Monster mutations

So there are two main genetic mutations that cause colon cancer in extremely young people like me, FAP and HNPCC. FAP basically turns your colon into thousands of polyps, and since I only had two polyps (one which had turned into my giant tumor, and one that was tiny), we are confident I do not have FAP. HNPCC, on the other hand, is typically diagnosed from a family history of colon cancers or other abdominal cancers. My closest relative with colon cancer is my dad's cousin, who was diagnosed at over 60 (not indicative of HNPCC), and the only other abdominal cancer in my family is my maternal grandfather's pancreatic cancer. Also, HNPCC-related colon cancers nearly always occur in the ascending or transverse colon, and mine is low in the descending colon. So despite my desire for genetic testing, my medical oncologist said she felt confident enough that I didn't have HNPCC that we didn't need to test for it before surgery.

Very often, if you have HNPCC, they will surgically remove the entire colon, because the risk of recurrent colon cancer is so high. The second most common cancer associated with HNPCC is endometrial cancer, so they often do hysterectomies as well. Sometimes they also take out the bladder and as much of the soft tissue as they can, to remove everything that you can possibly live without that might become cancerous.

But my oncologist had convinced me that I didn't have it and so we didn't need to test for it. The genetic tests take about 6 weeks to come back. I am nearly always in favor of more information, but she has proven herself to be so extremely competent (see the incident with the clot) that I just trusted her.

The surgeon who did my ovarian transposition (the surgery to move my ovaries up out of my pelvis so they didn't get fried in the radiation) said that if I were his wife or daughter, he would want me to have a hysterectomy at the same time as my major surgery. The rationale for this was that there's an increased risk that I could develop secondary uterine cancers as a result of the pelvic radiation, including a nasty form of incurable sarcoma, and my uterus is useless for childbearing, so why not just take it out and remove that risk. Additionally, the recovery time from my surgery would be no greater with a hysterectomy than without, and performing a hysterectomy later, after my pelvis is full of scar tissue from this surgery, would be much more difficult and time-consuming (though not impossible). The first time I heard this argument, it made a lot of sense to me. I'm pretty deeply risk-averse, and I think cancer has only made me more so, so why keep my uterus around if it's not going to serve its intended purpose and makes it more likely I'll have to go through this nightmare of cancer treatment again.

But then I went back to my medical oncologist and she said, essentially, surgeons like to cut out organs, of course he wants to remove your uterus. And she emphasized that everything he said is 100% true, but that it is highly unorthodox to perform a prophylactic hysterectomy on a woman of my age. She said her job was to cure me of my colorectal cancer, while leaving me as intact and normal as possible, and removing my uterus wasn't necessary and why not leave me as whole as we can. During radiation, I asked my radiation oncologist what he thought about a prophylactic hysterectomy, and his response was that he has treated thousands of patients for colorectal cancer, several of them young women, and he doesn't know of a single patient who had a hysterectomy without any evidence of disease or metastasis. So after that, I was fairly convinced that it was unnecessary. I'd also done some reading, and there is a small but real chance of fairly severe sexual dysfunction in young women following hysterectomy, which I'd like to avoid if at all possible.

But then on Wednesday, my surgeon announced that she feels strongly that I should have a hysterectomy. And she had pulled my original tumor biopsies from MGH (Mass General Hospital) to have her pathologist stain them for some specific markers (MSI) that are indicative of HNPCC. Prior to that revelation, we were confident I did not have HNPCC. But apparently at some point a test had been run that showed that my tumor was APC-negative, which dramatically increases the chances that I have HNPCC. So much so that my medical oncologist now agrees with my surgeon that even if the marker tests come back negative, it is still a good idea for me to have a hysterectomy.

And apparently even if the MSI-marker tests come back negative there is still a very small chance I could have HNPCC. If they come back positive, it is highly likely that I have HNPCC.

So my medical oncologist set me up with a genetic counseling appointment this afternoon (in an hour, actually) and put a rush on the HNPCC test. So we might have that back in a week or two, rather than having to wait the normal 6 weeks. I suck at waiting.

So it's back to the hospital for me! Whee!! And hopefully we'll hear about the MSI-marker results soon. I don't want to have to continue to deal with this uncertainty through the weekend. But at this point, I guess it almost doesn't matter. Because the MSI-marker tests aren't conclusive in either direction. And both my surgeon and my medical oncologist agree that I should have the hysterectomy regardless of what the tests say. I still just suck at waiting.

And I thought the scan results were the only thing I had to be worried about. HA! Cancer: full of freaking scary unknown unknowns.

Rollercoasters and roosters

So I feel a little like my life is a rollercoaster these days. When I'm feeling good, I feel very nearly healthy and thus great, but then inevitably the fatigue/nausea/anxiety catches up to me and I crater. It's so nice to have the good moments, though, that I'll take any/all of the craters just to be able to feel normal for a little bit.

Tomorrow I go in for a full day of appointments. I have a scan at 6:30am, then I meet with the surgeon to get the results of that scan at 9, followed by my pre-op anesthesia consult, and meeting with the ostomy team. I am NOT looking forward to it, mostly because everything that reminds me of surgery these days induces rather extreme anxiety. And while the prospect of getting good prognostic news from the scan is great, the fear of getting bad news totally outweighs the good. I have spent nearly all my life in search of data and information, and here for one of the first times I can remember, I think I'd really rather not know.

The main purpose of the scan is to give the surgeon a better sense of the odds that I will need a temporary colostomy, so that she can plan accordingly. Even if the scan looks great, it's still not going to be 100% that I won't need one, as there are apparently some issues that could pop up once she gets in there that would require a colostomy. But she said that if she doesn't have to do one, she won't, which is great news. But because there's still a chance I'll need one, I still have to meet with the ostomy team to go over where they're going to put it and caring for it and everything else. And, in the process of looking at the tumor to determine if I need a colostomy, we will also get my cancer restaged. And from everything I've read, stage at surgery is the single greatest predictive factor of survival. So if it's not good news it will be very hard.

The hospital also requires that I fill out advance directives/living wills/will wills before surgery, to make things easier on them in the event that I become incapacitated or die. While my surgery is long and complex (removing a large chunk of one's colon and rectum while allowing it to still function is not easy), it was not considered a risky surgery until I had my clot. Now, because of the clot, it is much higher risk, which makes me less than happy. But my surgeon hasn't ever lost a young patient on the table, so with any luck I won't be the first.

It's all just nerve-wracking. And to make things even more stressful, my husband is on the job market and has his first job talk on Monday! We fly out on Sunday morning (because with impending surgery/incapacitation, we'd rather be together than not, even for things like job talks), so he's terribly stressed about that aspect of his life, and about losing all day Wednesday to ferrying me around the hospital. As he likes to say, in any kind of normal world, going on the job market the first time should be the most stressful event in any young academic's life. Unfortunately, for us it's a distant second to my cancer treatment.

And the brakes on my husband's car went out on Friday in the midst of the big snow/ice storm, so we're still awaiting word from the mechanic on what the damage will be. Remarkably, they went out as he was stopped at a light, so he's fine, because with the roads covered in ice that could have been VERY bad. But it's an 11 yr old car, so if it's going to be a lot to fix, we might be in the market for a new car. Because that's not stressful either. So we're living in a state of basically constant stress and anxiety, which is less than fun.

But one week from today the first job talk and interviews will be done and we'll be on our way back to Boston, and two weeks from today, I'll be under anesthesia having my guts sliced and diced. So hopefully once we're through those two hurdles things will be less stressful. Though, to be fair, we were saying that as we were working 100+ hrs/week trying to work full time and finish the house in Michigan - once we sell the house and move, things will be less stressful. Oh how wrong we were!

Recap of the last few weeks

On Friday, I finished my six weeks of daily radiation with continuous chemo infusion, so I am very pleased to be able to check that particular hurdle off my list. While at its worst, the radiation was not as bad as the worst of the full chemo, even when I was at my most miserable during chemo, I knew I was going to feel human again in 3-4 days. With radiation, I started feeling pretty terrible in early week 4, and knew it was just going to continue to get worse for the next three weeks, which was very hard. But at least I can finally say that it should be nothing but improvement for the roughly six weeks until surgery.

In early week 3, I started having searing pain in my right shoulder, which was not an anticipated side effect of my treatment. Initially, my doctors just treated it with more and more oxycodone, until I was taking 40-50mg/day and it still wasn't really eliminating the pain. In addition, the extra nausea from the narcotics, on top of the nausea from the radiation and the nausea from the continuous chemo, made it impossible for me to keep anything down, and the mere thought of food was enough to make me gag. I had a hard time walking past the cafeteria in the hospital because of the food smells, and often had to leave the room when other people were eating so I wouldn't get sick. Thankfully, by the middle of week 4 my oncologist realized this wasn't working and brought me in for IV anti-nausea drugs, changed my painkillers to 60mg of slow-release morphine twice a day (which works MUCH better), and ordered a chest CT to see if I had a clot in my chest, which would be the most dangerous explanation for the shoulder pain. The CT showed minor fluid in my lungs, not enough to treat or explain my high level of pain, but no clot. So we kept on with the morphine, the nausea subsided somewhat after getting off the oxycodone, and we continued with the daily radiation treatments.

But my oncologist, bless her, was not satisfied with being unable to explain my terrible shoulder pain; she said it was keeping HER up at night because she didn't know what was causing it. So she took my CT to a second radiologist, who agreed there was no clot, and then to a third, who also initially said there was no clot. But with the third radiologist, my oncologist compared this recent CT to my initial diagnostic CT from May, frame by frame, and found something that might be a clot in my superior vena cava. She called at around 7 on Friday evening of week 5 to tell me that I might have a clot, and oh, by the way, this could kill me, so if I experienced any shortness of breath or new chest pain to head _immediately_ to the Brigham and Women's ER and stay there, being closely monitored, until they could perform the conclusive test, a venous MRI.

The venous MRI was scheduled for this past Tuesday, and sure enough, it showed a clot in my superior vena cava, just as my oncologist had thought. Apparently, it is a very difficult place to detect clots, because the standard methodologies don't capture that particular vein very well; we are lucky that Brigham and Women's has one of the best vascular teams in the country. But if there was any doubt that I was in the hands of extraordinary doctors, it has been completely erased. I am still a little amazed that my oncologist took the time and effort to go to three different radiologists, and was concerned enough about my pain to go to all of that trouble, despite flying off to conferences all over the world all the time. I am so very lucky to have such amazing doctors!

So they pulled the chemo pump on Wednesday, since the port-a-cath that I use for my chemo ends in my superior vena cava (which is almost assuredly why I got a clot there), so it wasn't safe to put anything in there any longer, for fear of dislodging the clot. I was scheduled for emergency surgery on Wednesday to remove my port from my left shoulder, but the hematologist refused to clear me for surgery, again concerned that mucking around in there could send the clot sailing directly into my heart or lungs. So instead, I started twice-a-day (very unpleasant) injections of blood thinners to break up the clot, and continued with my last week of daily radiation.

We're still continuing with the blood thinners, and I've been rescheduled for port-removal surgery on Tuesday, because the hospital is closed on Monday for Columbus Day. But once I recover from that, it really should be smooth sailing for the next several weeks. And I can tell already that I'm improving. The radiation fatigue was so intense that I had difficulty staying awake for more than about 4 hours at a time. Basically, any time I wasn't eating, in the bathroom, or at the hospital, I was asleep. But yesterday I was awake for 8 straight hours, which hasn't happened for several weeks! And the nausea is slowly subsiding, though still very present. Unfortunately, the recovery from radiation is very slow; it can take 4-6 months or longer for the fatigue to really fade. But I am so grateful for every improvement. Just knowing that it's not going to continue to get worse is a huge morale booster.

So we're plugging along. I'm very much looking forward to my month+ of no medical procedures! I know my major abdominal surgery will be awful. I'll be in the hospital for 1-2 weeks, and not walking independently for another 3-4 weeks. And I'll have to learn to deal with the colostomy and everything that goes along with that. But at least we'll have a brief respite of health before dealing with all of that.

A typical visit

Since many people have asked, I thought I'd give you a sense of what it's like when I get treated.

A bit of background: my port is a dual lumen port-a-cath in my left shoulder, just below my collar bone. I couldn't find a picture of the dual lumen port, but here's a single lumen:

The clear circle is the lumen - it's a sort of self-healing plastic, so that they can punch needles into it, but when they pull them out it never leaks. So instead of having one circle and looking like a spade, mine has two and looks like a figure 8. The tail is a catheter that threads through my subclavian vein into my superior vena cava (the giant vein that goes into your heart). The port makes it easier to draw blood, since I have terrible veins, but more importantly makes it so that the chemo gets diluted quickly once it's in. Apparently the type of chemo I'm getting can eat through veins if it's put into smaller veins, say in your arm. So I'm a big fan of the port!

Regular chemo:

I usually show up very early in the morning and head straight to lab services, where they access my port (i.e. stick a giant needle into my chest), dress the port with bandages since I have to leave it in for several days, and draw blood. Sometimes my port doesn't have a blood return (this is actually more common than not), so then they have to inject TPA (a kind of enzyme drain-o) to clear out the clog in the catheter. The TPA takes about an hour to dissolve the clog, so we just hang out and wait. There is an extraordinary amount of waiting involved in cancer treatment. Way more than I was expecting. So once the TPA is clear, then they can draw blood, which has to get run before I see my oncologist. The chemo I get (and I believe most chemo cocktails) destroys my bone marrow along with the cancer cells (also the cells in my mouth and gut, though not my hair cells!), which leads to low white blood cells, so they have to make sure my white (and red) counts are high enough before they give me my chemo. It takes about 20-30 min to run my blood, so then there's more waiting. They always check my vitals (weight, bp, temp, and blood oxygen levels), so if we're lucky they can do that while we wait for my blood. Eventually, we get in to see my oncologist and we go over my side effects from the last round and tweak my meds if needs be. And then I check in and wait for my infusion.

Once my oncologist writes the prescription for my chemo, it takes the pharmacy 1-2 hours to prepare it. But at least they have amazingly comfy (heated!) chairs for the infusion patients; the guest chairs are less spectacular, unfortunately. There are also volunteers that bring blankets, food, beverages, books, and all sorts of things past while you're waiting, which is very nice. I also get my pre-meds at this point, at least the ones that are taken orally - a ton of anti-nausea meds plus steroids. And they typically hook you up to saline pretty much as soon as you sit down (which then always makes me need to pee halfway through the infusion). Once the chemo gets there, I get to start my infusion. I'm on FOLFOX 6, which is a combination of Oxaliplatin, Leucovorin, and 5-FU. I get the oxali and the leucovorin via a 2.5 hr infusion, so they just drip slowly into my port while I sit there. Once that's done, then I get an injection of 5-FU, followed by the continuous infusion pump of 5-FU that I carry around with me for the next 48 hrs.

The pump is about VHS-tape-sized, and I wear it in a fanny pack across my stomach. Actually, it's a runner's hydration belt, but it looks like a fanny pack. I can run the tubing under my shirt, so it's less obtrusive, though the bulge from the needle into my port is clearly visible (as is the obviously medical device in my fanny pack). I typically feel reasonable the day of the infusion, worse the day after, and terrible the third day, but improving on days four and five. The anti-nausea meds make a huge difference, though by the end the steroids were starting to drive me a little crazy. Aside from the nausea, diarrhea and fatigue, the other main side effect is neuropathy from the oxali. Apparently oxali eats the myelin sheaths off your nerves, and it manifests initially as extreme cold sensitivity in your mouth, hands and feet. Basically, cold feels like electric shock. I can't eat or drink anything cold, so all my drinks have to be kept out of the refrigerator, and ice cream is strictly verboten. I wear gloves if I have to get anything out of the fridge or freezer, because it hurts to touch things that are cold. Slippers are an absolute necessity, as even regular room-temp tile is too cold for my toes. And washing my hands takes forever, because I have to wait for the water to warm up enough that it doesn't hurt.

The effects of oxali are cumulative, though they do typically recede with time. I'm now about 6 weeks out from my last chemo treatment and I can eat ice cream again, though my hands and feet still smart when they encounter things that are particularly cold. Because my treatment is split into 4 rounds of chemo, then radiation and surgery, and then another 8 rounds, instead of the typical 12 rounds all at once, I'm hopeful the neuropathy won't be as bad. Many people have to have their chemo doses reduced near the end of treatment because the neuropathy gets so bad, but the long-term disease-free rates are worse if you don't complete the full treatment. Eventually, the cold sensitivity morphs into constant tingling, followed by numbness. I haven't had to deal with too much of that yet, so I'm hoping it stays that way.

I am basically a sick mess Monday-Thursday of chemo weeks, but Greg has been good about getting me to take short walks every day to get me outside. And by Friday, I usually feel mostly human again. Every time it felt so good to feel not sick again - it feels similar to when you're getting over the flu and can finally enjoy being out of bed. It does definitely make me treasure the time I feel reasonable much more than I ever would have otherwise.

Baby pictures

I've mostly come to terms with the fact that I will never be pregnant. The gynecological oncology surgeon who moved my ovaries up and out of my pelvis so they wouldn't fry in the radiation, said that if I were his wife, he'd recommend doing a total hysterectomy when they go in to take out the tumor. The rationale for that is that I'll never carry a child, so why bother keeping my uterus around, given that there is a chance (albeit a small chance) that I will eventually develop uterine or endometrial cancer. My medical oncologist is against it, since the risk of secondary cancer is so small. Regardless, I've got a few months before surgery to make up my mind on that piece, and the point is that I'm used to thinking of and talking about my uterus as something expendable and useless.

But oh lordy do my friends' baby pictures on Facebook make me want to cry. I find myself in tears thinking about writing a short congratulatory note to friends with new babies, being jealous of their fertility and feeling robbed of my own. Greg and I had just started planning to start a family when I got my diagnosis, which somehow makes my fertility (or lack thereof) feel more like something that was violently snatched from me.

We completed an IVF cycle before I started my first round of chemo and have eight frozen embryos, and my amazing sister-in-law has already volunteered to be a surrogate for us if it is medically possible, so odds are good that we'll still be able to have biological children. And that at least provides some intellectual comfort, but does very little to blunt the emotional impact of looking at pictures of my friends and their partners glowing over their offspring. Even more than walking around with the needle in my chest and my pump in a fanny pack, tubing peeking out here and there, knowing that babies will be a long and fraught journey for us in the best case scenario makes me feel the incalculable losses that cancer inflicts.

I often barely have enough energy to get up to let the dogs out, so I'm very glad that we don't have young children as I go through treatment. But I do sincerely hope there are babies in our near future, one way or another.

No super powers

I'm now on my second week of radiation, out of a total of six weeks. It hasn't been too bad so far. The worst part is probably having to wear the pump all day, every day for six weeks. The pump is a VHS-tape-sized contraption that I wear in a fanny pack in front of me, and continuously pumps chemo (5-FU, to be precise) into the port-a-cath in my chest through a needle. The housing of the needle sticks up about an inch off my chest, and occasionally gets caught on things like seatbelts, but most of all makes it difficult to sleep. Also, the front of my left shoulder gets sore whenever my port is "accessed" (i.e. has a needle in it), which makes things like walking the dogs hard.

I was initially deeply apprehensive about having the pump 24/7 for six weeks, because it's the same pump and the same drug that I went home with after my regular chemo treatments, and I associated it so strongly with feeling absolutely miserable. Luckily, it turns out that the drug that really made me miserable is a different part of the cocktail, and 5-FU by itself isn't so bad. The main side effect is diarrhea, but, at least for now, immodium is sufficient to handle it.

The radiation treatment itself is entirely painless. I show up, change into hospital gowns, and eventually get called into the room where they scan my ID card to make sure they're giving the right radiation to the right patient. The first step is climbing up onto the table, which has a special cutout for my belly, so that my small intestines will fall down into the cutout and be further from the radiation field (apparently ovaries and small intestines are particularly vulnerable to radiation). Once I'm on the table, they turn on the laser cross-hairs and line my blue dot tattoos up with the laser marks, primarily by tugging on a sheet underneath me to move me where they need me to go. Then the techs head out, an alarm buzzer sounds, and I get a few seconds of radiation. I'm getting radiation in four fields, one from on top of me, one below me, and one on each side. Each field has a different lead wedge that needs to be inserted into the machine to balance the radiation dose, so the techs come in and out as the four different fields are radiated. Every Monday, they take a set of x-rays to make sure I'm still in the correct position. On non-x-ray days, I'm in the room for a total of around 8 minutes; x-ray days are slightly longer, but are still right around 10 minutes. By far the most time-consuming aspect is getting to and from the hospital in Boston traffic.

Tomorrow morning, very early, Greg will detach me from my pump so that I can really shower! You can't get the dressing over the port/needle wet, which makes showering difficult. We bought a hand-held shower head to replace our existing one, and tried the saran-wrap-and-tape approach that had been recommended by my nurse with reasonable success. I have to hang the pump (in a bag) on a hook outside the shower, and the tubing isn't all that long, so range of movement became a real issue. But overall it worked better than expected.

I get to take off the pump tomorrow morning because I have my weekly medical oncology appointment at 8 and my infusion appointment to get my new 7 day pump at 9. So it's okay if I'm off the pump for the few hours it takes me to shower, get to the hospital, meet with doctors and then get hooked up again.

October 7th (hopefully my last day of radiation treatment) cannot come soon enough!!

Treatment plan

The treatment plan proposed by Dana Farber was 4 rounds of every-other-week chemo, followed by 6 weeks of daily radiation with continuous 5-FU infusion, then major abdominal surgery to remove a large chunk of my colon and rectum along with the surrounding lymph tissue, then 8-12 more rounds of chemo, for a total of 10-12 months of treatment. And the radiation will destroy my uterus, so I will never be able to get pregnant at the end of all of this.

The timing of our visits to the infertility specialists worked out perfectly, almost ridiculously so. I had gone off my hormonal birth control as soon as we'd heard that I was going to be infertile, because I knew I wanted to try to squeeze in an IVF cycle if at all possible. After the big pow-wow with all the doctors on Wednesday, we had an appointment with the MGH infertility folks that Friday and they were able to a baseline ultrasound and apply for coverage with our insurance. It turns out that we have the only insurance in Massachusetts that covers IVF before cancer treatment (since I'm not technically infertile at the time of treatment), so that was a bit of luck, since the drugs alone are over $7,000.

We went away for the long weekend to a B&B in New Hampshire, and I got my period on Sunday, much to my surprise! We had an appointment with the Dana Farber infertility specialist the following Tuesday (since Monday was a holiday), and because everything was already in place, she was able to start me on hormones that very day. I even got my first injection in her office. Had my period not started exactly when it did, or our appointment been a day later we couldn't have used that cycle, and my oncologist wasn't willing to wait another month before starting chemo. So we were extraordinarily lucky on that one!! For the next 14 days, my wonderful husband gave me an injection every morning, and for the last 8 or 10 days I got another one in the evening. Somewhere in there I had surgery to install my port-a-cath in my shoulder, but it was entirely minor day surgery. Just made my left arm sore for several days.

Starting on day 8, I had to go in to the hospital every morning for blood work and an ultrasound so they could monitor how my eggs were developing. They took patients from 7:30-8:30, but you really needed to be there by 6:30 if you wanted to make it home before rush hour. So those were some really early mornings. Greg went with me to the first one, but it was so early and so mundane that he skipped the majority of the rest. He still had to get up at the crack of dawn, though, to give me my morning injection (since he typically left for work before I would get back from the hospital).

And then, less than two weeks after we started the hormones, they scheduled my egg retrieval for Sunday, the day before my first round of chemo. My retrieval was scheduled for Sunday at noon, so Friday at exactly midnight, Greg had to give me an IM hCG injection into my butt, which was less than fun. At least all my other injections were sub-q (nice 1/2" needs), rather than the giant 4" IM needles. Apparently the eggs are released exactly 36 hours after you get the hCG, so the timing is really important. And then, off to pre-op on Sunday morning with Greg. They put me under, so I don't really remember much after arriving and changing into a gown. But they got 14 eggs, which turned into 8 fertilized embryos, who are frozen and waiting for us once all of this is over!