LONG TERM
AGING WITH A NEURAL TUBE DEFECT
Adults with spina bifida have the normal aging medical problems in addition to those associated with their disability. Age associated changes can have an impact on medical and functional systems. In treating the adult patient, one has to evaluate the usual age-related medical problems as well as those unique to this population. Medical complications and cardiovascular disease may present at an earlier age. Successful transition to adult-based clinics appears to be based on a few key factors and include preparation, flexible timing, care coordination, transition clinic visits, and interested adult-centered health care providers (273). Adults with chronic conditions generally require more medical visits yearly and have an admission rate nine times more than the nondisabled. Adults with spina bifida in general are satisfied with life, but the area of largest concern is in self-care ability and partner relationships (274,275).
Spina bifida is associated with abnormalities in the brain and spinal cord. Approximately 90% of adults will have ventricular-peritoneal shunts. Shunt malfunctions can occur at any age and present with the classic symptoms of chronic headaches, vomiting, personality changes, concentration difficulty, and other neurologic changes. Shunt malfunction can lead to significant morbidity, mortality, and sudden death (276,277). Treatment is geared toward reducing pressure within the ventricular system either by shunting or ventriculostomy. Adult-onset tethered cord should be considered in a deterioration of neurologic status, bowel or bladder changes, increasing orthopedic deformities, and gait deviations.
There are several age-related musculoskeletal and orthopedic complications. Spinal deformities, including scoliosis, kyphosis, and lordosis, can increase over time and cause back pain. Chronic lack of sensation and muscle imbalances can lead to Charcot joints. Overuse syndromes are common in wheelchair and crutch users. Wheelchair mobility tends to cause stress on the upper extremities, while community ambulators develop knee and hip pain. Carpal tunnel and rotator cuff disease are well documented in wheelchair users (278). Gait abnormalities from underlying muscle weakness can cause undue stress on joints in the lower extremities.
Neurogenic bowel and bladder function is an important component of adult medical care. It is a rare individual with spina bifida who has a completely normal urinary system. Despite these abnormalities, more than 80% of adults are able to develop social bladder continence (68). Methods to achieve this goal include all those previously discussed. In the past, renal damage leading to renal failure and death was a major contributor of morbidity and mortality in adults with spina bifida; although this is much improved, it still remains a problem (279,280). There is also an association between the presence of a neurogenic bladder and the development of bladder cancer (281). Recent data suggest that this occurs at a young age in the population that develops bladder cancer, with variable pathology and has a poor prognosis (282). Neurogenic bowel function can change over time. Gastric motility seems to decrease with age and affects bowel programs. Treatment needs to be adjusted for these changes and includes different medication, dietary modifications, and newer surgical interventions.
The development of chronic skin problems is inherent in those with insensate skin. Aging causes changes in fat and muscle distribution, which can affect pressure ulcer formation. In the lower extremities, bracing can cause pressure and shear over bony prominences. Burns and abrasions can occur in unprotected skin. The wheelchair seated position results in pressure in the ischial and sacral areas. Prevention is imperative to avoid these secondary complications. The economic burden, along with psychological and functional impact, can be devastating (283,284).
Adults with latex allergies may have a higher rate than children for reactions, including anaphylaxis (285,286). This is probably related to repeated exposure to latex over the years, along with the increasing presence of latex in the environment.
Obesity is a health-related problem for both able-bodied and disabled adults. Nutritional studies indicate a decreased caloric expenditure with the disabled adult. Metabolic syndrome is more common in those with obesity and places these individuals at risk for coronary artery disease, diabetes, and hypertension. Interventions include nutritional counseling and healthy eating, exercise and fitness, and weight reduction. Interestingly, most researchers focus on obesity, although eating disorders also occur in the disabled population (284).
Sexuality and sexual function are often overlooked in the disabled population. It is a huge disservice not to address these issues. Current data shows that the majority of males and females with spina bifida have a desire for intimate relationships, including sexual contact (287). Recent data suggest 24% of adults have an active sex life, and gender and continence did not factor into this statistic (288). Men with spina bifida report ability to achieve erections in 72%, and 67% experience ejaculation, but only one-third are happy with the amount of rigidity (289,290). Sildenafil (Viagra) may improve erectile function in 80% of men (291). Fertility is impaired, as only 14% of men report fathering children, and neurologic level is an important factor (290). Women with spina bifida generally have normal menstruation, and 88% have adequate vaginal secretions during intercourse (289). Women with spina bifida are able to conceive and have children (284). Sexual counseling should inform individuals about the risk for pregnancy, sexually transmitted diseases, and contraception advice, including nonlatex condoms. Risk and benefits of Gardasil human papillomavirus vaccine should also be provided prior to sexual contact.
VOCATIONAL COUNSELING
Vocational counseling is an important aspect of transitional care of the individual with spina bifida, and current information in this area is limited. Recent data from the Netherlands reports a work rate of 62%, although 22% were in a sheltered environment. The definition of employment was based on at least 1 hour of paid wages per week. The best predictor of employment was level of education. This, along with gender and ability to care for self, was an important predicator of full-time employment (292).
Functional vocational planning should be started early in secondary school, assessing career interests, skills, and aptitude. The potential for success in a post-secondary school program should be explored along with vocational job training. A positive realistic approach may provide the best solution in planning for adult employment options.
