I think the title says it all. I’ve been so excited about writing this post because it’s the first time that I’ve been able to share that I’M GAINING WEIGHT! I am so overjoyed!
This past Wednesday, I headed to Fort Worth for yet another appointment at Cook Children’s. I knew I had been gaining weight, but I thought I was still at 104 pounds (what I had weighed Tuesday afternoon). To my surprise, when I stepped on the scale, it read I weighed a whopping 106.5 pounds. I started to cry. The nurse that was checking my vitals commented that I had been 100 pounds just six weeks prior at my last visit and I should keep at it! My oxygen levels were great, and I had even grown 1/4 of an inch. We headed back to my exam room and while I waited for someone to come in the room to visit with me, I texted my parents, Karli, and Jason telling them of the wonderful news and praising God for His goodness.
I wasn’t waiting long when my respiratory therapist came to get me to do my lung function test. I sat down in what I like to call “the giant breathing box” and waited for her to get the test set up. While we waited, she asked if anything new was going on in my life. So, of course I immediately held up my left hand to show her my engagement ring! She gasped as she grabbed my hand and she was so excited that the other respiratory therapists in the office had to come and see what the fuss was about. Everyone thought the ring was gorgeous and I was bombarded with questions about the wedding. When the interview was over, I performed my PFT and scored 89% consistently for all three tries. My lungs were too tired to do it again, so I stuck with that number. Unfortunately, it was down from the last visit which was 94%, but I didn’t even care because I was so happy about my weight. I thought it might even have something to do with getting my PEG tube a month ago.
Rachel, my dietitian (whom I fondly refer to as my favorite person), came to see me next with the biggest smile on her face. She was SO happy that I was gaining weight. We talked about the last month with the tube and we were in the middle of the conversation when she noticed my ring. She jumped up and said, “YOU’RE ENGAGED!” I laughed and held out my hand for her to see the ring. So we talked about that before getting back on track.
She asked about my appetite and was so glad to hear that I have been able to increase my feeds on my own and still have an appetite for breakfast. I told her that I’m definitely not as hungry as I used to be at meals, but that I eat literally all throughout the day, which I believe has also played a role in my weight gain. She was also happy to hear that I knew when to increase my enzymes before/after feeds and that I was doing it consistently (I haven’t missed yet).
When she left, word got out that I had a “beautiful rock” (said my social worker) on my finger and so I was constantly getting visitors to see my ring. Dr. Burke and Cyndy (his PA) came in and they too wanted to see the ring first thing. Cyndy commented that it was the most beautiful ring she had ever seen and she just really loved it.
The first thing Dr. Burke congratulated me on (after getting engaged of course) was my weight gain. He asked how I had been feeling since my lung function was down, but I told him I had been doing great and feeling wonderful. I mentioned that I actually thought my lung function would have been better because I had been feeling so good. Cyndy pointed out though that at my last visit I had been on VFEND (for the mold growing in my lungs) and that had helped with the lung function. But since my liver function had decreased from the VFEND, I had to stop taking it after that appointment and that is most likely why my lung function is down now. Ugh. So unfortunate. She also said that my sputum culture from the last visit showed that I was not growing the mold anymore, so we needed to see if it had come back since not being on the VFEND.
In Lubbock just a few weeks ago, I had orders to get blood drawn to send to Cook’s to see how my liver was doing. Cyndy said the results showed they were now almost back to normal after being three times what they should have been! (I can’t remember what the numbers were, but my eyes bulged when she told me).
We then got on the discussion of Orkambi, the newest drug for those with the double delta F08 mutation (Me!!) on the CFTR gene. Originally, I couldn’t take this drug with VFEND, so we were going to wait until I was off of it. So obviously, the time has come! This drug works specifically on the underlying cause of CF (the gene), and has been shown to increase lung function. I would have to take it twice a day, each time with at least 20 grams of fat. I figured that is one avocado or a Carnation Instant Breakfast packet with two cups of milk (this is what I normally have for breakfast anyway). So I have officially signed the papers to start looking into getting this $300,000 drug. If everything goes right, I should have it within a couple of months! Dr. Burke left after talking to me about having kids in the future and what that would look like for me, but that’s a whole different blog post 🙂
Carrie, one of the nurses that I talk to frequently came in to see me last. She gave me information on Orkambi, swabbed my throat for my sputum culture, and told me that I need to bring Jason to the next visit so he can see this part of my life AND so my care team can approve of him (haha!). I told her I would be more than happy to bring him!
I then went to the lab to have blood drawn. Throughout my appointment, I had had 3 Take5 candy bars (if you haven’t had one, go get one now), a water bottle, and half of a gatorade, so I felt prepared. The phlebotomist did get it the first stick, but my stupid blood started clotting in the line so she had to stick me again. But she got enough to re-do my liver function test and to do a genetic screening so the Orkambi people can have proof that I have the double delta F08 mutation.
I left the hospital in a great mood. I am so thankful that I have a care team that cares for me in such a personal way that they literally jump up and down when I get engaged or when I gain ten pounds. I am so excited to go back December 9th to not only see how much weight I will have gained, but to introduce Jason to everyone and let him see exactly what these appointments look like for me. God is SO good, y’all!!