My Ongoing Battle with Pseudomyxoma Peritonei (PMP)

Introduction

So, I haven’t told a lot of people about this. Truly, it has taken me a year to learn what I know now, and my journey isn’t finished yet.

I am writing this now to tell my story, in hopes that it will help others in similar circumstances to me – either medically or professionally – should something like this happen to them.

Bear in mind that these are simply my own personal experiences. If you’re going through something like this, your experiences WILL be different. Trust your doctors and your nurses and take this all with a grain of salt.

I live in Oshawa, Ontario, Canada. I am 40, a parent of 3 wonderful girls (ages 12, 14 and 18), happily married almost 17 years, and an entrepreneur. Needless to say, until all this began I already thought my life was challenging.

The Start

In January 2008, an emergency appendectomy alerted my family and I that I had (as I later learned) Pseudomyxoma Peritonei or PMP. PMP is a rare cancer (literally one in a million) that for me started as a tumour in my appendix (an adenocarcinoma), which grew and ruptured it, spilling mucous and mucous producing cells into my abdomen. I had no idea that week in the hospital that I was beginning a journey that would last at least the next year and a half.

The surgeon at Oshawa General Hospital who did the appendectomy was unfamiliar with the condition, and his uncertainty alarmed us a little. He did however find and refer us to a specialist (Hepatobiliary, Pancreatic and Gastrointestinal Surgical Oncology) at Sunnybrook in Toronto. The specialist was wonderful to talk to – a doctor who not only knew of my condition, but was tracking and treating other patients with similar circumstances. This was a great relief after talking to the doctor in Oshawa who really didn’t know what to tell us.

The specialist advised us that we needed to act within a couple years, but that we should take 6 months or so and determine more precisely what was going on in my abdomen. He advised us in April that there were a couple possible courses of action (wait and see vs. aggressive surgery), and then in September that he felt that I was a good candidate for the “Sugarbaker Procedure” (an aggressive and complex surgical procedure, with several stages, and many possible outcomes). The basis of this was that I was young, in excellent health otherwise, and I wanted to aggressively pursue the cancer so that there was as little chance of recurrence as possible.

I mentioned I am an entrepreneur. In January 2002 I started Three Wise Men – a software development company based in Oshawa. While I’d grown the company fairly steadily over 6 years, our staff remained fairly small. The news in April 2008 that I might have to take 3-6 months away from the company for surgery and recovery seemed almost impossible.

The Preparation

Needless to say, my 2008 was spent not only continuing to grow my business, but figuring out how it would run without me. The year saw some real ups and downs in my mood. I even considered shutting the business down at several points, thinking that it was an impossible task. In the end, some renewed drive led me to rediscover why I started the business in the first place. I couldn’t let it fail. I owe this renewal to some key friends and mentors I’d come to know well since I started the company – all entrepreneurs should remember that they don’t need to be an island, and either starting or joining a good business “mastermind” group is an incredible help dealing with business challenges, large and small.

My ultimate solution was focusing on systemizing and documenting my business. I joined a formal business coaching program geared towards owner-managed businesses which helped organize my thoughts, and I leapt ahead writing down and organizing everything about the business I could think of. I hired someone to help with sales and business development, and my most trusted co-worker (I can’t bear to call him an an employee, he’s an integral part of the business, a natural if somewhat reluctant leader and is entrepreneurial himself) rose to officially manage all production operations. I also offered official part-time positions to two of my key contractors who I’d also rather be more involved in the company.

In October, the specialist at Sunnybrook declared that he would be unable to do the procedure at his hospital, but had arranged several of his patients already to have the procedure done at the Victoria General / QEII Hospital in Halifax. We agreed, and scheduled an appointment in Halifax with the surgeon there to discuss the procedure. Turns out the procedure is only currently available in Canada from Halifax and Calgary.

We didn’t know how long it was going to be between the consult and the surgery, so we travelled to Halifax at the end of November on a 1-way ticket with no idea when we’d be home.

I have to give special thanks here to Francis, the wonderful woman who put us up while we stayed in Halifax. She is the cousin of my wife’s Mom’s friend and lives just north of Halifax proper in Lower Sackville. Turns out Halifax has an excellent transit system and the bus ride from Lower Sackville to the hospital was only about 45 minutes during the week. Francis was a warm and welcoming host, and life would have been so much more complicated without her.

As great as the doctor at Sunnybrook was to speak to, the doctor at Victoria General was even more reassuring. He spoke in great detail (and drew pictures!) about what I should expect. We didn’t know specifically how far the disease had progressed so there were many possible outcomes. He discussed the complexity of the surgery, all of the different decisions he might have to make on-the-spot during the procedure, how long it might take me to recover, and what the impact would be on my quality of life afterwards. We decided to move forward despite the risks (there’s always risk with any complex surgery, and this one takes the cake).

The surgery was scheduled for the end of January, so we returned home for Christmas to spend time with the family.

Thankfully, my preparation on the business had gone well and the office was running cleanly without me, so during December and January I spent little time there. I got a little bored in January (hey, what can I say, I’m a geek!) and I took on some programming work to help the guys out. I was happy to have the mental distraction.

Oh, and I should probably mention I’m a hardcore gadget geek. For example, I have our DVD collection at home archived to a USB hard drive I keep attached to our Sony Playstation 3 so that we can leave the DVDs in the basement and watch what we want on TV without running around looking for them (or having them get left laying around and get scratched or eaten by the dog).

Knowing I’d be spending weeks in the hospital, I transcoded our movie collection to a lower quality so they’d take up less room on my iPod Touch and I could fit more movies and shows onto it. I also picked up some extra TV series on DVD (the only way to watch TV!). The plan was to have my wife sync my iPod with my laptop, which would replace shows that I’d watched in the hospital with new material every few days. Happily even Season 4 of Battlestar Galactica was released a few days before I had to fly out for surgery.

Surgery

The word was, I should expect to be staying 3-5 weeks in the hospital so we booked another 1-way flight to Halifax on January 24, 2009.

When I was admitted to the hospital I was put on “contact measures”, a sort of quarantine because I’d been at Oshawa General Hospital for more than 24 hours within the previous year. This actually worked out for us, as I got a private room for the night before surgery. The staff were wonderful, and arranged a cot so my wife could stay with me in the room, and even found a way to get me some free TV for the night. We met several of the doctors through the day who would be involved in the surgery, each giving us the opportunity to ask questions and tell us about each of their roles.

As part of the surgery involved my bowel, I did have to drink another 4L of the bowel prep liquid that evening. Not my favourite stuff, and it took me until almost 8pm to finish it so I had a bit of a restless night with lots of diarrhea.

The morning of the surgery, I had a shower and was ready to go when they came in and got me around 7am. There was a little more time spent in the surgery waiting area, but before I knew it I was being wheeled into the operating room. I slipped onto the narrow surgical table and put my arms onto the special rests, and was put to sleep.

My parents and sisters arrived at the hospital while I was in surgery, and waited with my wife in a special waiting room. We were told the surgery would take between 10 and 20 hours, and it turns out I took 17 hours total. The main surgeon and his assistant were present the entire 17 hours, while other specialists came and went for each their own portions of the procedure. It was a bit of a grueling wait for my wife and family but the staff kept them up to date on the progress as best they could.

Basically here’s what they did (in no particular order):

• removed my right colon and some lymph nodes around it, thankfully my left colon was fairly lengthy and clear of disease so I got to keep it
• fashioned a new pouch at the end of my small intestine and attached it to the remainder of my colon
• removed my greater and lesser omentums (the fat on the inside of your belly)
• removed my entire peritoneal lining (protective lining around the inside of your abdomen)
• removed my spleen
• removed my gallbladder
• removed a thin outer layer from my liver
• filled my abdominal cavity with chemo fluid and “stirred” me for 2 hours (no idea what that means!)
• created a temporary ileostomy so that my bowel has a chance to heal (plan to reverse at 6 months)
• the incision took 59 staples to close, with 2 more sets of 3 covering incisions on my right abdomen
• They put 2 Jackson-Pratt drains out my left abdomen for drainage

I was quite lucky. I was able to keep a great deal of my colon, which means I will have normal bowel function after my ileostomy is reversed. And, the tissues removed and sent for biopsy came back normal or with only “nonviable” cells. It was as the doctors suspected during our consults, the amount and progress of my disease was certainly on the “good” end of the spectrum.

My family says when they saw me after surgery, I was very swollen. I was also quite a sight with all the various tubes and IVs sticking out of me. For IVs I had a line in each hand, and a central line in my neck with several feeds. I also had a catheter with a bag, an NG tube down my nose with a pump to drain my stomach into another bag, the two Jackson-Pratt drains on my left side, my ileostomy on my right side, and oxygen to my nose.

The next 3-5 days were a complete blur. While I know I spent 3 days in the ICU (Intensive Care Unit), and another 3 days in a “step-down” unit (1/2 way between Intensive Care and a regular ward) before I eventually hit the regular ward I only recall a few certain events.

One was on the day after surgery. I was awoken by two male nurses who asked me how my pain was (horrible!). Then, they said it was important for me to move around – so they were going to keep giving me painkillers until my pain was gone. I thought, “Yeah right” but they did it. After several shots my pain was somewhere else, and I stood – mostly on my own even. Not only amazing after that surgery, but amazing given that I was so high on the painkillers that whenever I closed my eyes I got lost in the most amazing hallucinations. They had me sit in a chair, where I got to see my sisters and my parents and my wife for the first memorable time after surgery.

The hallucinations were crazy, and went on for days. Visually, they seemed to only manifest when I closed my eyes, but they were auditory as well – and they happened all the time. It was kind of funny, I thought my IV pumps and those from other patients were talking to each other and to me. I don’t think my eyes focused both in the same place for the entire time.

They set me up in the ICU with an Incentive Spirometer (mine didn’t look like the one in the linked picture) – a device through which you inhale trying to move enough air to raise a little ball for a few seconds at a time. It’s amazing how much your lung capacity is reduced. These first few days I couldn’t do anything with it.

Recovery

When they moved me from the ICU to the step-down unit, they had to change the central IV line in my neck (something about incompatible with equipment in the step-down units and the wards). It didn’t hurt, but it was a little freaky as I lay there under just a local anesthetic feeling them poke around at my neck and stitch the new apparatus down. They also switched me from manual as-requested injections of the “Diloted” painkiller to morphine via a PCA pump. With the PCA pump, you get your own button you can press for an immediate small dose of painkiller, and it’s regulated to only allow you to fire it so often. The best thing about it is that it was through my IV so I got a break from the painful injections – they left my arms sore and bruised.

I didn’t know what my specific PCA restrictions were, and while I was awake and could see a clock I’d tend to click it every 10-15 minutes. It was a bit strange at night though – I couldn’t see a clock, and they just said to click it when I felt pain, which was pretty much all the time. At least one night I know I used a ton of it without realizing and the IV pumps around me seemed especially chatty.

Once in the step-down unit, I became more aware of what was going on around me. I became more aware of my wife at my side every day, mostly reading or stitching as I slept. We didn’t interact much, but it was a great comfort nonetheless to have her near. I started doing some regular things like brushing my teeth, and washing a little. Moving around was still a huge ordeal because I still had all 9 of my hoses and tubes sticking out of me, along with 2 IV poles full of pumps. I was being given TPN (2 bags, one a yellow fluid and the other a white milky fluid for nutrition), heparin, morphine via the PCA, and a variety of other medications on a periodic basis. They took the pump off my NG tube so my stomach just drained on its own.

I got better with my Spirometer, got up to 1/2 strength. I did not yet understand how critical this activity would become for me and didn’t practice it as much as I should have.

About a week after surgery, I was finally stable enough to be moved to a regular nurses ward, and within a couple more days things really started happening.

First, they took me off my PCA machine. I was sorry to see it go, but was happy that I could manage with less painkillers which meant I was more and more aware of what was going on around me. For the first time since before surgery I could start to focus both my eyes in the same place.

Within a couple more days (around day 10), they began removing tubes. At one point I found it more comfortable with my NG tube plugged (the one to my stomach), and they decided it was time to come out. They took it out and my catheter at the same time both of which were painless. Shortly after they took the two Jackson-Pratt drains from my left side (the tubes into my abdomen were very long and quite uncomfortable when pulled out). They even took out my central line – which was uncomfortable, but not painful. They took me off the TPN and put me on a basic fluids IV.

With my improved ability to focus and concentrate now I was very thankful for my prep work and planning with my iPod. It was a great help to pass the time watching some familiar and favourite movies and catching up on my favourite TV shows. Turns out that spending over $12 / day for 30 days on the hospital TV by the bed would have cost me almost as much as my iPod Touch.

Around day 11 I ran into a bit of trouble, and I blame not spending enough time with the spirometer and practicing my coughing. My lung capacity was so much reduced I simply couldn’t clear a tickle from my throat. It shouldn’t have been a problem, but I spend a couple nights where the tickle made me try and cough, but I couldn’t effectively do it and eventually started gagging which then turned into vomiting. I was still not quite mentally there so I didn’t fully realize at the time what was going on, plus my abdomen felt so different I couldn’t sort out what was nausea, hunger, pain from my incision, or pain inside. So when this occurred the nurses didn’t quite know what to make of it and I ended up on an anti-nauseant, which in hindsight wasn’t really what I needed.

The nurses thought that maybe the NG tube was taken out too soon, I didn’t really know at the time what to think. The doctors weren’t convinced however because I had stated that I did feel better with it plugged. I think the doctors were right and I do fully blame not doing more lung exercises and better preparing myself for such a simple thing like a tickle in my throat.

Also around day 11 they started me on clear fluids – jello, apple juice, cranberry juice, chicken broth, tea. I savoured my jello like the finest steak. For the first several days I stuck to very small then increasing amounts of the juices and jello, then started to venture to the broth. The broth scared me a little because it had bits in it (wasn’t perfectly strained) and they tickled my throat sometimes. Around day 17 they upgraded me to full fluids, which got me milk, oatmeal and cream soups. Then on day 18 I was upgraded to soft foods, which got me dry cereal, an apple muffin, cheese, shepherds pie and stews, and dinners like mashed potatoes, green beans or squash and roast beef or pork or broiled chicken or fish.

On day 14 the physiotherapy nurse started coming around regularly and I started walking and sitting more regularly. I started on a walker and after a couple days was walking on my own. I was working my way up to 3 walks a day up and down the hall. At this time I was only occasionally on an IV so I really appreciated the freedom being able to walk unencumbered.

The ET nurse came by and I got my first training on my ostomy. She explained everything about it and changed the pouch for me, and said there would be a few more visits where I would eventually be changing it myself. From this point on I was expected to empty it myself, which meant getting up to the bathroom, measuring its output, and marking it on my chart. It seems such a simple thing, getting up and going to the bathroom on your own, but I hadn’t done it in over 2 weeks. I had to get used to getting up when my body needed me to rather than just when I felt like it.

Since I wasn’t on any IV nutrition, I think they wanted to get me onto actual food as quick as my body would let them. And it worked, during my third week in the hospital I started to gain strength.

Until my next issue cropped up that is – which was spiking potassium levels. This was probably the most frustrating thing for me, mostly because of the way they handled it. I don’t know that there was any other way they could have, but it was frustrating nonetheless.

Normal potassium levels in your blood are something like 3.6 to 5.1 (this varies a little in the medical community I think). Mine was spiking between 5.3 and 6.0. Apparently if it gets to 7, you start to get some arrhythmia in your heart (scary). The normal treatment was this absolutely horrible liquid drug called Kayexalate. It was very thick, chalky, brown, with that slight fake fruit flavour you get sometimes in medicine. The first time they brought it to me, the nurse was so apologetic and I knew I was in for a treat. I had just had my first stew (chicken and vegetable) for lunch. I tried the fluid and it was so thick I gagged on it and completely lost my lunch.

Every day my potassium came back high, and every day they brought me 1/2 a styrofoam cup of the kayexalate. My wife suggested a popsicle, and it turns out that was the trick for me finally getting it down, which was basically tiny sips with a popsicle in between.

Kayexalate works by binding to the extra potassium and then passing it through your bowel. For me this translated into constant diarrhea, which basically meant my ostomy was liquid all the time and needed constant emptying. I was up every few hours day and night to the bathroom, which was exhausting. I don’t think at this point I was getting the nutrition I needed from my food because everything just went straight through me. As a result the strength I had been gaining seemed to slip away from me, and I ended up spending more time in bed.

The doctors had been fairly happy with my progress and were making noises like I might be able to go home soon. We started to talk about flight plans, but didn’t tell the family. As it turns out, until that release is actually signed and you’re leaving the hospital, it’s tough to count on when you’re actually going home.

Blood clots are a concern when you’re lying around in a hospital bed all day. Since surgery, I was wearing TEDs (tight stockings to keep clots from forming in your legs), on 3 Heparin shots per day, plus Aspirin and Advil to thin my blood. They urged me constantly to get out of bed and walk around or even just sit in a chair for a while. My reduced strength meant I was able to do this less and less.

Despite the precautions, on day 18 I had a pulmonary embolism – a blood clot, in my right lower lung. In the morning, after breakfast I started to get a spasm in my back. It was very painful and caused my whole body to tense, and my breathing to become more shallow. I tried walking but didn’t make it to the door of the room before I had to turn back. After a call to the doctor they prescribed an IV muscle relaxant (Flexural) and it helped some. It calmed down through the morning and started to act up again around lunchtime.

Through the afternoon it got progressively worse. By 2:00 the pain was constant and I could barely breath and they put me on oxygen. One of the senior nurses had a hunch and put an extra IV in me in preparation for any medication they might need to give me. I was gasping and taking the tiniest breaths as fast as I could while then ran tests. I had an EKG and an X-Ray at the bed, and they wheeled me down to the 3rd floor for a CT scan. They quickly identified the clot and had me up to the ward and on a Heparin drip pronto on the extra IV line.

The muscle spasm confused things a little, I truly thought that’s all that was wrong with me. And I guess while it’s not common it can happen along with a clot. Thankfully I hadn’t gone home yet, and my nurse was totally on the ball.

For the next 2 days they took blood 3 times a day and adjusted the Heparin drip accordingly so that it could dissolve the clot. My back was still spasming and I still found it hard to breath but it got steadily better over the next couple days.

By day 22 my INR (blood clotting factor) was at therapeutic levels and they switched me to Warfarin pills.

My potassium was still spiking, though less often, and occasionally my body was able to process food properly. I was very frustrated being on the Kayexalate but the doctors couldn’t pinpoint what was causing my potassium to spike. Their theory was that due to my high white blood cell and platelet counts cells were dying off and spilling extra potassium into my blood. Tests on my kidneys came back normal. One of the doctors from Medicine tested a theory that the Advil I was on might be causing it so that was discontinued.

I was gaining strength again now that the Kayexalate was less frequent and my small bowel was starting to work better. My body was processing food better, and I had more energy and got back up to my 3 walks a day and spending as much time as I could out of bed.

I even went down to the cafeteria to sit with my wife while she had her lunch a few times. It was a longer trip than just walking down the hall, and was a nice change of scenery. By this time, 3 1/2 weeks in the hospital, I was starting to feel a little caged.

Around day 26 they decided to hammer me hard with Kayexalate and try to push my potassium levels down as far as they could. They gave me a full cup at a time for a couple days to try and do this, but it didn’t really reduce it that far. Luckily by this time I had worked out a trick, they seemed to bring me the Kayexalate right after my meals (breakfast / lunch), but instead of taking it right away I’d wait until a couple hours after I’d eaten. This let my body process the meal better and helped with my gaining energy.

On day 28 I got a day pass, and my wife and I walked down the street from the hospital to Smittys, a nice independent restaurant a bit like Golden Griddle. The walk was fairly long and my hips got a little sore, but I had a great lunch. I ordered off their Seniors menu and had bacon, an egg, and 1/2 waffle. I was starting to feel like a real person again.

On day 28 the doctors also started talking about going home again. I was feeling pretty good, and especially after my day trip I figured I was ready, but was also a bit nervous. My wife booked a flight on day 31 in the morning and we crossed our fingers.

On day 29 I saw my surgeon for the last time. We talked about my recovery, flying home, reviewed what to expect in the next few months. He reiterated that my recovery was very good, despite the setbacks.

Going Home

On day 30, I was released. It was a very busy day, I saw the ET nurse for the final ostomy training, I saw a doctor from Medicine for my warfarin training and to get my prescriptions and a few extra Warfarin pills to last me until we could get our prescriptions filled at home. I saw one of the doctors on my surgical team for a final checkup and a few more immunization shots, and we gathered all of my stuff together in preparation to leave. By lunchtime all was said and done, and after I had my last hospital meal we stopped at the nurses station and said our goodbyes.

We stayed that night at a hotel near the airport. There was free Internet access at the hotel and I was able to use Skype on my laptop to call family to save the hotel phone bill. Dinner was a bit of a challenge because none of their meals fully fit my dietary restrictions, and they didn’t have any Tylenol (I had no pain medication at all, in hindsight I should have asked for some Tylenol and maybe a Morphine pill for the trip home). The night was a little restless, the bed was flatter than I was used to, I had to change my ostomy appliance for the first time without assistance, and we had to get up at 3:45am Halifax time to catch the shuttle bus to get to the airport and catch our 6:30am flight.

The morning was long, up at 3:45, shuttle at 4:30 to the airport (bumpy bus ride, it was very uncomfortable). I got a wheelchair at the airport, and we were settled at the gate at 5:00am. None of the airport shops were open, so I still couldn’t get any tylenol until 6, and then we boarded. We flew on Westjet and the 2.5 hour flight was not bad. The seat was comfortable enough (we were in the 3rd row) and unlike the bus there was no jostling around so it was reasonably comfortable. My wife’s dad picked us up at the airport and we had an hour’s drive home, which was a little bumpy and uncomfortable but not nearly as bad as the shuttle bus.

There’s something magical about being home after an ordeal like that. Beyond finally seeing my kids again. I think it’s true that I’ve recovered faster and easier since I’ve arrived home. Maybe it’s that I could finally relax. Maybe it was the reduced stress of having my own bathroom or wearing normal clothes, or my own bed. Maybe it was my wife’s cooking and getting back to our normal diet.

When I got home, the first thing I did was weigh myself. I was 215.5 lb before flying out for surgery, and 174.5 lb after returning home – 41 lbs lost.

Now, after almost 3 weeks at home, I’m able to make short trips to the store, walk for about 15 minutes before my hips hurt, and can do simple things like wash my own hair and put my own socks on. Stuff you really take for granted until you can’t do it.

Final Words

From here on in, I play the wait and see game. There will be regular visits to Sunnybrook and regular scans for the foreseeable future now as they monitor my abdomen and look for any recurrence.

My business is stronger than it has ever been, despite this building recession, and I attribute that fully to the strength of the staff and the preparations I was able to make in 2008 to assist them.

The whole experience worked out very well for me, all things considered. I know this was a combination of my age and good health, and lots of prayer.

I wish you all good health. I saw the mild side of cancer – and it pains me to think of all the people, some whom I’ve known, suffering or taken by this terrible disease.