Cerebral Palsy is a collection of motor disorders resulting from damage to the brain that can occur before, during and after birth. Congenital cerebral palsy indicates that a person developed cerebral palsy at birth which is the case of the majority of people with cerebral palsy. According to the Centers for Disease Control and Prevention (CDC), Cerebral Palsy (CP) is a group of disorders that affect a person’s ability to move and maintain balance and posture. It is the most common motor disability in childhood. It is estimated that an average of 1 in 345 children in the U.S. have cerebral palsy. For many years, it was thought cerebral palsy was due to lack of oxygen. Studies show this only accounts for 19% of all cases.
Prevalence and Characteristics
Around 764,000 people in the United states have at least one symptom of cerebral palsy
Around 10,000 babies are born each year with cerebral palsy
Boys are diagnosed more often than girls
Cerebral palsy is the mot commonly diagnosed childhood motor disability in the United States
Over 77% of children with cerebral palsy have the spastic form
More than 50% of all children with cerebral palsy can walk independently
African American children with cerebral palsy are 1.7 times more likely to need assistance with walking or be unable to walk at all
Around 41% of babies and children with cerebral palsy will have limited abilities in crawling, walking and running.
Around 41% children with cerebral palsy in the United states have some form of a cognitive disorder
Behavior problems are common in children with cerebral palsy including social skills and anger issues.
Seizures are a common associate disorder of cerebral palsy and can range from mild to extreme severe.
There is no known cure
What Causes Cerebral Palsy?
Studies show that about 10 to 20 percent of children with cerebral palsy acquire the disorder after birth. This includes through infections, jaundice, RH incompatibility and severe oxygen shortage in the brain.
Types of Cerebral Palsy
Ataxic- indicates the muscle tone is too low or too loose
affects 5 to 10 percent of people with cerebral palsy
movements are unsteady and shaking
have difficulty making quick movements
Spastic- refers to the inability of muscle to relax
is the most common type of cerebral palsy
70-80% of people have spastic cerebral palsy
will have difficulty moving from one position to another
Athetoid-uncontrolled twisting movements
Affects 10 to 20% of people with cerebral palsy
often have difficulty holding themselves in an upright position
muscles move involuntarily causing limbs to twitch
According to the Centers for Disease Control and Prevention (CDC), approximately 6.8% of children younger than 18 years in the United States have a diagnosed eye and vision condition and 3% of children younger than 18 years are blind and visually impaired. Visual disability is one of the most prevalent disabilities disabilities among children.
According to IDEA’s definition, visual impairment is defined s including blindness means an impairment in vision that even with correction, adversely affects a child’s educational performance. The World Health Organization (WHO), classifies visual impairment as occurring when an eye condition affects the visual system and one or more of its vision includes both partial sight and blindness
Classifications
The World Health Organization uses the following classification based on visual acuity in the better eye:
20/30 to 20/60- mild vision impairment
20/70 to 20/160- moderate visual impairment
20/200 to 20/400- severe visual impairment
20/500 to 20/1,000- profound visual impairment
More than 20/1,000- considered near-total visual impairment
No light perception- considered total visual impairment or total blindness
Types of Visual Impairment
Strabismus– a condition when the eyes do not align with each other (crossed eyes)
Congenital cataracts– a clouding of the eyes natural lens present a birth.
Retinopathy of prematurity– a blinding disorder that affects prenatal infants that are born before 31 week of gestation.
Coloboma- a condition where normal tissue in or around the eye is missing at birth.
Cortical visual impairment– a visual impairment that occurs due to brain injury.
Signs of Visual Impairments
Appears “clumsy” in new situation
Shows signs of fatigue or inattentiveness
Does not pay attention when information is on the chalkboard or reading material
Is unable to see distant things clearly
Squints
Eyes may appear crossed
Complains of dizziness.
Causes
The causes of childhood blindness or visual impairment is often caused by Vitamin A deficiency which is the leading cause of preventable blindness in children. Other causes include genetics, diabetes, injury and infections such as congenital rubella syndrome and chickenpox before birth.
Cortical Visual Impairment (CVI)
Cortical Visual Impairment in children is attributed to brain dysfunction rather than issues with the eyes. Causes included hypoxia, traumatic brain injury, neonatal hypoglycemia, infections and cardiac arrest.
Prader Will Syndrome is a genetic disorder resulting from an abnormality of chromosome 15 such as a loss of active genes. In most cases (70%) the paternal copy is missing and in some cases (25%), will exhibit two maternal copies of Chromosome 15. The genetic disorder was initially described by John Langdon Down and was named after Drs. Andrea Prader, Heimrich Willi and Alexis Labhart in 1956 and is found in 1 in 20,000 births affecting both sexes. It is also the most common recognized genetic form of obesity.
During childhood, individuals diagnosed with Prader-Willi Syndrome tend to eat constantly leading to obesity and for some, type 2 diabetes will develop. This complex disorder affects appetite,growth, metabolism, cognitive functioning and behavior.
Signs and Symptoms
People with Prader-Willi Syndrome (PWS) tend to never feel full (hyperphagia) which leads to constant eating. Signs in infants include, problems with strength, coordination and balance. Often there are feeding problems at birth, delayed speech and gross motor development. Children may be born with almond-shaped eyes and undeveloped sexual organs. Cognitive disabilities and developmental delays may also be present.
As children began to grow, constant craving for food often leads to behavior challenges including hoarding food, eating frozen food and food left in the garbage causing controlling or manipulative behavior.
Medical Issues
Medical concerns may include the following:
Sleep Apnea
Respiratory/Breathing
High pain tolerance
Severe stomach illness
Difficulty with vomiting reflex
Excessive appetite
Binge eating
Eye problems
Choking
Hypothermia
Leg Swelling
Consuming unsafe items
Negative reactions to medications
Teaching Strategies
Most people diagnosed with Prader Willi Syndrome fall between the moderate and mild levels of an intellectual disability meaning there may be challenges in the area of reasoning, problem-solving, planning, judgment, abstract thinking and learning. A child or student functioning at the moderate level may lag behind their peers in the area of language and pre-academic skills. Adults may function at an elementary school level and will require support in both work and daily living skills. For children and students functioning at the mild level, there may be difficulties in the area of reading, writing, math and money management. as children grow into adults, there may be a need for support in abstract thinking, executive functioning (planning, prioritizing and flexibility) as well as short-term memory and money management. Teaching strategies should focus on the following:
Aggression management
Anger management skills
Anxiety management
Emotional regulation
Personal safety
Social skills
Keep in mind that many children and adults diagnosed with Prader-Willi Syndrome may have additional challenges in learning due to medication. Some people take medication such as a growth hormone therapy which can cause fatigue. The following teaching strategies may also be useful when teaching a student diagnosed with Prader Willi Syndrome:
Use a multi-sensory approach. This involves a teaching style that includes auditory, visual, tactile, spatial, and kinesthetic (hands on activities)
Break learning into small steps. Check for understanding by asking the student to repeat back to you.
Teach a skill at least 2-3 times a day. This will help the student retain information.
Managing perseveration. Set up a rule where the student can a question no more than 3 times. After the third answer. Ask the student to repeat the response back to you.
Adult Day Program/Residential Setting
Most people with Prader Willi Syndrome due to their cognitive level, will be provided services in either a day habilitation program or live in a community providing residential services. Once a person becomes an adult, it becomes a little bit more tricky on maintaining issues especially behavioral. For instance, while living at home, a parent has the right to lock the refrigerator which is often suggested by experts. However, this becomes a violations of a person’s rights once they reach adulthood. Typically, committees meet to help make the right decisions along with family members and the adult diagnosed with Prader Willi Syndrome. Here are some suggestions.
Allow the person to have control of what is important to them. Have discussions on nutrition and staying healthy. Check to see if this may be an appropriate topic the person may want to improve by adding to their person-centered plan. Hold discussion groups in both day programs as well as in residential to discuss various topics on health and nutrition including holding classes on mindfulness and meditation.
Trips to shopping malls can be very tricky. Try to avoid mall’s eatery and plan if it is a group trip to have people bring their own lunches.
When teaching, allow time before giving additional prompts
Give praise as much as you can when it is appropriate.
Use visuals as much as you can including graphics and pictures.
Staff Training
Staff training on Prader-Willi Syndrome should include the following topics:
Overview of Prader-Willi Syndrome including, causes, symptoms, characteristics, nutrition, and self-regulation.
Impact on the family including the stresses families experience.
Teaching techniques including problem-solving, forward shaping and role-modeling.
March is Developmental Disabilities Awareness month! Although I blogged the definition of developmental disabilities here, I wanted to give you more information besides the Federal regulation. Quite often, people are confused between the definition of an intellectual disability and a developmental disability.
A developmental disability is described as an assortment of chronic conditions that are due to mental or physical impairments or both. For example, you may have a child or an adult with an intellectual disability or perhaps a person diagnosed with cerebral palsy and an intellectual disability. It is also considered a severe and chronic disability that can occur up to the age of 22, hence the word developmental. A developmental disability can occur before birth such as genetic disorders (i.e. cri du chat, fragile x syndrome,) or chromosomes ( i.e. Down syndrome, Edwards syndrome); during birth (lack of oxygen) or after birth up to the age of 22 (i.e. head injuries, child abuse or accidents).
The disability is likely to occur indefinitely meaning the person will require some type of ongoing service throughout their lives. Finally, the person must show limitations in 3 or more of the following areas of major life activities:
Self-care– brushing teeth, hand-washing and combing hair independently
Receptive and expressive language-ability to understand someone talking and to also be understood
Learning– ability to read and write with understanding
Mobilityability to move around without any assistance
Self-direction– time management, organization
Capacity for independent living– requiring no supervision
Economic self-sufficiency – having a job and purchasing what one needs
Here are some examples of a developmental disability:
Does everyone with a disability also have a developmental disability?
The answer is no. there are people with disabilities such as epilepsy and cerebral palsy simply have a disability based on the criteria listed above. However, many people with developmental disabilities quite often have a combination of disabilities. For example a child with autism may also have seizures and an intellectual disability or an adult may have cerebral palsy, intellectual disability and epilepsy. In addition there are many people in the spectrum of autism who also have ADHD and so forth.
So what’s the difference between an intellectual disability and a developmental disability?
A person with an intellectual disability falls under the category of a developmental disability meaning you can have an intellectual disability and a developmental disability. check here for the definition of an intellectual disability, you will see they are quite similar. Below is an infographic created by Centers on Disease Control:
February 13th is the first day of Duchenne Muscular Dystrophy Awareness Week. Here are some facts on Duchenne Muscular Dystrophy:
It is one of the nine types of muscular dystrophies
Duchenne muscular dystrophy was first described by French neurologist, Guillaune Benjamin Amand Duchenne in the 1860’s.
It is an inherited disorder
It is caused by an absence of dystrophin, a protein that bonds the muscle cell
It is characterized by progressive muscle degeneration
It occurs in about 1 out of every 3,600 male infants
Risks include a family history of Duchenne muscular dystrophy
Symptoms start appearing between the ages of 3-5.
By the age of 12, most males affected may lose their ability to walk
Breathing difficulties and heart disease usually start by the age of 20
Very rare are females affected by the disease.
Early symptoms include muscle weakness in the hips, pelvic area, thighs and shoulders.
By teen years, the heart and respiratory muscles are affected.
Duchenne muscular dystrophy carriers are females with one normal dystrophin gene on one x chromosome and an abnormal dystrophin gene on the other x chromosome
Most carriers do not show any signs or symptoms.
Affected children may have delayed motor skills including sitting, standing and walking.
Survival into the early 30’s is becoming more common due to advances in cardia and respiratory care.
Duchenne is associated with a heart disease that weakens the cardiac muscle
Between 400 and 600 boys in the United States are born with these conditions each year.
there are a few cases which results from new mutations in affected males
steroid drugs can slow the loss of muscular strength
There is no known cure for Duchenne muscular dystrophy
