21
Advocacy Issues: Sexuality
Janalee Heinemann, David A. Wyatt, and Barbara J. Goff
In the past, the primary caregiving concern for those with Prader-Willi syndrome was preventing life-threatening obesity. Although, of neces- sity, this remains the fi rst priority, better understanding and manage- ment techniques leading to signifi cantly lengthened life spans now allows for an additional focus on the quality of their lives. The joint focus on preventing life-threatening obesity through restrictions and limitations, while at the same time attempting to mainstream affected individuals into society, forces many to deal with previously latent ethical and legal issues. The primary issue of sexuality has placed many parents and professionals at odds and piqued new levels of discomfort.
Although sexual maturation in those with PWS is rarely complete, it does not mean that a person with Prader-Willi syndrome is asexual.
Indeed, sexuality is a “state of being” that incorporates gender identity, relationships, intimacy, and self-esteem as well as physical develop- ment and hormones.1 We are all sexual beings, including persons with PWS—who sometimes need intimate contact, sometimes dream about a romantic relationship, and always are infl uenced by what is on TV, read, or observed.
Until recently, it was believed that infertility in this population was universal. Parents, particularly mothers, of individuals with PWS have quipped, “We are the only parents who worry more about our teen and adult children going into their dates’ kitchen than into their bedroom!”
A new reality has emerged. Published reports now document three pregnancies: two young women with the chromosome 15 deletion form of PWS have given birth to children with Angelman syndrome (see Figure 21.1); the third young woman, whose PWS originated from maternal disomy, had a child who appears to be typically develop- ing.2,8,9 Since the ability to adequately parent is unlikely in even the most capable of persons with PWS, most parents and providers do not wish to deal with either of these situations.
This then raises a number of questions: What are the parameters of appropriate and allowable expressions of sexuality? Who decides—
parents or professional caregivers? If sexual activity is allowed, what
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kind of protections should be taken? Who does the teaching? Who does the monitoring? When an adult child with PWS lives away from home in supported care, how are parents assured that protection is really being utilized since many young women with PWS express a longing to get pregnant?
A logical corollary issue is that of marriage or cohabitation. When couples with PWS want to get married or live together, what is their right to have this type of relationship? Is it so wrong for young adults with PWS to want a romantic/sexual life? Some parents ask, “Isn’t it bad enough that they have to spend every day of their lives watching everyone around them enjoying the pleasure of eating? Do they also have to be deprived of this other basic pleasure and need in life?” On the other hand, others ask, “What is their potential for maintaining a long-term relationship?” These are a few of the many questions facing parents and caregivers for which there are no easy answers.
An even more troublesome situation might be when an individual with PWS is romantically involved with someone who does not have PWS. This is not an unusual situation for two reasons. Many individu- als with PWS do not want to be identifi ed by their disability and estab- lish relationships with people who are not as readily identifi able. The other reason is that the person with PWS knows that their non-PWS partner will have greater access to food and money. One parent reported that her daughter was dating a young man she knew through her Figure 21.1. A young woman with Prader-Willi syndrome and her daughter with Angelman syndrome (shown in infancy at left and at 11 months of age at right). Both mother and daughter were diagnosed by the FISH test (fl uorescence in situ hybridization), which identifi es chromosome deletions. A mother with the classic PWS deletion on her paternal chromosome 15 has a 50% chance of contributing a defec- tive chromosome 15 to her child; since the child receives it as a maternal chromosome deletion, the child develops Angelman syndrome instead of PWS (see Part I of this volume for details on the genetics of PWS and AS).
workshop. He did not have PWS and routinely provided her with snacks, either brought from home or purchased with his money. The mother liked the young man and spoke directly to him about the harm he could be causing by supplying her already obese daughter with food. He replied that he understood and he cared very much about the well-being of his girlfriend, but if he didn’t bring her what she wanted, she wouldn’t be his girlfriend anymore. He was quite correct.
While cognitive or emotional capacity is never the defi ning criterion for marriage or parenthood, cognitive capacity does impact the ability to understand right and wrong and allowable and disallowable moral and sexual behavior. A lack of understanding has led to problems of sexual charges against some young people with PWS. One instance involved a mid-30s couple, both with PWS, who had been dating two years. Although they were mutually consenting adults, when the young man’s fondling of his girlfriend caused slight bleeding in her vagina, she was taken to the hospital emergency room, and he was charged with “felony, fi rst-degree sexual assault and battery.” A much better response would have been to provide counseling to the couple with regard to their sexual expression.
Like most issues, the answer to what is acceptable sexual behavior is not a simple one. Some of the intertwining issues are listed below:
• Family’s religious and moral beliefs—The family may be strongly opposed to sex outside of marriage, yet marriage may be out of the question due to lack of maturity and the questionable ability to maintain a long-term relationship.
• Sexually transmitted diseases—How do you protect a vulnerable adult from picking the wrong partner? How can you be assured that they are adequately protected from sexual diseases?
• What is appropriate sexual behavior? Help from the Crisis Intervention Counselor at PWSA (USA) has been sought for situations in which poor behavioral choices have led to legal charges. Who is at fault when a person with PWS observes through peers, movies, and TV that others are getting affection and pleasure in more sexual ways?
Then, wishing to act normally, they subsequently make similar attempts that are unsophisticated—and perhaps socially unaccept- able—and that unexpectedly result in legal trouble.
The Role of the Service Provider
Because residential service providers have a mandated responsibility to address issues of sexuality for adults with PWS, the following are responses, from a provider’s perspective, to the questions posed at the start of this chapter.
What are the parameters of appropriate and allowable expressions of sexual- ity? This is dependent on the individual and his/her desires and capa- bilities. Where there is a question regarding competency to knowingly engage in sexual activity, a provider is responsible to conduct an assess- ment to determine competency and provide supports and supervision accordingly.
Who decides—parents or professional caregivers? Parents, quite natu- rally, wish to guide the lives of their children according to specifi c reli- gious or moral beliefs; providers, while respecting those beliefs, must also adhere to established agency and state standards of care, which include matters of sexuality and sexual expression. While there may never be complete agreement as to what is best for the individual with PWS, there must always be a process and a dialogue that keeps in focus the health, happiness, and fulfi llment of the individual with Prader- Willi syndrome.
Again, if the individual is an adult living in a residential program, competency must be established. If the person is deemed competent, then the policies of the agency and/or state must be adhered to. In some cases, sexual activity is prohibited within the residence itself, but other options may be explored. In other instances, individuals who are deemed incompetent to consent to sexual activity may be required to receive a certain level of supervision if sexual activity is suspected. In most all cases, the agency is obligated to provide sex education com- mensurate with the capacity of the individual(s) to understand and benefi t. This includes the use of protective devices.
What if the parent has guardianship? Having a guardian does not mean that an individual has to abide by their demands regarding sexual expression or lack thereof. The specifi c allowable interventions from a guardian vary from state to state and may, or may not, include infl u- ences into the areas of sexuality and sexual choices.10
If sexual activity is allowed, what kind of protection should be used? Any type of protection to be utilized during sexual activity would need to be medically approved and recommended and be of the type that the individual is most likely to use consistently and effectively.
Who does the teaching? The teaching of sexual information is delivered by the person(s) deemed to be most appropriate. For example, if a person has severe cognitive defi cits, a direct-service staff person with whom the individual has a close relationship and who possesses some level of expertise might be most appropriate. It might be prudent to have an established protocol wherein the staff person would function primarily as a conduit to introduce the individual to a sex educator/
counselor who specializes in these issues. In this way, staff are pro- tected so that accusations of inappropriate behavior may be avoided.
The staff member would reassure the individual that this counselor is safe to talk with in private. A counselor is needed to work on building relationships, not just to discuss sexual activity.
Who does the monitoring? Generally, it is the residential staff that moni- tors any developing romantic or sexual relationships. They do so in order to ensure the safety and well-being of the individuals involved.
Typically, there are agency guidelines to follow and supervisory staff to provide oversight with regard to sexual activity. If a sexual relation- ship is perceived as harmful, it most certainly will be stopped and investigated immediately. If it is assessed to be consensual and not harmful physically or emotionally, then most agencies would seek to provide education and counseling as appropriate.
When an adult child with PWS lives away from home in supported care, how are parents assured that protection is really being utilized, since many
young women with PWS express a longing to get pregnant? If a sexual relationship is being permitted after recommended assessments are completed and appropriate education provided, then the provider agency can only depend on the willingness of the individual to be honest and forthcoming in their discussions of safe sex. If agency staff suspects failure to follow recommended and agreed-upon protective measures, they may prohibit sexual activity until or unless the situation is rectifi ed.
These responses are typical but not refl ective of the practices of every provider agency. It is suggested that parents inquire as to any policies, procedures, or prohibitions a particular agency may have with regard to sexual activity prior to their child’s placement.
The issue of cohabitation or marriage is, again, one to be made on a case-by-case basis. Many agencies will design a residential program that would support a couple choosing to marry. However, such a serious decision would not be supported without a great deal of discus- sion, counseling, and education. Parent support for such relationships is a very powerful indicator of whether or not it will be successful.
Sexual Abuse
Even when parents and caregivers agree on allowable sexual expres- sion, the issue of sexual abuse remains a critical concern that is uncom- fortable to discuss but a reality with which we all must deal. Current statistics indicate that
• In the general population, at least 1 in 4 girls and 1 in 10 boys are molested before age 18.12 The risk is signifi cantly increased for those with disabilities.
• The average age of all molestation is around 8 years. Because males (and less obviously females) with PWS have more childlike genitalia, even adults with the syndrome remain at higher risk for abuse by pedophiles.
• The offender is often the person one would least suspect. National data indicate that the majority of offenders are not psychopathic and many are considered “pillars of the community.”3,6
Adult Sexual Abuse
In a study conducted in Connecticut over a 5-year period and pub- lished in 1994,5 researchers reviewed 461cases involving alleged sexual abuse of adults with intellectual disabilities. Of the 171 (37%) con- fi rmed cases, 48% of those abused had mild mental retardation, 28%
had moderate mental retardation, 15% had severe mental retardation, and 9% had profound mental retardation. Most were able to commu- nicate verbally and had few, if any, secondary disabilities (e.g., blind- ness, physical disabilities). Based on this description, we can see that a higher IQ is not protective.
Other fi ndings of the study included the following:
• 72% of cases involved female victims, and 28% involved males.
• Average age of the victim was 30.
• 88% of perpetrators were men.
• Most abuse occurred in the victim’s residence: institution 25.2%, group home 26.9%, family home 14.6%, community 7%, supervised apartment 6.4%, sheltered workshop 5.9%, other settings 14%.
• 92% of the victims knew their abuser.
Childhood Sexual Abuse
In one study investigating sexual abuse of females with mild mental retardation, 80% reported having been sexually abused at least one time.11 Estimates of sexual abuse against males with developmental disabilities have been as high as 31%.4
In another study of 43 children with global developmental disabili- ties who had been sexually abused, the following statistics were reported7:
• 46% were abused by a family member (compared with 38% for non- disabled children).
• 36% were abused by a nonfamily member (compared with 52% for nondisabled children).
• 18% were abused by both a family member and a nonfamily member (compared with 10% for nondisabled children).
The perpetuation of child sexual abuse is aided silently by two factors: (1) Most children don’t tell, due to fear, guilt, shame, and concern for others; and (2) most parents and professionals don’t suspect—or don’t believe the child when they do tell.
The failure to report occurs with increased frequency in those with cognitive disabilities. Many have insuffi cient language or understand- ing to adequately report. They may not even perceive or experience that what occurred was wrong. Just as often, the perpetrator threatens harm if the abuse is reported. As a result, many demonstrate an acute behavioral deterioration. Possible sexual abuse as the etiology of this deterioration is frequently overlooked resulting in misdirected treat- ment. Often the facts surrounding the sexual abuse emerge much later and frequently after others have also been victimized.
Why Children and Adults with PWS Are More at Risk
Individuals with PWS are especially vulnerable to being sexually exploited and abused for several reasons:
1. An uncritical trust of everyone—They generally have an emotion- ally and socially innocent nature.
2. An increased dependency on others—They lack control over their environment and thus are forced, due to their situation, to place an inordinate amount of trust in those who care for them.
3. An increased vulnerability to anyone who befriends them—Often it is diffi cult for them to socialize and communicate, thus they are more vulnerable to anyone expressing kindness. Many molesters use the
“special friend” and “shared secret” approach.
4. An increased vulnerability to bribes—Anyone who would give food secretly to a child or adult who has Prader-Willi syndrome would
be considered a friend for life, even if this friend was sexually abusive.
5. A normal need for physical attention, often coupled with a lack of adequate sexual education or appropriate outlets—Children with PWS typically receive and give many hugs and kisses. However, many students with PWS are not included in school-based sex education programs, as it is assumed the information is not relevant or will not be understood. As they grow into adolescence, the typical socializing and dating experiences are not available to them, and there may be problems during this period and adulthood with inappropriate sexual behavior.
6. An increased fantasy life—Being more socially isolated than the average child, people with PWS often build up great romantic fantasy lives of which a molester can take advantage. Also, their inability to think abstractly in many cases means they will be less able to make appropriate moral and social decisions.
7. An increased likelihood to be in settings where other adults have control over them—Foster homes, group homes, institutions, camping programs, and other supervised settings are all potential settings for molestation.
Why Parents and Providers Are Also at Risk
The previous section addressed the issue of why children and adults with PWS are at increased risk for sexual abuse. These same character- istics frequently cloud the issue of believability when a person with PWS reports such abuse, since a well-documented characteristic of children and adults with PWS is purposeful lying when angry, in an effort to hurt or get revenge against the perceived offender. And, as mentioned, many have active fantasy lives to compensate for the lack of social and emotional stimulation in their real lives, which can lead to made-up stories about adults that may seem real in their minds. As a result, some parents and providers have been unfairly charged with having committed physical or sexual abuse. It is this combination of increased vulnerability and increased propensity to assert an imagined, self-serving “truth” that makes it diffi cult to sort out the facts when allegations are made.
Conclusion
Providing a quality life for a person with PWS has layers of complexity and ethical issues that are not easily resolved. The rights and respon- sibilities regarding sexuality will continue to require a combination of an open mind, common sense, and ongoing dialogue among parents, caregivers, and most importantly, the person with Prader-Willi syndrome.
Perhaps the primary question remains: Who has the right to make these decisions? As with the general population, when considering the issue of sexuality for those with PWS, every parent, every caregiving agency, every ruling judge, and indeed, every culture and every
involved person has a different opinion regarding what is fair and appropriate and correct. Ultimately the decision must hinge on “what is best” for the person with PWS. And, just as often, what is best may not be what is “wanted” by that person or easiest for those providing care.
This chapter has dealt briefl y with several dimensions of the sexual- ity issue: the need to recognize the facts and fi ctions surrounding our beliefs about sexuality and people with PWS; the increased risk for abuse associated with those with disabilities; and the complexity and challenges of supporting individuals with PWS in fulfi llment of their hopes, dreams, and wishes. There are many ethical and legal questions surrounding the issue of healthy sexuality and sexual expression for those with PWS. Where care is shared between families and providers, both parties must come together with the individual to determine what makes sense for that person at a particular point in time. There are no easy or right or wrong answers—no simplifying blacks or whites—just shifting shades of gray.
References
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4. Finkelhor D. A Sourcebook on Child Sexual Abuse. Newbury Park, CA: Sage Publications, Inc.; 1986.
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Mental Retardation. 1994;32:173–180.
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