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Adolescent Gyn Series

Menstrual Management in Adolescents With Developmental Disabilities

Elisabeth H. Quint, MD; Anita Schwandt, MD

The pubertal girl with developmental delay has many of the same concerns as her nondisabled counterpart, but also some unique aspects to her personal situation. For some the transition is smooth, for others it is more troublesome.

The teenager with developmental disabilities needs attention to hygiene issues, menstrual irregularities, and the use of hormonal medications as indicated. In addition, care must be taken to provide optimal therapeutic options, respect the patient's autonomy, and enable her to participate in decision-making as much as possible.

EPIDEMIOLOGY

The age at menarche has remained relatively stable in the United States at around age 12.4 years.1 In girls with developmental delay, studies have found both earlier and later menarche than the general population.2,3 With regard to follicle-stimulating hormone (FSH) and luteinizing hormone responses to gonadotropin-releasing hormone stimulation in teenagers with developmental disabilities, an impaired response of the FSH-secreting pituitary cells in the initial pubertal stages seems to disappear during further sexual development.4 Ovarian sensitivity to FSH may be blunted in patients with Down syndrome, possibly due to lower growth hormone concentrations.5 Although one study found that eumenorrheic women with Down syndrome may be more prone to oligo-ovulation and luteal-phase defects,6 this could not be confirmed.7

While no data suggest any major differences in the cycles of women with developmental disabilities, other factors may contribute to menstrual irregularities. Women with epilepsy have an increased incidence of reproductive endo-crine disorders,8 and many anticonvulsants (except valproic acid) promote more rapid clearance of steroid hormones--which may lead to impaired contraceptive efficacy. Neuroleptics and metoclopramide can cause hyperprolactinemia with irregular bleeding, and occasionally hypoestrogenemia, leading to amenorrhea. Thyroid disease is common in women with Down syndrome, which can disturb the normal menstrual cycle.9,10 Low food intake or swallowing problems with placement of gastric tubes is common in the mentally disabled, resulting in low-weight hypotha-lamic hypogonadism and oligomenorrhea or amenorrhea.

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HISTORY

At menarche, the patient, her family, or caregivers often present with a request to help with or eliminate menses. A thorough menstrual history is essential to address the underlying concerns--eg, hygiene, menorrhagia, dysmenorrhea, pregnancy prevention, and/or sexual abuse. In terms of menstrual hygiene, patients who can manage their own toileting can usually be taught to use pads and other sanitary products.

The level of handicap determines whether the patient can express her own concerns. Sexuality must be addressed through counseling and appropriate services, overcoming the common tendency to regard the patient with disabilities as asexual.11,12 Other important topics for discussion include ability to want and consent to a sexual relationship, contraception, and risk of sexual abuse and sexually transmitted infections (STIs).

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GYNECOLOGIC EXAMINATION

Pelvic examination is seldom required in a teenager who presents for menstrual issues, unless STI testing is indicated. If polycystic ovary syndrome is suspected, a general physical examination should be performed to look for hyperandrogenism and clitoromegaly. Papanicolaou testing in this population should be initiated at age 21 years if there is no history of sexual activity, or within 3 years of the start of sexual activity.13

Abnormal bleeding in teenagers with developmental disabilities should be tracked with a menstrual calendar to document the scope of the problem. Irregular anovulatory bleeding due to an immature hypothalamic-pituitary-ovarian axis is very common during the first 2 postmenarchal years, and may persist for up to 5 years.14,15 Reassurance is often all that is needed. If the bleeding is excessive or leads to hospitalization, a thorough work-up is indicated for thyroid function, prolactin levels, and bleeding disorders.

Treatment can be considered if the bleeding leads to anemia, compromises quality of life, affects daily activities, or causes significant discomfort. Structural abnormalities are very uncommon in teen-agers, but ultrasonography may be helpful if standard treatments do not resolve the problem and the endometrial thickness is needed to decide between estrogen- or pro-gesterone-dominant treatment.

Given the potential problems with patient communication in this population, it is important to have good documentation of the menstrual cycle prior to initiating any treatment so that calendars can be used to assess efficacy. Before prescribing any hormonal treatment, the clinician should consider that such intervention may lead to life-long treatment in patients who may experience changes in supervision and less continuity of care. Periodic evaluation of the treatment plan is advised to prevent the unknown long-term effects of continuous hormone use over ages 30 to 40 years.

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TREATMENT

In teenagers with developmental disabilities and abnormal bleeding, the primary goal needs to be determined: either regulation of periods for menorrhagia and dysmenorrhea or induction of amenorrhea for hygiene concerns or dysmenorrhea (Table).

Table is not available.

TABLE. Treatment Options to Produce Amenorrhea

Nonsteroidal anti-inflammatory drugs can decrease dysmenorrhea and reduce menstrual flow by 22% to 47%.16 The appropriate dosage and dosing interval should be specified and the medication should be started several days premenstrually and continued throughout the entire menstrual period. Efficacy should be assessed at 3 months, with consideration of starting hormones if symptoms persist.

Combined Oral Contraceptives

If regularity or decrease in flow and cramping is desired, low-dose combined oral contraceptives (COCs) are a good choice. Many adolescents live at home with adequate supervision to support daily use. If oral intake is difficult, the pill can be taken with a favorite food. Although there are no data on risk of deep vein thrombosis (DVT) among users in wheelchairs, the chance of DVT is known to be slightly higher for preparations with third-generation progestins.17 Therefore, using a pill with 30 mcg of ethinyl estradiol and a first- or second-generation progestin would seem prudent. Patients using anticonvulsants (eg, phenytoin) may need higher-dose formulations in the presence of breakthrough bleeding (BTB).18 The COCs can be administered cyclically, or continuously by omitting placebo pills to prevent menses.

Combined Contraceptives, Other Delivery Systems

The contraceptive patch has not been studied specifically in this population, but is an alternative in patients without skin disorders. Partial/total detachment occurs at a rate of 3.8%,19 and some patients require daily monitoring so that they do not pull off the patch; placement on the back may help to prevent this. There may be a transient increase in breast tenderness, and contraceptive efficacy may be reduced in patients weighing more than 198 lb.20 The vaginal ring is another option, but issues with placement assistance may arise in a population with limited manual dexterity.

Oral Progesterone

In anovulatory patients, oral progesterone (eg, medroxypro-gesterone acetate, norethindrone, micronized progesterone) can be used for 10 days per month. Dosing is more complicated than for COCs, but cycles can be regulated. Administering the progesterone for the first 10 days of each calendar month may simplify scheduling. Continuous oral progesterone can produce amenorrhea, but may lead to BTB or mood disturbances.

Intramuscular Progesterone

Depot medroxyprogesterone acetate (DMPA), 150 mg intramuscularly every 12 weeks, can also produce amenorrhea. Weight gain related to DMPA use can be 5 to 10 lb per year, which can impair mobility and may make transfers in an inactive patient more difficult.21,22 There is a significant positive relationship between increased body weight on DMPA and obesity at initiation of treatment.22,23

Longitudinal studies have demonstrated a consistent loss in bone mass of 1% to 2% per year in DMPA users, regardless of age. This compares with an annual gain of 9% in bone density among adolescent nonusers,22 so that DMPA use in the adolescent may compromise adult peak bone mass. As women with developmental disabilities often have paralysis, contractures, spasticity, or deformities that decrease mobility, their bone density is significantly lower than age-matched controls.24,25 Neurologic impairment, increased difficulty of feeding, and anticonvulsant use are additional, independent contributors. However, bone density may improve after cessation of DMPA use, with at least partial recovery of bone loss after discontinuation.21,26

Despite concerns about DMPA-related depression in developmentally delayed adolescents with behavior or mood abnormalities, one study showed no statistically significant difference in the mood of DMPA users compared with controls.27

A single-rod, implantable progesterone preparation is forthcoming, with studies indicating 33% amenorrhea and no change in bone density.28 However, insertion may necessitate sedation or anesthesia.

Levonorgestrel-releasing Intrauterine System

The levonorgestrel-releasing intrauterine system (LNG-IUS) has not been described in this population, but may be appropriate for menorrhagia in patients with a normal uterine cavity. The LNG-IUS can produce amenorrhea rates of 44% at 6 months and 50% at 12 and 24 months, with 25% of patients experiencing only spotting.29 Placement may require sedation or general anesthesia. Insertion difficulties may be encountered in those with a small, nulligravid uterus and a narrow internal cervical os, but one study reported equal difficulty of insertion in nulliparous and multiparous women (14.9% versus 15.4%).30

Other Alternatives

Surgical alternatives include endometrial ablation; however, this does affect fertility and does not always cause amenorrhea and is therefore less desired.31 In some societies vaginal hysterectomy for menstrual hygiene has been used, but this is not usually used in the United States.32 This would also render the patient sterile, which has ethical and legal implications.33

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CONCLUSION

The management of menstrual issues in adolescents with developmental delay should be guided by the same principles as in the general teenaged population. If dysmenorrhea/menorrhagia cause great concern and affect daily activities, treatment should be initiated with periodic evaluation of the need for continuation. The health care team, patient, parents, and school/workplace or other caregivers should work together to find a solution that is in the best interest of the patient.

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Elisabeth H. Quint, MD, is professor, Department of Obstetrics and Gynecology, Division of Gynecology, University of Michigan Health System, Ann Arbor. Anita Schwandt, MD , is assistant professor, Department of Reproductive Biology, MetroHealth Medical Center, Case Western Reserve University, Cleveland, Ohio.

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