Our Sarcoma Story

For some time in 2006, into 2007, I had been after my husband, Adam, to have a lump on his lower back looked at. One thing after another kept happening, though, and the trip to the doctor just never really happened. Several months earlier, Adam had had his gallbladder removed due to “elevated liver levels.” Several months later, his levels were still very high, and he was always exhausted.

He did finally see his doctor, who noted that he thought it was likely a calcified broken rib from when my husband used to play college basketball, and that there was nothing to worry about. This did not sit well with me. It sat even worse when the “broken rib” lump started growing, aching, and turning hot. Adam went back to his doctor who suggested that this was probably a fatty tumor, again nothing, and that he would refer him to a surgeon for easy removal. We saw the surgeon, who suggested that it likely was a fatty tumor, but that for safety’s sake, before operating, he would like to have a CT scan done. I think since my husband was 31 at the time, healthy, a very tall former basketball player, that no one believed anything major could possibly be wrong with him.

Adam had the CT scan done, within two days we received a frantic call from the surgeon’s personal nurse. We needed to come in the next day for a biopsy, this was not a fatty tumor. The next day Adam was at the surgeon’s, we learned that whatever this thing was, that it had tentacles, and was pretty gnarly-looking. Within a day or two, we got a pathology report, saying this was benign. We relaxed, somewhat, despite no one seeming to understand what this thing inside of him was. Within two weeks Adam was being preped for surgery. At the time, we had four small children. The 7, 6, and 3 year-olds went to my mom’s, our 6 month-old rode along with me in the sling. I paced nervously at the hospital, baby on chest, as he was wheeled in to surgery. They told me to expect it to take 45 minutes.

Three hours passed, and we still paced. Suddenly a nurse burst through the waiting room door, asking me to okay emergency pain medication and a spinal block for my husband, who apparently was unable to consent. A few minutes later the surgeon came in, showed me a horrific Polaroid of my husband’s side, told me they had had to remove 2 ribs, a portion of his lung, that his lung had collapsed, that he had two active drain lines in his body now, that they had removed a tentacled tumor the size of a children’s football from my husband, that they had to repair part of his side with mesh, and that he would be shocked if this wasn’t it. “It,” I said, “cancer?” He said yes. I could tell that he was as shaken as I was by what had just happened. We would have to wait for pathology to see if there were clear margains (there weren’t), and Adam was headed to ICU.

My husband had entered the hospital, expecting to leave that evening, now he was being transferred to ICU, with intense breathing issues, and a huge portion of his torso missing. My head was spinning. The next week brought many struggles. We faced each of them with children running in and out of the hospital room, and a baby always present there in the sling. I had people ask, “how will you get through this? How can you all do this with a young family?” Our answer has always been the same, “how can we not, our other option is to give up fighting, and Adam dies, and that was never an option.”

The pathology showed a massive, invasive Desmoid-type tumor that is so rare that doctors can’t really even agree on what to classify it as. Some call it a fibromytosis and others a fibrosarcoma. Whatever it was, it was surely a cancer in my husband’s body. He went on to have another surgery a month later in January, to try and clear the margins, and then we waited.

We learned that only twenty-five people in the US each year would be diagnosed with Desmoid tumors, that they are particularly aggressive, and that they leave many people amputees. We found, and fired, a couple of oncologists who didn’t seem to get it. I got sick of doctors acting put out by having to deal with “rare cancers.” If one more person told us that if Adam had had something like breast or colon cancer, they would know exactly what to do, I would have screamed.

We finally found a wonderful Sarcoma Specialist in Dr. Howard Rosenthal in Kansas City (where we lived at the time). He not only knew about Desmoid tumors, but he had treated them. He pulled no punches and he sugar-coated nothing. He had read the research, and knew what other places were doing with Desmoid tumor patients. He laid out all the different treatment options, and as a team we decided what to do.

I realized then that one of the reasons I had disliked those prior oncologists is because they seemed wholly unconcerned by how drastically this affected our life. As if maybe the statement, “it may not even come back again,” was any comfort to us just because it was unlikely that Adam should have gotten this in the first place. Regardless, my husband had a massive twelve inch scar, and many missing body parts that did very much concern us for the future.

We decided to wait and see what happens, as we learned if it did come back, it was most likely to do so in the first two years. In June, we had a clear CT scan, we rejoiced. Maybe we could beat these odds, and make it past that two year mark. We were scheduled to return for another CT scan in October. By August, Adam was once again exhausted and I swore I felt a lump again. He promised we would call Dr. Rosenthal. We got in the next week.

Dr. Rosenthal felt what I felt, and sent us downstairs for another CT scan. And there it was, yet another tumor, a regrowth the size of a grape. Within two weeks Adam was back in surgery. They opened up his scar and by the time they got it out, the tumor was the size of a baseball. They were growing faster, literally sucking the life from him.

Once again, our team met, Dr. Rosenthal suggested radiation therapy. This was a one shot deal, though, we learned. Adam was 32. Once we killed this tissue, we would never have another chance at radiation again. We had to make a tough decision, did we burn our bridges now, roll the dice, hope this would fix it, and that we wouldn’t need treatment again, or did we try waiting again? If they came back post-radiation, Adam would be looking at a year-long stint of very aggressive chemo. The options were bad either way.

We rolled the dice, and by the end of October, Adam was receiving daily radiation therapy. He got blue tattoos to place the machine in the right spot and they irradiated a huge portion of his back every day. He got severely nauseous and tired, but worked through the radiation, as he is our family’s only income. I cannot describe for you the strength my husband possesses, I watched him endure burns, puking, exhaustion, and then get up the next day and not only do it again, but go to work and snuggle with the kids at night.

He did seven weeks of radiation, every day of the week except Saturday and Sunday. We made it together. He now has a large portion of his torso that is rough, leathery, extremely tender to the touch, and burned permanently brown, but so far, our luck has held. He finished radiation a year and a half ago. We go to visit Dr. Rosenthal, the sarcoma specialist, every six months, driving back to Kansas City to make sure it happens. We will visit him for the rest of Adam’s life, as even though the recurrence chance decreases after two years, it never goes away completely. I always laugh and say that we will probably personally put Dr. Rosenthal’s kids through college, but we’re very happy to do so, because he gives it to us straight.

As we approach the two year mark in December, I think back a lot to that time of cancer. Some of our kids remember it, others don’t recall it at all. Some days I would love cancer to be nothing more than a faint memory to them, and other days, I feel like it is so important that they know how close we came to losing daddy, and how important that fight was. We talk about the cancer freely, and there is no missing Adam’s massive scar and radiation burns, but as the memory of that horrible year and a half of fighting cancer fades into the past somewhat, it is almost surreal. I can‘t believe we did that with four small kids, that it all happened so fast, so furious. A few months after Adam finished radiation, we took the kids to Disney World to celebrate, and give them a break from the craziness. Nothing would make me happier than if we can take them every few years until we’re ninety. I plan to have Adam along for every single trip.

Even so, he is still so excited about the election. I wish he hadn’t had to go to work today feeling so yucky.

He IS really tired.  Last night he fell asleep at 9 pm.  Totally unlike him.  I have pictures of the fun battle scars where he’s marked for the radiation.  I will post them soon.  I’m too excited about the election right now for much of anything else.

She called me. I’m not sure why, except that I do handle most of the day-to-day stuff regarding the cancer. And she goes, that disk that you dropped off this morning with his original CT scan results, well, that makes things more complicated. And that’s okay, because it’s good for us to get a full picture of the area and the tumors, but wow. She goes, plus, Adam is a big guy. Well, she says, I guess you probably already know that, don’t you. And his scar is very large (12 inches), and it wraps around from his front to his back. And the area takes in bone, tissue, and organs. It is safe to say, she says, that this is one of the most difficult mappings that I have ever done. But we will get it. That said, I think we should take a little extra time, so that we make sure we get it right. We are going to take an extra week and push back the start of radiation likely until the end of next week or until the next week. This is a very difficult mapping. His is a very unique case. Also, I would suggest that if you need to ask anyone questions about the radiation, that you ask me personally, as I’m not sure most of the other folks who you’ll be working with are going to really have a handle on it.

I knew already that she would be out of the office for the next 2 weeks, as she is having knee surgery, but she said that she will continue to work on the mapping and that they will likely start radiation before she gets back, but that she will only miss about a week.

Sigh, could there be some fucking thing about this that doesn’t make us the one fucking crazy apple in the bunch, please? Could we get a big ole helping of totally normal and mainstream all up in here right about now?

1) Medical oncologist thinks that chemo is not the cure-all for these types of tumors that some think it is, and instead suggested that we do another drug therapy (which I need to go get filled at Target). I like this guy and he sounds like he knows what he’s doing, so we’re going to play along for now.

2) He also wants Adam to have a colonoscopy to rule out colon cancer and/or some genetic abnormalities that may accompany colon pollops (which we don’t know if he has, or not), as there is a correlation between colon cancer and these types of tumors. If he has the pollops, then we do genetic testing to see if there’s a genetic component. If so, there are currently no drugs to treat that genetic component of the cancer, but there are many drugs in the works that might 5-7 years down the line. That’s a lot of ifs, though, obviously. Hopefully we won’t get to the end of that line. The colonoscopy will happen post-radiation.

3) The radiology oncologist was awesome. Adam is scheduled to start radiation on October 27th. It will likely be a 5-6 week stint, everyday. They did a CAT scan today so that they can map the radiation over the next few days. He got his rad radiation tats so they know where to put the electrodes, et al. each day. We got a fun cancer bag of stuff.

4) I have to run around on Monday and get a few more pieces of medical imaging for the radiologist so that she can get the best mapping possible.

He was super nice, though. He left it up to us. He answered all our questions. The good part is that radiation on a whole in Desmoid tumors reduces the chance of recurrence by 2/3 (of course we will be the 1/3, I’m sure, but still…), it tends to delay the return of tumors in other cases, and it is generally considered the next course of action when surgery is not working to prevent the tumors.

The bad part is that this is it, we can only do radiation once. Then the tissue is destroyed, it will make it more difficult to both monitor and do surgery, because of where the tumors are (near the major organs) the radiation may do some damage to the organs, and it increases the likelihood (though only slightly) of contracting some other type of cancer down the line. It is much more successful than chemo at preventing recurrence, though it comes with more permanent and long-term negatives. It is, though, thought to be the best option for preventing recurrence. So, yeah, there we go.

I have to call a different hospital, though, to make an appointment for the radiaion treatments, as Dr. Coster’s office is way far away from where Adam works, and since he will have to go everyday for 5 weeks, it is just impractical to go there everyday. So I have to call the other hospital in a bit and get us an appointment there for them to map the area for radiation.

We should start relatively soon, he said, before the Desmoid cells begin regrowing, as it becomes less effective then. Adam will likely be fatigued and may have “intenstinal distress.” I can’t think of anymore information. So, you know what I know right now.

We are still going to see Dr. Myron, the medical oncologist, next week, to see what he has to say about all of this.