A Case Report of Usher’s Syndrome andAnorexia Nervosa

Victoria Chapman,1 Anton Valmana,1 and J. Hubert Lacey1*

1 Department of General Psychiatry, St George’s Hospital Medical School,London, England

Accepted 5 August 1996

Abstract: Objective: This report describes the rare combination of anorexia nervosa, deaf-ness, and visual impairment with a particular emphasis on management issues. To the au-thor’s knowledge, this is the first report of an eating disorder in a patient who is deaf with avisual impairment. Discussion: It describes the history of such a patient and the difficultiesencountered in her treatment. © 1998 by John Wiley & Sons, Inc. Int J Eat Disord 23:223–226, 1998.

Key words: anorexia nervosa; deafness; visual impairment

INTRODUCTION

This case report describes a woman who suffers from Usher’s syndrome and anorexianervosa. Usher’s syndrome is an autosomal recessive condition resulting in congenitaldeafness and retinitis pigmentosa. An association between eating disorders and deafnesshas been reported by De Leo and Santonastaso (1987), and there have also been reportslinking anorexia with blindness (Vandereycken, 1986). De Leo and Santonastaso (1987)suggest that prelingual deafness gives rise to communication difficulties which compro-mise language development and the potential to form interpersonal relationships, both ofwhich may predispose to an eating disorder. Vandereycken (1986) suggests that visualimpairment may accentuate a series of psychosocial problems which contribute to thedevelopment of anorexia nervosa. Deafness and limited communication skills pose sig-nificant problems with regard to the management of anorexia nervosa (Touyz, O’Sullivan,& Beaumont, 1994), and the added complication of deteriorating sight raises furthermanagement difficulties.

*Correspondence to: Professor J. H. Lacey, Department of General Psychiatry, St. George’s Hospital MedicalSchool, Cranmer Terrace, London SW17 ØRE, U.K.

© 1998 by John Wiley & Sons, Inc. CCC 0276-3478/98/020223-04

Prod. #1245

CASE REPORT

A 35-year-old woman with Usher’s syndrome (congenital deafness and retinitis pig-mentosa) and a 19-year history of severe food restriction, laxative abuse, and vomitingwas admitted to our eating disorders unit. She had an overvalued idea about the size ofher abdomen and thighs, and a phobia of normal weight. She had experienced longperiods of amenorrhea.

Her preoccupation with body image began at the age of 9 when she was teased at schoolfor being plump. She started restricting her food intake in her early teens and was finallydiagnosed with anorexia nervosa at age 16 years. Her first admission to a psychiatric unitwas at the age of 18 years and since then she has had 14 further hospital admissions.Throughout these years, her weight was maintained below 53 kg. She was diagnosed withUsher’s syndrome at the age of 21 years when her sight began to deteriorate. She currentlysuffers from a bilateral hemianopia, which is likely to get worse.

She was born in Ghana to a Ghanaian mother and a black South African father, thesecond of six children. Her oldest sibling died at 9 months from an unknown cause. Shehas one sister with Usher’s and a brother with schizophrenia. She was found to be deafat age 3 years and it was then that her parents brought her to England for specializededucation. From the ages of 5 to 16 years, she attended residential schools for the deafwhere she was above average academically. She described this period of her life as veryunhappy as she found it difficult to adjust to English culture and to cope with longseparations from her parents. A further cause for her unhappiness was that she wassexually molested by the 16-year-old son of her foster mother on several occasions in herearly teens.

She described her relationship with her family as difficult. She was not able to tell themhow unhappy she was at the school and cited communication difficulties within herfamily as a reason. No member of her family could use sign language, which led tofrequent misunderstandings. She described herself as a person with a strong need toachieve and throughout her life made friends only with people who are not deaf, manyof whom were carers she met through hospital admissions. She acknowledged feelingisolated within the deaf community and identifying better with the hearing world forreasons she found difficult to explain. She has lived alone for many years and has not beenable to complete her studies, mainly because of her frequent hospital admissions. Unsuc-cessful attempts have been made to encourage social contact using specialized education-al schemes and support groups for the deaf and people with Usher’s syndrome.

Management

The patient was admitted to the unit at a weight of 37.3 kg with a body mass index(BMI) of 14.3. She was initially placed on bedrest and started on a refeeding program. Themajor challenge encountered by the therapeutic team was the difficulty with communi-cation. At first, written notes were used which were time-consuming and complicated bythe patient’s additional visual problems. These difficulties were overcome by the use of asign language interpreter who contributed to the formation of a better therapeutic rela-tionship between patient and staff. However, she preferred the use of the written word asthe main method of communication as she felt the subtleties of her emotions were lostduring their translation into sign language.

224 Chapman, Valmana, and Lacey

The patient suffered from deteriorating tunnel vision and had been warned by special-ists that there may be a time when she could lose her sight completely. At the time oftreatment, she was able to see and communicate only with people directly in front of her.Communication within groups was very difficult. As a result, it was hard for her tointegrate into the ward milieu. A further aspect of her communication difficulties was thatshe somatized her anxieties, especially with regard to food so that at meal times shewould often throw herself to the ground, sometimes putting herself in danger. The staffhelped her by encouraging her to use written words to express her feelings.

During her treatment she received a range of therapies which included individual, art,family, and occupational therapy. In the individual therapy sessions, it was evident thatshe had always found it hard to accept that she was deaf and she had tried only tobefriend people from the hearing world. She also had great difficulty in accepting that hereyesight was deteriorating and these issues were worked through in therapy. In arttherapy, she was able to paint and talk about the work through the help of an interpreter.However, special care was taken so that she could see and talk about the work of othergroup members. Family therapy focused on the difficulties the family had in accepting thepatient’s disabilities and how they could communicate these feelings to each other. Oc-cupational therapy highlighted the problem areas of food management, safety, and socialisolation.

Although the basic principles of treatment of anorexia nervosa were applied, the pro-gram needed to be modified in a number of ways to adapt itself to this highly complexcase. These included individual occupational therapy sessions, the use of a psychothera-pist who could use sign language, and the exclusion from group therapy as it was felt thepresence of an interpreter would disturb the dynamics of the group. Efforts were made bystaff to integrate the patient into the unit by involving her in ward activities and makingnotes for the meetings. However, this was not fully achieved because some of her fellowpatients found it difficult to accept that she was being treated in a special way. Sheremained isolated and was unable to make friends.

She reached target weight after 14 weeks and commenced on the maintenance weightprogram for a further 2 months prior to discharge. During this time her anxieties anddenial about her disabilities, her continuing communication difficulties, and subsequentisolation needed to be addressed if she was not to relapse. There was also concern forher tendency to put herself in danger by walking across the roads in front of cars. Shewas encouraged to talk about these issues in individual therapy, to make contact withsupport groups, and to seek help with her disabilities, e.g. learning Braille and using acane.

Outcome

The patient spent a total of 25 weeks as an inpatient and, compared with her 15previous admissions, this stay was of average length. Since discharge over 1 year ago,she has been attending the outpatient program, maintaining her target weight, and livingindependently. She is no longer experiencing amenorrhea and regularly sees a specialistnurse for general support and food management. She continues to see her psychothera-pist and has a good relationship with the rehabilitation officer who helps with practicalissues. She has contact with her family, attends an Usher’s group, and is becoming lessisolated.

Usher’s Syndrome 225

DISCUSSION

This patient demonstrated an intellectual, emotional, and cultural isolation from thedeaf world and an inability to integrate herself fully into the hearing world. This isolationwas in fact due to her own denial of her deafness and her family’s difficulty in acceptingit. She was unable to express these conflicts through speech so her anorexia nervosa gaveher a sense of achievement which her personality required and a way of gaining controlover these emotions.

This case raises specific issues about managing an anorectic patient with communica-tion difficulties and chronic disabilities. First, the importance of a therapeutic relationshipin the treatment of anorexia has been described by Crisp (1980). This was found hard toachieve, but staff managed this by writing notes and using an interpreter and their ownpersistence. However, her full integration into the unit was more difficult to achieve. Thiswas partly the result of the patient’s own tendency to isolate herself, thus not formingrelationships, and partly the difficulty the other patients experienced in accepting a per-son whom they saw as special and treated differently by staff.

Second, the anxieties of the staff towards the treatment of a disabled patient wererelevant. These anxieties involved concern about the poor progress of treatment, theirability to communicate with the patient, and the belief that anorexia nervosa may be theonly solution for someone who is deaf and may lose her sight. These issues were dis-cussed in peer-support groups which helped to prevent demoralization.

Finally, the practical difficulties in treating a patient with chronic disabilities weresurmounted by adapting the treatment program to the patient. This involved giving thepatient more time individually, not only in the psychotherapies but in meal planning andcooking. Also important were the use of a sign language interpreter based on the ward,a psychotherapist who could sign, and liaison with a rehabilitation officer for the deaf.The patient’s tendency to put herself in danger may represent her desire for autonomy,her impulsive nature, or her need to raise anxieties. These issues are being workedthrough with the development of a trusting and supportive relationship with a carer inthe community.

In the treatment of any patient with anorexia nervosa, specific attention must be paidto the etiology and maintaining factors within the illness (Crisp, Kalucy, Lacey, & Har-ding, 1977). In the case of a patient in whom anorexia nervosa has developed from chronicdisabilities, it is especially relevant. Bemporad, Hoffman, and Herzog (1989) have foundthat anorexia and blindness have a particularly poor prognosis. With the added compli-cation of deafness or any other chronic disability, it is important to focus on the factorswhich contribute to a poor prognosis.

REFERENCES

Bemporad, J. R., Hoffman, D., & Herzog, D. B. (1989). Anorexia nervosa in the congenitally blind. Three trialconsiderations. Journal of the American Academy of Psychoanalysis, 17(1), 89–101.

Crisp, A. H. (1980). Let me be. In Anorexia nervosa (pp. 95–146). London: Academic Press.Crisp, A. H., Kalucy, R. S., Lacey, J. H., & Harding, B. (1977). The long-term prognosis in anorexia nervosa: Some

factors predictive of outcome. In R. A. Vigersky (Ed.), Anorexia nervosa (pp. 55–65). New York: Raven Press.De Leo, D., & Santonastaso, P. (1987). Anorexia nervosa in a prelingually deaf young man. A case report.

International Journal of Eating Disorders, 6, 317–320.Touyz, S., O’Sullivan, B., & Beaumont, P. J. V. (1994). Anorexia nervosa in an adolescent with early profound

deafness. International Journal of Eating Disorders, 16, 411–415.Vandereycken, W. (1986). Anorexia nervosa and visual impairment. Comprehensive Psychiatry, 27, 545–548.

226 Chapman, Valmana, and Lacey