“My child with cerebral palsy is now an adult. Will his medical problems change?” CLYDE E. RAPP, JR., MO., PENN VALLEY, PA
“Just because people get older doesn’t mean their disabilities disappear.” This statement was made by a developmentally disabled adult, which was included in a 2002 report of a 2001 Surgeon General’s conference devoted to meeting the medical needs of the developmentally disabled.
Indeed developmental disabilities, including cerebral palsy, do not disappear and may worsen without proper care. Therefore, if you are the parent of a child with cerebral palsy or have cerebral palsy yourself, it is important that you are informed about the medical problems of the adult with cerebral palsy Begin to inform yourself early (when your child reaches age 16 or 17) since many of the medical support systems (and individuals trained to administer care), are no longer as available after age 21. We know that 90 percent of all patients with cerebral palsy survive until adulthood.
Ninety-five percent of patients with spastic diplegia (where the spasticity is confined to the lower extremities) and seventy-five percent of patients with spastic quadriplegia (where spasticity involves all four extremities) survive until age 38. Average survival may increase even more as part of the increased survival of the population in general. For those patients who are already adults, many of them (as well as their families) may already be frustrated by the lack of an accessible repository of knowledge of cerebral palsy in adults.
Adolescence and Planning for the Future
Keeping in mind the increasing survival of individuals with cerebral palsy, it might be wise (if your child is an adolescent) to talk with your teenage child’s physician about the availability of care for adults with cerebral palsy. This may mean staying with your present physician if he or she is a family physician who is experienced in caring for adults with cerebral palsy. Some pediatricians will continue to care for patients with cerebral palsy even after they become young adults and a few internists (although certainly not enough for the number of patients who require care) may have had experience with cerebral palsy. In addition, some physiatrists (specialists in physical medicine) may be able to provide general care. There are a small number of hospitals which have special clinics experienced in the care of adult patients with cerebral palsy. Whatever physician you choose, they should not only have experience treating patients with cerebral palsy, but should also be familiar with many of the common medical problems of adults (such as hypertension, breast cancer, and coronary artery disease). Finding such a physician may not be an easy task, as there are very few training programs in medical centers in the treatment of adults with cerebral palsy (or the treatment of adults with other developmental disabilities).
Local United Cerebral Palsy offices may be helpful in locating a practitioner in your area who is experienced in caring for adults with cerebral palsy. (Look on their website, http://www.UCP.org. Search engines such as Google may also be helpful.)
There are, of course, continuing financial problems related to providing care at home particularly as parents age. Socially, there are often vexing problems of finding employment and living arrangements and achieving as much independence as possible. These problems, which are certainly important, have received more emphasis than the medical problems of the adult with cerebral palsy. The focus of this article is on the medical problems of this group, which may provide you with some information about what problems to expect during adulthood and some suggestions for avoiding them. The most common symptoms and problems caused by cerebral palsy will be described with an emphasis on those problems that tend to become worse when patients become adults or those that are unique to the adult with cerebral palsy. Hopefully, this will not only provide you or your relative with cerebral palsy with practical information but provide you with a basis for discussion with your physician when you or your relative reach adulthood.
Clinical Problems of Concern to Adults with Cerebral Palsy
HEARING AND VISION PROBLEMS
Problems with vision exist in one-fourth to one-third of adult patients with cerebral palsy. Usually the schools require yearly visual exams, but after age 21, this is usually left up to the family or the patient. It is important to have a visual exam yearly as an adult because of decreased visual acuity in some patients as they age. In addition, studies of intellectually disabled patients (including a number with cerebral palsy) of various ages have noted that approximately twenty-five percent have never had a visual exam. Children with cerebral palsy may have visual problems including cataracts, optic atrophy, and retinitis, and in these instances, a yearly follow-up by an ophthalmologist is important after age 21, as it is when they are younger.
Approximately ten percent of adult patients with cerebral palsy have hearing problems. As in the case of vision, hearing problems may become worse during adulthood, so yearly testing is advised.
THE CARDIOVASCULAR SYSTEM
The incidence of coronary artery disease (which causes heart attacks) and high blood pressure among adults with cerebral palsy is similar to that of the population as a whole. This of course means that monitoring cholesterol levels and blood pressure levels on a regular basis is still a necessity. How often they are monitored depends on the patient’s age and whether there are any abnormalities in either of these important predictors of heart disease. Cholesterol levels are occasionally significantly elevated even in young adults, probably as a result of inactivity, and there are of course medications available to lower cholesterol levels.
In the intellectually disabled patient with cerebral palsy (as with any intellectually disabled patient), the signs of chest pain or pressure may be subtle. Rather than pointing to his or her sternum or complaining about pain in the chest (if the patient is verbal), a family member or a caregiver may notice increased agitation or a change in skin color.
Adults (and children on occasion) may develop swelling or discoloration of the extremities due to abnormal involuntary nerve impulses which affect the tone of the veins or arteries supplying the lower extremities. Decreased temperature of the extremities usually does not lead to serious problems in these cases (except that the patient should wear warm trousers or stockings to avoid excessive cold in the winter), but the patient’s physician should be aware of such changes.
Swelling of the lower extremities due to poor venous tone can occur (again as a result of abnormal involuntary nerve impulses). These patients may need elastic stockings depending on the extent of swelling which can be evaluated by the patient’s physician. This type of swelling can occur in children as well as adults (as does decreased temperature of the limbs). Obviously due to the change in vascular tone in adults that occurs naturally with age, it is much more severe in a forty or fifty-year-old patient than a twelve-year- old child, in most instances.
Aspiration (as a result of poor swallowing or reflux of stomach contents) is a common problem in patients with cerebral palsy and varies greatly in frequency and severity. The remarks that follow concern the group with serious and/or frequent aspiration. Adults compose a large percentage of this group, since obviously adults are more likely to have a long history of recurrent aspiration.
Recurrent aspiration can cause fibrosis (or scarring), which effectively destroys a portion of the lungs. We are not able to detect damage to lung tissue with either measurements of oxygen saturation of the blood or pulmonary function tests, which measure such things as how much of the oxygen we breathe diffuses into the blood stream and the amount of viable or non-fibrosed lung tissue, until thirty to fifty percent of the lung has been destroyed. Therefore, if a patient has a history of frequent aspirations, particularly if his oxygen saturation decreases dramatically at the time of the acute episode, it is reasonable to assume that fibrosis is present. Certainly these clinical characteristics do not prove that fibrosis is present, but the assumption is a valid one based on postmortem (autopsy) studies.
The result of scarring in adults who aspirate frequently is that when they aspirate, their respiratory status can deteriorate quickly, and urgent medical attention is needed. Secondly, it is important for your physician to determine whether the aspiration is a result of a swallowing problem or reflux of stomach contents, since the treatment for swallowing difficulties involves a change in food consistency, and reflux of stomach contents may require the placement of a tube in the stomach (a gastrostomy tube) or the small intestine (a jejunostomy tube). Swallowing difficulties may also become serious enough to require tube placement as well. The cause of aspiration is important to detect in any patient with cerebral palsy, but it is more important to determine in a patient with significant fibrosis because of his or her medically fragile state.
THE GASTROINTESTINAL SYST\EM
Vomiting (with or without aspiration into the lungs) and constipation are common in some patients with cerebral palsy, and usually becomes more frequent as the patients age.
Although there is no scientific proof, both the vomiting seen in these patients as well as the constipation are thought to be a result of abnormalities in the involuntary (or autonomic) nervous system. These abnormalities delay the movement of undigested and partially digested food through the stomach (causing vomiting and occasionally aspiration as previous discussed) as well as dilatation of the small and large intestines. Poor movement through the large intestine (or colon) leads to constipation. We have no effective and safe medications which increase the rate of transit of intestinal contents. Therefore, in addition to the traditional means of preventing constipation such as adequate fluids and as much activity as the patient is capable of, vigorous use of laxatives and occasionally enemas is recommended.
Serious constipation may lead to the stool becoming impacted, which can, in turn, cause obstruction if constipation is persistent or prolonged. The use of appropriate laxatives is important because some simply do not work and some have serious side effects if used in excess. Therefore, once again, it is important to consult your physician.
As mentioned in a prior paragraph, the stomach occasionally does not empty properly in patients with cerebral palsy, causing reflux of the acidic stomach contents into the esophagus. This can lead to inflammation and even bleeding, usually after it has been present for years, which means such signs and symptoms of damage are more common in adults. We now know that acid reflux predisposes the patient to cancer of the esophagus, which is rare, but has been seen in adults with cerebral palsy who have reflux esophagitis. Endoscopy, where the lining of the esophagus is visualized by means of a flexible fiber-optic tube used to detect changes in the esophageal lining, is the best way of detecting such inflammation. If symptoms of reflux (usually burning over the front of the chest) are persistent, you should discuss the matter with your physician. If he or she concurs that endoscopy is necessary, it will not only verify the diagnosis of esophagitis (and rule out the remote possibility of cancer), it will also prompt the physician to explore the possibility that the symptoms may be the result of another condition. It bears mentioning that there is a condition called “Barren’s esophagus” (a change in the type of cells lining the esophagus) resulting from inflammation that can be seen by the endoscopist, which increases the likelihood of cancer, and therefore is an indication for more frequent endoscopic exams.
THE MUSCULOSKELETAL SYSTEM
Degenerative arthritis is more common in adults than children in the general population, and the adult with cerebral palsy has the added problem of increased stress on the joints because of muscle spasticity and contractures. Degenerative arthritis can obviously affect any joint. Most important in the adult with cerebral palsy is degenerative hip disease. Symptoms include increased pain while sitting and standing and pain in the groin and anterior thigh. These patients obviously require orthopedic referral.
Twenty-five to sixty-four percent of adults in institutions have scoliosis. It is of particular concern in patients with cerebral palsy since many patients are unable to shift their weight when lying or sitting down and decubiti may be the result of persistent pressure. Often scoliosis accelerates during puberty which can occur late (in the late teens or even the early twenties) in patients with cerebral palsy, so the patient’s parents or caregivers must be aware of the increase in the scoliotic curve during this period.
Osteoporosis is more likely to occur in patients with cerebral palsy because of decreased weight bearing, decreased exposure to sunshine, poor calcium intake, and often interference with the body’s production of vitamin D because of medications (Dilantin being the main culprit). Patients of all ages who have frequent fractures should have a measurement of bone density, usually by X- ray absorptiometry. Calcium supplementation is important for any female patient who is post-menopausal, or any patient (male or female) who has low bone density. The possibility of osteoporosis is always present in an adult with cerebral palsy. Therefore, it is certainly appropriate to broach the topic of measuring bone density with the patient’s physician, particularly since he or she may choose to use one of a group of medications (the biphosphonates) if the patient has low bone density.
It is worth noting that younger patients have spasticity where there is resistance to movement (resistance to extension of the arm on physical exam) but not absolute rigidity that does not allow the arm to be extended beyond a certain point. Where such rigidity exists, the patient is said to have a contracture. The reasons that spasticity develops into contractures is that there are certain changes in the brain and spinal cord with age and often because the patient does not continue some form of physical therapy including stretching. There is currently no method of controlling the changes in the brain and spinal cord, but adhering to a regular schedule of physical therapy and stretching will minimize the severity of contractures.
SEXUALITY AND THE PELVIC AND BREAST EXAM
There are no medical, social, or psychological reasons why adolescent or adult females with cerebral palsy cannot become sexually active, and they are therefore subject to the risks of unwanted pregnancy and sexually transmitted dis ease after puberty. Puberty often occurs late in patients with cerebral palsy, as mentioned previously, so parents and caregivers should not be surprised if sexual behavior begins late in adolescence or in early adulthood.
Given the above facts, counseling about sexuality should be an agenda item for the adolescent with cerebral palsy (for both males and females) to the extent that their degree of intellectual disability allows. Illustrations and educational videos may be helpful for this type of counseling, and very severely intellectually disabled patients may need close supervision because of the possibility of sexual activity as well as abuse.
Any female adolescent or adult with cerebral palsy who is sexually active or is eighteen years old or older should have a pelvic exam. Most patients have some anxiety about the pelvic exam and if your physician concurs, the use of a mild sedative may be necessary. The discomfort of the exam may be decreased in patients whose spasticity prevents them from spreading their legs completely by having the patient lie on her left side and the use of a small (or pediatric sized) speculum. Consulting a physician who has experience performing pelvic exams on patients with cerebral palsy or other types of physical disabilities is recommended if such an individual exists in your area.
Because of the technology and design of the X-ray equipment for performing mammograms, it is often difficult to perform a mammogram on patients with cerebral palsy who have contractures or a spine which is fixed in a flexed and curved (kyphoscoliotic) position. Occasionally mammograms, which patients should begin having on a yearly basis at age 40, cannot be done. In this case, annual breast exams should be supplemented by monthly exams by the patient or by a qualified professional, such as a visiting nurse or nurse practitioner. Ultrasound exams are used to supplement the breast exam if an abnormality is suspected, but are not a substitute for mammography.
Studies have indicated that children with cerebral palsy have a decrease in lean body mass (which essentially means that muscle mass is decreased) and the same is true based on the observations of many physicians who care for adults. From a nutritional standpoint, this means that it is important to maintain adequate caloric intake and particularly important to maintain adequate protein intake, so that they do not lose further muscle mass and their resistance to infection (which to some extent depends on adequate protein intake) will not decrease. Many family members are hesitant to give permission for the insertion of a gastrostomy tube, since they feel that this means that the patient will no longer be able to enjoy meals. It may be necessary for proper nutrition in some patients, and the patient may still be able to enjoy small snacks, so that the taste of food and pleasures of eating can be maintained to some extent.
Studies of adults with cerebral palsy have also shown that a number of patients are deficient in iron and calcium intake. Periodic blood counts and serum iron and serum calcium levels will detect any deficiencies which require supplementation with calcium or iron.
Particularly since the increase in life span of adults with cerebral palsy has resulted in a greater number of adults with cerebral palsy, it is important to become aware of the medical problems they encounter. Many of these problems are simply problems that existed when they were younger and have become more serious, and some are seen almost exclusively in adults.
It is the author’s hope that cerebral palsy will become a condition that one lives with rather than one from which one dies. Hopefully, with the help of new training programs in medical schools and hospitals (as now proposed by the American Academy of Development Medicine and Dentistry along with the help of advocates, physicians, and other caregivers), this vision will soon become a reality.
Whatever physician you choose, they should not only have experience treating patients with cerebral palsy, but should also be familiar with many of the common medical problems of adults.
Counseling about sexuality should be an agend\a item for the adolescent with cerebral palsy to the extent that their degree of intellectual disability allows.
CLYDE E. RAPP, JR., M.D., PENN VALLEY, PA
Dr. Rapp is currently an Adult Developmental Disabilities Consultant and is an internist. Prior to his present role, he ran the Center for Adults with Developmental Disabilities at Albert Einstein Medical Center in Philadelphia for twelve years. He is also a member of the Executive Board of the American Academy of Developmental Medicine and Dentistry.
Copyright Psy-Ed Corporation Oct 2004