THE OFFSPRING OF PEOPLE WITH CEREBRAL PALSY

00

6 N 6 d m

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The few studies of people with congenital cerebral palsy (CP) who have had children have dealt mainly with the quality of labour of the disabled mothers (ClaytonJones 1962, Bengston et al. 1989, Winch et al. 1989). Hansen (1960), in his detailed study of the CP population of Denmark, mentioned that 17 per cent were married but said nothing of any children. The subject probably has been neglected because of general assumptions that these patients do not have children and that, since the cause is (or was) thought to be birth injury or preterm birth, it could be of no genetic interest. But the number of people with CP who marry and have children is undoubtedly increasing because of better care, and obstetricians may be concerned as to whether such mothers experience special difficulties during labour. As a general neurologist, the author encountered several adult patients with families (and remarkable life-stories), and had also in the course of three decades seen over a thousand children with CP of all kinds. The matter merited investigation for two reasons. One was a general interest in these individuals and their lifehistories. The other was more complex. Detailed analysis of the data obtained from the records of the cases of congenital CP had shown (a) that the severity of the ultimate condition is not

related to the nature of birth (Foley 1992), and (b) that two-thirds of all cases can be regarded as immature as judged by their birthweight ratios (Keen and Pearse 1985, Brooke et al. 1989, Morley et al. 1990), though the ratio seemed to bear little relationship to clinical severity. But the question arose as to whether the fundamental cause is a placental metabolic insufficiency: and this raised the further question of whether such a defect, due possibly to a maternal or paternal mutation, might be transmitted. The answer to this question was the primary goal of the limited survey reported here. There must be many individuals with CP who have had children, but they lead their lives in obscurity, do not find their way into the medical literature and are difficult to locate. Purists will argue that information derived from a postal survey, in which respondents were not examined personally, is worthless: but it is better than none, and the precision of the respondents' replies came as a pleasant surprise.

Material and method Questionnaires were sent to as many people with CP who had married and had children as could be contacted through various sources. Most of the contacts were made through the social workers at various local branches of The Spastics

Society, but some came from the author’s own records, and some from patients themselves. After the exclusion of one case with acquired CP and two with familial spastic paraplegia, there were satisfactory replies from 88 parents. The questionnaire, accompanied by an explanatory letter, consisted of 13 main questions, and was filled in with remarkable precision by the affected parents-only 9 per cent, for example, failed to supply their postcodes. Doubtful points were clarified by further letters. The responders were asked to place themselves in one of five diagnostic categories, being supplied with brief definitions, and did so as follows: athetosis and athetoid quadriplegia (42), spastic diplegia (29), hemiplegia (1 6 ) and one person was uncertain. There were 15 couples who both had congenital CP, and a further seven with CP had married a person with another kind of disability: the rest had married unaffected people (Tables la, b, c). Regarding diagnostic categories, athetosis was defined as CP dating from birth and characterised by irregular, unwanted movements, with or without some rigidity: diplegia as weakness and spasticity of both legs, with minimal weakness of the hands; and hemiplegia as involvement of one side of the body only. The responders had 122 children and 19 grandchildren. These responding parents of course would represent the less disabled and most enterprising of the CP population, and a small fraction at that, but it is assumed that the pathology is similar in mild and severe cases, only less in extent in the former. 27 used a wheelchair, one had a communication aid and another one wished for one; and of the 27 fathers with CP, one is a minister and two hold civil decorations. They were asked as a matter of interest to what they attributed their condition, given a choice of birth injury, preterm birth, Rh-incompatibility, ‘uncertain’ and heredity. Most of the athetoid parents blamed birth injury; the preterm diplegic parents blamed immaturity or were ‘uncertain’; the term diplegic parents blamed birth injury; while those with hemiplegia were equally divided between the three first choices. Most of the

TABLE la People with CP married to normal spouses

00 P QI

r4 rQI

Male Female

Children Normal Abnormal

d m r4QI

Diplegia I Athetosis/ athetoid quadriplegia Hemiplegia 0 Total 8

18

30

7 9 43

16 1 7 74

1

0‘

37 6 8

TABLE Ib Both parents with CP Male

Female

DPL DPL DPL ATQ ATH

DPL DPL DPL ATH HEM ATQ ATH ATH ATQ ATQ ATH HEM DPL ATH HEM

2;s

ATH ATH ATQ ATHO ATHO HEM ‘HEM HEM Total

Children Normal Abnormal 1

1 3 1 1 1

3

2 1 3

2 I

1

2 2

2

24

3

TABLE Ic One parent with CP, one with another disability Male

Female

Children Normal Abnormal

DPL ATQ Acquired CP Friedreich’s ataxia Spinal deformity ATH Phocomelia Total

Epilepsy Poliomyelitis DPL

3 3

HEM

I

DPL Partial sight HEM

2

1

1 -3

12

I

DPL = diplegia; ATH = athetosis; ATQ = athetoid quadriplegia; HEM = hemiplegia; ATH 0 = reported by CP spouse but not one of responders.

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3 4"

TABLE I1 Offspring of mothers with congenital cerebral palsy

i

2 -

A

Diagnosis

N

2

9

-n 0

z

a L

0 M

.L c

i;

Preterm diplegia 14 Mean SD Range Median 8 Term diplegia Mean SD Range Median Hemiplegia 14 Mean SD Range Median Athetosis (1 1) Athetoid qudriplegia (10) Quadriplegia (4) 25 Mean SD Range Median

Mofhers Mean GA (wks)

Mean BW

29.14 (14). 3.1 26-34 28 '

984.1 (14) 502 568- 159 I 1136

40.25 (8) 38-40 40

3216 (8) 784 2045-4190 2954

36.6 (10) 5.2 24-42 38

2165 (10) 827 909-3352 2315

38.5 (19)

3027 (15) 866 1591-4204 3239

(8)

Children

N children Age a1 birih Mean GA of first child (wks)

Mean BW

w

27 29.5 (14) 5.2 21-41 28

37.6 (27) 3144 (27) 7.6 787 37-40 2528-3977 39 2997

27.6 (8) 3.9 23-34 27,5

38.7 (12) 3345 (12) 1.2 606 35-42 2363-4261 38.5 3277

28.3 (14) 4.7 18.5-35 31

39 (20) 1.3 38-40 39.5

27.6 (25) 5.1 19-37 28

38.6 (40) 3097 (40) 2.45 610 21-42 966**-4500 39 3153

I2 1.15

20 3195 (20) 583 2131-4346 3323

51 3.8 28-32 40

*Numbers for whom information available. **Baby with trisomy 18.

children were born between 1961 and 1988, the mode being 1981. With regard to their children, they were asked about pregnancy, birth, birthweight, gestational age, neonatal problems, malformations and birthmarks, motor or behavioural difficulties and any subsequent health problems. There were no questions about social conditions.

Results

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The mothers Sixty-one mothers with congenital CP supplied details. Their mean age was 28-3 years at the birth of the first child (SD 9, range 18.5 to 40.8). They had had 132 pregnancies. Three were terminated, one resulted in a stillbirth, and there were 18 miscarriages (14 per cent). Toxaemia occurred in 5 per cent. The frequency of an abnormal pregnancy was the same for the mothers with an able-bodied husband as for those with a CP husband (x2= 0.6, p < 0 . 5 ) , though it was slightly higher for the mothers with hemiplegia. The diference in the number of abnormal pregnancies between hemiplegic mothers and those with the other forms of CP was

significant ( x 2 = 9*7,p