The birth of a child with a congenital anomaly : some psychosocial implications for the family

THE BIRTH OF A CHILD WITH A CONGENITAL ANOMALY: SOME PSYCHOSOCIAL IMPLICATIONS

FOR THE FAMILY

by

BLANCHE DE WET

Dissertation presented for the Degree of Doctor in Philosophy at the University of Stellenbosch

Promotor: Professor J.J. de Villiers

ACKNOWLEDGEMENT

My sincere appreciation to the parents of patients for their open and courageous sharing of experiences with the researcher in order to make the present study possible. It is with pleasure that I record their generous assistance.

To my promotor, Prof J.J. de Villiers my thanks for practical advice on the logical sequence of presenting research findings and for making his extensive experience in this field available to me. The co-promotor, Prof Cywes, not only suggested the present study, but greatly influenced my thinking through both his positive concern for patients and their parents, as well as through his constant attitude of empathy towards them. At the same time his easy accessibility and regular advice was indispensible and very highly valued.

The assistance of my colleague Lesley Henley, by means of 'constructive criticism and pertinent questions largely facilitated pragmatic thinking in the formulating of recommendations based on the findings of the study and is acknowledged with much appreciation.

Without the financial support of the Dorothy de Villiers and University of Stellenbosch bursaries, the study would not have been possible. Both are gratefully acknowledged.

My thanks are due to Loretta Jakubiec and Jenny Altschuler for line drawings and to Mr G Knight for assisting with statistics. I am indebted to Rita Hebler for so ably typing the manuscript.

CHAPTER 1

INTRODUCTION

~A. INTRODUCTION

...

...

C. METHODS

...

1. The choice of a research design 5

Literature search and pilot study

The study sample

Demarcation of the study •••••••••••••••••••••• A conceptual framework for studying the family

of the child with a congenital anomaly •••••• 10

14 15 16 16 17 19 20 22 23

.

.

Selection of the sample

Representativeness of the sample

Sample bias .

Geographical distribution of the sample ••

(a) (b) (c) (d) Retrospective study Interviews 2. 3. 4. 5. 6. 7.

D. PROBLEMS EXPERIENCED IN THE STUDY 24

E. DEFINITION OF TERMS 25

(ii)

CHAPTER 2

THE CHILD WITH CYSTIC FIBROSIS AND HIS FAMILY: A LITERATURE STUDY

A. CYSTIC FIBROSIS: THE DISEASE

...

B. PSYCOSOCIAL ASPECTS OF CYSTIC FIBROSIS

.

.

1:

3. Parental response to cystic fibrosis... 40

(a) The pre-diagnostic stage ••••••••••••••••••• 40

(b) The confrontational stage •••••••••••••••••• 41 (c) The long-term adaptational stage ••••••••••• 42

--j{ 4.

5.

Family functioning and cystic fibrosis •••••••••

Genetic issues in cystic fibrosis .•••••••••••••

46

48

_j 6. Impact on the siblings of patients with cystic

7.

fibrosis

The impact of cystic fibrosis on the patient •••

50

52

8. Mourning the death of a child with cystic

fibrosis .

C. SUMMARY

...

56

(iii)

CHAPTER 3

THE CHILD WITH A CONGENITAL ANOMALY AND HIS FAMILY: A LITERATURE STUDY OF OESOPHAGEAL ATRESIA, HIRSCHSPRUNG'S DISEASE AND ANORECTAL MALFORMATIONS

A. OESOPHAGEAL ATRESIA: THE ANOMALy •••••••••••••••••

1. Aetiology .

2. Clinical features .

3. Treatment .

4. Prognosis .

5. Psychosocial aspects of oesophageal atresia

58 59 59 61 62 63

Psychosocial aspects of Hirschsprung's disease. HIRSCHSPRUNG'S DISEASE

Incidence and aetiology Clinical features 67 67 68 68 69 70

...

.

.

·

.

·

.

4.

Psychosocial aspects of congenital anorectal

malformations •••••••••••••••••••••••••••••••••• 73 ANORECTAL MALFORMATIONS

Clinical features and treatment

Incidence and aetiology

70 71 71 72

...

·

.

D. THE CHILD WITH A CONGENITAL ANOMALY AND EARLY MOTHER/ INFANT SEPARATION ••••.•••••••••••••••••••••••••••• 76

(iv)

CHAPTER 4

THE ONSET OF PRELIMINARY SYMPTOMS AND LEARNING THE DIAGNOSIS

THE PRE-DIAGNOSTIC PHASE IN CYSTIC FIBROSIS •••••• 1. Duration of the pre-diagnostic phase •••••••••• 2. Difficulties experienced at the institution of

THE FAMILIES .. 1. Mortality . 2.. Family size .. 3.. Age .. (a) Patients ••••••••••••••••••••••••••••••••• (b) Mothers . (c) Fathers •••••••••••••••••••••••••••••••••• A. B. 4. 5. Social class

The utilization of social work services

80 80 81 82 82 84 84 85 86 87 88 birth 90

(a) Infants directly transferred to a hospital 90 (b) Patients discharged undiagnosed, but with

suspicion of ill health ••••••••••••••••. 90 (c) Patients discharged without suspicion •••• 91

C.

D.

3. Seeking a diagnosis in the community THE DIAGNOSTIC PHASE IN CYSTIC FIBROSIS

1. The immediate response of parents to the

diagnosis .

2. How parents were told ..

THE PRE-DIAGNOSTIC PHASE IN OESOPHAGEAL ATRESIA 1. Duration of the pre-diagnostic phase •••••••••• 2. Type of oesophageal atresia diagnosed •••••••••

92 95 95 97 100 100 100

(v)

3. Difficulties experienced at the institution of

105 106 101 103 104 104 birth .

(a) Infants directly transferred to a hospital •• (b) Patients discharged undiagnosed, but with

suspicion of ill health •••••••••••••••••••• 109 THE DIAGNOSTIC PHASE IN OESOPHAGEAL ATRESIA ••••••• THE PRE-DIAGNOSTIC PHASE IN HIRSCHSPRUNG'S DISEASE •• 1. Duration of the pre-diagnostic phase •••••••••• 2. Difficulties experienced at the institution of

birth .

E. F.

G. H.

(c) Patients discharged undiagnosed, without

suspicion of ill health ..•....•...•...•.

3. Seeking a diagnosis in the community ••••••••••• THE DIAGNOSTIC PHASE IN OESOPHAGEAL ATRESIA ••••••• THE PRE-DIAGNOSTIC PHASE IN ANORECTAL MALFORMATIONS • 1. Duration of the pre-diagnostic phase ••••••••••• 2. Difficulties experienced at the institution of

110 110 113 116 116 (a) (b) birth

Infants directly transferred to a hospital. Patients discharged undiagnosed but with suspicion of ill health •••••••••••••••.••• (e) Patients discharged undiagnosed but without

117 117

118

suspicion of ill health •••••••••..•••••••• 119 3. Seeking a diagnosis in the community ••••••••••• 119 I. THE DIAGNOSTIC PHASE IN ANORECTAL MALFORMATIONS 120

(vi)

CHAPTER 5

THE CONFRONTATIONAL PHASE: COPING WITH THE ACUTE STAGE OF THE CONGENITAL ANOMALY

A. THE HOSPITAL EXPERIENCE

...

1. 2.

3.

Hospital doctors and parents ...••...•..

Parents and nursing staff ••••••••••••••••••••• Parents and other hospital staff ••••••••••••••

136 139 142 B. C. D.

SEEKING A CAUSE FOR THE ANOMALy ••••.••••••••••••• OTHER MAJOR LIFE EVENTS •••••••••••••••••••••••••• BRINGING THE PATIENT HOME

143 148 151

1. Fear of discharge ••••••••••••••••••••••••••••• (a) Cystic fibrosis ••••••••••••••••••••••••••

(b) Oesophageal atresia ...•..•...•...•... (c) Hirschsprung's disease •••••••.••••••••••• (d) Anorectal malformations ..•...•..•....• 151 152 153 155 157

Wishes to have aborted •••••••••••••.•••••••••• Mother-child enstrangement •••••••••••••••••••• 2. 3. 4. Death wishes

.

.

(vii)

CHAPTER 6

THE LONG TERM ADAPTATIONAL PHASE: LEARNING TO LIVE WITH THE ANOMALY IN THE COMMUNITY

A. HANDLING SYMPTOMS AT HOME 168

1. 2. 3. 4. Cystic fibrosis .. Oesophageal atresia ..••..•..•..•••••.••.•••.• Hirschsprung's disease .. Anorectal malformations .. 169 175 179 190

B. COMMUNITY REACTION AND PARENT ATTITUDE 195

1. 2. 3. 4. Cystic fibrosis .. Oesophageal atresia .••.••..•.•...••...•..•• Hirschsprung1s disease .. Anorectal malformations .. 195 198 199 201 C. D.

CONTACT WITH PARENTS OF OTHER PATIENTS

DISCUSSION

..

..

(viii)

CHAPTER 7

THE CHILD WITH A CONGENITAL ANOMALY AND SOME FAMILY RELATIONSHIPS

A. THE MARITAL RELATIONSHIP 213

1. 2. 3. 4. 5. Cystic fibrosis . Oesophageal atresia ..•••••..•••••••.••••••••• Hirschsprungfs disease . Anorectal malformations •••••••••••••••••••••• Some general factors

214 217 218 221 222

(a) Absence of the father

...

(b) Mothers' source of main support

...

(c) Subsequent pregnancies

...

...

3. Hirschsprung's disease and anorectal malforma-tions

DISCUSSION

230