Friday, 5 April 2019

Sexuality in PWS

I often hear the question about whether women with PWS can have babies.  The answer is, yes, they can, but it is not common.  Babies to women with PWS have been documented in Scandinavia, USA, and New Zealand.

IPWSO's Clinical & Scientific Advisory Board has written an article on sexuality and fertility in PWS which you can see on our website, which has the technical explanation, but it is important to understand how sexuality might be interpreted by the person with PWS.
Mother (PWS) and baby (AS)

The CSAB article says,

"Romantic relationships may be perceived in many different ways by persons with PWS, and it is important to discern fantasy from a more actionable situation. Some may claim to be engaged to be married and indicate that a marriage date has been set, even though they have minimal contact with or personal knowledge about the identified person. Others may have a real girlfriend or boyfriend and describe a dating relationship of variable intensity; they may be content to display affection through hand holding and kissing without actual sexual contact. A few may enter into what can be described as a serious relationship that might include sexual intercourse.

"First, it is important to evaluate their level of understanding of sexual function and “where babies come from.” Second, their understanding of the rules and responsibilities, boundaries, and personal safety around sexual behaviour must be explored. Finally, their knowledge of the risks of sexually transmitted diseases must be assessed. "

Equally important is the need to ascertain whether sex is being exchanged for food or whether the person with PWS is being taken advantage of sexually. If this is discovered, a referral to the proper agency for investigation is required.  Finally, individuals with PWS often display interpersonal conflict in their relationships. They may have difficulty sharing, meeting the expectations of others, or making empathic connections. Their level of emotional maturity may not be conducive to the level of judgment, commitment, and intimacy necessary in a marriage, even  with the help of parents or guardians.

Many young adult men and women with PWS will express a desire to have a baby. In most cases, this idea comes from an earlier psychological stage of the family romance, such as playing with dolls. This wish can be acknowledged, discussed, and addressed through counselling. It is often managed through substitution, such as caring for a pet, being involved in the lives of nieces and nephews, being directly supervised in activities with small children, or accepting that not all adults in society have children. When the desire for a baby is complicated by actual fertility, it may be difficult for the woman with PWS to accept the use of contraceptives. In these cases, anticipatory guidance requires the involvement of the parent or guardian. Family, religious and social values should play a major part in these discussions. At least one abortion has occurred.

Articles and narratives of the five pregnancies in women with PWS suggest that the gestations and deliveries were uncomplicated. Three babies were delivered by planned caesarean sections. All of the mothers were unable to breast feed and bond with the children, and the infants were cared for by others, most often relatives. Common for all was also an incapacity to put the needs of the child ahead of their personal needs and desires, especially about food. As described in one case “As long as the baby didn’t cry or want to be fed when the mother wanted her own meals, all was fine”.

"The genetics of the children born to mothers with PWS has varied. Theoretically, the chances of a fertile woman with PWS having a healthy baby depend on what genetic type of PWS she has. A woman with maternal uniparental disomy (UPD) is likely to have a healthy baby, everything else being equal. A woman with a deletion has a 50% chance of having a baby with Angelman syndrome.

There are no known cases, yet, of a male with PWS fathering a child."

Studies have also shown that in spite of hypogonadism being a major feature, sexual interest and activities are common.  Treatment with sex steroids may increase this. A low level of testosterone and inhibin B (produced by the testis) and an imbalance in other hormones (LH and FSH) mean that fertility in males is very unlikely.

In women with PWS the same hormonal pattern was found, but in a few women the hormones were normal which indicated fertility was normal.  The Israeli study went on to say that "treatment with sex steroids in both genders would decrease fertility further.

There are many women and men with PWS who have very affectionate and caring relationships with each other, or with a person without PWS.  Many of these relationships are long-lasting and very important to them.  Some people with PWS marry and live with support from carers, and many are just content to be able to say they have a "boyfriend" or "girlfriend".  There are many who have an active sex life, both males and females.  Our job is to support them in their choice.


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