Gynecologic Care for the Mentally Handicapped Individual

Pediatric and Adolescent Gynecology
Sue Ellen Koehler Carpenter

There is a great need to provide quality gynecologic care to mentally handicapped individuals. In general, these individuals now have greater life expectancy than previously. Those with mild-to-moderate retardation are being encouraged to fulfill their highest potential by living in society at large or in sheltered situations with a high degree of normalization. Severely affected individuals, who might have succumbed at a young age to the medical complications of their disorder, are living through puberty and surviving their parents. Issues of sexuality, contraception, menstrual hygiene, and premenstrual syndrome with severe behavioral disorders can become important. Management of common gynecologic disorders, such as vaginal discharge, dysfunctional uterine bleeding, and pelvic pain, becomes difficult due to limitations in communication and examination. In some institutions, multispecialty clinics have been created to serve these patients.

MENSTRUAL HYGIENE

Issues of menstrual hygiene are common. Frequently, behavior modification successfully trains mildly and moderately retarded women. These programs may be less successful for profoundly affected patients unless toilet training has been successful previously. Oral contraceptive pills can be useful for limiting the volume of menstrual flow. Alternatively, menstrual suppression with depot medroxyprogesterone acetate can be used. After 1 year of use, amenorrhea is induced in 50% of patients using the standard contraceptive dose of 150 mg every 3 months. A more frequent schedule can be used to induce amenorrhea more quickly. Long-term use of Depo-Provera carries the risk of bone mineral loss. The benefits of menstrual suppression must be weighed against this risk. Some patients may be able to cooperate with periodic bone mineral density assessments.

Vaginal hysterectomy has been reported as a means of menstrual management. In general, a less invasive form of therapy consistent with the patients' goals will be chosen. Hysterectomy will be reserved for patients with documented failure of training programs, gynecologic disease, severe anemia, or contraindications to hormonal therapy. A case of hepatitis-positive blood being splattered uncontrollably and posing a substantial threat to caregivers and housemates has been reported to justify a hysterectomy. Endometrial ablation may provide an acceptable alternative to hysterectomy.

Dysfunctional uterine bleeding certainly will exacerbate problems of hygiene. Accurate menstrual records should be kept. Alterations in previously normal cycles warrant investigation, as for any patient. Obesity and thyroid dysfunction, which occur commonly in patients with Down syndrome, may predispose this population in particular to abnormal bleeding.

PREMENSTRUAL SYNDROME

Premenstrual syndrome with exacerbation of autism, temper tantrums, irritability, restlessness, disobedient behavior, and seizure activity has been noted. The value of an accurate menstrual calendar for interpreting these behavioral changes is obvious. Some behavior problems reflect dysmenorrhea, which severely handicapped persons are unable to communicate. This will respond to timely, generous administration of nonsteroidal antiinflammatory drugs. Others may respond to oral contraceptive pills or Depo-Provera. Selective serotonin reuptake inhibitors can be used, but the collaboration of an interested psychiatrist may be required as the incidence of polypharmacy in these patients is quite high and the clinician's ability to assess a patient's progress and monitor side effects may be limited.

SEXUALITY

Issues surrounding sexual behavior are best dealt with in group educational and individual counseling sessions. Individuals who are integrated into the community often cannot interpret media representations of sexual behavior. They need help understanding the nature of appropriate relationships and learning to take responsibility for their actions. They must be taught to avoid situations that could lead to sexual abuse. Parent groups focus on helping parents learn to accept their children's sexuality and establish realistic goals for the maturing family member. More severely affected persons need limitations placed on unacceptable public displays of sexual behaviors and sexual self-abuse.

CONTRACEPTION

Providing adequate contraception can be difficult. Oral contraceptives and barrier methods demand a level of compliance that may be beyond the scope of an individual's abilities. However, oral contraceptives can be supervised by a caregiver. The risk of thromboembolism must be considered. Patients with impaired circulation or decreased mobility may be at increased risk. The intrauterine device may be appropriate in a few cases; however, it will be unacceptable to the extent that it increases dysmenorrhea and menstrual flow. Some patients will be unable to reliably report the symptoms that may accompany complications.

Depo-Provera is commonly used. The most common side effect is irregular bleeding during the first year of use. Thereafter, 50% of patients experience amenorrhea. Weight gain, abdominal bloating, headache, mood changes, and nervousness may occur, but usually are minor relative to the benefits of therapy. Bone mineral loss associated with long-term use has been reported, and clinicians need to be mindful of this potential effect  Norplant (subdermal levonorgestrel implants) provides protection for as long as 7 years. It does not carry the contraindications of estrogen-containing products. Although Norplant has been offered to patients with mental retardation, acceptance of the method has been limited .

STERILIZATION

The question of involuntary sterilization inevitably arises in a clinic where patients with mental retardation who are deemed incapable of giving informed consent are seen. The history of the procedure in the United States dates from the turn of the century, when the eugenics movement advocated involuntary sterilization to avoid genetic transmission of mental retardation. In 1927, the Supreme Court affirmed the constitutionality of eugenic sterilization in an opinion on Buck v Bell written by Justice Oliver Wendell Holmes: “It is better for all the world if instead of waiting to execute degenerative offspring for crime or let them starve of their imbecility, society can prevent those who are manifestly unfit from continuing their kind...three generations of imbeciles are enough”.

Today, most readers of the opinion of Justice Holmes would find it very disturbing. However, the decision set the stage for a 15-year period during which 37 states passed eugenic sterilization legislation. In 1942, the Supreme Court, with a completely different membership, heard Skinner v Oklahoma. Skinner had been convicted three times of shoplifting and other similar offenses. Under the Oklahoma habitual criminal sterilization act, anyone convicted of specific crimes (including larceny) could be sterilized. The law excluded other equally serious offenses. Skinner argued that this differential treatment violated his right to equal protection. The opinion of the Supreme Court held the Oklahoma statute unconstitutional. In the words of Justice Douglas, “We are dealing here with legislation which involves one of the basic civil rights of man. Marriage and procreation are fundamental to the very existence and survival of the human race. The power to sterilize, if exercised, may have subtle, far reaching and devastating effects...there is no redemption for the individual whom the law touches...He is forever deprived of a basic liberty”.

The Skinner decision set the stage for the current debate on involuntary sterilization. The physician is placed in a difficult position when a patient and her family seem to have a reasonable request for sterilization because the individual cannot or does not wish to meet the demands of child rearing. Vining and Freeman   have appealed for a moderate approach that is guided by the level of retardation of the patient. In some cases, the level of supervision required for the severely affected patient makes sexual activity and, thus, the fear of pregnancy unreasonable. In the case of mildly retarded individuals who are able to marry and maintain themselves in the community, sterilization should not be an issue. For a middle group, living in a sheltered situation, increased efforts at normalization have led to acceptance of the right to sexual satisfaction, and sterilization may offer the best chance for normalcy. Advocacy for individual rights should include the possibility of obtaining sterilization under circumstances where the procedure allows patients to best fulfill their own potential.

Currently, the physician liability issues with regard to involuntary sterilization depend on state laws. There are three categories to consider: (a) potential liability when a state statute authorizes involuntary sterilization, (b) liability when a court orders sterilization at the request of the guardian without a specific statute, and (c) liability when there is no statute or court order. Approximately 19 states now have legislation enabling sterilization of mentally incompetent persons. The statutes generally have been upheld if they contain enough procedural protection for the patient. The New Jersey court invoked the parens patriae doctrine to order sterilization. The purpose of the doctrine is to protect the rights of mental incompetents. The judge interpreted sterilization as a procedure to which the patient had a right when it was clearly in her best interest. The court sought to protect that right. In some states where there is no statute, it may be possible to obtain a court order for sterilization when due process and equal protection under the law are emphasized. Other state courts have been unwilling to conclude that there is anything in their common law powers that enables them to mandate sterilization.
The substituted consent of parents alone, without a valid court order, will not suffice. The current attitude of the courts in such cases implies that physicians incur tremendous liability if they perform such procedures.

Rauh et al. have advocated a moderate approach to the issue: a statute that would enable sterilization under specific circumstances. Applications would be reviewed by local courts or hospital review committees. Their proposed model statute would include the following:

1.The individual is presumed capable of procreation.

2.The individual is, or is likely to be, sexually active.

3.Pregnancy would not usually be intended by a competent person facing analogous choices.

4.All alternative contraceptive methods have proved unworkable or been shown inapplicable.

5.The individual's guardian agrees that sterilization is the best course of action in the case.

6.Comprehensive medical, psychological, and social evaluations recommend sterilization for the individual.

7.The individual is represented by independent legal counsel with demonstrated competence in dealing with medical, legal, social, and ethical issues involving sterilization.

8.The individual, regardless of her level of competence, has been informed in full by one able to make her understand and up to her level of competence shows awareness of the method of sterilization, the consequences of sterilization, the likelihood of success, alternative methods of sterilization and birth control, and the consequences of pregnancy and parenthood.

HYSTERECTOMY

Frequently, patients' families present to the clinic with requests for hysterectomy. This must be considered on an individual basis. The least harmful form of care consistent with the patients' best interest should be used. Families sometimes present with this request even before menstruation begins or when the patient is amenorrheic. Patients often have significant medical risks for surgery, which the family does not consider in their request. The family's specific concerns must be addressed with sensitivity, practical advice, and assurances of support. Questions of menstrual hygiene should be answered with adequate training programs and medication as needed. Contraception should be provided. Information that hysterectomy will not ameliorate premenstrual syndrome or prevent sexual abuse should be presented. Parent support groups and social work intervention may be encouraged. At times, a parent requests hysterectomy as part of a larger, sometimes overwhelming, agenda dealing with the physical, emotional, and financial demands that the maturing dependent places on the family.

McNeeley and Elkins   reported their experience with major gynecologic surgery in 15 mentally handicapped women. The indications for hysterectomy were menorrhagia (n = 3), leiomyomata (n = 9), and ovarian neoplasm (n = 3). All decisions for major surgery or sterilization in their clinic are presented to a societally based committee before admission . Six of the 13 women undergoing major abdominal surgery in this series developed significant complications. All three quadriplegic patients with cerebral palsy developed aspiration pneumonia. Pelvic cellulitis occurred in one case. Urinary tract infection and fecal impaction occurred in two others—one with associated small bowel obstruction. In this report, another 22 minor procedures (including dilatation and curettage, and laparoscopic sterilization) were performed without incident. Abdominal hysterectomy for sterilization alone certainly is contraindicated by its increased morbidity. Most of the patients in this series presented with advanced gynecologic pathology probably due to the difficulty of providing routine gynecologic examinations in this population.

ROUTINE CARE

The initial evaluation of patients with mental retardation who present for routine gynecologic care should include a complete medical history, gynecologic history with a menstrual calendar, if available, and physical examination. In this population, chronic illness and subnormal weights are not necessarily associated with amenorrhea. If a recent social work evaluation and psychological evaluation (with IQ testing) are available, they are helpful.

A pelvic examination should be performed, if possible, and a Pap smear obtained in patients with abnormal vaginal bleeding or vaginal discharge, patients over age 30 years, or patients who are sexually active. If a speculum examination is impossible, a Pap smear can be performed blindly using a moistened cotton-tipped swab directed to the cervix by an examiner's single finger. When a pelvic examination cannot be performed, a pelvic ultrasound may be a useful alternative. However, an examination under anesthesia will be required on some occasions. Office sedation may provide a convenient alternative to general anesthesia.

Routine complaints, such as vaginal discharge, require a practical approach. Urinary frequency, crying on urination, or foul-smelling urine suggest a urinary tract infection. Anal itching suggests the presence of pinworms. Vulvar irritation can result from or cause excessive masturbatory behavior. Hypoestrogenism may be a cause of discomfort, especially if hygiene is difficult. This responds to a regimen of frequent sitz baths, air drying, and soothing ointment such as Balmex. Hydrocortisone cream 1% may be used as well. Recent antibiotic use or a thick white discharge suggest Candida infection, and an empiric trial of an antifungal agent is appropriate. If the discharge is persistent or recurrent, a complete pelvic examination to rule out foreign body and to obtain complete cultures for Candida, gonorrhea, chlamydia, streptococcus, and fecal pathogens is required. A saline preparation should be examined for Trichomonas and Gardnerella. Appropriate treatment then is instituted. If cultures are positive for a sexually transmitted disease, rapid plasma reagin (RPR) and human immunodeficiency virus testing along with an investigation for sexual abuse may be required if consensual sexual activity cannot be confirmed.

In summary, the gynecologic care of mentally retarded adolescents requires special care. It is best provided by individuals with an interest in the field and the ability to consider the patient's unique medical and social circumstances when providing services. A commonsense approach to the patient's best interests is needed. The breadth of experience in the academic center clinics may be informative for those who provide community-based care for these special patients.