Factors that contribute to secondary musculoskeletal impairments and pain include:

• Physical growth
• Inability for muscles to lengthen in proportion to bone growth
• Excessive biomechanical forces through joints
• Overuse syndromes
• Immobility
• Fatigue
• Obesity
• Stress and depression
• Early joint degeneration

Transition From Secondary Education: Although the Individual Education Act mandates transition planning by age 14 for students in special education, many young adults have few options for post-secondary education or employment.

Challenges of Adults with Cerebral Palsy

• Secondary Impairments
• Possible deduction in motor impairments
• Limited specialized health care
• Access to assistive technology
• Restricted social and leisure pursuits
• Low employment rate
• Often dependent on family for living arrangements
• Fulfillment of social roles more restricted the more severe functional limitations in mobility and self-care
• Parents of adult children with severe disabilities are faced with lifelong responsibilities for care.

Health Related Quality of Life: Understanding of health related quality of life is limited by the lack of information on the personal values and goals of adolescents and adults with cerebral palsy. There are multiple research methods:

• Qualitative
• Participatory
• Predictive
• Experimental

Research Priorities

• Understand perceived health-related quality of life issues of adolescents and adults with cerebral palsy and their families including social participation.
• Evaluate the consequences of secondary impairment, environmental factors, and personal factors on changes in communication, self-care, and mobility of adults and adolescents with cerebral palsy.
• Determine optimal methods for improving physical fitness and prevention of secondary health conditions in adolescents and adults with cerebral palsy.
• Identify the determinants for successful use of technology for adapted function including availability, training, personal and environmental factors
• Determine the resources and supports associated with successful transition from secondary education to post-secondary education, employment and independent living.

There are three kinds:

• Upper Brachial Plexus (Erb’s palsy): The upper part of the nerve plexus is damaged. Along with total brachial plexus palsy, this is the most common form of the disorder. These infants cannot move the shoulder and keep their arms extended and turned inward, giving the appearance of the “waiter's tip hand.”
• Total or Global Brachial Plexus Palsy: The entire brachial plexus is affected. Infants with total brachial plexus injury have no movement of the arm, wrist, or hand; the arm is limp. This type of injury is more often associated with nerve roots being detached from the spinal cord, which is more difficult to treat.
• Lower Brachial Plexus Palsy: Isolated lower plexus injury is exceedingly rare. Typically the lower brachial plexus is involved as part of a more extensive injury, such as total brachial plexus injury.
• Bilateral Injuries: The brachial plexus can be injured on both sides of the body, although such cases are rare. Bilateral injuries can be mistaken for other problems, such as spinal cord injury.

Natural History: Approximately 80-90% of children will spontaneously recover within two months. The recovery is attributed to a stretch across the plexus and these children will have normal upper extremity function, recovery via nerve regeneration. This yields a useable extremity with impairment on limb length, shoulder motion and strength.

Changes from infancy to adulthood:

• Infant: Diminished workspace and delayed ability to achieve developmental milestones with both hands
• Adolescent: Difficulties with overhead reaching and certain activities of daily living; such as dressing, washing one’s hair and reaching behind his or her back.
• Adult: Accepted limited motion; made certain occupational and recreational adjustments; and developmental of arthritis and contralateral limb pain more conjecture than science.

Research priorities:

• Inform science and policy of different impairments and degree of injury
• Understand the natural history of BPBP
• Inform providers and policy makers about early interventions
• Study ability of the impaired infant to obtain development milestones after BPBP.
• Conduct research regarding the infant’s and child’s actual and perceived disability after BPBP.

Incidence

• Generally there are between 7,600 and 10,000 new cases of Spinal Cord Injuries (SCI) per year
• There are between 1,520 and 2,000 cases in children and adolescents nationally

Ways SCI impact Children’s lives and how it persists throughout their lives

• Impairment
• Function
• Less community participation
• Environmental issues
• Life Satisfaction

Life Expectancy:

If a child has no SCI and is 10, life expectancy is 67 years, if he is 15, life expectancy is 62 years, and if he is 20, life expectancy is 57 years.

If a child has a SCI but is not affected above the injury and is 10, life expectancy is 62 years, if he is 15, life expectancy is 57 years, and if he is 20, life expectancy is 53 years.

If a child has paraplegia and is 10, life expectancy is 56 years, if he is 15, life expectancy is 51 years, and if he is 20, life expectancy is 46 years

If a child has a SCI and the injury is between C5-C8 and is 10, life expectancy is years 50, if he is 15, life expectancy is 46 years, and if he is 20, life expectancy is 41 years.

If a child has a SCI and the injury is between C1-C4 and is 10, life expectancy is 46 years, if he is 15, life expectancy is 41 years and if he is 20, life expectancy is 37 years

If a child has a SCI and the injury that requires he ventilator and is 10, life expectancy is 35 years, if he is 15, life expectancy is 30 years and if he is 20, life expectancy is 27 years.

Children with a SCI may be at increased risk of complications associated with aging, such as hypertension, obesity, and metabolic syndrome, because of their relativity long life span and their sedentary life style.

Differences between pediatric-onset and adult onset SCI

• Children with SCI have a long life span and increased duration of exposure that increases frequency and severity of long-term consequences of SCI-related complications; such as pressure ulcer and urological complications.
• Scoliosis and hip dislocation are unique complications of pediatric-onset SCI.
• Pre-injury experiences, such as employment or illness differ in children; therefore adult outcomes in participation, quality of life and health may be unique in the pediatric SCI population.

Prospective Study of Adult Outcomes of Pediatric-onset SCI

• 265 subjects- sustained a SCI when they were 18 or younger, with a mean age of 13 and half years; follow them up when they were 24 or older, with a mean age of 29.9 years, range 24-40
• Standardized Measures included:
- Functional Independence Measures
- Craig Handicap Assessment and Reporting Technique- participation
- Short Form-12, health-related quality of life
- Satisfaction with life scale

• Mortality: Of the eligible subjects, 4% were dead and 20% were lost to follow-up. The mortality implications establish a survival curve, identify causes of death, delineate predictive factors of death and design strategies to prevent death.
• Medical complications commonly occurring in adults with pediatric-onset SCI:
- Urinary tract infection (UTI) 74%
- Pain 69%
- Spasticity 57%
- Autonomic dysreflexia 54%
- Pressure ulcers 44%
- Respiratory complication 33%
- Chronic Medical Conditions 20%
- Pressure ulcers and severe UTI affects employment, independent living and life satisfaction. Spastiscity affects employment and independent living. Pain affects employment and life satisfaction.

Medical Complications implications

• Develop interventions that prevent complications associated with significant outcomes
• Do these interventions improve adult outcomes? Is participation and life satisfaction improved by preventing pressure ulcer sores?
• Longer-term follow-up is needed to more clearly delineate the implication of SCI related complications such as pressure ulcers and shoulder pain and general health problems such as metabolic syndrome.
• Participation is significantly associated with a variety of factors
• Do interventions that impact significant predictive factor, such as education, community mobility, functional independence and medical complications, improve participation?
• Life Satisfaction is significantly associated with community mobility, marriage, use of street drugs and health status. Do interventions impact these factors and improve life satisfaction?

• Occurs in 2% of all people
• Most starts in childhood
• 70% is well controlled with medication
• 20-30% of children continue to have difficult-to-control seizures. Many of theses children have multiple disabilities including cerebral palsy, mental retardation, and behavior and learning disabilities
• Children with epilepsy alone need new and better treatment so they can lead normal lives.
• Children who have epilepsy along with multiple disabilities are similar to children with multiple disabilities without epilepsy. All these children need day care, schooling and job training.
• Adult with epilepsy are further “disabled” by the lack of transportation, and myths and misunderstandings about epilepsy and its effects on intellect, function and job performance.
• Research in epilepsy could fill in some of the gaps in knowledge, and inform science and policy makers.

Research Priorities

• Research into the meaning of threshold and the mechanism of epileptogenesis could prevent much of the disability of epilepsy.
• Develop medications, which are effective against the pediatric epilepsies, and evaluate the effects of these drugs on the developing nervous system.
• Develop strategies to avoid the psycho-social disabilities of epilepsy
• Research on the patho-physiology of infantile spasms and develop therapies that could prevent epilepsies and allow the development of more effective therapies.

The Prevention of Childhood Epilepsy:

• Why does epilepsy begin in childhood?
• Threshold for seizures increase with age; research into the meaning of threshold and the mechanisms of epileptogenesis could prevent much of the disability of epilepsy.
• What are the components of threshold?
• Can they be identified genetically?
• Can they be modified to prevent epilepsy?
• Can newer imaging detect small foci?
• Will early detection lead to early surgery and better outcomes?

Childhood Epilepsy is different than adult epilepsy. Reasons why adult epilepsy is mainly studied:

• Temporal Lobe Epilepsy (TLE) is commonly studied and is used to evaluate new medications
• TLE is rare in children
• Most epilepsy in children is cortical or sub-cortical
• Pediatric epilepsies need to be studied and medications need to be developed which are effective against them
• These medications need to be evaluated for their effects on the developing nervous system

Can the psycho-social disabilities associated with epilepsy be avoided?

• Children with epilepsy have a higher incidence of behavior and learning disorders and school failure
• Adolescents with epilepsy fail to attend and drop out of school at higher rates than others
• Adults with epilepsy have depression, unemployment and decreased of quality of life

Ways to Avoid these Problems:

• Project All Children Together (ACT): teaches child care workers to deal with seizures and other problems that lead to a child’s dismissal.
• VEPAE (Maryland’s program): documents if adolescents are provided with ob-skills training and work experience, they were:
  -More likely to attend school
  -Less likely to drop out
  -More likely than peers without epilepsy to be in school or in jobs-2 years later
  -Early interventions could prevent many problems
  -Psychosocial research could validate these and other programs

Studies of Infantile Spasms and the Lennox-Gastaut Syndrome (L-G-S)

• Infantile spasms and L-G-S are age-specific
• They are common causes of both
• Understanding the patho-physiology of these catastrophic epilepsies could lead to better treatments could lead to better treatments, and perhaps to prevention of mental retardation.

Mechanisms of Action of the Ketogenic diet: Understanding its mechanism of action will give insights into the pathogenesis of pediatric epilepsies and allow the development of more effective therapies.

• This high-fat, adequate protein, low carbohydrate diet is more effective in controlling difficult-to-control seizures than any medication
• It is well tolerated –when effective
• It can be discontinued after two years- without seizure recurrence
• The reasons why the diet works is unknown.

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