General/Lifestyle - IntellectAbility

Dementia and Intellectual Disabilities

Written by Craig Escudé, MD FAAFP, FAADM, September 2023.

Dementia can be a pretty scary thing for anyone. For people with disabilities, changes or a decline in cognitive functioning can be quite challenging to recognize, especially in people who do not use words to communicate.

When we are looking to diagnose dementia, clinicians frequently use tests like a mental status exam where a person is asked to remember lists of items, draw a picture of a clock with numbers on it, and recall current events like naming the current president and the city and state where they live.

For some with disabilities, questions like these may not have ever been able to be successfully answered due to a different baseline of knowledge and comprehension.

So how, then, can we attempt to accurately diagnose dementia in people with differing levels of cognitive baseline functioning?

One way to do this is to look at changes in functional status over time. There is a tool developed by the National Task Group (NTG) on Intellectual Disabilities and Dementia Practices called the Early Detection and Screen for Dementia (EDSD) (i), which is specifically geared to help assess levels of functional decline over time in people with intellectual and developmental disabilities. This tool is designed to be used by family members and other supporters. Looking at serial measurements annually or every six months can help determine if a person is having a functional decline, but does this mean that the person definitely has dementia? No, it does not. Ruling out other causes for symptoms consistent with dementia is also very important. Mental health conditions, untreated sleep apnea, metabolic toxicity including alcohol, medication side effects, vitamin deficiencies, brain tumors, and thyroid, kidney, and liver disease can all present with signs resembling dementia. However, having the type of information that the EDSD can provide can be very helpful to a clinician in determining whether or not a person has evidence of dementia. When we see changes in cognition, we should never hastily conclude that it is due to dementia but instead look for treatable underlying causes such as those mentioned above. Working with a clinician knowledgeable about dementia in people with disabilities is advisable.

People with Down Syndrome are at a much higher risk of Alzheimer’s type dementia. People with Down Syndrome have an extra copy of chromosome 21, which also carries a gene responsible for producing a protein called amyloid precursor protein. Too much buildup of this protein in the brain leads to beta-amyloid plaques, which are implicated as a significant cause of Alzheimer’s disease. It is estimated that 50% or more of people with Down Syndrome will develop Alzheimer’s, and they are more likely to develop it earlier than those without Down Syndrome. It’s recommended that screening for dementia using a tool like the EDSD be started at age 40.

There have been significant advances in research on Alzheimer’s disease, including the development of blood tests and more sensitive brain scans that can help diagnose Alzheimer’s dementia. While these tests are currently not available everywhere, soon, we should have widespread availability of blood testing to diagnose the condition. There have also been advances in medication treatment options for dementia, and current trials are promising for the availability of effective treatment options in the near future. As with any new treatment, caution is warranted before starting medications, and one should always weigh the risk and benefits of treatment and include frank discussions with the clinician, the person for whom treatment is being considered, and the person’s support network to help ensure that the best decision is made for each individual person.

People who are supporting those with dementia also need support. The NTG also has a Family Support Committee. Activities of the committee include providing written and public comments on federal issues related to dementia, an inclusive, monthly, national online support group for family caregivers, and participating in a Facebook group for caregivers and professionals supporting individuals with Down Syndrome and Alzheimer’s disease. You can learn more about the NTG Family Support Committee by visiting www.the-ntg.org/family-caregivers.

Realizing that someone we love is showing signs of dementia can spark a wave of fear and anxiety. Ensuring a proper diagnosis, involving compassionate clinicians, and aligning ourselves with others facing similar situations can help us gain understanding and position ourselves to continue to grow and thrive in a life that, despite challenges, is filled with many joys and blessings.

References:

[i] National Task Group on Intellectual Disabilities and Dementia practices. NTG – Early Detection and Screen for Dementia. (NTG-EDSD)

[ii] National Institute on Aging. 2020. Alzheimer’s Disease in People With Down Syndrome

How Healthcare Technology Shapes the Lives of People with Intellectual and Developmental Disabilities

How Healthcare Technology Shapes the Lives
of People with Intellectual and Developmental Disabilities

Written by Dr. Craig Escudé

People with intellectual and developmental disabilities (IDD) experience numerous challenges accessing healthcare and receiving services and supports that result in poorer overall health, lower life expectancies, and reduced possibilities to live a full life of their choosing. Technological advances are facilitating earlier detection of illnesses that are often missed and improving health outcomes for a population with significant health disparities. These advances additionally have widespread applicability extending to people who are aging, people with cognitive decline, traumatic brain injury, and other differences.

Health Risk Informed Telemedicine™

The Health Risk Screening Tool (HRST) is a web-based application used to screen for health risks in people with IDD. Supporters of people with IDD answer simple health-related questions about the person they support, and the tool then provides a person-specific health risk profile to enlighten support teams and clinicians. Beyond identifying those risks, the HRST delivers action steps specific to the person’s risk profile that supporters utilize to mitigate those risks. Using this tool helps supporters to recognize signs of illness sooner and empowers them to act upon them with confidence. Earlier identification of health conditions leads to better outcomes.

In a new health model called Health Risk Informed Telemedicine™, IntellectAbility’s HRST risk profile technology is being integrated into telehealth companies’ electronic health record platforms like StationMD that provide vital telemedicine services to people with IDD. The telehealth clinician then has access to their patient’s most significant health risks, enabling them to make better-informed decisions about the person.

“Watch” That Seizure

Did you know your watch can detect seizures? Electronic watches with motion sensors can detect body movements consistent with seizure activity. They can alert staff or family members of the possible seizure and prompt a check-in on the person.

Improving Safety and Accuracy with Medication Usage

People with IDD often rely on medications to treat health conditions. Still, delivering those to the person can be complex, especially in enterprise settings like group homes and supported living arrangements when relying on others to assist in administration, record keeping, and other related processes.

Technology, through the use of apps tailored to IDD Residential workflows along with their app-connected medication boxes that only provide access to the right medications, at the right times, for the right people, can dramatically improve medication and regulatory compliance and independence for the individual.

Additionally, this technology, such as that from ImpruvonHealth, can drastically reduce the number of medication errors, improve safety, and reduce the time needed by nursing and direct support staff to deliver medication to the person.

Monitoring From Afar

Remote monitoring systems can enable people who need extra support to live more independently. They may come in the form of web-connected alarms that can alert a support staff member if the oven is left on, if a smoke alarm goes off, and if a person falls. Or, they can prompt people to perform activities such as daily grooming or taking their medication at the right time, allowing them to live more independently without having someone in their home 24/7.

The independence gained from utilizing such supports can be life-changing to the person, allowing for more privacy and independence while still assuring safety. Companies like SimplyHome focus on using these remote supports to improve independence for people with IDD.

What Did You Say?

Everyone communicates. We may not all use words, but we all definitely communicate. People with IDD can frequently comprehend more than most people assume, but they may not be able to communicate effectively through speaking and writing. However, there are technological advances that improve their ability to communicate.

There are numerous devices that can facilitate communication, as well as apps that can be used on tablets and phones to assist someone who has a limited ability to use words to communicate to be able to type in words or utilize pictures to communicate more effectively with others.

Getting From Here to There

There are also apps that have been developed to assist people with IDD to navigate more successfully in their communities. Other apps can prompt them to stay on task and digitally connect to a virtual job coach when they need support at work. Apps like these help foster greater independence while reducing the need for paid, in-person support during these activities.

Technology is advancing in so many ways and at such a pace that one can only imagine the future. I encourage “techies” out there to give attention to research and development of technology related to improving health, wellness, freedom, and independence for some of society’s most at-risk. And to take it one step further, imagine what collaboration between these different technologies could offer.

Unlocking Behaviors: Gastrointestinal Distress

Co-written by Risley “Ley” Linder, MA, MED, BCBA & Craig Escudé, MD, FAAFP, FAADM

This article is part of a co-authored series on behavioral presentations in which a physician and a behavior analyst provide insight into real-life case studies to share their expertise on how behavioral issues can be addressed in an interdisciplinary fashion.

Rick is a 43-year-old man with a moderate intellectual disability. He is very active and is described as a “high-energy” person. He was transferred to a new group home after his last one closed due to the inability to find enough staff to meet the needs of the people they supported. It was immediately noted by the new support team that Rick frequently searched for things to eat, and most of it was not food. He would look for objects on the ground, find whatever he could, and eat them. A repairman was working on the heater, and Rick managed to grab a small screw and swallow it before anyone could stop him. There was a tear in the vinyl flooring in his room, and staff noted one morning that the tear was a bit larger. Later, they saw him picking the flooring, breaking small pieces off, and eating them.

His staffing needs increased to monitor him more closely and prevent him from ingesting harmful objects. His level of aggression began to increase as staff would attempt to keep him from eating non-food items.

Medical Discussion

Eating things of non-nutritional value or non-food items is called pica. People may search for objects on the ground, pull stuff off the wall, or constantly search for some object to chew or swallow. It is more commonly seen in children, pregnant women, and people with conditions such as autism, intellectual disabilities, and schizophrenia. Many forms of pica involve one particular item that a person ingests.

Examples include:

Chalk
Clay
Dirt
Coffee grounds
Ice
Paper.

Sometimes pica is associated with underlying medical conditions. One of the more commonly seen conditions in the general population is iron deficiency which may be associated with an ice pica.

In people with intellectual and developmental disabilities, a condition that is noted to be commonly associated with pica behavior is gastroesophageal reflux disease (GERD). One suspected reason for this association is that a person learns that if they swallow more frequently, the acid that causes the pain is washed back into their stomach. They then begin looking for items to eat to stimulate salivation to wash down the acid.

Another commonly seen presentation of GERD is someone who likes to chew on a sock or other object. If you support someone who does this or has pica behavior, you might consider asking for an evaluation for GERD. They may be chewing to stimulate salivation to wash the acid down to relieve pain. Consideration should also be given to checking for Helicobacter Pylori infection, which has been implicated as a cause of GERD.

Behavioral Discussion

When examining pica behaviors, the first question is, “Are there immediate health and safety concerns?” As a few quick examples, is the person in immediate danger from consuming toxic chemicals, uncooked food, sharp items, or unknown items/substances? If so, medical assistance should be sought immediately.

Second, “What is the historical dynamic of this behavior?”

In the case of Rick, long-term pica behaviors can result from undiagnosed/untreated medical conditions in conjunction with learned behaviors that actually have logic in their engagement.

To continue with Dr. Escudé’s point, if a person consumes the brittle, chalk-like substance comprising drywall, they can learn that this eases the discomfort in their stomach, throat, and mouth (e.g., GERD symptoms). The presentation we, as behavior analysts hear is, “They are eating the walls!” In reality, they have found an “antacid” in their environment that is managing the discomfort they are experiencing.

Behaviorally, pica behaviors should be presumed to require a multidisciplinary approach until proven otherwise. The rare but eye-catching “Rapunzel Syndrome” illustrates how pica behaviors are complex and require insight from various professionals. The combination of trichotillomania (pulling out your hair) and trichophagia (chewing/consuming hair) requires a multi-faceted plan of care that requires behavioral management, psychiatric consultation for potential underlying diagnosis, routine primary care check-ups, and crisis management for potential medical needs – to name a few.

Chronic pica behaviors can be reduced, particularly if the underlying cause is related to a medical condition. However, the person may still exhibit the pica behavior “out of habit.” Using the example of a person consuming drywall, they have learned that this behavior effectively alleviates the discomfort they feel. At the beginning of treatment (e.g., taking medication), they may continue to engage in the behavior, as they have to learn that taking medication is a more effective means of reducing discomfort. The person should be monitored after the introduction of a medication to see if the behavior decreases, stops, or even increases, as the medication type, dosing, or other factors may not be the “right fit.” In other words, if the medication is ineffective in reducing the discomfort, the pica behavior will likely continue.

If the behavior abates after the introduction of any intervention (e.g., medical and/or behavior) but suddenly re-appears, there could be an acute stimulus that is exacerbating the existing diagnosis and should be evaluated by a medical professional. The pica behavior should also be considered as potentially indicating other medical concerns as the person may have generalized the pica behaviors using the logic, “Well, this helped me the last time my stomach hurt.”

Outcome

Thanks to an astute direct support professional and a responsive team that was aware of the association between pica and GERD, Rick was taken to see a gastroenterologist who performed an endoscopy. He noted numerous gastric and esophageal erosions consistent with GERD. Rick was started on a proton pump inhibitor, and over the next several weeks, his pica behavior slowly lessened. His suffering from GERD symptoms, his overall health risk, and the need for additional staffing were all reduced, resulting in improved quality of life for Rick, his supporters, and the support agency.

Author Bio:

Ley is a Board-Certified Behavior Analyst with an academic and professional background in gerontology and applied behavior analysis. Ley’s specialties include behavioral gerontology and the behavioral presentations of neurocognitive disorders, in addition to working with high-management behavioral needs for dually diagnosed persons with intellectual disabilities and mental illness. He is an officer on the Board of Directors for the National Task Group on Intellectual Disabilities and Dementia Practices, works closely with national organizations such as the National Down Syndrome Society, and is the owner/operator of Crescent Behavioral Health Services based in Columbia, SC.

Dr. Craig Escudé is a board-certified Fellow of the American Academy of Family Physicians and the American Academy of Developmental Medicine and President of IntellectAbility. He has over 20 years of clinical experience providing medical care for people with IDD and complex medical and mental health conditions. He is the author of “Clinical Pearls in IDD Healthcare” and developer of the “Curriculum in IDD Healthcare,” an eLearning course used to train clinicians on the fundamentals of healthcare for people with IDD. He is also the host of the “IDD Health Matters” podcast.

Unlocking Behaviors: Dental Health

Co-written by Risley “Ley” Linder, MA, MED, BCBA & Craig Escudé, MD, FAAFP, FAADM

Published by Helen Journal

This article is part of a co-authored series on behavioral presentations in which a physician and a behavior analyst provide insight into real-life case studies to share their expertise on how behavioral issues can be addressed in an interdisciplinary fashion.

When it came to tennis, 37-year-old Tim was a huge fan. He loved watching how fast the players hit the ball back and forth, especially his favorite player Roger Federer. He knew the general timing of the US Open and other major tennis events and would watch as many matches as possible.

Tim enjoyed snacking on almonds and other nuts while watching tennis. One year, during the US Open, he decided he didn’t want almonds and even knocked them off the table a couple of times. He began to get a bit agitated at various times of the day or night, and his support staff wasn’t sure what was going on. After a few weeks, Tim became quite a challenge, hitting out at others, wanting to stay in his room more, and even eating less.

Tim was taken to his primary care physician for an evaluation. He underwent a physical exam, and nothing unusual was found except for a 5-pound weight loss since his last visit six months prior. Because of his agitation, he was then seen by a psychiatrist. Staff were concerned that he was endangering himself and others during his episodes of agitation. He was started on psychotropic medication in an attempt to reduce his aggression. It seemed to work for a while, but then the agitation ramped up again. After several psychiatry visits and medication adjustments, the recommendation was made to move Tim to an in-patient psychiatric setting for evaluation.

A relatively new nurse who had recently attended a conference about behavioral changes in people due to underlying medical conditions attended Tim’s final team meeting before he was to be committed. She recalled hearing at the conference how dental issues can cause agitation. She recognized that Tim had not had a dental evaluation in the last three years because of COVID restrictions and his inability to wear a mask.