Janine: Cushing Syndrome

Dear Trudy,

Before describing the effects of Cushing syndrome on the bladder and bowels, I feel it only fair to give a “Readers Digest” definition of the condition.

Cushing Syndrome is a disease caused by an abnormally high presence of cortisol in the body. Cortisol is a steroid produced by the adrenal glands. Unfortunately, excess cortisol (hypercortisolism) lends to a myriad of unpleasant symptoms; the most popular being drastic weight gain, especially in the mid-section, while maintaining thin arms and legs.

Other symptoms may include but are not limited to excessive body hair (hirsutism), buffalo hump, moon-shaped face, purple stretch marks (striae), fragile skin that bruises easily, acne, slow healing, missing periods (ammenorrhia), high blood pressure, depression, diabetes, osteoporosis, fatigue and muscle weakness.

Cushing patients are often misdiagnosed, and can wait years for their diagnosis due to the overlapping of the symptoms with other diseases. Dr. Harvey Cushing was the first to discover Cushing Disease in a 1912 study; so us, “cushies” use his birthday as a time to spread awareness about this disease because it’s not a very well-known illness.

As you can see from the symptoms above, Cushing syndrome affects the body in many different ways. The adrenal glands are responsible for the production of cortisol and incidentally sit right atop the kidneys as can be seen by the picture below (courtesy of Wikipedia “polyuria”).

An overproduction of cortisol can cause high blood sugar and often diabetes. Anyone that has known someone with diabetes or had diabetes, knows that excessive thirst and urination that comes along with it.

Most Cushing patients will tell you they wake up multiple times during the night to void and tend to have a tremendous output of urine. Also, people with Cushing may do something called “cycling.” This means that their cortisol will be tremendously high at some points and tremendously low at others. Low cortisol can cause diarrhea, nausea and tremendous stomach upset.

My experience was slightly different. I had tumors on one adrenal gland that were causing the excess cortisol; however, I also had tumors on the other adrenal gland causing excess aldosterone. Aldosterone is another steroid produced by the adrenal glands that directly affects the breakdown and absorption of salt in the body.

You would be surprised by just how important this is. Not only does hyperaldosterone cause extremely high blood pressure, but it creates something called salt-wasting. The excessive urination is so intense that potassium and salt just come right out in the urine. Low potassium is called hypokalemia and can be extremely dangerous if not treated.

Now, I mentioned that people with Cushing tend to wait a long time for a diagnosis. I became sick at the age of 19. I doubled in weight in less than a year and my blood pressure was running easily 200/95 most days. Yet my doctor continued to feed me blood pressure medication until the age of 23.

He finally figured that maybe I wasn’t just being lazy, overeating or just depressed. He sent me to a nephrologist to scan my kidneys and see if the arteries were narrowed and causing my high blood pressure. Thank goodness he finally did!

The nephrologist saw something called an abdominal aortic aneurysm (AAA) in the scan and had me immediately admitted to the hospital. An AAA is no joke. It is basically when the aorta is under such immense pressure for such a long time that it sort of splits and balloons out. Therefore, allowing the blood to channel through two openings. The ballooning of the outer wall or second opening is weak. In the elderly it is much more weak and subject to bursting. I should have been dead. My youth is what saved me.

The hospital that discovered my AAA was not equipped to deal with such a matter, so they transferred me to the city hospital. The first course of treatment was to assess the damage and lower my blood pressure. An endocrinologist walked in and took one look at me and was able to surmise I had Cushing. He was right. I was a textbook example of someone with Cushing.

Within the year I had my right adrenal gland fully removed and my left mostly removed. They left behind a piece of the left hoping it may someday be able to function again on its own. The withdrawal I went through from steroids after surgery was intense.

When a person has adrenal glands removed he or she may become dependent on steroids for life. Without those steroids the person can go into a state of adrenal insufficiency or AI. AI is a serious matter, which amongst other more serious symptoms may be diarrhea, nausea and vomiting. I was lucky because when my surgeons saved my partial adrenal, it eventually, continued to make aldosterone. That meant I only needed to replace cortisol putting me at less risk for AI.

As you remember, I had been diagnosed with an AAA prior to the adrenalectomies. Unfortunately, it had grown to a point at which it needed to be repaired. I went in for surgical grafting of my aorta in November of 2006. If anything could go wrong it did. I had infections, was in and out of surgery, but most importantly for our purpose here; suffered a spinal cord infarction.

Something like that had always been a risk of the surgery, but I never thought it would happen to me. I remember feeling very tired and unable to get out of my hospital bed. Then…I woke up and was unable to feel my legs. I was paralyzed from the waist down. They told me that the fluid in my spine had been temporarily blocked. That I may never walk again. Not only did I have this gaping hole in my side where they were still continuing to operate every other day to debreed the wound and try to rid me of infection, but now I was paralyzed?

I was extremely lucky in the sense that I gradually regained feeling in my legs. When I say gradually, it was extremely slow and painful. I also began physical therapy. During that time I had a catheter in and my bowels had become impacted. The impaction was horrible. I had a very nasty and not well meaning nurse manually remove the impaction. It was both humiliating and painful. I will never forget her long finger nails…

Turns out that this would become common-place. My bowels were flaccid. So they started giving me so many stool softeners I learned quickly that I had no control over my bowels. I was constantly having accidents and started wearing diapers. Next they removed my catheter. I had to pee so badly and I just couldn’t. I didn’t understand. I had no idea this could happen. I remember sitting on the toilet in my room running my hand under warm water from the sink just praying I would pee. Nothing happened.

The nurses would come in and straight cath me. Unfortunately, I have always had a large urine output, with or without Cushing. So the 4-6 hours they thought was normal would set me into a state of agony. My bladder was so full and I was in so much pain pleading with them to cath me.

Their responses: “You can’t need to be cathed again.”

Finally a kidney doctor came in and said there was nothing wrong, I just had a larger volume than most and that they should restrict my fluid intake. I had my water held ransom and because of the paralysis couldn’t get up to get any. The result was that I was still putting out an abnormal amount of urine and I became dehydrated. They returned my water.

The hardest part in all this was that I had to learn how to catheterize myself. The therapist tried to help me. She gave me a mirror and told me to lay in bed and do it with a self-contained kit. Ummm, I am over 300 lbs, I haven’t seen my urethra since I was 19, are you kidding me?

I would just stab at myself upset and blind because I had to pee so bad. Not to mention, I couldn’t help think, how would I ever do anything in the real world if I constantly had to do this? Then I would have to beg the nurses to do it for me. Forget sleep. This would happen every couple of hours because I had to go so often.

I was so lucky because I met this wonderful woman with MS in therapy that said she had been cathing for years. She was a little bit on the heavier side, but not as big as me, and said she used these little 14French sticks and emptied her bladder right into the toilet. She described to me how she did it and it only took a small while before I was able to do it too! I am forever thankful to her for teaching me what the therapists and doctors could not.

So now I want to skip ahead to a couple of years later. I have got onto a bowel plan that isn’t perfect but my cathing frequency is becoming an issue. I was cathing upwards of 30 times a day! I couldn’t take a half hour car ride without being so uncomfortable I wanted to cry. Not to mention the infections… I always had one.

I decided to see a local urologist. I told her all of my symptoms and what I had been through and she didn’t even have to physically examine me. She gave me a bunch of pamphlets and a video and explained interstim implants and sacral stimulation. She said it would get my frequency under control and there was a good chance I would pee again.

Six surgeries later… Yes , I said SIX, and $3,000 later, the implants had not helped at all and they were burning under the skin. I won’t get into the details of why I had so many surgeries, but let’s just say she sold me on the false hope she gave, because I was so desperate. She had even gone as far as to let me believe the implants would improve my bowel function. I felt betrayed.

I had also gained back the 100 lbs I had lost prior to their insertion due to all the depression and recuperation from the surgeries. I had to start going to pain management because the weight was causing too much stress on my surgical sites in my ribs and abdomen.

Let’s fast forward again, to about two more years later and about two and a half years ago from now. I decided to see a urologist in the city. He was very nice and said we needed to do something called a urodynamic study so he could assess the extent of my bladder function.

He was extremely honest and blunt in his assessment. He said that the interstim implants could have never helped me. I was both devastated and relieved by his honesty. He basically explained that the nerve damage caused from my spinal infarction was causing my nerves to work overtime trying to work making spasms in my bladder. He further explained that the damage was too severe for me to ever push out urine on my own again. The best course of action was for me to try and control the frequency of my urination with medication. I tried so many medications and still only got down to cathing around 20 times a day. He also said that he would remove the implants for me whenever I wanted.

Not much time had passed and I was constantly online looking for any sort of new procedure that might help me at all. It had escalated to the point that I began leaking if I didn’t make it to a bathroom in time.

Finally, I came across and article on botox. I called my urologist immediately and asked him if I was a candidate. He said I was, and scheduled me. I had the botox injection treatments and interstim implants removed at the same time.

Here I am two and half years later. I just had my fifth set of botox injections and I am so much more content. I am only cathing an average of ten to fifteen times a day. I don’t leak anymore either. It’s still not perfect or ideal, but so much better than before. My bladder isn’t running my life. I get the series of shots about every six months in the hospital under twilight. Some patients have it done right in his office without any sedation, but I am not interested in finding out what twenty or thirty some-odd shots to my bladder feel like without being sedated. I will say that I am always on the lookout for the next best advancement.

I wanted to share my story with all of you. No one should have to endure all of this unnecessary pain, embarrassment, humiliation and just plain danger. Had I known anything about Cushing prior to my diagnosis, there is a very good chance so much of this could have been avoided.

Think of this as a cautionary tale. I cannot emphasize how important it is to educate yourself. If I have taken anything away from my experience, that would be it. It’s ok to question things. Doctors are people just like us. Some are good, some are not so good. During the month of April and Cushing awareness please remember people like me. We have so much knowledge at our fingertips with the internet…please use it. It could be life-saving and life-changing all at once.


Posted on

September 29, 2020