Now That It Is Over (Maybe) I Can Talk About It

The last few weeks of my life have been the most terrifying and joyful I have ever known. The purpose of this blog has never been to document every aspect of my life, and I purposefully kept my pregnancy quiet because of all the complications that could have arisen as a result of the disease. But I do think it’s important to talk about the disease, if only for other people in the world that have Wegener’s Granulomatosis. If someone searches and ends up here, and knows about what I’ve gone through, maybe it can help someone else going through something similar.

We managed to make it to 34 weeks without very many problems. In fact, all of the problems that I did have, swollen joints, exhaustion, some other minor things that could all easily be explained by pregnancy. There’s just one thing… they could just as easily been explained as the disease flaring. It was impossible to tell what was what: was I developing pre-eclampsia or was it the disease or was it just pregnancy in my case. Because it’s so rare, both my disease and then women with my disease having babies, there were so many unanswered questions. The main thing I knew was that I was being followed by exceptional doctors. Then, my lab results showed disease activity, and that was that, things were flaring. It was minor and we’d get it under control. Only it didn’t end up being minor at all. It ended up being dramatic, violent and life-threatening.

Five weeks ago my lungs started bleeding. I had just seen the OBGYN that afternoon and was so very tired. For the previous couple days, I couldn’t catch my breath, but when the baby gets big enough, it too pushes on your lungs. Hence, yet another explanation for a disease symptom that can be confused with pregnancy. But when I coughed and a chunk of blood the size of a toonie landed in the sink, my heart stopped. That’s how the disease first presented itself. That’s how I got sick in the first place all those years ago. So, I sent a quick email to my pregnancy nephrologist (doctors give you email addresses now!) and asked her what to do. She sent us to the Labour and Delivery section of Mt. Sinai and then the ordeal really started. It’s called hemoptysis — I always used the term hemorrahging to describe what the disease does to my lungs — but essentially, they were bleeding internally and it wasn’t stopping. Because it’s an OBGYN ward and not a disease ward, there was some confusion about what to do with me and the first night I spent in the hospital, the orders didn’t get transferred over. I spent a miserable, freezing cold, uncomfortable night with a bone dry IV and coughing up bucket loads of blood for hours until they started rounds in the morning.

By then, the severity of the situation had dawned, and the chaos began. The words “life-threatening” were uttered more than once and they floated around the idea of putting me in Intensive Care. In the end, they moved me back to Labour and Delivery for one on one care. Through the course of the day, I saw, no word of a lie, 52 doctors that first day. They decided to treat the situation aggressively because of the baby. Let’s not forget that I am still pregnant. They decided to do plasma replacement therapy. It’s kind of like dialysis. They take out all the bad plasma, the stuff that contains the antibody that is the cause of the disease, and replace it with donated blood product. The machine is hooked up to you via a central line, and out goes all your plasma into a bag that gets destroyed. The treatment takes about three hours all tolled and then you get unhooked. We did seven days of this, plus IV steroids, to try and stop my lungs from bleeding. It took over 1o days but it worked. The disease somewhat settled down at that point, and the “plex” therapy as they call it is quite amazing — in short, once it was finished I could feel no disease in my body whatsoever, but it only lasted until the next treatment. It did, however, contribute to my condition becoming stable. We had one little glitch with the plex, and on the second day my hemoglobin dropped, and so I had my very first blood transfusion. Of course, it didn’t work the first time, so we had to do it twice. I signed a lot of consent forms and tried not to worry about all the risks. I suppose when you weigh the pros and cons, it’s better not to be dead.

Things looked up from there, at least for a while, until I started developing pre-eclampsia, which is pregnancy induced hypertension. With women with underlying renal problems, the likelihood of pre-eclampsia developing is high and, of course, it’s hard to tell whether the disease encouraged the pre-eclampsia or whether or not my pregnancy would have ended up with the complication anyway. The higher my blood pressure went, the more medication I had to take, everything, luckily, is safe for the baby, who, by the way, is perfectly fine. So, I am in the hospital, taking more medicine than ever, sicker than I’ve actually been since I was first diagnosed with the disease, and pregnant on top of it. The prednisone’s making me a little batty, so I’m cleaning my hospital room at night and trying not to lose it being hooked up to all the machines, poked for blood everyday. The one saving grace was listening to the baby twice a day on the monitor and knowing that he (we didn’t know “he” was a “he” at this point) was just fine.

After I had been in the hospital for two weeks and seen probably over 70-odd doctors, they called an “all doctors” meeting to decide what to do. According to my OBGYN, the plan was to get me stable and then let me go home and deliver the baby naturally, but when the pre-eclampsia started to rear its ugly head, they changed their mind and they decided to induce at 36 weeks. And when they make that decision, it starts immediately. Like, that same day. So, over we go to Labour and Delivery again and they being that process. It takes forever. I was still doing work because I was so bored just sitting there waiting for it all to happen. It’s all a bit clinical, but it was the safest way to deliver the baby, and when my contractions really started they gave me an epidural because they didn’t want my body to be under any more stress and for the disease to go haywire again.

So, we were mid-way through the next day, Friday, October 22, when they discovered that my placenta had really started to act up and was causing the baby distress. So, no labour for me — they whisked me into an operating room and we had a c-section. Our RRBB was born at 3:04 in the afternoon and weighed an impressive 6 pounds 9 ounces. Not bad for 36 weeks.

The next few days are spent just dealing with being new parents, something that many people go through, we had plenty of rough starts, namely with feeding, and only because we didn’t quite realize that just because the baby falls asleep after eating that he’s actually full and/or had enough to eat. I was also really anemic and taking tonnes of medicine, so it took forever for my milk to come in. In the end, they let us go home, finally, after two weeks and five days in the hospital. Oh, but before we left? We had another transfusion because the surgery dropped my hemoglobin to 72. Imagine this — having a blood transfusion while trying to breastfeed, the tubes stuck in your arms, the baby in your arms, grabbing at everything, but it worked and my iron counts were much better the next day. That gave me some much needed energy to just be home after all those days cooped up in a tiny hospital room.

We spent a glorious 24 hours at home.

Then the baby developed a little jaundice and the family doctor’s scale miscalculated his weight so we were back at the hospital for him. That was a rough night. We weren’t in our nice, private room any more but on the maternity ward. The couple next to us had their own baby, and baby schedule, so we didn’t sleep a wink. Of course, my blood pressure was sky high, from worrying about the baby and from no sleep, so when they checked it for me, it was in a state where it wasn’t safe to let me go home. We were admitted back into the hospital again, and I almost lost my mind. The resident, who was only trying to help, wouldn’t let us leave. I tried to explain that I could monitor my BP at home (I had a cuff) and was seeing a team of doctors in a week, all of whom know about all of the conditions — she kept trying to diagnose me and I kept saying that all I needed was proper rest and to get my baby out of the hospital. So, I tried to check myself out at night and the nurses and my RRHB convinced me to stay — they didn’t put anyone else beside us and my BP came down slightly. But that was it for me, it was now Friday and I had been in hospital for three weeks, one of which with my poor newborn who had seen his sweet bassinet only once in his life.

Then I did something completely out of character and checked myself out against doctors orders. My BP by the time I got home and relaxed and got some sleep? 123/71. Perfectly fine, as I knew it would be. But those last couple days were the breaking point for me. I just couldn’t take anymore. Luckily, things are now starting to calm down, we think. The disease is still scary and active, and I am still recovering from all of it, but the baby is thriving, gaining weight and doing really well with breastfeeding. The last time I was this sick, it took over two years to get better, and who knows if my body will ever recover. Funny, all the doctors told me it would be fine — well, that there would be risks but that they’d catch the disease before anything too terrible would happen. Oddly, just like when I was first diagnosed, the disease is mysterious and difficult to diagnose until it’s on you like a tsunami, dragging you down and drowning you in your own blood.

Now, the really hard part begins. The recovery. Dealing with the exhaustion is one thing, I mean, we have a glorious little newborn at home, but dealing with everything else that happened on top of it took its toll. I feel a lot like I am just coping. Just getting through the days hour by hour, and that’s all I can do: plasma replacement, IV steroids, bucketloads of drugs, two transfusions, another disease (pre-eclampsia that apparently lasts for six weeks post-partum), a life-threatening flare, surgery, a new baby, and prednisone-induced crazies. It’s a lot. I am at loss for words but we are also incredibly lucky. We have a lot of support, my RRHB is amazing, and we have some of the greatest friends and family two people could ever have surrounding us.

Plus, we have him. And he’s beyond words.