Amy has Trikafta!!!!!

An update on Amy for all those who were wondering about her declining health. There is some bright news in our lives today.

Amy’s lung function is currently under 30% the predicted value. That’s about the point when I was listed for a transplant. However, she is mostly stable so unless she has a sudden crash, she isn’t eligible for the lung transplant program. She feels healthy in the sense that she has no acute infection but her lungs are terrible from years of scarring and any cold, flu, or virus (!) could kill her.

And she’s starting Trikafta today! Trikafta is a triple combination drug that is for those with the most common CF genetic mutation. It’s a breakthrough that was on a lot of Top 10 most exciting scientific discoveries lists at the end of last year. Those in CF community have been promised life changing breakthroughs before but these new meds actually seem to be delivering on the promise. They're taken for life but the hope is that it'll make CF a manageable disease that lets people live until old age.

The drug is not approved in Canada. Vertex, the pharmaceutical company, hasn’t submitted it for governmental review yet. Advocates in the CF community and within CF Canada are working hard on reaching an agreement between Vertex and Health Canada to have this orphan drug approved but it’s moving slow and people with CF are dying in the meantime.

Everyone in government is currently busy trying to prevent the general population from flooding the hospitals with COVID19 cases – but when Trikafta does get approved and as long as it’s affordable, it will most benefit those who still have their relatively healthy lungs. It’ll increase quality of life, reduce hospital stays. Provide people with CF the opportunity to plan for the future instead of wondering if they’ll live past 35. Trikafta has been approved in the UK and the US and those who have access to the drug before their lungs are "critical"are having lung function increases of 20-30%. It’s working wonders. To use the word infuriating that a medication exists that can dramatically change peoples lives but it’s just out of reach is putting it mildly.

Back to the positive, Amy is getting it! She has it. She has it in her hands.

She was approved through Vertex’s compassion care program which is providing Trikafta for Canadians with CF who have critically low lung functions. This does mean though, that the 20-30% increase in lung function that other people are seeing will probably not happen with her.

The doctor’s best guess was a 5% increase due to all the previous damage to her lung tissue. But 5% is still 5%. And if it stabilizes her lungs, that would be crucial to her not crashing out. We don’t know yet how well it’ll work. Today is Day 1 and we’re all hopeful and celebrating that Amy has the chance to try it.

Thank you to everyone who has been checking up on me during the outbreak.

I'm off from work right now for the foreseeable future. While Isaiah is still working, he's in a small office and hoping to work from home at least 50% of the time.

My doctor has told me that being post-transplant and a diabetic, I'm more immunocompromised than their general population of people with cystic fibrosis (which is already high risk) so I need to do everything I can to avoid the virus. I'm a bit anxious mentally when I read about people still going out to social gatherings or to pubs and restaurants. Breaks from the news and social media helps but I also compulsively like to know what is happening.

I've have so many hobbies to keep me busy at home from all the time I've spent laying low before and after my transplant and during chemo. And social distancing doesn't mean having to avoid nature. I just have to avoid the lodges.

Everyone has read all the stats and most people will get through this healthy. But staying home is about more than "getting through this." It's about keeping the vulnerable (me!) safe and not overwhelming our health care system. Our health care system that has people treated in the hallways on the best days. It'll break in seconds if we're not careful.

Be safe and wash those hands. ❤️

5 year lungiversary!!!

In a week it's going to be my 5 year lungiversary. 5 years!! I can barely believe it. 5 years since that night in the hospital when the nurses came to tell me that there were donor lungs for me. 5 years since I wasn't sure if I was going to live for another few weeks. 5 years since I was too tired to brush my teeth or get out of bed.

If you had told my past self that five years after my transplant I would have gone to a surf camp in Hawaii, bought a house, and be working an almost-full-time job, I wouldn't have believed you. 

Or that I would have travelled to Europe and had a book published, I still wouldn't have believed you.

Our cute little house!

I never thought I was going to get five years out of my donor lungs. I mean, I hoped I would but I never let myself believe it was possible. Especially with the mess that happened during the first year after my transplant. 50% of people post-double lung transplant live for five years. 50% of people who develop post transplant lymphoproliferative disorder are alive after two years. I've been unbelievably fortunate.

I'm not going to list everything I've been able to do because of my donor as the list would be endless. The first item on that list though is “breathe.” Just being able to breathe. Breathe in cold air without my lungs seizing. Breathe in humid air while showering which I no longer dread.

After 5 years I remain grateful that I can walk up stairs without wheezing. I can sleep without needing 3 pillows to prop me up. I can laugh without having a coughing fit afterward. I can stop worrying about how many calories I'm consuming and not have to supplement my diet with Boost or Ensure.

(So much for not starting a list.)

I know everything I'm saying I've said before but it's just so amazing to be reaching this milestone. Alive and not spending all my time in the hospital. After my rough start, I'm not very high priority for any of my doctors anymore. I still have quarterly checkups with my respiratory team but my hematologist has pushed me to annual appointments and will most likely kick me off her rotation soon. I don’t even have to go Toronto this year for my annual appointment which I’m a bit sad about because I love being a tourist in Toronto for a few days. I’ve been deemed stable enough that all the annual tests have been done in Halifax. The only new medical process that happens at 5 years is that they start regular colonoscopies because the risk of colon cancer is quite high. So that’s...poopy.

The risk of skin cancer also increases significantly at 5 years  and although I’ve been followed by a dermatologist since the transplant, I need to become more diligent about sun protection.

There's still a small part of me that keeps waiting for the metaphorical health shoe to drop. I get up in the morning and put on a fun dress, make a giant coffee, and go to work and think “this is too easy” or “when is this all going to change?” I know it's not necessarily rational but it's hard to shake off the feeling like something bad medically is about to happen as soon as I've gotten parts of my life together.

Hiking Cape Chignecto!

I wish I could just relax but it's also that paranoid part of me that keeps me from taking too many risks and reminds me to stop and prioritize my health. I am, and will forever be on my anti-rejection medications which lowers my immune system and leaves me vulnerable to catching any bacterial or viral infections. The medication is to prevent organ rejection – if my immune system were healthy, my body would start attacking my lungs and we don't want that. However, having a lowered immune system means I have to be cautious about getting an infection. I know people who have died post-transplant after contracting the flu virus so the seriousness of infections shouldn't be minimized (get your flu shot!).

Because of this, I avoid people who I know are sick. I wash my hands a significant amount. I nap when I'm tired. Isaiah gets to eat any and all sketchy leftovers from our fridge. I worry about catching the measles whenever there is an outbreak anywhere nearby (as in anywhere east of Montreal). The fear keeps me cautious even though I know I can do everything right and still catch something that could kill me.

But being careful isn't why I've managed to avoid organ rejection, people get rejection for many reasons and I don't want you to think that it's brought on by the person's own actions. It's a thing that happens and I suspect it'll one day happen to me.

And when it does, I’ll deal with it at the time. Life is too short to worry about hypotheticals although it’s much easier said than done.

I kept trying to wrap this up with some tear jerker story about all lessons I've learned in my bonus time on this planet. But what is there to say that hasn’t already been said? Basically, life is short and precious so love deeply, take risks, and follow your dreams.

And while that’s true, the reality is that part of me living every day to the fullest means sometimes I spend way too much time on the couch eating chips, watching TV, and scrolling through social media. Downtime is also an important part of life that's often overlooked but that's a rant for another day.

There’s no possible way for me to distill my gratitude for my donor, their family, my health team, the transplant team, the medical system, and my support network into a simple phrase except to say Thank You. Thank you to everyone who has been there for me and my family. You’ll never know how much your messages and letters meant to us.

Next week I'll officially be 5 years post-transplant so on Saturday Isaiah and I are hold a house warming party/5 year lungiversary (everyone welcome!). On the actual 26^th I'm taking the day off so I can sleep in, have Isaiah make me a fancy breakfast (he doesn’t know this yet), and spend the day with my loving family to celebrate being alive.

5 years. I never believed it would happen. 

New job!

After years of saying that I wasn't looking for work more than the one and half days a week I was doing at the library, this week I'm starting an almost-full-time job at the desk of the Mount Allison Library. I'm both nervous and excited. It's going to be a huge change for me, working five days a week, having to wear something other than sweat pants, not being able to nap whenever I feel sleepy. Now it'll be all professional clothes, commutes to work, and probably an increased caffeine addiction along with everyone else. I'm excited about the actual job itself – more responsibilities, new computer system to learn, a university setting – and am mostly concerned that I'm not going to have the stamina for a five day a week position.

I'm going to have to be pro-active about putting my health as my top priority and realize that everything else will have to take second place. Especially for the first month when I imagine I'm going to be quite tired while learning the new position as well as the energy required in just being at work five days a week and moving more.

So I've been meal prepping all week, baking bread, making mini meatloafs, and freezing calzones that I can just grab and go for a solid meal. Isaiah would normally happily cook meals for us but he got Bell's Palsy last month and has been off work due to his very slow recovery. He's going back part-time this week but he's still unable to close his eye or chew food. Needless to say, he's also going to be quite tired for a few weeks.

I had always thought if I was going to switch jobs it would be more for maybe another part-time job or for a rec position but this one came up and Isaiah convinced me to apply even though I was positive I wasn't qualified. I figured it wouldn't hurt to apply and if nothing else, I would have updated my resume and then, to my surprise but not Isaiahs, I got a job interview. I wasn't hired for the actual job that had been posted but for a very similar one that is considered a 'temporary' position. So after next April, I may not have any job but at least I'll have tried something different and I'll know by then if I'm comfortable and healthy enough to work more hours.

You may be asking “but why would you take a job if you don't know that you can do it?” and the answer is “because I want to know if I can.” I'm almost five years post-transplant now and I want to push myself a bit and see if I'm ready for it. If I am, that's great. Once this job is over, if it's not renewed than I'll know I can start looking around at other positions. If it's a disaster than that's an answer as well. I'm really trying my best to recognize that this may not work out for me and that's okay. Sometimes it's enough just to see if it's possible and learn that it's not. Or so I keep telling myself. Eventually I'll believe it.

Word on the Street!

This past weekend I participated in Halifax's biggest book event, Word on the Street. It's the event's 25th year and while it use to take place on the waterfront, it's now in and around the Halifax Central Library. Friday night I attended the welcome reception for the authors and exhibitors on the beautiful 5th floor where we ate delicious canapes and sipped wine as the moon rose over the Halifax harbour. If you've never been to the library, the view alone from the balcony is worth the trip. It was a fun night even though I'm not great at mingling or inserting myself into conversations.

My room!

Saturday was the main event where I did a quick book signing at the Nimbus tent and presented as part of a "Life Stories" time slot and then had another quick book signing in the main signing area. I thought I would have more time to attend panels but all I managed to do between events was grab a quick snack. I did manage to attend a panel at the end of the day about romance writers of Atlantic Canada which was motivating to me to keep writing the romance novel I've been picking away at.

The event had a great energy to it and while I'm pretty sure I spent more time chatting to other authors than the general public, it was a fun day that required a 2 hour recovery nap once I got back to where I was staying.

So many good authors!

It's me!