virus type I in Jamaica. Int J Cancer

1985; 36: 37-41. AE, Fang CT, Slamon DJ,

12. Williams

et al. Seroprevalence and epidemiological correlates of HTLV-I infection in U.S. blood donors.

Science 1988; 36: 37-41. 13.

Murphy EL, Figueroa JP, Gibbs WN, et al. Sexual transmission of human T-lymphotropic virus type I. Ann Intern Med 1989; 111: 555-60.

19.

Boulos R, Halsey NA, Holt E, et al HIV-1 in Haitian women 1982-1988. J Acquir Immune Defic Syndr 1990; 3: 721-28. 20. Anderson DW, Epstein JS, Lee TH, et al. Serologic confirmation of human T-lymphotropic virus type I infection in healthy blood and plasma donors. Blood 1989; 74: 2585-91. 21. Griner PF, Mayewski RJ, Mushlin AI, Greenland P. Selection and interpretation of diagnostic tests and procedures. Ann Intern Med 1981; 94: 553-600.

Hartley TM, Khabbaz RF, Cannon RO, Kaplan JE, Lairmore MD. Characterization of antibody reactivity to human T-cell lymphotropic virus types I and II using immunoblot and radioimmunoprecipitation assays. J Clin Microbiol 1990; 28: 646-50. 23. Khabbaz RF, Hartley TM, Lairmore MD, Kaplan JE. Epidemiologic assessment of screening tests for antibody to human T-cell lymphotropic virus type I (HTLV-I). Am J Public Health (in press). 24. Khabbaz RF, Hartel D, Lairmore M, et al. HTLV-II infection in a cohort of New York intravenous drug users: evidence for an old infection. J Infect Dis (in press). 25. Lee H, Swanson P, Shorty J, Zack J, Rosenblatt J, Chen I. High rate of HTLV-II infection in seropositive I.V. drug abusers in New Orleans. Science 1990; 244: 471-75. 22.

14. Okochi K, Sato H, Hinuma Y. A retrospective study on transmission of adult T-cell leukemia virus by blood transfusion: seroconversion in recipients. Vox Sang 1984; 46: 245-53. 15. Quinn TC, Zacarias FRK, St John RK. HIV and HTLV-I infections in the Americas: a regional perspective. Medicine 1989; 68: 189-209. 16. Centers for Disease Control. Licensure of screening tests for antibody to human T-lymphotropic virus type 1. MMWR 1988; 37: 736-47. 17. Rosenblatt JD, Gasson JC, Glaspy J, et al. Relationship between T-cell leukemia virus-I I and atypical hairy cell leukemia: a serologic study of hairy cell leukemia patients. Leukemia 1987; 1: 397-401. 18. Lairmore MD, Jacobson S, Gracia F, et al. Isolation of human T-lymphotropic virus type 2 from Guaymi Indians in Panama. Proc Natl Acad Sci USA (in press).

VIEWPOINT

Helping a child to understand her own testicular feminisation

Children do not think as adults do. They would therefore be less worried than adults are about a diagnosis with serious or ominous implications, yet they are commonly left uninformed until someone judges that they are old enough to understand. For most, this means delivery of painful information during the very vulnerable teenage years. A better approach is to unfold the truth stage by stage, matching simple statements to the child’s conceptual until the personal implications are finally realised as part of a maturing process. Use of this approach for a child with testicular feminisation is described.

growth

Introduction We have over the years come to a deeper understanding of how children think.12 Whereas at one time it was customary to withhold distressing information until a child was old enough to understand it, it is now recognised as more helpful to tell the truth stage by stage as understanding unfolds. This approach is recognised as the best for matters such as adoption, conception through a donoror the diagnosis of a malignant disorder.4 It should also be used in any chronic disorder of early onset, including a chromosomal abnormality such as Turner’s or Kleinfelter’s syndrome. I describe here how it relates to the condition of persistent testicular feminisation.

Case-report The child was the firstborn of healthy parents and weighed kg at birth. Abnormalities of the genitalia (hyper-

2-82

trophied clitoris, prominent labia, and closely approximated vaginal introitus and urethral orifice) were noted at birth. Buccal smear showed no Barr bodies but a Y chromosome. At 9 days, normal male chromosomes were reported. At 2 weeks, examination under anaesthetic showed a moderate phallus, a female urethra with no verumontanum, and a normal vagina with a cervix. Laparotomy at 3 weeks of age showed a normal uterus with bilateral fallopian tubes and abnormal gonads. A biopsy sample showed cryptorchidism as in testicular feminisation. After discussion, the parents, paediatric surgeon, and clinical geneticist agreed that the child should be raised as a girl. Phallidectomy and excision of the gonads were carried out when she was 17 months old. By then she had a normal 6-month-old sister. Paediatric follow-up was arranged.

Subsequent developments When the child was 2 years 9 months, her mother mentioned that she had been told to withhold the truth about the child’s condition. Advice from other senior experienced doctors was that this was generally the correct ADDRESS

Paediatric Department, North Staffordshire Hospital Centre, Stoke-on-Trent, UK (J Goodall, FRCPE). Correspondence to Dr Goodall at Melton, Burrington Drive, Trentham, Stoke-on-Trent ST4 8SP, UK

34

but that "later" the child would need to know about her abnormal genitalia and future infertility. Current paediatric experience, however, suggested that a child should be helped to understand at each developmental level facts that could be built on later. She was clearly an intelligent, though timid, child so her mother was first advised simply to tell her that she had needed an operation when she was a baby and to indicate its site, but to attempt no further explanation yet. Follow-up was resumed a few years later, the unplanned lapse being due to the child’s course

timidity. When the child was nearly 8, her sister noticed differences in their genitalia, but both childen accepted without further curiosity the explanation about a past operation. The parents were now advised to take opportunities to mention casually that not everyone has children of their own. When the child was 9, a diagram was used to explain the names of perineal orifices and the function of urethra and anus. Internal anatomy and menstruation were not described. At about 11 years old the introduction of replacement hormones was explained as some people’s needing such treatment to help to change shape. Since her younger sister was already showing signs of early puberty, treatment of the patient was introduced to give her the lead. At this time, an excellent programme was shown on BBC television. It described diagrammatically and clearly how the condition of persistent testicular feminisation arises and also interviewed an elderly woman who had the disorder but had led a normal married life and adopted two children. It took time to trace and obtain a copy of the film (Body Matters, April, 1987). It was shown first to her parents, who approved of its clarity and helpfulness. When she was 12 years old the young girl watched the film twice with her mother and paediatrician. By now, her understanding of it was mature enough for her to express appreciation and enlightment. She now realised that all embryos begin as girls in habitus, despite chromosomal make-up, but whereas her chromosomes should have dictated masculinisation she had actually stayed as a girl. Thus, her "building blocks" carried a different label (XY) from her bodily configuration (XX). By now, hormonal treatment was producing early breast development, but not, as yet, menstruation. Her only question was how long her treatment would last. Although her mind was not yet ranging so far ahead, opportunity was taken to reinforce the happy experience of the woman seen on the film. Follow-up continues.

Discussion

Occasionally, we read in the press of some young athlete find that her chromosomes are male when her lifelong image has been totally female. Such a painful crisis of identity is likely to cause severe emotional difficulties. If such information is available in babyhood, it seems kinder to give it to the child gradually and so prevent her building her life on a total misconception. We have had a lot to learn about the way in which children’s minds develop and their concepts unfold. Under 2 years old, the approach to life is totally egocentric, and a child is unable to think in other peoples’ terms. From then until just over 6 years, classification of things that match each other means that differences between one child and another are not made much of. Children are more involved in their own internal world than in making more than superficially disparaging comparisons, such as something’s

shocked

to

being bigger or smaller than is desirable. In any case, when young they would not worry about being in a minority group, egocentricity usually being a self-affirming state of mind. As

matching skills advance, recognisable differences register and slowly evoke comment. A 3-year-old may be able to copy the drawing of a face, but only later chooses to alter the facial expression spontaneously. Although intelligence, stimulation by older siblings, or being very experienced can all speed up the process, 6-7 years is about the age at which average children start making deductions and sometimes faulty conclusions. This was the age at which the two sisters commented on the difference in their genitalia. The ability to draw other parts of the body

accurately develops gradually; the "draw-a-man" test’ is one test used to gauge conceptual development according to the details portrayed. Between 7 and 11years, the average mind starts to range a little more but is still not thinking in adult terms. Deductive ability improves and the making of correct connections is now possible. As the child described here showed, a diagram can readily be transposed in imagination to the external features of one’s own body; internal imagery generally starts towards the end of this age range and even then can be very rudimentary.6 Many adults have no clear concept of internal anatomy. The matching idea of "shaping up" like the other girls, even though used with the strange phrase "hormonal replacement", aided the concept of being like everybody else, so important in pre-teens as well as to teenagers. Familiarity with a medical term was useful, though understanding would come later. By the time that the illustrative film actually arrived, the girl was almost at the perfect age for viewing it. Looking back into embryological life and imagining the body’s internal schema was now comprehensible with visual aids, but would have been difficult without them. Her forward ranging question about the length of treatment was again a mark of her age group, mental gymnastics having now been acquired. By now, too, she could understand that the removal of her gonads (sources of hormones) meant that she had to continue to replace that loss. In any long-standing disorder this pattern of feeding information should be followed. It could be helpful, to both patient and doctor, to have support from a child psychiatrist or clinical psychologist. Children do not view their troubles as adults do; attempts to protect them from adult knowledge may not only leave them vulnerable to a shocking revelation at exactly the age when conformity with peers and sexual identity are important, but could also breed non-compliance with treatment. Characteristically, non-compliance happens at two stages in a child’s life. The first of these is at about 6-8 years, when a child starts to recognise that "no one else has to take this stuff, so why should I?" It may also happen in the mid-teens, when peer group conformity takes precedence over parental insistence on doing certain things, among which may come the taking of treatment. If the child has grasped while younger that treatment is essential for the maintenance of personal health rather than being some perverse adult whim, as an adolescent he or she can take much more responsibility for it. The child described here will not be without difficulties. In her late teens she may have an emotional crisis of identity, but it should be less severe than if she had been offered shattering new information at that age without preparation.

35

In later adolescence, awareness of her state may help her to understand some of her own attitudes. She remains a rather shy and sensitive person, intelligent and with a love of reading. Fortunately, athletics is not her favourite pastime; she may go on to higher education which, for many women, means less full-time preoccupation with affairs of the home and family than may be so for others. Her family have been very supportive throughout. Her sister is curious about the pubertal changes after treatment but has so far been told simply that some people have to take tablets to help them change shape. Hormonal replacement is already such a well-known phrase that should her school peers hear of it they may feel it beneath their sophistication to ask exactly what it means. The decision about whom the girl tells about her

chromosomal

do so, will be hers older people who understand and will support her when that time comes.

M. Shingleton and W. Glenn Hurt. New Churchill Livingstone. 1990. Pp 520. 60. ISBN 0-443086419.

Edited by

Hugh York/Edinburgh:

emphasis on in-vitro fertilisation and prenatal diagnostic techniques, it is refreshing to find that this beautifully produced book is devoted to the less fashionable problems of women who have completed their families, and who are perimenopausal or postmenopausal. The fifteen contributing authors come from Richmond, Virginia, or Birmingham, Alabama, and reflect local views. Indeed, there are whole chapters devoted to subjects rarely considered in depth by gynaecologists this side of the recent

Atlantic. The book starts with an introduction to the biology of aging and a review of the physical and mental health of menopausal and postmenopausal women, which includes a discussion of exercise, weight control, osteoporosis, and nutrition, with a useful table of potential drug-nutrient interactions for commonly used drugs. A chapter on outpatient surveillance describes the commoner causes of death and suffering, with current US screening recommendations for women aged 40 years and older, including the frequency of mammography, faecal occult blood tests, and serum cholesterol measurements. This all-round approach is supported by another chapter on the gynaecologist as primary care physician, in which commoner medical diseases are discussed-but only in sufficient detail to serve as an introduction to each subject. European readers may be struck by the apparent life-long attachment of many American women to the obstetrician who delivered their babies-but perhaps this seems a small compensation for the costs of US obstetric malpractice

insurance. Considerable attention is paid

to

are

‘

1. Beadle M. A child’s mind. London: Methuen, 1972. 2. Goodall J. How children think. J R Soc Health 1987; 107: 224-26. 3. Brandon J, Warner J. AID and adoption: some comparisons. Br J Soc Work 1977, 7: 335-41. 4. Spinetta JJ. Disease related communication: how to tell. In: Kellerman J, ed. Psychological aspects of childhood cancer. Illinois: G. C. Thomas, 1980. 5. Goodenough FL, Harris DB. Goodenough-Harris drawing test. New York: Harcourt Brace Jovanovich Inc, 1963. 6. Eiser C, Patterson D. "Slugs & snails and puppy-dog tails"—children’s ideas about the inside of their bodies. Child Care Health Dev 1983; 9: 233-40.

is overlooked. Three different the difficult and perhaps increasingly approaches common problem of vault prolapse are then discussed: abdominal culdoplasty is recommended to prevent vault prolapse, and the technique is beautifully illustrated. Investigation of dysfunctional and postmenopausal bleeding is dealt with in a fairly standard manner and hysteroscopy is recommended as an office procedure. There is also clear advice as to how far gynaecologists should go in the management of malignant disease, and when to call on an oncologist, and an authoritative review on breast diseases, particularly breast cancer. However, despite the statement that "the gynecologist should be comfortable in dissecting about the ureter and vessels within the retroperitoneal space", there is little detailed advice on what has to be done when the ureter is damaged and even less when there is injury of the iliac vessels. A lot of information is provided in over 550 pages of double columns, with clear print, helpful tables, and figures and drawings that illustrate the text well—except in the chapter on breast diseases, where the drawings are not up to the high standards elsewhere. All the chapters have up-to-date and useful references. Although largely a reflection of North American gynaecological practice, the authors’ refreshing approach to gynaecological problems, particularly the common disorders that bring so many women to outpatient departments, will be of much wider interest.

repair

to

Postreproductive Gynecology

Amid

and when

REFERENCES

Manchester

BOOKSHELF

make-up,

alone, but she knows that there

to

benign gynaecological

conditions and urinary disorders. In the chapter devoted to operations for stress incontinence the Burch colposuspension is recommended, but the MarshallMarchetti-Kranz procedure is not mentioned. Similarly, when surgical treatment of prolapse is discussed the

Department of Obstetrics and Gynaecology, New Cross Hospital, Wolverhampton WV10 OQP, U K

Diabetes: Clinical

ALAN M. SMITH

Management

by Robert B. Tattersall and Edwin A. M. Gale. Edinburgh: Churchill Livingstone. 1990. Pp 382. C37.50. Edited

ISBN 0-443022739.

In their preface the editors, quite rightly, express apprehension as to the need for yet another volume on the management of diabetes. Although one cannot help but share their sentiment, it quickly becomes apparent that this book is different: a very refreshing approach to the subject

makes it years. The

one

of the best such manuals

to

emerge for many

strength of the book is its practical approach to the management of diabetes: it is written by clinicians -for