The story of Joann
Hi my name is Joann and I was diagnosed with Familial adenomatous polyposis and Gardner syndrome in 1978. Familial adenomatous polyposis, called FAP or classic FAP, is a genetic condition. It is diagnosed when a person develops more than 100 adenomatous colon polyps. An adenomatous polyp is an area where normal cells that line the inside of a person’s colon form a mass on the inside of the intestinal tract. The average age for polyps to develop in people with FAP is in the mid-teens. If FAP is not diagnosed and treated, there is a very high likelihood that a person will develop colorectal cancer.
Individuals with FAP also have an increased chance of developing cancer in other organs, including the stomach, small intestine, the pancreas and biliary tree. Risk for hepatoblastoma, a rare type of liver cancer, is increased in young children with FAP. A locally aggressive, non-cancerous tumor type called desmoid tumors/desmoid fibromatosis and a rare type of brain tumor called medulloblastoma can also occur in some individuals. Risk for papillary thyroid cancer is also increased.
This is my history of what I’ve been through because of FAP.
I had a total colectomy in 1978 when I was 11 which left me with an ileostomy. I had a koch pouch put in when I was 13, and also in my teens I had multiple surgeries for blockages caused from adhesions and I had Desmoid tumours removed. Then I went a few years without any surgeries until 1991 when I had to get my koch pouch reattached to my abdominal wall as it was torn from it. Also in 1991 I had another benign tumour removed from my left thyroid. In 1997 I had a fibroid tumour removed from my ovary. In 2003 I had another fibroid tumour removed from both of my ovaries and lost my right ovary and was left with 1/4 of a ovary on my left side. I also had my rectum and anus removed because they were full of polyps. I also had my Koch pouch removed and went back to having an ileostomy because my Koch pouch was half dead and full of polyps. In 2009 I had my gallbladder removed because of polyps. In 2012 I had the worst experience of my life that started with a total blockage where part of my small intestine was dead and they had to remove a big amount of small intestine leaving me with short gut syndrome and only 6 feet of small intestine. This means that everything I eat or drink only takes about a hour to go through me. While healing in the hospital, there was not enough blood getting to my stoma causing it to die but they couldn’t just just go in and remove it – they said it had to completely die and rot until it fell off leaving me with a really small stoma that was below my skin level. My incision also opened up and I became septic with a infection just under my skin near my stoma. It was the worst three months I spent in the hospital. My last surgery was to remove a large bone growth from my jaw and they also removed all my teeth due to my FAP and they just kept breaking off and getting abscess all the time. Now I get ERCP scopes and gastroscopy scopes every six months due to the amount of polyps I have in my duodenum, ampulla, stomach, and small intestine which get sent to pathology to see if they are cancerous. I also had to get a port-a-cath put in to get liquids by IV every week and to get vitamins. I’m low in iron because of my short gut syndrome.