Missed Diagnosis: How Many Times Have You Seen This Happen?

Jerome is 22 years old. He enjoys NASCAR racing, going to the beach, and eating out at restaurants. He works 12 hours per week at a local grocery store and, in his leisure time, likes to watch sports on TV with his dad and brothers. Jerome uses a wheelchair and requires assistance with activities of daily living, including eating, dressing, and bathing. He communicates primarily through gestures, facial expressions, noises, and pictures, using the basic communication device he received in high school. He lives at home with his parents, who are his primary caregivers. He also receives personal care and employment support during the day from a paid supporter named Cecelia.

Cecelia recently approached Jerome’s mother because she has been noticing changes in his behavior over the past several months. Typically upbeat, Jerome has been frowning more and is less interested in activities that used to make him happy. Last week, when asked if he wanted to stop at a local café for lunch, he shook his head to indicate “no,” yelled, and banged his wheelchair. And this week, Jerome used his communication device to indicate “sick” and “stay home” on a workday-something he had never done before.

Jerome’s mother was also seeing subtle changes in Jerome that were concerning. She shared with Cecelia that Jerome was groaning a lot, not sleeping well at night, and seemed to be eating less than usual at mealtimes. He recently yelled at his brother and purposely knocked a bowl of snacks on the floor while getting ready to watch the Sunday afternoon football game- something he usually loves to do.   He had also started chewing on his hands, causing chafing and redness.

When asked about how he was feeling, Jerome dropped his head, grimaced, looked away, and, using his communication device, indicated he felt “sick,” “tired,” and “mad.”

Jerome’s mother scheduled an appointment with his primary care provider. Unfortunately, this appointment did not go well all around. The provider was new to the practice, as was Jerome, having recently transitioned from his long-time pediatric provider. Jerome and his mother waited for almost an hour past their scheduled appointment time, and when the provider came into the room, she did not acknowledge Jerome, speaking only to his

mother. A cursory exam was done at the same time Jerome’s mother was filling the provider in about his recent behavioral changes and his complaints of feeling sick and mad. In less than 15 minutes, the provider concluded that Jerome was “probably depressed given his situation” and recommended that Jerome be started on tricyclic anti-depressant medication.

In the meantime, Cecelia contacted Jerome’s case manager to inform her of her discussion with Jerome’s mom and to let her know the outcome of his appointment with his primary care provider. The case manager, who was also relatively new to Jerome, thanked Cecelia for the information. Recognizing that Jerome’s symptoms represented a change in health status, she accessed his web-based Health Risk Screening Tool (HRST) record and updated it accordingly.

 

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).

Meanwhile, Jerome was taking his anti-depressant but wasn’t feeling any better. His behavior continued to deteriorate; he lost 12 pounds and called in sick to work so often that he was in danger of losing his job. He was exhausted from lack of sleep, as were his parents.

The case manager called Jerome’s mother to share the service consideration produced by the HRST and assisted her with obtaining a referral to see a gastroenterologist. As luck would have it, the gastroenterologist had a brother with disabilities, was happy to meet and talk with Jerome and his mother, and quickly recognized that Jerome’s “behaviors” were probably related to acid reflux which can cause pain and discomfort at mealtimes and is exacerbated when lying down in bed. Diagnostics were completed, and a diagnosis of GERD was made.

Jerome’s diet was adjusted, and his bed was positioned so that the head of the bed was higher than the foot of the bed. Medications, including antacids and H-2 (histamine) blockers, were ordered. Jerome’s primary care physician was consulted, and the tricyclic anti-depressant was discontinued because a diagnosis of depression no longer seemed appropriate and because this class of medications is to be avoided in people with GERD because it can actually worsen their symptoms.

Within two weeks, Jerome was back to his baseline, smiling, eating, working, and watching the NASCAR races with his brothers.

What happened in this situation?

Jerome was likely the victim of “diagnostic overshadowing,” a phenomenon that stems from cognitive bias and poses a serious health risk for people with intellectual and developmental disabilities (IDD). Often, symptoms that would otherwise be addressed through immediate medical evaluation are discounted and attributed to the person’s IDD. No further assessment is conducted, differential diagnoses are not considered, and medical conditions continue untreated, often while psychotropic medications are being given to “treat” the person’s symptoms. This bias is usually unconscious and can be addressed through healthcare provider education.

Fortunately, Jerome has attentive and knowledgeable caregivers and advocates who understand the importance of “looking beyond the symptoms,” access to a robust health risk management tool, and a disability-competent health care provider. However, there are many others who do not. In order to ensure health equity for all people with IDD, there is a need to:

  1. Ensure health care providers receive education to ensure they are disability healthcare competent
  2. Effectively monitor, identify, and address emerging health risks
  3. Educate support staff to recognize and report symptoms of the “Fatal Five Plus,” conditions most likely to lead to morbidity and mortality for people with IDD: aspiration, constipation, dehydration, seizures, sepsis, and GERD

IntellectAbility can help. Contact us for more information about the Health Risk Screening Tool (HRST), the Curriculum in IDD Healthcare, and our eLearn course on the Fatal Five.

People with IDD at Risk-The June 2022 Joint Commission Sentinel Event Alert

Juanita is a 53-year-old woman. She has a long history of anxiety and depression. Juanita also has cerebral palsy and an intellectual disability and lives in a community residence for people with intellectual/developmental disabilities (IDD).   

For the past couple of days, Juanita has been experiencing increased anxiety. She tells her supporters she feels more tired than usual, has a poor appetite, and has been having “dizzy spells.” Her supporters reached out to Juanita’s primary care provider, who recommended they give her lorazepam, as outlined in her as-needed medication protocol. But it doesn’t help, and Juanita now says she feels like she is going to die.   

While it is not uncommon for Juanita to suffer from acute anxiety, her supporters contacted the primary care provider again, saying, “something just isn’t right with Juanita.” A telehealth appointment was arranged, during which the provider met with Juanita and her supporters. The provider ultimately recommended Juanita be seen in the emergency room to evaluate her symptoms. A diagnostic workup was done, and it was determined that Juanita was having a heart attack and needed immediate intervention. 

Fortunately for Juanita, she can communicate her symptoms, has supporters who know her well and have been educated in healthcare advocacy for people with IDD, and has a relationship with a primary care provider who understands the value of “looking beyond the behaviors” to explore the potential for an undiagnosed medical condition. But this outcome is a best-case scenario and not the typical outcome for people with IDD because of a phenomenon known as “diagnostic overshadowing,” the topic of a recent Sentinel Event Alert issued by The Joint Commission,  a global driver of quality improvement and patient safety in healthcare and a leading accreditation body for healthcare entities.  

Diagnostic overshadowing is a harm that stems from cognitive bias and poses a serious health risk for people with disabilities. Often, particularly in the case of people with IDD who have co-morbid behavioral health conditions, symptoms that would otherwise be addressed through immediate medical evaluation are discounted and attributed to their IDD. No further assessment is conducted, differential diagnoses are not considered, and medical conditions continue untreated, often while psychotropic medications are being given to “treat” the person’s symptoms.   

“Diagnostic overshadowing is a serious safety and quality concern as an initial misdiagnosis can have a significant impact on quality of life, including the physical and psychological wellness of patients,” says Ana Pujols McKee, MD, executive vice president, chief medical officer, and chief diversity, equity and inclusion officer, The Joint Commission. 

According to the alert, the medical literature provides extensive evidence that diagnostic overshadowing exists within clinicians’ interactions with patients of all ages with physical disabilities or diagnoses such as autism, mobility disabilities, and neurological deficits. Unfortunately, most clinicians are unaware of this because medical and nursing schools do not typically include curricula on healthcare for people with IDD, and most practicing clinicians have not had the benefit of education in disability-competent healthcare.  

The Joint Commission Alert recommends the following to address the serious, life-limiting, and sometimes life-threatening consequences of diagnostic overshadowing:  

  1. Create an awareness of diagnostic overshadowing during clinical peer and quality assurance reviews and by addressing it in training and education programs. 
  2. Use listening and interviewing techniques designed to gain better patient engagement and shared decision-making. 
  3. Collect and aggregate data about pre-existing conditions and disabilities and create EHR prompts for clinicians. 
  4. Use an intersectional framework when assessing patients in groups prone to diagnostic overshadowing to overcome cognitive biases and look beyond previous diagnoses. 
  5. Review your organization’s ADA compliance using the added perspective of diagnostic overshadowing to ensure that it meets the needs of patients with physical disabilities. 

IntellectAbility can help.  

The online Curriculum in IDD Healthcare (CIDDH) includes a section specifically designed to instruct health care professions students and practicing clinicians on how to “unlock the language of behavior.” The training emphasizes how in-depth assessment of behavioral or psychiatric symptoms can lead to diagnosis and treatment of underlying medical conditions. 

The CIDDH also provides concrete recommendations for directly and actively engaging people with IDD in their health management and includes effective communication strategies for those supporting people who may not communicate using words. The training also emphasizes the key role of supporters and family members during the diagnosis and treatment process.  

Upon completion of the online modules, physicians and nurses earn Continuing Medical Education (CME) credits or Continuing Education Credits (CEUs) and are eligible to receive the IntellectAbility Recognized Provider in IDD Healthcare award, indicating their commitment to providing quality healthcare to people with IDD.  

According to Dr. Craig Escude, developer of the CIDDH, “As The Joint Commission recognizes, when providers set aside bias and assumption, they can listen to patients more effectively and therefore treat them more effectively. At IntellectAbility, we have decades of experience teaching person-centered methods to clinicians and caregivers who work in intellectual and developmental disability services. Our programs can help medical providers acquire the skills called for in The Joint Commission Sentinel Event Alert.”

 

Feel free to call us at 727-437-3201 or email us at Inquiries@ReplacingRisk.com.

Protective Equipment and Persons with IDD

Although people might be getting a little weary of hearing about the COVID-19 pandemic, it is still a very real issue and we need to remain highly vigilant. That includes wearing protective equipment when appropriate. When should masks be worn and how do you safely put them on and off? The World Health Organization (WHO) offers simplistic and helpful demonstrations and explanations regarding masks.

It is difficult for many people to wear these articles. If a person wears glasses and the mask is not put on appropriately, it steams them up. Or, the mask slips down on the face and it must be retied or adjusted. It could just feel very odd so that people are constantly trying to adjust it for comfort. Maybe the person is claustrophobic and has a feeling of suffocation if they can’t breathe in fresh air. What if the person with I/DD is fabric-sensitive, smell or touch sensitive? What if they are simply noncompliant? These are challenges that support persons are dealing with every day.

Here are a few things you can try:

1) Try putting masks on stuffed animals. Let the person put the mask on the toy and then apply their own mask or allow staff to apply it. The support person may have to put it on the stuffed animal. Leave it on the animal for a while so that they can see it isn’t bothered by wearing a mask. It may take several times of putting the mask on the stuffed animal before the person will allow a support person to put a mask on their face or put the mask on their own face.

2) If fabric masks are being used, have several pieces of fabric options and allow the person to choose what they want. Try to have different textures, colors and prints available. Select elastic that is not too tight so that it won’t hurt their ears. If elastic is not available, use large hair ties. The fluffy hair ties that are elastic may be used, too. Try masks with no elastic but instead have simple ties that go around the head. Again, allow them to select the elastic or ties.

3) Let them have a mask to carry around and hold and feel. Let the person try it on and take it off. It doesn’t matter if they destroy it, give them another type to play with and see if there is more success with it. Slowly try to desensitize. Let them wear a mask for ten seconds, then take it off. You may even have to start with a shorter time and add a second or two each time you put the mask on them. You can even make an activity out of it. “Mask time” is when everyone can demonstrate putting on and taking off a mask appropriately. You may try a reward system after they wear the mask for a while such as picking a special treat, a trip to the park, FaceTiming with family (make sure they have the mask on and give the person a lot of praise) or simply spending 1:1 time with their favorite staff.

4) Let them personalize their mask. Again, allow them to select the fabric. If using disposable masks give them a Sharpie® and let them draw on the mask and make it theirs. Provide different stencils that the support persons can help them with, such as cars, butterflies or action figures. Find pictures in magazines and hot glue to the mask. (Be very careful if you are using a hot glue gun near the person! Not only the glue gun, but the hot glue itself can burn the person.) Allow them to make two masks so they can choose which one to wear. This can also be done on a fabric mask, but if drawing, they are stuck with only one or two drawings.

5) The support person can also go to a party supply store, or again use a stencil, and get some crazy big glasses. Hot glue the mask to the glasses making sure the glasses won’t interfere with the fit. This can be done with cloth or disposable masks. If the glasses interfere too much with the fit, make some using a stencil and heavy construction paper. The person can select how they want their glasses to look and again they personalize with drawings or pictures. Be mindful the the glasses do not obscure the person’s vision.

6) The staff can have a “Western” time. Everyone puts on a cowboy hat and has a bandana. Watch old Westerns where many of the characters wear a bandana mask. Allow the person to try a simple bandana mask. This is much better than wearing nothing. (I would suggest you avoid The Lone Ranger TV series, because the main character only wears a mask that covers the eyes and obviously that would not be effective against COVID-19.)

Suggested Old Western Series:
a. Bonanza
b. Roy Rogers
c. Gunsmoke
d. Hop Along Cassidy
e. The Rifleman
f. Wagon Train
g. Maverick

The shows can often be found on ME TV or H and I TV channels. If you find some that are appropriate, DVR them and you will always have something to watch.

I hope this gives you some suggestions that might not have been tried. Even if all the people in support have no problem with masks, most of these suggestions can still be used to personalize their masks. All any of us can do is hope this nasty virus leaves us soon!

By Sherry Neal, RN-BC, CDDN

Physical & Nutritional Supports (Part 2)

Last month, we talked about Physical Supports and the concept of supporting a person 24/7 to achieve better health. In this month’s article, we’ll touch on Nutritional Supports. I credit HRS founder, Karen Green McGowan, RN, with enhancing my knowledge about this important way of support.

Few people with intellectual and developmental disabilities (IDD) are born with bony deformities. This is more often a function of failing to initiate the battle with gravity that begins with spinal shaping from a belly-down position. If, instead of moving the head and spine against the opposing force of gravity that normally changes the spine from a big C into an S curve, the person remains on their backside with little active movement, the body will take a new shape that reflects the primary pattern of movement. The head and spine will often flatten and reshape into scoliosis, kyphosis and a flattened chest. These deformities can interfere with normal bodily functioning including the ability to safely swallow.

Nutritional Supports are basically supporting a person in safely achieving good nutrition.

Consider someone that eats with her head rolled back and to the left. This position prevents an adequate closure of the flap that protects the windpipe. Bits of food can just fall right on into the airway. She develops aspiration pneumonia which leads to fibrosis in her lungs. Even if the poor eating posture is thereafter corrected, the fibrosis will predispose her to further pneumonias.

The key lesson here is prevention. Nutritional Supports are basically supporting a person in safely achieving good nutrition. Below are a few general tenants of good nutritional support.

Basics of proper alignment for eating:

  • Align the person starting with the proximal joints and work your way out to the arms and legs.
  • The trunk should between 45 and 105 degrees with 90 degrees the theoretical optimum.
  • We can eat in other positions besides sitting, such as prone on forearms or side lying. These positions are usually reserved for people who have deformities such that sitting is not a good position for them.
  • Keep the trunk as straight as possible with the nose, navel and knees pointing in the same direction.
  • The head should be pointing forward with the nose in midline. The head should not be tilted forward or back as that interferes with the function of the epiglottis in covering the airway. The most dangerous of these is tilted back as it opens the airway right up. It is very dangerous to eat this way.
  • The person may need to have their head supported in neutral and a little help to pull the jaw forward.

The above is general information and should not be implemented for any particular person without consulting with their healthcare professional. Always refer to a person’s Physical and Nutritional Support Plan for individual guidance of positioning and proper techniques for safe eating.

Physical & Nutritional Supports (Part 1)

I’ve practiced medicine in the field of healthcare for people with intellectual and developmental disabilities for many years, and sadly, I had never heard of the term “Physical and Nutritional Supports.” Sure, we did some of these things in our clinical support of people with IDD, but we never had fully understood what I consider this “lost art.” It’s time to put an end to that way of thinking. In this newsletter, we’ll talk about Physical Supports, and in a later newsletter, we’ll delve into Nutritional Supports. Much of what you read in these articles was adapted from the writings of HRS founder Karen Green-McGowan, RN. I’m thankful for the opportunity to have learned this and so much more from her.

The Concept of Physical & Nutritional Supports

People with physical disabilities often have conditions like cerebral palsy, spasticity and contractures of their extremities. Often, they are prescribed physical therapy for range of motion. There’s nothing wrong with that, but let’s look at it this way. We’ll compare weight loss to physical therapy to illustrate the concept.

Physical therapy is often delivered in doses to a person who needs modification of life-style 24 hours a day.

Consider a person who is morbidly obese. Doses of therapy, such as a 300-calorie meal three times a week, will seldom impact the problem unless movement and intake are modified 24 hours a day. Many clinical services are delivered in doses, but most major issues, such as obesity or recovery from a stroke, require a major alteration in lifestyle

With that in mind, consider a person who has a physical disability. They go to see a physical therapist for three doses of physical therapy per week. They then return home and are placed in a chair for the rest of the day before being placed in bed. That physical therapy is wasted and will have little, if any, impact on the health of the person. Ninety minutes of stretching a week compared to the remaining 9,990 minutes in the week spent sitting or lying down with little active movement just won’t do it.

Physical therapy is often delivered in doses to a person who needs modification of life-style 24 hours a day. Babies born with hemiplegia, the most common form of cerebral palsy, want to use only their good side and leave the involved side dragging behind. When they are forced to use the involved side from the get-go, within a year or so, one can often not tell that the toddler had a disability at all.

This makes the case for 24-hour planning that utilizes many forms of supports to get the function of a healthy body. It is not only up to the therapist or clinician to manage the person’s health. It is up to each person who supports them to understand the forms of support this person requires and how they fit in. Techniques to support people 24/7 are relatively easy to learn and to implement by all staff including direct support professionals when guided properly by a physical and nutritional supports plan. Rather than focusing on doses of therapy, we should develop a therapeutic lifestyle. It just makes sense.

Physical & Nutritional Supports

For most all of us, getting up and eating our daily meals, plus a few snacks here and there, is something we take for granted. Running through a drive-thru window for our favorite beverage on the way to work is routine. But what if our meals had to come through a tube, or be pureed or liquids thickened? That would certainly turn our daily routine upside down!

As service providers, some of the people you provide supports for receive tube feedings or their food and fluids are otherwise specially prepared. This is their routine. Doesn’t sound very appetizing, does it? What if you could help a person MAYBE eat a little more independently or have a more typical presentation of their meals and drinks? I think you would agree that would be the better way to go. Physical and nutritional supports may help you do that.

What if you could help a person MAYBE eat a little more independently or have a more typical presentation of their meals and drinks?

Don’t just think of physical supports as a specialized chair or positioning device. Physical supports are a whole range of positions that a person can assume throughout the 24-hour day to help assist that person move better. It may consist of placing a person in a position where they are required to reach out for an item or loosen up a tight joint. This might allow him or her to eventually use that arm to eat by being able to reach their utensils or a glass of fluid. As a caregiver, you may be trained in special techniques to help control jaw movement or teach a person to drink from a straw. These special positions and procedures are often used by physical and occupational therapists to achieve a more independent eating style for the person.

There are many ways that therapists can assist a person with eating. Therapeutic positioning is one of the means they use to help a person gain more control. Therapeutic positioning involves putting a person into a “working” position. A working position is one where muscles, bones and joints must “work” to hold them in that position. These positions help reform those connections and the movement ability. A person should be in working positions throughout the entire day. The appropriate therapist prescribes what those positions should look like.

Therapists may also help persons with adaptive feeding methods or other physical supports. There are many eating utensils that enable a person to eat more independently. You may be familiar with some items such as built up handles on a spoon or a rocker knife, but there are a lot of other devices available. Sometimes, a person could eat more by mouth with special presentation techniques. The therapist can determine if they have one side of the mouth that is stronger than the other and determine where food needs to be placed on the tongue. Another method is to trigger reflexes of the lips in order for a person to pull food off of a utensil.

Physical Supports

When you think of physical supports, do you only think of things like wedges, custom fit wheel chairs and seat belts? If that’s the case, you are missing out on a lot of things you can do to improve the lives of the people you serve.

Physical Supports allows you to provide the external supports necessary throughout the 24-hour day to help the person become more functional. Think about the supports you need throughout the day. Are you a little on the shorter side and need someone else to reach the top shelf of the cabinet? What about an extra pillow at night to prop up your arm so it doesn’t go to sleep? Glasses in order to read? These are very minor, but they are still physical supports.

When we are on a diet, we don’t just diet 3 days a week or only at meals then binge the rest of the time. It’s the same principle when a person is getting physical therapy: we can’t just let them be inactive and have therapy 3 times a week. During the time they are not actively receiving P.T. we can be placing the person in ACTIVE or WORKING positions. If a dead person could maintain the position, it is not an “active” or “working” position. Just lying on your side is not an active position. Putting someone into a prone on forearms position so that they have to hold their head up is a working position.

Physical Supports allows you to provide the external supports necessary throughout the 24-hour day to help the person become more functional.

We often can see these positions in infants as they develop. The infant will start raising its arms up or holding up their head on their own. They can’t hold these positions for long until they build up strength. They are building against gravity. A person that has a contracture in an elbow for instance, may have their arm placed in a working position that allows gravitational force to help move the arm down into a more usable position.

There are also physical supports that are not active. Positioning a person to prevent pressure injuries is a physical support. Sometimes we do it with special equipment, sometimes it is just with bed pillows. Just know that we are positioning them for prevention when they are in EVERY position throughout their entire day, not just at night.

Physical supports can help prevent pneumonia, help with bowel function, keep skin healthy, help the person to become more independent and many, many other things. Please keep in mind that even the most minor change can make an enormous difference in someone’s life.