Since I was only knee high, I’ve dreamt of being a mom. I always hear people say they were blessed with their children. While I absolutely agree that my three beautiful kids are blessings in my life, I made the conscious decision to bring them into this world so that I could love them, cherish them, care for them, provide for them and protect them. It’s what I feel I was created for and it is one of the greatest joys in my life. However, in all my dreams of being a mother, FAP didn’t exist. From the moment they told me my babies were healthy after they were born, I never once imagined that this would be our life. Guilt has never plagued me because I didn’t know I had FAP during my years of having children, but I have questioned every decision I’ve ever made for them. I wouldn’t be honest if I said I put up a good fight every single day, but my journey has taught me when to fight and how to fight.
I was raised by my mother and adoptive father. I knew of my biological father and his family, but I wasn’t a part of their lives until I was in my late 20’s. In 1992, when I was 15 years old, my mom received a call from my biological father expressing concern that I needed to have a colonoscopy to check for polyps. My grandmother had died in the 70’s due to colon cancer and my uncle had just died from colon cancer in his 30’s. My dad had a colonoscopy after my uncle died. He, too, in his 30’s, had a colon blanketed with polyps and he was diagnosed with Familial Adenomatous Polyposis. My grandmother and uncle had FAP, as well. At the time, the medical field was still baffled by this rare disease. The emphasis on regular screenings for people with a family history of genetic disease was almost non existent. They told my dad if he had a colectomy it would save his life. He underwent a successful surgery shortly after. Not long after his surgery, my first colonoscopy was scheduled when I was 15. My colon was completely clean. Not a single polyp. They told me to have another colonoscopy in 10 years. My mom didn’t know the importance of regular surveillance and I continued on with my life without feeling the threat of this disease.
Seventeen years and 3 kids later, I had already begun my FAP battle, but I didn’t know it. In 1997, my oldest son was 18 months old, and tripped and fell causing him to hit his head very hard. He cried continuously for hours holding his ear. After taking him to the ER, and after numerous tests, they found he had a very large polyp in his ear. They were unsure what had caused it but told me that I shouldn’t worry. Sometimes those things just happen, they said. He was treated and later I learned he had lost 30% of his hearing in that ear due to the polyp perforating his ear drum and growing into his inner ear. A couple of years later he started having dental problems. The enamel on his teeth was damaged, he had missing teeth in some places and extra teeth in other areas. The dentist was convinced that it was due to him being ill as an infant and toddler and needing so may antibiotics, along with running high temps. I took care of the issues and moved on, still not knowing that his life was being affected by FAP. He dealt with multiple sebaceous cysts, another complication from FAP, over the years. Most of the cysts being on his forehead and in his knees. The cysts that were in his knees were painful and caused issues with sports and running. He always kept going though. It never stopped him.
Skipping ahead a couple of years, it was time for the family to get our annual eye exams. Several years earlier, Ashton, my oldest son and the one I mentioned above, and I, were diagnosed with CHRPE (congenital hypertrophy of the retinal pigment epithelium), also known as retinal tumors and another complication from FAP, and diagnosing him was missed. I was told that I must’ve had a virus while I was pregnant with him and it caused us both to have CHRPE. During that appointment, Ashton needed glasses for the first time. He had a longer haircut and I went to brush his hair away from his face to get a good look at a pair of glasses he was trying on. Instantly, I felt a large egg-sized lump at the edge of his hairline. It was concerning, but he always had trouble with cysts in that area and I thought the lump was most likely the same thing. I still remember the conversation we had sitting in the waiting room at the optometrist’s office. Ashton was 13 and had kissed a girl for the first time at school that day. Mom moments. I’m still grateful for that pair of glasses. Every single day, I’m grateful. Our lives changed after that, but later it would save Ashton’s life and mine.
I made an appointment with our family doctor the following day and she ordered an x-ray of Ashton’s head. The x-ray was inconclusive so they ordered a CT scan. The doctor called several hours later to tell me that Ashton had over 20 boney growths on his skull and jaw. The radiologist was unsure of what they were and we were sent to a pediatric neurosurgeon. I can’t tell you how many horrible thoughts and ideas I had about what could be wrong or what they would need to do, but not once did I think it would be linked to colon cancer. We met with the pediatric neurosurgeon, who ordered more testing. After two weeks of tests, he told me that they were benign bone tumors, osteomas, and that Ashton was just born that way. I don’t know about any other parent, but that answer or reasoning did not sit well with me. I walked out of his office with a feeling of terror that something more significant was being missed. I have a good friend that is married to a neurosurgeon. He doesn’t treat children, but I called her to see if he would at least look at Ashton’s CT scan and point me in a better direction. I brought the disc to him later that day and after looking at it he took it to Children’s Medical Center in Dallas. He met with several doctors to get their opinions. Hours later he called me and told me to schedule Ashton for a colonoscopy, as soon as possible. Strangely, I instantly knew it was connected to the disease that my father’s side of the family had. Dr. Morgan felt confidant the osteomas were caused by FAP, but Ashton would need more testing to confirm the diagnosis. I didn’t waste any time. Ashton had his first appointment with the GI doctor the next week and she did his colonoscopy/endoscopy 3 days later. Results showed that he had 50+ polyps in his colon. She biopsied 10, all results showing they were adenomas and he was diagnosed with FAP. I agreed to have him scoped every 6 months, until she felt it was time to have his colon removed. Our goal was to get him through high school, which was only 5 years away. At the end of that appointment, his doctor gave me all the facts that she could pull out of her brain, out of books and from other doctors she had consulted with. She informed me that because Ashton had FAP, it meant that I had to have it, as it came from my side of the family and does not skip a generation. You have a 50/50 chance of passing it to your children. Ashton was not my only child. I have another son and daughter. Before I even began the next leg of my journey, I was already overwhelmed.
I went to see my GI and scheduled my scopes 4 weeks after Ashton was diagnosed. It was April of 2009 and I was 32 years old. I was prepared for the worst because I had already been told that I was nearing a scary age for cancerous changes in people with FAP. When I woke up from anesthesia in the holding area, I already knew. I knew by the look on the nurse’s faces and by the extra tender tone in their voices that something had been found. I had a colon blanketed with precancerous polyps, along with my esophagus and stomach. They clipped as many polyps as they could from my stomach and esophagus, but my colon needed to go. Up until this point, I had a healthy life. I didn’t feel ill, but I had a ticking time bomb inside my body. I knew I needed this life saving surgery, but I knew it was, also, going to change my quality of life. I scheduled my surgery for a week later. People tried to talk me out of it, tried to make me wait a little longer, but I knew without a shadow of doubt, that I had to be proactive. If not for me, for my children. I didn’t want to wait. I knew waiting and assuming and trying to predict how it all may go would make me crazy, so I jumped in with both feet. I did as much research as I could that week, ate all the foods I knew I would miss, had several glasses of wine, celebrated life with my kids and cried a whole lot behind closed doors. I knew I needed better perspective and my perspective was that I was doing this before Ashton had to and I was going to get through it resiliently so it helped him be brave when it was time for him to face it himself.
I had the first part of my surgery on April 7, 2009. I had 16 people in the waiting room supporting me and just making sure I knew that they were there. I don’t know if I would’ve been as at peace without my waiting room army. My surgery lasted 8 hours. They came to the waiting room during hour 5 to tell my husband that there were polyps in my rectum and they would need to remove it, as well. I knew it was a possibility and had told him beforehand, if it came to that, to agree to it. That seemed to be hard for my mom and dad, but they carried on with my wishes. I woke up the next day for the first time, ostomy bag in place, and ready to face my new circumstances.
The next 3 month were tough, but I learned to adjust to my new, but temporary, situation with the ostomy. Originally, I was very against doing the surgery in two parts because I did not want the ileostomy and I had to gain new perspective again. It was allowing things to heal and rest and it was temporary. I could do it, and I did. July 7, exactly 12 weeks after step 1, I had the second part of my surgery and they reversed the ileostomy and connected my j-pouch. I was in the hospital for 7 days and then home for 7 days before I passed out while I was left alone with my 5 year old daughter. I had become terrified to eat. It was the strangest fear I’ve ever had. I, somehow, convinced myself that eating more than a few bites was going to cause everything to get blocked. In the process I almost starved myself to death. They put me back in the hospital for 2 weeks while they taught me about nutrition, managed my pain and let me get some much needed rest. When I came home the second time, I was in a better place physically and mentally. 6 months after my surgery, I was doing ok. Still getting use to my frequent bathroom visits, but overall doing well. Then, before I knew it, it had been almost a year since my first surgery. I woke up one day, literally, and realized that I felt good. It wasn’t like what life was before my j-pouch, it was a new norm, but I felt good. The immediate threat seemed to be gone and now I could focus on preventative care for myself and surveillance for Ashton.
IT’S NOT JUST ABOUT YOUR BUTT
18 months after I had my first surgery, I started to notice a lump near my right hip. It was causing some pain, as well. My first thought was that it was a hernia. I ran a daycare out of my home and I was constantly picking babies and toddlers up. I went to the doctor a couple of times as it grew larger, but they seemed to think it was scar tissue. Several weeks later, another lump near my belly button started to show and became painful. At that point, I couldn’t ignore it anymore. I was taking pain meds around the clock for the pain. I, also, had a new pain in my lower back. The masses had become large enough that they showed through my clothes and multiple times people asked if I was pregnant. Walking into the surgeons office for my appointment, a bird decided my shirt needed a little extra something. I just remember thinking, “THIS CAN’T BE A GOOD SIGN!” Nothing like a bird deciding to dump on you while you’re walking into the colorectal surgeon’s office. I hadn’t seen my surgeon in a year and we were both happy that everything had gone so well. We were discussing that as he pulled up my shirt to look at the masses protruding from my stomach. I will never forget the next words that he said, “Shit, Jessica. I’m sorry.” Sorry? What? Why are you sorry? He said he was 99.9% sure it was Desmoid tumors, but he needed to get a CT to make sure. He sent me down immediately to have one. Later that night, he called me with results. His diagnosis was correct. I had 4 Desmoids in my abdomen. The biggest, most invasive one, was wrapped around a leg of my aorta going to my intestines and had completely blocked my right ureter. My kidney was 3 times as big as it should be and I was in immediate danger. He said I needed a stent placed in my ureter so that my kidney could drain, immediately. The next day I went into surgery. Into the second hour, they came out to tell my husband and mom that they couldn’t even get a stent the size of a hair in there. They needed to place a nephrostomy tube through the skin in my back, into my kidney, so it could drain into a bag to relieve my kidney. When I woke up with the nephrostomy bag, I was shocked and confused. I knew I was about to face another battle from this disease. The immediate threat was managed, but now I needed to go to M.D. Anderson hospital in Houston to see the Sarcoma specialist, since there were no specialties in fibromatosis near me.
After 3 days of testing at M.D. Anderson, I met with a team of doctors to discuss what I needed to do to save my life. I needed to start aggressive chemotherapy right away. They wanted to hit the Desmoids with three different drugs for 72 consecutive hours in the hospital, every 3 weeks, until we had enough shrinkage to save my kidney and other vital organs that would start being affected as the tumors grew. After asking every question I could think of, I finally reached my final question…what happens if chemo doesn’t work? They said I only had a 30% chance that it would work because of the size of the tumors, and if it didn’t, I had about 16 weeks left to live. I don’t think anyone forgets hearing those words being said about their life. Is this really happening? WHY? It isn’t fair. WHY? I can’t do this. This is too hard. I will never forget what my oncologist said to me next. She said, “Jessica, you only have time for one thing right now. You only have time to be a chemo patient. You don’t have time for anything else. If you put all of your fight into this battle, you will continue to get to be a mom, a wife, a friend and a daughter, but right now, you’re a chemo patient. You’re fighting for your life.” Then, images of my three kids flashed across my mind. I think my heart, for a moment, ached for them at the thought of me leaving them. It was then that the invisible gloves appeared.
CHEMOTHERAPY – DESMOIDS
Just like my oncologist said, all I had time for over the next 6 months was being a chemo patient. Over a period of 180 days, I spent 123 of them in the hospital. I was either in there for chemo, side effects of chemo, nephrostomy tube changes due to getting staph infections in my kidney’s or bladder, pneumonia, anemia, blood transfusions, GI infections that I caught in the hospital. It was a lot of only being a patient. Right at my 7 month mark of chemo, I was in the hospital with pneumonia. It was close to Thanksgiving and I was trying so hard to be well enough to go home to be with with my family for the holiday. I had some friends come in that I had not seen in a while. I was enjoying their company when I was suddenly in pain. They thought one of my Desmoids had liquified and ruptured. From what I’m told, because I don’t remember much after being in pain, my vitals dropped, I was severely vomiting and then passed out. The doctor saved my life that night. They took me into surgery so they could place a tube in my abdomen to drain the fluid. Thankfully, they took me into surgery. It wasn’t the tumor that had liquified, my kidney had ruptured. At that point I was in complete kidney failure and my doctors made the decision to remove my right kidney. My surgery went well and now I just had to make sure my left kidney kept doing the work for both of them. Chemo shrank all four Desmoids enough that my organs were safe and not being damaged and I made the decision to stop chemo. It was late in the year of 2011.
In October of 2012, I had been doing well for almost a year. I had started to really enjoy life again. It was time for my 6 month scopes and I was feeling hopeful that everything would be ok. Those were the only scopes that I didn’t feel fearful that they would come back with negative results. I like to think of it now as the calm before the storm. The polyps in my ampulla had started having significant cancerous changes. My GI told me I needed an ampullectomy to remove that part of my stomach. I had surgery the next week. During surgery they place a stent in your main pancreatic duct to allow fluid to continue to flow through it due to swelling from surgery. He told me most people wake up with pancreatitis, and if I did, they would hospitalize me after the procedure. Of course, I woke up with severe pancreatitis. I think aside from natural child birth, that’s the worst pain I’ve ever endured. For the next year I had chronic pancreatitis. It took over my life. I was in pain often, couldn’t eat well, or at all. I lost 30 pounds in a matter of 3 months. At 95 pounds, they threatened to put a feeding tube in place. I told them that day that eating would have to be another fight that I would have to win. I was NOT getting a feeding tube. No more tubes…no more bags. I had to make a conscious effort to eat over the next year. It felt like food and calories were all I thought about. Food didn’t taste good, look good, smell good and it hurt. At times when my pancreatitis was the worst, I couldn’t even stand water. It took a year and another surgery to clear up my pancreatic duct, before I gained 15 pounds. I still fight to maintain my, “no feeding tubish” figure of 110 pounds.
Things seemed to be moving along again and then it was time for Ashton’s annual scopes in June of 2013. The results weren’t so great that time. They think Ashton’s had polyps since he was 8 or 9 years old. At 13 he had 50+. These new set of scopes, at 17, showed a blanketed colon with changing polyps. He was already on the brink and I could’ve missed it by days by not pushing my insurance to let him have scopes twice a year. I can not emphasize enough how important close monitoring is in the life of FAPers. It’s a part time job for some and a full time job for others, but it has to be done. It was Ashton’s senior year in high school, and although we didn’t make it through graduation, I felt like a tiny victory had been won by us coming 6 months shy of it.
In July 2013, a week shy of his 18th birthday, Ashton had the first step of his two part colectomy. He was in surgery for 6 hours and the surgery went well. On day 3 of his recovery, he was incredibly nauseated and vomiting bile, as he didn’t have any food yet. The vomiting didn’t stop overnight and they become concerned that his intestines were kinked. They tried doing several contrast tests, but nothing was passing through. They finally took him into surgery on day 5 after the NG tube continued to drain bile from his stomach. Those were some of the hardest days of my life. He was barely holding on. He was so ill that he didn’t speak, he barely moved, but he cried. I’m so thankful that he doesn’t remember any of it. If anyone has ever met Ashton, they know he never stops talking. I don’t think I’ve ever appreciated hearing him talk as much as I did after those days in the hospital. The day they took him into exploratory surgery, it was an odd day and no one could get to the hospital to be with me. I was incredibly nervous. My gut told me that we were wasting time. After being in surgery for 2 hours, our surgeon came out to talk to me. Ashton and I have been his patients for 6 years. I have incredible trust in him and have built a relationship over time. The look on his face walking in to speak to me said a million things before he said a word. I just asked him, “What?” He said when he opened Ashton up, his intestines were blue. He said he yelled, “F@$*! I’m not walking out there and telling his mom that I lost her son. I’m not losing a 17 year old kid!” He said he prayed that when he untangled Ashton’s intestines, that they would turn pink. He untangled them and they did. By the time he got that far into telling me that Ashton was ok, I had already lost him 3 times in my mind and came close to falling on the floor when he finally said his intestines turned pink. Ashton would be ok. I’ve never hugged someone so hard. Four days after surgery, Ashton’s incision became infected with staph and they had to open it up so they could put a wound vac in. He was still pretty medicated at that time, but during the process of them trying to get it inserted, he passed out from the pain. I made them stop and told them to leave the room and give him a break. It was in that moment that I learned how important advocacy is for any patient, especially when advocating for your child. He spent 22 days in the hospital before we went home. At home, his stoma constantly had issues and it made keeping an ileostomy bag on very difficult. There were some days when we had to change it 5 or 6 times and his skin hated us! Finally, right before his takedown surgery, they were able to remove his wound vac and that gave him a little freedom. He weighed 90 pounds and looked like a 70 year old man. Those 3 months were awfully hard on him. Finally, in October of 2013, he had his ileostomy reversal and takedown. They didn’t let him try liquids until day 7. They wanted to make sure his intestines didn’t try to tangle themselves again. He was not happy. I don’t think anyone begged for or talked about food as much as he did for those 7 days. Bless his heart. He finally came home after 16 days in the hospital.
ASHTON’S EMERGENCY SURGERY – DESMOIDS
18 months later, in April of 2015, after several blockages, they admitted him from the ER with severe abdominal pain and vomiting. He was 20 and was living on his own. He hadn’t gone to the bathroom in 3 days and didn’t tell anyone he was having trouble. Again, after testing to see that nothing was passing through, they took him into surgery. They found that bands of overgrown fat, due to the overgrowth of cells from FAP, had wrapped themselves three times around his small bowel. 2 inches of his intestines had died and they resected that part and removed the bands, along with the lower portion of stomach muscle where the bands had been growing from. While they were operating, they found two Desmoids. Both in areas where I have them and one of them near his kidney and ureter. They are small and for the past 6 months have shown minimal growth. We will continue to monitor them frequently. That was a tough day for he and I. Lots of tears were shed between the two of us. He’s old enough to remember what I’ve been through and he’s old enough to understand the destruction Desmoids can cause.
SAGE AND EMMA
Even with regular surveillance and preventative care, this disease can attack from any and all angles. The fight is never over. FAP doesn’t feel compassion, it doesn’t discriminate. It seeks to destroy. I’ve learned to never be surprised and always keep my head and fists up. Our fight isn’t over. I still have a 15 year old son and 11 year old daughter that show signs of the disease, but have no polyps. I finally won the battle with insurance to cover genetic testing so that I don’t have to continue to put them through scopes. I get frightened by the slightest sign that they may have FAP, but if they do, we will continue on fighting as we always have.
THE COMPLICATIONS CONTINUE
It’s been 5 years since the nephrectomy of my right kidney. My left kidney has been slowly losing function over the years. Recently, learned that I was in late stages of kidney failure. They’ve told me that at some point I will need dialysis and a kidney transplant. Desmoids thrive on invasive surgeries. It’s a huge concern to put me through that, so my team of doctors, and myself, are working hard to combat continued deterioration of that kidney. I’ve now added a renal dietician, hematologist, pain management and nephrologist to my team of 5 specialist, including oncologist, gastroenterologist, urologist, gynecologist and colorectal surgeon. There’s many appointments, many waiting rooms, expenses and time put into keeping me going. Without this team I couldn’t be able to continue to care for my loved ones. I’m grateful daily that I have access to the medical care that I do. Not everyone with this disease is as fortunate as I am. Hard decisions are ahead but I will cross each road when I arrive at them.
This is our life. This is our norm. I don’t know that I’ll every accept our circumstances. I’m afraid the day that I accept it that I’ll be defeated. Instead, we keep doing a whole lot of living while we can, and when we can’t, the thought of the days ahead, when we will, keep us going.