Unlocking Behaviors: Cognitive Changes
Unlocking Behaviors: Cognitive Changes
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 cogntivie changes can be addressed in an interdisciplinary fashion.
Jamie is a 48-year-old dynamic, energetic, fun-loving person who happens to have Down syndrome. She lives in a small community-based group home with two other people. She’s known as the jokester in the group and always has a way of making her friends laugh. She is an impeccable dresser and fancies cool hairstyles, which she highlights with fashionable hair accessories.
Jamie experienced a pretty big traumatic event in her life when she lost her mother two years ago. It had quite an impact on her. She became a bit withdrawn. She was less interested in many of her usual activities, and as her friend said, “She just wasn’t acting like Jamie anymore.” Her friends noted that she was less concerned about what she wore, sometimes wearing the same clothing two days in a row. Her hairstyle became much more straightforward and even appeared a bit messy at times. Her usual witty jokes seemed more challenging to come by for her, and often, she took long pauses before answering questions.
Over time, Jamie seemed to need more assistance with usual activities such as preparing meals, and later, she even seemed to have difficulties feeding herself. She occasionally chose a short-sleeved shirt to wear when it was 30 degrees outside, and more than once, she’d put her shirt on backward.
Behavioral Discussion
In the behavioral realm, one of the most challenging things is delineating between psychiatric symptoms (e.g., Jamie’s possible depression symptoms), dementia symptoms, and normal aging in a person with intellectual disabilities. Over time, I have developed an approach of “prove to me it is dementia.” I have adopted this approach because, far too often, dementia is a diagnosis that seemingly is arrived at too quickly and easily amongst interdisciplinary teams, including physicians and other medical professionals. There are many reasons a person can exhibit changes in cognitive status, mood, affect, or simply changes in their preferences. As behavior analysts, it is critically important for us to keep an eye on all the possible reasons a person could be exhibiting changes in their behavior as we work to delineate changing behavioral presentations as a person ages.
One of my most commonly used tools for making this delineation is a dementia screening tool developed specifically for people with intellectual disabilities – The National Task Group on Intellectual Disabilities and Dementia Practices – Early Detection Screen for Dementia (NTG-EDSD). The NTG-EDSD can be used for the early detection screening of those adults with an intellectual disability who are suspected of or may be showing early signs of mild cognitive impairment or dementia. The NTG-EDSD is not an assessment or diagnostic instrument but an administrative screen that can be used by staff and family caregivers to note functional decline and health problems and record valuable information for further assessment (NTG, 2013). Learn more here: https://www.the-ntg.org/about-the-ntg .
Jamie would likely benefit from the completion of a dementia screening tool, such as the NTG-EDSD, which is recommended on an annual basis for individuals with Down syndrome at the age of 40. This would allow her staff, with a distal and working knowledge of Jamie, to provide insight into the specific areas of her life in which changes have occurred, such as language & communication, activities of daily living, behavior and affect, and potential medical issues, to name a few. These areas can help guide the interdisciplinary team’s decision-making as they work to “prove that it is dementia.”
Medical Discussion
Jamie has many classic signs of depression and had a good reason for being depressed with the loss of her mother. However, because Jamie has Down syndrome, she is also at a much greater risk for Alzheimer’s dementia, and the signs and symptoms can overlap. 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 major 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 at an earlier age than those without Down Syndrome.
It’s important to rule out other causes for symptoms that are consistent with dementia. The following can present with signs resembling dementia:
- Mental health conditions
- Brain tumors
- Vitamin deficiencies
- Medication side effects
- Thyroid, kidney and liver disease
There is new and promising research in Alzheimer’s disease, including blood tests 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. Learn more about Alzheimer’s and other dementias at https://www.alzheimers.gov/.
Outcome
Jamie was initially evaluated by a neuropsychiatrist and was diagnosed initially with depression with secondary concern for Alzheimer’s. Medication treatment for depression did improve some of her symptoms, but after testing, talking with her support team, and reviewing her NTG-EDSD screen, she was diagnosed with Alzheimer’s dementia. She is currently receiving support from her team and friends, who have been educated on best practices for supporting people with dementia.
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.
Health-related Resources for People with IDD and Their Supporters
With significant health disparities noted in people with IDD, it is important that people with intellectual and developmental disabilities (IDD), their supporters, and healthcare providers educate themselves on the different health risks that are more commonly seen in people with IDD and about what can be done to prevent serious complications. Supporters and healthcare providers are often challenged in finding helpful information related to healthcare for people with IDD. As a physician who started practicing in this field in the 1990s, finding clinically relevant information about healthcare for people with IDD was challenging. Fortunately, over the past several years, more resources have been developed that relate specifically to healthcare issues and improving health equity for people with IDD. In this article, you’ll find a listing of websites, tools, and training available to provide information and guidance to you, whether a family member, paid supporter, healthcare provider, or person with IDD.
Using the information provided by the case manager, the HRST recognized the possibility of an undiagnosed gastrointestinal condition as the potential cause of Jerome’s behavioral symptoms and produced a “service consideration” suggesting the need for clinical follow-up to rule out Gastroesophageal Reflux Disease (more commonly known as GERD).
Aspiration is usually listed as the most common cause of death in institutional settings, including nursing homes and large group care settings for people with IDD. Aspiration pneumonia is a common discharge diagnosis following hospitalization. It occurs when bits of food or liquids make their way into the windpipe instead of into the esophagus where it should be. Aspiration can occur during the swallowing process, but also occurs when stomach contents come back up the esophagus as a result of vomiting or gastroesophageal reflux. It often begins subtly and damages increasing portions of the lungs. Feeding tubes, which carry their own increased risks, are often utilized in an attempt to bypass the swallowing process and reduce aspiration episodes, but are not always entirely effective. Other factors which may lead to aspiration are poor body positioning, particularly in individuals who cannot control their own movements well, and behavioral issues related to eating. One of the most common signs of aspiration is a cough noted during eating or drinking or shortly thereafter. When a cough is noted under these conditions an evaluation should be initiated to look for possible aspiration.
Constipation and Bowel Obstruction are almost always at the top of the list of causes of illness and preventable death in people with IDD. One of the most important root causes of bowel obstruction is the use of multiple drugs with constipating side effects. Add to this the fact that most of us have dietary habits that contribute to the problem, chief among them, diets that are low in fiber and adequate fluids. Lack of mobility is a contributing factor to constipation, as well. We often see people on one or more medications to help improve elimination, but ironically, drugs intended to improve constipation often place the person at higher risk for impaired bowel function. Adequate active movement or exercise is also important to bowel function. Controlling these factors, along with training caretakers to recognize the signs and symptoms of bowel problems at the earliest moment can greatly reduce occurrences of illness or death from bowel obstruction.
Seizure deaths can occur from episodes of prolonged, uncontrolled seizures as well as something called SUDEP or sudden unexplained death in epilepsy. For reasons that medical science has yet to explain, people who have epilepsy have a higher chance of dying suddenly unrelated to actual seizure activity. Drug toxicity and medication side effects can further impact the health and well-being of people who takethese medications, and the balance between adequately controlling someone’s seizures and the risk of adverse drug reactions must always be at the forefront of the treatment plan. Life expectancy for people with active seizure disorders has been shown to be up to 10 years less than those without epilepsy.
Dehydration is all too common and easily preventable in most cases. People who do not swallow well are particularly likely to refuse fluids or indicate fear when they get them, often resulting in dehydration. Dehydration is also likely when fluids are restricted in an attempt to prevent incontinence, not realizing that lack of fluids can contribute to constipation and increased seizure frequency, not to mention drug toxicity and other health problems. Awareness of fluid loss through sweating in warm and humid conditions or from loss due to vomiting or persistently elevated blood glucose levels in people with diabetes is important, as well. The presence of fever also increases a person’s fluid needs. Anyone supporting people with IDD should be aware of a person’s fluid requirements and work to ensure that they receive the necessary fluids to prevent dehydration.
Sepsis, sometimes called “the silent killer” is an ever-present threat to all. Sepsis is a condition which results from a significant infection in the bloodstream and the body’s response to that infection. Early signs of sepsis include fever, chills, rapid heart rate, low blood pressure and mental status changes. Some of these symptoms are common with may illnesses, but one’s index of suspicion should remain high for worsening signs of a serious infection and referral for a medical evaluation should be considered earlier rather than later. The risk of death increases significantly for each hour that passes when sepsis is left untreated. Remember, “When in doubt, send them out!”
Gastroesophageal reflux disease (GERD) is the backing up of stomach contents, including acid, into the esophagus. GERD is frequently undiagnosed in many people, including those without disabilities, until major harm has been done to the bottom of the esophagus. Medications that cause constipation also contribute to GERD. Individuals who are overweight, particularly when they carry excess weight around the abdomen or wear clothing that is too tight have a higher risk of GERD. Ill-advised dietary choices, immobility and improper positioning also contribute greatly to the incidence of GERD. As this disorder continues without treatment, discreet or frank aspiration, life-threatening gastrointestinal bleeding and esophageal cancer become increasingly common. Recognizing signs of GERD, especially in people who do not communicate with words, is important to reduce the risks associated with this condition. People who exhibit food refusal, coughing when lying down, physical or verbal aggression particularly around meal times, or distress in the middle of the night may be telling you that they are experiencing GERD. Pica, a behavior where people eat things of non-nutritional value, may also be a sign of GERD and should prompt an evaluation.
