The May 9-10, 2003, Evidence-Based Practice in Spina Bifida Conference was a milestone: the first time ever that researchers and medical providers from all over the country gathered to assess the current state of research in spina bifida and plan a much-needed, long-term research agenda. And not a moment too soon!
Although there are many more adults living with spina bifida than ever before, there’s very little high-quality research on spina bifida in general—and even less on how spina bifida affects people throughout the life cycle.
Our current system does not promote ongoing, coordinated health care for adults living with chronic conditions, which creates significant health management challenges for adults with spina bifida. Most physicians who treat adults were not trained in what were once considered pediatric disorders. According to Timothy Brei, MD, “We've grown beyond the boundaries of established medical research. We are the pioneers. Many adults with spina bifida know more about their bodies than the physicians they see.”
Mark Merkens, MD, suggests that adults who lack access to an adult spina bifida clinic might want to look for a rehabilitation physician for primary or specialty care.
Health Management and Aging With a Disability
As people age, they experience various physical manifestations of the aging process. Muscle strength, flexibility and endurance decrease, and the risk of osteoarthritis and osteoporosis rises. Aging affects the central nervous system, decreasing sensation and balance. The skin thins and becomes less elastic.People with spina bifida experience the usual manifestations of age, but since spina bifida puts pressure on many body systems, age-related declines in affected areas may occur sooner or be more severe.
"SB docs at national conferences always talk about aging "in SB years". That is to say, things that happen to people as they age, happen to people with SB faster. Because of how we're built, and/or the way we walk/roll in wheelchairs, we develop things like arthritis sooner. Rotator cuff issues. Hip problems. This also applies to internal organs. Kidney damage may occur sooner. Heart and lung issues from scoliosis some of us have. We just age faster. Nobody knows how long a lifespan those of us who are shunted can have!! I know of people with SB in their 50s and 60s and 70s.....but none with hydrocephalus. Had they been born with hydrocephalus in the 1930s, 40s and 50s, they probably would not have survived. Since 80% of people with SB also have hydrocephalus, we are the first generation to survive in large numbers, thanks to the advent of the modern day shunt in 1958."~~~ Jesus Arroyo
Shunt Failure
Shunt failure happens to adults, even those whose shunts have not been working for years. The symptoms of shunt failure in adults are usually the same as those in children. Dr. Merkens describes the symptoms as follows: “The symptoms of shunt failure may creep on you. Your thinking may be slower, you may be less facile with words, more hesitant in general and a little clumsier on your feet. Neurosurgeons who treat adults may be skeptical of shunt failure in adults, especially if the shunt hasn't been working for a while. But what I tell my students is ‘If there’s a neurological problem, it’s the shunt, stupid.’ It’s the mantra in new generations of young neurosurgeons.”
Chiari Brain Stem Compression
Chiari brain stem compression is likely to cause sleep apnea or loss of respiratory drive. The symptoms may be subtle at first. Dr. Merkens says, “Maybe you've been doing fine for years, but now you've contracted pneumonia a couple of times and seem to have more trouble recovering.”
According to Dr. Merkens, it generally doesn't happen for the first time in adulthood. But neurosurgeons say that when you’re starting to think about chiari brain stem compression, it’s a good idea to also check the shunt. A shunt failure increases pressure in the brain, pushing the skull down into the cervical spine—exactly what chiari brain stem compression is. It could present with a weak voice, pneumonia, more difficulty swallowing, difficulty with vocal chords and sometimes even problems with the eyes.
Spinal Cord Tethering
Spinal cord tethering can occur for the first time at any age, although it’s more likely to occur if there has been a previous tethering and detethering, which may have created scarring. Tethering usually presents the same signs and symptoms in adults as in children: basically anything that changes below the level of the spina bifida—the level of the spine where the defect occurred. There may be urinary changes, such as infections or difficulty staying dry, or sudden changes in bowel patterns.Skin sores may be another symptom of tethering. Orthopedic changes, such as a foot starting to turn out, or scoliosis that progresses fairly quickly may signal tethering, as can a change in sensory level. Dr. Merkens says, “If you used to be able to feel down to your shins but now can’t feel your knees, tethering may be responsible.”
However, before contemplating detethering surgery, the shunt should be checked. If a pediatric, orthopedic or urology exam shows changes that suggest tethering, the next step is to get an MRI of the entire neurological system, to check for tethering, spinal syrinx and shunt failure. If the results are inconclusive, a pressure check of the shunt may be necessary.
Urinary and Bowel Systems
The rate of renal failure has declined significantly since the introduction of CIC(intermittent catheterization), but we’re not home free. Medical providers know that CIC reduces urinary tract infections (UTIs) and prevents urine backups, but its long-term effects are still unknown.
Dr. Merkens mentioned two young men with artificial sphincters who ran into serious trouble by not following their programs and said, “We don’t do enough to identify good candidates for artificial sphincters. Good candidates must catheterize religiously and have a low incidence of UTIs. An artificial sphincter basically puts a cork in the bladder, and urine must be emptied completely several times a day, or else kidney damage may result.”
People who have had bladder augmentation need to be followed closely over their lives for any signs of cancer, problems with mucus or kidney stones. The long-term effects of augmentation are still unknown. Adults with illeal loops must be followed closely, because there’s a chance that over time the loops may allow urine to back up into the kidneys.
Most difficulties with bowel programs relate more to behavior than to the level of spina bifida. Success generally depends on a consistent diet, regular exercise and timed evacuation. Doctors are not yet sure about the effects of chronic enemas, rectal bulbs and similar practices, so affected areas need to be checked regularly. Dr. Merkens raised the issue of mega-colon which refers to the bowel becoming larger from regularly holding large amounts of stool. Though it is certainly an excellent method of bowel management, we don’t yet know of potential complications of the Antegrade Colonic Enema (ACE) over time.
Dr. Merkens says, “We are learning to be more aggressive about bowel programs earlier in life to prevent later complications such as mega-colon In Great Britain, for example, the goal is for children to be in a functional bowel program by the time they are 6-years-old.”
Orthopedic and Skin Issues
According to a 10-year-old German study, over 50 percent of adults may have poorly fitting, poorly functioning braces. Premature arthritis is often a problem for adults with spina bifida, especially for those who use their shoulders to ambulate. Knees are also prone to arthritis, particularly in adults with a wobbly gait who don’t use crutches. Lots of back-and-forth and side-to-side motion is very hard on the knees.Scoliosis can progress in adults if it wasn't fused in adolescence. Skin breakdowns can cause bone infections, especially at the heel or toes, which can be difficult to heal. Orthopedic problems can occur as a result of pressure and abrasion, poor circulation, neurological changes and edema. Dr. Merkens advises adults with spina bifida whose feet swell to elevate them several times a day. If your feet are purple or red, wearing pressure stocking is a good idea. Adults need to check their skin often. Wheelchair users need to use a mirror to check their rear daily and remember to shift positions every 15 minutes to avoid pressure sores.
Dr. Merkens recommends that adults visit a podiatrist annually, to ensure that their feet remain healthy.
Latex Allergy
Latex allergy is more likely to affect those born from the late 1980s to the mid-1990s. The rate of latex allergy in people with spina bifida born from 1987 to 1995 is over 50 percent, but the rate since then is much lower, almost certainly due to improvements in the latex purification process. Almost all large, teaching hospitals have latex-free operating rooms, but patients may have to specifically request a latex-free environment at smaller hospitals.Before visiting the dentist, adults need to request a latex-free environment, which means that the patient in the previous appointment slot will need to receive latex free treatment, too. In fact, it’s a good idea to ask for the first appointment in the morning, when the room is cleanest and most latex-free.
Hypertension
Dr. Merkens reports seeing elevated blood pressure in many of his adult patients, at younger ages than usual. If blood pressure goes up, he suggests having a urologist evaluate the kidneys, because kidney problems can raise blood pressure. Elevated blood pressure—higher than 140/90—needs to be treated and followed closely.
Lung Health
There are two types of lung disease: obstructive and restricted. Obstructive is when something is blocking breathing, such as in sleep apnea. Restrictive lung disease is due to the inability of the lung to fully expand when taking a breath, which may be caused by scoliosis, Chiari, or even a weakness in the abdominal or chest muscles. Some of the signs are very subtle. But Dr. Brei advises adults who snore, feel tired or have to take naps during the day to see a physician for an evaluation.
Obesity and Exercise
Obesity is major health problem in the general US population, but it’s even more problematic in adults with spina bifida. According to Dr. Brei, “As we move into our teens, we have a tendency to become heavier, which contributes to high blood pressure, diabetes, heart disease and sleep apnea.”Many adults with spina bifida have decreased mobility and spend more time in wheelchairs. Those who walk often tend to walk less as they age. Dr. Brei says, “Exercise is tremendously difficult for many of us, but it is so important. And vigorous, heart-pumping exercise is what we really need to maintain our cardiovascular health and manage our weight. Starting a program may be very difficult, but it’s essential to find a physical therapist, recreation therapist or fitness trainer you can work with.”
Regarding regular exercise, Dr. Brei says, “If I had the answer for this, I’d be rich. There are institutional barriers that are difficult to overcome, but it’s also true that some of us are not very motivated to exercise. I absolutely despise exercise. I've never felt those little endorphines that are supposed to make you happy while you work out. But I go to the gym as religiously as I can.”
Interpersonal Relationships
Interpersonal relationships are very important for everyone, including adults with spina bifida. Studies show that satisfying relationships improve both longevity and mental health. Dr. Brei says, “It’s not easy to go out and try to meet people. It’s often uncomfortable at first and it exposes us to hurt. But if we don’t seek out relationships, they’re probably not going to find us.”
Pregnancy
Spina bifida does not appear to affect a woman’s ability to become pregnant. Although the evidence is sketchy, it appears that spina bifida can complicate pregnancy, and although most difficulties may disappear when the pregnancy is over, there are some reports of women losing mobility and not regaining it after pregnancy. Women who want to become pregnant should work closely with an obstetrician specializing in high-risk pregnancies.They should also talk to their doctor about taking prescription strength levels of folic acid prior to pregnancy since they are at higher risk of having a child with spina bifida.
Pain
Adults with spina bifida often experience considerably more pain as they age, which may be due to joint stress, muscle pain or arthritis. Dr. Brei advises adults in pain not to suffer in silence, but to tell their doctor how they feel. There are many pain management programs that can help.
Substance Abuse
Some research shows substance abuse as higher in adults with spina bifida than in the general population. If that’s true, it could be self-medication for pain, depression, isolation or any of a number of other issues. Dr. Brei says, “I worry because substance abuse hampers your ability to be independent, to succeed, to achieve goals. If you feel depressed or anxious, seek help. Counseling can help and several drugs out now are very effective in treating depression and anxiety.”
Basic Health Care Guidelines
Adults with spina bifida need to follow the guidelines for periodic health checks on all body systems: blood pressure, height/weight, cholesterol levels, colon health, tetanus shots, pap smears, breast exams, testicular and prostate exams, urinalysis, renal ultrasound, renal blood test, spine x-ray, eye exams and more. SBAA publishes guidelines on health maintenance for people with spina bifida, which Dr. Merkens is in the process of updating.In summing up, Dr. Brei offers this advice: “Avoid smoking, drugs, and drinking too much. Find work you enjoy. If that’s a problem, volunteer your services. Develop social supports, such as finding others who enjoy the same hobbies you do, or join a group at church. If you’re working, get together with people at work. Meet your neighbors. Get lots of sleep. Find exercise you can do and stick with it.”
Adults living with spina bifida need to monitor their health regularly and seek out health care at the first signs of trouble. Health care is one of those areas where being proactive pays big dividends—and waiting too long to react can create serious problems.
+++This article was adapted from the 2003 SBAA Annual Conference session “Health Care Issues for Adults With Spina Bifida,” presented by Timothy Brei, MD, and Mark Merkens, MD.
***This information does not constitute medical advice for any individual. As specific cases may vary from the general information presented here, Spina Bifida Family Support advises readers to consult a qualified medical or other professional on an individual basis.
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