The initial treatment addressed most of my symptoms. About six months after the initial symptoms I was feeling close to normal.  The only indications that I had Cronkhite-Canada Syndrome was my nails were still not normal. My thumbs and index fingers were “clubbed.” They would grow real thick.  Every week or so I took a heavy duty emory board and sanded them down. I also had a scar to the right of my belly button – starting about 3 inches below and extending to about 6 inches above. This was related to having my ascending Colon removed.

During a follow-up colonoscopy and endoscopy – I believe the third from Emory over about a 5 month period,- for it took place a few days before the 4th of July, my Colon was perforated. The colonoscopy identified an area of concern. The doctor had taken many biopsies and there was a particular area of my ascending colon she was concerned with. Before I left to go home from the colonoscopy she had made me an appointment to have another procedure done (I can’t remember if it was MRI or CCT) – I had to drink some nasty contrast before the procedure.  I believe the Colonoscopy was on a Tuesday and the MRI/CCT was scheduled for Friday. Thursday was the 4th of July. We went to the fireworks like we always did and had a good time.

I woke up about 3am with a significant pain on my side. I got up and took some aspirin and got dressed. My appointment was for an 8am procedure. I left for the clinic around 6:30 and called the doctor around 7am. She answered her personal cell phone that she had given me in case of an emergency. I told her what was my symptom. She said she would call the clinic as she wanted someone to look at the results as soon as possible and that I should call her after the procedures. Which I did.

I called her on my way home, just after I left the hospital. She asked that I not go home, to park close by and she would call me after she talked with the doctor that would interpret the test results.  In hind sight I believe she had a good idea of what had occurred.

She called me back about 20 minutes later. The test had identified the colon was perforated in the area where she had taken several biopsies. It appeared that part of the colon was diseased.  I turned around and checked myself in to the hospital. Later that day the infected area was drained. A tube was left in for additional drainage – but there was little drainage after the it was initially drained. I was on an IV and an antibiotic for 8 days.

A few weeks after I left the hospital I was back – to get my Ascending Colon removed. At this point even though I was not having any external symptoms I had to many polyps to count. In some places on the color pictures from the colonoscopy there was as much white (the polyps) as pink – the healthy condition. The removal of the Ascending Colon was uneventful. The reason for the big scar was the doctor wanted to get a good look at my colon to get a better sense of the overall condition – that was okay.

Initially the doctor had talked about removing more of the colon – but I would only agree to remove  what was dieased.  He ended up agreeing that was the best approach.

I have an annual colonoscopy and endoscopy. So I had the procedures completed about a year after my surgery to remove my ascending colon. This was around 18-20 months after my first symptoms. The results were positive in that the number of polyps in my colon were significantly reduced. There were fewer in my stomach but still many. The duodenum was worse. This is a pattern that occurred a couple of times.   Initially the colon was worse, then the stomach and then the small intestine. The remission followed this same pattern – with the colon the first area to go in to remission.

You can debate if my Cronkhite-Canada Syndrome has ever went in to remission – in that I have never been polyp free since the initial on-set. However, for 6-7 years I had no external symptoms and the number of polyps in my colon and stomach continued to be few. I always had a section in the duodenum with many polyps.

The number of polyps in my second annual set of procedures were significantly better in both my colon and stomach. For several years I had a few polyps in my colon that were often removed during the colonoscopy. This ranged from 3-4 to 10-12. A few times they were not removed but were documented and watched. They were always benign. Sometime around year four or five I actually did not have any polyps in my colon. Around 3-4 were identified in the prior colonoscopy. The doctor was amazed as this was the only time that she had ever seen polyps go away.  The number of polyps in my stomach at that time were minimal. At this time you might of considered me in remission as I was having no symptoms, no polyps in my colon, a few in my stomach and the duodenum had also improved.

During this time my treatment was unchanged. I was taking the three presciptions; sulindac, rantidine and loratadine. Vitamins B12 and D along with a general vitamin. I had annually both a colonoscopy and endoscopy. Generally there were a few polyps – but not many.

In the fall of 2015 I had my annual colonoscopy/endoscopy.  For the prior 2-3 years I had either no or few polyps in my colon, a few in my stomach and many in the duodenum. Over the last year one symptom had reappeared.  My nails – while not dying – were no longer normal.  I had to cut them frequently or they would break or become ingrown.  The nails on my thumbs were a little thicker.  Before the procedures I told the doctor that unlike the previous years where I always thought there would be no polyps – I thought there would be more – based on my nails.  Unfortunately, I was right.

I had many polyps identified during my 2015 procedures.  In the colon in the range of 15, in the stomach there were to many to count.  There was also an increase in the duodenum.  It was not welcome news.

The doctors recommendation was to treat the new polyps aggressively with additional medications.  Two new prescriptions were added; Lialda (mesalamine) and mercaptopurine.  My perception is these drugs have the potential for higher side affects or consequences for the medications I have been taking for a long time – NSAID, Zantac and Claratin – are taken for years by many individuals .

In the fall of 2016 my annual colonoscopy and endoscopy was performed.  There was a slight improvement in there were a few less polyps in the colon, the stomach and duodenum were about the same, with the duodenum having many polyps.

The primary external symptom continues to be my nails.  I do have mild bouts of diarrhea from time-to-time – generally 2-3 months apart. I have no other symptoms.

For the last several years my liver enzymes have not been normal – but just a little high.  During my normal physical in the summer of 2015 the blood tests identified continued high values for AST and ALT. My doctor thought is was time to look at this in more detail and referred me to a hemotolgist.  He defined many blood tests to be performed.  They identified that my iron levels were high – very high.  The normal range for Ferritin is 24-336. My test results in July were a little above this range. I had many blood tests performed over the next several months. In March of 2016 my levels were over 3.300 – almost 10 times what was considered normal.  Additional tests were performed to try to identify the root cause of the high iron levels.  One of the tests would have identified if hemachromotis was the cause – the test was negative for this condition.  A liver biopsy was performed – the biopsy showed my liver was normal – with a little fatty liver. The doctor said all of the test results were consistent with hemochromotis – that I had tested negative for.

The net is the root cause was not identified.  The cause is likely related to either Cronkhite-Canada Syndrome or one of the medications.  The hematologist believes CCS is the likely cause as there is a strong relationship between the intestinal system and the liver.

I had eight phlebotomies (one a week) that brought my Ferritin levels down in to the normal range.  The current approach is to have blood tests performed every 8 weeks with one phlebotomy performed every six months.  My iron remains in the normal range – though not as low as the doctor originally targeted.  I meet with the doctor in a few weeks to determine if I will continue on this schedule.

At the risk of over simplification or being inaccurate – I will attempt,  to define a “syndrome” and why it is different than a “disease.”  My understanding is that a disease has a known cause.

A syndrome is defined by it’s symptoms – so for Cronkhite-Canada Syndrome, it would include the hundreds of polyps, weight loss, hair loss, loss of appetite, hyperpigmentation, loss of nails, etc.  However the cause of those symptoms is not known.

So if you have a disease and somone else has the same disease – you would be having similar reactions to something similar that is going on in your bodies.  If you and someone else have the same syndrome you would both have the same symptoms – but the cause of the symptoms would not be known.

I read something that made the most sense to me – it was a progression of the understanding of something that is happening in the body.  From least understood to most understood – the progression was; symptom, syndrome, disorder and disease.

I have talked with two other individuals that also had or have Cronkhite-Canada Syndrome.  One person sold school buses and my sister managed a trade show for school principals and school boards.  Hundreds of suppliers to school systems participate in the show, from selling milk to selling buses.  This individual was at all of the shows my sister managed until a few years ago when he talked with my sister and said he could not attend for he had this strange illness with no known cause.  He started to describe his symptoms.  My sister told him her brother also had a similar strange illness.

To shorten the story he also had Cronkhite-Canada Syndrome and called me.  We talked several times.  About a year  before I started having the CCS symptoms – I had an accident and broke both legs close to my ankles (fibula and tibia in my left leg and the tibia in my right).  I had issues with my left leg healing correctly.  I had four surgeries over about a years time.

Bob, the bus salesman, had a bad motorcycle accident – I believe it was about 1-2 years before he started having CCS symptoms.  Likely a coincidence – but who knows.
I asked Ron, the other person that had Cronkhite-Canada Syndrome I talked with, if he had any trauma in his life.  The answer was no – the first time he had every been in a hospital was when he was admitted to perform TPN – to provide him nutrients for he had lost 90 pounds.

So with a sample of two I thought it was possible that trauma played a role. With a sample of three – likely not.