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Use of infliximab in CCS

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Introduction

Due to the rarity of Cronkhite-Canada syndrome (CCS) it is not a well understood disorder partly due to difficulties in gathering a large number of patients in order to perform robust studies. Therefore there is no strong evidence for specific treatments, no clear understanding of the aetiology (the cause), and the pathogenesis is relatively unknown (the biochemical steps that underlies the disorder). There is some evidence that CCS is an autoimmune disorder, meaning that something causes the bodies own immune system to start targeting itself1–3. Treatment for CCS is largely anecdotal, generally composed of nutritional support, steroids, or immune suppression4.

Steroids

Currently most patients are treated with glucocorticoids (part of the steroid “family”; commonly known as corticosteroids) which include medications such as prednisone, prednisolone, and cortisone. It is also used in many other disorders such as rheumatoid arthritis, sarcoidosis, and inflammatory bowel disease. The rationale behind this, is that the corticosteroids suppress the immune system by suppressing T cell activation, inducing lymphocyte death, and altering lymphocyte recirculation. By decreasing the immune response, there should be a decrease in inflammation resulting in a decrease in cellular damage and symptoms5. However this is generally for symptomatic relief meaning it can help ablate the CCS symptoms of diarrhoea and malabsorption whilst it doesn’t deal with the underlying cause of this disorder.

Anti-TNFα

Tumor necrosis factor alpha (TNFα) is a cytokine known to be a key part of the inflammatory process. It is also associated with autoimmune disorders such as rheumatoid arthritis, ankylosing spondylitis, and inflammatory bowel disease as it essentially increases the inflammation. Although the exact cause and mechanism of CCS is still unknown, a 2010 case report highlighted a patient with elevated levels of TNFα, providing some evidence that it plays an important role in the development of this disease6.

Infliximab (trade name: remicade) is an anti-TNFα drug. It targets the aforementioned TNFα and prevents it from activating. The drug is known as a monoclonal antibody – it is an antibody made in the lab, so it is like a homing missile targeted and specific just for TNF molecule. Due to this, it is also relatively expensive. It is believed that if TNFα is blocked by infliximab or other “anti-TNF” drugs, then this stops a key step of the CCS pathway (inflammation) allowing time to heal and thus halting progression.

Following this 2010 report, there have been 3 reports in the literature of anti-TNFα being used. They are summarised below.

  1. Tran et al. (2010)7: first case report of infliximab being used for refractory CCS. The patient was a 37 year old male with 45 lb weight loss and low albumin. He was treated with oral prednisone, subcutaneous octreotide, antibiotics, ranitidine, and cromolyn, however over a 12 month period his symptoms continued to wax and wane, eventually leading to a relapse. As this was not responding to treatment it is known as refractory. Infliximab and total parenteral nutrition (TPN) was started and at 12 weeks the patient displayed weight gain and albumin increase. After 12 months of infliximab therapy the patient was asymptomatic.

  2. Watanabe et al. (2014)8: a 57 yo male with severe CCS was treated with corticosteroids, however symptoms persisted. Infliximab was used “His general condition improved, and colonoscopy performed 20 months after induction of anti-TNF therapy showed complete remission with the disappearance of polyposis. He now receives 200 mg of infliximab every 8 weeks, and has been symptom free for 3 years since the initial administration”.

  3. Boland et al. (2016)4: a 40 yo female presented with CCS. TPN and intravenous steroids were administered without improvement. Azathioprine and infliximab regiments were then started which resulted in rapid clinical improvement.

To conclude, there are now three reported cases of infliximab being used to very good effect. However a sample size of 3 is not large and depending on the country infliximab can be very expensive. Further research must be performed investigating the use of infliximab as a first line therapy for CCS.

References

1. Zong, Y. et al. Case report—malignant transformation in cronkhite–canada syndrome polyp: Medicine 96, e6051 (2017).

2. Ota, S. et al. Cronkhite–canada syndrome showing elevated levels of antinuclear and anticentromere antibody. Clinical Journal of Gastroenterology 8, 29–34 (2015).

3. Kopáčová, M. et al. Cronkhite-canada syndrome: Review of the literature. Gastroenterology Research and Practice 2013, 1–9 (2013).

4. Boland, B. S. et al. Cronkhite canada syndrome: Significant response to infliximab and a possible clue to pathogenesis. The American Journal of Gastroenterology 111, 746–748 (2016).

5. Head, B. M. Experimental and clinical pharmacology: Corticosteroids in autoimmune disease. Australian Prescriber 22, 9–11 (1999).

6. Martinek, J. et al. A fulminant course of cronkhite-canada syndrome. Endoscopy 42, E350–E351 (2010).

7. Tran, V. T., Reicher, S., Chung, D. S., Eysselein, V. E. & Pham, B. V. M1252 infliximab therapy in refractory cronkhite canada syndrome. Gastroenterology 138, S–364 (2010).

8. Watanabe, D. et al. Successful treatment of cronkhite–canada syndrome using anti-tumor necrosis factor antibody therapy. Endoscopy 46, E476–E477 (2014).

Author

Please contact Aaron Khoo akhoo1@uq.edu.au if there are any queries or concerns.

Tom’s Story

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Personal Background

I was born in December 1949. My father was a piano tuner and mother was a piano teacher.  She got them out of tune and my dad put them back in tune. I have one younger brother.  Before we were born my father was in the Navy and was a tail gunner in WW II. After the war he was transferred to Alaska and served his remaining time in the Navy as a carpenter building barracks in the Aleutian island chain.  My mother worked in a ship yard during the war in Seattle and was a riveter. My brother and I were born and raised in Amarillo, Texas.  My dad made one of the bedrooms a studio where my mother taught in our home.  She was a stay at home mom and was able to work at home.  My brother and I were raised in a loving musical family. My dad sang in the church choir and mom was a substitute church pianist and organist. We were taught early on about volunteering, helping others, mowing lawns and shoveling snow in the winter.  Looking back I consider myself lucky to have had such supportive and guiding parents.

 

I was married to Jana in 1988 and we have one son.  When he reached 6 years old we decided to home school him.  When he was in the first grade, the aerospace company I worked for spun off the aircraft support group I was in to another company in New Hampshire and I wanted to stay with the original company.   I started looking within the company and found a position as an avionics maintenance field engineer in international programs.  I found a position to support aircraft in Taiwan.  Jana had previously taught grade school and had lived overseas before and knew how to network.  She established relationships with some homeschool groups over the internet before we ever left the US.  We ended up living in Taiwan for 8 years before coming back home.  I could not have done it without her.  I sometimes wonder if living there had anything to do with my current condition. There were many factories and you would hear about liver problems and cancer from airmen  While living in Taiwan we became very active SCUBA divers. In December 2004, we moved back to Texas and in 2013 after 30 years of service, I retired.

Life Changes

I was 55 when we came back from Taiwan in December 2004. The following year we planned a SCUBA diving trip to Bonaire. Before the time came to go I had a gall bladder attack and ended up in the hospital and had to have my gall bladder removed. This was the second time I had been in the hospital.  The first time was in Taiwan where I had kidney stones.  In 2014 we decided to sell our house and move to the Houston area.  It was a lot of work to prepare and de-hoard 30 years of acquiring stuff.  By the summer, I started showing signs of jaundice.  I had a few instances of abdominal pain late spring but didn’t think it was anything serious. On July 13, 2014, Jana, my mother-in-law and our son were looking at me across the table and told me I was yellow.   I had not been feeling good for several weeks and so we went to the Emergency Room at Harris Methodist Hospital in Fort Worth, TX.  I was examined and admitted with a diagnosis of acute pancreatitis, Jaundice, and Elevated LFTs. Dr. Virat Dave (pronounced Dahvay) suspected I had a blocked bile duct. They did a CT scan, Ultrasound, and MRI but could not find any blockage.  For 5 days I was on saline, vitamins and antibiotics intravenously.  The doctor told me this would give my GI system a rest.  After release from the hospital I had to go back every week to the Gastrointestinal doctor (Dr. Dave) for a blood panel test for liver and pancreas.  It took six weeks for the blood tests to return to normal.  After that, I felt much better and we finished getting our house in top shape for viewing and listed it with a realtor. It sold quickly and we rented a storage space and moved most of the stuff in the heat of summer.

Retirement

We moved into an apartment for six months and de-hoarded some more.  While I was still working we bought a 40 year old sail boat and had it trucked to a local lake in Fort Worth.  It needed a lot of work and I worked on it a lot and by the time we sold our house we had moved it down to Clear Lake in the Houston area.  After six months we moved on to Adagio Gul, our sailboat, with our two cats.  We thought we would be cruising within six months but ended up living on the sailboat for two and a half years.  We had to install solar panels, update the navigation instruments and practically refit and upgrade the whole boat.

Changes in Health

In 2017, we gave one of our cars to our son and the other one to my brother.  Our goal was to eventually get down to the Florida Keys but we told everyone ‘Let’s just get to Louisiana’.  In February, I had finished replacing a head gasket on the 4 cylinder diesel engine. It took three months. In January 2017 I had a complete physical and everything was good.  We left May 10, 2017 and missed a weather window to go off shore so we went the Intercostal Water Way (ICWW) instead.  On May 17, we were circling around waiting for a barge to come through the Leland Bowman Lock and ran aground.  I lowered the dinghy and expended a lot of energy trying to kedge Adagio Gul over to pull her off but to no avail.  I wore myself out and put the dinghy back in the davits before dark.  It was real hot that night. This may have been the trigger event to start my CCS plus my eating habits weren’t that good either.  I would boil an egg and put lots of Worcestershire sauce on it. (lots of vinegar) I put Tabasco sauce on everything.  I started noticing about 3 hours after breakfast I would feel like I had hunger pangs and would eat a sandwich or some crackers to make the feeling go away.  Since living on Adagio Gul I would have a cup of strong French press coffee and a cinnamon raisin bagel every morning.  Early the next morning a barge came by and caused waves and lifted Adagio Gul up and down.  We started her up and were able to back into deeper water.  We went through the Leland Bowman Lock May 18th and on through Louisiana. 

 

The locks in New Orleans lifting us up to the Mississippi and down to the other side were very interesting and somewhat stressful.  We went through the Mississippi river in a thunderstorm and while Jana was at the helm I had to go below and add transmission oil to the transmission with the engine running.  When I came up into the cockpit I saw a big log go by and water was swirling and really moving.  Then we hit something and noticed the prop was unbalanced.  We made it to Seabrook Harbor Marina and boatyard late that night and decided to haul out two days later.  Sure enough there was a chunk out of the prop.  We removed the prop and the boat yard sent it out to a prop shop for repair.  The boatyard let us and the cats stay aboard Adagio Gul and since we were not in the water we could not use the AC so it was hot.  After repairs we splashed back in and went into the Mississippi Sound and barrier islands.  On June 25th, 2017 we got to Dauphin Island in Alabama we anchored out in the afternoon and late in the evening the wind changed directions. We had to reset the anchor.  Jana at the helm and I at the bow.  This was one of those times when it took several tries to reset the anchor.  It was midnight and I was very tired by the time we had reset the anchor.  I was puzzled about how tired I was.  We did not sleep very well that night.  The next day we left early and made it to Lulu’s, a marina owned by Jimmy Buffet’s sister.

 

Sailing and cruising is one of our dreams but maybe the stress of the heat and physical exertion may have contributed to the onset of the CCS.

 

Over July 4th, we stayed at Barber Marina.  It was here that we got out our folding bicycles and pedaled 5 miles in 95 degree heat to a small marina for a cheeseburger and a pizza to take back for later.  When we left Barber Marina we left the ICWW at Pensacola and went out into the Gulf of Mexico and cruised overnight and arrived in Panama City the next morning.  We were dog tired and when we got there we tied up, checked in, walked to a small restaurant, had breakfast and came back and crashed.  At breakfast I noticed my taste was blunted and everything tasted the same.  The next day is when I started feeling bad in my stomach. I called my Health Maintenance Organization HMO back in Houston and discovered that the only option they would cover is going to the ER at a local hospital. I waited two days but finally decided I needed to go.  I had lost 12 pounds.  A couple who we became good friends with took us to Gulf Coast Medical Hospital and they checked me for diabetes, heart attack, and did a CT scan.  The ER doctor prescribed Lansoprazole 15mg for 14 days and wrote me a prescription to have an endoscopy.  I felt better on the Lansoprazole but after 14 days the same feeling came back and I had diarrhea.

 

We decided to rent a car and go back to Texas.  My mother-in-law had two empty bedrooms so she let us stay there.  We left the cats with my mother-in-law in Mansfield, TX and drove to Houston to see my primary care doctor.  He sent me to Quest Diagnostics for a blood panel and referred me to a GI doctor for an endoscopy. I had lost 4 more pounds. He prescribed two different antibiotics and 40mg time release omeprazole. The endoscopy appointment was 2 weeks later.  We drove back to Mansfield and two weeks later drove the 250 miles back down to Houston for the endoscopy. I had lost 4 more pounds. Four samples were taken during the endoscopy.  The endoscopy pathology report was negative for H Pylori with marked erosive duodenitis, and inflamed and eroded hyperplastic polyp with no evidence of carcinoma or lymphoma, and chronic esophagitis. We drove back to Mansfield and after evaluation of the endoscopy it was decided I should have a EUS endoscopy. They wanted to look at my pancreas with ultrasound.  It was scheduled for three weeks later. When I showed up for the EUS endoscopy I had lost 6 more pounds. This was a total of 26 pounds. All food had no taste and I was tired all the time and had no stamina.  I noticed a big indentation on my right index finger fingernail and reasoned the distance from the indentation to my cuticle was a time marker for when my condition started.  I noticed my hair was starting to get brittle and thin and the palms of my hands had large freckles under my skin. The doctors called it hyperpigmentation. Other symptoms were toes and fingers tingling at night and just a general tired and weak feeling all over.  The EUS endoscopy diagnosis was Celiac Disease but they sent me to Quest Diagnostics for a celiac blood panel to confirm the diagnosis.  We drove back to Mansfield and waited for blood panel results.  It was negative.

 

At this time Jana and my sister-in-law applied online to Mayo Clinic in Rochester MN and gave the Internal Medicine group details and they sent me a questionnaire. I filled it out and was accepted for a 01 November, 2017 appointment.  The reason for Mayo Clinic was because my mother-in-law had 12 surgeries at Mayo Clinic at Rochester. Jana and her sister traveled with her on a few trips and they experienced first-hand how excellent the patient care was.  We flew there and after a multitude of tests the diagnosis was Cronkhite-Canada Syndrome. The Dermatology department took pictures of my fingernails and took a swab sample from my mouth. The pathology report from that revealed major Candida albicans yeast blooms and they told me that was what was blunting my taste! They prescribed 10mg Clotrimazole anti-yeast lozenges (troches) 3 times daily. After returning home and a week after taking these lozenges my taste returned!  Food started tasting good again. They also gave me two iron infusions because my iron was so low I was borderline anemic.  They started me on a prednisone tapering regimen. It began at 40mg per day, taken first thing when I woke up and each week I would step down 5mg per week. We flew back to Mansfield and I started on the prednisone. I started looking at labels on all foods.  I looked at my blood panel tests and noted what elements and nutrients I was low in and sought out food that had what I needed. It was a big adjustment but I started a food log so I could look back the previous day and determine what produced solid stools and what produced gurgling stomach and diarrhea.  While I was at Mayo Clinic I visited the Barbara Woodward Lips patient education center and read a lot between appointments.  I decided I should try to stop all consumption of alcohol.  One thing that I knew but hadn’t taken seriously was that I was not drinking near enough water.  It is recommended to drink a minimum of 8 glasses of water a day. A glass is 8oz.  It took several days for me to adjust to that amount.  I started eating 5 or 6 times a day. All small meals.  10 minutes before I got up I would take a chewy gummy multivitamin and a chewy gummy calcium-vitamin D.  I think there is better chance of absorption when your saliva mixes with the chewy gummies versus swallowing a pill vitamin. Then I take a 20mg pepcid, 50mg of zinc, and chase it with a glass of water and lie in bed with several pillows propping me up. Then I get up after 10 minutes, weigh myself, heat water, and while it is heating I feed the cats. I put in a bag of green tea (helps reduce inflammation) when the water is boiling and let it steep while I prepare a serving of Cheerios mixed with a serving of All Bran and a serving of raisins. I pour 8oz of 2% milk in the mix and that is breakfast.  Lately I have been adding a banana on the side. I was low in potassium, iron, and calcium. These cereals are fortified with potassium, iron, B6, B12, and many other nutrients. I take my prednisone dose with breakfast.  I try to not have any food two hours before bedtime. Right before bed I take another 20mg pepcid.  After two weeks of carefully planned meals I started gaining weight. My stamina and strength started to return. I started walking and was able to gradually lengthen the walks each week until I was up to 6 to 7 thousand steps a day.

 

The third week in February when I was on 10mg of prednisone per day we flew back up to Rochester for a follow up colonoscopy/endoscopy and the inflammation was much reduced but there were still too many polyps. The next day blood panel revealed my iron levels were two points below the normal range which were much improved.

 

The prednisone tapering regimen plan is when I get down to 5mg per week I will step down in 1mg increments until I get to 0.  If my symptoms return I would let my doctor at Mayo Clinic know and I would be switched over to Imuran which is a trade name for Azathioprine.  I am at 4mg per day per week now and so far so good. I have gained 25 pounds back.  I weigh first thing when I get up in the morning.  I am also taking 20mg Over the Counter OTC Famotidine which is Pepcid when I wake up in the morning and before I go to bed. Sometimes if we go out to eat or plan on eating a spicy meal I will take a Pepcid before the meal.  I’ve stopped all alcohol use and also very spicy foods.  I expect to be on some maintenance medication in the end because CCS is auto immune and the condition will most likely require some medication to keep it in remission.

Ron’s Story

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Cronkhite – Canada Syndrome

My experience living with a diagnosis of a rare condition known as Cronkhite – Canada Syndrome (CCS)

 INTRODUCTION :

What is CCS ?

In 1955 two doctors one by the name of Leonard Cronkhite and the other Wilma Canada discovered CCS. Since 1955 there have only been approximately 450 – 500 known cases in the world of this condition.

Most of the cases have been found in Japan. The ratio is estimated to be 1 case per million population. The onset of CCS occurs on average around the age of 59. The range of age is 31 to 86.

 MY PERSONAL BACKGROUND  :

Born in May 1957, I was diagnosed with the CCS in 2016 at the age of, you guessed it, 59.

I was very privileged to be raised by two great and supportive parents, a stay at home Mom and a hard working father who by trade was an electrician and dairy farmer. I have one older brother and a younger sister. We were all raised in a very loving family environment. We were taught that honesty and hard work are the way to succeed in life.

Married in 1981 to Nancy, together we have raised three, very highly motivated, career oriented children, two daughters and a son. We are very proud of all of them and were blessed with our first grandchild in November of 2015.

Life it seemed, for my first 59 years, was textbook. I enjoyed playing various sports, but mainly baseball, golf, hockey and curling. I also played in a band for a number of years until our children were old enough to start their own lives in an active manner. Once they were old enough to play sports, I became involved in coaching their sports teams while they were growing up. Both my wife and I tried to stay involved in their lives. My wife and I also enjoyed a very active social life and very much valued the many friendships we have established over the years and still do.

I retired in April of 2015 at the age of 58 after 35 years of service with a public sector employer. I was rarely sick as was evident of the fact that I rarely if ever took a sick day, and was rarely if ever late for work. My career in the public sector environment included the operation of vehicles for the purpose of road and highway maintenance. My responsibilities included supervising various road related projects and finally for the past number of years was the maintenance manager for the Department, managing a large staff of employees, equipment, housing and a very large municipal budget.

EARLY SIGNS of PROBEMS AHEAD            

For the first 58 years of my life I was very healthy, having never spent an overnight in a hospital and breaking only one bone (a finger) over the many years that I played sports. I lived a very active lifestyle.

The first sign of problems ahead came in September of 2015 while attending a family function. I noticed that I could not eat or drink very much and started to feel very severely bloated. I left the gathering and went for a walk but the feeling persisted. Later that afternoon we went to my daughter’s place where I tried again to walk off the severe bloating feeling. That evening we went out for dinner and I still could not eat a thing.  After a good night’s sleep, I felt much better and the bloating feeling never really returned to that extreme.

A few days later I started to experience bleeding in my stools. Initially this did not trouble me as I had had issues like this in the past and they always were treated with over the counter products. However this time was different, these treatments did not work and the condition did not improve. I went to my doctor who assured me that there was nothing to worry about and prescribed over the counter medications. Again after a couple of weeks the condition did not improve.

(My weight at his time was 240 lbs.)

During the first week in November 2015, I knew something was really wrong when I lost my ability to taste food. Food started to taste metallic, really awful, nothing tasted as it should. I made an appointment to see my family doctor. I told him that the bleeding problems were no better and that now I had lost my ability to taste food as well as my appetite. I literally told him that he needed to book me for a colonoscopy, something that he had been reluctant to do because of the results I had received from stool sample screening that he had me complete every two years.

I must mentioned that, at this period in time, my mental state, for the first time in my life, had become very fragile. I started to feel a great deal of anxiety. I will say that in order to counteract the negative feelings I was having over my deteriorating health ( Weight Loss, Inability to Taste Food and lack of appetite) I went for many, many long walks. Even during some very stormy weather, I would get out and walk to relieve the anxiety of what was happening to my health. Little did I know that my journey was just beginning.

 On December 12th   2015 I had my first colonoscopy, a week later the surgeon told me that he had removed three very large suspicious polyps, luckily none of them were cancerous. However he stated that I was a very UNIQUE individual. Not being sure what he meant by this, he explained that he had never seen so many polyps in a colon before. He explained that as a result of what he had discovered in the scope of my colon, there was an immediate need for him to go back in and remove more polyps.

On December 22, 2015 I had my second colonoscopy, There were an additional 11 polyps removed. On January 22, 2016 the results of the pathology report were revealed. Thankfully the report indicated that all of the polyps removed were benign. However, due to the number of polyps still in my colon the surgeon wanted me to see a geneticist to have testing done, which I agreed to. The request was made at this time by the surgeon, however I never heard from the geneticist in London until June 2016. I was booked for an appointment at St Joseph’s Hospital in London in mid July 2016.

It was during this period of time (January 22, 2016) that I made a conscious effort to take a more positive approach to my feelings of negativity about my deteriorating health. I decided that I needed to begin to think positive and work toward a solution of trying to regain my health and search deeper for the reason behind my condition. Little did I know that it would be a few more months before the answers would come. However thinking more positively, greatly improved and totally eliminated my anxiety.

In mid February 2016  I started to experience constant diarrhea. I went to the emergency department at the hospital after about a week of this condition. Tests, as I was told by the doctors, determined that there was nothing wrong with me and I was prescribed probiotics to correct the problem. However they did not work.  By this time I had lost in the neighborhood of about 30 lbs since November 2015.

 The emergency doctor while conducting this examination discovered that my blood pressure was extremely low. I had up to this point always had normal blood pressure. This condition would exist for many months.

By March 2016, new symptoms were starting to surface, my fingernails were starting to deteriorate. I questioned my doctor about this and he had no idea what was causing this. Shortly thereafter I noticed that my hair became very brittle and started to fall out. By the end of March (2016) there was not one hair left on my body. Eyelashes were gone, all body and facial hair were also gone.

It was becoming very difficult, mentally, to comprehend what was happening to me. I was being questioned almost daily from staff at my workplace wondering if I was OK. My answer was, to the best of my knowledge and what doctors were telling me, there is nothing wrong with me that could be explained.

During this period of time, I had continued to finish out the curling season at our curling club. There was one guy that I had known for a few years that I had been curling with. He and his wife had left for a couple of weeks on a vacation, when they returned to the curling cub he did not recognize me, as we curled on the ice the night of their return. My weight loss along with my hair loss had changed my appearance to the point where people that had known me for many years had difficulty recognizing me.

The Doctors continued to search for answers, to the point where I had completed ultra sound on all my vital organs, nothing of any concern was found.

On APRIL 29th I retired from my job of 35 years. This decision had been made many months before. My decision to retire had nothing to do with the illness that was consuming me.

The waiting game continued until June 2016 when I finally received a notice in the mail informing me that an appointment was set up for me for mid July with a Geneticist in London. After meeting with the Geneticist, it was determined through doctors reports and research by the geneticist that I could possibly have a condition called Cronkhite Canada Syndrome.

To try and determine the validity of this diagnosis, I was set up to meet with Dr Hussein a Gastrologist in London Ontario,  on the first week of August. He would conduct an Endoscopic Procedure on my stomach as well as another Colonoscopy.

It was determined after these procedures that I indeed had Cronkhite Canada Syndrome. The next day I was immediately sent to Doctor Adam Rahman in the London Health Sciences Center who would become the main physician who was going to be treating me for this condition moving forward.

December 2016

By mid December 2016, I was very weak. I had lost over 95 lbs since October 2015. Due to my alarming weight loss and fast deteriorating heath, a conference call between my wife and I and Doctor Rahman was set up.  My wife explained to him that something needed to be done immediately due to my frail condition. Dr Rahman asked me if I would agree to start a treatment of Imuran and prednizone knowing the risk factors. I agreed, saying that I had nothing to lose.

Started taking IMURAN and PREDNIZONE

January 2017

I was admitted to the London Health Sciences Center January 13th, 2017  to have a PICC line inserted so that I could start TPN treatment (12 hour infusions per day of nutrients directly into my bloodstream). My weight at this point was about 145 lbs. I spent 6 days in the hospital until the nutritionists were satisfied that the correct level of TPN had been achieved.

Within 5 weeks I had regained not only some much needed energy I had gained approximately 30 lbs and by mid to late February was up to approximately 180 lbs. I was feeling much better physically and had much needed energy and strength back in my legs and body to start getting out and about.

February 2017

By the end of February I was totally weaned off of Prednisone. (No longer taking IMURAN by this time)

March 2017

Started REMICADE treatment on March 14, 2017. I received the first two infusions over the next two weeks then was put on a schedule of taking an infusion once every 8 weeks and continue on this treatment schedule to date.

My weight by late October 2017 was between 230 lbs and am having to exercise and watch my diet to not only keep my weight down, but help to regulate a high cholesterol count in my blood tests. My fingernails have regrown and my hair has come back to pre- November 2015 condition.

November 2017

I went through a colonoscopy and an endoscopic procedure on November 1st at London University Hospital by Doctor Rahman so he could look at the progress and my recovery to health over the past several months.

The procedures indicted that the polyps in both the stomach and colon have been significantly reduced in size since my last colonoscopy. I was scheduled to return to London on March 28, 2018 to possibly have some of the larger remaining polyps removed by Dr. Hussein.

March 2018

Dr. Hussein at St Joe’s Hospital conducted an endoscopic procedure on my stomach and a colonoscopy. It was determined by Dr Hussein that the remaining polyps have been reduced in size but there are still many polyps. It was decided that I would return to his office on April 24, 2018 to find out the results of biopsies of some of the polyps that he took samples of. We will then discuss the path forward and how we should proceed with determining how to deal with the remaining polyps.

Surgery is scheduled for June 1st 2018 to remove more polyps.

END  (April 3,2018)

To Be Continued

My Story

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The objective of this web site is to provide information about Cronkhite-Canada Syndrome (CCS) based on my experience as someone that has CCS. There is limited information available regarding Cronkhite Canada Syndrome as it occur’s so rarely.  I am not aware of any research that is being performed to better understand CCS. I found a single instance several years ago where a doctor at the University of Texas may have been doing some basic search, using finger nail clippings, but it appears he lost his funding and the research was stopped. Most of the information available on-line is a summary of literature for no primary research is being performed. Thus the value of the information to someone that has or believes they have CCS is limited. If you have CCS, the available literature may cause you concern when you read of a 50% survival rate.

I have lived with Cronkhite Canada Syndrome for almost 10 years and to a large extent symptom free. I am not trying to provide medical information or suggestions. I am trying to share my experience – for example, how my diagnosis was made, the treatments I continue to take and the results to date. Lastly, feel free to contact me via this web site. I would be more than happy to talk with anyone who has CCS for we belong to a small club.

Thanks,

Pete Densmore

Symptoms

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The week before Valentines day I had been traveling on business.  At the end of that week I had a bout of diarrhea. That was my first symptom of Cronkhite-Canada Syndrome (CCS), though I did not know it at the time. That was in February of 2006. The second symptom was the following week. I sat down to a nice Valentines meal with my wife. I believe it was a Caesar salad and a nice steak. I did not eat it all. My wife noticed right away – for I did not often leave steak on my plate. I made a statement at the time – that in hindsight should of made me think something was not right – but did not. I told my wife the steak tasted “tinny.” For the next several months food did not taste good.

I saw an article recently about a company that was trying to farm a berry that made food taste good. Their target market was cancer patients. It appears that for some cancer patients the food tastes “tinny.” I found that interesting.  Here is a link….  http://www.miamiherald.com/news/local/community/miami-dade/homestead/article122716684.html

While the first two symptoms were fairly close together. It was 4-5 weeks before the third symptom occurred. During that time – with diarrhea that was getting progressively worse – I had visited my doctor – a general practitioner in a small town close to where I lived. He had referred me to a gastrointestinal specialist. He prescribed some medicine that had no effect and after another few weeks he performed a colonoscopy. Amazingly he did not identify anything amiss – other than colitis. It was not long after my colonoscopy that the third symptom appeared.

I lost my hair – all of it – not an eye lash left – in a matter of a week. The shower drain would clog when I took a shower.  The day after my hair started to fall out I was able to get in to my doctor as I was quite concerned with what was going. He referred me to a Dermatologist specialist, that confirmed I had alopecia. and I would lose all of my hair.  After 3-4 days I went and had my head shaved.

I knew that As soon as my hair began to fall out there was something significantly wrong. My diarhia was bad and getting worse. The idea of food was awful. I tried all kinds of food – all tasted bad. My go to diet, that I believe played an important role in my health during this time, was Gatorade and Ensure. I got to where I really disliked the taste of Ensure. I tried all flavors – some were better than others – but it was easy for me to chug an ensure. I would try to drink 4-5 ensures a day. I drank Gatorade throughout the day. With no information to support it – I believe this diet was very helpful – for while I was losing weight – I never felt weak. Though this diet did little to help my diarhia.

I was now also bald and in this first 4-5 weeks I lost around 20 pounds. I am not sure when the next symptom occurred, for it was so mild. My skin, and I mostly saw it on my hands, became almost baby smooth and was pigmented, blotchy.

The above four symptoms appear in many gastrointestinal diseases. I believe the last symptom is unique to CCS and it allowed me to self diagnosis CCS.  My nails began to die about a third to a half way up – there was a red line that divided the healthy nail from what was not. It happened in both my finger and toe nails. The finger nails were first and the toe nails were a few weeks later.

The above symptoms all occurred in about a 4-6 week period. At the end of this period, I had frequent diarrhea, food was distasteful, I was bald, hands as smooth as a baby’s butt and my nails were dying.

With strong suggestions from my wife – I looked for a different health care environment that was better equipped to address my emerging health needs.

Diagnosis

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About 5-6 weeks after I first had symptoms I changed my health care provider from a local doctor to Emory Clinic. I choose Emory for two reasons; 1) even then there practices were integrated with all medical records on-line and available to all of the other doctors and 2) I thought that a teaching hospital would be better equipped to identify and treat a medical condition that was unknown.

I identified an internist at Emory based on his location as his office was the closest located to me and his medical school – that had a good reputation. I met with him and discussed the symptoms. It took about 2 months from the time I met with him to a final diagnosis of Cronkhite-Canada Syndrome.  From an emotional point of view that was, and has been, the most difficult time for I knew I had a significant medical issue – but it was unknown.

There were many tests. Multiple blood tests, MRI’s, CCT’s, colonoscopy – three times (once at the GI specialist associated with the local doctor and twice at Emory), and endoscopy. I swallowed the pill that took a 6-7 hour video of my intestinal system.

Several possibilities were identified including; cancer and leukemia. The process to isolate and test a possible diagnosis would take about a week. The internist was calling to get me appointments with the different specialists. They would review the symptoms, review prior tests results, have me take additional tests, wait a week to receive the test results eliminate what they thought it might have been, communicate they did not know what it was and then repeat – the next week – trying to either eliminate or confirm a diagnosis. This went on for around 6-7 weeks.  The internist’s plan was to go another week or so and if there was still no diagnosis to have me check in to the hospital.  This would allow the tests to be performed concurrently.

I was spending hours on-line trying to identify my condition. The symptoms of diarrhea, alopecia, loss of appetite and blotchy and smooth hands (hyperpigmentation) are associated with several diseases. With the symptom of dying nails – I was able to narrow down the possible conditions. Eight years ago there were fewer articles on Cronkhite-Canada Syndrome than there are today – not that there are many today – for most of the articles provide summary information and mostly say the same thing. I identified two possibilities; 1) radiation poisoning and 2) Cronkhite-Canada Syndrome.

In addition to these symptoms were the results of the tests and procedures. Hundreds of polyps existed in my colon, stomach, and small intestine (though with an endoscopy only the duodenum was visible). There was a pattern – initially my colon had the most polyps, then the stomach and then the small intestine. In the first colonoscopy performed by Emory my colon had hundreds of polyps and my stomach a modest amount. Around 4-6 weeks later another colonoscopy and endoscopy was performed. The doctor that performed the procedure was concerned for I had progressed to hundreds of polyps in the stomach and many were located at a bad spot – where the contents left the stomach and entered the small intestine. She was concerned that I could get a blockage. Though I never did.

Prior to the second procedure I told the doctor that I believed the symptoms were associated with one of two conditions; radiation poisoning or CCS. She laughed about radiation poisoning.   Cronkhite-Canada Syndrome was the diagnosis from the Emory pathologist.  At last there was a diagnosis.  It took two months from the time I went to Emory and over 3 months since I had the first sympoms.

I then spent many hours researching the information available that was limited. The first article I read on Google communicated within the first paragraph that the survival rate was around 50%.

Initial Treatment

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After the diagnosis by Emory I wanted a second opinion. I went to the Cleveland Clinic for in the articles I had read there was a doctor there that was a polyposis specialist and may have treated one Cronkhite-Canada Syndrome patient. Since then I know that Mayo Clinic has treated at least two patients with CCS.

The Cleveland Clinic confirmed the CCS diagnosis. One doctor did the colonoscopy and the doctor that I had identified as a specialist did the endoscopy. They also were in agreement with my initial treatment.

The initial treatment was; Sulindac (a prescription NSAID) Rantidine (Zantac) and Loratadine (Claratin). Sulindac was to reduce inflamation. Zantac is a H2 blocker – that is how it reduces acid in the stomach. Claratin is a H1 blocker. The literature indicated that other cases had treated CCS with a combination of these three prescriptions. Ten years later I still take these three drugs.

After the diagnosis I had an extensive battery of blood tests done. At that time I was diagnosed with low vitamin B12 – so I started taking that. A few years later I was diagnosed with low vitamin D – so I started taking that. I still take both.

Before my diagnosis I had went on a Prednisone ladder over about a 5-6 week period. It made a significant difference in my diarrhea. The principle behind the treatment was to treat the symptoms – for there was not a known cause. The Cleveland Clinic supported that approach. Though there was one exception – I also took a strong antibiotic for 7-10 days – as that was also mentioned in one of the articles. I have no idea if that helped or not.

I took the above prescriptions for a month. I was not getting any better. The gastroenterlogist relied on a recommendation from the doctor that specialized in nutrition. He recommended that I take an anabolic steroid for a month. At the beginning he said it would only be for a month and he would not extend it. He also recommended for me to take mega doses of zinc. I can’t remember the dosage but I was taking 3-4 large zinc pills 3-4 times a day. Three weeks in to this treatment – seven weeks after the initial diagnosis I began to notice a difference. My appetite was returning. I had also went on a second predisone ladder (at a lower dosage) as I continued to have diarrhea.  After I went off prednisone the second time – my diarrhea did not return. In a few more weeks I began to grow hair – yeah. It took several more months for my nails to turn to almost normal.

Initial Recovery

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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.

Remission and Steady State Treatment

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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.

It’s Back

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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.