Little did I know my dad’s announcement would alter my life so much. When I was five years old my father was diagnosed with Familial Adenomatous Polyposis (FAP). After being diagnosed with FAP, my dad had surgery for a colectomy. They took biopsies from several polyps, only to find out that he had three malignant tumors. He had to go through six months of chemotherapy. Four years later, cancer spread to his liver and the lining of his small intestine. He had a few more surgeries. They did all they could and gave him six months to live. He died five months later at the young age of 32; I was 10 years old.
Shortly after his surgery, we were told the bad news. This is a hereditary disease consisting of multiple adenomatous polyps. If left untreated, the polyps would turn into cancer as it did in my dad. This meant that my brother, sister, and I were going to all be tested annually. This began for all of us at age five. Hundreds of adenomatous polyps were found in my brother and I around age 13, and we were diagnosed with FAP. We were told we needed to have surgery to have a colectomy, which was done on both of us on the same day. I was 17 and my brother was 18. Five years later my younger sister was diagnosed with the disease as well and had to have the same surgery. We were told that we all would need to be followed annually. Through being tested annually, adenomatous polyps were being found in my rectum. My doctor strongly suggested that I have another surgery called the J-Pouch surgery.
This surgery is a two-step process: the first step is to remove the rectum and create an internal pouch out of the small intestine. This required me to have an ileostomy. The second-step was called the take-down, to get rid of the ileostomy. I really was not looking forward to having this surgery and having an ileostomy, but I knew I had to. I had my first surgery February 19, 2004. I had complications during and after the surgery. My surgeon, Dr. Dietz, did not even think he was going to be able to complete the surgery due to a desmoid tumor located by the left ureter. During surgery, Dr. Dietz accidentally nicked my left ureter. It was a God send that happened because a stent was placed in my ureter making it possible for Dr. Dietz to be able to complete the surgery. Complications after surgery included: staples that had come loose in my J-pouch, requiring me to go back into surgery the next day; a bad wound infection, causing me to have my wound packed daily for four weeks and then a wound vac for four weeks; and an abscess in my pelvic cavity, causing me to have a drainage tube for six weeks.
Soon after I was healed it was time for the second step of the surgery, which I had in mid May. I was excited to get rid of the ileostomy, but nervous at the same time, as I had complications with the first surgery. I had problems with this surgery as well. I could not eat without severe abdominal pain and vomiting. After being in and out of the hospital for over two months, Dr. Dietz finally figured out what the problem was. My small intestine had collapsed at the point of the reconnection. I had to have surgery to repair the problem and received TPN through a PIC line because I was unable to eat for such a long time. I also had acute renal failure, a dangerously high heart rate, and a wound infection. Finally, after being in the hospital for 23 days, it was time to go home.
I went to work on that floor two years later, and a resident told me that they had almost lost me. One year later, I had to have surgery to remove a superficial desmoid tumor. There are still four desmoid tumors located in my abdominal and pelvic cavities. I am currently taking medication to control the growth of those tumors, along with yearly CT scans to keep an eye on things. So far the medication has been working. I also have had adenomatous tissue in my duodenum and major papilla, which had to be removed for three consecutive years. I asked Dr. Dietz if my children would have to worry about these problems and he said, “Yes, it seems to run in the family. If one person in the family has them, then all of them tend to have them.” I wasn’t happy to hear that! In 2013 I had an ampulectomy in my major papilla due to past years of adenomatous tisssue turning into large polyps that could turn into cancer. Doctors felt it was time to do something. During the procedure I needed a stent put in my pancreatic duct and in my common bile duct that had to be removed two weeks later. It was now time for my children Colton, 13, and Cassie, 10, to be tested. I was dreading this time for years. The good news was they could be genetically tested since my sister and I were genetically tested by Washington University, where they found the mutation of the gene causing FAP. Colton and Cassie were tested in May of 2006.
Six weeks later, I got the phone call that I was dreading. Both Colton and Cassie had the gene mutation. I just could not believe it; I thought I went through all the problems I did to save my children. I guess I was wrong! One month later, Colton and Cassie had to have their first colonoscopies, and they both had hundreds of polyps already that after biopsies proved to be adenomatous. None of us were prepared for this news. We thought they wouldn’t have them for a while, especially Cassie at age 10. I was devastated and felt so much guilt that it’s all my fault, as I’m the one who passed the gene on to them. It was suggested by doctors that they both have the J-Pouch surgery. We felt lucky that we found a pediatric surgeon who could perform this surgery laproscopically. Colton, age 14, had his first surgery on March 19th and Cassie, age 11, had hers on March 20th of 2007. Colton had the worst bowel prep that the floor had had. He was throwing up all night. Colton began throwing up again after surgery when he was able to eat. They found free-floating air under his diaphragm, which could only be caused by having a leak out of his intestines. He had to undergo another surgery to repair the leak. Cassie would not eat or drink after seeing her brother go through what he did, because she was scared she was going to need surgery too. After twelve long days it was time to go home.
Colton had a really hard time emotionally with everything that he went through and especially the ileostomy. He was mad at me and thought it was all my fault that he had to go through everything. He got to the point that he would not speak to or even look at anyone. He just focused on the T.V. He even spoke with a therapist because I, as well as my husband, mother, medical staff and doctors, felt it was necessary for him. That didn’t seem to help either. He got better as time went on. May 29th was the day for both Colton and Cassie’s take-down surgery. Cassie went in and the surgeon found that she was not ready. She had a very tight stricture, as well as grayish mucus and granulation, and he thought she had a little leak in her pouch. Next it was Colton’s turn, and thank God he was ready; he had already been through so much. The next four weeks Cassie had to go into the OR for dialation procedures.
Finally, on July 3rd Cassie was ready. Cassie had been a real trooper through it all! It was such a relief that it was all over with. Now they also need annual testing just like me, in which adenomatous tissue has been found in their duodenums. They didn’t find it in me until I was in my 30’s. Even though there have been some positive improvements for this disease such as genetic testing and laproscopic surgery, there are still more and more negative conditions being found every day. The conditions I am aware of are: adenomatous tissue can be found in any part of the digestive system, desmoid tumors, being more prone to cancers such as adrenal, and the gene seems to be a very strong gene thoughout each generation.
Who knows what else will come up in the future; it seems more and more keeps popping up. Up until a few months ago the children thought they had cancer. I feel I should have raised them knowing about the disease like we did instead of hiding the disease their whole life. It was too much of a shock to them.