. 2018 Apr;64(4):254–279.

Table 3.

Canadian consensus guidelines on the primary care of adults with IDD: Recommendations withare strongly recommended; thesymbol indicates clinicians’ top recommendations;orindicates that reviewed publications pertain to the guideline background; blue indicates a stronger or especially relevant knowledge subtype for the recommendation compared with gray (see Tables 1 and 2).

BACKGROUND	RECOMMENDATION	TYPES OF KNOWLEDGE
Approaches to care
1. *A person-centred approach to care* of adults with IDD can positively affect their health and well-being.¹² In person-centred care relationships, the person is at the centre of communication, planning, and decisions regarding care. This might require additional time and supports to meet individual needs	1.1 Engage patients and their caregivers to find effective ways of collaborating¹³	Expert Experiential
	1.2 Identify with them someone who knows the patient well who will attend health care appointments, help to coordinate care, and monitor ongoing health and social needs^13,14	Expert Experiential
	1.3 Provide sufficient time and supports to ensure that individuals can make their specific health concerns, needs, and perspectives understood^14–16	Expert Experiential
	1.4 Discuss with patients and their caregivers their goals of care and values to guide health care decisions¹⁷	Expert
2. *Effective communication* is considered essential to good health care by people with IDD and their health care providers.^18–20 Adults with IDD, however, might have challenges in communication (eg, language comprehension, expression or social interaction)^21,22	2.1 Address patients directly¹³	Expert Experiential
	2.2 Find ways of engaging the patient. Attend to both verbal and nonverbal cues²³	Expert Experiential
	2.3 Use the patient’s preferred communication method and tools (eg, Easyhealth easy-read leaflets) to facilitate communication^18,24–26	Expert Experiential
	2.4 Slow down in communicating^13,27,28 (see Communicating Effectively tool²⁷)	Expert Experiential
	2.5 Involve caregivers familiar with the patient to help communicate, but be attentive to inappropriate taking over of decision making²⁸	Expert Experiential
	2.6 Try to have a short period alone with the patient to address safety or other concerns²⁹	Expert Experiential
3. *Capacity for decision making* is relational. Many patients with IDD can participate to some extent in decision making if provided with accommodations and supports by health professionals and family and other committed caregivers (eg, for communication [see guideline 2]³⁰ or deliberation. Decision-making capacity varies with the complexity of a decision. It can be affected by factors such as the patient’s limited or adverse previous health care experiences, level of distress, and mental health conditions. These factors can be difficult to recognize in people with IDD but can often be addressed when identified (see guidelines 9 and 28)^17,31	3.1 Assess decision-making capacity of adults with IDD using a tool that is adapted to them and considers their need for accommodations and supports (eg, the Decision-Making Checklist³²). Screen for factors that can affect decision-making capacity, such as those listed in the background, and address with interventions or supports. When uncertain, refer to a psychologist or other professional who is familiar with assessing people with IDD or similar needs^31,33	Expert Experiential
	3.2 In assessing decision-making capacity and supporting a patient’s decision making, adapt communication to the patient and involve family and other caregivers familiar with the patient, as in guideline 2²⁷	Expert Experiential
Deliberating on interventions that both meet the health needs of the patient and reflect the patient’s preferences and intentions is the goal of person-centred decision making. Patients with IDD might need support to deliberate. Patients who do not meet the legal requirements for giving or refusing consent have life histories and are able sometimes to provide signs of their preferences and intentions to trusted family and other caregivers that can inform deliberations. Substitute decision makers should be guided by knowledge of this person gained from those closest to her or him^17,31	3.3 Engage in a shared decision making process with patients and their caregivers. When the legal substitute decision maker does not know the patient well, propose consulting trusted family and other caregivers to inform deliberations^31,33	Expert Experiential
4. *Families and other caregivers* often experience considerable mental, physical, or economic stress in balancing the person with IDD’s support needs with other responsibilities^34,35	4.1 Regularly screen for and proactively attend to the support needs of caregivers.^36,37 Recommend interventions that reduce BTC in people with IDD (eg, positive behaviour support^38,39) and increase coping and reduce stress experienced by caregivers (eg, mindfulness^40,41)	Empirical Ecosystem Expert Experiential
An increase or change in needs or a negative life event can lead to a crisis situation.^42–44 Families’ need for respite has been increasing⁴⁵	4.2 When concerns arise, assess and monitor family or caregiver stress (eg, through the Brief Family Distress Scale⁴⁶) and advocate for respite or additional supports⁴⁷	Empirical Ecosystem Expert Experiential
5. *Interprofessional health care teams* can assess and address holistically a range of health and developmental needs⁴⁸ and, with sufficient supports, can improve outcomes of care.⁴⁹ Such teams should be integrated (eg, the Patient’s Medical Home model)^8,14	5.1 Engage in or support developing an integrated health care team of professionals, preferably ones who are familiar with adults with IDD (eg, medical specialists, pharmacists, audiologists, speech and language pathologists, dentists, dietitians, occupational therapists, physiotherapists, psychologists, behaviour therapists, and those providing community supports)^14,50,51	Empirical Expert
	5.2 Designate someone to lead, coordinate, and integrate team input⁴⁸	Empirical Expert
Assessments and considerations important for all care
6. *Health assessments* that attend to specific health issues of people with IDD increase preventive care actions and disease detection^52,53	6.1 Perform a periodic comprehensive health assessment using guidelines for primary care of people with IDD and adapted tools (eg, the Preventive Care Checklist Form).^10,11 Include a physical health examination, mental health assessment, and review of the adequacy of financial and other community supports^14,51	Empirical Expert
Success in implementing health assessments depends on making changes to the context and organization of primary care practices^54,55	6.2 Monitor and seek ways of improving rates and outcomes of comprehensive health assessments of patients with IDD in your practice (eg, by proactively inviting patients, training staff, and aligning such assessments with guidelines for primary care of people with IDD)^10,11,55,56	Empirical Expert
A health action plan helps to set goals, clarify responsibilities for health care, and facilitate partnerships among patients, caregivers, and members of the interprofessional health care team²⁹	6.3 Based on health assessments, develop a health action plan that identifies health and related issues in an order of priority and with timelines acceptable to patients and their caregivers. Give a copy to the patient and caregiver⁵¹	Expert
7. *The cause* of IDD is important to establish because it can inform preventive care, support, and treatment decisions^57–59	7.1 Determine whether an assessment of cause has ever been done. If cause was established, consult available relevant management guidelines (eg, Health Watch Tables for specific syndromes^57,60,61)	Expert
Advances surrounding genetic and environmental causes of IDD continue to increase the proportion of disorders with known origin. Clinical geneticists carry out assessment of causes and offer genetic counseling^62,63	7.2 If cause is unknown, contact a genetics centre for advice on referring patients for an assessment or reassessment^62,64–66	Expert
8. *Cognitive ability and adaptive functioning* are assessed for a diagnosis of IDD and can vary throughout the lifespan^67–70	8.1 If there is a concern about cognitive ability or adaptive functioning and there has been no assessment during adolescence or adulthood, refer to a psychologist or, for adaptive functioning, to an occupational therapist with the expertise to assess^67,68,70	Expert
	8.2 Use tools such as the Adaptive Functioning and Communication Tool⁷¹ as a guide if specialist assessments are unavailable or delayed	Expert
Changes in cognitive ability and adaptive functioning might affect eligibility for support services and can also account for behavioural changes^67,69	8.3 Reassess cognitive and adaptive functioning to inform planning for expected service transitions (see guideline 12) or whenever a substantial change in behaviour occurs^67,69	Empirical Expert
9. *Pain and distress* can manifest atypically in patients with limited communication and can be difficult to recognize. Nonspecific changes in vital signs, appearance, and behaviour, including being less responsive and more withdrawn or new onset of BTC (see guideline 27), might be the only indicators of pain and distress^72,73	9.1 With caregiver input and adapted tools, such as the Chronic Pain Scale for Nonverbal Adults with Intellectual Disabilities^74,75 or the Non-Communicating Adult Pain Checklist,^76,77 assess for pain and its intensity	Empirical Expert
Common sources of pain include injury, dental caries, GERD, arthritis, constipation, and dysuria.^78–80 Distress can be a response to pain, challenges in the person’s environment (eg, sensory hypersensitivity), lack of supports, or some negative life experiences^72,73	9.2 Employ a comprehensive and systematic approach to assessing BTC that might be communicating pain or other causes of distress (see guideline 27)^14,72,81,82	Empirical Expert
10. *Polypharmacy and long-term use of certain medications* are prevalent among people with IDD.⁸³ This has been shown to cause considerable adverse drug reactions and effects that negatively affect patients’ quality of life.^84–86 The UK campaign STOMP-LD (Stopping Over Medication of People with Learning Disabilities, Autism or Both) addresses inappropriate psychotropic medication-related polypharmacy⁸⁷	10.1 Review regularly (eg, every 3 mo) the date of initiation, indications, dose, effectiveness, and adverse drug reactions or unwanted effects of all medications.⁸⁸ Involve a pharmacist to review medications whenever possible⁸⁶	Empirical Ecosystem Expert
	10.2 For potentially toxic medications, or those with drug-organ or drug-drug interactions, establish a baseline for the patient and monitor at the recommended interval for each⁸⁸	Empirical Ecosystem Expert
	10.3 Educate the person and caregivers about appropriate use of medications, interactions with over-the-counter, alternative, and as-needed medications, and to report potential adverse drug events. Provide patients and caregivers an opportunity to discuss questions about medication.^89,90 Determine the patient’s capacity and need for supports for both decision making and medication adherence.⁹¹ Simplify the medication administration routines and recommend appropriate medication administration aids (eg, dosettes, blister packs) and other needed supports	Empirical Ecosystem Expert Experiential
11. *Abuse, exploitation, and neglect* of people with IDD occur more commonly compared with the general population.^92–95 These are often perpetrated by people known to the victim.^92,94 They include emotional, verbal, physical, or sexual abuse, and financial exploitation.⁹⁶ Abuse can present as unexplained changes in physical health (eg, malnutrition⁹⁷) or mental health (eg, anxiety, depression), as well as changes in behaviour (eg, withdrawal, disruptive behaviour, inappropriate attachments, sexualized behaviour).⁹⁸ Neglect can present as a recurring pattern of inadequate care (eg, missed appointments, nonengagement and nonadherence)⁹⁹	11.1 Assess for risk factors (eg, residential living) and for possible indicators of abuse, exploitation, and neglect (see background column)⁹³	Empirical Ecosystem Expert Experiential
	11.2 Report suspected abuse, exploitation, or neglect to the appropriate authorities (eg, police or, for Ontario, via ReportON)^100,101	Empirical Ecosystem Expert Experiential
	11.3 Refer all victims of abuse, exploitation, or neglect to appropriate local resources and services for support,⁹⁹ especially those with expertise in working with people with IDD	Empirical Ecosystem Expert Experiential
12. *Life transitions*, such as to adolescence, adulthood, frailty (which can have an early onset), and end of life, are periods of change that are among the most challenging for people with IDD and their caregivers. These are times that require different or greater supports^44,102–104	12.1 Proactively discuss the effects of anticipated transitions with patients, their caregivers, and other members of the health care team^44,105,106	Empirical Expert Experiential
Changing expectations and roles during transitions can result in emotional, behavioural, social, and spiritual distress due to changes in and loss of previous relationships and supports¹⁰³ and limited ability to cope with such causes of distress¹⁰⁷	12.2 Proactively engage psychosocial and spiritual supports (eg, meditation, participation in a support group or faith community) to enhance resilience and coping skills of patients. Refer to a behaviour therapist or psychologist for interventions like social skills training (see guideline 26).^108,109 Promote friendships and positive social networks¹¹⁰	Empirical Expert Experiential
Effective communication and planning contribute to successful transfers of health care during life transitions, such as change of health care providers, systems, and settings of care and support	12.3 Collaborate with the individual, caregivers, and involved partners in care to develop a transition plan, comprehensive medical summary, and completed transition readiness checklist (eg, Healthcare Transition Tools and Resources¹¹¹ or Ask: a health advocacy program¹¹²). Regularly review and update the transition plan^113,114	Empirical Expert
Physical health
13. *Physical inactivity and obesity* are prevalent among people with IDD and are associated with cardiovascular disease, diabetes, constipation, osteoporosis, early mortality, and other health risks. Being underweight, with its associated health risks, is also common^115,116	13.1 Monitor weight trends regularly and assess risk status using body mass index, waist circumference, or waist-hip ratio measurement standards^117–119	Empirical Ecosystem Expert
	13.2 Counsel patients and their caregivers annually regarding targets for an optimal diet and level of physical activity using general population guidelines by age. Advise patients regarding possible changes to their daily routines to meet these targets^120,121	Empirical Ecosystem Experiential
Environmental and social factors often contribute to obesity and low physical activity rates^122,123	13.3 Address modifiable risk factors for obesity such as medications and environmental or social barriers to optimal diet and physical activity^120,121	Empirical Ecosystem Experiential
A health promotion program can improve attitudes toward physical activity and satisfaction with life,¹²⁴ but people with IDD often have difficulty finding programs that will accommodate their needs	13.4 For anyone who is not meeting diet and physical activity targets, refer to interprofessional health promotion resources (eg, dietitians, support workers, and community programs adapted for people with IDD)^125–127	Empirical Experiential
14. *Vision and hearing impairments* among people with IDD are often underdiagnosed and can result in substantial changes in behaviour and in social and adaptive functioning^128–130	14.1 Perform an annual office-based screening of vision and hearing (eg, Snellen eye chart and the whisper test) or when symptoms or signs of visual or hearing problems are noticed, including changes in behaviour or function¹³¹	Empirical Expert
	14.2 Refer for optometry assessments to detect glaucoma and cataracts every 2 y after age 40^132,133	Empirical Expert
	14.3 Refer for audiology assessments based on screening and for age-related hearing loss every 5 y after age 45^131,134	Empirical Expert
Cerumen impaction resulting in substantial hearing loss is more prevalent among people with IDD than in the general population and is a reversible cause of hearing loss¹³⁵	14.4 Screen for cerumen impaction every 6 mo and address (eg, by advising periodic use of mineral oil drops)^134,136	Empirical Expert
15. *Oral disease* (eg, periodontal disease, caries, missing teeth and dental erosions that are associated with GERD) is more prevalent among people with IDD than in the general population^137,138	15.1 When dental erosions are detected, assess for GERD^139,140	Empirical Expert
Poor oral health substantially affects nutrition and respiratory and cardiac health and can contribute to behavioural disturbances.^140,141 However, people with IDD can have difficulty maintaining oral hygiene routines and accessing dental services that accommodate their needs¹⁴²	15.2 Monitor oral health status and encourage daily oral hygiene routines, periodic professional dental care, and other preventive oral care measures¹⁴³	Empirical Expert
16. *Cardiovascular disease* is prevalent among people with IDD, especially those with Down, 22q11 deletion, and Prader-Willi syndromes.^144–146 Risk factors for cardiac disorders are increased owing to physical inactivity, smoking, obesity, and prolonged use of certain psychotropic medications^147,148	16.1 Screen for cardiovascular risk factors earlier and more regularly than in the general population and promote prevention^149,150	Empirical Ecosystem Expert
The proportion of adults with IDD who have CHF is 3 times higher than in the general population¹⁵¹; CHD, which is very common in individuals with Down syndrome, can lead to CHF^152,153	16.2 Assess annually for signs and symptoms of CHF and cardiac decompensation or when these are detected. If detected, refer the person to a cardiologist or, if the cause is CHD, to an adult CHD clinic.^154–156 Consult the Canadian Congenital Heart Alliance for clinic locations¹⁵⁷	Empirical Ecosystem Expert
17. *Respiratory disorders* (eg, aspiration pneumonia) are among the common causes of death for people with IDD.¹⁵⁸ Swallowing difficulties can increase the risk of aspiration or asphyxiation.^159-163 Such difficulties are prevalent among people with neuromuscular dysfunction (eg, cerebral palsy) or those who are taking medications with anticholinergic effects	17.1 Assess annually for overt or silent aspiration and signs of swallowing difficulty (eg, throat clearing after swallowing, coughing, choking, drooling, long meal times, aversion to food, weight loss, and frequent chest infections or asthma) or when these signs are detected¹⁶⁴	Empirical Ecosystem Expert
	17.2 Refer to a speech and language pathologist to assess swallowing function when signs and symptoms of swallowing difficulty are detected¹⁵⁹	Empirical Ecosystem Expert
Asthma is more prevalent among people with IDD than in the general population^165,166	17.3 Screen for asthma. Consider other causes of a cough or wheeze, such as aspiration¹⁶⁵	Empirical Ecosystem
	17.4 Use spirometry or pulmonary function tests to confirm the diagnosis of asthma. These might need to be adapted to the needs of some people with IDD who find these tests difficult¹⁶⁵	Empirical Ecosystem
COPD is more prevalent in adults with IDD than adults without IDD¹⁶⁵	17.5 Ask about smoking and exposure to second-hand smoke. Screen for COPD as in the general population¹⁶⁵	Ecosystem
	17.6 Screen for obstructive sleep apnea (see recommendation 25.3)	Empirical Expert
18. *Gastrointestinal problems* are common among people with IDD and are manifested by food aversions, changes in behaviour, or weight loss^167–172	18.1. Screen annually for GERD or when symptoms or signs are detected. Manage GERD. If introducing medications (eg, NSAIDs) that might aggravate GERD, monitor for onset of GERD symptoms¹⁶⁸	Empirical Ecosystem Expert
	18.2 If unexplained weight, gastrointestinal, or behavioural changes are detected, assess for common issues (eg, constipation, GERD, peptic ulcer disease, celiac disease, and pica)^{169,173–175}	Empirical Ecosystem Expert
People with IDD are at increased risk of acquiring Helicobacter pylori infection if they have ever lived in group-living settings^176,177	18.3 Test for H pylori infection in symptomatic patients or asymptomatic patients who live or have lived in a group residence.¹⁷⁸ Retest at regular intervals (eg, 3–5 y)¹⁷⁹	Empirical Expert
People with IDD frequently experience constipation related to neurologic dysfunction, lack of physical activity, a low-fibre diet, or medications with anticholinergic effects, which can lead to substantial morbidity. Pain associated with constipation might present as distress, sleep disturbance, or behavioural changes including self-harm^{169,173,180,181}	18.4 Ask about frequency and consistency of bowel movements regularly to detect constipation (eg, using the Bristol Stool Chart) and address reversible medical causes.¹⁷³	Empirical Expert
	18.5 For patients with constipation or diarrhea, use a bowel movement monitoring chart¹⁸² that patients and caregivers can complete	Empirical Expert
19. *Women’s gynecologic and reproductive health.* Menstrual disorders adversely affect the health and well-being of some women with IDD. Issues include menstrual-related pain, excessive bleeding and anemia, reduced seizure control, negative effects on hygiene, functioning, and behaviour, social isolation, and increased caregiver burden^183,184	19.1 Ask about menstrual-related symptoms and concerns regularly. Provide education regarding symptom management and options, including the use of nonhormonal interventions (eg, NSAIDs)¹⁸⁵	Empirical Expert
There is insufficient evidence currently to recommend one method of menstrual regulation or suppression over others for women with IDD^186,187	19.2 Discuss methods of menstrual regulation with women with IDD and their caregivers. In deciding together on a method, consider safety and effectiveness, the patient’s health circumstances, and the patient’s and caregiver’s views on the benefits and burdens to the patient^184,187	Expert
Medical considerations regarding fertility regulation for women with IDD are similar to those for other women. In Canada, the law prohibits parents or other substitute decision makers from consenting on behalf of persons with IDD who are incapable of giving consent to surgical sterilization for contraceptive purposes¹⁸⁸	19.3 Screen for sexual exploitation and unintentional risky or harmful sexual practices. When these are present, facilitate deliberation with the patient and her caregiver of a range of methods to reduce risk of infections and to regulate fertility	Expert
Pregnancy poses increased risk of adverse health outcomes for women with IDD. They have higher rates of obesity, epilepsy, polypharmacy, mental health issues, and poverty than similar-aged women in the general population. They are also more likely to have complications of pregnancy, such as venous thromboembolism and preeclampsia, and to undergo labour induction and cesarean section than pregnant women without IDD are^189,190	19.4 Provide women with IDD with increased monitoring during pregnancy (eg, longer and more frequent appointments)¹⁹¹	Ecosystem
	19.5 Address modifiable risk factors (eg, smoking cessation) before or as soon as possible during pregnancy¹⁸⁹	Ecosystem
	19.6 Engage local resources that can support and educate mothers with IDD and their partners as soon as possible during pregnancy and after childbirth¹⁸⁹	Ecosystem
Menopause occurs earlier among women with IDD, especially those with certain genetic disorders such as Down syndrome, than among women in the general population. Women with IDD are often unaware of symptoms associated with menopause (eg, disturbed sleep)¹⁹²	19.7 Ask perimenopausal women with IDD about menopausal symptoms at an earlier age than women without IDD¹⁹²	Experiential
Relationships, intimacy, and sexuality are important issues that are often not considered or addressed in the primary care of adolescents and adults with IDD¹⁹³	19.8 Ask male and female patients, their family, or other caregivers about the patient’s relationships, intimacy, and sexuality (eg, sexual behaviour, gender identity, sexual orientation, genetic risks).¹⁹⁴ Provide or refer for education and counseling services that are adapted to the needs of people with IDD¹⁹³	Expert
20. *Neuromuscular and skeletal disorders* (eg, scoliosis, contractures, spasticity, and ligamentous laxity) are possible sources of unrecognized pain and occur frequently among people with IDD, especially those with cerebral palsy. This can result in reduced mobility and physical activity with associated adverse health outcomes^195–197	20.1 Promote mobility and regular physical activity (see guideline 13)^198,199	Empirical Expert
	20.2 Consult a physical or occupational therapist regarding adaptations to promote mobility and physical activity (eg, wheelchair, walker, modified seating, splints, orthotics, and safety devices such as handrails)¹⁹⁸	Empirical Expert
	20.3 Actively monitor foot health and ensure properly fitting footwear in consultation with a podiatrist or orthotist²⁰⁰	Empirical Expert
Osteoporosis and osteoporotic fractures are more prevalent and occur at a younger age among people with IDD than among those in the general population^201,202	20.4 Screen both male and female patients for osteoporosis starting in early adulthood^201,203	Empirical Ecosystem Expert
	20.5 Seek advice from a radiologist regarding alternative methods to assess risk of fragility fractures if the patient cannot be assessed using the usual nuclear BMD test, such as by assessing the patient’s forearm only²⁰⁴	Ecosystem Expert
Risk factors for osteoporosis specific to people with IDD include increased severity of IDD; long-term use of certain medications such as anticonvulsants, glucocorticoids, and injectable long-acting progesterone in women; vitamin D deficiency; prolactinemia; immobility; and presence of certain genetic syndromes^202,204,205	20.6 Recommend early and adequate intake or supplementation of calcium and vitamin D unless contraindicated (eg, among people with Williams syndrome)^202,204	Expert
	20.7 Be aware of concurrent medical conditions and medications in patients with IDD when considering osteoporotic treatment options (eg, renal insufficiency or swallowing difficulty) and seek advice (eg, from an endocrinologist or pharmacist)^202,204	Expert
21. *Epilepsy* occurs in about 1 in 5 people with IDD compared with 1 in 100 people without IDD.²⁰⁶ The prevalence increases with the severity of IDD. It contributes to early mortality.²⁰⁷ It can be difficult to diagnose.^208,209 Choice of medications and importance of regular monitoring are the same as for those in the general population²⁰⁸	21.1 For people with IDD and epilepsy, make a comprehensive epilepsy health action plan involving patients, family, and other caregivers.²⁰⁸ Follow guidelines for diagnosis and management of epilepsy^208,210,211 and use tools for periodic reviews²¹²	Empirical Expert
	21.2 Review this plan at least annually. Review more frequently (eg, every 3 to 6 mo) the efficacy and safety of antiepileptic medications, including any drug reactions adversely affecting cognitive functioning or behaviour²¹⁰	Empirical Expert
22. *Endocrine disorders* can be challenging to diagnose in people with IDD. They have a higher incidence of thyroid dysfunction compared with those in the general population²¹³	22.1 Test annually for thyroid function in patients with elevated risk of thyroid dysfunction (eg, people with Down syndrome). Also test when changes in behaviour or adaptive functioning are detected^214,215	Empirical Expert
	22.2 For patients taking lithium, atypical or second-generation antipsychotics, or seizure medications, establish a thyroid function baseline followed by annual reassessment^216,217	Empirical Expert
The prevalence of diabetes is higher among people with IDD compared with those in the general population and slightly higher among women than men^218,219	22.3 Include patients with IDD among those at high risk of diabetes. Screen for type 2 diabetes at an earlier age than is recommended for the general population²¹⁸	Ecosystem Experiential
	22.4 Provide diabetes education to patients with IDD who have diabetes and their family and other caregivers that is adapted for people with IDD^220–222	Expert Experiential
23. *Infectious diseases* (eg, influenza, Streptococcus pneumoniae infection, and hepatitis B). People with IDD living in group residential settings are at higher risk of developing such infections^223–225 but have lower rates of immunization and screening compared with the general population^226,227	23.1 Include patients with IDD in routine immunization programs targeting high-risk populations for influenza and S pneumoniae infections^223,227	Empirical Ecosystem Expert Experiential
	23.2 Offer hepatitis A and B immunization to all at-risk patients, such as those who require long-term, potentially hepatotoxic medications or who live in group settings^225,228	Empirical Ecosystem Expert Experiential
People with IDD are at a greater risk of developing chronic, invasive, and life-threatening forms of infection because of factors such as increased infectious sources, compromised immunity, inability to report symptoms, and absence of fever and other signs of infection.^226,227 They are also at greater risk of not having these infections detected promptly	23.3 Screen patients for infectious disease according to guidelines for high-risk populations and other special risk factors (eg, group residence, sexual practices, IV drug use)²²⁹	Empirical Expert
	23.4 Reduce risk factors for invasive lung infections, such as by supporting safe feeding practices, positioning to enable secretion clearance, and respiratory therapy²²⁶	Expert
	23.5 If a patient manifests changes in behaviour or mental status, perform a head-to-toe examination to detect infection. Alert caregivers to signs and symptoms of infection	Expert
24. *Cancer screening* (eg, for cervical, breast, and colorectal cancer) is less likely to be received by people with IDD than by those in the general population.^230,231 Factors include lack of knowledge of family history to inform screening intervals, logistic challenges performing certain screening, lack of adapted patient education tools, and family member reluctance and fears related to the perceived excessive burdens of possible cancer treatment^232,233	24.1 Proactively obtain information on family history of cancer and review annually²³⁴	Empirical Ecosystem Expert Experiential
	24.2 Use clinical tools adapted for people with IDD to promote education and uptake of cancer screening tests²³⁵	Empirical Ecosystem Experiential
	24.3 Discuss concerns regarding cancer and symptom management with family and other caregivers and provide information regarding management and palliative care²³²	Empirical Ecosystem Experiential
People with IDD are less likely to be supported to self-monitor and report early symptoms and signs of cancer. Those who do develop cancer often have more advanced cancer at the time of detection than those in the general population do²³⁶	24.4 Perform a total-body screen for skin lesions and breast and testicular examination in adults with IDD during periodic health assessments. Use easy-to-read materials to inform people with IDD about these examinations before assessment (eg, Easyhealth easy-read leaflets).²⁶	Empirical
	24.5 Proactively instruct patients regarding self-monitoring, as is considered routine among those in the general population (eg, being breast aware, reporting gross hematuria, melena, and changes in moles)²³⁷	Empirical Expert
	24.6 Instruct family and other caregivers regarding observable signs and symptoms of cancer and, if detected, to seek prompt medical attention^237,238	Empirical Expert
25. *Sleep problems* (eg, difficulties settling, night walking, or early awakening) are common among adults with IDD and often result in increased caregiver stress²³⁹	25.1 Screen regularly for sleep problems and for associated caregiver stress.²³⁹ Review with and educate patients and their caregivers on helpful sleep hygiene practices²⁴⁰	Empirical
Sleep problems can be caused by physical health issues (eg, GERD, pain that the person with IDD might be unable to communicate,²⁴¹ or obstructive sleep apnea); environmental factors (eg, noise, electronic screen time); distressing life experiences (eg, bereavement or adjustment to change); or effects of medications (eg, certain psychotropics, antiepileptics, and antidepressants). Also, BTC and psychiatric disorders can be associated with sleep disturbance²³⁹	25.2 Review medications that might negatively affect sleep and other physical health issues, as well as environmental, life experience, and psychiatric issues that might cause sleep problems²³⁹	Empirical
	25.3 Assess for obstructive sleep apnea in patients, especially those who are obese (see guideline 13) and those with craniofacial abnormalities or certain genetic disorders (eg, Down syndrome)^241–243	Empirical Expert
Adults with IDD and sleep problems can benefit from behavioural interventions, such as sleep hygiene training or environmental modifications²³⁹	25.4 Refer to a behaviour therapist or occupational therapist familiar with people with IDD to assess or address sleep hygiene and environmental factors²⁴⁰	Empirical
Mental health
26. *Psychosocial context and mental well-being*. Mental health disturbances, common among adults with IDD, are associated with sensory impairments, negative life events,²⁴⁴ lack of suitable supports (emotional, social, community, work, and recreational),²⁴⁵ stress,²⁴⁶ and coping capacity^107,247	26.1 Screen for sensory impairments (see guideline 14), negative social circumstances, stressful life events (eg, abuse, neglect, bullying, and exclusion), and coping capacity (see guidelines 11 and 27)	Empirical Ecosystem Expert Experiential
	26.2 Promote friendships, social networks, and accommodations for inclusion and participation to decrease isolation and loneliness²⁴⁸	Empirical Ecosystem Expert
	26.3 Engage with community services and interprofessional partners or teams to enhance coping skills⁷³	Empirical Ecosystem Expert
The effects of these circumstances can best be understood by inviting and appreciating the perspectives of persons with IDD, in combination with those who support them^249–251	26.4 Get the person with IDD’s perspective on their situation whenever possible. Use visual aids as well as words (eg, Books Beyond Words)^24,110	Expert Experiential
27. *Behaviours that challenge* (eg, self-injury, aggression, outbursts of anger, and irritability) are not psychiatric disorders; BTC often communicate underlying distress, sometimes from multiple causes^72,73,252 BTC sometimes occur owing to an absence of necessary environmental accommodations, insufficient supports (eg, inappropriate expectations and environments that are stressful, unresponsive, understimulating or overstimulating, noisy, restrictive, or intrusive, or that lack privacy), and a lack of adaptations for coexisting disabilities such as cerebral palsy and hearing and vision impairments An “enabling environment” exists when an individual’s developmental needs match their supports.⁷³ BTC can be symptoms of negative life experiences (eg, bullying, disappointment, losses, grief, and trauma)^253–256 BTC might be a symptom of psychiatric illness. A systematic diagnostic formulation, such as HELP, will identify whether BTC that appear “psychiatric” might be due to one or more other causes^72,257	27.1 Develop a diagnostic formulation (eg, HELP) that considers causes sequentially and systematically, such as the following⁵⁰: H—Assess for possible physical health problems, (consult the above physical health recommendations for head-to-toe sequence of common medical concerns), pain, and adverse and other side effects of medications E—Facilitate “enabling environments” that meet these unique developmental needs and can diminish or eliminate BTC. Work with an interprofessional team and caregivers to address problematic environmental circumstances (see guidelines 1, 2, 26).⁷³ Ascertain whether existing supports match needs (see guideline 8).²⁵⁸ Plan for a functional behavioural assessment by a behavioural therapist or psychologist L—Screen for distressing life experiences that might be contributing to BTC (see guideline 12)^{72,252,259,260} P—Having attended to the above, consider psychiatric conditions (eg, adjustment difficulties, mood and anxiety concerns). Refer as needed for assessment to an interprofessional mental health team (see guideline 5)^{48,72,252,261,262}	Empirical Expert Experiential
Despite the evidence of non-benefit and concerns regarding potential harm, psychotropic medications are often used to manage BTC^263,264	27.2 Review regularly (eg, every 3 mo) the rationale and use of prescribed psychotropic medications, including those used as needed.^{263,265–268} See guideline 29 for psychotropic prescribing practices for psychiatric disorders	Ecosystem Expert
Efforts should focus on the need to adequately assess the cause of BTC rather than solely using medication to suppress them	27.3 Use psychotropic medication to manage BTC of people with IDD only as a last resort and for a short term to attend to risk of harm while ascertaining causes^72,263	Expert
For some people with IDD, additional supports might first have to be provided for successful discontinuation of medications^269–271	27.4 Consider tapering and stopping, at least on a trial basis, long-term psychotropic medications not prescribed for a specific psychiatric diagnosis.^266,268 Refer and work with an interprofessional mental health team for this purpose^266,268,271	Empirical Expert
Antipsychotic medications are often inappropriately prescribed for adults with BTC and IDD in the absence of a robust diagnosis of a psychotic disorder^269,272	27.5 Do not use antipsychotic medications as a first-line or routine treatment of BTC^268–270	Empirical Ecosystem Expert
28. *Psychiatric disorders.* People with IDD have increased vulnerability to mental stress compared with those in the general population. Developmental challenges, transitions, greater exposure to adversity and traumatic life events, limited coping skills, and insufficient supports contribute further to this vulnerability. If adjustment issues are not adequately addressed, they might give rise to adjustment disorders, which are diagnosed when a life event or trigger has contributed to the mental distress²⁶⁰	28.1 Screen for antecedents, life events, and other triggers of mental distress. Explore the importance of the event for the person and obtain collateral history²⁶⁰	Expert
	28.2 Proactively plan with the person with IDD, caregivers, and appropriate services to attend to predictable developmental challenges and stressors and to ensure that the necessary supports will be in place (see guidelines 12 and 29)²⁵⁹	Expert
	28.3 Offer or facilitate supportive counseling before developmental challenges and life stressors occur (see guideline 29)	Expert
Psychiatric disorders (eg, mood and anxiety disorders) are common among adults with IDD, but the signs and symptoms might be seen as part of the IDD (ie, diagnostic overshadowing) rather than a change indicating a psychiatric disorder. Consequently, psychiatric disorders might not be recognized and addressed^{252,273–275}	28.4 Screen for a possible psychiatric disorder by looking for changes from baseline in mental state and behaviour (see psychiatric symptoms and behaviour screening tool, page 75)²⁵⁸	Ecosystem Expert
Establishing a diagnosis of a psychiatric disorder in adults with IDD is often complicated by communication barriers or atypical presentation.^274,275 In general, anxiety, mood, trauma, and stressor-related disorders (eg, PTSD and adjustment disorder) are underdiagnosed while psychotic disorders are overdiagnosed in people with IDD^{252,276–280}	28.5 Seek interprofessional assessment from specialists in psychiatry, psychology, or speech-language pathology, preferably those with expertise in IDD. Review previous psychiatric diagnoses. Consider use of self-report and informant questionnaires developed for people with IDD (eg, Glasgow Depression Scale for People with a Learning Disability, Glasgow Anxiety Scale for People with an Intellectual Disability, Glasgow Depression Scale–Carer Supplement)^281–283	Empirical Ecosystem Expert
Some IDD syndromes are associated with increased risk of particular developmental, neurologic, or behavioural manifestations and emotional disturbances (ie, “behavioural phenotypes”)^284,285	28.6 Consult available information regarding behavioural phenotypes associated with specific syndromes (eg, autism spectrum disorder,²⁸⁶ fetal alcohol spectrum disorder,²⁸⁷ and Williams syndrome)²⁸⁸	Expert
PTSD is underdiagnosed and might manifest as anxiety, mood disturbance, or change in behaviour (eg, “noncompliance,” self-injury, aggression, outbursts of anger, irritability) and might occur in response to events not typically considered to be traumatizing (eg, siblings leaving home)^289–291	28.7 Explore for possible trauma, possibly unknown to new care providers; be alert for signs of PTSD such as reexperiencing (eg, psychotic presentations and behavioural enactments; avoidance, which might be interpreted as noncompliance; and increased arousal, which can present as irritability)²⁹¹	Expert Experiential
Psychotic disorders, which include schizophrenia and traumatic psychosis, are especially difficult to diagnose in people with IDD when their delusions and hallucinations cannot be expressed verbally.^279,280,292 Developmentally appropriate fantasies and imaginary friends might be mistaken for delusions, and self-conversation mistaken for hallucination^279,280,293	28.8 Work with an interprofessional team to help clarify diagnoses in patients with limited or atypical use of language^294,295	Expert
Sexual abuse occurring during childhood can be associated with hearing voices in adulthood^296,297	28.9 Screen for trauma²⁹¹	Expert
There is increased risk of psychosis associated with visual²⁷⁷ and hearing²⁹⁸ impairment and social isolation	28.10 Screen for and address possible hearing and vision impairments (see guideline 14)	Empirical
	28.11 Work with caregivers to ensure optimal communication^299,300 (see guideline 2) and inclusion to prevent social isolation (see guideline 11)	Empirical Expert
Emotional, psychiatric, and medical conditions can coexist and give rise to clinical and diagnostic complexity.³⁰¹ A biopsychosocial approach assists in unraveling these complexities^50,252,302	28.12 Implement a systematic and sequential approach, such as the HELP approach (guideline 27), to identify contributing causes or seek consultation from an interprofessional mental health team^276,277	Empirical Expert
29. *Mental health intervention. Psychological therapies.* When people with IDD experience emotional distress, various individual or group interventions are effective (eg, counseling, CBT, positive behaviour support, psychotherapy, trauma-informed therapies, mindfulness practices).^108,252,290 Adaptations to the needs of people with IDD and supports can improve access to these therapies	29.1 Inform patients of psychological interventions and supports available from the interprofessional team or community. Ensure that supports to access these services are provided (eg, transportation) and include the active participation of an IDD-informed support person¹⁰⁸	Expert
*Psychotropic medications* can be effective, along with other psychological therapies, when a psychiatric disorder (eg, ADHD, anxiety and mood disorder) is confirmed by comprehensive assessment.^{216,252,263,303} Psychotropic medications, however, should be used judiciously with people with IDD. Some might be taking multiple medications and thus be at increased risk of adverse drug-drug interactions (see guideline 10), have atypical responses, and have adverse reactions or unwanted effects even at low doses. Adults with IDD might be unable to describe harmful or distressing effects other than through changes in their behaviour^{264,304–307}	29.2 When a psychiatric diagnosis is confirmed by comprehensive assessment, consider indicated, disorder-specific psychotropic medication along with other possible interventions as outlined below^252,263	Empirical Ecosystem Expert
	29.3 Consult prescribing guidelines for psychiatric disorders in IDD^88,263,267	Empirical Ecosystem Expert
	29.4 Refer to medication algorithms for specific diagnostic categories (eg, ADHD, anxiety, mood)^216,303	Empirical Ecosystem Expert
	29.5 Start low and go slow in initiating, increasing, or decreasing doses of medications^88,305	Empirical Ecosystem Expert
	29.6 Seek and document informed consent using adapted tools, such as the Informed Consent Checklist³² (see guideline 3)⁸⁸	Empirical Ecosystem Expert
	29.7 Educate and arrange to receive feedback regularly from patients and their caregivers during medication trials^252,303	Empirical Ecosystem Expert
*Antipsychotic medications* are commonly prescribed to adults with IDD despite the absence of a diagnosis of a psychotic or mood disorder.^83,264 Adverse drug reactions and unwanted effects can be considerable. Antipsychotic medications increase the risk of CNS effects (eg, sedation, behavioural disturbance), extrapyramidal symptoms (eg, parkinsonism, akathisia, tardive dyskinesia), anticholinergic effects (eg, swallowing difficulties, bowel dysfunction), cardiovascular effects (eg, orthostatic hypotension, tachycardia), and endocrine effects (eg, metabolic syndrome, sexual dysfunction), as well as longer-term adverse effects^267,308	29.8 Review response, adverse reactions, or unwanted effects every 3 mo (see guideline 10). Review the psychiatric diagnosis and appropriateness of prescribed medications whenever there is nonresponse, worsening of symptoms, behavioural change, or patient or caregiver request^88,263,306	Empirical Ecosystem Expert
	29.9 Monitor and address adverse drug reactions and unwanted effects of antipsychotic medications, such as those listed in the background column ⁸⁸	Empirical Ecosystem Expert
	29.10 If adverse drug reactions or unwanted effects are present, consider dose reduction, tapering, or switching medications^263,308	Empirical Ecosystem Expert
*Other therapeutic interventions* include environmental modification with attention to sensory hypersensitivities and hyposensitivities; education and skill development; communication aids; behaviour support; art, drama, and music therapies; healthy lifestyle and physical activity interventions; and caregiver support, training, and stress-reduction practices^124,126,252	29.11 Educate patients and caregivers to incorporate a healthy diet, physical activity and good sleep habits into their lifestyles⁸⁸	Empirical Expert
	29.12 Advocate for and consider referral to professionals, agencies, and community programs that can be of therapeutic benefit, especially those that can be adapted to the needs of people with IDD²⁵²	Empirical Expert
30. *Behavioural crises* arise from various circumstances and in response to stressors. When behaviour cannot be managed safely in the current environment, a situation can escalate to a crisis level and require assessment and management in an ED	30.1 Psychotropic medications can be used to ensure safety during a behavioural crisis and might need to be continued during assessment of the underlying causes. This use should be temporary and ideally stopped after 72 h³⁰⁹	Expert
Some people with IDD who have visited EDs for behavioural crises do not have any follow-up with a primary care provider or psychiatrist within 30 d of discharge.³¹⁰ Follow-up is crucial for continuity of care, prevention of recurrence, and the need to review any medication prescribed to manage the crisis³¹¹	30.2 Debrief with the patient and caregivers as soon as possible (eg, within 72 h) after an ED visit in order to minimize the likelihood of recurrence. Include a review of crisis events and responses (eg, de-escalation measures, medications) and identify the possible triggers and underlying causes of the behavioural crisis^268,311	Ecosystem Expert
Hospital staff, people with IDD, and caregivers consider crisis plans helpful in managing crises.^312,313 People with IDD with primary care involvement and crisis plans are less likely to visit an ED when a crisis arises³¹⁴	30.3 If the patient is at risk of recurrent behavioural crises, involve local ED staff and other stakeholders to develop a proactive, integrated crisis plan (eg, Crisis Prevention and Management Plan).³¹⁵ This should be available in the ED and updated regularly³¹⁶	Empirical
31. *Addictions* are underrecognized among adults with IDD.³¹⁷ High risk of addiction is associated with mild IDD, persons who live independently, males, those with psychiatric disorders, and those with legal issues.³¹⁸ The most commonly used substance is alcohol,^319,320 followed by cannabis.³²⁰ Lower amounts of alcohol consumed can lead to functional impairments in those with IDD relative to the general population^318,319	31.1 Screen for risk and vulnerability factors for addictions as part of a comprehensive health assessment³²¹	Empirical Ecosystem Expert
	31.2 Educate adults with IDD, family, and other caregivers about risks and vulnerabilities, including peer pressure and exploitation³²²	Empirical Ecosystem Expert
	31.3 When addiction is present, review the environment, social relationships, and supports. Understanding how aware the person is of the problems and associated risks can assist in developing appropriate interventions^322,323	Empirical Ecosystem Expert
	31.4 Work with the person, their family, and other caregivers using a harm-reduction approach, including planning regular office visits³¹⁸	Empirical Ecosystem Expert
There are few addiction-related services adapted to the needs of people with IDD.³²⁴ They are very likely to have comorbid psychiatric diagnoses and a range of physical health issues that require a multifaceted approach to management^317,318	31.5 Advocate for adaptations to and arrange additional supports for people with IDD in order to benefit from generic addiction-related services designed for people without IDD.^321,325 Promote opportunities for meaningful socialization that do not involve exposure to addictive substances	Ecosystem Expert
32. *Dementia* (major neurocognitive disorder⁶⁸) is more prevalent among adults with IDD compared with the general population (age of onset 60–65 y), with a statistically significantly increased risk in adults with Down syndrome and at an earlier age (50–55 y)^326,327	32.1 Educate caregivers to recognize and report possible early signs of dementia, such as new onset of forgetfulness, incontinence, loss of personal skills, and changes in sleep patterns, personality, and behaviour^328,329	Empirical Expert
Diagnosis might be missed because changes in emotion, social behaviour, or motivation can be gradual and subtle. A baseline estimate of functioning against which to measure change is needed³²⁶	32.2 When signs of dementia are present, investigate for potential reversible causes including infections, thyroid disorder, cardiovascular disease, hearing and visual impairments, nutritional deficiencies, or medication effects³³⁰	Expert
	32.3 Refer individuals who are known to be at risk of dementia to a psychologist for a baseline assessment of cognitive, adaptive, and communicative functioning after age 40 y and at age 30 y for people with Down syndrome³²⁶	Expert
Differentiating dementia from depression and delirium can be especially challenging in adults with IDD³³¹	32.4 Monitor with tools designed for people with IDD and dementia (eg, NTG–Early Detection Screen for Dementia)^329,332,333	Empirical Expert
	32.5 Apply the differential diagnosis criteria for dementia and delirium as in the general population using a systematic and sequential approach, such as the HELP framework (see guideline 27), to review signs and symptoms of concern³³⁰	Expert
	32.6 After addressing any medical findings and precipitating life events, consider the possibility of depression and a trial of an antidepressant medication to resolve symptoms and support the diagnosis of depression³²⁷	Expert
	32.7 Refer to the appropriate specialist (eg, IDD mental health team, geriatric service, or neurologist) if it remains uncertain whether symptoms and behaviour are due to emotional disturbance, psychiatric disorder, or dementia³²⁶	Expert

ADHD—attention deficit hyperactivity disorder, BMD—bone mineral density, BTC—behaviours that challenge, CBT—cognitive-behavioural therapy, CHD—congenital heart disease, CHF—congestive heart failure, CNS—central nervous system, COPD—chronic obstructive pulmonary disease, ED—emergency department, GERD—gastroesophageal reflux disease, IDD—intellectual and developmental disabilities, IV—intravenous, NSAID—nonsteroidal anti-inflammatory drug, NTG—National Task Group, PTSD—posttraumatic stress disorder.