Shortly after I came up with the idea for this ride I asked my mom if she would tell the story of her family’s ongoing battle with Familial Adenomatous Polyposis (FAP). From a mother’s perspective. Chronicling how a family umm blessed with this rare disease has been affected by all aspects of it. From when my dad was first diagnosed with FAP to present day.
Like so many times in my life, she said yes. She truly is an amazing woman. She has given so much of herself to her family. And, continues to. One of life’s givens.
As you will see below, she did a wonderful job capturing what it’s like living with FAP and having loved ones afflicted with it.
So, enough of my rambling. I hope you will take the time to read her story. My family’s story.
My Family & FAP
- -By Mom.
- Ted’s FAP Diagnosis & Initial Surgeries
- Recurrence of Ted’s Cancer
- Effects on the Family
- Ted’s Final Days and Death
- Life After Ted and Children’s Testing for FAP
- Todd, Kim & Laurie’s Initial Surgeries and Related Problems Afterwards
- Kim’s J-Pouch Surgery, Learning About Desmoid Tumors and Other Problems
- FAP Research and Genetic Testing
- Colton & Cassie’s J-Pouch Surgeries
- Todd’s Plans for J-Pouch Surgery
- Todd’s Continuing Medical Issues
- Laurie’s J-Pouch Surgery
- Take Care of My Family
Ted’s FAP Diagnosis & Initial Surgeries
When my youngest child was about two months old, my husband Ted was diagnosed with Familial Adenomatous Polyposis (FAP). He was having problems with diarrhea and crampy pain in his abdomen for a couple of years prior to his diagnosis. He even scheduled an appointment with the doctor two years before, but cancelled it because he didn’t want to take off work. He sometimes commented that maybe he had cancer. We were a young couple with three young children, and we had never had any experience with anyone close to us having cancer. Therefore, I did not take his comments seriously.
He finally went to our family doctor the day before Christmas in 1973, since there was no work that day. After having a colonoscopy, he was told that he had multiple polyps in his colon – hundreds of them – and that he had to have surgery to remove his colon. With this disease there is a 100% chance that the polyps would turn to cancer if left untreated. We found out that the disease is hereditary, and that our children had a 50% chance of developing polyps. This was the beginning of a life changing experience for our family.
At the time of Ted’s first surgery, he was 27 years old and I had just turned 28. Our son Todd was almost 6, our daughter Kim was 5, and our daughter Laurie was about 3 months old. We lived in California. My family lived in Wisconsin, as did most of Ted’s family. He had 2 brothers in California, one of whom we saw very little. His other brother’s wife was a great help to us during that time. In fact, her mother was the one who suggested that we see if Ted would qualify to be treated at City of Hope. He was approved for treatment there, which was a great blessing for us. City of Hope did not charge anything over and above what your insurance would cover. With all of the surgeries, tests and doctor’s appointments Ted had, and then tests and appointments for the two older children, we could have been under great financial stress, along with the stress of the illness itself.
The surgery to remove his colon went pretty well. Ted’s sisters from Wisconsin and Hawaii had come to be with him. My sister-in-law in California, along with our neighbors, helped tremendously by taking care of the children while I was at the hospital. A couple of days after the surgery I got the phone call from Ted saying that they had found three malignant tumors from the biopsies that were taken during the surgery. I think I was in shock, as I didn’t realize the ramifications of this information. My visiting sister-in-laws and I still stopped to shop at the Sears surplus store near our house on the way to the hospital. I think the one from Hawaii, being a nurse, wondered how I could do this. I just didn’t know exactly what this meant. But I was soon to find out.
Ted had to go to City of Hope every week for chemotherapy, usually on Fridays. That way, after the recuperation time from the surgery was over, he could work Monday through Thursday. About six months after the first surgery, he had to have another exploratory surgery to see if there was any more cancer. The surgery went well and everything looked ok. Thank God!
But right after the surgery was over, I got called to the nurses’ station for a phone call. It was a fireman calling to tell me that my daughter Kim had run through a sliding glass door and that I should get there right away. He didn’t tell me the location or extent of her injuries, so I was really scared. City of Hope was about an hour away from our home in the San Fernando Valley and the hospital Kim was at. That was probably the most panic-filled drive of my life. When I got to the hospital, the doctor let me in to see her. She had a scratch on her forehead, but most of the cuts were on her legs. When the doctor pulled the flap of skin open in one area, I was so upset that he had me leave the room. The plastic surgeon had to put over 300 stitches in her legs, in layers. For quite some time she had plaster casts on one side of both of her legs, with ace bandages wrapped around her legs. This was mostly to keep her from bending her knees.
A friend had been taking care of the children at her house the day of the surgery. She had to go to pick up a partial at the dentist, and since her sons were in 7th and 8th grades, she felt it would be ok to leave the kids with them for a short time. My daughter was running out of the living room, through a glassed-in patio and on to the pool. The patio was enclosed and had it’s own door, so the glass door from the living room to the patio was usually left open. Kim didn’t realize that someone had shut that door, and ran at full force through it. The glass shattered to the floor. Eight-month old Laurie was crawling around the living room. The boys had gone out to the garage for a few minutes. Six-year old Todd was the one who called the emergency personnel. The boys contacted the nurse who lived across the street, and she came over to help out until the firemen got there. When I got to the hospital, my friend was sitting in the waiting room, and I could tell how bad she felt about what had happened. I never blamed her, and I didn’t want her to blame herself.
City of Hope gave us a wheel chair for Kim to use. After Ted got out of the hospital, he was off work for a couple more months. During that time some of our friends introduced us to camping. We all enjoyed this very much, even Kim, who still had the casts on and couldn’t go into the water. We continued to enjoy camping throughout our time together as a family.
For the next four years Ted had to go to City of Hope for checkups every three months. The two older children began to have annual tests there, too. They had barium enemas and/or flexible sigmoidoscopies done. The worst part of the procedures for the children seemed to be the preparation, which included liquid diets and laxatives. They thought I was terrible for coming to school during their lunch hour to have them take caster oil. Thinking back, they were probably right. I should have let them stay home the day before the procedure, as well as the day of the procedure.
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Recurrence of Ted’s Cancer
In December 1977, again right before Christmas, we found out that cancer had shown up in Ted’s liver. The doctor said he could have 5-10 years left to live. He was told he needed surgery as soon as possible. But Ted wanted to wait until after Christmas to mention this to anyone, so that it wouldn’t ruin anyone’s holiday. Our niece from Hawaii was visiting that year during the Christmas season. Ted also wanted to go back to Wisconsin to visit family and friends before the surgery, and he didn’t want to tell them anything about what was going on, so that everyone could enjoy the time we spent together. So, it was February by the time the surgery took place.
The exact details and timing of the events during the next five months are a little fuzzy, but I’ll present them to the best of my memory. After Ted had been in surgery for several hours, I was looking out the waiting room window and saw his surgeon walking out of the hospital. I couldn’t figure out what had happened. Finally a hospital staff member, who would come out with periodic updates, came to let me know that the surgeon had gotten sick and had to leave and that someone else was finishing up Ted’s surgery. After what seemed like forever, a total of about 8 hours, I was told the surgery was finished, but that the doctors had to do something else before they could talk to me. When we finally got together, I was told that there were also tumors on the lining of Ted’s small intestine, and that if he hadn’t had the surgery when he did, he would probably have been dead within a few weeks. Because of his age, they did some things that they wouldn’t have done for an older patient. But they said that he only had about six months to live. I couldn’t believe it! They said it would probably be best if we didn’t tell him the time frame, as it would probably depress him, and he wouldn’t do as well. Ted’s sister from Wisconsin agreed with that theory.
I’ll never know if that decision was right or not. Ted and I never really discussed his death before it happened, even though I’m sure he knew that it was inevitable. And, therefore, we never discussed it with our children either. I do now feel the whole situation was not handled correctly. Ted spent most of the next five months in the hospital, and he wanted me to be there with him all of the time. That is pretty much what I did. This is another decision I made that I question to this day. My children were taken care of by friends and relatives, sometimes at their houses and sometimes at ours. My mother had planned to come out to stay with the kids when we thought Ted would only be in the hospital for a couple of weeks. Then the day before the surgery, my mother was hospitalized. She had a bad reaction to the prednisone she had been taking for shingles and ended up needing blood transfusions. I don’t remember her even telling me she had shingles.
There were cottages on the City of Hope grounds that families of patients could rent, which is what I did for a while. The children would stay there with me some weekends, as did Ted’s sister when she was there from Wisconsin. When the kids were out of school, a very close teenage friend of ours came down to help out and to be with them, so that they didn’t have to spend all day in the hospital room.
Every time they would try to give Ted food, his system could not handle it. Gastric fluids would seep out of his small intestines. The doctors explained that it was like a kink in a hose that caused a leak. Ted had two or three surgeries to try to repair these kinks, but they didn’t seem to work. Sometimes he would get fistulas, where the fluid would create a hole in Ted’s skin and would come out.
There were two women at the hospital that provided much support to me. One was the woman who checked in with me during Ted’s surgeries, and I can’t remember what her title was. The other was a social worker. Ted’s sister was there at the time of one of the subsequent surgeries, and I remember her saying something to me right after he was taken into the operating room that hurt me a lot. What she said included, “Blood is thicker than water”, insinuating that she was closer to him than I was. I ended up going to the social worker’s office to wait by myself until that surgery was finished.
My aunt, who was also my godmother, came from Wisconsin for about a week at Easter time to help with the children. I came home from the hospital at night during that time. After she left, I found that I really wanted to spend more time with the kids. So, for a while I would come home after the kids got home from school, and the teenage neighbor would come over at bedtime to stay with them so that I could go back to the hospital to sleep in Ted’s room.
There came a time when it looked like Ted was able to keep his food down and he would be able to come home. That was at the same time my mother was finally able to come out to help with the kids. She came anyway, and we were all at home. However, it didn’t last long. Ted was not able to keep anything down once he got home. He also had an open fistula, which I had to clean and dress. Ted wouldn’t call the doctor to let him know about his problems, as he didn’t want to go back into the hospital. After about a week he had an appointment with the doctor for a check-up. While at the hospital, I let them know what was going on, and Ted was readmitted.
The doctors decided to take Ted back into surgery and insert a broviac catheter, which was a permanent feeding tube. The nurses taught me how to irrigate the line in his feeding tube, how to hang the bags with his liquid food, how to change the I.V. tubing, etc., in the hopes that Ted would be able to go home with the feeding tube. Ted was allowed to leave the hospital for two days at a time. While at home, he could not have food and could only drink water. I would have to irrigate the line on his feeding tube every eight hours. It was a direct line to his heart, and if any air got into the line, it could have been deadly. City of Hope would not allow Ted to leave the hospital permanently with me being the one having these responsibilities, because they could have been liable if something went wrong at home. The doctors checked around and found that UCLA Hospital would allow it though. So we considered transferring Ted there.
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Effects on the Family
During this time I was staying at the hospital in Ted’s room all of the time, partly because of him wanting me to be there, and also so that I could get practice working with his feeding line. It was baseball season for Todd now, so we tried to plan the days Ted came home around Todd’s games so that we could be there to watch. We were usually home two days every week. It was good to spend family time at home together and to spend time with friends. At some point during the time that Ted was coming home for these two-day visits, his Dad came from Wisconsin to spend time with us.
I know it was not easy for the children while Ted was in the hospital. Their caregivers changed frequently. We were so lucky to have such a wonderful support system, including friends and relatives that went out of their way to do whatever they could for us. Besides my mother and my aunt coming out to California, my sister-in-law from Arizona (the one who had previously lived in California) came up to take care of the kids for a week. The teenage friend who came to the City of Hope cottage and the teenage boy that stayed overnight with the kids were a great help. The children stayed for quite a while with some friends who lived in the Simi Valley, which was over a half-hour north of us. They both worked in Los Angeles, which was about the same distance south of Sylmar, where the kids went to school. Laurie wasn’t in school yet, except for three half days of pre-school each week. So our friends had to drop her off at another friend’s house in Sylmar. Her son was in pre-school with Laurie. Besides taking Laurie to school, she would take care of all three children after school until the other couple picked them up on their way home from work.
During the time the kids were staying with the friends in Simi Valley, Laurie developed pneumonia. I wasn’t allowed to see her for quite awhile, three weeks I think. The doctors were afraid I would pass the pneumonia on to Ted. That was very hard for me. Our friend had to take some days off work to stay with Laurie, and sometimes another friend in her neighborhood would stay home from the college classes she was taking to be with Laurie.
There was a time while Ted was in the hospital that Kim wasn’t allowed in, because she had a severe case of poison oak. She had to stay away as long as there was any oozing from blisters.
Todd was having a hard time with his emotions. He would start crying sometimes in class, so the teacher would send him into Kim’s classroom. She had the same teacher that he had the previous year when he was in fourth grade. That teacher was a good friend to the children and to me. I knew her quite well, as I worked as a teacher’s aide in the school. I didn’t go to work at all during the time Ted was in the hospital.
About the time we were ready to pursue my being able to take care of Ted at home, the doctors found that his kidneys were failing. They said that he could possibly live for another three months, but that he would not be able to go home except for his two-day visits. School was out for the summer, and Todd’s baseball season was coming to a close. Our nephew from Wisconsin (the son of Ted’s sister that had previously come out) came to visit. We all went to Todd’s baseball recognition picnic. That was the last time that Ted came home from the hospital. In fact, it was very hard for him to come home that time, as by then he was in a lot of pain and was receiving quite a bit of morphine in the hospital. I don’t know if Ted tried to hang on until school was out and baseball season was over, but it sure seemed that way to me. I do know that it was a blessing from God, a part of His master plan.
We decided that it might be easier and more enjoyable for Todd to go back to Wisconsin to stay for a little while, since we thought that Ted had about three more months to live. So, the day after the picnic, I took Todd and my nephew to the airport. I decided to sleep at home that night and go down to the hospital with the girls in the morning. Ted called first thing in the morning to see when we would be there. He thought it was already night time. When we arrived, he seemed quite confused. The cancer had now gone to his brain.
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Ted’s Final Days and Death
By the next day he was in a coma, and the doctors said he didn’t have much longer to live. I called my sister-in-law, so that we could make arrangements for Todd to come back to California. Her reaction to the news of Ted’s condition was so strong that Todd knew something terrible was going on. I had to tell him on the phone that his father would probably be dying soon. Ted’s sister was going to fly back with Todd, but she called me during the night before they were to leave to say she just couldn’t take seeing Ted the way he was and that Todd would have to fly back by himself. I felt so bad for him! During the flight back, Todd took pictures of the clouds out of the plane window. That may have been the beginning of his passion for photography.
The day after Todd got back Ted woke up again. It seemed like he knew that Todd was there, and he wanted to spend some more time with Todd, along with the girls and me. But the next day he was unconscious again, and he never woke up. Five days later the phone rang while the kids and I were asleep in the cottage. It was Ted’s nurse who said that he would probably be passing away sometime soon. So we got dressed and went over to the hospital.
After the end of the school year, the children had started staying with me in the cottage. One of the nurses on the afternoon shift had small children, and she asked if Laurie could come over to her house during the day. She would pick Laurie up in the morning and bring her back to the hospital when she came to work about 3:00 p.m. Before she was picked up that last day, I told Laurie that her Dad would probably be dying that day. She was only 4 years old, and her reply was, “Then can we go home and go swimming?”
About noon that day Kim and I went down to the lounge for a few minutes. I ate an egg salad sandwich. Kim couldn’t eat anything, because she was scheduled for a test later that day and had been preparing for it. The evening before she had to drink a laxative in the bathroom of Ted’s hospital room. They had switched to having the kids drink something white instead of castor oil. Kim gagged on it, and spit it out all over. I had guilt feelings for a long time for eating in front of her and for leaving Ted’s room when I knew he could die at any time. Todd was alone in the room with him. As I was walking out of the lounge, I saw the nurses scurrying down the hall towards his room. I rushed down there; but by the time I arrived in Ted’s room, he had passed away. I felt terrible! The male nurse that was in the room asked if I had some things I wanted to say to Ted, and I said that I did. So he took Todd and Kim out for a walk in the gardens while I said my good-by to Ted. The priest from the nearby parish had been coming regularly to visit us, and the thing that got me through this all was knowing that Ted had made his peace with God and that now he was in heaven with Him, with no more suffering.
I made some phone calls to let people know about Ted’s death, including one to his work. His best friend came right down to the hospital to help with details. Kim still had to have her test that afternoon, because it wasn’t fair to her to have to go through the preparation again if we rescheduled the test. Todd hadn’t been scheduled for tests that day, because we thought he would still be in Wisconsin.
Ted’s doctor told me that day that he wasn’t sure that they should have done what they did to prolong his life in the February surgery, as the quality of Ted’s life afterwards was not good. They also asked if they could do an autopsy, due to the rareness of familial polyposis, the disease that caused his death from cancer. I agreed to that.
The next day Ted’s friend from work went with me to make the arrangements at the funeral home, because the company paid for the entire funeral. They had paid Ted’s salary up until his death, knowing that he would never return to work. The secretary ordered all of the flowers, and they paid for two of the employees to fly back to Wisconsin to be pallbearers for his funeral here. He had started working at Anthony’s Manufacturing Company when the company was small, and he was well liked and appreciated there.
Our parish priest was out of town, so we had a priest from a near-by church for the funeral service at the funeral home in California. He asked several questions about Ted, and gave a very meaningful sermon. He said that Ted was up in heaven playing baseball with the angels. That was such a wonderful vision! I believe that during that service I felt closer to God than at any other time during my life. The room was filled to overflowing with all of our friends, Ted’s co-workers and mine, Todd’s baseball coach and the entire team, Kim’s Girl Scout leader and many of the girls. I felt so much love and support in the room, but especially I felt the love and support of God.
A couple days later we flew Ted’s body back to Wisconsin for his funeral and burial. His Dad, who was also very supportive, had planned it all for me. I felt bad for him. This was the third child he had lost, two daughters had died in separate car accidents when they were 18 & 23. Ted’s mother was also deceased. Many of our Wisconsin friends and relatives attended this funeral. After the meal at the church some of the people came over to my parents’ house, and after they left I felt somewhat empty.
The next day a friend from high school, who was also a relative of Ted’s, stopped by to see if there was anything he and his wife could do to help out. He told me to put the smile that he remembered back on my face. We stayed back in Wisconsin for the remainder of the summer, spending some time with his family and with another family from Wisconsin, who we became especially close to. The children became friends with their children and with the children who lived next door to my parents. Their mother also became a good friend of mine. I also rekindled some old friendships and made some new ones, along with spending much time with family. I think it was very helpful to spend that time in Wisconsin, instead of having to go back to our home in California right after Ted’s death and funeral.
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Life After Ted and Children’s Testing for FAP
We got back to California in time for school to begin. Laurie started kindergarten, Kim was in 5th grade, Todd was in 6th grade, and I went back to my job as an aid. We spent Thanksgiving, our first holiday without Ted, with my sister-in-law and her family in Arizona. It was about a 7-hour drive, the furthest I had ever driven. Ted had always driven when we went any distance. My parents and brothers came out to spend that Christmas with us. The following summer I figured that if I could drive to Arizona, I could drive back to Wisconsin. I wouldn’t drive much more than 7 hours each day to get there. So we spent that whole summer visiting friends and family again.
With all of the memories of what we had gone through at City of Hope, even though the doctors and staff were wonderful there, I didn’t want to go back for the children’s doctor’s appointments and tests. So we started going to Los Angeles Children’s Hospital. We no longer had health insurance, as it had been through Ted’s work and I only worked part-time through the school system. The two doctors we saw at Children’s Hospital did not charge us for their services, because of the disease being so uncommon and them wanting to know more about it. We did have to pay for the procedures that were done though, both at Children’s Hospital and at USC Medical Center. Todd and Kim had their first colonoscopies during that time. The liquid diet was longer back then, 3 or 4 days. And there were the laxatives and enemas. Poor Kim threw up on the bridge coming from the parking lot to the hospital the day of the colonoscopy. They were put out for the procedure there. Kim was sleepy the rest of the day, but Todd came out of the procedure raring to go. Thank God, no polyps showed up!
The third summer after Ted’s death we only went back to Wisconsin for three weeks. A friend talked me into looking for houses back here. Of the houses we looked at, I liked one better than the rest because the basement was finished. After having lived so long in California, where there were no basements, I had a fear of them. There was already an offer on the house, contingent on the sale of the couple’s present home. The children wanted to move to Wisconsin, but I wasn’t sure if it just seemed exciting to them. So, I decided that if we got the house, we would move to Wisconsin; otherwise, we would stay in California. I guess it was part of God’s plan for us to move back here, as we were able to buy the house. The children stayed in Shawano with my parents, so that they could begin school here, and I went back to California to pack. My brother Jim flew out to drive me back, pulling a u-haul behind us. We moved into our house on October 1, 1980.
Again we were blessed by not having to pay medical expenses for the children’s FAP follow-ups. Ted was part Menominee Indian, and he was on the roll. This meant that his children, being first-generation descendents, were eligible for medical treatment at the Menominee Tribal Clinic. If there are required services that are not provided there, you can apply to Contract Health Services at the clinic to see if they will pay for the services at another facility. Approval is based on the availability of funds and the seriousness of the patient’s illness. The children’s application was approved.
We were advised to see a gastroenterologist at Marshfield Clinic, which is where the children began to go for their doctor appointments and tests. It was then time for Laurie to start being tested, too. First she had to have a test done here, before the doctor could send a referral to Marshfield. The doctor from Keshena performed the test at Shawano Community Hospital. I watched as he inserted a proctoscope into her rectum. I don’t think he gave her anything to alleviate the pain, and I felt so very bad for her as I watched her feeling so much pain during this exam.
For a couple of years nothing showed up on Todd and Kim’s annual colonoscopies. But then the gastroenterologist found multiple polyps in both Todd and Kim’s colons; and after taking biopsies, he told us that they were the kind of polyps that go along with FAP. He encouraged them to have surgery done to remove their colons, the same surgery that their Dad had for his first surgery. He referred us to a surgeon at Marshfield.
The surgeon agreed that the children should have the surgery done soon. Since both doctors said that there weren’t many cases of FAP patients developing cancer in their teens, I decided to wait another year. Then after their colonoscopy the following year, I decided to put it off for another year. I kept hoping that a cure would be found before that time came. The surgeon also mentioned a new surgery, where the rectum would also be removed. The surgeon would create a J-pouch from part of the small intestine, to take the place of the rectum. This is a two-part surgery. The children would have to have a temporary ileostomy, while the J-pouch healed and the doctors made sure that it was functioning properly. Then they would have another surgery to have the ileostomy reversed. Neither Todd nor Kim wanted to have the “bag”. Also, I was skeptical, since the J-pouch surgery was so new, and no one knew the long-term effects.
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Todd, Kim & Laurie’s Initial Surgeries and Related Problems Afterwards
Finally the time came that we couldn’t wait any longer, and both of the surgeries to remove their colons were scheduled for the same day, shortly after school was out for the summer. Todd was 18 and had just graduated from high school, and Kim was 17 and was between her junior and senior year of high school. What a scary time that was for them and for me! By that time I had remarried, and we were lucky to have my husband Tom there with us to support us throughout this difficult time.
The children were admitted to St. Joseph’s Hospital in Marshfield the day before the surgeries. That night Kim was very, very nervous. She was actually shaking. The doctor had the nurse give Kim something to calm her nerves. Thank God that both of the surgeries went well, as did the time they spent in the hospital. Their rooms were across the hall from each other, and I went back and forth between rooms. Tom had to go back to work, but he came down on the weekend. The children were in the hospital almost two weeks. I stayed in a hotel across the street from the hospital that was mostly occupied with families of patients. Kim ended up needing a couple of pints of blood to help her get stronger. The kids had an NG tube, and they had to pass gas and have a bowel movement before they could go home. Kim accomplished this first, and I can still see Todd continuously walking the halls to get his system going.
Todd and Kim had the rest of the summer to recuperate before school started in the fall. Todd left Shawano to go to Milwaukee Institute of Art and Design, and Kim started her senior year at Shawano High. Their lives were pretty normal, except that they had to watch what they ate, and they went to the bathroom many more times a day than they did when they had a colon. They had to go in for annual checkups at Marshfield to check for polyps in their rectum.
Todd had to spend a few days in the hospital in Milwaukee twice with blockages due to food that he had eaten getting stuck where his small intestine was joined to his rectum. The doctor had Todd put on I.V. fluids to rest his system for a few days. The second time he had a blockage, the doctor also had an NG tube inserted through Todd’s nose, which went to his abdomen to remove gastric fluids. Thankfully, these actions took care of the problem.
Kim also had to go into the hospital for a few days once due to a blockage. The emergency room doctors in Appleton insisted that she was probably having so much pain because she was pregnant and was having a miscarriage. She explained that was not the case and told them about the surgery she had. After a couple days without eating and being on I.V. fluids, a wad of gum showed up in the toilet. That is what had caused her blockage.
Laurie kept going to Marshfield Clinic for her annual check-ups, which eventually consisted of having colonoscopies. You would think that with a 50% chance of getting the disease, Laurie would be the one of my children not to have the disease. However, at about the same age that the polyps showed up in Todd and Kim, they showed up in Laurie. She was seeing the same surgeon that performed Todd and Kim’s surgeries, and he again suggested the J-Pouch surgery. But Laurie did not want to have the temporary ileostomy, especially since she would be starting college in the fall. So the surgery to remove her colon was scheduled for shortly after Laurie graduated from high school when she was 17.
The surgery and the time in the hospital again went as planned. But after Laurie was home for a couple days, she started having a lot of pain and throwing up. So we had to take her back to Marshfield, and she spent another week in the hospital. By then it was after the Fourth of July. A little over a month later, Laurie started college in Madison. But after a few weeks, she was feeling weak and tired, probably because she was not fully recuperated. So she came back to Shawano for a semester and worked at a day care center. She began the second semester at the University of Wisconsin in Whitewater.
The following year, while she was again a student at Whitewater, Laurie had a blockage. She was in a lot of pain and had someone take her to the hospital in a nearby town. They didn’t give her anything for pain, because they wanted to see what the cause of the pain was. The doctors there eventually told her that they wanted to perform surgery the following day to see what was causing the blockage. Laurie stayed overnight in the hospital, and the next morning I drove down. We had her transferred by ambulance to Marshfield, so that her regular surgeon could treat her. Laurie was in so much pain that she couldn’t lie down. It felt better for her to sit in a more upright position. She couldn’t wait to have an NG tube inserted, because she knew that would help with the pain. They did have to do surgery and found that the blockage she had was caused by scar tissue from the first surgery. Laurie was in the hospital for about a week, but this time she recuperated faster and was able to return to school a few weeks later.
When the children had their first surgeries, we had thought that would pretty much take care of the disease as long as they continued to go in for follow-ups on their rectum and have any polyps that appeared burned off. However, as time went on, more was learned about FAP and other problems that were related to the disease. The doctors recommended that my children have the J-pouch surgery to remove their rectum, so that they wouldn’t have to worry about developing cancer in their rectum. The doctors recommended that this be done before the children turned 40 years old.
We were told that people with FAP could also develop pre-cancerous and cancerous polyps in their upper digestive tract, duodenum, and biliary tract. Cancers of the thyroid, pancreas and adrenal gland are more commonly found among FAP patients. Therefore, the children now have regular upper endoscopies, which involve more preparation, diet, sedatives and concern. They have found polyps in Kim’s duodenum and papilla, which have to be monitored. Todd had polyps in his ampulla, and the gastroenterologist removed the entire ampulla, which is a small organ near the pancreatic ducts.
As a mother, it is very hard to watch your children go through all of these procedures and surgeries, and to see them in pain. I wish I could go through some of it for them. It is hard to keep from being preoccupied by thinking and worrying about all of them. But in my mind I know that worry does not help them or me. So, even though I am always concerned about them, I have learned that there is nothing I can do about their physical problems. I can only be there to love them and to support them. And I have learned to trust in God to take care of them. That is what gets me through all of the situations that arise. I know that He loves them and He loves me, and He will always do what is best. I also have the support and prayers of many friends, which helps me a lot.
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Kim’s J-Pouch Surgery, Learning About Desmoid Tumors and Other Problems
After much encouragement from Kim’s gastroenterologist, because of the amount and size of the polyps she was getting in her rectum, Kim decided to go forward with the J-pouch surgery. Her surgery was scheduled for February 2004, just after her 36th birthday. At that time Kim lived in Collinsville, IL, which is just across the bridge from St. Louis, MO. The surgery was scheduled at Barnes Jewish Hospital in St. Louis, which is where she still goes for follow-ups with her surgeon and gastroenterologist. Tom and I went down to be with Kim for the surgery, and Todd came down the day of the surgery to be there when she came out of surgery. It was a very long day, in which we learned about another problem that FAP patients can have.
After Kim had been in surgery for four or five hours, her surgeon came down to talk with us. He said that Kim had a desmoid tumor, which was in the way of him being able to create the J-pouch. The J-pouch is created when the surgeon pulls down part of the small intestine and forms a J, which will take the place of the rectum. A desmoid tumor, which we had never heard of before that day, is a non-cancerous tumor, which can grow and take over other organs. Kim’s surgeon had accidentally nicked her left ureter while trying to create the pouch. So, while he was down talking to us, a urologist was up in the operating room inserting a temporary stent into Kim’s ureter. The surgeon told us that since he could not create the pouch, Kim would either have to have a permanent ileostomy or come in for more frequent check-ups on her rectum. We could not make that decision for her, so he said he would go back to the operating room and close her up after the urologist was finished. He said she should be out of surgery in about a half-hour. We waited and waited, for what seemed like forever. Finally, the nurse came over to tell us that the surgeon was able to create the J-pouch. It seems that after the stent was in place in the ureter, it was easier for the surgeon to work in the area where the desmoid tumor was located. So the nick the surgeon made ended up being a Godsend.
Kim was in surgery for over eight hours that day. In fact, the surgery that was supposed to be done after hers had to be rescheduled to another day. When Kim finally came down from recovery, she looked terrible. Her face was all swelled up, and she had a lot of tubes. Todd was so upset when he saw her that he had to leave the room for awhile. Kim had several complications before she could go home. Some of her staples had come loose, which caused her to go back into surgery to be repaired. She had an abscess in her pelvic cavity, which caused her to have a drainage tube for six weeks. Again she needed a couple pints of blood to make her stronger. But the worst thing was that Kim’s wound became badly infected. First the infection was in the upper portion of her incision, but eventually it went into the lower part of the incision also. This meant that her wound was open, and deep. It had to be cleaned and packed daily, and in order for her to go home, I had to learn how to do this. I never thought I would be able to do such a thing, as I have to turn my face when they show blood in movies and I didn’t do very well when the children were injured as they grew up. But I knew I had to do this for my daughter and as it turned out, it really wasn’t that bad. I doubt I could do it for a stranger though. It was very tricky to change her ostomy bag, with the wound being so close to the stoma for the ileostomy.
Finally, after about two weeks, Kim was able to come home. Tom and Todd had gone home to go back to work. Kim had gotten a divorce and was a single mother at the time, so I took off work and planned to stay with her as long as she needed me. Luckily, I worked for the Federal Government and I could use my own sick leave to take care of my daughter through the Family Leave Act. I had a lot of sick leave built up. The day Kim came home she couldn’t wait to eat a tuna sandwich. However, she became very sick to her stomach after eating it. She doesn’t eat tuna to this day.
One night after Kim was home, her son Colton came upstairs about midnight complaining about a very severe pain in his side. When I looked up his doctor in the phone book, I ended up calling him at home by mistake. He told us to take Colton to the hospital. I told Kim I would take him, but she insisted on going along. We took her daughter Cassie to a friend’s house and headed to the hospital. After several tests, they found that Colton had appendicitis, and he had to go in for surgery. The hospital gave Kim a wheelchair to use while she was there, they let her use the other bed in Colton’s room, and they gave us supplies for me to take care of Kim’s wound. Colton was in the hospital a couple days. So now I walked the halls with him. He was such a good patient.
A home health care nurse came to check on Kim periodically, and she had some visits at the wound-ostomy clinic at Barnes. The nurses suggested that she get a wound-vac to help speed up the healing of the open wound. Her insurance approved this, and the machine was ordered. Gray foam was packed into the wound, and the machine was constantly running. You could see blood being pumped out. If the foam came loose at all and air got in, the foam would have to be taken out, and new foam put in. It was very painful for Kim when the foam was pulled out. I could not do that. We had to contact the home health care nurse to come and take care of it. The constant draining of the wound-vac also became painful for Kim, especially in the lower part of her incision area. She was so relieved when she stopped using it. I was able to go home about that time, but not for long.
In mid-May, soon after she was healed, it was time for the second step of Kim’s J-pouch surgery. It was time for the reversal of her ileostomy. The surgery itself went okay, but Kim again had problems after the surgery. She could not eat without having severe abdominal pain and vomiting. She was in and out of the hospital for several weeks. She was back in the hospital when I was at my aunt’s funeral in West Bend, WI. I had brought extra clothes along, just in case Kim wanted me to come down to St. Louis to be with her. While I was at the luncheon after the funeral, I received a phone call from Kim’s roommate in the hospital. She said that Kim was so sick, and that I needed to come down. So off I went, and Kim ended up being in the hospital for 23 days. Luckily, I still had leave time left to stay there during that time, as we were able to take 3 months of Family Leave each year. I took it right to the limit.
Kim was feeling miserable. I can remember her throwing up during scans that were taken. I felt so bad for her! Her doctor finally figured out what the problem was. Her small intestine had collapsed at the point of reconnection. Kim had to have another surgery to repair the problem. She had to receive nutrition through a PIC line, because she was unable to eat for such a long time. She also experienced acute renal failure and had a dangerously high heart rate.
I spent a lot of nights sleeping in Kim’s room. One night the young doctor on call, who reminded me of the TV character Doogie Houser, checked her incision. He said that it was infected, and that he would have to open it up where the infection was. I became very upset, remembering what Kim had gone through with an open wound previously. I tried to get him to wait, but he said it needed to be done. Thank God, this time the open wound was only a few inches long.
The staff felt bad for Kim for all she was going through. One day two of the patient care techs planned a spa day for her. They did her hair, put cucumbers on her eyes, and gave her a facial and a pedicure. Everyone liked Kim. She was a very good patient. I was really proud of her!
Towards the end of the time Kim was in the hospital, my daughter Laurie gave birth to her second child. Laurie lived in Indiana, and I had planned to be with her, as I had been when her first child was born. Her mother-in-law went down for a week, and I planned to go to Indiana when she went back to Wisconsin. The following Monday, several days after Kim got home from the hospital, we went to the wound/ostomy clinic at Barnes to have her open wound flushed, packed and dressed. While there I received a call from my brother letting me know that my mother, who was in a nursing home a couple of blocks from my house in Shawano, was not expected to live much longer. My Dad had passed away about 8 months earlier, after spending 4 years in the nursing home. He had a stroke, which resulted in him developing dementia and not being able to speak. He had been a salesman, so that was very hard for him and for all of us to see. Laurie called me the day after his funeral to tell me that she had just found out she was pregnant. I felt like God was giving us a gift.
The wound/ostomy specialists gave us supplies, and they told us that it would be okay for Kim to drive with me to Wisconsin. We went home to pack and headed for Wisconsin. I called my mother on the drive home, and even though my brother didn’t think she could talk, I said I wanted to talk to her. She said something like, “Jolane, you’re coming home?” She seemed relieved to know that. We got to Shawano about 8:00 that evening and went straight to the nursing home to see my mother. She was worried that Kim would catch pneumonia from her. But I explained that she didn’t have the kind of pneumonia that was contagious. We spent most of the next day with her. Todd came up from Milwaukee, too. Later, while we were at home for supper, I received a call from the nursing home letting me know that Mom had passed away. We all went back to the nursing home. The nurse asked if we wanted a priest to come. The priest from our parish was on vacation, so the priest from a nearby parish came to pray with us. Our priest agreed to come back early from his vacation, and we had the funeral on Friday. Following the funeral luncheon, we all went down to Indiana to see Laurie’s new son Gabriel. A friend of Kim’s had brought her children up to Wisconsin for the funeral. The four of them went back to Collinsville at the end of the weekend, Tom and Todd went back to Wisconsin, and I stayed with Laurie for about a week to help out and to spend time with my new grandson and his sister Zoe. And then I went home and back to work.
The following year Kim had to have surgery to remove a superficial desmoid tumor. She still has four desmoid tumors located in her abdominal and pelvic cavities. She takes medication to control the growth of these tumors, along with having annual CT scans to check on them. Also, as previously mentioned, she has had adenoma tissue in her duodenum and major papilla. Kim asked her doctor if her children would have to worry about these problems, and he told her that if one person in an FAP family has them, then all of them tend to have them. We just keep on praying.
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FAP Research and Genetic Testing
Through the years research was done to determine what gene was associated with FAP. Our family was asked to participate in this study and all three children and myself submitted samples of our blood to be used in this research. It was determined that FAP is caused by a mutation in the Adenomatous Polyposis Coli (APC) gene. A blood test can locate the change in the APC gene in about 80% of families with FAP. If a child has the gene mutation, he or she will eventually develop FAP. If the gene cannot be detected in the parent with FAP, their children must continue to have the regular colon screening that my children had. We considered this new way of testing a blessing for our grandchildren. They would not have to go through the discomfort and preparation for testing at an early age as my children did. It was recommended that children have the DNA test done by age 10.
Kim, the children’s father and I met with a genetics doctor at Washington University, which was attached to Barnes Jewish Hospital in St. Louis, to discuss how to proceed with their children Colton and Cassie. Colton had some blood in his stool when he was about 10; so, because of his family history, he had to have a colonoscopy at that time. No polyps showed up, and it was determined the blood was from strain. However, since he had had the colonoscopy, the doctor said it would be ok to wait to have his DNA test done until Cassie reached the age of 10. Colton was 13. First, Kim had to have a DNA test done to identify the mutation of the gene in her. It turned out that she was one of the 20% of FAP patients where the APC gene mutation could not be detected. We contacted Johns Hopkins to have them look at the results of the blood we had submitted to them. It turns out that they also couldn’t detect the mutation in Kim’s sample, but they could in her brother Todd’s and her sister Laurie’s. So, they sent information to Laurie to take to her doctor, who in turn took blood for a DNA test on Laurie. This provided the information that was necessary in order to do DNA testing on Colton and Cassie, who were tested in May of 2006. The vials of blood from Colton, Cassie, Kim and Laurie were all sent from Washington University in St. Louis to a hospital in Philadelphia to be genetically tested. Through that process they were able to find the gene mutation in Kim’s blood. Six weeks later, we found out that both of the grandchildren had the gene mutation, and therefore had FAP. What a shock it was for all of us!
We had been told that if the disease does not show up in a child of a parent with FAP, that would end the dreaded disease for that family. It does not skip generations. We certainly had hoped and prayed for that to happen. Kim felt very guilty for passing this disease on to her children. When we lived in California, all the doctors advised that my children not have children because of passing the disease on. However, their surgeon at Marshfield disagreed. He said that because removing the colon could treat the disease, and because a “so called” normal child could die crossing the street; if he was in this situation, he would have children. It was very hard for my children to decide what to do. We continued to hope and pray that there would be positive breakthroughs in the future. Kim and Laurie have each been blessed with two beautiful children.
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Colton & Cassie’s J-Pouch Surgeries
About a month after receiving the DNA results, Colton and Cassie had their colonoscopies. We assumed that Cassie, being so young, would not have any polyps. However, we were in for another shock. Both of the children already had hundreds of polyps, and the doctor suggested that they both have the J-pouch surgery. The children went to a pediatric surgeon at Cardinal Glennon Children’s Hospital in St. Louis. He was set to schedule their surgeries with the same long incision down the front of their abdomens that my children had. Kim and the children’s father had been divorced for quite some time, and now she was remarried to Rob. The children were covered under Rob’s health insurance, which is why they went to Cardinal Glennon. After January 1, 2007, because of a change of insurance, they were able to go to St. Louis Children’s Hospital, which is a part of Barnes Jewish Hospital. That was another great blessing.
There was a pediatric surgeon there that would perform laparoscopic surgery. Colton and Cassie were scheduled to have surgery right after school ended for the summer. And I planned to retire at that time. I had been eligible to retire when I turned 60 in January of 2005, but I found it hard to make the break. I had worked at my job at USDA Rural Development for over 24 years, and I just couldn’t seem to decide on a time to leave. The job was such a big part of my life, and my co-workers were such good friends. I decided that when the grandchildren had their surgeries, I would finally take the step, so that I could be with them and help Kim out. Well that time came sooner than I expected. The children’s surgeon planned to move out to California to take over the private practice of a doctor out there when he retired. And that doctor decided that this was the time that he wanted to retire. Colton and Cassie’s surgeries were moved up, so that they could be done before the surgeon left for California.
Colton’s surgery was scheduled for March 19th, 2007, and Cassie’s was scheduled for the following day. They were admitted to the hospital the day before the surgeries, so that a NG tube could be inserted, while they were awake, and prep fluids could go through the tube instead of the children having to take them orally. Colton had a rough time of it, but all in all, they were such troopers through this. Tom and Todd also came down for these surgeries. It was hard for all of us to watch what the kids had to go through at such a young age. Both surgeries went as planned, but again there were some problems during the time they were in the hospital.
Colton began throwing up after he was able to eat. The doctor found free-floating air under his diaphragm, which was 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 anything after seeing her brother go through what he did. She was afraid that she was going to need another surgery, too. There was a wonderful children activities director there, who Cassie got very close too. She suggested that we keep a chart for Cassie, and every time she ate or drank something, she would get stars to earn a reward. This seemed to help.
Colton also had a very hard time emotionally. He seemed angry and didn’t want to talk to anyone, or even look at us. The staff recommended that he talk with a therapist. So he was visited by a counselor, and also by an art therapist. He got better as time went on. Colton and Cassie both finally got to go home after twelve days in the hospital. An ostomy nurse came several times to teach us how to change their ostomies and to give the kids and us some helpful hints. Kim and I already had pretty much experience with ostomies, but all of this was new to Rob.
Colton and Cassie didn’t go back to school while they had their ostomies. The school arranged for homebound teaching for the remainder of the year. The retired teacher who came to the house got assignments from their teachers, and it worked out quite well. At this time Kim was going to school to become a nurse, and she was also working part time as a patient care tech at Barnes Jewish Hospital on the colorectal floor. I traveled back and forth to their house to help out, planning my trips according to both Rob and Kim’s schedules.
Colton and Cassie’s takedown surgeries to reverse the ileostomies were scheduled for May 29th. The surgeon who did their previous surgeries had moved to California, so their current surgeon performed these surgeries. Cassie went into the operating room first, and the surgeon found that she was not ready for the reversal. She had a very tight stricture, along with grayish mucus and granulation that the surgeon attributed to a leak in her pouch. Colton felt bad for his sister, as he went into the operating room. Thank God, he was ready, and his surgery went as planned.
Cassie had to go into the operating room for anal dilation procedures every Tuesday for the next 4 weeks. She took this setback very well. She had a contrast study done, and finally, the surgeon thought she was ready. But he wouldn’t know for sure until he started the surgery. The surgery for Cassie’s takedown was scheduled for July 3rd. The surgery went as planned. What a relief for everyone! The following day there was a 4th of July celebration in the children’s activity room that was a few floors down. Cassie wanted to go, even though it was so soon after her surgery. We took her down in a wheel chair, and the activity director was so happy to see her. The feeling was mutual. I had to leave to go home three days after Cassie’s surgery, because my stepdaughter was getting married the following day 7/7/07. I made it home just in time for the rehearsal. Cassie was upset that I had to leave while she was still in the hospital, and I hated to leave her. But I had no choice.
In 2009 Colton and Cassie had a thyroid ultrasound, which noted no abnormalities. They have also had upper endoscopies, in which they both have had adenoma tissue in their duodenum. They will have to have these scopes done regularly, as well as regular flexible sigmoidoscopies.
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Todd’s Plans for J-Pouch Surgery
Todd did graphic design work for advertising agencies in Minneapolis and later in Milwaukee. During those years, while he had health insurance through work, he was pretty faithful in having his regular checkups done. Then he decided to do free lance work, and he had no insurance. He didn’t go in to be checked out for over five years. This was a very dangerous chance to take, as he could have developed cancer in his rectum or upper digestive tract during these years. He often complained of gastric pain, and went to the bathroom often, and always had to go right after every meal. We were all worried about him and tried to convince him to go to the doctor.
When Laurie’s husband went to Iraq with the National Guard, Todd moved from Milwaukee to Green Bay to stay with his sister and her two children. When Laurie went to her gastroenterologist for an appointment, we talked to her doctor about Todd. He said that Todd needed to get in to be seen, and that he should schedule an appointment for a flexible sigmoidoscopy as soon as possible and just make very minor payments each month. Laurie and I convinced Todd to do this in August of 2007. After completing the procedure, the doctor reported that Todd had many polyps and that several were very large, over a centimeter in size. He recommended that Todd have the J-pouch surgery done very soon.
Since Todd did not have insurance, this was very difficult to do. In September 2007 Todd moved back to Shawano. My parents had passed away, and my brother J.A. decided he couldn’t handle the financial responsibilities of the house and moved into an apartment. Todd moved into the house to refurbish it to be sold. He did much repair work, painting, and redid the hardwood floors. J.A. and I spent a lot of time cleaning out all of the cupboards and closets and the basement of the big two-story home. It was bittersweet, as the house held a lot of memories. One day J.A. told me that he wasn’t feeling well, so he was going home to his apartment. I called him when I left for home several hours later, and he told me that he felt better after lying down for a while. I called him a couple days later, and he didn’t answer. I tried again the next morning, and he still didn’t answer. I picked Todd up and we went over to check on J.A. His car was there, but he didn’t answer the door. The door was locked, so Todd took out the living room screen and went into the apartment. He found J.A. lying on the bathroom floor, and he was unresponsive. We called 911, and the ambulance was there in minutes. They confirmed that J.A. was not alive. He had died of a heart attack. This was such a shock, and something that still gives me much anguish. It helps me to know that now J.A. is in Heaven with Mom and Dad, who he was so close to in life, and he is also with God.
We checked with the Menominee Indian Tribe to see if they could again help Todd with his medical expenses. After a lengthy application process and the 90-day residency requirement, Todd was approved by contract health services. He was required to live within 30 miles of the reservation. By then it was December, and the grant funds for the year had been used up, so Todd had to wait until the new funds were awarded in January.
The gastroenterologist had recommended a surgeon in Green Bay, but after checking with his office, it didn’t appear that he had performed many of these surgeries. So I checked out surgeons at Marshfield Clinic and at University of Wisconsin Hospital and Clinics in Madison. One doctor at Madison sounded very well qualified, so I called his office and he called me back. He was very knowledgeable and very interested in treating Todd. He had even studied under the doctor that had invented the J-pouch surgery.
The surgery was tentatively scheduled at UW Hospital for the third week in January 2008. Todd was required to have a CT scan done prior to the surgery, which was done at the hospital in Shawano. A few days later he received a surprising phone call from the local doctor informing Todd that he saw an abnormality in Todd’s left ureter and kidney. So, Todd had to meet with a urologist at UW Clinic before he could have his surgery. Todd had tests done to determine the extent of kidney damage. The flow test showed that Todd’s right kidney was doing two-thirds of the work. After reviewing the results of the flow test, the surgeon that would perform the J-pouch surgery expressed to Todd that he was surprised to see that the kidney was functioning at all, because before the flow test was done he had thought the kidney was already dead. The test results meant that the urologist would also have to be in the operating room when the J-pouch surgery was done, so that he could insert a stent in Todd’s left ureter. It took quite a bit to coordinate the surgery date into both surgeons’ schedules, but it was finally set up for April 3.
Todd had been in surgery for several hours, when a resident came to talk to Tom and me in the waiting room. It seems that Todd also had a desmoid tumor that was interfering with the surgeon’s ability to create the J-pouch, and Todd’s surgeon said it would be impossible to do. The resident wanted me to give permission for the surgeon to give Todd a permanent ileostomy. I could not believe this was happening again! We could not make that decision for Todd, which is what I told the resident. A short time later the surgeon came down and discussed the situation with us, while the urologist was putting the stent in. We called Kim for advice, since she had been in the same situation as Todd. She was put on speakerphone for the conference. She agreed that the decision had to be made by Todd. Kim had been planning on coming up to be with Todd the following day, but she was so upset that she decided to leave for Madison right after this phone call. She was at school, but talked to her professor and left to go home and pack.
The surgeon went back to the operating room to close Todd up. He said he also closed some tears, put an anti-adhesive barrier to prevent scar tissue, and put clips around the desmoid tumor. He said that if the clips moved on an x-ray, it would show that the tumor had grown. They had also taken biopsies of the desmoids, even though it was almost certain that they would not be malignant.
We waited for Todd to come back from the recovery room. When he got back to the room, he kept feeling around for the temporary ileostomy bag that was supposed to be attached to his abdomen. He couldn’t find it and was very curious and upset. He knew that something was not right. Todd said that he had already noticed this while he was in the recovery room. It had gotten so late that the doctor had gone home after the surgery, before he had a chance to talk to Todd or to me. He called me on the phone, and I told the doctor about Todd’s frustration. The surgeon told me that Todd would be so out of it that night that he would forget about it. He said he would explain it to Todd in the morning. But he didn’t know how Todd reacted to anesthesia, as Todd was awake enough to understand. To make matters worse, a nurse or tech had mentioned to Todd that he heard that Todd would have to be having chemotherapy, which scared him more. So, I explained to him what had happened in the operating room and I also explained about the chemotherapy. The surgeon had mentioned to us that Todd would be referred to an oncologist to treat the desmoid tumor, and he said this would be for chemotherapy. But that term refers to oral medicine, as well as I.V. medicine, because it is a chemical.
The anesthesiologist had inserted an epidural for pain medicine after surgery, which made it much less painful than previous surgeries had been. The pain medicine was centered at the location of the pain, instead of having to work it’s way through Todd’s system. This made it easier for Todd to get out of bed and walk around, which he did a lot of because of the stress he was under about the outcome of the surgery. He has a tendency to pace when he is upset. Todd was in the hospital for eight days after surgery, and it was not an easy time for him emotionally. Kim had stayed for several days, and Tom went home after the weekend. When Todd could finally go home, we had a three-hour drive. The bumpy ride through Madison, because many road repairs were needed, and the long drive to Shawano, caused Todd to be nauseated. His recuperation went well, but he had many other medical procedures ahead of him.
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Todd’s Continuing Medical Issues
Todd was going to Madison every six months for appointments with the oncologist, which included CT scans to monitor the growth of the desmoid tumors. They said that the desmoid tumor that caused the problem with the surgery was about 8 centimeters, where the small bowel looped on itself. The two oncologists Todd met with did not know much about desmoid tumors, as they are uncommon. The doctors mostly dealt with cancerous tumors. They said there was a lot of talk at medical conventions about desmoids, but not much consensus throughout the country on what to do about them. They prescribed higher doses of Sulindac and Tamoxifen. Todd took these oral medicines initially, but eventually went to taking Sulindac sporadically, and now I don’t think he is even taking that. Both of his sisters are taking Sulindac. Eventually, Todd only met with the younger doctor, who did research desmoid tumors, but still was not sure what the best route to go was. The CT scans never offered much information, so the doctor ordered a PET scan, which also was not much help. He said he would try an MRI next, but Todd became so discouraged with the outcomes of these appointments that he decided not to go back to the oncologist. The doctor tried to contact Todd, but Todd did not respond.
Todd went to a gastroenterologist at Madison in June of 2008 for a flexible sigmoidoscopy and an upper endoscopy. The doctor removed about 25 polyps that were over 5 mm and a couple over a centimeter in size. Biopsies were taken, and thankfully, none had turned to cancer. The doctor said he would remove the rest of the smaller polyps when Todd came back in six months. The upper endoscopy showed that Todd had abnormalities in his ampulla, a small organ located near the pancreatic ducts. Biopsies were taken, and the doctor said that if they showed that it was adenoma tissue, which is precancerous, he would schedule a procedure to remove the whole ampulla. It turned out to be adenoma tissue and the procedure to remove the ampulla was done in early December. Later in December Todd went back to the gastroenterologist to have the remainder of the polyps removed from his rectum. He went back again last December to have polyps that had appeared during the year removed. Thankfully, there were no large ones.
Most of Todd’s trips to Madison have been to the urologist. He has had various renal scans done, and he has had to go for outpatient surgery to have his stent changed every three months since his surgery in April of 2008. At first it was difficult for the surgeon to insert the new stents, because Todd’s ureter is not straight. It is all curved up because of the desmoid tumor. It seemed to get easier as time went on. After a few times, the urologist started using a larger stent for better drainage. He found there was a slight improvement. The doctor was very frustrated with Todd’s situation, too. He didn’t know what would be best for Todd overall. It is true that people can live with only one kidney, but because of the possibility of Todd developing a desmoid tumor near the right kidney, he didn’t feel it was safe to let the left kidney die. He said that if the stent was removed, the kidney would probably die sooner, rather than later, probably in a few months. He said that if he pulled the stent out and the kidney died, it could be painful. He mentioned putting in a metal stent, which he himself had never done before. This type of stent could stay in for about a year. The doctor said that it might be problematic to get it into Todd’s ureter. He said he would research it more, and that he might be doing this procedure with a patient he had at the Veteran’s Hospital. He indicated that he would set up an appointment to discuss this with Todd before trying it. However, when Todd went in for his last stent change in July of this year, the urologist said he had decided to insert a stainless steel stent, which he did. Todd was a little leery about this, having had no advance notice.
Todd often has had blood in his urine, sometimes with blood clots, ever since he has had a stent in his ureter. This usually happens right after the surgeries to change the stents, and when he does any heavy lifting or strenuous work. He also has a lot of pain in his left side flank, the area of his stent. It seemed that after the stainless steel stent was inserted, he had more problems with this. So he called the doctor, who scheduled an appointment. The urologist ordered a urine sample and an x-ray of the area where the stent is. There was no infection in the urine, and the x-ray showed that the stent appeared to be in the correct position. He did suggest removing the metal stent and going back to the original type of stent, which would have to be changed every three months. But Todd was uncertain how long he would be in Shawano and be eligible for medical assistance from the Tribe, so the doctor said he would leave the stainless steel stent in.
I went with Todd to most of his appointments in Madison, and I also became very frustrated with a lot of the results. The doctors and staff were very good and supportive, but the issues seem to be so uncommon and no one knows exactly how to treat them. I definitely can understand where Todd is coming from when he gets discouraged.
Todd was not happy living in Shawano. At times he became very depressed with his living situation and with his medical problems. In November of 2008 my parents’ house was sold, and Todd moved into the upper floor of a duplex. At first I helped him with his rent payment, but eventually I decided that he had to take responsibility for it himself. I was not helping him by supporting him. I wondered how much damage I had already done to him, by letting it go on this long. He did some work for some of his friends who were in the art field. And he had a close friend from Green Bay, who also helped him out. From month to month, we didn’t know if he would come up with his rent payment. Todd went through times of severe depression, and I worried about him and what he might do. He went for counseling for a while in Keshena. Todd stopped going to the counselor, because he didn’t think it was helping. Sometimes he would get angry with me and hold grudges for things I said. I only wanted what was best for him, but I didn’t know what to say or do. I started going to some prayer groups, and I got support there, both from the other women and from God. These women, along with many other friends of mine, also prayed for Todd. I wish that he could have the kind of support that I have and the faith in God. Even though it is sometimes hard to believe, I know that God has a plan for Todd. When I start to worry, I say this prayer, “Jesus, I trust in You. Please take care of Todd”.
In July Todd decided it was time for him to leave Shawano. At the end of the month, his friend from Green Bay and I helped him to move down to southern Wisconsin to stay with the friend he was doing work for. He stayed there for about a month, and now he is staying a short distance from there. His best friend from Milwaukee Institute of Art & Design, who Todd has remained friends with and done much hunting with throughout the years, has a studio down there. It is in an old church and his offices are in the house that is next to the church. There is also a bedroom, bathroom and kitchen in the house that Todd is using for his living quarters. Todd is doing some work for this friend in exchange for staying there.
While staying there, Todd came up with an idea, which he is still refining. He would like to find some backing to take a motorcycle ride across the country, possibly starting at the City of Hope Hospital where his father was treated, and where Todd began having his tests for FAP. He would like to bring awareness to his disease, along with several other causes. He would document his ride in video, photos, and writings. He always has his camera with him and has been taking many beautiful pictures lately. He sends some to me, and I keep telling him how talented he is and that he has so much to offer. Todd was so motivated, and he sounded in better spirits than he had in a long time. I was so happy for him, and I pray that he will follow in this direction.
The problem with Todd living away from this area is that now he has no medical coverage. The urologist had scheduled an office appointment for him this month that he had to cancel. The urologist’s office called back to remind Todd that he needs to have his stent changed in six months. It is lucky, or part of God’s master plan, that the doctor put in the stainless steel stent, and Todd didn’t have to have his stent changed this month. Todd’s gastroenterologist scheduled an appointment for scopes to be done next month that he also will have no coverage for. Jesus, I trust in You. Please take care of Todd.
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Laurie’s J-Pouch Surgery
Laurie started to have more polyps in her rectum, between 36 and 47 each time, when her gastroenterologist performed her flexible sigmoidoscopies. He removed all of them by lasering off on the surface of her rectum. The doctor said he wanted to save the rectum by removing all of the small polyps and with closer follow-ups, every 3 or 4 months, instead of every 6 months. He did recommend a surgeon in Green Bay in case Laurie wanted to start thinking about having the J-pouch surgery. After seeing the problems that both Kim and Todd had with their surgeries, Laurie decided to make an appointment with Todd’s surgeon in Madison. At this appointment, Laurie decided to go ahead with the surgery after school was out in June of 2008.
Laurie had an upper endoscopy done at the end of April, when Todd went in for his post-op appointment. No abnormalities showed up on Laurie’s scope, and none ever have on any upper endoscopies that she has had. She also had to have a sphincter test done, because this surgeon also planned to remove Laurie’s anal lining, which Kim and the grandchildren’s surgeons did not do. Laurie had a CT scan done in May, with no problems noted, and her surgery was scheduled for June 6.
Thank God, Laurie’s surgery went well, but not quite as planned. Her surgeon was planning to do Laurie’s pouch in the shape of an S or W, which was a newer method making the pouch larger. That would help her not to have to go to the bathroom as often. But it was a good thing that Laurie had the surgery done when she did, because the surgeon found several good sized desmoid tumors, about the size of a nickel, that caused shortening and tethering (fine suturing) of the pouch. So she ended up having a J-pouch.
Tom was again there with me, along with Laurie’s husband Jerry, and Kim and her family came up the day of the surgery and stayed for the weekend. Kim stayed in the room with Laurie at night and Laurie said that she was a big help. The following day there were thunderstorms, with tornado warnings late in the afternoon and into the evening. All of the patients had to be moved out of their rooms and into the hallways. Laurie sat out there in a chair, nodding in and out of sleep. People were eating their dinners, but Laurie couldn’t eat yet, so she had to smell the aromas. Jerry drove home through the storms, as he had to work the following day. Todd came down to Madison on his motorcycle through the storms. He spent that night in the room with Laurie. He wanted to be there for his sister, but it was a reminder of what had gone wrong with his surgery and that he was not able to have a J-pouch, and it became difficult for him to be there. Kim’s family and Tom went home on Sunday, and a friend of Laurie and Todd’s came down that day. Todd and the friend stayed in Madison for several days, and Jerry came back down during his next days off. There was another storm and tornado warning the day Jerry drove back to Madison, which was also the day that Todd rode his motorcycle back to Shawano. Todd had a passion for riding his motorcycle, but the rides to and from Madison were very wet and quite adventurous.
Laurie also had an epidural for pain, which helped. She was in the hospital for a week, and then also endured the bumpy, long ride home to Green Bay. We had to have her pain medication prescription filled on the way home. Laurie had a lot of gas pains for the first few days, and spent much time walking to relieve them. She also had problems sleeping in bed, and spent a lot of time in the recliner. A home health care nurse came to help with changing her ileostomy bag and to check on her, and I stayed at her house for a while to help out. Her recovery progressed, and on July 24 she went down to Madison to be dilated. On July 28 she went down for a barium check of her pouch and for the pre-op for her takedown surgery, which was scheduled for August 8. That surgery went well, and she was only in the hospital for four days. Jerry and Tom had again come down for the surgery and the weekend, and Todd and his and Laurie’s mutual friend also came down to visit Laurie the evening after her surgery.
She had to go back for another dilation in September. At her post-op appointment her surgeon told her to call for an appointment with him in 1 ½ – 2 years see if he has to dilate again, but to call him sooner if she has problems, especially with strain in going to the bathroom. He also told her to check with him before she has any other type of surgery, as that could disrupt the pouch.
Laurie has not had any major problems since her surgery. She found that she was more tired than before, so after some blood tests, they found she was low on iron. She now takes iron supplements. Most foods agree with her system better than they do with her brother and sister. She said that she thinks she goes to the bathroom less now than she did before her 2008 surgery. She continues to have upper endoscopies with her gastroenterolgist in Green Bay. She also was going to Madison every six months for CT scans and to meet with the same oncologist that Todd met with. She is taking Sulindac. At her last appointment we mentioned that her sister was only going in for annual CT scans and appointments to discuss the results. Laurie’s doctor agreed that it would be ok for her to come in annually also.
Laurie’s children, Zoe (8) and Gabe (6), are the only members of our family that still have to be checked for FAP. Laurie and her husband Jerry have met with a genetics specialist, who confirmed that they should have the DNA testing done by the time the children reach the age of 10. The doctor suggested that Laurie and Jerry take a life insurance policy out on the children before they are tested. We all are hoping and praying that Zoe and Gabe will be the ones in our family that do not show up with the mutation in the gene. I think it’s about time the odds are with us.
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Take Care of My Family
After 37 years of dealing with this life changing experience for our entire family, it has become a part of me as well as of my children, their father, and our grandchildren. It will also affect the lives of future generations who inherit the FAP gene mutation. Even though I have not had anything to deal with physically, the disease has affected me in many other ways. It has been, at times, emotionally draining. I have tried to be as supportive and compassionate as I can with my children and grandchildren. I have learned so much about Familial Polyposis and spent so much time in hospitals and at doctor appointments, that sometimes I feel like I am in the medical field. But, I am not feeling sorry for myself. My children and grandchildren have provided me with much more joy than worry and sorrow. In most ways, they can go about living their lives normally. And I will always love them unconditionally and continue to pray for them. Jesus, I trust in You. Please, take care of my family!
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