This pediatric module features aging with a disability, why adult physiatrists should treat these kids. This was updated in 2020 and Kevin Murphy initiated this module and has exquisite expertise in this lifetime care model. Thanks for his assistance with this. Lifetime care for pediatric patients with disabilities is very important as more and more are surviving till much older ages. Family medicine and internal medicine providers are often unable or uncomfortable because they do not understand the disabling primary condition the child has when they become an adult. And adult physiatrists are uniquely qualified to take over these cares. Pediatric PM&R providers would happily continue seeing many of these patients but their institutions may not allow it. And frankly there are a lot of adult problems that come with aging that we are not necessarily equipped to deal with and that adult physiatrists have great expertise in. The focus of course is on maintaining function with these young people as they age with their primary disability, have accompanying aging disabilities and have to deal with the social emotional living situation and change in equipment factors that accompany their primary difficulties. We'll first focus on adults with cerebral palsy. Cerebral palsy is a group of disorders that affect movement and posture. Most importantly it is a non-progressive static brain injury. So if a patient is worsening over time you cannot always attribute this to the primary injury that they have. The onset is in the first couple years of life and often is perinatal. Thus children that have been in a car accident, have drowned in a pond or have metabolic disorders when they are very young are often categorized as having a brain injury and associated with CP. Periventricular leukomalacia is a very common problem related to prematurity where the blood vessels break and cause scarring and this is most commonly seen with diplegic cerebral palsy which affects the lower extremities. Adults with CP are living longer and longer and there are a larger number in the United States at this time and it is growing. The quality of life that they have is also improving because society has included those with disabilities in the school system and in jobs more frequently in the last decade. Better medical care is available because we now understand what problems they may face and there are improved living situations for adults with disabilities who can no longer live with their parents. Unfortunately those with cerebral palsy have higher risk factors for some specific medical difficulties including breast cancer because of the frequent x-rays that may have been done for scoliosis, cardiovascular issues because many are deconditioned, not able to get physically active and may develop obesity, respiratory issues because of potential aspiration over the years and bronchopulmonary dysplasia from being born prematurely and then the typical high rates of diabetes, asthma, hypertension, stroke, depression that accompany anyone who is aging. Osteopenia and idiopathic fractures because of a non-weight-bearing status can also be difficult. So access to a physician who can coordinate care, understand and proactively address these issues and communicate in a language that they understand is exactly why adult PM&R fits the bill for this care. If a patient with cerebral palsy seems to have functional deterioration, don't blame the primary diagnosis initially. Make sure you look for vision or hearing loss which can have a greater impact on these patients than it might on others. If they are G-tube fed there may be nutritional deficits because no one is really monitoring anything except their weight loss or weight gain. Medication interactions are common as things may be added by a variety of different physicians because of sleep difficulties or behavior and depression is common because of the changes in social situation that may occur. Anxiety can be a big concern when patients are switching from living at home to living with a group home or in another setting. Arthritis and degenerative joint disease from walking on legs that have been biomechanically challenged over the years as well as having back pain can be problematic and many patients are very independent and will crawl around their homes versus give up that independent mobility. Thus their pain needs to be controlled and we also need to be aware that seizures, early dementia or stroke can be concerns as well. Of course as children age they get less therapy, their braces become outgrown, their wheelchairs and every piece of equipment needs replacement or repair and their caregivers will likely change as well. The adult PM&R physician can be the one who is proactive in addressing these concerns as many group homes, guardians or primary care physicians are not going to pay attention to these things. Long-term care planning, anticipating that parents will not be able to continue caring for them as they age is essential to discuss as early as possible. Spasticity management plays a huge role in most kids with CP's medical life as a child and shouldn't stop when they become adults. They continue to need orthotics, Botox, Baclofen, pump refills and injections into joints that are painful. They may need referral for orthopedic surgeries including hip replacements as they get older. Orthopedic issues are particularly difficult in CP patients because simple joint replacements that you might do in someone else are in a different biomechanical and muscular situation than in a typical adult. Hip dysplasia is very common as is scoliosis. Severe end-stage hip dysplasia can cause extreme pain and loss of ambulation. It can cause difficulties with seating due to asymmetrical pressure and pain. Many patients will have injections of steroids into the joint spaces to try to prolong the time before they might need surgery. Griddle stone procedure or resection of the femoral head is a salvage procedure done when nothing else can relieve their symptoms. For some patients a total joint replacement can be an option but needs to be done by an experienced surgeon who understands the altered biomechanics of someone with CP and who has worked with these patients over time. Otherwise failure is a big concern. Spondylolisthesis can be common in the ambulatory adult who has had a swivel gait and a lot of early arthritis is also an issue. Cervical stenosis can be a problem. Scoliosis is very common in adults with CP who have not been ambulatory and we certainly hope that this would have been addressed before they get to see you. Scoliosis procedures have made dramatic leaps in terms of growing rods and there is really no excuse for a patient to look like this because of the seating, skin and respiratory difficulties it causes. Back to concerns about the hip. Osteopenia is also common because many people are not appropriately weight bearing or have been vitamin D and calcium deficient and idiopathic fractures can be easily missed. This patient was noted to have a behavioral disorder which was really related to pain and an undiagnosed fracture. Patella ulta is very common and can lead to knee pain. Foot surgery such as a triple arthrodesis is common in children or young adults and can lead to early degenerative arthritis. But by and large the most common complaints of adults with CP are fatigue and pain because they have high energy expenditures to maintain their mobility and often do not have access to the exercise programs that could help reduce pain. Energy conservation principles need to be discussed and implemented. Labs need to be checked and treatment for osteopenia initiated. Counseling and an understanding that aging combined with their pre-morbid cerebral palsy related orthopedic issues can amplify and speed up some of the decline that they may not have anticipated would happen. Everyone wants to remain independently ambulatory and we need to insist that our patients use a device that will help them maintain that. They may need to crawl, scoot or find another means of mobility within their homes rather than walking if it is causing pain and would reduce their potential to continue walking when needed. Stand pivot trancers are essential to maintain so that caregivers who often refuse to use lifts can continue to care for their children. Neurogenic bladder is common in children with CP and is often treated with Botox injections or antispasmodic medications. If you have to go to the bathroom every 20 minutes, you are not going to be able to be toilet trained and it can be socially difficult to find a job if this is an issue for you as an adult. Neurodynamic studies can identify these issues early on and may continue to be repeated as adults. Hypoactive overstretched bladders can also lead to incomplete emptying which would require catheterization. In those cases, a metrophinol procedure can allow much easier catheting through a continence stoma on the abdomen, especially for women that may have difficulty otherwise. If the patient is having urinary difficulties, referrals are essential because reflux can lead to hydronephrosis and kidney failure. Bowel issues are also lifelong and constipation is a very difficult problem for many to solve. Most are on bowel programs with stimulants as well as softeners, but this can be time consuming and difficult for them to do. The use of dietary changes and assistance with enemas or continence stomas may be needed. The MACE procedure is often used in those with spina bifida, but can be used in cerebral palsy and allow much more independence. Next, we're going to discuss spina bifida, which is a neural tube defect that occurs very early in pregnancy. Folic acid has been shown to greatly decrease the risk of this, but there is a familiar recurrence rate and it is more common in specific populations. During pregnancy, this can be diagnosed quite early on, and if prenatal surgery is done, there is a definite reduction in the need for shunting. This is one of the most important signs of spina bifida. Next, we're going to discuss spina bifida, which is a neural tube defect that occurs very early on, and if prenatal surgery is done, there is a definite reduction in the need for shunting. things that has been recognized but as yet not all centers are capable or offer this at the early enough time to make it effective. Children with cervical level spina bifida often do not survive. The higher the level, the more likely they will have shunting as well as cognitive and learning disabilities and even if an incomplete picture is present, they will have limited control of their extremities and often are ventilatory dependent. Treatment for these children is similar to that of a traumatic spinal cord injury. Those with a thoracic level spina bifida are very similar to traumatic spinal cord injury paraplegics although often they have incomplete sensation and incomplete and asymmetric motor control. Hyphosis and scoliosis are common as are foot deformities and most of these patients are only ambulatory for short distances for exercise or in the home. They must use KAFOs to stand and these can be cumbersome to get on as well as heavy. Scoliosis needs to be addressed early on and there are now growing rods as well as a variety of different scoliosis procedures that can allow early surgery but continued axial growth. Lumbar lever patients with spina bifida have strong hip flexors and trunks but still are not able to ambulate in a community setting. They often have scoliosis as well and often early hip dislocation. Hip dislocation may not be a problem if it is symmetric and bilateral and they have no sensation but if it is asymmetric it can cause gait and seating difficulties which can lead to skin problems. Generally these patients are full time wheelchair users. A lower lumbar level of L4 will have weak ankle dorsiflexion and need lower extremity bracing for mobility and they can typically walk around their classroom at school, get around their homes but would not go community distances without a wheelchair. They also have hip dislocation, scoliosis and calcaneal foot deformities. One of the main difficulties that one might see as they age is skin integrity especially over the surgical site or the gibbous deformity that has formed over the years. This needs to be addressed through appropriate seating including positioning in bed, in the car or in their wheelchair. The lumbar level L5 is going to have a patient who is independent in community ambulation. They do have some asymmetry side to side and often have a loping gait with calcaneal feet. However, they are able to get around although they may use crutches. The sacral level of spina bifida may be independently ambulating for long distances but still have neurogenic bowel and bladder and skin integrity issues. Sensory deficits and neurogenic bowel and bladder are present at all levels of injury and can be very problematic if the patient is not on surveillance for the sensory issues. Bladder surveillance is important because of the risk of hydronephrosis as well as bladder cancer. Bladder augmentation can increase the risk of cancer but decrease the risk of hydronephrosis. Thus these patients have a cytoscopy every one to two years typically and will have renal ultrasounds on an annual basis. Cataracts are also done fairly frequently as children and may be repeated as adults. Clean catheterization versus sterile catheterization is typical and most patients will need to do this for life. The metrophin off continent catheterizable stoma placed on the abdomen allows women in particular to have easier access for catheterization without undressing and without moving out of a wheelchair. For bowel control, the Malone antigrade continent enema procedure is an antigrade enema where fluid is flushed into the colon and the patient sits until the colon is emptied. This catheterizable stoma can be done surgically typically by a urologist and can be life-changing for patients that have had constant constipation. Arnolkiari malformations are common and syrinx either in the thoracic or lower lumbar region are also common. Shunting is fairly global at this point because patients that have had prenatal surgery are still not the majority of patients. You should anticipate that adults with spina bifida will have shunts and these do need to be checked to assure that malfunction has not occurred. Tethering in the spinal cord is another common issue often seen during growth spurts but can occur as adults as well. There might be an increasing scoliosis, asymmetrical sensory loss, changes in bowel or bladder function or changes in gait or pain. An MRI can be diagnostic but is not always diagnostic and surgical decisions are typically based on symptoms. Latex allergy has been known since about the 1980s when it became clear that patients with spina bifida had a huge rate of sensitivity, much higher than any other children despite the fact that they had the same number of surgeries. Latex can cross-react with a variety of fruits that come from trees related to the latex tree and thus bananas, avocados and kiwis are common foods that children or adults with spina bifida may avoid. Having an EpiPen and a medical alert bracelet is crucial. Secondary conditions associated with spina bifida include obesity and short stature, osteoporosis due to limited weight bearing, overuse and underuse of joints and nonverbal learning disabilities which impact vocational choices. Nonverbal learning disabilities are typically diagnosed and addressed in the school system as patients progress but neuropsychology testing should be repeated in high school and may inform on vocational decisions. Medications for attention may be very beneficial and these would need to be continued in adult patients. Vocational and career planning should begin by the age of 16 years in the school system but many young adults stay in the system until they are 21 and at that time if those issues have not started to be addressed a referral for vocational rehabilitation should be made. Employability often relates to the ability to manage one's bowel or bladder independently but education is the best predictor of vocational achievement. Sexuality issues are often neglected in any adult with spina bifida or cerebral palsy and the majority of this is because of discomfort on potentially the part of pediatric practitioners addressing it and the assumption by adult practitioners that none of these patients are sexually active. However, advice regarding STDs, contraception, making sure that the patient has had an HPV vaccine if their primary care physician did not mention it and assistance with other sexual issues should be addressed and the PM&R physician is perhaps going to be the one that has the relationship with the adult that can address these most comfortably. What's with Down syndrome? Down syndrome patients are living longer than ever before and to the 6th and 7th decade of life. They also have an increased risk of multiple medical issues similar to those with spina bifida and CP. Hypothyroidism, however, is one to really not miss as up to 40% of these patients have this and it can masquerade as depression and obesity or conduct disorder and be assumed to be related to their primary diagnosis. Sleep apnea is also very common and needs to be addressed aggressively. Cataracts and glaucoma as well as hearing loss can cause behavioral or mobility changes that the PM&R physician is easily able to address and recognize. Cardiac issues are also of concern and patients need to be screened for this and referred to cardiology. Unfortunately adults with Down syndrome are also at a higher risk for cancer, specifically for leukemias and some solid tumors. These issues also need to be discussed and proactively addressed. Things have changed in the world of atlanteo-axal instability. As previously, children all had cervical spine films and there was a great deal of concern about laxity. Today the AAP no longer recommends routine spine films and suggests they only be done if someone is involved with high-risk activities or if they are asymptomatic. However, if a patient with Down syndrome is being anesthetized, care needs to be taken with neck manipulation. Cervical spine pain can also be an issue and the adult physiatrist is perfectly suited to do interventional pain management. Other musculoskeletal conditions that are common and can cause pain include late hip dysplasia or degenerative joint disease, knee pain, foot pain, scoliosis pain from fusion or from hypotonia, inflammatory arthritis and osteoporosis. Cognitively patients with Down syndrome have intellectual disabilities. Their academic performance is low which means that their competitive employment rates are going to be low as well. However, the impact of shunts which we may no longer be seeing in the future can significantly alter this picture and we should not underestimate the need for or the benefit of neuropsychology testing. Patients with Down syndrome, as mentioned, can be sexually active but they rarely reproduce and they do have a higher risk of having a child with Down syndrome so this should be part of the counseling as well. Childhood functioning does predict adult function in Down syndrome. Many families are overprotective but many more children are being placed in public schools where transitional programs are setting higher expectations for them than in the past. The need to address long-term care for these patients as their parents age, however, is something that is important to start having conversations about in young adulthood so that there are no abrupt transitions later on. Depression and hearing issues need to be checked as hearing loss is common, as mentioned, are cataracts. Mental health issues and autism can accompany Down syndrome but again need to be evaluated to ensure that they are not related to pain, hypothyroidism, etc. Depressive disorder and depression are particularly common as well as early onset dementia. Down syndrome population has a higher incidence of this with an onset at an earlier age. Medications can be helpful if started early and this should precipitate an early referral if the patient is not seeing a neurologist or a geriatric specialist already. Sensory deprivation from social changes, living changes, and not being with their familiar parents, having hearing or vision loss are a major concern as well. Thus, in lifetime care the adult physiatrist really is the most able to care for the functional needs of the adult that has a childhood onset condition. Questions In a diplegic patient with CP who is complaining of back pain, what would you do? C. Mobility issues impact biomechanics so do not assume that scoliosis is the cause of pain or that an assistive device will help. 2. A 45-year-old with L2 spina bifida has been acting aggressive and irritable and her group home providers want you to give her something to help her relax. What would you consider as alternative options? E. All of these issues can cause behavioral problems. 3. A 55-year-old Down syndrome patient has stopped interacting with her family and just wants to sit in her room. What would you do? All of these may be treatment recommendations, but the most important would be to screen for thyroid disorder first.

adult physiatrists

pediatric patients with disabilities

aging disabilities

primary disability

proactive care

specific medical issues