Parental responses to consanguinity and genetic disease in Saudi Arabia

Sot. Sci. Med. Vol. 33, No. II, pp. 1295-1302, 1991 0277-9536/91 $3.00 + 0.00

Printed in Great Britain Pcrgamon Press plc

PARENTAL RESPONSES TO CONSANGUINITY AND GENETIC DISEASE IN SAUDI ARABIA

CATHERINE PANTER-BRICK Department of Anthropology, Durham University, 43 Old Elvet, Durham DHI 3HN, U.K.

Abstract-In-depth interviews of 36 Saudi families whose children suffered from neuro-metabolic disorders were conducted at a specialist hospital in Riyadh in order to examine parental understanding of disease and attitudes towards future births and consanguineous marriages. Parents had difficulty accepting a genetic explanation for diseases that did not affect all children at the time of birth, they also expressed religious or folk beliefs to account for illness. Coping behaviours included denial and resignation to the situation, divorce and remarriage. Some families adopted a cautious approach to cousin marriages and future births; this was significantly related to their education level, but not to previous infant deaths. Awareness of medical facts brought little emotional comfort to parents but allowed for preventive measures through screening adult carriers and identifying affected infants. This study presents new material from Saudi Arabia to strengthen current awareness that the range of religious beliefs, social attitudes and reproductive behaviours adopted by families in a society undergoing rapid change is of direct relevance to health care.

Key words-consanguinity, counselling, religion, metabolic diseases, screening, family planning, Islam

INTRODUCrION

This study examines parental understanding of genetic inheritance and responses to the morbidity of children admitted to King Faisal Specialist Hospital and Research Centre (KFSH), Riyadh, Saudi Arabia. Parental views about consanguinity and disease were ascertained in the hope of enhancing supportive care and genetic counselling in a country undergoing rapid change, especially in the fields of formal education and medical care [l-7]. While parents might not always express their true feelings to a foreign investi- gator, their responses provide a valuable indication of what they believe is appropriate or socially acceptable, what problems they perceive, and how they cope with them [8,9]. Patients and physicians often assign a different meaning to the experience of sickness, and the discrepancy between explanatory models affects their relationship and the efficacy of medical care [lO-121. This study describes the beliefs and attitudes of Saudi families who were counselled by practitioners who were trained in the West.

Genetic diseases are frequently expressed in Saudi Arabia because of three factors: the widespread practice of consanguineous marriages, the high fertility of Arab families, and the reduced mortality due to infections. A recent national survey, the first demo- graphic study of its kind, showed that over half of Saudi marriages were contracted between relatives, most of them between first cousins (parallel, cross- cousin, matrilineal and patrilineal), with an even greater prevalence of consanguineous marriages amongst the Bedouin (Table 1). An independent study of 4498 women randomly selected from delivery wards in four Riyadh hospitals [ 131 reported a consanguinity rate of 54.3% overall (31.4% for first cousins).

The incidence of inherited metabolic and neuro- degenerative disorders in Saudi Arabia is still unknown. However, the referral of cases to the

KFSH, a tertiary care centre for the whole Kingdom, indicates frequent occurrence as compared to the West. Maple syrup urine disease (MSUD) is rare in Europe [lo] and the United States [14], while the diagnosis of 16 MSUD cases during an l&month period in 1987-88 suggests that it is 50-100 times more common in Saudi Arabia. Likewise, biopterin- dependent phenylketonuria (PKU) is extremely rare in the West, while the KFSH detected four new cases, all members of the same tribe. The carrier rate for Sandhoff disease is around l/298 world-wide, but could be l/32 in some tribes of Central Arabia [15]. Other genetic diseases have been described in Saudi Arabia, with particular reference to consanguinity [16]. Inbreeding-related morbidity is reported elsewhere, in Arab or Muslim communities of India [16, 181, Sudan [19], Iran [20], Egypt [21], Iraq [22], Pakistan [23], Kuwait [24] and Britain [2S].

METHODS AND SAMPLE

The thirty-six families included in the study were a cross-section of those admitted in Pediatrics at KFSH over a four-month period (June-September 1988). These were all parents whose children suffered from metabolic and neurological disorders; nineteen were in-patients staying in the hospital for several days or weeks, while seventeen were out-patients attending a clinic. This sample is not representative of the general population; it constitutes a group of families referred to a specialist hospital by other centres (all medical treatment is free of charge), and counselled by a physician (not specifically by a genetic counsellor). Parents were interviewed after their consultations, with the assistance of Arabic-speaking hospital personnel who volunteered their help. Most translators were female in order to gain the confidence of women. The author learned Arabic and could follow conversations.

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1296 CATHERINE PANTER-BRICK

Table I. Consanguinity in Saudi Arabia (Tabbara er al., unpub- lished) King Khalid Eye Specialist Hospital National Survey

In general In the Marriages between: population Redouin

First cousins 28% 59% Second cousins 5% 7% Distant relatives 20% 23% Other 47% II% Total 100% 100%

Exact n unavailable (close to 9 million people in Saudi Arabia).

The interviews were semi-structured questionnaires which included questions demanding a yes/no type of answer and open-ended queries, amenable to quanti- tative data analysis and contextual descriptive statements. Knowledge about the disease was ascertained from the family’s belief in its underlying cause, recognition of symptoms, expectation of progress or fatal outcome, and cognizance of special treatment. The social, financial and emotional burden of care for the sick child was then discussed. Attitudes towards marriage and family planning were explored by asking parents whether they wished to have more children, preferred cousin marriages to non-consanguineous unions, and would arrange their child’s marriage with a close relative. Their course of action in seeking medical help was also elicited, together with comments about medical services. The consistency of parental responses, in having acknowledged heredity and taking a cautious viewof marriage and reproduction, was subsequently evaluated against educational level, family size and number of affected births using Fisher’s exact test.

Parents were met on two or three separate occasions, each spouse individually. In fourteen cases, only one spouse could be met. A total of sixty-five interviews were conducted, each lasting between twenty and ninety minutes. No one refused to participate in the study.

Parents were genetically related in all but four cases (Table 2). Their educational background is shown in Table 3. Fathers, averaging 34 years of age (SD = 7.32), worked as qualified teachers, accountants or engineers (n = 17), in other occu- pations (n = 9), in the National Guard (n = 8), or were unemployed (n = 2). Mothers, aged 25.9 years (SD = 5.6), were all housewives except for two primary school teachers. All respondents were Sunni Muslim. Eighteen lived in the larger Riyadh area, seventeen resided elsewhere in the Kingdom, and one was referred from Yemen.

Families averaged 4.58 (SD = 2.83) total births and 3.83 (SD = 2.55) live children, with a range from 1 to 10 births. Two-thirds had one or two affected children but no previous deaths, while one-third had experienced up to four infant deaths. Children were 1-87 months old (mean 20.30, SD = 21.26) at the time of study, and had been hospitalised for an average of four months during their lifetime. Just over half the families lived as nuclear households; a

Table 2. Genetic relationship between spouses in this study

Coefficient of relationship Spouses Total

Relationship I n = 36 %

Double first cousin l/4 I 3 First cousins II8 24 67 Second cousins l/32 4 II Distantly related <l/32 3 8 Unrelated’ 0 4 II

‘Includes ‘milk cousins’ who breastfed from the same nursemaid.

third employed a maid or a nanny to help with childcare. Six fathers were polygamous.

The diseases included thirty-one cases of inborn errors of metabolism, most of them autosomal reces- sives, and five other neurological disorders (Table 4). Defects in amino acid metabolism were amenable to some dietary control, but no cure was available for the lipid storage diseases or mucopolysaccharidoses [26, 271.

PARENTAL RESPONSES TO ILLNESS

Parental emotions usually passed through the four distinct stages (denial, anger, depression, resignation or action) common in the West (28-301. A first defence mechanism involved a powerful denial of the situation: parents could not accept a bleak prognosis and sought medical help elsewhere; they might claim a retarded child had reached normal developmental milestones; or they refused to tell relatives that the disease was inherited and have them tested. Blame and resentment were often directed towards medical personnel and caused many disruptions in family life [31]; there were considerable child-minding difficulties in cases of neuro-degenerative diseases because of the children’s obvious disabilities, and maternal bonds were disturbed right from birth when newborns with metabolic disorders could not suckle. Worries about the threat of divorce, fear for the child’s life, bouts of general depression were commonly expressed. Parents eventually accepted their situation with serenity or resignation, and lived “one day at a time”. Others resorted to more drastic behavioural strategies, such as remarriage and the start of a new family.

PARENTAL EXPLANATIONS FOR DISEASE

Statements accounting for the presence of disease included the following: “God’s will”, “genetics”, “I don’t know”, the “evil eye”, and “illness during childbearing”. All parents believed God determined their fate in granting health or illness. Some acknowledged a genetic basis for the disease without truly accepting or understanding it. Many denied any knowledge of possible causes, mostly because they did not believe given medical explanations or were reticent to disclose beliefs of a non-medical nature.

Education in years

Husbands Wives

Table 3. Educational background of respondents

Enrolment Primary Secondary Further O-2 yr 3-6 yr 7-l2yr >12yr

3 (8%) 9 (25%) I (20%) I7 (47%) I2 (33%) IO (28%) 4 (II%) IO (28%)

Total n (%)

36 (100%) 36 (100%)

Parental responses to genetic disease 1297

Table 4. Nature of the diseases affecting patients and families

Diseases Aetiology Trcatmcnt Cases

Defects in amino acid metabolism Maple syrup mine disease AR diet 5 Phenylketonuria’ AR diet 3 Organic acidcmias AR diet? 5 HMG CoA deticicncy AR diet 2 Carboxylase deficiency AR diet? 1 Acyl CoA deficiency AR diet? I Tyrosinemia2 AR diet I Hyperglyccrolemia XR diet I

Defects in lipid metabolism Sandhoff AR none? 2 Niemann-Pick AR none’ 3 Gaucher AR none? 1 Ceroid lipofuscinosis AR none’ I

Other metabolic defects Mucopolysaccharidoses AR none 2 Glycogen storage disease AR diet I Zellweger syndrome AR none 1 Familial hypercholesterolemia AD diet 1

Neurological syndromes Unclassified disorder XR none 1 Congenital hypoparathyrodism UN none 1 Pierre Robin malformation’ SP therapy I Microencephaly AR none 2

Total 36

Autosomal recessive, AR; Autosomal dominant, AD; X-linked recessive, XR; unknown, UN; sporadic, SP.

‘Malignant or biopterin-dependent PKU. ‘Suspected but ruled out on the basis of further tests. ’ Receiving genetic counseling in the pediatric unit. ?No cure. but bone marrow transplant a possibility.

Genetics (wiratha)

In Arabic, wiratha means “inherited” or “trans- mitted” from one generation to another, but does not refer exclusively to a biological process. Parents did not accept the inheritance of disease unless the family history made this obvious. They thought that a genetic illness should affect all children, rather than a few, immediately after birth, rather than a few months later. They stressed the fact that relatives had married cousins but were blessed with normal children, or that brothers who married outside the family had affected children. Spouses did, however, acknowledge they were carriers where the disease clearly ran in the husband’s or the wife’s line. But the risks to future pregnancies were poorly understood.

Two-thirds of respondents (n =24 or 67%) acknowledged that the disease could have a genetic basis, but only a third (11 or 3 1%) of them were sure. Eight parents quoted a probability for future children being affected, four of them accurately: where recur- rence risks were l/4, families quoted “SO-50”, “30-70”, “four will be ill, six healthy”, or “after the fourth, the child will be all right”. Others could not remember (7 cases), claimed they were not told (7 cases), or did not know because doctors had yet to reach a final diagnosis (2 cases). One mother “frankly did not care” about probabilities so long as the possibility for a birth defect did exist.

Religion

Parents centered their explanations around religious belief, whether or not they had acknowledged heredity, because in many ways it provided a better reason for the occurrence of disease (see Discussion). One mother expressed this as follows: “the disease

runs in my family. But only God knows why some children are fine and others are not”.

Parents also used religion as a vehicle for express- ing their feelings (“Insh’Allah” or “God willing” being one common expression). Many popular attitudes towards health and disease were rooted in the body of Islamic teaching elaborated in the Qur’an, narratives of Prophet Mohammed’s life (hadith) and the writings of theologians [32,33]. Although few parents substantiated their feelings with excerpts from the Qur’an or hadith, their responses closely reflected religious teachings. They believed their burden was willed by God and should be borne patiently (Qur’an LVII, v. 22-23: “Naught of disaster befalleth in the earth or in yourselves but it is in the Book before We bring it into being (. . .), grieve not for the sake of that which hath escaped you”) [34]. This helped parents to overcome feelings of anger or helplessness [32, pp. 448-449; 33, p. 4881: mothers would say “I must accept His will”. The more devout families perceived suffering as a test of their faith (Qur’an II, v. 155-156: “And surely We shall try you with something of fear and hunger, and loss of wealth and lives and crops; but give glad tidings to the steadfast, who say, when a misfortune striketh them, we are Allah’s and unto Him we return”).

Religion also made possible the denial of responsi- bility (Qur’an XXIV, v. 61: “No blame is there upon the sick . . .” ). Unlike in the West [30,35], only a minority of parents acknowledged guilt for having given birth to a child which had to suffer. While most mothers worried that future births would be affected, others expressed no feelings of this nature, since “God would provide”.

Lastly, religious beliefs allowed hope for the child’s survival. One mother quoted from a ha&h: “there is a cure for all diseases except old age”; in fact, it states: “there is a healing for all diseases . . , except death” [36, p. 4001. Muslims emphasise that surrendering to God’s will does not prevent them from seeking a cure. According to an authoritative hadith, the Prophet said: “0, servants of God seek medical treatment. God has put a remedy for every malady, clear to whoever knows it and unclear to whoever does not know it” [33, p. 341, and also: “No disease Allah created, but that He created its treatment” [36, p. 3951.

Folk explanations

Belief in the “evil eye” (‘ayn) also helped to explain why certain children, perfectly healthy at birth and growing normally, were suddenly afflicted with a serious illness. The “eye’‘-also known as the “look” (nathra)-is cast by females, who may be jealous, envious or simply wicked, and who intentionally or unintentionally harm a child at a glance. An example will make this clear. One father related that his newborn son became sick moments after a female relative, upon seeing the child suckle, asked rudely why the baby was not bottle-fed and left the room without blessing him. At the hospital, a Saudi doctor who was also a motawwaa (religious advocate) offered to counteract the evil influence of her eye by reading verses from the Qur’an, washing a coffee cup and date stones left behind by the woman, and cleansing the baby with this water. Thereafter the


child’s condition improved remarkably-he had MSUD and was placed on a special diet since he could not metabolise the protein in breast-milk. The coincidental events leading up to the baby’s sickness and recovery were perfectly congruent with both medical and “folk” explanations.

Belief in the “evil eye”, prevalent throughout the Middle East [3741], is a sensitive issue, an “old- fashioned” conviction which parents are unwilling to discuss with doctors. However, ten respondents thought that it was substantiated by the Qur‘un (CXIII: “I seek refuge with the Lord of the Daybreak (. . .), from the evil of the envier when he envieth”) and hudith [36, p. 4261, and that illness could be attributed to it. Over a third (39%) of the respondents had resorted to traditional methods of healing, taking their children to a reputed reader of the Qur’un or to a local healer (tabib shuubi). Local healers are men or women who apply ointments on the head or cauterize selected parts of the body, such as the neck, back and stomach, with hot iron nails or wooden sticks [42-44]. This treatment is said to counteract the physical symptoms of an illness, and the effects of the “evil eye”. Some parents saw local healers before they had tried hospital care, but others went after their child, still feeble, had left KFSH. One father of an MSUD child even interrupted the hospital’s prescribed dietary treatment for five months because the “bums” had been so successful. Interestingly, while the Prophet Mohammed ordered ruqyu (the rect&ion of Quranic verses) if there were danger from an evil eye, he is said to have forbidden cauterization (36, pp. 396 and 4261.

Lastly, infectious illnesses (e.g. meningitis) and taking the contraceptive pill during pregnancy or early lactation were also cited as a cause for a child’s failure to thrive. In accordance with the variety of explanations held, families sought a combination of cures from among Quranic readers, local healers and empirical medicine.

REPRODUCIIVE PLANS

Births and marriages

Half the families expressed a desire to have more children, 42% did not, while 8% maintained that the birth of children depended solely upon God’s will; five mothers were already pregnant at the time of study, Those who wished to limit births had not necessarily achieved a large family size: six had few children but wanted to take no further chances of a child suffering (Table 5). Six other families, with past experience of infant morbidity, were, however, planning another pregnancy; often the couple felt compelled to give birth to healthy children, particularly sons. Thus no significant relationship was found between reproductive plans and the number of live children or affected births.

Contraception and abortion were issues discussed with a total of 30 families [see 45). In half these cases, women took the contraceptive pill: fourteen in order to space births, and one to avoid menstruation and associated ritual pollution during her pilgrimage to Mecca. One woman contracepted by breast-feeding. Others had not yet considered birth-control, believed it was forbidden by religion and adverse to health, or had their wishes overruled by their husbands. In this sample, contraceptive use was not significantly associated with parental education.

Twelve families said they would consider terminat- ing a pregnancy if the doctors could tell the fetus was affected and advised this as the best course of action. They would seek clarification of Islamic law-it allows abortion within the first 120 days of pregnancy if the mother’s health is endangered [4648]. Three fathers were prepared to travel abroad since abortion is not practised in Saudi Arabia. In contrast, sixteen parents would refuse a therapeutic abortion on religious grounds (Qur’un XVII, v. 31 “Slay not your children . . .” ) and because one could never be sure that the child would be unhealthy. Indeed one family, offered curettage in Germany, decided to keep the affected baby. One father declared he was con- sidering “contraceptives or divorce, but certainly not abortion”.

Polygamy and marriage after divorce are two options available to Saudis. Fourteen women thought that their husbands might remarry; the latter inde- pendently confirmed this and said their spouses had the choice to stay or leave. The two wives with independent financial security said they were “like all other women” who could not prevent their husbands from remarrying, for “Saudi men like many children”. This upset many women [see 491, particularly younger wives, but others calmly stated this was “our culture and God’s will”, that “men had the right to take up to four wives”, or that “all our neighbours had several wives”. Three women had advised their own husbands to take another spouse who would bear them healthy children; given the value placed on family life, they perceived this as “the right thing for a man to do.”

The men who would not remarry were either highly educated, already polygamous, unemployed, or fathers-to-be. Their wives confidently stated: “he is a good man”, or “why should he remarry? the disease is not from me”.

Views on consanguinity

Attitudes towards marriage between close relatives were more or less equally divided between those in favour and those against. Thirteen parents thought cousin marriages were preferable because the families knew one another and because this strengthened relationships between them; they recognised no

Table 5. Reproductive plans, current family size, and affected births

Wish more children

Yes NO Up to God n

Current family size Affected births Total

< 5 children > 5 children 0 deaths 1-4 deaths n (%)

13 (36%) 5 (14%) 12 (39%) 6 (17%) 18 (50%) 6 (17%) 9 (25%) 9 (19%) 6 (17%) I5 (42%) 3 (8%) 0 (0%) 3 (8%) 0 (0%) 3 (8%)

22 (61%) 14 (39%) 24 (66%) I2 (34%) 36 (100%)


particular problems with this custom (take the case of three daughters promised to the three sons of a maternal uncle: one had a child with PKU, but the parents would not change the wedding plans of her sisters). Fourteen parents rejected such marriages, because they foresaw that families might be torn apart if the marriage failed, or because they realised that, from the point of view of health, it was best to marry an unrelated person. In two cases, the spouses held conflicting opinions. Other couples specified that it was up to each family to make the decision.

[N--54], although education and familiarity with the disease will be important [28,55]. These studies also show that “genetic counselling” is not always conse- quential for those who plan the birth of another child [56]; the perceived burden of having another affected birth, family size, and prior reproductive plans are far more likely to influence their reproductive behaviour [29, 57-591.

Parents’ statements ranged from: “a spouse you know is better than one you don’t know and a cousin takes better care of you . . . our custom brings the family closer . . . when you have beauty, intelligence and money, why marry out of the family?’ to conversely “I could not marry my cousins, they are like brothers . . . relatives fight like scorpions . . . there is the genetic problem, so marry out to get a strong child . . . cousins should not marry if they are carriers, but if they like each other . . .“.

Fourteen parents said they would discourage their children from marrying a close relative, and another three said they would merely advise them against it. One father wanted to ban all consanguineous marriages for the next generations, while two mothers said “the modern way” was to undergo testing before the wedding to establish whether a couple carried any diseases. Some had already spoken with their friends and family, but their advice generally went unheeded, as examples of consanguineous marriages with healthy progeny were also to be found.

EVALUATION OF PARENTAL RESPONSES

In just over half the cases, parents professed a good understanding of heredity and coupled this awareness with a cautious attitude towards future births and marriages (Table 6). The consistency of their responses was significantly associated with the education of both husbands (P = 0.003) and wives (P = 0.04). Contrary to expectation, the type of parental response was not significantly associated with past experience of the disease.

Saudi parents may well have difficulty comprehending the complex rules of genetic inheritance, especially if they have had little schooling; women, in particular, felt unable to question male physicians [see 59,601. Parents may also be unwilling to accept a genetic explanation for what is a very sensitive family matter. Religion offers them a more acceptable explanation for the disease, resolving confusion and incertitude with respect to the timing of illnesses and the outcome of future births. While Mendelian inheritance stipulates that carriers will have a given pro- portion of affected children, it cannot specify why this particular child is spared while another is afflicted. Under these circumstances, parents prefer to relate the reason for individual suffering to God’s will rather than chance events: there is a purpose to God’s action that cannot be attributed to “blind” probability. Thus, where the relationship between disease and death as one of cause and effect is well understood, two more pertinent questions remain unanswered: “why this person rather than another and why at this present time?’ [62,63]. Religious belief did not avert distress and disturbance in family relationships, but it did enable parents to make sense of a confusing and uncertain situation and to shoul- der the burden of raising sick children. Furthermore, it was a socially accepted way of responding to illness; while respondents unanimously professed submission to the will of God, their emotional responses ranged from anger and bitterness to acceptance of their plight with remarkable outward serenity. Thus parents found in religion a way of explaining disease which fell into line with the larger framework of culturally sanctioned Islamic beliefs and behaviours.

DISCUSSION

Studies conducted in Western countries have demonstrated that medical and genetic information is often incompletely assimilated or misunderstood by families affected by an inherited disorder. Parents have great difficulty comprehending in statistical terms the risks of having normal or affected children

Table 6. Parental responses and education

Wife’s schooling Husband’s schooling Type of response <6yr >lyr n <6yr >lyr n

Acknowledged

Despite repeated efforts, the medical staff may also have been unable to convey all the necessary counselling information [64, 651. Linguistic difficulties, temporal pressure, and cultural and educational differences often hamper hospital personnel, who are in the great majority foreign. To enhance the com- munication of medical and genetic information, families can be made aware of at least three important facts: that the onset and expression of a genetic trait can vary, that consanguinity increases the risk of affected births where families carry deleterious genes, and that medical counselling does not exclude all reference to religion since it focuses upon general rules of inheritance while the latter addresses particular instances [63, 661.

Counselling may have very little impact if consanguinity is not widely discouraged; however, marriage

heredity- 10 I1 21 3 19 22 Other 12 3

patterns can be very resistant to change (in one 5 14

n 22 14 :: I: 24 36 illustrative case, a Saudi father counselled at KFSH

Fisher’s exact test (two-tail): P = 0.003 for husbands; P = 0.04 for divorced his wife, a paternal first cousin, only to

wives (association between spouses’ education: P = 0.07). remarry a maternal cousin). The custom of marrying

‘Where parents acknowledged a genetic basis for disease and held one’s cousin is deeply rooted in Arab culture, and is attitudes towards births and consanguinity which reflected an thought to predate Islam (Omar Ibn Khattab, the understanding of heredity. Prophet’s companion, had to advise: “seek outsiders

SSM 33111-F


for marriage, so that your progeny does not become weak”). While the Qur’an (IV, v. 23) forbids certain marriage relations of consangunity (as in the cases of mother, daughter, sister, niece and aunt), fosterage (foster-mother and sister) and affinity (wife’s mother and daughter, son’s wife), it does not forbid unions between other relatives. The Prophet himself married a first cousin, but also took wives from outside the family [67, pp. 129-1301. Cousin marriages prevailed over time for social reasons as they strengthened family ties and kept property within the pattilineal group. This custom may decline among the highly educated urban elite [13, 68-701, but it is still widely practised by the Bedouin [71,72].

While recent studies have shown that inbreeding effects on fertility and mortality may not be as severe as-originally suggested, as in South India and Japan where inbreeding has been practised over many generations [73-781, rare deleterious recessive genes are clearly more commonly expressed in inbred populations [79-811. A recent survey of childhood blindness in Saudi Arabia, which showed significant association with consanguinity, recommended “a comprehensive programme of religious and govern- mental intervention to discourage marriage between persons having the same grandfather or grand- mother” [82]. Yet it is difficult to effect such a program in Saudi Arabia, as this touches upon a very sensitive issue which most people believe lies solely in the family domain. Nowadays, general education in genetics is available only to those who reach high school. Recently however, local newspapers and some television programs have drawn public attention to the subject [83].

A screening program for identifying families at particular risk of genetic disease is a realistic objective in Saudi Arabia. It is possible to distinguish the heterozygous carriers from affected homozygotes in a small number of disorders, such as Sandhoff disease, MSUD and PKU [26,27]. The KFSH already screens all neonates for PKU and offers amniocentesis to high-risk women, and recently conducted a pilot survey to screen Sandhoff carriers [15]. Towards the end of this study, a meeting between doctors and parents was convened in the hope of forming a support and discussion group. It was encouraging that parents showed great interest in the rules of genetic inheritance and the possibilities for screening relatives before marriage. Awareness of medical facts, while bringing little emotional comfort to parents, does allow for future preventive measures through pre-marital screening of adult carriers and timely identification of affected infants.

In turn, an appreciation of the range of religious beliefs and social attitudes is of direct relevance to health care [ 11,84-86, p. 21, 87,88, p. 13771. In the first place, Saudi families expressed a variety of views on health and explanations of disease, based on Islamic teachings, popular beliefs and secular medical facts, which provided acceptable medical and moral explanations for their ordeal. Heterogeneity of medical ideas as well as large areas of uncertainty are only to be expected in a society undergoing very rapid change (891. Second, these beliefs shaped both health care utilization and subsequent reproductive behaviours. Out of concern for their children, parents

sought health care from several practitioners, including local healers and medical doctors. Their coping strategies were also varied, including a cautious approach to consanguineous marriages, divorce, polygamy, a wish to limit births or conversely a compelling desire to give birth to a healthy child. Knowledge of genetics has thus profound ramifica- tions on family relationships, which are perhaps the central element of Saudi culture.

In conclusion, this study focuses on a situation of great anthropological and medical interest resulting from the high rates of consanguinity on the Arabian Peninsula. It also strengthens current awareness of the importance of relating cultural background and religious beliefs to medical practice.

Acknowledgetnenfs--I thank Dr F. A. Jabbar, Chief Execu- tive Director, and Dr R. Lambrecht for inviting me to KFSH. I am indebted to Dr P. Ozand for his excellent diagnostic work in the Pediatric unit. I also thank all physicians, nurses, social workers and fellow research workers for their invaluable assistance, and for having invited me on many occasions to present the findings of this study.

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Parental responses to consanguinity and genetic disease in Saudi Arabia