Severe Physical Handicaps By Andrea Opel Sondra Deurloo

Severe Physical Handicaps By Andrea Opel, Sondra Deurloo, Caitlin Robles, and Danielle Harrington

Definition and Eligibility

IDEA Definition/Eligibility • Americans with Disabilities Act (ADA): “a person is considered physically handicapped if he or she has an impairment that substantially limits one or more of life’s daily activities. ” • IDEA 2004: ▫ A person with an orthopedic impairment, brain injury, or other health impairment who, by reason of that impairment, needs special education and related services is considered to have a physical disability. ▫ The condition must interfere with or substantially limit the child’s ability to take part in routine school activities. ▫ A physical disability or health condition need not limit activity; instead, it may involve other restrictions, such as a special diet (i. e. celiac-disease) or the student’s need to use medical equipment.

Special Education Eligibility (cont. ) • When physical disabilities adversely affect academic performance, the following special education eligibility categories may be considered: ▫ ▫ Orthopedic Impairment Other Health Impairment Multiple Disabilities Traumatic Brain Injury

Anatomy of Severe Physical Handicaps

The Nervous System A network of specialized cells (neurons) that coordinate the actions of an animal and transmit signals between different parts of the body Facts to know: ØPeripheral nervous system, the Øsympathetic nervous system : “fight or flight” Øparasympathetic nervous system : “rest and digest” ØVentricles: open spaces in the brain which are filled with Cerebrospinal Fluid (CSF) ØThe left half of the body is controlled by the right side of the brain and vice versa. ØSodium and Potassium are vital to neural operations.

Occipital Lobe Parts of the Brain Frontal Lobe The ability to concentrate and attend, elaboration of thought, abstract reasoning, problem solving, judgment, muscle memory. Also includes Broca’s area. Parietal Lobe Touch perception and goal directed voluntary movements Temporal Lobe Hearing ability, memory acquisition and long-term storage (hippocampus), sense of identity, behavior and emotions (amygdala). Also includes Wernicke’s area. Occipital Lobe Primary visual reception area Brain Stem Involuntary responses and actions (breathing, heart rate, swallowing, startle response). Damage here can affect level of alertness and ability to sleep. Cerebellum Regulation and coordination of movement and balance

Neurological Disorder Terms • Aphasia – Language impairment usually due to left hemisphere damage to Broca’s (impairing expression) or Wernicke’s (impairing understanding). • Agnosia – Can’t recognize every day objects • Ataxia – lack of gross motor coordination • Apraxia – inability to carry out directed motor functions even though directions are understood

Musculoskeletal System • Composed of bones, muscles, cartilage, tendons, ligaments, joints and other connective tissue • Provides form, support, stability and movement to the body

Musculoskeletal System (cont. ) Elements of the musculoskeletal system: • Bones – provide support for the body, protect body organs, produce blood cells, store minerals • Cartilage – a firm substance that prevents bone to bone contact • Tendons – attach the muscle to the bone • Ligaments – attach bone to bone • Skeletal Muscles – attached to the bone by tendons; move bones through contracting ▫ Type 1: slow/endurance ▫ Type 2: fast/sprint

Useful Root Words for reading Medical Records A- Away or not – eg. Ataxia (not ordered) Distal Away from the center – eg. Fingers are distal to the body Dorsal Towards the back – eg. Ears are dorsal to the nose Hemi- Half – eg. Hemiplegic (paralyzed from the waist down) Hyper- Over or above – eg. Psuedo-hypertrophy (fake muscle mass increase) Hypo- Under or below – eg. Hypoxia (not enough oxygen) Myo Muscles - eg. Fibromyalgia (muscle pain) Para- To one side – eg. Paraplegic (paralyzed on one side of the body Proximal Towards the center – eg. Shoulders are proximal to the body Quad- Four – eg. Quadriplegic (paralyzed in all four limbs) Ventral- Towards the front eg. Chest is ventral to the back.

Causes of Severe Physical Handicaps • Physical disabilities and health conditions are classified as either congenital or acquired. ▫ Congenital : either are born with physical difficulties or develop them soon after birth ▫ Acquired : developed through injury or disease while the child is developing normally. The age at which a condition develops often determines its impact on the child.

Causes (cont. ) Cause Meaning Trauma means injury. Many people become injured through accidents. In these cases the skeletal, muscular or nervous systems may become damaged. Trauma usually happens after birth. Illness There are many illnesses that can cause damage to body systems that control movement and may lead to physically disabilities. Many illnesses are caused by a variety of bacteria and viruses. Some illnesses may be inherited and there are some illnesses for which scientists still don't know the cause. Congenital Something that is "congenital" means present when a person is born. In these cases, a person is born with a physical disability – doesn’t have to be genetic. Genetic means that something is inherited from a person’s parents. This means that one or both parents passed a gene that carried that disease or disability. Genetic disorders can sometimes be caused by a mutation of a gene. Scientist usually don't know why these mutations happen and when they can happen.

Convulsive Disorders

Convulsive Disorders Definition • Characterized by having seizures • Seizures occur when a burst of electrical impulses in the brain escape their normal limits. They spread to other areas and create an uncontrolled storm of electrical activity. The electrical impulses can be transmitted to the muscles, causing jerking or convulsions. • Though seizures can be concomitant with other disorders, but when it occurs alone, it is considered epilepsy

Convulsive Disorders Causes • In 70% of cases, the cause of epilepsy is unknown. • When the cause is known it is usually due to a brain injury of some kind. • Other possible causes: hypoxia, stroke, abnormal levels of substances (e. g. , sodium, blood sugar) that temporarily deprive the brain of required nutrients • About 30% of those with epilepsy are children

Convulsive Disorders Types • There are two main categories of seizures: Generalized Seizures and Partial Seizures • Two most common types of seizures*: ▫ Grand Mal seizures (loss of consciousness, collapse, violent jerking) Video Clip ▫ Absence seizures (mostly in children, brief loss of consciousness, no jerking, may occur several times a day) Video Clip * There are other types of seizures; however, these two are the most common

Convulsive Disorders Academic Implications Common medication side effects §Inattention/Concentration problems §Hyperactivity §Memory problems §Excessive fatigue §Depression §Physical aggression/rage/tantrums/irritability §Cognitive impairment related to injuries and scarring in the brain §Tiredness §Speech slurring §Balance issues, clumsiness §Dizziness and nausea §Mood changes §Memory loss §Irritability §Vision disturbances §Hyperactivity

Convulsive Disorders Recommendations • • Social Skills Groups Structure and consistent schedule Frequent breaks Clear and consistent rules at home and at school with consequences for behavior • Check for understanding frequently • Be aware absence seizures may be occurring throughout the day, which may affect memory

Cerebral Palsy

Cerebral Palsy Definition and Causes • Cerebral palsy is a condition, sometimes thought of as a group of disorders, that can involve brain and nervous system functions such as movement, learning, hearing, seeing, and thinking. • Caused by injuries or abnormalities of the brain • Usually occurs during pregnancy • Risk Factors: premature birth, infections, severe jaundice, hypoxia, traumatic brain hemorrhage, toxicity

Cerebral Palsy Symptoms • Most common symptoms: ▫ ▫ ▫ Muscles that are very tight and do not stretch. Abnormal walk (gait) Joints are tight and do not open up all the way Paralysis May affect one arm or leg, one side of the body, both legs, or both arms and legs

Cerebral Palsy Symptoms • Other Symptoms: ▫ Speech problems ▫ Seizures ▫ Cognitive Impairment (around 50% have ID) ▫ Hearing/vision problems ▫ Difficulty swallowing

Cerebral Palsy Academic Implications/ Recommendations • Due to physical limitations and speech problems, the following accommodations/modifications are usually necessary : ▫ ▫ ▫ Physical therapy Occupational therapy Speech therapy Communication devices/computer technology Special Day Classes, depending on the cognitive and communication level of the student • Video clip

Fredreich’s Ataxia

Fredreich’s Ataxia Definition and Causes • Genetic disorder caused by a defect in the gene Frataxin (FXN), which causes the body to produce too much of part of the DNA called trinucleotide repeat (GAA). • Symptoms are caused by the wearing away of areas of the brain and spinal cord that control coordination, muscle movement, some sensory functions, and may also affect the heart.

Fredreich’s Ataxia Symptoms • Common symptoms include: ▫ ▫ ▫ ▫ ▫ Abnormal speech Changes in vision, particularly color vision Hearing loss - occurs in about 10% of patients Jerky eye movements Loss of coordination and balance, which leads to frequent falls Muscle weakness Unsteady gait and uncoordinated movements (ataxia) Muscle problems (can lead to scoliosis) Heart disease Diabetes (later stages)

Fredreich’s Ataxia Academic Implications/ Recommendations • • • Speech therapy Physical therapy Occupational therapy Walking aids or wheelchairs Access to keyboard or computer May need to alter PE requirements Longer time for tests and in class assignments Use of a scribe Letting the student leave a few minutes early for lunch or between classes to miss crowds

Neural Tube Defects

Neural Tube Defects Definition and Causes • Neural tube defects are birth defects of the brain, spinal cord, or vertebrae. • Causes: environmental (e. g. , diabetes, obesity, drugs), nutritional components (e. g. , folic acid deficiencies

Neural Tube Defects Types • Anencephaly: Most of the brain does not develop. Most fetuses spontaneously abort and those that are born rarely survive. • Encephalocele: The bones of the skull do not completely close and a sac like formation with brain tissue and spinal fluid protrudes outside the head skull. • Spina bifida: the fetal spinal column doesn’t close completely during the first month of pregnancy. There is usually nerve damage that causes at least some paralysis of the legs.

Neural Tube Defects Types of Spina Bifida • Spina bifida occulta: The bones of the spine do not close but the spinal cord and meninges remain in place and skin usually covers the defect. Mildest form. • Meningoceles: The tissue covering the spinal cord sticks out of the spinal defect but the spinal cord remains in place. Rarest form. • Myelomeningocele: is a birth defect in which the backbone and spinal canal do not close before birth. Most severe form.

Neural Tube Defects Symptoms/Academic Implications • Symtpoms: ▫ ▫ Varying degrees of paralysis of the lower limbs Most children will have some form of a learning disability Possible bowel and bladder complications May have hydrocephalus • Academic Implications: ▫ ▫ ▫ May need a bladder management program Difficulties with attention Trouble expressing or understanding language Problems with reading or math Depending on cognitive level, may benefit from more intense services such as placement in an SDC class

Muscular Dystrophy

Muscular Dystrophy Definition and causes • Also called Inherited Myopathy or MD • Muscular dystrophy is a group of inherited disorders that involve muscle weakness and loss of muscle tissue, which get worse over time. • Incidence: about 1 in 651, 450 persons in the United States. • May occur in childhood or adulthood • The more severe forms tend to occur in early childhood.

Muscular Dystrophy Types • There are many different types of muscular dystrophy. They include: ▫ ▫ ▫ ▫ Becker muscular dystrophy Duchenne muscular dystrophy Emery-Dreifuss muscular dystrophy Facioscapulohumeral muscular dystrophy Limb-girdle muscular dystrophy Myotonia congenita Myotonic dystrophy • Duchenne’s and Becker’s muscular dystrophies affect males almost exclusively. • Severity depends on type. Duchenne MD is deadly, while others can cause little disability.

Muscular Dystrophy Symptoms • Different types of MD present different symptoms and affect specific muscle groups. • All of the muscles may be affected (including the heart). Or, only specific groups of muscles may be affected, such as those around the pelvis, shoulder, or face. • Reading disabilities are common • Mental retardation is present in some types of the condition Symptoms • Muscle weakness that slowly gets • Frequent falls worse • Loss of strength in a muscle or • Delayed development of muscle group of muscles as an adult motor skills • Loss in muscle size • Difficulty using one or more muscle groups • Problems walking (delayed walking) • Eyelid drooping (ptosis) • Drooling

Muscular Dystrophy Academic Implications/Recommendations • There are no known cures for the various muscular dystrophies. The goal of treatment is to control symptoms. • In some cases, surgery on the spine or legs can improve functioning • Treatment may include: ▫ ▫ Physical therapy Orthopedic appliances such as braces and wheelchairs Corticosteroids (especially for children) Maintaining an active lifestyle

Traumatic Paraplegia and Quadriplegia

Traumatic Paraplegia and Quadriplegia Definition and Prevalence • “Traumatic” injury: Characterized by damage to the bones of the spine that surround the spinal cord, often resulting in damage to the nerves inside the spinal column. • Traumatic quadriplegia: Spinal cord or nerve root deficit not involving the cranial nerves above and including C 8, T 1 roots. • Traumatic paraplegia: Spinal cord or nerve root deficit below and including T 2. • Complete: Complete motor and sensory deficit below the level of the injury. • Incomplete: Any sensory or motor sparing below the level of injury including perianal sensation.

Traumatic Paraplegia and Quadriplegia Common Causes • Broken neck or back neck caused by: ▫ ▫ ▫ Vehicular accidents 37% Violence 28% Falls 21% Sports-related 6% Other 8% (e. g. , complications following surgery) ▫ ▫ Infection of the spinal nerve cells Cysts or tumours pressing on the spinal cord Interruption of the blood supply to the spinal cord Congenital medical conditions (e. g. , spina bifida) • SCI can also be caused by so-called ‘non-traumatic’ cord injury, such as:

Traumatic Paraplegia and Quadriplegia Symptoms Common symptoms include: • Loss of sensation and motor function • Dysfunction of the bowel and bladder • Impaired or lost sexual functioning • Men may have their fertility affected, while a women's fertility is generally not affected. • Low blood pressure • Reduced control of body temperature • Inability to sweat below the level of injury • Chronic pain.

Level Abilities Functional Goals Limited movement of head and neck Breathing: Ventilator or implant Communication: Talking can be difficult, very limited or impossible. Daily tasks: Needs assistive technology for independence Mobility: Can operate an electric wheelchair with head mouth, or chin C 4 Usually has head and neck control. May shrug their shoulders. Breathing: May initially require a ventilator for breathing Communication: Normal, may have weaker voice projection Daily tasks: May have limited independence with specialized equipment C 5 Typically has head and neck control, can shrug shoulder and has shoulder control. Can bend his/her elbows and turn palms face up. Daily tasks: Need some specialized equipment but will have independence with some daily activities (e. g. , eating, drinking, grooming) Mobility: Power wheelchair with hand controls. Driving may be possible. C 6 Has movement in head, neck, shoulders, arms and wrists. Can shrug shoulders, bend elbows, turn palms up and down and extend wrists. Daily tasks: Can perform some daily tasks using specialized equipment (e. g. , feeding, bathing, grooming, personal hygiene, dressing, and light housekeeping) Mobility: Can use a manual or power wheelchair. Some independent transfer. C 7 Has similar movement as an individual with C 6, with added ability to straighten his/her elbows. Daily tasks: Able to perform household duties. Need fewer adaptive aids. Health care: Able to do wheelchair push ups for pressure relief. Mobility: Daily use of manual wheelchair. Can transfer with greater ease. C 8 -T 1 Has added strength and precision of fingers that result in limited or natural hand function. Daily tasks: Can live independently without assistive devices in daily living. Mobility: Uses manual wheelchair. Can transfer independently. T 2 -T 6 Has normal motor function in head, neck, shoulders, arms, hands and fingers. Has increased trunk control. Daily tasks: Should be totally independent with all activities. Mobility: Possible limited walking with extensive bracing. Requires high energy, offers no functional advantage, and can lead to damage of upper joints. T 7 -T 12 Has added motor function from increased abdominal control. Daily tasks: Able to perform unsupported seated activities Mobility: Same as T 2 -T 6. Health care: Has improved cough effectiveness. L 1 -L 5 Has additional return of motor movement in the hips and knees. Mobility: Walking can be a viable function, with the help of specialized leg and ankle braces. Lower levels walk with greater ease with the help of assistive devices. S 1 -S 5 Depending on level of injury, there are various Mobility: Increased ability to walk with fewer or no supportive devices degrees of return of voluntary bladder, bowel and sexual functions. C 1 -C 3

Traumatic Paraplegia and Quadriplegia Academic Implications & Recommendations • Functioning will vary based on location of damage and severity of symptoms • Many require assistance for personal care ▫ ▫ ▫ ▫ ▫ Physical therapy Occupational therapy Counseling Adapted power wheelchairs Tape recorders Computers Page turners Mouth control (sip and puff) units Voice activation, chin control, head control, eyebrow control, or eye blink ▫ Balanced forearm orthosis (brace) forearm and wrist stability

Connective Tissue Disease

Connective Tissue Disease Definition & Causes • Mixed connective tissue disease (MCTD): uncommon autoimmune disorder that features the connective tissues as a primary target for pathology. • Connective tissues: The structural portions of our body that essentially hold the cells of the body together. • Characterized as a group by the presence of spontaneous overactivity of the immune system which results in the production of extra antibodies into the circulation. Leads to inflammation in tissues. • Classified under autoimmune disorders • Most commonly diagnosed in women in their 20’s and 30’s • Some are inherited (e. g. , Marfan Syndrome, Ehlers-Danlos syndrome); others have no known cause or are believed to be triggered by infection

Connective Tissue Disease Types • Causes overlapping features of primarily three connective tissue diseases — ▫ lupus ▫ scleroderma ▫ polymyositis • May also have features of rheumatoid arthritis • Each can be identified by a blood test and distinctive, classic symptoms • Can be “undifferentiated” at first, as symptom onset can be gradual • Pediatric MCTD occurs in children under the age of 16. MCTD is three times more frequent in girls than boys. • 93% of children with pediatric MCTD have arthritis

Connective Tissue Disease Symptoms • Symptoms: ▫ Raynaud's disease — blood vessel spasms that interrupt blood flow to the fingers, toes, ears and nose ▫ Fatigue ▫ General feeling of being unwell (malaise) ▫ Muscle pains (myalgias) ▫ Joint pains (athralgias) ▫ Mild fever ▫ Joint swelling ▫ Swollen hands and puffy fingers ▫ Shortness of breath and chest pain (uncommon but dangerous; could be pulmonary hypertension) • Often begins with fever, decreased energy, and weakness • Can range from mild to life-threatening

Connective Tissue Disease Academic Implications & Recommendations • Can be treated with medication; may require only for flare-ups, or all the time • Prognosis can vary; some go into remission, and others have more serious, long-lasting symptoms but can still lead an active, productive life • Pay attention to medication side effects (e. g. , nausea, weight gain, hair loss) • Recommendations will vary by symptoms

Resources • • Epilepsy. com: www. epilepsy. com • • • United Cerebral Palsy: www. ucp. org Epilepsy Foundation: www. epilepsyfoundation. org Spina Bifida Asspciation: www. spinabifidaassociation. org The Nemours Foundation. Provides information for kids, teens, and parents: Kidshealth. org 4 My. Child: www. cerebralpalsy. org The Friedreich’s Ataxia Research Alliance: www. curefa. org Muscular Dystrophy Association: www. mdausa. org American Autoimmune Related Diseases Association, Inc. : www. aarda. org

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