Issues of sexuality in Down syndrome

Don Van Dyke, Dianne McBrien, and Andrea Sherbondy

As human beings, individuals with Down syndrome have the right to emotionally satisfying and culturally appropriate sexual expression. As patients, they have the right to routine reproductive health care provided to the general population. Cognitive and language disabilities may predispose this population to unwanted pregnancy, sexually transmitted disease, and sexual exploitation. Sex education tailored to cognitive level, learning style, and living arrangements is essential to the education of children and young adults with Down syndrome.

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Van Dyke, D, McBrien, D, and Sherbondy, A. (1995) Issues of sexuality in Down syndrome. Down Syndrome Research and Practice, 3(2), 65-69. doi:10.3104/reviews.53

Introduction

Issues of sexuality accompany each stage of human development. Sexual tasks for infants and pre-school aged children include the development of physical and emotional attachment to parents and other family members; issues for school aged children are the emergence of modesty and privacy as well as the beginning of masturbation [Table 1] (Haka- Ikse and Mian, 1993; Grant, 1995). Personal safety and self-esteem become important issues for adolescents and young adults [see Table 1] (Haka-Ikse and Mian, 1993). Little has been written about psychosexual development in Down syndrome.

There have been major advances in interdisciplinary and preventive medical management of individuals with Down syndrome. Improved medical care, laws ensuring appropriate education and related services for the disabled, and slow changes in societal attitudes have brightened the outlook for individuals with Down syndrome (Van Dyke et al, 1995). An active life, including participation in work, social, and worship environments, is now possible. As individuals with Down syndrome take their place in these areas, we can expect some of them to assume the sexual roles that the general population takes for granted: lover, spouse, parent (Van Dyke et al., 1995). Review of developmentally appropriate sexual issues should be part of the medical and psychological evaluation of all individuals with Down syndrome.

Sexual Development and Behaviour

All individuals, regardless of disability, are sexual beings. Individuals with Down syndrome and other mental disabilities who engage in sexual behaviors, however, may encounter societal prejudice as well as significant parental anxiety (Van Dyke et al., 1995). Historically common sex-segregated living arrangements in now-defunct institutions and the absence of available social outlets point to a societal denial of sexuality in people with disabilities (Edwards, 1988).

The development of a secure sexual identity is a difficult task even in the absence of a physical or mental disability (Grant, 1995). The emergence of sexual behavior in the individual with Down syndrome alarms some parents and caretakers who may rightly fear that their child's cognitive deficit makes him or her especially vulnerable: to unwanted pregnancy, sexual exploitation and abuse, and to sexually transmitted disease.

Masturbation, a rhythmic self-stimulation of the genital area, is a healthy and normal part of self-discovery (Haka-Ikse and Mian, 1993). It may also provide self-gratification; it may or may not prelude sexual intercourse (Monat-Haller, 1992). In some severely mentally disabled individuals, it may also appear as a form of self-injurious behavior (Van Dyke et al., 1995). The incidence of masturbation in individuals with Down syndrome has been reported as 40% in males and as 52% in females (Rogers and Coleman, 1992; Pueschel, 1986). Rates of masturbation are not significantly higher in individuals with Down syndrome than those in the general population (Myers and Pueschel, 1991); reports demonstrate the general population incidence to be 100% in males and 25% in females by the age of 15 (Ertem and Leventhal, 1995).

Sexual Abuse

Numerous experts in physical and sexual abuse acknowledge that the mentally disabled individual is particularly vulnerable to sexual exploitation and abuse (Schwab 1992). In a 1987 study by Elvik et al., 37% of mentally disabled women demonstrated pelvic exam findings deemed consistent with prior vaginal penetration (Elvik et al,1987). Schor's review of 87 non-institutionalized mentally disabled individuals indicated that 50% of mildly disabled subjects had had sexual intercourse (Schor, 1987; Chamberlain et al, 1984). Rape or incest had occurred in 33% of mildly disabled subjects and 25% of moderately disabled subjects (Schor, 1987; Chamberlain et al, 1984). The mentally disabled individual is vulnerable to sexual maltreatment for several reasons: isolation; communication deficits; small peer group; and limited mutual support services (Schor, 1987). Loneliness and frustration may drive an individual to accept any form of individual attention whether it be negative or positive (Schor, 1987). Other contributing factors include multiple living situations and transient caregivers, some of whom may be paedophiles. (Schor, 1987)

Personal safety education should begin early in childhood (Haka-Ikse and Mian, 1993). Young children and those individuals with Down syndrome who have severe cognitive or language impairment may learn best from a good touch/bad touch model : "Just say no to unwanted advances or touching" (Haka-Ikse and Mian, 1993; Monat-Haller, 1992). Older children and individuals with mild language and cognitive deficits may be able to learn the "Circles Concept", a paradigm of physical and emotional distance (Walker-Hirsch and Champagne, 1992). In this paradigm, colored circles represent levels of personal relationship and physical intimacy. Individuals learn appropriate touching behaviors for each circle of intimacy, and are cautioned that "sometimes a friend may want to be closer to you than you want. You must explain to your friend and say "STOP" (Walker-Hirsch and Champagne, 1992)". Like typical young people, then, students with Down syndrome need early sex education accompanied by open discussion Because of significant variations within this population in cognitive levels, learning styles, living arrangements, and health problems, they require an individualized approach to sex education (Van Dyke et al, 1995).

Dating

There are no studies of dating patterns or related social behaviors in Down syndrome. Dating is a normal part of adolescent social development (Fegan et al, l993). The social skills needed for dating can be taught as part of a life-skills based curriculum (Fegan et al, l993). Such preparation is best provided before the actual dating experience. Those who doubt that dating occurs in the Down syndrome population should read the chapters on "Girls and Sex" and "Marriage and Children" in the book Count Us In: Growing Up with Down Syndrome. Written by two young men with Down syndrome, Count Us In provides eloquent proof that adolescents with Down syndrome have typical teenage concerns:

"And that's part of the big problem to fix that, because I want to have more social experience with dating. Getting together with boys is okay as friends. But girls, getting to know them, I'd be happy to do it. ... But, I would like to keep my experience up by dating again and now I'm making decisions of what my values are and what kind of things and responsibilities for me to do in the future of marrying Tami. It's premature but it's okay... after you have the experience to decide if you want to get married."

Marriage

Few papers have described marriage in the Down syndrome population. Studies of marriage in the mentally disabled population by Edgerton (1983); and by Koller et al (1988) have shown that individuals with cognitive deficits who married had only mild mental disabilities. The most troubled marriages were those in which both partners were mentally disabled (Koller et al, 1988). Of 38 married individuals with Down syndrome including 35 female subjects, all had spouses without Down syndrome (Edwards, 1988). In this study, couples lived in an environment that was closely and consistently supported and in some cases supervised by other family members or advocates (Edwards, 1988).

Reproductive Issues

In general, adults with Down syndrome under-use the health care system (Elkins et al, l987). Women with Down syndrome demonstrate significantly lower usage of gynecological and reproductive services than women in the general population (Elkins et al, 1987). While it is recommended that all females with Down syndrome have a baseline pelvic examination and pap smear between 17 and 20 years of age, this recommendation is infrequently followed (Rogers and Coleman, 1992). To address this problem, some care providers have developed special clinics for adults with Down syndrome (Doty, 1995). Men with Down syndrome need to learn testicular self-examination as their cognitive level permits; likewise, women need to learn breast self-examination and the necessity for regular gynecological care (Elkins et al, 1987; Doty, 1995).

Contraception is often a major concern of parents of individuals with Down syndrome (Van Dyke et al, 1995). Parents may be uncomfortable discussing sexual and reproductive issues with a physician (Van Dyke et al, 1995). Health care providers and professionals need to initiate structured, nonjudgmental discussion of contraception and to provide clear information tailored to patient developmental levels (Grant, 1995).

Methods of contraception in males and females with Down syndrome are outlined in Table 2. As in the general population, the only nonsurgical method available to males is the condom. Because barrier methods require application at the time of each coitus, they may not be practical for individuals with Down syndrome who may need supervision in using such devices (Elkins, 1990). There are a number of contraceptive methods for females including condoms, spermicidal foams and gels, diaphragm, sponges, cervical cap, IUD, oral contraceptives, Norplant, and Depo-Provera. No form of contraception is totally contraindicated for individuals with Down syndrome (Schwab, 1992).

Oral contraceptives are frequently used by females with Down syndrome. Contraindications for this therapy in Down syndrome are the same as in women in the general population; both absolute and relative contraindications are outlined in Table 3 (Heaton, 1995; Laros, 1993).

Medroxyprogesterone (Depo-Provera) and Norplant (levonorgestrel implant) are frequently used depot forms of contraception. In one study, 50% of women receiving Depo-Provera were amenorrheic; this therapy may considerably simplify menstrual hygiene (Laros, 1993). One year of quarterly Depo-Provera injections costs approximately $200 (Laros, 1993). Norplant is a progestin-impregnated silastic implant that is usually inserted into upper arm subcutaneous tissue. It is usually effective for five years but may be removed sooner (Laros, 1993). Reports of complications requiring removal - including irregular vaginal bleeding - have dampened some of the initial enthusiasm for Norplant. Cost is estimated between $300-$400 a year (Laros, 1993).

Surgical contraceptive procedures include laparoscopic tubal ligation and total abdominal hysterectomy. Both procedures require informed consent and involvement of the patient and parent. A helpful reference regarding sterilization issues appears in the 1988 publication by the American Academy of Obstetrics and Gynecology on Ethics. The process of obtaining a sterilization procedure can be emotionally draining and financially burdensome for parents (Van Dyke et al, 1995).

Sexually Transmitted Disease

It is important to remember that the condom is the only contraceptive method available which also helps prevent the transmission of disease. Consistent and correct use of barrier methods such as condoms may be not practical in the Down syndrome population (Elkins, l990). Individuals with Down syndrome may be at increased risk for sexually transmitted disease (STD). Transmission risks for some STDs are outlined in Table 4. A 50-90% transmission risk of gonorrhea has been documented (Stone,1994). While no reports estimate the incidence of HIV infection in Down syndrome, HIV has been reported in the mentally disabled population (Kastner et al, 1989). Clearly, other sexually transmitted diseases including syphilis, chlamydia, herpes simplex, venereal warts, and hepatitis B are potential concerns in the sexually active individual. In a national study by Marchetti et al (1990), a total of 45 developmentally disabled individuals in 11 states were identified as having HIV infection (Marchetti et al, 1990). Risk factors associated with HIV infection in Down syndrome are identical to those in the general population and include heterosexual activity, homosexual activity, IV drug abuse, and contaminated blood transfusions (Simonds and Rogers, 1992).

Pregnancy

Significantly impaired fertility of both sexes is evident in the Down syndrome population (Rogers and Coleman, 1992). While males have long been assumed to be sterile, Sheridan reports one case of a cytogenetically normal male infant that was fathered by a man with Down syndrome (Sheridan et al, 1980). Women have impaired but still significant fertility: a number of reviews document women with Down syndrome carrying pregnancy to term and delivering infants with and without Down syndrome (Bovicelli et al, 1982; Rani et al, 1990). Infants born to mothers with Down syndrome are at increased risk for premature delivery and low birth weight (Bovicelli et al, 1982). Pregnancy outcomes obtained from a study of mothers with Down syndrome are displayed in Table 5. Whether a woman with Down syndrome constitutes a high risk pregnancy depends largely on cognitive level and medical status. Obviously, the presence of maternal cardiac, thyroid, or hepatic disease, as well as seizure disorder, complicates a pregnancy. The high incidence of congenital heart disease in any offspring with Down syndrome contributes to pregnancy risk, including stillbirth and neonatal death (Gordon, 1990). Offspring without Down syndrome have a greater than average number of congenital anomalies (Bovicelli et al 1982) (see Table 5).

Parenting

Only scant information is available on the parenting abilities of individuals with Down syndrome (Van Dyke et al, 1995). The issue of parenting by a mentally disabled individual is a controversial one. Some professionals maintain that children of mildly retarded couples are probably no worse off than children of individuals who are "classified as culturally deprived" (Nigro, 1975). Other authors opine that mentally disabled parents may be more likely to neglect their children through acts of omission but that purposeful abuse may be infrequent, especially in the setting of adequate societal and family supports (Tymchuk, 1992).

Summary

In summary, sexuality is an intrinsic aspect of human development. Individuals with Down syndrome need individualized instruction and education to develop appropriate sociosexual behaviors. While individuals with Down syndrome have unique and sometimes complex medical needs, they still require routine reproductive health services recommended for the general population including urological care for men, gynecological care for women, and preventive medical services. Education and counseling to prevent unplanned pregnancy, abuse, and sexually transmitted disease should be part of the routine medical care and education for individuals with Down syndrome. All individuals with Down syndrome have the right to develop and express sexuality in an emotionally satisfying and socially appropriate manner. It is the responsibility of care providers to facilitate communication, research, and support services to accomplish this goal.

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