Don’t fall into the dementia trap when treating a developmental disability

by Lockup Doc

In addition to correctional psychiatry, I spend about half of my professional time treating patients with developmental and intellectual disabilities (mental retardation). The majority of my patients have severe or profound mental retardation and are completely nonverbal.

Over the years I have observed that when many of them are admitted to the hospital for acute medical or surgical problems, because they are severely cognitively and functionally impaired, they are treated as though they have terminal illnesses. The treatment approach is often akin to that for patients with end-stage Alzheimer’s Disease.

By definition, a developmental disability (DD) occurs before the age of 18 and is a static condition. Most of my DD patients have genetic syndromes or have suffered from perinatal brain injuries. They have been far behind the curve in meeting developmental milestones, and many never will be able to walk, talk, or have the motor or communication skills or level of independence that most of us enjoy.

However, despite these deficits, each of them has unique strengths. Even those who require significant assistance with their activities of daily living still seem to be able to enjoy various activities in their lives. It would be easy to judge them as having little quality of life, but the longer I work with this population, the more I believe it to be a mistake for the developmentally normal to judge the quality of life of the chronically disabled.

On the surface, these patients are obviously severely impaired, and in many ways some of them do not look much different from those with severe dementia. The key point, though, is that their developmental disabilities are not progressive or terminal illnesses.

In contrast to those with developmental disabilities, patients with end-stage Alzheimer’s Disease have a progressive and ultimately fatal disease. In advanced Alzheimer’s Disease, patients often become incontinent of bowel and bladder, become nonverbal or minimally communicative, have difficulty eating or stop eating altogether, and eventually require assistance with all activities of daily living.

Primary care physicians are familiar with this latter scenario since Alzheimer’s Disease is fairly common. Often their end-stage dementia patient previously will have been a person with normal intellectual functioning who was quite independent. Considering hospice and withholding treatment can be the right decisions in advanced dementia. We often think of such patients as having very little quality of life because of the dramatic losses that have been inflicted by their dementing illness. They clearly are not the same people they used to be.

However, since severe and profound mental retardation are much less common than dementia, and because those with severe developmental disabilities may, on the surface, resemble those with advanced dementia, I believe it is easy to mistakenly think of the two as being in the same end-of-life category even though they are not.

When this mistake occurs, DD patients may have necessary diagnostic and therapeutic interventions withheld even though their physicians may be very competent and believe that they are doing the right thing by not treating these “obviously declining” patients too aggressively.

The key to avoiding falling into the dementia trap when treating a patient with a severe developmental disability is to learn as much as possible about the patient’s baseline level of functioning. For example, if a particular patient has always been nonverbal, incontinent, and gastrostomy-tube fed but does not seem to be losing skills, then the diagnostic and treatment approach likely will be similar to that for any other non-terminal patient. However, if the patient is obviously declining relative to baseline, then a more conservative approach may make sense.

The author is a psychiatrist who blogs at Lockup Doc.

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  • Marie

    This is such a good reminder to never take ANY diagnosis for granted or at face value. As you so eloquently point out, it is an easy trap to fall in to, but the patient will suffer unnecessarily.

    This especially timely for me personally. My 76 year old mother has been diagnosed with dementia. However, the ‘dementia’ didn’t start until literally the day she had her pacemaker changed in March of 2009. As a nurse, I believe she had an adverse event during the minor surgery. But my non-medical siblings and father have sadly accepted the diagnosis without question. They say “What difference does it make?”

    It frustrates me beyond words because her treatment would be so different if she was correctly diagnosed. I believe her physicians are completely irresponsible. No one goes from functional to demented overnight without a catastrophic event. She now languishes in a long term care facility because it is too easy to label a 76 year old as demented if they are acting inappropriately than to take the trouble to explore what might have happened to her 11 months ago.

    Thank you for a thought provoking and compassionate article.

  • drbell

    Not only do I agree with this on a professional level but also on a personal level. I work with these issues professionally on a daily basis but also have experience with dealing with this issue in my immediate family. Many times I have marveled at the courage and dignity those with disabilites exhibit.

  • Dr. J

    A very thought-provoking piece that addresses an issue that very few of us in the medical profession even consider. Too often, we project our own views about the “quality of life” of an individual with a developmental disability. And as you described, the point on a person’s journey through life, when comparing someone with end-stage dementia to an individual with a static encephalopathy, is very different. We could certainly use more DD training in medical schools and residency programs to help new physicians understand this issue a little better.

  • Jen

    Excellent post. I have autistic children, and I often worry about what their medical care will be like when I’m no longer around to oversee it. Too many medical professionals consistently underestimate them, and more education around DDs could certainly assist with that.

  • mc

    As a pediatric ICU resident, I once took care of a patient who had complications after surgery. This patient had a known developmental disability at baseline, but immediately on arriving in the unit was intubated and sedated. When the patient’s condition began to deteriorate, ECMO was considered, but we were completely dependent on the input from the family and the surgeon (who had been taking care of the patient for some years) to determine is this would be appropriate or not- they told us this patient had a good quality of life and that ECMO was appropriate. We were fortunate to have this input available, because without it we had no idea what the quality of life of this individual was.
    Fortunately, this patient turned around and recovered without ECMO, but very scary to think about.

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