Intellectual Disability Treatment & Management

Updated: Nov 16, 2021
  • Author: Ari S Zeldin, MD, FAAP, FAAN; Chief Editor: Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA  more...
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Treatment

Approach Considerations

Early identification of children with developmental delays is necessary to begin receiving early intervention services for children from birth to 3 years of age and early childhood education services for children aged 3–5 years, which are known to improve outcomes.

The mainstay of treatment of intellectual disablity (ID) is developing a comprehensive management plan for the condition. The complex habilitation plan for the individual requires input from care providers from multiple disciplines, including special educators, language therapists, behavioral therapists, occupational therapists, and community services that provide social support and respite care for families affected by ID.

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Medical Care

Preventive care

Unfortunately, routine preventive care for children and adults with ID is lacking. Adaptive equipment (eg, for nonambulatory patients) and extra time (eg, double time slots) may be required to accommodate such patients. In addition, family members or other support persons may be helpful. Written plans (such as the Massachusetts Department of Developmental Services Annual Health Screening Recommendations and Health Record) are helpful for interdisciplinary team communication.

Lifestyle

Physical activity and obesity are major contributors to disease in ID. Very few programs exist that target healthy lifestyles (nutrition/diet, exercise, self-care, stress reduction) in those with ID. Annual counseling and referral on these issues to community agencies and programs is recommended. [29] Medications (eg, antipsychotics) should be titrated to reduce the risk of obesity and metabolic issues.

Pain

Manifestations of pain in people with severe to profound ID include crying, screaming, grimacing, protective postures (eg, arching, fetal position), rocking, and aggression. Parent/caregiver input is key to interpretation of these behaviors, though validated tools have been used as adjuncts (such as the Pediatric Pain Profile).

Common causes of acute pain include dental caries/abscesses, GERD, constipation, UTI, spasticity (when ID is associated with cerebral palsy), pressure sores, and fractures.

In addition, neuropathic pain due to dysautonomia or motor spasms may create chronic disturbances. Treatment should be prompt and include NSAIDs or acetaminophen for mild pain, tramadol or equivalent for moderate pain, and opioids for severe pain as indicated, and management of sources of pain. Some suggest use of gabapentin for neuropathic pain if no sources are identified and there is a history of surgery, symptoms suggesting visceral hyperalgesia (eg, associated with feedings or bowel movements), or symptoms of autonomic dysfunction and spasticity.

Communication

Written, verbal and pictoral forms of communication as well as gestures and demonstrations are helpful for those with ID to ensure mutual understanding and improve treatment adherence.

Sedation/anesthesia

Patients with ID requiring anesthesia may have different reactions than the general population, such as paradoxical reactions to benzodiazepines, and care should be taken to use the lowest dose and titrate slowly.

Sexuality/abuse

A significantly higher proportion of children and adults with ID have experienced some form of abuse, with some estimates of up to 70%, which contributes to mental health issues. This should be addressed at each medical visit and especially in the setting of changes in behaviors, such as increased aggression.

Cognition

No treatments are available specifically for cognitive deficiency. Although the pharmacologic enhancement of cognition (eg, use of donepezil in patients with Down syndrome [30] ) is an area of interest, research on such nootropic (ie, knowledge-enhancing) compounds is limited. Such drugs have not become part of the routine or even experimental clinical management of this population.

Other concerns

See the list below:

  • Individuals in the United States older than 18 years are no longer under the guardianship of their biological parents. No exceptions are made for children with ID. Most of these individuals, particularly those in the range of mild ID, are capable of making appropriate legal and medical decisions when adequately and appropriately informed of the decision outcomes.

  • Physicians have the duty to ascertain whether patients with ID have the capacity to consent for medical treatments. This may be challenging and outside information and supports (eg, family, caregivers, social workers) may be required to confirm the patients' understanding of the risks, benefits and alternatives to the procedure.

  • Some individuals may not be capable of comprehending the implications of the medical or legal matter at hand. In such cases, the decision is best made by a member of the biological family. The family member may obtain guardianship status for power of attorney over these matters. If a family member is unavailable to serve as guardian, then a guardian ad litum can be assigned by the court for assistance in such legal and medical matters. If a patient with ID does not have the capacity to consent, then the patient's assent should be sought if possible.

  • Subsequent to the long history of forced sterilization of girls/women with ID, varied federal, state, and local laws regulate sterilization of individuals with ID. The American College of Obstetrician/Gynecologists provides guidance on informed consent for sterilization procedures in patients with ID. [31]

  • Complex decisions, particularly those involving end of life, are perhaps best handled with the assistance of the ethics committee of the involved medical institution.

  • Failure to identify a genetic cause of ID with risks to other family members or risks to the patient for future medical complications are potential medical/legal pitfalls.

  • Perhaps 1 in 8 convicts on death row in the United States has ID. Many persons cannot fully comprehend the Miranda Rights and other critical concepts necessary to maneuver through the criminal justice system.

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Consultations

Consultations with the following may be helpful:

  • Developmental pediatrician or psychologist

  • Child neurologist

  • Geneticist and counselor

  • Psychiatrist

  • Dentist

  • Podiatrist

  • Special education/educational therapist

  • Occupational, speech and/or physical therapist

  • Behaviorist

  • Pharmacist

  • Durable medical equipment providers

  • Social services agencies/social workers

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Activity

Because obesity is more prevalent in those with intellectual disability (ID), regular physical activity should be included in the management plan. [32]  Adaptive exercise programs for those with concomitant physical disabilities should be recommended as needed. [29]

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Long-Term Monitoring

Children with intellectual disability (ID) should be evaluated regulalry by a neurologist or neurodevelopmental pediatrician with a special interest in the etiology and management of cognitive disorders. The physician should have adequate knowledge of the educational, social, and support services available in the community; assessing the appropriateness of the patient's individualized habilitation is important.

To maximize the individual's functional independence, the following areas should be addressed by the physician at least annually:

  • Treatment of associated impairments

  • Pharmacotherapy

  • Behavior management

  • Educational services

  • Recreational needs

  • Family counseling

The annual visit requires routine preventive medicine and coordination of specialized services such as dental and gynecologic care under sedation. Supplemental vaccines, including the influenza and hepatitis B vaccines, are particularly prudent for those in residential placements. A careful behavioral history is important to identify newly emerging maladaptive behaviors that may be treated effectively with behavior management. Examples of health care guidelines for individuals with ID include the Massachusetts Department of Mental Retardation checklist. [33]

If patients have coexisting motor impairments, the physician should monitor for secondary orthopedic disease. Advanced knowledge in the pharmacologic management of spasticity and rigidity allows the clinician to refer the patient for botulinum toxin injections or baclofen pump insertion when appropriate. Arthroplasty for progressive hip dislocation and/or tendon releases for progressive contractures due to spasticity may be required.

The health maintenance schedule for individuals with Down syndrome is well recognized. The American Academy of Pediatrics and American Academy of Family Practice have provided practice guidelines on the preventive care of children and adults with Down syndrome. [34]  Ongoing vision and audiologic evaluation, thyroid function tests, and screening for atlantoaxial instability and obstructive sleep apnea are some important components.

The American Academy of Pediatrics has recently provided practice guidelines for the health maintenance of children with Prader-Willi syndrome. [35]

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