Karen Found
Toronto Ontario - Glucagonoma.
My symptoms appeared, starting in the summer of 1982 when I was 40. I came home from a trip to Disney world, with a deep vein thrombosis in my right leg. I had previously had a severe thrombosis in my left leg, in 1965, when I was first married and was on the pill and they attributed the thrombosis to the pill, which was called Ortho Novum, one of the very first birth control pills on the market at that time. In 1965, I was honeymooning in Florida, when the first thrombosis occurred. My leg had gone blue and had swelled up like an elephant. I was considered an acute emergency and had to have emergency surgery at a teaching hospital in Gainesville, the J Hillis Miller Health Center, to save my leg, and ultimately my life as the thrombosis could have killed me at any moment. I was 23. My parents in Canada got in touch with Canadian doctors who advised that deep vein surgery would not have been attempted in Canada at that time, so I was very lucky that I was able to be immediately The thromboses diagnosis and treatment in 1965 were to make the initial diagnosis of my Glucagonoma more difficult, (in 1989).Doctors afterwards were always looking for something else.
I was blessed with four children in the following five and a half years, three boys and a girl. In 1982 I was again treated for a deep vein thrombosis, which I later discovered is a symptom of Glucagonoma. This time my right leg was affected. I knew what it was because of my experience in 1965. I waited four days to make absolutely sure I knew what it was, not wanting to bother doctors unnecessarily, and then drove over to Sunnybrook Hospital in Toronto. I was admitted immediately. I was in hospital for 10 days on an IV Heparin drip, which dissolved the clot. In 1985 I again experienced what I thought was a deep vein thrombosis in my right leg, and tried to ignore it. I went up to Muskoka, on visiting day at my childrens’ summer camp, and there and then, on Highway 400 I ‘threw’ the clots from my leg to my lungs, which is what happens when DVTs move.
I got through the day, even climbing the hills of Muskoka, and going to all my kids’ cabins, drove home, and again waited three days, (it was holidays and my doctor was away), and on the fourth day after not being able to lie down to sleep, because I couldn’t breathe if I lay down, I then delivered my son’s newspapers for him, (as he was at camp), and then drove straight to Sunnybrook. (Thinking back I don’t know why I am still alive).
I was admitted again, and again it was the summer time. Perhaps the heat of summer has an effect on the formation of blood clots.
I was in hospital for 10 days but this time they could not dissolve my clots. I had clots in the bottom of my left lung and later discovered, after I had a digital subtracting pulmonary angiogram, that I had a large clot in my right lung. The doctors were mystified.
Two nurses came in and prayed over me that night, in 1985. That night I also wrote my will, on a paper napkin. My 98 year old year old roommate witnessed it.
The doctors were concerned before the angiogram that the clots were old, but the angiogram confirmed that the clots were new. The heparin routine I was put on in the hospital eventually controlled my clots and most of them eventually disappeared. Since 1985 I have I have been on different varieties of blood thinners continuously.
At that time one of my doctors began to wonder why a person of my age was experiencing so many blood clots. It was Dr Livingstone, a young doctor, about 31 at the time, from Sunnybrook. He was the first doctor to be interested in my case. He had to get special permission to administer a special blood test to try to determine what was causing these blood clots.
The test was done, and the test results came back. They were negative. I don’t know what the test was for. Whatever cause Dr Livingstone was looking for was not there.
So we went on from there. In 1987, I was admitted to Sunnybrook with anorexia. I had passed out on the bathroom floor, said I’d tripped, but my four kids woke up, and standing looking at me, were ready to call 911. I persuaded them I only tripped and was OK, but the next day when I couldn’t stand up I phoned my GP and he said enough is enough, and sent me to Sunnybrook.
I was at Sunnybrook again, for 10 days, and this time I was sent to the psychiatric ward. My doctors were psychiatrists. I insisted I wasn’t anorexic and that something else was causing the weight loss. I knew that I had no appetite and that the cause was not emotional. The doctors didn’t believe me. They were very kind, and kept looking at my wrist for scars as they suspected I might have attempted suicide at different times.
I was taken in a wheelchair to an amphitheatre where I was the subject of Psychiatric Grand Rounds. I sat there and was questioned by a number of students in the amphitheatre. There were at least 100 medical students there, and they were very curious, wanting to know how long I had been anorexic and why.
Near the end I was asked by one student what part of my body did I think was still too fat. I said,” None”. I weighed 94 pounds at the time. Another student asked why did I not admit that part of my body was fat. Another student then said that it was because anorexics tell lies. I was mystified by these revelations. Even at that time I was amazed that doctors could not see beyond their own discipline.
The Hospital would only discharge me after 10 days in the psych ward if I had an appointment to see a psychiatrist. I also was somewhat depressed, which I discovered later can be a symptom of Glucagonoma. It also didn’t help that at that time I was in the middle of a divorce. However the doctors did not know I was going through a divorce.
I also had to be 104 pounds to be discharged. They had checked to make sure I wasn’t throwing food away or hiding it under the mattress, but their constant feeding allowed me to gain weight and be discharged. I was fed very high calorie foods. I managed to maintain this weight for two years by drinking a lot of milk shakes, and eating high calorie foods. It was fun.
Then in 1989 I developed infections in my two big toes. The Sunnybrook podiatrists and chiropodists were mystified as to how to clear up the infection. Eventually the podiatrist sent me to a dermatologist. My GP at the time did not know what to do. He was unable to help me with any solution to my problem.
So I went to the dermatologist, again at Sunnybrook. His name is Dr Neil Shear. I saw him twice and he tried working on my toes. He was attempting to find the source of the infection in my toes.
Again I had to go to Muskoka to visit my children at Summer Camp. This time I couldn’t walk on my infected feet. By now a severe rash had appeared and was all over my feet, up my leg, and down my arms. My feet were filled with open, wet, miserable, sores. I put my feet in a bath of Epsom salts and that did absolutely nothing for them.
I made another appointment to see Dr Shear and he saw me within days. On seeing the rash, Dr Shear insisted on a biopsy of the rash, which was also on my buttocks.
Two of the three biopsy samples came back negative, and one was positive for necrolytic migratory erythema, which is only seen as a symptom of Glucagonoma syndrome. He immediately wanted me admitted, and I was.
I had wonderful care. I had a little tent placed over my legs, and I was driven everywhere in the wheelchair while in the hospital. They gave me sitzbaths. I was hoisted up on an elevator and lowered gently into these baths, which did nothing for me, but were a nice treat nonetheless. Many doctors came to take snapshots of my lesions. They would come up to within an inch of my lesions to take pictures. The doctors were always very kind and always asked permission to take the pictures.
At one point Dr Livingstone came into see me again, and we had a wonderful discussion, about what had happened when he was a very new doctor, and he had tried to discover what was wrong, and he observed with a laugh, and we both laughed, that had he known then what he now knew, he would be sitting with his feet on his desk and his hands behind his head...basking...and we both kept laughing.
I was so grateful to him in those early days as he was the only doctor at that time who tried to find some non psychological answers. I was taken to Grand Rounds in a taxi for a few days to several different hospitals, and young medical students would observe my lesions and discuss with their professors how to identify them. I was then sent to Toronto General Hospital Investigations Unit. I had been told that the doctors and nurses in that unit had been briefed on my Glucagonoma diagnosis before my arrival, and that they had been reading everything they could lay their hands on about this syndrome. They had a difficult time, however, finding books or articles on the subject.
I was an in-patient in the Investigations Unit for three months. This unit no longer exists in the same form. It was a fascinating place. It had 12 beds.
The mission was to locate the tumour. The doctors had read enough to know that the gut was generally the location where Glucagonoma tumours are found. The rash had been just another symptom that was only found with Glucagonoma. At that time, they said I was the 102nd patient in Medical History to have Glucagonoma syndrome.
I had many medical pictures taken as the rash continued over three months. My entire body was wrapped in burn gauze soaked in something. Each time the dressings would be on for 15 minutes. The dressings were changed three times a day. Unfortunately they had no effect.
I was still anorexic but, thankfully, psychiatrists were not involved. The team knew by now that anorexia was another symptom of Glucagonoma.
I was given Ensure, which I hated, with every meal, also for snacks, to fatten me up. I wanted a milkshake, which I loved, which they would not give me. Ensure had all the vitamins, which I needed.
I was given two MRIs during my three month stay at the investigations Unit. Also CT scans, ultrasounds, the whole works. Nothing was spared.
A normal glucagon level is approximately 300. At diagnosis, my glucagon level was 14,000. By this time I had already been started a new drug, which was called Sandostatin, which was delivered intravenously. The doctors and researchers knew I had Glucagonoma, but they just hadn’t found it yet. When I was later sent home I was directed to give myself subq sandostatin shots four times a day.
Endocrinologists were brought in to observe my specific case during my three month stay at the Investigations Unit, because it had been determined that my tumour was endocrine in nature.
My tumour was not found until a portal vein study was done sometime after discharge from the Investigations Unit. It was a last resort to find the tumour. A catheter was stuck through my ribs to get to the portal vein, and fed through the portal vein into various organs. Blood didn’t seem to be a problem. The catheter would stop at different organs or different parts of the organ and little pieces of tissue would be taken out of this or that portion of the organ and stacked up inside the catheter. The process took several hours. The researchers could see where they were going but they were unable to recognize tissues as tumours on the scan monitor. They had to go to a lab to test each piece of tissue to find out which corresponding organ sample had the highest concentration of Glucagons. It turned out to be the tail of the pancreas where the concentration of Glucagons was the highest, and that was where the tumour was eventually located. Once the doctors knew where the tumour was, and I had been discharged on sandostatin subq injections, I was at home on and on Sandostatin.
I came home to discover I had lost my job as a volunteer coordinator at a Special Needs school. This was 1990.
In September 1990 I was offered an operation to remove the tumour in the tail of the pancreas but I had just started a new job so I decided not to have the operation till I had been back at work for a year.
My operation was July 2nd 1991. It was performed at Toronto General Hospital. A little more than half my pancreas was removed. I was in hospital again, for about 10 days. There was great optimism that everything had gone well.
However I had not yet been told that my tumour was malignant. I was curious and refused to go home until I was told what kind of tumour had been removed. The nurses kept assuring me that I could go home, and that they could not give me that information. I said again I wasn’t going to go home till I found out if the tumour was malignant. Before the end of the when I was scheduled to leave my resident, who had been responsible for my care for 10 days, very sheepishly said, in response to my refusal to go home unless I knew the results of the tumour biopsy, “I was hoping you wouldn’t ask”.
Then he said,’ It is malignant but we think we got it all.” I was glad I knew the truth. It did not change anything. Life was going to continue as usual. I went off sandostatin after my operation, because the tumour was gone, and the doctors considered me cured. I considered myself cured too. This was 1991. I was 49.
I was off sandostatin for 9 years. I went for checkups every six months for a few years but findings were uneventful and not wanting to waste my doctors’ time I discontinued my checkups.
Around 1997 I was feeling fine but decided I should get another check-up. I went back to Toronto General. I had a CT scan at that time. The results showed two small tumours in my liver. At that time nothing was going to be done except wait and watch. Doctors concurred with the idea of ‘wait and see’ I kept having checkups. After a year, around 1998 a few more tumours appeared in my liver, plus my pancreas had developed two more tumours. I also had one in the lymph nodes and in the lymph nodes of my aorta. I had not yet been put back on Sandostatin.
The more tumours I developed the more curious I became and at that time I was referred to Princess Margaret Hospital. At Princess Margaret the doctor, before she even introduced herself looked at me and said,”There is nothing I can do to extend your life expectancy.”I was somewhat taken aback. I thought, that’s an interesting way to introduce yourself. Later we established a good humoured and relaxed relationship.
After some time when I did not die and all I had were checkups, my doctor told me she was focusing on my quality of life, and not on my quantity, but she told me that down the road there might be something for me.
In 2000 I decided to seek out-of-country advice and saw Dr Fatourechi, in the Division of Endocrinology and Metabolism at the Mayo Clinic. I was told Dr Fatourechi had some experience with Glucagonoma. The hospital, in Rochester, Minnesota, was so wonderful, and so organized, and I was treated like royalty. I was there three days. I was seen by numerous doctors in different specialties, by oncologists, surgeons, and endocrinologists. Their recommendation was a liver resection, as I already had seven liver tumours, and they also recommended removing to portion of the liver that had the most tumours.
When the liver was open, they felt that they could then get into the other half of the liver and embolize the remaining tumours. Nothing was said, I am pretty sure, about the Mayo recommending any Sandostatin at that time. I realized the cost of the operation was going to be prohibitive, so I thanked them very much and took their findings home. I did not give them to any local doctor. I regarded the trip as an interesting education.
Back at Princess Margaret Hospital I continued to be monitored and in 2004 it was recommended I go to London, Ontario, to see the London team. When I went to London I was prescribed Sandostatin 30 every 28 days. I have continued it all along, even through chemotherapy.
Between 2003 and 2006, I had some chemotherapy, consisting of epirubicin and carboplatin, doxorubicin, and 5FUat Princess Margaret Hospital and it was successful in keeping the tumours stable. I was able to work continuously during the first round of chemo as I never felt ill. I had it again in 2006. Both times I lost my hair. I wore a wig the first time, but the second time I wore scarves. I have been going almost exclusively to London since 2004.
As of writing, in 2007 I have 18 four centimetre malignant tumours in my liver. I am on 30 mg every 28 days. I love going to my London doctors and the Hospital in general. I truly believe it has bought me time.