As noted by The Gupta Guide* researchers recommend a nine-step approach for practitioners when assessing health and function in adults with intellectual disability suspected of having MCI or dementia. These include:
• Taking thorough history, with particular attention to "red flags" that potentially indicate premature dementia such as history of cerebrovascular disease or head injury, sleep disorders, or vitamin B12 deficiency
• Documenting a historical baseline of function from family members of caregivers
• Comparing current functional level with baseline
• Noting dysfunctions that are common with age and also with possible emerging dementia
• Reviewing medications and noting those that could impair cognition
• Obtaining family history, with particular attention to a history of dementia in first-degree relative
• Noting other destabilizing influences in patient's life such as leaving family, death of a loved one, or constant turnover of caregivers, which could trigger mood disorders
• Reviewing the level of patient safety gleaned from social history, living environment, and outside support
• Continually cross-referencing the information with the criteria for a dementia diagnosis
* Sunjay Gupta, The Gupta Guide, Aug 21, 2013, citing work done by Moran et. al.,"The national task group on intellectual disabilities and dementia practices consensus recommendations for the evaluation and management of dementia in adults with intellectual disabilities" Mayo Clin Proc 2013; 88(8): 831-840.
What are some first steps?
Complete a function and behavior reporting form, like the NTG-EDSD
Talk over with others to gain consensus on observations and impressions
Arrange for a clinical assessment by a nurse, psychologist, or medical doctor
Organize an diagnostic appointment with a clinic or practitioner, preferably one knowledgeable about dementia and intellectual disability
Be prepared to discuss the NTG-EDSD information with the assessor
Cognitive Assessments and Care Planning
CMS Cognitive Assessment and Care Plan Services
Examining adults with intellectual disability as part of the Annual Wellness Visit or other assessment opportunities is often difficult for medical personnel who may be unfamiliar with intellectual disability or the adult who has an intellectual disability. This difficulty may impede an accurate assessment of current functioning and ascribing a diagnosis of a neuropathology related to a disease or condition resulting in dementia. Differentiating presenting behavior due to cognitive decline from pre-existing cognitive limitations is often difficult absent 'personal best' functioning data or a recent history information about changes in function and behavior. This may be problematic for primary care practitioners or other clinicians being asked to undertake a cognitive impairment assessment during the Annual Wellness Visit or other clinical visit.
To provide more guidance for clinicians who may be undertaking a cognitive impairment assessment with the population-at-large, CMS issued new guidance for cognitive assessment and care plan services. The guidance notes that these assessments can help detect cognitive impairment as part of a routine visit through direct observation or by considering information from the "patient, family, friends, caregivers, and others." CMS suggests also that clinicians may also use a brief cognitive test and evaluate health disparities, chronic conditions, and other factors that contribute to increased risk of cognitive impairment. CMS notes that if the clinician detects cognitive impairment at an AWV or other routine visit, he or she may perform a more detailed cognitive assessment that should evolve into a care plan. Such an additional evaluation is necessary to diagnose a person with dementia, stemming from Alzheimer’s disease, and to identify treatable causes or co-occurring conditions such as depression or anxiety.
The following extract from the CMS guidance identifies "What’s Included in a Cognitive Assessment? "
The cognitive assessment includes a detailed history and patient exam. There must be an independent historian for assessments and corresponding care plans provided under CPT code 99483. An independent historian can be a parent, spouse, guardian, or other individual who provides patient history when a patient isn’t able to provide complete or reliable medical history.
Typically, a clinician would spend 50 minutes face-to-face with the patient and independent historian to perform the following elements during the cognitive assessment:
Examine the patient with a focus on observing cognition
Record and review the patient’s history, reports, and records
Conduct a functional assessment of basic and instrumental activities of daily living, including decision-making capacity
Use standardized instruments for staging of dementia like the Functional Assessment Staging Test (FAST) and Clinical Dementia Rating (CDR)
Reconcile and review for high-risk medications, if applicable
Use standardized screening instruments to evaluate for neuro-psychiatric and behavioral symptoms, including depression and anxiety
Conduct a safety evaluation for home and motor vehicle operation (if applicable)
Identify social supports, including how much caregivers know and are willing to provide care
Address advance care planning and any palliative care needs
The instruments cited above may be applicable for some adults with minimal intellectual disability, but may be less useful for the majority adults with more notable lifelong intellectual disability. Some of the suggested procedures are appropriate, particularly if they do not rely on the individual to be the informant or pose an expectation that the individual may respond to requests in the same manner as neurotypical adults. Of particular concern is the use of generally applicable functional assessments (noted in the guidance) which do not recognize limitations in lifelong functioning or pick-up on decline or changes in function from previous levels.
Unfortunately, also missing from the extensive guidance is a stipulation for augmenting the assessment for persons with pre-existing cognitive impairments, such as intellectual disability. The NTG suggests that the current CMS guidance should be augmented with a notation of what alternative measures and procedure may be applied with conducting cognitive impairment assessments with adults with lifelong intellectual disability or other neuroatypical conditions. Such procedures should include
(a) drawing upon information from persons close to the individual, who have an understanding of the individual's history and optimal functional abilities,
(b) using functional assessment instruments developed for specific use with adults with intellectual disability (and particularly for those who may be experiencing decline), and
(c) working with primary caregivers, whether family or staff from host agencies, to design dementia care plans.
Many host agencies have medical and health records that can help examining clinicians with discriminating typical functioning from those associated with emerging neuropathologies. Further, many host agencies may also have records of the use of intellectual disability-specific standardized screening instruments that can provide insights in the history or frequency of occurrence of behavioral symptoms. Many sources have recommended using the NTG-EDSD for collecting such information and for holding discussions with informants.
Although the guidance doesn't specifically reflect the use of measures applicable to adults with neuroatypical conditions, we recommend using specialty measures germane to adults with intellectual disability to minimize misdiagnoses.
The CMS guidance provides these specifics as to the written care plan and its preparation:
The care plan should reflect a synthesis of the information acquired as part of the assessment. It should be written in language that is easily understood, indicate who has responsibility for carrying out each recommended action step and specify an initial follow-up schedule. Some clinicians find it useful to organize the care plan into broad components, such as:
• Specific characteristics of the cognitive disorder (e.g., type and severity of cognitive impairment; special hazards such as falls or orthostatic hypotension in Lewy body dementia; or referral to a dementia specialist for further diagnostic assessment or complex management).
• Management of any neuropsychiatric symptoms, including referrals for caregiver stress and behavior management training or psychiatric care for the patient as indicated.
• Comorbid medical conditions and safety management, including any changes needed to accommodate the effects of cognitive impairment.
• Caregiver stress and support needs, including primary care counseling and, as indicated, referrals to community-based education and support, specialized individual or family counseling, or in-home care, legal or financial assistance.
See also 'Dementia Care Planning'