On July 18th, Vertex Pharmaceuticals announced positive early study results for next-generation CFTR modulators. Why is this exciting news? Because this is what many of us with cystic fibrosis have been waiting for: a medicine that will treat the underlying issue of the disease for those of us who have a minimal function mutation. While patient accessibility is still a little ways off, the bottom line is, these medicines aregoing todrastically change lives! Here is Vertex’ press release and then the Cystic Fibrosis Foundation announcement is here.
At the beginning of 2017, in hopes of giving these lungs a good year-long clearing, I set a goal of running 10K (6.2 miles) each week for a total of 322.4 miles for the year. Up until I got sick a couple of months ago, I was averaging 7 miles a week. There were a few weeks in June/beginning of July where I fell short of the goal distance but now I’ve been back at it for the past 3 weeks. Miles run in 2017=189 so far. I’m not stopping anytime soon:) Hoping to reach the goal before the end of the year!
I’ve been taking the CF supplement Indrepta for over 7 months now. You can read about this supplement here. There are now 3 versions of Indrepta available. On November 26, 2016, I began taking Indrepta A for a couple months. I then switched to Indrepta B for a couple months, and when C came on the market, I decided to try that version for two months. After trying each of the versions, I decided that Indrepta B (click here to read about which version might work best for you) was the most beneficial for me and so I have decided to stick with B and about 4 weeks ago, I added Taurinac into the mix. I had already been taking PharmaNAC fizzy tabs for a few years but Taurinac supposedly has some additional benefits specifically for cystic fibrosis so I switched.
I have not experienced dramatic changes yet; however, since adding Taurinac, I have recovered from a respiratory virus amazingly well. And that is not the way it usually goes for me. I don’t usually get back to baseline lung function after fighting a respiratory virus. While I was very sick with this most recent respiratory virus, I did in fact rebound and my lung function is now back to where it was before I got sick in June. While the other positive changes have not been drastic, they are still worth noting.
My weight has been extremely stable, even during this last bout of sickness.
My sinuses seem a little less congested. (still doing 3 saline sinus rinses per day)
It’s been a little easier to work the mucus out of my lungs during treatments.
I’m not sure if my most recent recovery and/or these other changes are from the addition of Indrepta and Taurinac but I am definitely going to keep taking these together and hopefully reap more benefits the longer I continue.
We can all think of something to be thankful for. This week I’m thankful for:
Jim and cousin Isaiah- climbing monkeys
The safe arrival of my cousins who traveled here for a month from Uganda to visit family and friends. My cousin serves as the executive director at Cure Children’s Hospital of Uganda and his family will be returning there next month. We have missed them so much and it is such a treat to have them here!
July 4th canoe adventure
Continued progress on the health front. I had another follow up doctor’s appointment on Monday and my FEV1 is back up to 32%! I had my picc line pulled 🙂 My resting oxygen sats have been great this week and I have been able to sleep better than last week. My endurance while jogging is also getting a little bit better each day. I’ve appreciated being able to get out of the house for a change of scenery on many occasions and even went to work on Wednesday….watch out, here I come!
I’m thankful for an ice cream treat with a good friend. It’s always refreshing to spend a little time with someone who lifts you up. Strawberry cheesecake has been my go to flavor this summer. So good!
I had a follow up appointment this week and my doctor seems to agree that things are moving in the right direction. I’ll be doing at least another week of IV antibiotics and go back for another appointment on Monday. I’ve been able to be oxygen free while at rest during the day and that is such a gift.
I’m thankful for simple things that put a smile on my face these days. During workouts in the basement I keep seeing these cute little birds come to bathe in the puddle outside of our basement door. They seem to have such a fun time with each other. I’m reminded that God has a purpose and a plan for every creature, great and small.
Sometimes pets are the best medicine. I’m thankful for a sweet puppy to cuddle with while I’m not feeling well.
I’m thankful to have been well enough to celebrate Jim’s birthday out at Queen Lake over the weekend. My parents and family helped me throw him a birthday party😊 He is my favorite and such a special guy!
Let us hold unswervingly to the hope we profess, for he who promised is faithful. ~Hebrews 10:23
I’ve been really sick recently. It always starts with a tickle in my throat and then things sort of fast forward to a chest cold/fevers/all around feeling lousy in less than 24 hrs it seems. I’ve been here many times before but never with a baseline lung function this low. I went to the hospital for a picc line placement a week ago and will probably be receiving IV antibiotics from home for 3 weeks. Maybe this doesn’t seem like a big deal and in the grand scheme of things, I’m hoping it’s not but I found myself discouraged a few nights ago.
For the last 7 months, I’ve been going through a lung transplant evaluation at a center that is close to my home (60 miles away) in hopes that I may be able to have my surgery in New England. If I had a choice, I’d like to be near our support of family and friends during recovery. Just before I came down with this cold, I was told that this center will not be able to perform my lung transplant because they discovered that I have a mild case of portal hypertension. It is now very unlikely that any lung transplant program in New England will take my case.
There are too many details to write in this post but my heart just sank when I heard this news. This was not the phone call I was hoping to get. I’d been told up to this point that I am an ideal candidate for lung transplant surgery. When I caught this cold, I found myself really struggling to fight back. I didn’t and don’t have things figured out and secured. What if I didn’t improve and get better this time? And when I get sick, I get sick. Days run together. I don’t get sleep. Antibiotics make me feel lousy. Fevers make me so loopy. O2 24/7. Breathing is so hard. All. The. Time. Not just at certain times. All the time. Let’s just say, I wasn’t in a good place a few nights ago.
I was very honest with God about my feelings. I told him I didn’t have the strength to do this and that I’m just so tired of everything being so hard. In fact, I cried out to him frequently. Pleading with him for relief, strength, and comfort. Everything just seemed so impossible.
And then all of a sudden a few nights ago, I actually slept!
I’ve seen a little bit of improvement each day since that night and was even able to be without my O2 for a little while this afternoon. I am not sure if I will fully recover and I am really not sure of anything having to do with lung transplant surgery at this point but I do know one thing. I continue to experience the faithfulness of God. He is always good and he is always with me. He is able. That is all the security I need.
Thank you for your support this year. Team Emily’s Athletes raised $11,260 for cystic fibrosis research and drug development in 2017! We are so thankful to everyone for their generosity in supporting this cause.
May is Cystic Fibrosis Awareness month. Walking in the Cystic Fibrosis Foundation’s Great Strides walk is one of the things that Jim and I do each year to raise awareness about cystic fibrosis and support the effort to find a cure. Our team, Emily’s Athletes, will be walking on May 20th. We are so thankful to the many of you who have generously supported us this year and in years past. If you haven’t contributed yet and you’d like to help, we’d love to have your support. Check out our team, Emily’s Athletes here 🙂 Nearly 90 cents of every dollar donated goes directly to cystic fibrosis research and drug development.
I am so thankful for the Cystic Fibrosis Foundation and all they do to add tomorrows for those of us who live with cystic fibrosis. Their effort and commitment to the fight against CF have added decades to my life and many others. I’m excited for what the future holds and I’m confident that cystic fibrosis will be stopped in its tracks very soon.