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Question:

My brother just turned 58 years old. Other than a diagnosis of Alzheimer’s he enjoys good health. He lives in an apartment with 2 roommates and 24-hour staff. I noticed over the summer that he was going to the bathroom a lot and he was in there for a long time. I assumed it was constipation and took him to the doctor 3 weeks ago. We are adding prune juice to his diet, and he is seeing a gastroenterologist next month. One of the staff suggested it might be a prostate issue. I emailed his doctor and inquired about a prostate exam. I’m concerned how He will handle a digital rectal exam (DRE). Should we start with a blood test for PSA? I know nothing about the prostate. He has also lost 17 lbs. in the last year. I attributed it to new roommates and improved eating habits, but I’m wondering if it’s symptomatic of something else.

Answer:

In our practice, it has frequently been reported to us that a person with Down syndrome (DS) is spending a long time in the bathroom. There are many possible reasons for this.

One observation is that some people with DS will use the bathroom as a quiet place or a place to avoid the activity of their residence. The bathroom may be a place of escape. One time of life this may be noted is if/when the person develops Alzheimer’s disease (AD). When some people with DS develop AD, it is noted they may have reduced tolerance of noise and busy activity going on around them. These may upset them, and they seek places away from the noise and commotion and the bathroom is one such place.

Another reason for spending more time in the bathroom is constipation. Constipation is more common in people with DS. After doing scores of evaluations of people with DS including taking their history, doing a physical exam, and ordering and reviewing labs, I am convinced a large percentage of people with DS go through life at least mildly dehydrated; some quite dehydrated. Dehydration is a common cause of constipation. Many people with DS and AD appear to develop further impairment of their thirst mechanism. They don’t ask for fluids and often don’t drink unless it is offered to them. This may lead to more dehydration. It is important to be proactive with providing fluids and encourage the person to drink regularly. It is also important to be aware if the person doesn’t appear to be having regular bowel movements and address that as needed. Additional information on constipation in people with DS is available here.

Difficulty urinating is also a reason for spending more time in the bathroom. It is often due to the person having difficulty initiating their stream (urinary hesitancy) or they feel like they aren’t emptying their bladder (urinary retention). We have long recognized people with DS can have difficulty emptying their bladder, and we published a paper in 2015 on a study we did on assessing for urinary retention in people with DS. There are many reasons why a person with DS may not be emptying their bladder. Causes, symptoms, assessment, and treatment of urinary retention are discussed in the article linked here.

In an older man, hypertrophy (increased size) of the prostate is a potential cause of urinary retention. This is true for men without DS and men with DS. The urethra (the tube that carries urine from the bladder outside of the body) can become blocked or narrowed when the prostate is enlarged. A digital rectal exam (DRA) can give some but limited information. A prostate ultrasound can also provide information about the prostate. However, both of these require insertion into the rectum (finger or ultrasound transducer) and this can be difficult for some men with DS to tolerate.

There is testing available to assess urinary flow and whether the person can empty their bladder. This can be done for men with prostate enlargement and for men and women with urinary retention for other reasons. Extensive urinary flow studies are available (for men and women) that can provide a lot of information about what the person is experiencing, what he feels when his bladder is full, how the urine flows, and whether the bladder is being emptied. We have found this to be a difficult test for many people with DS. Therefore, we usually choose a different test that can measure the volume of urine before and after urination. While this does not yield as much information, this is usually better tolerated and often is a good next step in the assessment. It can be done with a bladder scan in the office or with an ultrasound of the bladder ordered as a pre- and post-void study. This can demonstrate if the person is able to empty his or her bladder.

There are other reasons for the urethra to be narrowed or blocked. Scarring or strictures, particularly after the person has had instrumentation (e.g., a cystoscope) or a catheter in the past, can cause reduced flow. Sometimes the narrowing may have to be enlarged with a cystoscope. The cystoscope can be used to open the urethra by removing the scar tissue. The narrowing from the scar tissue may recur and the cystoscope procedure itself may contribute to more scar tissue. This procedure sometimes must be repeated.
In an older man who is not emptying his bladder, especially if a large prostate has been detected, treatment with medications that improve urinary flow can reduce the time needed in the bathroom to start the urinary stream. Looking into the bladder with a cystoscope can also be used to visualize the effect of the enlarged prostate on the urethra and can also be used as part of a surgery to reduce the blockage from the prostate.

For some men and women with DS, the problem is not that the outlet of the bladder (the urethra) is obstructed, it is due to the bladder not contracting well (the bladder doesn’t push the urine out). The muscle of the bladder often has low tone in people with DS. We think the difference in the function of the autonomic nervous system (the part of the nervous system that functions without us thinking about it) in people with DS compared to people without DS likely contributes to decreased tone of the muscle in the bladder. In addition, in our experience, many people with DS don’t urinate regularly and “hold their urine” causing their bladder to get overstretched. Like a rubber band that has gotten overstretched, it can be difficult for the bladder to regain the function to contract normally.

A PSA (prostate specific antigen) blood test is likely to have limited utility in someone with these symptoms. PSA is used as both a screening test for prostate cancer and to monitor prostate cancer treatment. If the PSA is elevated, testing as described above is still needed to see what is causing the blockage or reduced ability to empty the bladder. In addition, prostate cancer is much less common in men with DS (as are most solid tumors in men and women with DS). In our discussions with our patients and their families, we usually don’t recommend ordering a PSA whether it be as a screening tool or when there is a concern about inability to empty the bladder. For more on prostate cancer screening in men with Down syndrome, please see this resource.

Another consideration is his kidney function. Kidney function is often mildly impaired in people with Down syndrome, probably due to a reduced number of kidney cells caused by having an extra 21st chromosome. In addition, chronic urinary retention can injure the kidneys as well and, therefore, diagnosing and treating urinary retention can not only improve symptoms but also prevent kidney injury.

Weight loss can have many causes. People with DS often have a change in their hunger. Some people with DS and AD stop asking for food and only eat when it is offered to them. Similarly with fluids as noted above, those aiding someone with DS and AD must be aware of this and ensure the person is eating adequately.

There are many other causes for weight loss including diabetes mellitus, kidney disease or injury, hyperthyroidism (overactive thyroid), loss of muscle mass due to inactivity, and others. There may also be metabolic changes in AD that are not well understood at this time. In addition, healthy eating and being more active with new roommates may also explain the weight loss. Monitoring of food intake and doing an assessment for other possible factors can help delineate the cause.