In November 1994, a newsletter called “Brand News” published Emily Perl Kingsley’s description of what it’s like to be the parent of a child with a disability. “Welcome to Holland” compared pregnancy to planning a trip to Italy — and giving birth to a child with a disability to unexpectedly arriving in Holland.
Kingsley asserted that after getting comfortable with the challenges of raising a disabled child, “you begin to notice that Holland has windmills … and Holland has tulips. Holland even has Rembrandts.”
Just like the numerous interlaced highways of the world, cerebral palsy is a complex condition that is as individual as the child who possesses it. But armed with the right information and the proper mobility devices, a child with CP can travel the world. In fact, mobility devices “may mean the difference between a life of relying completely on others, to going off to college and living in a dorm,” says Tim Caruso, a senior physical therapist at Chicago’s Shriners Hospital for Children and a private practitioner and president of Chicagoland Performance Consultants.
Most importantly, mobility devices may help a child with cerebral palsy express his or her own inner potential and beauty.
Getting to Know the Place
Some 764,000 children and adults in the United States are estimated by United Cerebal Palsy to manifest one or more of the symptoms of cerebral palsy. Currently, about 8,000 babies and infants are diagnosed with the condition each year. In addition, some 1,200-1,500 preschool-age children are recognized to have CP each year. UCP estimates that between one and three of every 1,000 births will have CP.
In fact, CP is the most common cause of childhood disability in the United States and possibly the world, said Brett Nirider, an experienced pediatric therapist at Good Samaritan Community Healthcare in Duyallup, Wash.
Clinicians define cerebral palsy as a permanent brain injury that causes muscle weakness and poor control. While the damaged area of the brain does not progress, in time, change is possible for the secondary conditions of CP, such as muscle spasticity (increased muscle tone). These symptoms can become better or worse, or remain the same. Cerebral palsy is not considered a disease, but rather a non-communicable condition. Although CP is not “curable,” commitment to training and therapy can help improve function.
Despite a unifying definition, individuals with CP manifest a variety of symptoms depending on the specific area of the brain injury. As a result, this particular condition can be quite confusing and difficult to identify. Muscle tightness or spasm, involuntary movement, and disturbance in gait and mobility are common effects. The severest symptoms of cerebral palsy will prevent a person from walking and require lifelong care, while a milder case might only make walking awkward.
Abnormal sensations and perceptions; sight, hearing or speech impairment; seizures and tremors may also accompany CP. Related problems may include difficulties in feeding, loss of bladder and bowel control, GI problems, difficulty breathing due to posture, skin disorders from pressure sores and growth problems. Convulsive seizures occur in about 25 percent of patients, most often in those with spasticity, according to Children’s Hemiplegia and Stroke Association (CHASA). Short attention span and hyperactivity are also common.
Cognitive manifestations of CP include developmental disabilities, such as learning disabilities; however, developmental disabilities are the exception, not the rule. Approximately 50 percent of those with spastic CP and 35-40 percent of the total CP population are developmentally disabled, according to Cerebral Palsy Information Central. Learning disabilities may range from trouble with a few subjects and learning other things quickly to more severe learning disabilities in which everything is learned at a slower pace.
“Many kids with CP are bright, but have difficulty in learning because of deficits in visual-perceptual skills, auditory processing or body awareness that affect their ability to learn,” said Nirider.
CP is usually diagnosed within the first 18 months of life, as children with CP are slow to reach development milestones such as sitting up, crawling, walking or smiling, said clinicians. Brain injury leading to CP ususally results during early stages of development, either in fetal, perinatal or early childhood. The extent and location of brain injury determine the severity of impairment.
Fetal brain injuries are the result of anoxia (a problem with the umbilical cord); maternal infection, such as rubella, toxoplasmosis (an infection caused by parasites that produces lesions on the fetus’ central nervous system) and herpes simplex; metabolic disorders such as diabetes, a heart condition, hyperthyroidism and severe asthma; RH factor; abdominal injury; or absence or lack of prenatal care.
In the perinatal phase, CP can manifest from anoxia; asphyxia; analgesics (administering of drugs that affect the respiratory system); trauma, specifically to the head during labor and delivery, such as hemorrhage, forceps application, poor position of the infant or breech delivery; pressure changes in delivery, either too fast or too slow; or complications with premature birth.
Early childhood CP can be caused by trauma to the head; infections, such as meningitis, encephalitis and high fevers; vascular problems; anoxia due to strangulation, carbon monoxide poisoning, smoke inhalation or near drowning; or neoplasms of the brain including cysts, tumors and hydrocephalus (caused by fluid in the brain).
Although CP is not a terminal condition, it is commonly believed that the mortality rate of those diagnosed with CP is somewhat higher than that of the general population, though few studies exist to substantiate that theory. Poor nutrition caused by the muscle problems that make eating difficult contributes to the likelihood of infection, which decreases the chance of survival. According to a 2002 Danish study, those with mild CP had near-normal lifespans, especially those with walking abilities, while those with more severe forms, such as spastic quadriplegia or a side condition of epilepsy, had shortened lifespans compared to the general population. Community Health Care, Inc., based in Wisconsin, estimates that more than 90 percent of infants with CP survive into adulthood.
“Theoretically (those with CP) can have the same life expectancy as everyone else,” said Julianna Arva, OT and pediatric product specialist at Permobil. “Yet you don’t see many old people with CP, at least not very involved ones. The main reason is that society did not know how to care for them for the longest time. Most lived in nursing homes in not very good situations … sitting in bad chairs, lying in bad beds. Once you are in a chair and incorrectly seated and without appropriate therapy, you can develop many secondary complications — urinary tract infections, pressure sores, kyphotic postures — that place a large amount of pressure on your GI organs. It is these secondary complications that end up killing people with CP before it’s time. This, however, is getting better and better, so with improved care they can live a long and full life.”
“Many of our more severely involved kids have respiratory, gastrointestinal, cardiac and visual problems,” Nirider agreed. “In those cases, life expectancy can be compromised, largely due to seizures, or respiratory infections. Many of the kids will have typical life spans.”
Because the brain injury location is not the same for each person affected, it is impossible to provide a one-label-fits-all diagnosis. Therefore, the types of CP vary greatly between individuals.
Nirider explained that it is important to remember that CP is classified by both attributes as well as distribution.
“In other words, some attributes might be spastic, athetoid (or dyskinetic), hypotonic, ataxic, or mixed. These are words that describe the attributes of the child’s movements,” Nirider said. “There are also classifications of distribution, or what part(s) of the child’s body is (are) affected. So you may see words such as diplegia (involving legs more than trunk and arms), hemiplegia (involving one side of the body much more significantly than the other), and quadriplegia (involving arms, legs, trunk on both sides of the body). So, it is possible to identify a child, for instance, who has spastic diplegia, or spastic athetoid quadriplegia with a hypotonic trunk, or some other combination of attributes and distribution.”
Many organizations including UCP and the Centers for Disease Control and Prevention group CP by four main types — spastic, athetoid (or dyskinetic), ataxic and a catch-all category of mixed types — and subdivide these types by limbs affected, such as hemiplegic or quadrapledic.
Spastic Cerebral Palsy
In the 1860s, William Little, an English surgeon, wrote the first medical descriptions of cerebral palsy, specifically spastic diplegia. He observed children in the first years of life who had stiff, spastic muscles in their legs and arms. These children had difficulty grasping objects, crawling and walking, and did not get better or worse with age. The condition was known as Little’s disease for many years.
Spastic cerebral palsy is the most common form of CP, accounting for about 70 percent of all CP cases, states CHASA. Clients with spastic cerebral palsy experience increased muscle tone, which causes stiff muscles and makes moving difficult and awkward. Upper motor neuron involvement is the cause of spasticity, which may affect motor function to a varying degree.
Wheelchairs (manual and power), strollers, adaptive sitters, standing frames, trikes, bikes, adaptive electric riding toys, walkers, canes, poles, orthotics (feet, knees, hands, wrists, elbows, trunk supports) are just a few of the adaptive equipment children with CP use. The need for adaptive equipment may change as the person develops, or as new treatments are introduced.
“Independence is the main goal,” said Arva. “So the least amount of technology that provides efficient mobility