Gynecologic and Obstetric Issues Confronting Women with Disabilities

Approximately 21% of women in the United States have some type of disabling condition.¹ Many other women suffer from chronic conditions that, despite significantly impairing functional capabilities, fail to meet classic definitions of “disability.” Women with disabilities are less well educated and less likely to be married than nondisabled peers. They are more likely to live in poverty, to lack employment, and to rely on publically funded health coverage.² The medical community frequently overlooks clinical issues important to this very large population, including many unique gynecologic and obstetric problems.

Basic screening and the pelvic examination

Physicians caring for disabled women must perform pelvic examinations in a way that assures patient access, safety, and comfort. Adjustable tables that lower to the standard wheelchair height of 19 or 20 inches facilitate access for the examination. Handrails on either side of the table enhance safety and allow independent movement up or down the table. Alternative stirrups, particularly padded boot stirrups, increase comfort for women with limited range of motion of the lower extremities (Fig. 1) (Welner Universally Accessible Examination Table, patent issued 4–96, no. 507050). An assistant should attend each examination and when necessary, manually support lower extremities, individually adjusting adduction/abduction, flexion/extension positioning for patients with spinal cord injury (SCI), stroke, cerebral palsy (CP), orthopedic injury, multiple sclerosis (MS), and even obesity. When padded boots are available, the need for extra personnel is minimized, as boots can stabilize spastic limbs and those with limited range of motion. In patients with CP, who often have very severe flexion contractures, it may be preferable to use knee-chest, side lying, or heel-to-heel positions. Above all, physicians must allow more time to examine a disabled patient.³

Spinal cord-injured women with lesions above T6 pose a particular problem during a pelvic examination because these patients are at increased risk for having an episode of autonomic dysreflexia (AD) during this type of examination (see section titled Autonomic dysreflexia). Before obtaining a pelvic examination, physicians should advise these patients to empty their bladders and to perform standard bowel programs the night before to minimize the risk of triggering AD and bowel accidents during examination.

Rapid pulling movements to adjust leg positioning should be avoided as this can cause increased muscle spasticity. Liberal application of lidocaine gel to the perineum before insertion of the speculum can lessen the likelihood of developing AD. Topically applied lidocaine gel can also be useful to decrease discomfort when examining disabled nulliparous women or those who are elderly.⁴ Despite all precautions, AD can still occur in a predisposed patient. Care givers should have rapid-acting antihypertensive agents available and should monitor blood pressure periodically during the pelvic examination.

Breast cancer screening

Breast cancer is the most common gynecologic malignancy. Not only are women with disabilities at increased risk for underdetection of breast disease but they also may harbor an increased risk for developing such malignancies. Women with disabilities may be at increased risk for underdetection of breast pathology as a result of a variety of factors. Some clinicians and patients, for example, wrongly believe that mammography can be performed only when a woman is standing, neglecting to offer this essential screening tool to women in wheelchairs. Some women with disabilities have restrictions in upper body movements, making it difficult for them to perform breast self-examination. Physical limitations may impact the quality of the mammography image, limiting views to the anteroposterior dimension and restricting the ability to obtain lateral views. Unfortunately, access barriers such as high examination tables may limit a woman's ability to obtain regular clinical breast examination. Clinicians need to work closely with women to overcome obstacles in breast health care. Initiatives sponsored by the Center for Disease Control (CDC) and the Association of Professors for Gynecology and Obstetrics (APGO) have attempted to educate women's health care providers and improve access to mammography.^{5, 6}

In addition to delayed diagnosis because of access barriers, women with disabilities may harbor an increased risk for developing breast cancer. These women, because of decreased physical activity and limited food choices are statistically heavier than other women, with obesity being one of the risk factors linked with breast cancer.⁷ Early exposure to ionizing radiation to the spine, a common occurrence in girls with cerebral palsy, scoliosis, and other conditions has also been linked to an increased risk for the development of breast cancer.⁸

Menstrual cycle

For many disabled women, regular menstruation signifies normalcy as well as the opportunity for childbearing. Other disabled women discover that normal and particularly abnormal menstruation causes hygiene problems and increases the need for attendant care. Parents of girls with disabilities – especially severe cognitive disabilities – frequently worry about perceived mood changes and pain during menses, leading them to request hormonal or even surgical suppression of menstruation. Physicians evaluating disabled women must therefore know the effects of various disabilities on the menstrual cycle while remembering that disabled women often experience the same menstrual disorders that trouble the nondisabled.

CP, spina bifida, and other congenital disabilities usually do not affect the onset or pattern of menarche. Young girls who suffer traumatic brain injury (TBI) or SCI may experience delayed menarche or even precocious puberty.⁹ Most women with SCI will resume normal menstruation within 6 months postinjury, though roughly 25% of women in one study reported increased perimenstrual dysautonomia – gooseflesh, headaches, flushing, and sweating.¹⁰  

Women with previously normal menstruation may experience transient menstrual disorders after becoming disabled. Women with chronic disease states, such as diabetes mellitus or obesity, may experience menstrual irregularity. Patients with rheumatoid arthritis retrospectively report later onset of menarche and increased rates of ovulatory dysfunction.¹¹ Women with SCI, TBI, and MS have an increased frequency of hyperprolactinemia.^{12, 13, 14} Indeed, in one systematic review of pituitary dysfunction following TBI, hypopituitarism was discovered in more than one quarter of patients 5 months postinjury.¹⁵ Consensus guidelines of some organizations now include routine screening for hypopituitarism after moderate and severe TBI.¹⁶  If no structural abnormalities of the pituitary are discovered and menstrual cycles are regular, no further therapy is warranted. Disabled women with hyperprolactinemia may have significant hygienic problems due to related menstrual irregularities. Moreover, many have low levels of 17β-estradiol, which intensifies their risk for osteoporosis. Patients with hyperprolactinemia and menstrual irregularities either severe enough to cause anemia or hygienic issues require pharmacologic therapy.

Contraception

Although disabled women require safe and effective contraception, they lack comprehensive data to guide their choices.

Women with impaired mobility face particularly difficult issues given their increased risk for the development of deep vein thrombosis due to lower extremity venous stasis. Although spasticity may diminish venous stasis, it is unknown how greatly spasticity decreases overall risk of deep vein thrombosis. Likewise, it is unclear how much immobility truly increases the risk of venous thrombosis among partially immobilized women or chronically immoblized patients who continue to engage in a variety of physical activities.¹⁷ Most clinics for women with disabilities continue prescribing combination oral contraceptives provided the patient lacks a personal history of thrombosis. Nuvaring™ (Schering Plough, Kenilworth, New Jersey), a vinyl ring worn in the vagina for 3 out of 4 weeks per month, administers estrogen and progesterone transvaginally without the need for the patient to ingest pills or remember to take a daily pill. Although some women with disabilities lack manual dexterity to place Nuvaring™, caregivers or family members can often do so for the patient. Nuvaring™ offers excellent cycle control with relatively less total estrogenic exposure than most combination oral contraceptives. Although the OrthoEvra™ (Ortho-McNeil Pharmaceutical, USA) transdermal patch offered many of the same advantages of NuvaRing, FDA issued updated labeling in 2005 following reports of increased venous thromboembolism among users.¹⁸ Three weeks of patch use exposes patients to roughly 71% more estrogen than a similar low dose oral contraceptive.¹⁹ Although the absolute risk of thromboembolism remains small, providers should use the patch more cautiously in chronically immoblized patients until more data become available.

Because estrogen-containing contraceptives increase the risk of thrombosis, physicians may wish to avoid prescribing them in this population, substituting progestin-only pills ("POPs" or “minipills”), depot medroxyprogesterone acetate  (Depo-Provera™) injections, implants containing levonorgestrel (Norplant™, Jadelle™) or etonorgestrel (Implanon™) or IUDs. The mini pill is somewhat less effective compared to combination oral contraceptives and should be combined with a barrier method for improved efficacy.²⁰ Because of the high prevalence of latex allergy in women with spina bifida and some other chronic disabilities, nonlatex methods may be appropriate in these patients.²¹ Nonlatex condoms and barriers greatly benefit this population.²² Barrier methods of contraception, though safe, may be difficult for many disabled patients to insert or use properly without their partner's assistance. 

Unfortunately, progestin-only methods often pose troublesome side effects. Patients may experience irregular bleeding with associated hygiene problems. Although many women appreciate improved hygiene associated with amenorrhea, Depo-Provera™ can also cause weight gain, depression, and hair loss. Hypoestrogenism induced by Depo-Provera™ might predispose patients to osteopenia.²³ Cromer and colleagues reported a 3% reduction in bone mineral density among adolescents whose bone density would typically have increased 9% during the same time period.²⁴ Due to concerns about how chronic Depo-Provera™ use in teens might impact peak bone mineral density and lifelong risk of osteoporosis, FDA issued a "black box warning" for Depo-Provera™ in 2004.²⁵ Although many authors refute the significance of these findings – and neither the American College of Obstetricians and Gynecologists nor the American Academy of Pediatrics advise against prolonged use of Depo-Provera™ – physicians caring for chornically immoblized patients or patients with chronic bone disease should make patients aware of the potential such agents might have to compound existing bone fragility.²⁶

Implanon™ (Schering Plough, Kenilworth, New Jersey) an etonorgestrel subdermal implant available in the US since 2006, offers many of the advantages of other progesterone only contraceptive methods but is not user dependent, it contains no estrogen and exerts no impact on bone mineral density. Approximately 10% of women discontinue Implanon™ due to unpredictable bleeding, a significant hygiene issue in chronically immobilized patients.²⁷ Some medications commonly used by disabled patients, particularly antileptics such as carbamazepine, may reduce contraceptive efficacy.²⁸

Despite the proven safety and efficacy of intrauterine devices (IUDs), many myths about them permeate US healthcare culture. Fortunately, US clinicians have begun to challenge unwarranted bias, liberalizing usage of IUDs among nulliparous patients, teenagers, and other groups of women who require effective contraception.²⁹ This includes women with disabilities. In the past, providers have worried that women with decreased pelvic sensation might have reduced ability to sense signs of pelvic infection or spontaneous expulsion of the IUD. Women with decreased manual dexterity cannot feel for the string to demonstrate appropriate placement of the IUD and some patients have questioned whether increased menstrual flow associated with copper IUDs might worsen dysreflexia among SCI patients with injuries above T6. Few of these problems warrant abandoning IUD use. Pelvic infection, rarely complicates IUD use and partners or caregivers may just as easily feel for strings. Progestin bearing IUDs, particulary the LevoNorgestrel IntraUterine Sytem (Mirena™, Bayer HealthCare Pharmaceuticals, Wayne, New Jersey, USA) decrease menstrual flow, offering improved menstrual hygiene and, at least theoretically, the opportunity to reduce menstrual associated dysreflexia among "high spinal" patients.³⁰

Women with chronic disease states face a variety of problems that often limit their choice of contraception. Patients with chronic, debilitating cardiac disease should avoid estrogen-containing contraceptives because they increase the risk of thrombosis. Physicians may prescribe progestin-only methods, or they might consider the use of intrauterine devices in women wishing to avoid hormonal agents. Although women with rheumatoid arthritis may experience decreased severity of symptoms while using combined oral contraceptives, women with other autoimmune conditions, such as systemic lupus erythematosus (SLE), may experience adverse side effects.³¹ SLE patients experience a significantly increased frequency of thrombotic events.³² Thus, they should use IUDs, progestin-only methods, or barrier methods.

Sexually transmitted disease

Unfortunately, many health care providers and family members still view disabled women as asexual. Although the disabled woman may therefore find it uncomfortable sharing intimate needs with health care providers and family, she may also be less likely to self-detect signs of sexually transmitted disease (STD). In many cases, an STD may be the only sign of an abusive relationship (see section titled Abuse). Thus, anyone caring for the disabled patient must become knowledgeable about the particular problems that STDs pose within this population.

Signs and symptoms of STDs used by physicians to diagnose infection reliably in nondisabled patients may prove less reliable in disabled women. Women with pelvic sensory impairment may not feel pain or cramping as do nondisabled women with chlamydia, gonorrhea, or pelvic inflammatory disease.³³ Malaise or increased spasticity may be the only warning signs voiced during the examination. Visually impaired women may not detect or accurately describe a lesion that could represent syphilis, herpes, or human papillomavirus infection. Although a complete discussion of all STDs exceeds the scope of this chapter, several infections warrant particular discussion.

SYPHILIS

The disabled woman with syphilis may present with mild or severe symptoms, either unnoticed by the patient, partner, attendant, or practitioner or mistaken for consequences of her disability.

Women with visual or sensory impairment may not detect the ulcer or chancre of primary syphilis before its spontaneous regression 3–12 weeks after infection. As with nondisabled patients, physicians should maintain a high index of suspicion when considering a diagnosis of syphilis, and not hesitate to perform serum screening tests, which generally detect infection 4–6 weeks after exposure.

Patients with secondary syphilis most commonly present with flu-like symptoms or skin rash. The rash often involves the palms, soles, trunk, and upper extremities. Women whose disabilities require that they take multiple medications may mistake skin rashes for yet another medication reaction, particularly because manifestations vary considerably from patient to patient.

In approximately 30% of patients, tertiary syphilis will develop if the initial infection is not treated. Sequelae of tertiary syphilis include optic atrophy, tabes dorsalis, and even generalized paresis. MS patients also develop this constellation of findings. Thus, a physician treating a woman with MS, who develops visual disturbance and sensory or balance abnormalities, may falsely attribute these findings to MS. Patients with symptoms of neurosyphilis may exhibit a change of mental status or the onset of a new physical disability. Such findings often confuse the patient, the patient's family, and her care givers, who may have overlooked infectious etiologies, such as syphilis, that they might not overlook in nondisabled patients.

HERPES SIMPLEX VIRUS

Approximately 50% of adults test positive for exposure to herpes simplex virus (HSV), and nearly 60% of these persons remain entirely asymptomatic. Both primary and recurrent lesions present problems for the disabled, infected patient.

Patients with primary HSV typically present with painful genital ulcerations and flu-like symptoms. Women with sensory impairment may fail to detect the lesions, but may report malaise or increased spasticity. Although healing of lesions may improve with adequate ventilation of involved areas, immobilized patients may have difficulty attaining or maintaining positions that allow ventilation.

Recurrent genital herpes, though usually milder in severity than primary outbreaks, may also go undetected by the disabled patient. Clinicians and personal care attendants must monitor women with sensory impairment and a known history of HSV infection for skin lesions consistent with HSV and not falsely assume that lesions in the perineum or other weight-bearing areas are simply related to pressure ulcers. Often, women with recurrent HSV become familiar with less traditional prodromes, such as generalized malaise or spasticity, and can help inform their care givers accordingly.

HEPATITIS B and C VIRUSES

Hepatitis B and C viruses (HBV) are transmitted through sexual contact, transfusion, and intravenous drug use. Many disabled women became physically impaired as a result of risk-taking behaviors, or required transfusion after traumatic injury before routine screening of blood for HBV or HIV viruses, and yet never underwent testing during their rehabilitation. Complicating this dilemma, health care providers may confuse constitutional symptoms such as fatigue, malaise, arthritis, and flu-like symptoms with exacerbation of certain disabilities, such as MS or rheumatologic diseases, and not screen for blood borne infections. Patients who continue to engage in high-risk behavior after the onset of a disability should undergo vaccination, just as is recommended for all nondisabled patients. Vaccination may also be warranted in patients with a history of TBI, who may have impaired judgment.

HUMAN PAPILLOMAVIRUS

Between 10% and 60% of the population demonstrate DNA evidence of exposure to human papillomavirus (HPV).³⁴ Many HPV infections are asymptomatic, requiring regular screening for diagnosis. HPV lesions are inadequately detected in women with mobility impairments, who often do not undergo regular screening because of access issues, and therefore these women are at increased risk for premalignant and malignant cervical diseases commonly associated with HPV infection. Not surprisingly, the more severe a woman's disability, the less likely she is to receive either PAP smears or mammograms.³⁵ Concurrent autoimmune disease compounds this risk: women with such disorders may have a 10–30% increased risk for cervical dysplasia compared to women who do not have autoimmune diseases or are otherwise autoimmune suppressed.³⁶

Many medical practitioners find colposcopic examination of the disabled woman cumbersome and unduly uncomfortable for the patient. Wall-mounted colposcopy units and examination tables that allow support of altered leg adduction will help both patient and physician. Women with SCI lesions above T6 risk development of AD during any cervical manipulation necessary to diagnose or treat dysplastic lesions. These patients require careful monitoring of blood pressure, the immediate availability of antihypertensive agents, and personnel familiar with AD and its treatment to be on hand. Physicians might wish to consider performing colposcopic examination in a monitored setting, such as an outpatient surgical suite in patients with lesions above T6, particularly those with a history of AD.

HUMAN IMMUNODEFICIENCY VIRUS

HIV infects more than 1 million Americans. As of 2005, roughly 26% of new US AIDS cases occurred in women, most often women who acquired HIV through sex with a high risk male partner. The epidemic disproportionately impacts African American women and other minorities.³⁷ Though no database provides an accurate representation of the prevalence among women with different types of disabilities, the inability of some disabled women to negotiate barrier use, higher risk taking after disabling injury – the so called "promiscuous phase" – and histories of transfusion following traumatic injuries all likely place disabled women at heigtened risk of acquiring the virus. 

As with other infectious diseases, care givers may mistakenly attribute signs and symptoms of HIV infection to other afflictions encountered by disabled patients. Patients using wheelchairs, who are already prone to frequent bouts of candidal vaginitis and vulvitis, may not realize that recalcitrant infection may represent a more serious problem. Mucocutaneous ulcerations due to HIV may masquerade as decubitus ulcers of the perineum and buttocks. Clinicians may less commonly test disabled women for HIV than their nondisabled counterparts, buying into the false notion that HIV is not a disease of women and that disabled women are not sexually active or vulnerable to abuse.

Infertility

Women with a variety of disabilities may be more prone to ovulatory dysfunction. Disabilities such as SCI, TBI, and MS may be associated with hyperprolactinemia. Although many patients exhibit concomitant menstrual irregularity or galactorrhea, others remain asymptomatic. Practitioners should maintain a high index of suspicion when treating these patients, and not neglect to obtain a pituitary evaluation. Similarly, chronic disease states such as diabetes mellitus, Sjögren's syndrome, and myasthenia gravis may – through altered thyroid function or other associated endocrinopathies – result in oligo-ovulation. Drugs commonly used by disabled women, such as corticosteroids, nonsteroidal anti-inflammatory agents, phenytoin, and tricyclic antidepressants may also affect ovulation.^{38, 39, 40}

Although the evaluation and treatment of infertility is the same in disabled and nondisabled women, women with disabilities may require special consideration in a variety of circumstances. Women with pelvic contractures, such as is caused by CP, may have difficulty attaining adequate positioning for many required procedures. Those with impairment of vision and movement may require assistance interpreting basal body temperature or other ovulation testing. Women with SCI above T6 may exhibit AD in response to endometrial biopsy, hysterosalgpingography, or hyperstimulation caused by ovulation-induction regimens. The majority of women with physical disabilities have unaffected fertility potential and clinicians should avoid assuming that the male partner need not be evaluated.

Menopause

More than half of women in the United States are over age 50 and the likelihood of disability – chronic or acute – increases with advancing age. Like their nondisabled counterparts, the majority of women with disabilities will have sequelae of estrogen deficiency, including osteoporosis, but their disabilities often complicate standard modes of therapy.

Disabled women may respond somewhat differently in symptomatology to decreased estrogen levels in menopause. These include vasomotor instability, which can be especially troublesome in women with high spinal cord lesions or in women with MS who are sensitive to temperature fluctuations. Women with MS may experience worsening of symptoms, but this may improve with estrogen replacement therapy (ERT).⁴¹ Urinary symptoms such as incontinence, increased frequency of cystitis, and bladder pain often complicate menopause. SCI patients and others with bladder regimens may need to modify their routines throughout the menopausal years. Cognitive impairment, often linked to estrogen deficiency states, may further complicate ongoing cognitive impairment related to TBI, stroke, or MS. Osteoporosis is responsible for significant morbidity and mortality in general, but especially so in menopausal women with disabilities.

Chronic immobilization and medications such as corticosteroids increase bone loss.  Patients with MS have an increased risk of vitamin D deficiency, potentially resulting in secondary hyperparathyroidism and resultant osteoporosis. Many MS patients, therefore, may benefit from daily supplementation of at least 600 IU of vitamin D.⁴² Women with SCI, in addition to being at risk for osteoporosis associated with menopause and chronic immobilization, may also show neurogenic osteopenia and heterotopic ossification (development of bone around joints). Such patients often benefit from bisphosphonate therapy.^{43, 44} Menopausal women with stroke and resultant unilateral weakness may have impaired balance and may be prone to falling. Many such women are elderly and may have underlying osteoporosis, which may be exacerbated by long-term heparin therapy given to stroke patients.⁴⁵

Clinical studies have prompted the use of many different serum markers for osteoporosis, including alkaline phosphatase, serum calcium, osteocalcin, 25-hydroxyvitamin D, parathyroid hormone, urinary hydroxyproline, and urinary pyridinolenes.^{46, 47} Furthermore, a variety of radiologic procedures – most notably, dual X-ray aborptiometry – are commonly used to quantify bone mass compared to control populations.⁴⁸ Unfortunately, there are no control data regarding expected bone mass for women with disabilities, particularly women younger than age 50.

Treatment of osteoporosis in women with disabilities needs to be individualized. Options that have been used include calcium supplementation, weight bearing exercise, vitamin D, calcitonin, bisphosphonates such as alendronate, and hormone replacement therapy (HRT). Intranasal calcitonin helps prevent bone loss during immobilization and may also produce an analgesic effect. However, there are drawbacks, including tachyphylaxis with long-term use and lack of beneficial effect on cortical bone.⁴⁹ HRT protects both cortical and trabecular bone, and these benefits are enhanced with concomitant administration of calcium, vitamin D, and magnesium.^{50, 51}

Many women experience hot flushes, irregular menstruation, and other climacteric symptoms before cessation of menses. In the absence of other medical contraindications, low dose oral contraceptives often provide cycle control, relief from flushing, and many other noncontraceptive benefits. Although physicians previously prescribed HRT following menopause both to prevent osteoporosis and to lower the risk of cardiovascular disease, several recent studies have changed the standard of care. The Heart and Estrogen/Progestin Replacement Study Follow-up (HERS II) failed to demonstrate improvement in cardiovascular outcomes.⁵² The Women's Health Initiative (WHI), the first prospective longitudinal trial evaluating the benefits and risks of hormone replacement therapy, was stopped early due to an increased risk of coronoary events, stroke, and invasive breast cancer. Increased risk was eventually documented in those receiving estrogen as well as estrogen/progestin in combination following 4 or more years of use.⁵³ As a result, physicians no longer prescribe HRT as a primary preventive therapy, though many continue to prescribe for the acute and chronic treatment of hot flushes and other symptoms related to hypoestrogenism.

It is essential to screen all women for breast cancer, particularly those after age 50. Some women with irregular upper body contours or restricted range of motion, such as those with CP, stroke, MS, and quadriplegia, may find mammography more cumbersome and may require referral to special mammography centers for alternative imaging techniques.

Sexual response in women with disabilities

Physicians and family members often forget that disabled women are sexual beings and experience the same sexual response cycle as their nondisabled counterparts.

Although disabled women experience sexual desire in response to standard erotic stimuli, fatigue or discomfort caused by chronic diseases or disabilities may suppress excitement. Soft touch of areas that have preserved sensation, erotic videos, and sensual music can help stimulate sexual desire. Couples may find gentle touching of all areas arousing, sharing in the knowledge that they are being touched by each other. Patients may wish to alter analgesic regimens to permit sexual activity during periods of maximum pain relief. Women with some chronic conditions experience increasing fatigue at different times of day and it would be prudent to avoid attempting sexual activity during these periods.

Some women with SCI experience spasticity in the lower extremities, causing discomfort and difficulty in adjusting positions. Slow and gentle muscle stretching can help relieve spasticity and can become an important part of foreplay.⁵⁴ Although some women with SCI will not experience genital vasocongestion as a result of neurologic injury, many experience reflex genital vasocongestion despite a complete lack of subjective arousal. Women with lesions above T6 may even experience AD in response to any phase of the sexual response cycle. Clinicians should discuss these issues with an SCI specialist.

Abuse

“Abuse” is preventable emotional or physical harm that results from intentional human action. Although approximately 20% of the general female population experiences abuse, nearly 50% of women with disabilities suffer this violation. Sadly, half of all cases may go unreported.^{55, 56}

Most cases of abuse result from one person's taking advantage of an actual or perceived power differential. Such interactions can occur between parents and children, spouses, personal care attendants and clients, physicians and patients, and caretakers and clients living in long-term care facilities. Women with physical limitations may become accustomed to relinquishing some control to others. This dependency may take the form of needing to hold a person's hand when going downstairs, requiring someone to lift her wheelchair into a car, or needing even more basic support with activities of daily living (e.g., getting out of bed, dressing, toileting, feeding). Moreover, parents, siblings, or others who discount a woman's positive attributes and focus instead on her physical limitations, may help lower a woman's self-esteem, thereby increasing her vulnerability to abuse. In one study of 181 women with disabilities, women reporting abuse more often perceived themselves as sexually unattractive with low body image and low self esteem. These perceptions and the woman's concomitant desire to remain in a relationship increased her likelihood of remaining in an abusive relationship.^{57, 58}

Combating abuse may overwhelm any woman who feels trapped and powerless. Health care workers should try to identify abused women, looking for subtle signs (e.g., idiopathic chronic pain, headaches, unexplained traumatic injuries, centrally located bruising, emotional disturbance, weight changes). Frequently, the abuser may accompany the woman to visits. Thus, physicians should insist on speaking with the patient privately, whenever possible. After identifying situations of abuse, health care workers should locate accessible shelters, counseling services, and alternative living situations.^{59, 60}

The psychosocial and sexual needs of women with disabilities have many things in common with those of nondisabled women. Access, education, and empowerment can improve communication between these women and their medical providers, ultimately improving their quality of care.

Families and health care providers often needlessly discourage disabled women from becoming pregnant. Disabled women who have researched the effects of pregnancy and childrearing on their disability usually encounter health care providers who lack basic information necessary to counsel them, or who fuel possibly unfounded fears regarding the clinical consequences of pregnancy, labor, and delivery. Ironically, the same providers more often fail to offer disabled women information regarding contraception, even though fertility usually remains unaltered after a severe traumatic injury. Preconceptional counseling is critical for the disabled patient but often fails to occur because the patient fears how health care providers and family will respond to their decision to have a family. 

 The following areas should be addressed with the disabled woman who is planning a pregnancy:

•   The effects of her particular disability on pregnancy, labor, and delivery
•   The effects of pregnancy, labor, and delivery on her disability
•   An inventory of the effects of medications on the fetus
•   Modifications of health that could enhance childbearing
•   Family adjustments for child care
•   Community resources available to the disabled pregnant woman and her family.

Disabled women interviewed after pregnancy and delivery consistently advise health care professionals to respect the expertise the patient has acquired in the process of managing her disability. They recommend that professionals discover what the patient can and cannot do for herself, and that they introduce her to relevant support groups, particularly a group of other disabled women who have parenting in common. Most importantly, obstetricians must accept their role as primary-care physicians, orchestrating the cacophonous disciplines often involved with the disabled woman's antepartum, intrapartum, and postpartum care.⁶¹

Antepartum issues

The Americans with Disabilities Act defines disability as “a) physical or mental impairment that substantially limits one or more major life activities, b) a record of such impairment or c) being regarded as having such an impairment.”⁶² Although this definition encompasses a vast array of physical and mental disorders, each of which impacts pregnancy differently, there are a variety of common problems most disabled women face. SCI exemplifies the problems associated with many disabilities. These patients confront physical barriers and suffer the effects of chronic immobilization faced by patients with other chronically debilitating and neurologic diseases. There is also more literature written about women with this particular disability, either because women with acquired disabilities are more likely to desire pregnancy or because AD, a disorder commonly associated with SCI, strikes more frequently and insidiously during pregnancy. Regardless, pregnant disabled women have many of the same concerns about prenatal care as nondisabled pregnant patients. Good prenatal care still begins with a thorough history and physical examination and assessment of these issues. After that, the obstetrician must deal with the attitudes and issues more particular to the disabled population.

Prenatal testing and the disabled patient: concerns of the disabled

The disabled community has shifted the approach to disability from models based on charity and medicalization to a social-contextual approach that mainstreams disabled persons into the general community. Rather than viewing disability as cause for pity or medical intervention, they view disability as adaptable variation often made unadaptable by fear, prejudice, and lack of accommodation. This view of disability conflicts with traditional prenatal screening, which seeks to avoid the individual and social costs of disability.

Efforts to restrict a woman's right to terminate an undesired or undesirable pregnancy resemble age-old attempts to restrict the autonomy of disabled women in choosing other medical therapies. Moreover, few disabled women contest the use of prenatal testing to ameliorate a disability through prenatal therapy or to prevent the social disruption that often occurs when an undiagnosed disability surprises a couple at the time of birth. Many disabled women, however, criticize termination of some abnormal pregnancies based on the “altruistic” impulse to spare the fetus future pain or harm. Perhaps the perceived incapacity prompting many people to terminate a pregnancy is not the authentic experience of those with the disability. Is it better not to exist or to have a disability?

Because most disabilities result from trauma or the effects of age, it is unlikely that prenatal screening will reduce the gross social cost of disabilities.⁶³ Prenatal screening that fails to incorporate the subjective experience of the disabled themselves costs society enormously when it eliminates the contributions of gifted, diverse individuals.

Obstetric concerns of the disabled pregnant patient

In addition to standard obstetric questions, a disabled patient would likely want to know whether disability in general, and her disability in particular, will influence the outcome of her pregnancy, labor, and delivery. Unfortunately, most of the reports in the literature relating obstetric outcome with disability have been studies of small series of patients or have reflected the anecdotal experience of select providers. Although a thorough discussion of all disabilities and how they relate to pregnancy exceeds the scope of any single review, the experiences of spinal cord-injured women and those with MS warrant particular attention given the amount of published data and frequency of occurrence in common obstetric practice.

SCI does not appear to alter the physiology of pregnancy or childbirth. Although one report suggested an increased frequency of congenital anomalies and stillbirth among women with SCI, more recent studies have found no such association.⁶⁴ Some series have suggested an increased risk of preterm labor and delivery among women with SCI; others have demonstrated no such risk.^{65, 66, 67} Although many consider rapid labor a risk for women with SCI, Baker and colleagues⁶⁸ were unable to demonstrate this – whether the lesion was above T10, in which case the patient has decreased sensation of labor, or below T10, in which case labor sensation is normal.

MS, the most common acquired demyelinating disease of the central nervous system, is also the most common cause of chronic neurologic disability among uninjured young adults. Because MS afflicts women twice as often as men and manifests during reproductive age, it often complicates pregnancy and prenatal planning.⁶⁹

Obstetricians can reassure MS patients that their disease does not likely jeopardize fertility or obstetric outcome. In at least one study, the number of pregnancies in women with MS was not different from that of control groups.⁷⁰ The rate of spontaneous abortion, congenital malformation, and stillbirth also does not appear increased in MS patients.⁷¹ Most studies have found MS to have no effect on the course of pregnancy, labor, or delivery including no change in the frequency of preterm labor, preterm rupture of membranes, or difficult delivery.^{70, 72, 73, 74}

MS, like other autoimmune diseases, tends to improve during pregnancy, although it worsens postpartum.⁷⁵ Fortunately, no difference in long-term disability is observed in comparisons of parous and nulliparous MS patients. Moreover, pregnancy or a history of pregnancy do not increase the rate of disease progression.⁶⁹

Despite legitimate obstetric concerns, physicians must reassure their disabled patients that together they can anticipate and overcome most obstetric problems through common-sense preventive measures. Many strategies exist to help patients with SCI, MS, or other neurologic impairments, as well as their care givers, discern the onset of labor. A private tour of birthing facilities allows the visually impaired patient the opportunity to examine physical boundaries, and the neurologically impaired patient the ability to resolve access issues before labor's onset. Similarly, the hearing-impaired patient can identify her interpreter before delivery or meet with labor and delivery staff, who can assure the patient that they have access to transparent masks and that they know not to speak too quickly or exaggeratedly if she lip reads. Regardless of the disability, the disabled patient needs extra reassurance from all obstetric staff that her pregnancy will most likely result in the normal, term delivery of a normal, healthy infant.

Common antepartum problems of spinal cord injured and other chronically immobilized disabled women

DECUBITUS ULCERS

Decubitus ulcers complicate the chronically immobilized woman's pregnancy because of her increased weight, altered center of gravity, and decreased ability to transfer. They most commonly involve the sacrum, heels, and ischium. Anemia, also increased in the pregnant disabled patient, further predisposes her to pressure sores and may itself be exacerbated by the presence of ulceration.

The obstetrician must respect the severe clinical ramifications of these lesions. Decubitus ulcers predispose the patient to bacteremia and may induce a nutritionally catabolic state (i.e., negative nitrogen balance), increasing the likelihood of intrauterine growth retardation.

To prevent these lesions, the patient must continue practicing weight shifts and push-ups in the chair, despite the increased difficulty usually encountered during pregnancy. Those caring for the patient should remember to check wheelchair cushions and investigate the patient's complaints promptly. A pressure relief program that has prevented skin breakdown before pregnancy might not withstand the increased weight gain and hormonal changes associated with pregnancy.

URINARY TRACT INFECTION

Urinary tract infection complicates the pregnancies of most women with SCI and other disabled women with chronic bladder denervation. Indeed, in Hughes and associates'⁷⁶ series of 17 pregnant women with SCI, cystitis developed all but one woman. Comarr⁷⁷ showed that paralyzed pregnant women have a higher incidence of cystitis than nonparalyzed pregnant women, independent of the existence of catheters or calculi. Cross and co-workers⁶⁷ compared the frequency of urinary tract infections among groups using catheters (either intermittent or indwelling) versus groups using no catheters; the results were 77% and 31%, respectively.

Although many obstetricians might dismiss urinary tract infection as a minor prenatal complication, it can become life threatening in SCI patients if it precipitates AD.

Most patients with neurogenic bladder perform intermittent self-catheterization. Nurses must remind the pregnant disabled patient of physiologic changes accompanying pregnancy that increase her risk of urinary tract infection. They should teach measures to reduce the likelihood of infection, such as adequate fluid intake, catheter care, and minimization of postvoid residual volumes through complete bladder emptying. It is unclear whether the heightened risk of pyelonephritis in this population warrants chronic prophylactic antibiotic therapy or serial cultures. Some authors have recommended suppression therapy with methylmandelic acid in patients with SCI.⁷⁸

ANEMIA

Although anemia often poses only a minor problem for the nondisabled pregnant patient, it can severely compromise the disabled patient's pregnancy. Anemia can exacerbate fatigue, already heightened in the immobilized patient, further reducing her ability to transfer, self-catheterize, or perform other daily functions. Although failure to perform such tasks on a regular schedule can increase the risk of a variety of problems, anemia directly predisposes the patient to decubitus ulceration. Oral iron therapy exacerbates chronic constipation, and resultant fecal impaction and disimpaction can precipitate AD in women with SCI.

Care givers should prescribe stool softeners and high-bulk diets early in gestation, even in the asymptomatic patient. Some have even recommended routine blood transfusion of patients with hemoglobin values less than 11, though this datum usually reflects older, more liberal transfusion practices currently curtailed because of appropriate concern of transmission of HIV and other blood-borne pathogens.⁷⁹

RESPIRATORY COMPROMISE

Respiratory function often declines in disabled pregnant patients. Not only does the pregnant uterus exert increased pressure on the diaphragm, decreasing residual volume and functional residual capacity, but injuries above T5 prevent voluntary use of abdominal wall musculature, impairing cough and clearance of secretions, and increasing the risk of pneumonia. Women with high thoracic or cervical lesions may require ventilatory support during late pregnancy or labor.⁸⁰

THROMBOEMBOLIC COMPLICATIONS

Despite the theoretically increased risk of thromboembolism in chronically immobilized disabled patients, most studies have failed to confirm an increased risk unless other risk factors were present. Most investigators do not recommend routine prophylactic anticoagulation of pregnant women with SCI or other immobilizing disabilities.

Intrapartum issues

Most pregnant women worry about whether they will recognize the onset of labor and will have an uncomplicated vaginal delivery. For the disabled patient, such commonplace problems often assume uncommon complexity, and many new concerns arise specific to that patient's particular disability.

DIAGNOSIS OF LABOR

Many patients with SCI, MS, or other debilitating neurologic diseases face an increased risk of unattended labor and delivery because of difficulties sensing contractions and other signs of labor.⁸¹

Women with cord transection above T10 have painless labor, because pain impulses enter the spinal cord between T10 and L1. Those with lower lesions may have dulled pain sensation, and often fail to recognize contractions while asleep. Perhaps equally problematic, the disabled pregnant patient is often unable to sense rupture of membranes, and her care givers may erroneously attribute leaking fluid to incontinence, which also increases with advancing gestational age in patients with neurogenic bladders. Whether labor is more rapid in patients with SCI remains the subject of controversy. Baker and colleagues⁶⁸ were unable to demonstrate this for lesions above and below T10.

Strategies to prevent unattended birth in the disabled population have included serial examination, early hospitalization, and the use of home uterine activity monitoring (HUAM). Hospitalization is usually unnecessary, although it might be helpful for preventing unsupervised term delivery in the small subset of patients completely unable to sense contractions. Although routine admission may have some advantages in patients with SCI, it completely disrupts family life. HUAM, as with any kind of tocodynamometry that utilizes abdominal belts, can precipitate AD.

Care givers affiliated with the Rehabilitation Institute of Chicago favor the use of serial examination complemented by education of the patient regarding ordinary signs of labor and ways she might detect them. The disabled patient can be trained ahead of time in abdominally palpating contractions or, if possible, observing for an increase in vaginal discharge. Pathologic changes commonly associated with AD (e.g., pilomotor erection, rhythmic headache, diaphoresis) may also help alert the patient that labor is beginning.

AUTONOMIC DYSREFLEXIA

AD, also known as autonomic stress syndrome or the mass reflex, occurs when afferent impulses enter the spinal cord, initiating reflex arcs unmodulated by higher centers. This syndrome occurs primarily in patients with lesions at or above T10 and especially above T6, because lesions at this level eliminate hypothalamic control over sympathetic spinal reflexes. Although AD can complicate the normal daily functioning of patients with SCI, it presents particular problems for the obstetric patient and her health care providers.

Many stimuli can elicit the panoply of clinical events characteristic of AD. Distention, contraction, or manipulation of the cervix, bladder, or rectum; strong suprapubic bladder pressure; perineal manipulation during disimpaction; excessive deep breathing; labor; and immersion of the feet in cold water have all triggered this potentially life-threatening complication of SCI. Symptoms include severe hypertension with headache, bradycardia, arrhythmia, diaphoresis, flushing, tingling, nasal stuffiness, and respiratory distress. Rhythm disturbances include prolonged PR intervals, second-degree atrioventricular block, multiple premature ventricular contractions, and bigeminy. Obstetric care givers often express surprise at the degree of hypertension noted in these patients, with pressures often exceeding 300/150. Indeed, if unrecognized, AD can result in intracranial hemorrhage, coma, and maternal death.^{4, 82} Concomitant uteroplacental vasoconstriction can lead to severe fetal asphyxia.

Although many modalities exist to help treat AD, the cornerstones of therapy remain prevention and early recognition. Attentive nursing care can help eliminate bladder distention and fecal impaction. Physicians performing pelvic examinations should consider coating the speculum with anesthetic jelly before insertion or prophylactically giving patients sublingual nifedipine 30 minutes before procedures known to elicit AD.⁸³ All care givers should make sure monitor belts are not too tight, because AD has been reported in response to monitor belt usage. Despite the theoretic advantages of HUAM, the possible occurrence of AD remains a relative contraindication to its use in particularly susceptible patients.

No matter how meticulously care givers attempt to avoid AD, it still occurs in some patients. Early recognition helps prevent many of the severe sequelae. Many of the case reports of cerebral hemorrhage or other negative outcomes involve cases where AD had initially been ignored or mistaken for pregnancy-induced hypertension (PIH)/preeclampsia.⁸² Care givers must remember that AD develops early in labor. One helpful way to distinguish AD from PIH is to note changes in blood pressure with contractions. In cases of AD, blood pressure builds rapidly with contractions and abates. Indeed, patients should be forewarned during antenatal visits to report periodic headache, because this often serves as the only subjective clue to AD and the onset of labor! Once present, AD can be treated by removal of the eliciting stimuli or the use of parenteral antihypertensive agents, regional or general anesthetics, or other pharmacologic interventions.⁸⁴

The most reliable method of rapidly eliminating AD is induction of spinal or epidural anesthesia extending to the T10 level. Indeed, care givers should strongly consider the placement of epidural catheters before the onset of labor, if possible. Many have recommended epidural meperidine or other spinal or epidural narcotics to avoid nonselective motor, sensory, and sympathetic blockade more common with anesthetic agents.⁸⁵ Severe sympathetic block can lead to hypotension, bradycardia, and even asystole. Traditional antihypertensive agents used to treat PIH, such as nifedipine and labetolol, may also be used in the patient with AD to control acute symptoms. Other drugs that may help alleviate acute symptomatology include diphenhydramine hydrochloride, promethazine hydrochloride, and diazepam. Although obstetricians may shorten the second stage via forceps or vacuum extraction in the patient with AD, they should perform cesarean section only based on standard indications.

Further management remains supportive. ACOG recommends continuous monitoring of cardiac rhythm and blood pressure via an art line in all patients with hyperreflexia, but despite the widespread availability of technologic assistance, care givers must not forget to rely on a more conventional – and, perhaps, more effective – alternative: listening to the patient. Most patients with SCI patients can describe events that have commonly led to AD or are aware of prodromal symptoms and signs. Perhaps nowhere is a critical alliance between medical and nursing staffs and these patients more necessary than in dealing with this syndrome.⁸⁶

Postpartum issues

Disabled parents face additional challenges posed by their unique disabilities. Most patients, including those with SCI, produce breast milk and should strongly consider nursing their infants. Early mobilization and physical therapy help prevent deep vein thrombosis in chronically immobilized patients. Although most sources report similar frequencies of wound breakdown in disabled and nondisabled populations, others have warned that denervated tissue absorbs catgut suture less effectively than normal tissue and advise against its use in perineal repair.⁶⁴

Very few studies adequately document the effects of disability on childrearing. Although some authors have reported detrimental effects, most of these studies fail to distinguish between physical and mental disabilities.⁸⁷ One Swedish study of parents with SCI confirmed low divorce rates, excellent child adjustment, and little family requirement for external support.⁸⁷ Certainly, mentally disabled patients might be unable to interpret infant cues necessary to breastfeed and often have unrealistic expectations of children. Deaf patients might wish to have their children referred to an auditory stimulation program. All patients and their families, regardless of disability, might benefit from a family meeting with their health care provider before delivery to anticipate common postpartum problems. Health care providers should remain cognizant of the pervasive fear many disabled parents have that an external agency or health care professional might attempt to have children removed from the care of the disabled parent. Indeed, in one survey of more than 1000 parents with disabilities, 15% reported attempts to remove children from their households.⁸⁸

 In 1998, Through the Looking Glass (TLG), an advocacy group dedicated to assisting families with disabilities since 1982, became the first National Center on Parents with Disabilities. Funded by the National Institute of Disability and Rehabilitation Research, TLG provides information to disabled parents and performs research regarding issues pertaining to parenting with a disability. Research performed at the center has demonstrated the ability of patients with a range of disabilities to adapt to childrearing. They have also assisted with the development of accessible baby care equipment.

Despite the obstacles, most patients interviewed after childbirth voice no regrets, and many patients followed at the Rehabilitation Institute of Chicago have had more than one child. Most disabled women enjoy sharing their experiences with similarly disabled women and constitute an essential resource underutilized by health care professionals.

Those in need of further information regarding parenting with a disability or pregnancy among disabled women may contact the following resources:

The Center for Women With Disabilities

http://www.ric.org/conditions/community/women-disabilities-center/index.aspx
  Rehabilitation Institute of Chicago
  345 East Superior Street, Room 106
  Chicago, IL 60611, USA
  (312) 238–7997

The Center for Research on Women with Disabilities

Baylor College of Medicine

http://www.bcm.edu/crowd/

Americans with Disabilities Act: Questions and Answers

 http://www.ada.gov/q&aeng02.htm

 Through the Looking Glass

  National Center for Parenting with a Disability
  Contact: (800) 644–2666

http://lookingglass.org/index.php