Gynecologic Issues in Adolescents with Developmental Delay
Elisabeth H. Quint
Susan D. Ernst
Introduction
Adolescence is a time of transition for all teens and their families. For families with teens with developmental disabilities, the issues of menstruation, hormonal changes, and reproductive concerns can be daunting. Concerns about safety, abuse, and unwanted pregnancy can contribute to a sense of feeling overwhelmed by parents and teens. Health care providers can have a significant and positive impact on the lives of these young women, their families, and their caregivers. This chapter will discuss some of the more common dilemmas faced by teens with developmental disabilities (DDs) and their families. Cases are used to illustrate the complexity of the issues and the importance of the social context in which they occur.
Communication and History
The gynecologic visit with a teenager with DD should be comprehensive. Gynecologic visits for typical adolescents would include a confidential portion to screen for sexuality issues (see Chapters 1 and 2). Confidentiality is often a new and possibly uncomfortable phenomenon for the teen and her caregivers. Teens with cognitive disabilities are often viewed as asexual and not in need of the usual questions and screening for any sexuality issues or risky behavior. However, teens with DDs have sexual thoughts and experiences similar to normally developing teens. Therefore, while often omitted, the need for a confidential part of the history is crucial.
Other important issues to consider when communicating with teens with developmental disabilities include the level of cognitive development and the degree of hearing or speech impairments. The clinician needs to adjust language and teaching to the level of development by using basic language and illustrations. Patients with hearing impairment will have a preferred mode of communication other than speech, such as written communication, lip reading, or sign language, and may require an experienced interpreter. Patients with speech impairments may use letter boards, picture communication, eye gaze systems, or computer-based assistive technology devices.
When obtaining a history, the clinician needs to be sure to address the desired outcomes and goals of the visit, both with the patient and her caregivers. Menstrual cycles and their effects on health and hygiene are discussed. Education to both patient and family may focus on sexuality, safety and puberty, the menstrual cycle (e.g., mood, seizures), and/or the need for contraception or menstrual suppression.
Physical Examination and Screening
Pelvic examinations in teenagers are less frequently indicated than in the past (see Chapter 1), with Papanicolaou (Pap) testing beginning at age 21 and noninvasive screening for sexually transmitted infections (STIs) available (1,2). To visualize the external genitalia, the frog-leg position can be helpful. Other positions include lying on one side or having an attendant lift both legs together up straight, since abducting the legs may cause a strong reaction in some patients. If a patient cannot be examined due to communication or physical issues, an examination under anesthesia may be indicated, but only if the examination is imperative for the diagnosis. If a Pap test is indicated and the patient cannot tolerate a speculum examination, a “blind” Pap test can be done. In this technique a gloved finger is inserted in the vagina to locate the cervix and a cytobrush is guided over the finger to the cervical os to collect the sample. Although lower rates of endocervical cells are found, this still can lead to satisfactory Pap tests (3).
Human papillomavirus (HPV) vaccination should be considered in all women ages 9 to 26 years (4) and should be discussed with all parents and teens with DDs. Although their risk for cervical cancer may be lower than that of the general population, women with DDs may not be able to tolerate screening with Pap tests at the recommended frequency and the vaccination can decrease the potential risk for cervical cancer (3).
Education
Case scenario: A 14-year-old teen with Williams syndrome and mild cognitive impairment is brought by her parents to the clinic to discuss her menses and talk about contraceptive options. She is verbal and is a very social young woman. She attends middle school and is integrated in some regular education classes. For extracurricular activities she enjoys soccer and horseback riding and is a singer in a band.
An important component of the evaluation of adolescents with DDs is the assessment of knowledge regarding puberty, menstrual hygiene, sexuality, and self-defense against sexual abuse. This includes inquiry regarding her ability to accurately identify body parts, male and female; her ability to perform her own toileting hygiene; her understanding of the basic mechanics and consequences of intercourse; her ability to distinguish good touch from bad touch; and her understanding of appropriate social interactions. If she expresses an interest in sexual activity, her ability to provide informed consent should be assessed. These questions could help direct educational efforts in the clinical setting and lead to appropriate referrals to resources that may be used in the home and school for continued education.
Many adolescents with DDs and their families visit their clinician or a specialist for counseling and education even prior to the onset of puberty to help prepare for this change (5,6). Families often seek information about how the adolescent with a DD might predictably respond to the physical, psychological, and hormonal changes associated with puberty. The literature suggests that puberty is normal in sequence and timing for the majority of adolescents with developmental disabilities. However, there are some exceptions (5). Girls with neurodevelopmental disabilities are 20 times more likely to experience premature sexual development (7). Girls with autism spectrum conditions may experience a slight delay in the onset of menarche (8), while adolescents with cerebral palsy may have a slightly earlier onset of pubertal changes (9). Girls with Down syndrome seem to have an average age for menarche (10,11). Individuals with Prader-Willi syndrome have some early pubertal development that is characterized by normal adrenarche followed by pubertal arrest (12).
Initially many teenagers will have irregular cycles, even up to 5 years, due to the immaturity of their hypothalamic–pituitary–ovarian axis (13). In teens with DDs there are additional factors that may affect menstrual cycles. For example, adolescents with Down syndrome have a higher incidence of thyroid dysfunction (14), and women with epilepsy are more likely to have menstrual cycle abnormalities and polycystic ovarian syndrome (15). Valproic acid in some studies has been associated with the development of polycystic ovarian syndrome (16), and other medications, including haloperidol, risperidone, and metoclopramide, can lead to hyperprolactinemia and amenorrhea (15). Cervicitis from Chlamydia causing irregular bleeding can be overlooked in teens with DDs. Adolescents with communication difficulties may present with behavioral challenges at the time of puberty because of limited ability to express emotional changes and pain that are often associated with menses (17). Adolescents with disorders affecting mobility and fine motor control may have trouble with menstrual hygiene due to inability to manipulate pads or tampons.
General teaching of adolescents with DDs should begin before the onset of puberty (18). The use of simple terms and repetition of subject matter is important, as is coordinating educational efforts with parents, schools, and community groups. Using a variety of different teaching aids, including pictures, anatomically correct dolls, or videos, is recommended.
The discussion starts with the basic information of anatomy for both boys and girls. An explanation of where the menstrual blood comes from and reassurance that this bleeding is considered healthy and normal is important. Hygiene education should include wiping of the genital area from front to back, changing pads on a timed schedule, changing pads and clothes when soiled, and general hand washing and bathing. A mother or sister may be able to model menstrual hygiene, which can provide reassurance, normalize the menstrual cycle, and reinforce the hygiene information. In general, adolescents who manage their own hygiene for toileting can also be taught to master their own menstrual hygiene.
Sexuality Education
Sexuality education is necessary but often neglected for adolescents with DDs. Multiple studies indicate that 50% to 80% of individuals with DDs do not receive sexual education in their schools (19,20,21). In addition, only 28% to 39% of family members are reported to have discussions regarding sexuality with individuals with DDs (22). Recent research indicates that adolescents with mild cognitive impairment have similar rates of sexual attraction and sexual experience as normally developing peers. However, adolescents with DDs have been shown to have higher rates of unprotected intercourse, lower rates of contraceptive use, higher rates of unplanned pregnancy, and higher rates of STIs (23). These data emphasize the importance of addressing sexuality education in the clinical setting and suggest that resources for continued education should be supplied both at home and at school.
Individuals with mild to moderate cognitive impairment need straightforward information regarding the mechanics of sexual activity, male and female anatomy, public and private body parts, and public and private activities. The clinician or educator should review self-stimulation or masturbation and suggest appropriate places where this may be performed. The difference between “good touch” and “bad touch” and appropriate social boundaries and interactions should be reviewed along with a detailed discussion of pregnancy, contraception, and prevention of sexually transmitted infections.
For teens with more severe cognitive impairment, sexuality education may need to be further simplified. Because they cannot understand the risks and consequences of their behavior, it is unlikely that these individuals would be able to consent to sexual activity, and as a result they are also subject to coercion and intimidation (21). One technique for teaching social boundaries is the “circle technique” (24): this technique places the adolescent with a DD in the inner circle and teaches where people belong from inner to outer circles in order of familiarity and the appropriate behavior that goes with that (e.g., hug relatives, shake hands with friends, no touching by strangers).
Unfortunately, many characteristics of individuals with DDs predispose them to physical and sexual abuse. They are dependent on others for many personal activities of daily living such as toileting and hygiene. They can be affectionate or of a loving nature and have learned compliance with requests. They may also have physical challenges that leave them vulnerable and not mobile and they may have decreased communication skills (25). Rates of sexual assault in women with DDs vary from 25% to 99% (26,27,28). Two recent studies in Massachusetts estimated that 35% of women with disabilities report sexual assault and 88% of these women know their perpetrator (29). Several studies have shown that individuals with DD may be taught self-defense skills to prevent sexual assault (30,31,32): how to recognize unwanted touch, how to verbally refuse or physically remove oneself from an unwanted situation, and how to report the incident to a trusted adult. This basic assault prevention model has been referred to as “NO, GO, TELL” (33). These basic concepts may be difficult for those with cognitive impairments and limitations in mobility and communication skills. Saying “no” or removing oneself from an unsafe environment requires both a strong enough sense of self to defy an adult or authority figure and the physical ability to escape. Telling a trusted adult requires communication skills that demand an understanding of anatomy and sexual acts. Clinicians, parents, and caregivers need to be vigilant in looking for signs, symptoms, or behaviors that may be indicative of sexual assault and promote comprehensive prevention programs.
Case resolution: Education was done with the teen and her parents about her menstrual cycle and her ability to cope with menstrual hygiene and it appeared that she was tolerating her menses well. Her parents were concerned because this teen is so social that she would potentially be persuaded to engage in sexual activity. We assessed her understanding of anatomy, public and private body parts, and sexual activity; her interest level in boys; her ability to distinguish appropriate and wanted touch from inappropriate touch; and her ability to refuse unwanted advances. We used multiple tools including anatomically correct dolls, diagrams, and pictures to assist in the evaluation of knowledge and education. A sexual health counselor experienced with working with adolescents with DDs taught her the basics for sexual assault prevention and resources were given to her parents for further education at home. Her family decided to wait on contraception as the teen was not interested in sexual activity and they felt comfortable with her ability to refuse an unwanted advance.
Medical Issues
Menstrual Concerns
Teens with DDs who present with environmental, behavioral, or medical issues related to the menstrual cycle should be carefully evaluated and the goal of treatment defined. If a teen comes in with the complaint of heavy or abnormal bleeding, there may be health concerns such as anemia. However, other considerations, including desire for menstrual cycle suppression or effects on overall quality of life, may also be important.
Case: A 16-year-old girl with moderate developmental delay of unknown etiology comes in with her mother. She has had periods for 1 year and they are monthly, lasting 3 to 4 days, of moderate flow. She goes to the regular school system and has an aide in the classroom. She does her own toileting. A problem arises when she will not allow the aide to help her with her pads. However, she keeps forgetting to put a clean pad on after she goes to the bathroom and that causes accidents. The school now wants her to stay home on the days of her periods. Her mom is interested in stopping the periods altogether.
Menstrual Manipulation
Complete amenorrhea is difficult to obtain with any method. Often hormonal manipulation of the cycle will increase the likelihood of unpredictable spotting, which may be even more problematic for the teen than scheduled regular bleeding. Thus, the impact of irregular bleeding needs to be discussed prior to initiating treatment.
Combined Hormonal Contraception
Combined Oral Contraceptives
Combined oral contraceptives (COCs) used cyclically result in less menstrual blood loss in most patients. COCs can also be used on an extended basis in an attempt to reduce the total days of menstrual flow. Two common strategies include the use of 84 or 91 days of hormonal pills with a 7-day break, and indefinite use of continuous hormonal pills. The incidence of unscheduled bleeding with the extended regimen is significant, but decreases over time. In one study 53% of patients reported complete amenorrhea at 1 year and an additional 26% had only spotting that did not require sanitary protection (34). Episodic unscheduled bleeding may be particularly troublesome for a teen with a DD due to possible reliance on others for hygiene care. The solution to the unscheduled bleeding, after pregnancy is ruled out, includes supplementation with intermittent estrogen or institution of a 4-day hormone-free interval in response to persistent bleeding (35). For those teens who have difficulty swallowing or with a gastrostomy tube, a chewable COC is available. Antiepileptic medications that induce the hepatic cytochrome P450 system can decrease its efficacy (see section on epilepsy).
Contraceptive Patch
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