Chapter 549 Gynecologic Care for Girls with Special Needs
Adolescence is a challenging time for all children and their families, but especially for teens with special needs; the hormonal changes occurring and the start of menstrual cycles can profoundly affect the lives of teens and their families. In addition there may be concerns about sexual activity, safety and abuse, and unplanned pregnancies.
Adolescents with special needs can have physical and/or developmental disabilities. They are often seen by society, including their families and care providers, as asexual, and therefore sexual education might not have been provided or considered necessary. Physically disabled teens are as likely to be sexually active as nondisabled teens. The care provider needs to assess the teen’s knowledge of anatomy and sexuality, her social knowledge of relationships, and her ability to consent to sexual activity. Education regarding HIV and other sexually transmitted infections (STIs), disease prevention, and contraception, including postcoital contraception, should be offered at a developmentally appropriate level. Teens with disabilities may be more at risk for isolation and depression during adolescence.
The risk for sexual abuse in teens with disabilities is difficult to estimate. Studies show that teens with physical disabilities are just as sexually active as their nondisabled counterparts but that more of their activity is nonvoluntary. Screening for abuse is mandatory. Abuse prevention education can include the No! Go! Tell! model. For teens with limited verbal capacity or developmental delay, abuse may be very hard to detect. The care provider needs to be vigilant in looking for signs on physical exam, such as unexplained bruises or scratches, or changes in behavior that may be indications of sexual abuse in those adolescents (Chapters 37.1 and 113).
A pelvic exam is rarely indicated in teens who are not sexually active, unless they have vulvar issues such as discharge, irregular bleeding, suspicion for abuse, or foreign body and an external inspection can be performed. A speculum exam is not performed, and if the vagina or cervix needs to be visualized, an exam under anesthesia by a gynecologist should be considered. Testing for STIs can be accomplished by urine testing or vaginal swabs (Chapter 114).
Irregular menstruation is common in teenagers, especially the first 5 years after menarche, due to immaturity of the hypothalamic-pituitary-ovarian (HPO) axis and subsequent anovulation (Chapter 110). Several conditions in teens with disabilities are associated with an even higher risk of irregular cycles. Teens with Down syndrome have a higher incidence of thyroid disease. There is a higher incidence of reproductive issues, including PCOS in teens with epilepsy and on certain antiepileptic drugs (AEDs) (Chapter 546). Antipsychotic medication can cause hyperprolactinemia, which can affect menstruation.
The main issue with menstrual cycles, whether they are regular, irregular or heavy, is the impact of menstruation on the patient’s life and her normal activities. The history should focus on this aspect, and menstrual calendars may be helpful to document the cycles, behavior, and the impact of treatments. Most adolescents who self-toilet can learn to use menstrual hygiene products appropriately.
The evaluation for abnormal bleeding is the same as for all teens. Areas requiring particular attention for the child with special needs are the possible need for menstrual suppression for hygiene or cyclical behavioral issues, like crying, tantrums, or withdrawal, and a request for birth control, especially coming from a caregiver and not from the teen, which requires an evaluation of the teen’s ability to consent and evaluate the safety of her environment.
If after documenting the impact of the cycles on the patient’s well-being (often through menstrual or behavioral charting for several months) the care provider, patient, and family decide on menstrual intervention, several options are available. Menstrual regulation is not different from that in the nondisabled teenager. Menstrual suppression leading to complete amenorrhea is usually difficult to obtain, so treatment goals should be set early on. Infrequent scheduled bleeds may be easier to manage than unpredictable spotting, a common side effect of treatment, for certain patients. Outcome goals can be to decrease the heaviness of flow, regulate cycles to predictable bleeding, relieve pain or cyclical behavior symptoms, provide contraception, and/or obtain amenorrhea.
After treatment has started, continue to monitor cycles, ideally with continued menstrual or behavior calendars. Guidelines for office follow-up include menses that are too heavy (in excess of 1 pad/hour), too long (>10 days), or too frequent (< 20 days from day 1 to the next cycle day 1).
If menorrhagia or dysmenorrhea (occasionally leading to cyclical behavior changes in nonverbal teens) is the main concern, the patient can be started on nonsteroidal anti-inflammatory drugs (NSAIDs). These can decrease the flow by up to 20% in adequate doses and can be used alone or in combination with other treatments.
Cyclical oral contraceptives usually lead to regular, lighter cycles. Extended cycling through the use of continuous use of oral contraceptives can suppress cycles, with amenorrhea rates improving with time. Some unpredictable spotting is usually unavoidable, and often teens with special needs prefer to have predictable cycles several times a year. A chewable oral contraceptive is available for those with swallowing issues.
The contraceptive ring is usually used in a pattern of 3 weeks on and 1 week off, but it can be used in a continuous 4-week pattern, which can lead to less bleeding. However, the contraceptive ring may be difficult to use if the teen cannot place it herself, and help with placement has clear privacy issues.
The patch can also be used in a continuous fashion. Some teens with developmental disabilities remove their patch erratically, and placement out of reach (e.g., on buttocks or shoulder) is advised. Pharmacologic data indicate that estrogen exposure is higher for the contraceptive patch than for oral contraceptives or the vaginal ring. It is unclear exactly how this might affect the risk of deep vein thrombosis (DVT), especially in nonmobile women.
Immobility per se is not a contraindication to estrogen-containing contraceptives. However, there are minimal data on the risk of DVT in immobile teens in wheelchairs with or without extraneous estrogen. Obtain a thorough and extended family history for hypercoagulability before initiating estrogen therapy. Careful use of lower-dose estrogen preparations may be advisable, and 3rd-generation progestin combinations and the patch should only be used if other methods have failed.
Intramuscular medroxyprogesterone acetate (DMPA) has long been very useful in menstrual suppression. Two issues are particularly relevant to teens with disabilities. Studies on a decrease in bone density associated with longer-term use of DMPA and a black box warning by the FDA have raised concerns about use of these products in young women, although recent data indicate that the lowering of bone density improves after the medication is stopped. For teens with mobility issues or those with very low body weight who are already at risk for low bone density, this can be of more concern. The second issue is weight gain, especially in obese teens, which can lead to health and mobility issues. If long-term DMPA is considered for a specific patient, calcium and vitamin D supplementation is recommended and bone density could be measured after several years of use.
Continuous oral progestins can also be very effective to obtain amenorrhea. These include the progesterone-only pill and other progestins used daily, such as norethindrone 2.5 mg or micronized progesterone 200 mg.
The progesterone IUD for off-label use of diminishing menstrual flow is not contraindicated in teens, but might require anesthesia to be inserted if the exam is very difficult due to contractures or a narrow vagina. Checking for strings in a clinic setting may be challenging; however, the IUD location can be confirmed by sonography. There may be a significant amount of irregular bleeding in the first several months, but there is 20% amenorrhea after insertion and 50% amenorrhea after 1 year of use.
Surgical procedures such as endometrial ablation and hysterectomy are available for treatment in adults, but they should only be used in very extreme situations for teenagers where all other methods have failed and the patient’s health is severely compromised by her cycles. Ethical considerations and consent issues are difficult in these situations, and state law varies on this topic.
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