TIS                         

The influence of chronic illness on children’s development is of paramount importance to therapists working with this population of patients. Researchers have suggested that chronically ill children might develop in different ways due to their condition, in addition to family and environmental factors. The manner and extent to which a chronic illness may modify typical developmental processes depend to a large degree on the illness, its severity, its natural history and expected prognosis, the degree of limitation associated with it, the presence or expectation of mental retardation, its genetic background and implications, necessity for care, and the degree to which the illness if visible and thus known to the wider community.

Chapter 3 will present common chronic illnesses in children and suggest therapeutic interventions appropriate for each.

There are varying degrees of chronic illnesses to those that are severely affected to those that have mild or minimal symptoms. However, research is showing that mildly affected children are actually poorer at coping with their disease and are subject to increased emotional and psychological problems than children that are more severely involved. It seems that unlike severely affected children, who constantly have to battle with illness-related difficulties and are able to recognize their own limitations, mildly affected children try to deny or hide their conditions and behave as if they were without any risk of medical difficulties. It has also been suggested that less severely affected children may also try to function in both worlds, that of the normal and that of the sick child, leading to inability to function effectively in either world.

CHRONIC ILLNESS - refers to an illness that can last an extended period of at least 3 months and often for life, and cannot be cured. Thus, medical care is aimed at management of the illness through pain control (sickle cell anemia), reduction of exacerbations or attacks (asthma), bleeding episodes (hemophilia), or seizures (epilepsy). Treatment is aimed at reducing the potentially negative effect of the chronic illness to enable the child to adopt a normal life style.

CHRONIC JOINT DISEASES

Juvenile rheumatoid arthritis (JRA) - a group of diseases characterized by chronic joint inflammation thought to be an autoimmune disorder in response to infection or trauma.

1. Systemic JRA- multisystem involvement in addition to multiple joint pain and inflammation. May include pericarditis, myocarditis, hepatosplenomegaly.
2. Polyarticular JRA - Involvement of 5 or more joints usually symmetrical distribution. Knees and ankles frequently involved; elbows, wrists, and fingers less often and rarely cervical spine. More prominent in girls.
3. Pauciarticular JRA - most frequently seen of the JRA types with joint involvement of 4 joints or fewer with 2 further subgroups.

Treatment interventions should be directed at functional limitations dependent on pain and joint mobility limitations and consideration of any organ system involvement. Gait is usually affected through diminished velocity and stride length with antalgic characteristics. Crutches, or a walker may be necessary to maintain ambulation. Gait determinants should be considered with emphasis on functionality and stability while working within the limitations of pain. Endurance should be worked toward and effective self-range of motion and positioning programs to rest inflamed joints while reducing the risk of contracture or increasing deformity. Elbow, wrists, and finger involvement may also affect activities of daily living and may also negatively impact the ability of the upper extremities to bear weight in gait.

Sickle cell anemia - blood disorder predominantly seen in individuals of African descent where the red blood cells assume a crescent shape. This disorder causes intense localized muscle and joint pain with the possibility of embolic-like cell clusters causing paralysis or secondary paresis. Limited respiratory reserve is noted with contraindications for any cardiorespiratory challenge.

Treatment interventions include identifying and minimizing behaviors that may lead to a sickle cell crises by avoiding high demand challenges. Muscle and/or joint pain is difficult to treat with physical or occupational therapy alone. Appropriate interventions for neurological complications should be considered that may include normalization of tone, weight-bearing on affected extremities, functional activities and instruction with assistive devices as needed.

CHRONIC MUSCLE DISEASES

Muscular dystrophy - genetically related intrinsic muscle disease that may be progressive in nature. There are several different types of dystrophies categorized as MD.

Duchenne’s muscular dystrophy - x-linked recessive with mostly males and rarely females being affected. This is a progressive disease progressing to death in late adolescence or early adulthood. The weakness from proximal to distal with delayed motor milestones and loss of ambulatory abilities young.

Becker muscular dystrophy - is a milder variant of Duchenne’s with onset of weakness of 5 - 15 years or later with proximal to distal progression.

Limb-girdle muscular dystrophy (Erb’s dystrophy) - autosomal recessive with a group of disorders in both males and females characterized by proximal weakness. The onset of weakness begins in second decade of life with slow progression.

Facioscapulo-humeral Dystrophy (Landouzy-Dejerine Muscular Dystrophy) - autosomal dominant with onset of weakness noted after the second decade with initial involvement in face, shoulder girdle, and progression to pelvic girdle.

Myotonic Dystrophy (Steinert Syndrome) - autosomal dominant characterized by absence or weakness of sternocleidomastoid muscle. Weakness progresses distally to proximally with a variable onset.

Central Core Myopathy - autosomal dominant with non-progressive weakness and generalized hypotonia.

Nemaline Myopathy - autosomal dominant with a variable onset of weakness marked with hypotonia and arachnodactyly. Cardiac involvement may be fatal.

Myotubular Myopathy - autsomal recessive with hypotonia onset in infancy with ptosis, facial and optical paralysis and slow progression of weakness.

Treatment interventions for muscular dystrophies should be individualized to include:
• Parent education of the role of genetics in the disease process
• Maintaining mobility as long as possible by encouraging functional activities rather than specific strengthening regimes; utilize adaptive equipment; encourage recreational activities; promote cardio-pulmonary fitness.
• Anticipate contractures and use positioning programs and devices to maintain optimal alignment and minimize need for stretching.

CHRONIC NEUROLGIC DISEASES

Spina bifida - is a congenital malformation of the spinal cord and/or vertebral malformation. There is some evidence that hot baths early in a pregnancy, and/or the lack of folic acid may increase the likelihood of this infant manifestation. This disorder is often associated with hydrocephalus. There are several grades and type of spina bifida:

Cranial meningocele and cranial encephalocele - superior neural tube defect which may be amendable to surgical repair with optimistic outcome

Spina bifida occulta - typically vertebral defect only with nonfusion of vertebral arches and no spinal cord involvement or weakness.

Spina bifida aperta - total absence of spinal cord closure and may be anencephaly if it occurs at the uppermost part of the spinal cord resulting in minimal brain development and absence of a cerebrum.

Spina bifida cystica - external appearance of a fluid-filled sac
Meningocele - the sac contains cerebrospinal fluid only and the spinal cord remains intact with little or no weakness following surgical repair.

Myelomeningocele - the sac contains cerebrospinal fluid and the spinal cord is protruded into it. The amount of impairment is directly associated with the level of the defect and the amount of cord involvement. This is the most common type of spina bifida.

Diastematomyelia - development of two hemi spinal cords separated by a bony spur or block. There may be no neurological symptoms or there may be rapid onset of scoliosis, lower extremity hypertonis and urological symptoms.

Treatment interventions are highly variable depending on location and severity of spinal cord involvement. Symptoms range from minimal bowel and bladder incontinence with club feet to limited paraparesis, to complete paraplegia. Often there are weak or absent gluteus maximus muscles and hip adductors leading to tibial band tightness and hip flexor contracture. The therapist should anticipate problems and develop proactive interventions including:


• Observing for shunt malfunction
• Anticipate hip flexor contracture and emphasize full hip extension from an early age; mininize dislocation potential of hip
• Consider sitting and positioning programs to negate hip flexor tightness and kyphotic back alignment institute standing and weight bearing when ace appropriate
• Promote optimal trunk and extremity alignment
• Work toward gross, fine, and oral motor skills through cognitive objectives at age appropriate intervals promote independent locomotion whether by ambulation, scooter board, or wheelchair.
• Encourage independence with adaptive tools as needed for activities of daily living
• Provide and consult on assistive devices such as braces, reciprocating gait orthoses, crutches and walkers and wheelchairs.

Cerebral palsy - represents a group of prenatal, perinatal, or postnal disorders resulting from maternal infection, trauma, cranial hypoxia, or consanguinity. Frequently associated with placental insufficiency, prematurity, and Grades III and IV intraventricular hemmorhage.


Spastic cerebral palsy - typically has increased muscle tone in antigravity muscles. Muscle imbalance across joints may lead to range of motion limitations and ambulatory and motor difficulties.

Spastic quadriplegia - denotes four extremity involvement as well as throat, neck, and trunk musculature. Symptoms may include neonatal feeding dyscoordination and generalized hypotonia that gradually transitions into muscle imbalance and spasticity by the first year of life. Seizure, hip subluxation, cognitive, visual, auditory and motor deficits are often present.

Spastic diplegia - describes mainly lower extremity involvement characterized by a gait pattern of short stride length, excessive hip adduction and internal rotation, and ankle plantar flexion. Reciprocal movements may be difficult to attain. Often associated with prematurity. Visual defects and oral motor difficulties may also be present.

Spastic hemiplegia - describes paralysis of one side of the body with the child’s tendency to neglect the affect side and compensate with the opposite side. May be associated with strabismus, seizures, learning, and perceptual disorders.

Ataxic cerebral palsy - generalized decreased muscle tone throughout with an initial infant diagnosis of "floppy baby syndrome". Characteristics include a wide base of support in weight bearing positions along with ataxia and incoordination of ambulatory efforts. Occasionally hop abduction contractures may occur. This type of CP is associated with abnormal cerebellar development and may include poor visual tracking and speech delay.

Athetoid cerebral palsy - is a form of dyskinesis with extraneous movements, postural instability, fluctuating muscle tone with difficult during speech, feeding, and upper extremity activities. This is a common sequelae following Rh incompatibility factors with the mother. Cognitive ability may be normal an hearing loss may be present.

Mixed cerebral palsy - describes the presence of athetosis and spasticity together.

Treatment interventions should be directed at the individual child’s needs and developmental level. Common interventions include:
• Maximize movement capabilities and avoid contractures
• Intervene early with mobility and developmental activities
• Simulate age-appropriate gross and fine motor experiences
• Initiate standing through supported weightbearing by 9-10 months
• Provide trunk and upper extremity alignment through proper positioning to facilitate fine motor and feeding skills
• Assist child to initiate or complete transitions between positions
• Address oral motor functions in conjunction with feeding and speech
• Work with normalization of tone
• Correct head and truncal asymmetry
• Emphasize elongation of hip adductors and internal rotators
• Emphasize elongation of spastic hamstrings to prevent posterior pelvic tilt that leads to kyphosis
• Generalize trunk and neck strengthening exercises
• Utilize sidelying positioning in adduction and extremity loading through kneeling or standing
• Integrate all interventions into the child’s daily activities as possible

Other: there are many other supplemental treatments for the various forms of cerebral palsy that may be used in conjunction or in addition to therapy. These include direct neuromuscular electrical stimulation (NMES), neurosurgical procedures such as spinal cord stimulation and rhizotomy, pharmacological interventions, and orthopedic interventions that may include casting and bracing.


Brachial Plexus Injury - results from compression or traction injury to the brachial plexus, typically unilateral and may occur secondary to shoulder trauma from prenatal or postnatal events or from anomalies such as cervical rib or abnormal thoracic vertebrae. Fractures of the clavicle or humerus, shoulder dislocation, and facial or phrenic nerve damage may coexist. Sensory impairment is usually present with motor development compromised.

Erb’s Palsy - results from an upper plexus injury to C5 and C6 nerve roots with resulting weakness or paralysis of the levator scapulae, rhomboids, deltoid, serratus anterior, supraspinatus, infrasprinatus, biceps brachii, brachialis, brachioradialis, forearm supinator, and forearm extensiors of the wrist, fingers, and thumb.

Klumpke Paralysis/Palsy - is a lower plexus injury to C7, C8, and T1 that results in distal weakness or paralysis of the wrist and finger flexors and extensors, and the intrinsic muscles of the hand.

Erb-Klumpke Paralysis/Palsy - mixed involvement which may include nerve rootlets from C5 to T1 with resulting muscle weakness or paralysis.

Treatment interventions are dependent on the severity and extent of sensory and motor impairment in the involved arm. Infants may recover from traction injuries spontaneously. However, recovery from avulsion injuries may be limited. Shoulder subluxation and contractures may develop secondarily to the muscle imbalances. Treatment goals should be directed at avoiding further damage through positioning programs and gentle range of motion; minimizing or avoiding contractures with consideration of appropriate soft splints of the shoulder, elbow, wrist or fingers. Splinting is considered controversial in the shoulder region. As the infant matures, age-appropriate dynamic weightbearing or loading of the extremity is recommended. Neuromuscular electrical stimulation may be considered and sensorimotor activities are recommended.

CHRONIC MEDICAL CONDITIONS

Asthma - may or not be a genetically based chronic lung disorder often associated with allergies. BPD of infancy may progress to asthma conditions. Medications usually can control the symptoms and the child should be able to participate in all activities normally when in control medically. Activity should be encouraged along with self-instruction to even the youngest child to recognize triggers and appropriate preventative routines.

Cystic fibrosis - inherited progressive disorder of the exocrine glands that produces pancreatic insufficiency, hyperplasia of mucous-producing cells in the lungs, excessive electrolyte secretion by the sweat glands, and digital clubbing.

Therapy interventions are generally directed at cardiopulmonary issues including bronchial hygiene with percussion and vibration in addition to inhaled medications; chest wall mobility exercises to maintain balanced proportions of the rib cage; optimal postural alignment to avoid kyphosis or scoliosis and allow the lungs as much space as possible to function; and cardiopulmonary conditioning to maintain as active a lifestyle as possible.

Congenital heart conditions - there are several structural cardiovascular anomalies associated with arrested development occurring during gestational days 18 - 50. Rubella infection, maternal drug and alcohol ingestion, radiation exposure, maternal infections, and maternal diabetes have all been suggested as contributory.

Patent ductus arteriosus - is failure of ductus arteriosus to close soon after birth, allowing blood flow from high pressure aorta to pulmonary artery and left to right shunt. Often associated with prematurity and may close with medication alone though surgical intervention may be necessary.

Atrial septal defect(s) - include single or multiple openings in the wall separating the two atria thus allowing flow going directly into the ventricles.

Ventricular septal defect(s) - include single or multiple openings in the wall separating the ventricles resulting in significant left to right shunt with pulmonary engorgement and issues. Surgical patching is indicated.

Coartation of the aorta - denotes narrowing of the aorta in close approximation to the ductus arteriosus with increased resistance to blood flow in the left ventricle. Surgical correction is necessary.

Tetralogy of Fallot - includes ventricular septal defect, pulmonary stenosis with right ventricular hypertrophy, and aortic dextroposition resulting in significant right to left shunt and cyanosis with severe exercise limitations and tolerance.

Transposition of the great vessels - results from incomplete division of embryonic exit trunk from heart, resulting in aorta coming from the right ventricle and the pulmonary artery from the left with septal defects. Cyanosis and severe restrictions maybe present.

Hypoplastic left heart syndrome - describes the condition of the left heart incapable of providing sufficient blood flow to maintain life and corrective measures include heart transplant.

Therapeutic interventions - include monitoring exercise tolerance; efforts focused on gross motor developmental delays that are common in infants and children with severe anomalies. Following surgeries treatments may need to focus on pain issues and limited range of motion particularly of the neck, thoracic area and shoulders.

Hemophilia - x-linked recessive disorder resulting in impaired blood clotting capability which causes pain and reduced range of motion and muscle strength in joints into which bleeding occurs. The hinge joints of the knee, elbow, and ankle are typically affected and may lead to arthritic conditions. Intracranial bleeding can lead to death.

Treatment interventions include minimizing joint trauma and hemorrhages, proactive muscle strengthening exercise, and encouraging recreational activities to promote cardiopulmonary fitness. Muscle hemorrhages should be treated with elastic pressure bandages and ice while joint hemorrhages may require immobilization and gentle range of motion.

The same developmental tasks and challenges experienced by healthy children are also experienced by chronically ill children. However, the ability to master these tasks and cope with the typical stresses of childhood can be made more difficult by the presence of an illness. Physiological aspects of the disease itself, medications and other forms of treatment, frequent hospitalizations, disruptions in daily activities, and alterations in family relationships are some of the potential factors that may restrict the normal development in chronically ill children. Thus, the therapist is challenged to create therapy interventions that allow for functional progress while being mindful to the chronicity of the underlying conditions.




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