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Medical Conditions Commonly
Associated with Developmental Disabilities

Cardiovascular Disorders
Communicative Difficulty
Decubitus Formation/Skin Breakdown
Dental Care
Failure to Thrive/Malnutrition
Gastrointestinal Disorders
Hydrocephalus
Orthopedic Disorders
Psychiatric Disorders
Pulmonary Complications
Seizure Disorders
Spasticity
Urinary Tract Disorders
Sexuality and Health
References

Cardiovascular Disorders

Some developmental disabilities may be associated with alterations in the anatomy and physiology of various organ systems. For example, newborns with Down syndrome are up to 50% more likely to experience congenital heart defects-the most common of these being mitral valve prolapse, and/or atrial or ventricular septal defects (ASD or VSD). It is vital that healthcare providers be aware of the potential for endocarditis in such individuals. Patient education concerning prophylactic antibiotic therapy before invasive procedures-including dental work- is imperative.

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Communicative Difficulty

Speaking difficulty (dysarthria) is common among persons with certain developmental disabilities, including cerebral palsy, autism, and mental retardation. Poor control of the muscles of the lips and tongue may make speech sound slurred, garbled, or unintelligible. Children with autism may experience expressive language delay. Some persons may not be able to speak at all. Children with developmental disabilities should be assessed early and frequently for speech development and pathology. Referral to a speech therapist should be made early in order to prevent delay in speech development. Great emphasis should be placed on facilitating optimal communication. For individuals with severe communication difficulties, an assistive technology speech augmentation device may be required. Care must be taken not to assume retardation in individuals exhibiting dysarthria or aphasia.

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Decubitus Formation/Skin breakdown

Individuals with developmental disabilities such as cerebral palsy or spina bifida often experience limited mobility due to spasticity or paralysis. Spasticity may preclude 'normal' motor function by primary neuromuscular effects or by resulting contractures. Differing degrees of immobility may be experienced by a person with cerebral palsy or other disability.

Immobility results in areas of prolonged diminished circulation to the skin and deeper tissues. Initially these areas present as persistent reddened patches of skin most frequently located over bony prominences. Without intervention, these areas often progress to frank necrosis (death) of affected tissues. An able-bodied individual continually shifts his or her position in response to discomfort signals generated by the nervous system. This shifting of weight thereby prevents formation of pressure ulcers. Conversely, the person experiencing neurological deficits may not either adequately sense the discomfort nor have the motor control necessary to shift his or her position in response. In cases where mobility is significantly restricted, constant attention must be paid to skin integrity and protection.

Another factor which may exacerbate skin breakdown is bowel and bladder incontinence. Persons with cerebral palsy and spina bifida often experience difficulty maintaining bowel and bladder control. Constant irritation from contact with urine and/or feces may irritate skin in areas where circulation is frequently already compromised. Hygiene in these areas is imperative in order to prevent infection from occurring.

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Dental Care

The child/adult with significant developmental disability often presents a challenge to caregivers and other providers when attempting to provide oral hygiene and dental maintenance. Many factors such as hyperactive oral-motor reflexes (e.g., tongue-thrust, tonic bite), poor oral-motor function, and excessive salivation (sialorrhea) make oral hygiene difficult to maintain. Poor bolus formation and/or difficulty swallowing may result in oral stasis of food. This in turn leads to bacterial colonization of the oral cavity - and dental caries often result. Oral infections also produce offensive odors. Individuals using certain anti-seizure medications may incur overgrowth of the gums (gingival hyperplasia). Additionally, poor dental occlusion may lead to abnormal wear of tooth enamel. Regular dental exams should begin by age two years.

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Failure to Thrive/Malnutrition

Adequate nutrition can be a significant challenge in children and adults with developmental disability. Infants and children often present with varying degrees of feeding difficulty and are often underweight for age. Several factors may lead to malnutrition in the person with cerebral palsy in particular. Lack of exercise due to diminished mobility may decrease appetite. Oral-motor dysfunction may limit dietary choices. Gastroesophageal reflux (GERD) is often present and may result in vomiting and/or severe esophageal erosion. Individuals affected by oral-motor dysfunction and GERD are also at risk for aspiration. Assessment of nutritional status is imperative as some children may require the placement of a gastrostomy tube to provide adequate intake and promote growth.

Other factors which may influence growth and development in individuals with disability include alterations in sensory perception (such as in autism), neuropsychiatric disorders, medical illness, and/or side effects of medication.

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Gastrointestinal Disorder

Constipation related to a variety of causes is often a problem in children and adults with developmental disabilities. Decreased mobility may lead to diminished appetite, which can result in inadequate roughage in the diet. Additionally, certain disorders such as cerebral palsy may induce oral-motor dysfunction, which restricts the amount and type of food consumed. Gastrointestinal transit time is increased substantially in individuals with cerebral palsy - resulting potentially in stasis of stool, bloating, and impaction. Decreased mobility and/or perineal sensation may result in constipation. Constipation itself causes decreased appetite, thereby inducing a 'vicious cycle.' Chronic constipation may thus lead to delayed growth and/or general malnutrition in affected individuals. Gastroesophageal reflux disease is also common among persons with developmental disabilities.

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Hydrocephalus

Approximately 85% of infants born with meningomyelocele (a form of spina bifida) experience hydrocephalus, requiring placement of a ventriculo-peritoneal shunt. Hydrocephalus is a condition characterized by increased cerebrospinal fluid (CSF) pressure. This increase in CSF pressure is caused by blockage to the flow of cerebrospinal fluid within the central nervous system. Well-infant checks of children at risk should include assessment for symptoms of increased intracranial pressure (ICP) such as lethargy, irritability, or nausea and vomiting. Left untreated, increased ICP may progress to profound lethargy, vomiting, increased blood pressure, lowered heart rate, brain stem herniation, and death.

A Ventriculo-peritoneal (VP) shunt placement is usually necessary to lower CSF pressure in infants/children exhibiting clinical hydrocephalus. A plastic catheter is essentially placed into the ventricle of the brain. The catheter is brought out beneath the scalp and tunneled under the skin to the abdomen. The shunt is then inserted into the peritoneal cavity as a drain for the excess CSF. Placement of a VP shunt is not without risk. Encephalitis, meningitis, and immune reactions are a concern.

Parents should be educated in the function and observation of a VP shunt, as well as the signs and symptoms of increased ICP associated with shunt failure. Health care practitioners should be aware that children with VP shunts are more likely to experience allergies to latex - containing supplies. Latex exposure, especially during surgical intervention, may result in life-threatening anaphylaxis.

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Orthopedic Disorders

Individuals with cerebral palsy often are affected by musculoskeletal disorders such as hip subluxation/dislocation, scoliosis, and contractures. Abnormalities in skeletal muscle tone result in incorrect alignment of joints and bones. Hip subluxation or frank dislocation may occur in children with severe cases as early as 2-3 years of age. Careful attention must be paid during the physical examination to detect the presence of hip dislocation in order to prevent further deterioration of the hip joint. Conservative measures such as positioning and physical therapy may be effective if applied early.

Scoliosis is an abnormal curvature of the spine which may result from uneven muscle tone and spasticity in the muscles of the back and pelvis. Greater spasticity in the child/adult with cerebral palsy leads to a greater chance of earlier and more significant orthopedic problems. Continual spasticity frequently leads to permanent shortening of the muscle, or contracture. Contractures in turn result in further misalignment of the joints.

Contractures are a frequent occurrence in persons with both neurological and mobility problems. Misalignment of the joints induced by contractures may result in chronic pain for the person with cerebral palsy. Ambulatory individuals particularly may experience gait disturbances due to shortening of the hamstring. The body may be 'frozen' into awkward and uncomfortable positions which further limit mobility. A thorough examination is required to assess the presence and extent of contracture. Physical therapy may be helpful in the initial stages to maintain muscle length. Treatment is best aimed at prevention; however, often contractures do form and may require surgical intervention.

Persons with developmental disability also may be more prone to fractures due to the de-mineralization of bone induced by immobility, as well as potential propensity for falls. Aggressive or impulsive behaviors may also lead to injury in individuals with behavioral disturbances.

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Psychiatric Disorders

It is estimated that persons experiencing developmental disability may be 3-4 times more likely to experience psychiatric disorders. Affective disorders, such as depression and ADHD, are the most common. Psychotic disorders such as Schizophrenia are not commonly found in this population. An accurate diagnosis will require the practitioner to consider relevant input from the family - as well as the primary care physician.

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Pulmonary Complications

Children with cerebral palsy often are affected by asthma, as well as an increase in primary respiratory disorders. In addition, premature infants frequently develop respiratory distress syndrome (RDS) at birth related to immaturity of the lungs. Occasionally a child will continue to need supplemental oxygen for an extended time. Winter months may be particularly stressful for the child with asthma due to increased exposure to viral pathogens. Respiratory viral infections should be monitored closely to determine the need for clinical support. Children with developmental disability may also experience decreased pulmonary reserve as a result of obesity and/or chest wall or spinal deformity.

Upper airway obstruction is a potentially life-threatening condition which must be guarded against in the individual with symptoms of oral-motor dysfunction. Difficulty swallowing, (dysphagia) tonal abnormalities of the lips and tongue, regurgitation, and hyper-reflexive movements are factors which increase the likelihood of an airway obstruction occurring during feeding or eating. Additionally, general weakness and incoordination of the muscles involved in chewing and swallowing often cause problems with bolus formation that may result in aspiration. In severe cases where there is substantial risk of choking, alternate feeding routes may be explored.

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Seizure Disorders

There is a greater occurrence of seizure disorders in children with cerebral palsy. Seizure activity refers to chaotic electrical activity in the brain. This activity usually manifests itself through involuntary movements and/or alterations in sensation or consciousness. Seizures are categorized by their degree of involvement of the brain and observed clinical phenomena, such as loss of consciousness. Seizures may be demonstrated by sensory phenomena such as altered smell. Autonomic symptoms may also be present such as sweating, pallor, dilated pupils, or flushing. Hallucinations and altered sense of time have also been reported.

Seizure activity often accompanies fever in all children. Thus, not all seizure activity requires medical intervention. Pharmacological management should remain as conservative as possible with the lowest therapeutic dose used.

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Spasticity

Approximately 80 percent of all individuals with cerebral palsy will have some degree of spasticity. Persons with spina bifida may also experience spasticity, depending on the presence of paralysis. Spasticity refers to the muscles' resistance to passive stretch. The muscles of an individual with cerebral palsy exhibit a lowered threshold in the stretch reflex. Spasticity is also a primary indicator of upper motor neuron involvement. Persons with spastic cerebral palsy experience increased muscle tone at rest. Spasticity is often accompanied by other symptoms, such as ataxia, (poor balance) incoordination, involuntary movements, primitive reflexes, paralysis, weakness, and fatigue. The rigidity of muscle fibers is problematic in that it may induce gait disturbances, oral-motor dysfunction, scoliosis, contractures, and hip dislocation, as well as chronic pain for the individual with spastic cerebral palsy.

It is important when examining the individual with neurological disability to assess the degree of muscle spasticity, as well as any associated symptoms. Physical therapy, medication, and surgery are potential treatment modalities which may be used to increase functional abilities and quality of life.

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Urinary Tract Disorders

Individuals with developmental disabilities such as cerebral palsy and spina bifida are at increased risk for the development of several urinary problems secondary to deficits in neurological function. Alterations in both sensation and neuromuscular control of voiding often result in symptoms such as urinary frequency, urgency, incontinence, or urinary retention. Overactive contraction of the bladder (hyperreflexia) is often present, causing frequent emptying and resulting small bladder size. Urinary retention is problematic due to increased risk of infection. Inability to empty a full bladder allows residual urine to develop overgrowth of bacteria. In some persons with cerebral palsy, detrusor muscle contraction by the bladder occurs without urinary sphincter relaxation. When this occurs, urine from the bladder may be forced backward into the ureters and/or kidneys. This phenomenon is known as vesicoureteral reflux (VUR) and may result in dilated ureters and/or kidneys, as well as infection of the kidney itself (pylelonephritis). Although VUR is not common in children with cerebral palsy, caution is warranted when assessing urinary function.

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Sexuality and Health

Historically, sexuality is a topic which has been sorely neglected for persons with disabilities, particularly developmental disabilities. Children with developmental disabilities will experience the hormonal effects of puberty just as their unaffected peers do, but often with added emotional anxiety. It is very important that all children approaching puberty receive proper information concerning their sexual health, sexual activity, and responsibility. Parents are often reticent to provide sexual education to their children for various reasons. Parents of children with developmental disabilities often fail to recognize their child as a viable sexual being. Additionally, the extended caregiving relationship required by some children with cerebral palsy may cause parents to assume lack of maturation in this regard. It is important to discuss sexual changes both with parents and children to address concerns. Girls reaching menarche should receive regular gynecological exams and may require contraceptives to help regulate their menstrual cycle.

Adolescents with cerebral palsy and other developmental disabilities may experience feelings of frustration related to their concept of body image, peer review, or degree of independence. Although these feelings may frequently be experienced by all teens, the individual with cerebral palsy often has a multitude of additional issues to confront. Limitations in mobility, problems with continence, excessive drooling, and/or physical effects of spasticity may work to interfere with the development of self-esteem, which is imperative at this stage. It is important to listen to concerns voiced by the individual and offer support. Counseling should be suggested for those experiencing undue anxiety or symptoms of depression.

Many adults with developmental disabilities may never have explored their own sexuality. Overprotection by parents and/or caregivers may be one cause. Failure of others to see the individual with significant disability as having sexual needs is another. Sexuality should be openly discussed with the individual and encouraged as a healthy part of human life.

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References

Bakheit, A.M.O., Bower, E., Cosgrove, A., Fox, M., Morton, R., Phillips, S., et.al. (2001). Opinion statement on the minimum acceptable standards of healthcare in cerebral palsy. Disability and Rehabilitation, 23(13), 578-582

Beckung, E., & Hagberg, G. (2002). Neuroimpairments, activity limitations, and participation restrictions in children with cerebral palsy. Developmental Medicine and Child Neurology, 44, 309-316

Chance, R.S. (2002). To love and be loved: Sexuality and people with physical disabilities. Journal of Psychology and Theology, 30(3), 195-208

Chong, K.F. (2001).Gastrointestinal problems in the handicapped child. Current Opinions in Pediatrics, 13(5), 441-446

Day, H. (2004). Preventing bone fractures in immobile children. Pediatric Nursing, 16(2), 1-5.

Delfico, A.J., Dormans, J.P., Craythorne, C.B., & Templeton, J.J. (1997). Intraoperative anaphylaxis due to allergy to latex in children who have cerebral palsy: A report of six cases. Developmental Medicine and Child Neurology, 39, 194-197

DiGiulio, G. (2003). Sexuality and people living with physical or developmental disabilities: A review of key issues. The Canadian Journal of Human Sexuality, 12(1), 53-68

Fitzpatrick, L.A. (2004). Pathophysiology of bone loss in patients receiving anticonvulsant therapy. Epilepsy and Behavior, 5(2), S3-S15

Graham, H.K. (2002). Painful hip dislocation in cerebral palsy. The Lancet, 359, 907-908

Henderson, R.C. (2004). Predicting low bone density in children with cerebral palsy. Developmental Medicine & Child Neurology, 46(6), 416-419

Hockstein, N.G., Samadi, D.S., Gendron, K., & Handler, S.D. (2004). Sialorrhea: A management challenge. American family physician, 69(11), 2628-2635

Houlihan, C.M., O'Donnell, M., Conaway, M., & Stevenson, R.D. (2004). Bodily pain and health-related quality of life in children with cerebral palsy. Developmental Medicine and Child Neurology, 46, 305-310

Isaacs, D., Kilham, H.A., Somerville, H.M., O'Loughlin, E.V., & Tobin, B. (2004). Ethics Forum-Nutrition in Cerebral Palsy. Journal of Pediatrics and Child Health, 40(5-6), 308-312

Jenson, M.P., Engel, J.M., Hoffman, A.J., & Schartz, L. (2004). Natural history of chronic pain treatment in adults with cerebral palsy. American Journal of Physical Medicine and Rehabilitation, 83(6), 439-435

Koman, L.A., Smith, B.P., & Shilt, J.S. (2004). Cerebral palsy. The Lancet, 363, 1619-1631

Nickel, R.E., and Desch, L.W. (2000). The physician's guide to caring for children with chronic disabling conditions. Baltimore, MD: Paul Brookes Publishing

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