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.

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 (178,179). 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.

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 (38). 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 (181,182). There is also an associa­tion between the presence of a neurogenic bladder and the development of bladder cancer (183). 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 (184). 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 (185,186).

Adults with latex allergies may have a higher rate than children for reactions, including anaphylaxis (187,188). 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 able­bodied 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 (186).

Sexuality and sexual function is 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 (189). Recent data suggest 24% of adults have an active sex life and gender, and continence did not factor into this statistic (190). 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 (191,192). Sildenafil (Viagra) may improve erectile function in 80% of men (193). Fertility is impaired, as only 14% of men report fathering children, and neurologic level is an impor­tant factor (192). Women with spina bifida generally have normal menstruation, and 88% have adequate vaginal secretions during intercourse (191). Women with spina bifida are able to conceive and have chil­dren (186). Sexual counseling should inform individu­als about risk for pregnancy, sexually transmitted diseases, and contraception advice. Risk and benefits of Gardasil human papillomavirus vaccine should also be provided prior to sexual contact.