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SEXUALITY AND MOVING INTO ADULTHOOD: INFORMATION CAN EASE TRANSITIONS


Sexuality can be a difficult subject to discuss within any family. For parents who have a child with CdLS, it can be intimidating and fall to the wayside when numerous medical issues need immediate attention. However, most older children, regardless of their level of development, will at some point begin to express their sexuality. People with CdLS will experience physical changes as well as behavioral/social issues as they become adults that require guidance and support from their caregivers.

Three professionals from our Clinical Advisory Board share their expertise regarding sexuality and the transition into adulthood for people with CdLS.
Antonie Kline, M.D., Howard Levy, M.D., and Natalie Blagowidow, M.D. have all participated in CdLS clinics for adults and adolescents and have advised many families supporting their children through puberty. Following, they discuss physical issues for both girls and boys, as well as social issues for mildly, moderately and severely involved young adults.

The menstrual cycles are usually regular, and normal in length and flow. Painful menses can occur, and can be treated with anti-inflammatory medications such as Advil or Anaprox. The birth control pill is very effective in relieving menstrual cramps.

Premenstrual Syndrome (PMS) is a common complaint, leading to worsening behavior problems in some. It may be necessary to adjust medications targeting behavior during the premenstrual portion of the monthly cycle.

Menstrual cycles are sometimes heavy or may create a hygiene challenge for caretakers. The birth control pill will make flow lighter, and there are recent formulations (for example, Seasonale) which allow only one menses every three months. DepoProvera, a hormone injection given every three months will stop menstrual flow. This medication can also help reduce seizures, but is associated with bone thinning while taking it. The Mirena IUD provides release of hormone just to the uterus, and thus has very few side effects. All of these options are effective birth control methods. Some caretakers opt for surgical procedures to avoid menses and birth control challenges. This option should only be considered when medical treatment has been unsuccessful.

Gynecologic exams with Pap smear are recommended to start by age 21, and sooner in those who are sexually active. The pelvic exam can be conducted under sedation or anesthesia in women who have difficulty cooperating with the exam. This can be scheduled when another procedure with anesthesia is already planned. Ultrasound examination can also provide helpful information in some cases. It is very common for boys with CdLS to be born with undescended testes, meaning the testes are found low in the pelvis or in the groin, rather than in the scrotum.

There is a small chance that undescended testes can become cancerous, so they should always be surgically repaired within the first year or two of life. Some boys have hypospadias, in which the urethra (the opening for urine to come out) is located somewhere along the underside of the penis, rather than at the tip. This should usually be surgically repaired in order to improve toileting skills. It is also common for boys with CdLS to have a smaller than average penis, but this usually doesn’t cause any problems.

Most boys do go through puberty, although it might be a year or two later than expected. Some do not develop full secondary sexual characteristics, such as hair growth in the armpits and beard area. There is often an increase in aggressive and/or self-injurious behavior around the time of puberty.

Low testosterone (hypogonadism) is found in some men with CdLS, even those who have two normallysized testes. Among other things, hypogonadism can contribute to dry skin, high cholesterol, weight gain, low bone density, and depression. Testosterone supplements can be prescribed, and probably improve most of those symptoms. Testosterone supplementation, however, will also tend to increase aggressive behavior and sexual drive, which may be problematic. There are alternative ways of treating dry skin, high cholesterol and low bone density, when necessary.

Depression can be addressed with a psychologist or psychiatrist.

There appears to be a tendency of premature aging in CdLS, and thus symptoms of prostatic hypertrophy (prostate enlargement) might occur at a younger age (perhaps as early as the 40s) than in the general population (usually in the 50s or older). Prostate enlargement is a very common problem for all men as they age. As the prostate gland grows, it begins to compress the urethra and obstructs the urine flow. Common symptoms include difficulty starting to urinate, reduced force of the urinary stream, dribbling at the end of urination, and needing to urinate multiple times overnight.

Testosterone stimulates prostate growth, and it is possible that men with hypogonadism might have a reduced risk of symptomatic prostatic hypertrophy. 

Hormonal changes in children with CdLS may occur slightly later than in their unaffected peers, and, in a rare minority, may not occur at all. As the body undergoes physical changes under the influence of changing hormones, the adolescent with CdLS will experience emotional and behavioral changes. This is true both for those children who are severely involved as well as those who have mild involvement.

MODERATELY TO SEVERELY INVOLVED

The child with CdLS who is moderately to severely involved, in terms of organ system effects and mental development, may have no or little apparent awareness of body changes and emerging sexuality.

He or she will need to be managed and monitored by the parents or caretakers. Maintaining usual routines is of prime importance to reassure a child in this transition. As for many adolescents, self-touching and/or masturbation may increase, and it is important for parents and caretakers to acknowledge the need for this but stress the importance of privacy. Some individuals may start undressing in public places or using inappropriate touching or words with others. It is advised to encourage only specific places (for example, bedroom or bathtub) for these behaviors. Many parents have shared that the use of overalls, fastenings in the back, or specific clothing that makes access to “private parts” more difficult can be very helpful.

A rewards system in stressing good behaviors may also work to help children maintain appropriate dress and behavior in public. In terms of emotional issues, aggression may worsen or develop.

Anxiety and depression may also begin or increase. Frustration, just as during the toddler years, typically leads to or compounds many of these emotions.

Counseling can be helpful during this time and, for some, medications may help with stability. By late adolescence and early adulthood many of these “difficult” behaviors abate.

"A rewards system in stressing good behaviors may also work to help children maintain appropriate dress and behavior in public. In terms of emotional issues, aggression may worsen or develop. Anxiety and depression may also begin or increase.”

MILDLY INVOLVED

The child with CdLS who is mildly involved may have an appropriate or heightened awareness of his/her differences, particularly in comparison to peers. Middle school is the time when differences can become more apparent. If puberty arrives later than most, height will remain shorter and body changes will not become evident until much later than peers, sometimes creating a sense of isolation. Lack of a sense of personal hygiene can be more obvious in a middle school setting and compounded if puberty arrives later. The sexual and social needs of the adolescent will be present and could lead to increased anxiety about all of these changes. There is a need for the child to be educated as soon as possible about future changes and other issues, or social isolation could be detrimental. Personal safety may become an issue as well.

Young women need support to be protected from both disease and pregnancy in the event of sexual maturity and should learn about birth control options as they age.


Tonie Kline

Three professionals from our Clinical Advisory Board share their expertise regarding sexuality and the transition into adulthood for people with CdLS.
Antonie Kline, M.D., Howard Levy, M.D., and Natalie Blagowidow, M.D. have all participated in CdLS clinics for adults and adolescents and have advised many families supporting their children through puberty. Following, they discuss physical issues for both girls and boys, as well as social issues for mildly, moderately and severely involved young adults.

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Last modified by Gerritjan Koekkoek on 2022/05/31 16:35
Created by Gerritjan Koekkoek on 2021/11/01 11:50

                                                                                                                                                                                                                                                                                                                                                                                                     

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