My name is Ina. I’m now 63, and my journey with Familial Adenomatous Polyposis[FAP] (little did I know) began when I was a mere child. This journey started with very little knowledge or understanding and over the course of my life I’ve learned more than I ever wished I would need to know.
My grandmother died of cancer when I was about nine years old. Grandma had an ileostomy and treatment for a while before she passed away. My father had surgery in which the large bowel was removed and the small intestine reconnected to the rectum (total colectomy) around 1960. He survived the surgery and recovered fairly well but was not able to handle the stress of farm work and left the farm to work with his brother painting houses.
When I was in high school, I started having routine rectal exams by proctologists, who found small polyps and burned them out just about every year. Once I graduated high school, the doctors started performing barium x-rays in addition to the yearly rectal exam. My understanding was that if a polyp was found to be cancerous I would undergo the same surgery that my father had undergone — resection and removal of the large intestine. The doctors never discussed any details with me and I was not well-enough informed to know what questions to ask. This yearly routine continued without incident for several years, until the exam I was given shortly after I gave birth to my daughter.
The doctor found a half dollar sized cancerous polyp this time, and the cancer had progressed into the intestinal lining, but not into the lymph nodes. My Proctologist referred me to a gastroenterologist (GI) for an EDG and a colonoscopy. When I asked about what surgery I would be having I was completely taken aback when I was informed that my colon and rectum would be removed, leaving me with a permanent ileostomy and no options for any alternatives. My surgeon did not provide any options to this complete removal procedure, which included the rectal muscles, preventing any future options like a pouch or pull through. Of course, at that stage in our journey we were not aware that other options were even possible.
This situation really drove home the need for all patients to do some research before committing to radical procedures, just because the doctor recommends it. I had complications during the surgery; among other issues, they had to remove my gall bladder because I had stones and the scheduled four-hour surgery lasted nine hours. My husband visited me in recovery for a few minutes before I went back into surgery because my stoma was not pink and healthy. I ended up with a short bowel and arrived in ICU around 5 AM the next morning.
I was in pretty bad shape after the surgery, but the ICU nurses allowed my husband to bring our daughter into the ICU for a couple of days and this certainly spurred my desire to get well and get home. However, due to the double surgery and a few setbacks during recovery, it was almost a full month before I was released. Once I was in a regular room, my husband faithfully brought our three- month-old baby daughter every day to visit. Even with my baby as an incentive, I still got pretty depressed before I was released.
After I got home and made somewhat of a full recovery, I faced another challenge — small bowel obstructions. Several foods proved to frequently cause problems (green beans, chicken, even oranges) and could cause me to visit the ER, which generally ended up with a three or four day hospital stay and the always — welcomed NG tube. I still have this problem, although I make every effort to be smart and aware of the dangers.
Research later confirmed that my baby daughter had a 50% chance of having FAP. We assumed that our daughter would need to start exams when she was in high school, just like I had. My husband and I had discussed having three children, but with each child having a 50% chance of having the FAP gene and my much reduced general health, my husband convinced me that the risk of childbirth outweighed the advantages of more children. He said he would much prefer a wife and one child than more children and no wife.
When our daughter was in first grade she started having stomach pain. Her pediatrician did a blood test, which did not indicate anything, and would not refer her to a gastroenterologist. I changed doctors as soon as I could, and after she started the second grade and the chronic pain came back, her new doctor referred her to a specialist. The GI doctor scheduled an EDG, which showed that her stomach was in a pre-ulcerous state; her stomach bled at any site the probe touched. During the doctor’s visit, we discussed the family history and were informed that FAP polyps could form anywhere in the digestive tract and that our daughter should have annual EDGs and colonoscopies. A polyp was discovered during this annual exam in her ninth year and was found to be cancerous. This was not welcome news.
In the spirit of doing what you have to do, we scheduled surgery shortly after the school year ended and not long before she turned ten years old. [At every hospital stay we witnessed other families’ plights and struggles with their own burdens and felt very blessed with our situation. An extended visit to the children’s floor really helps to open one’s eyes to the possibilities.] We discussed the surgery options with her GI doctor and surgeon and chose the j-pouch option. The surgery went well — she was back home in a few days and the future seemed pretty good.
Soon after, we had to take her to the ER with stomach pain, and on the second visit, the residents (no real doctors anywhere to be found) discovered an incision infection. She was home for a couple of days before going back to the ER with pain again. Lack of knowledge and experience on the ER staff’s part and our own lack of knowledge delayed the standard barium x-ray that should have been performed. When the test was finally performed, her intestine was twisted so badly that she, too, ended up with a short bowel. Her ileostomy was repositioned to her right side and the j-pouch procedure plan was changed to a straight pull through for the next summer. The next summer, before she turned eleven, the attempt was made to do the pull through procedure. The surgeon was not successful and was not very sympathetic, either — his attitude was that all anyone should wish for was an ileostomy.
By this time, our daughter was traumatized by just the mention of getting an IV, as she was generally stuck at least two or three times unsuccessfully. In fact, this has not changed. During an outpatient visit in 2011 for a CT-Scan, she was stuck six times before an IV was successfully inserted. She had the closest thing to PTSD that we want to experience. While in her sophomore year in high school, we started sending her to a therapist at her request. In her junior year, her GI doctor directed us to discuss a possible new surgery with a different surgeon. That summer she had a straight pull through procedure completed, somewhat successfully (with her short bowel and massive amount of adhesions and scar tissue, her condition is not normal), but had a lot of recovery problems and had to be on TPN most of that summer.
To this day she experiences diarrhea on a regular basis, has to take B-12 shots, and extra iron. In spite of these problems, she has survived and been successful in completing a master’s degree and having a career and has made a happy marriage. It was an amazing amount of comfort to have loving family members and a caring church family who offered support and prayers along the way.
It is a lot easier to deal with your own health issues than it is to see your child go through so much trauma and tribulation. My husband and I had a lot to accept and had to eventually come to terms with the fact that there was nothing we could do — we were not in control. I felt so very helpless throughout all of my child’s medical treatment. Throughout the whole ordeal I made the very best decision I could at each step of the way with the knowledge that was available. Some of the decisions, however wisely made, had unfortunate consequences. The knowledge that the best possible path was chosen at each step, given the circumstances and the medical advice offered and using our own limited knowledge and best judgment, helps keep guilt at bay.