Breathing Easy

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I always look forward to writing a new post to share with my readers, but I am especially excited about this one. This past Wednesday was my regular 3-month check up with my CF doctor in Fort Worth. As has been the case for the last couple of appointments, I was excited to go so I could see how much weight I had gained- thanks to Joey and my feeding tube!

As some of you may already know, I got my button replaced about a month ago due to the high volume of formula that my previous button was leaking out when I fed. I am happy to report that after the very successful operation and the two-day recovery period (SO much better than last time!!), I am feeding every night without a single drop of formula leaking out. Praise Jesus!

Recently, I have also increased my feeds to 3 cans per night (instead of 2.5 cans) to give me 1,500 calories per night. This was just easier for me when preparing the feeds each night, and I still eat throughout the day, drink two Carnation Instant Breakfast’s each day, and have a pretty good appetite! Having a major in Nutritional Sciences and Dietetics and having an in-depth knowledge of nutritional support (tube feeding) really comes in handy because I can increase or decrease my feeds without consulting my doctor or dietitian, and I am able to tell if I am tolerating my feeds or not.

So anyway, back to the day of my appointment.

It had been decided at my last appointment by my CF Coordinator, that now that I was engaged, my fiance needed to come to my appointments and be involved in my health care since he would be my primary caregiver in the (very) near future. Of course, I was all for it! So Jason woke up early Wednesday morning and we headed to Fort Worth-just the two of us.

Jason and I always enjoy road trips together, but this was the first one we had been on where we were going to a much bigger area than Lubbock or any surrounding towns located in the “flat lands”. He was nervous about driving, and I was thankful he was driving so I didn’t have to be nervous about driving! Ha. But the trip down there wasn’t bad.

When we finally made it to downtown Fort Worth, we decided to stop at a Potbelly’s sandwich shop and eat lunch before heading to Cook Children’s Medical Center. Well thanks to our nerves and my not-so-great driving directions of where to park, we ended up turning down a one-way street. After a brief moment (or two) of panicking, we made a quick u-turn and eventually found a place to park where we had to pay $10. Worth it? I don’t think so, but at this point we had no other option. Jason said, “We definitely won’t be moving here.” I agreed.

We ate lunch on the patio of Potbelly’s surrounded by tall buildings and clear skies. I was reminded of being in New York with Karli; it was so pretty.

After lunch, we found our way to Cook Children’s. After checking in and grabbing a mask to keep from breathing germs on others, we headed upstairs to the Pulmonary floor.

The first thing they did was weigh me. I stepped on that scale and BEHOLD. 111 POUNDS.

I was overjoyed. It was the most I’ve ever weighed in my entire life.

At my previous visit, I was weighing in at 106 pounds, which meant not only had I gained 5 pounds since my last visit, but that I had gained a grand total of 15 pounds with my button! No regrets. I love being able to NOT see my ribs when I look at myself in the mirror. You have no idea.

My oxygen was great, as well as my blood pressure.

I was shown to a room where Jason and I waited for just a few minutes before my nurse, Candace, came into the room. She asked the standard questions about if I had changed my medications, how my feedings were going, and how I was doing on Orkambi. It wasn’t long before it was time to do my pulmonary lung function test (PFT). I introduced Jason to the respiratory therapist and explained to her that he was my fiance and had never been exposed to what I do at clinic. So I performed the test, and I got a whopping 98% on my third try! It was up from the 89% that it was last time, and was, in fact, the highest it had been all year. I wanted to cry.

Jason and I went back to my exam room after that and we waited again. But soon, my dietitian, Rachel, entered the room. Again, I introduced her to Jason and she started off by asking how wedding planning was going and how I was doing with school. I told her how I had just finished finals the day before and what classes I had taken this semester. We then talked about how she was going back to school and how her life was. We proceeded to talk about my feedings and why I had decided to up my intake of formula and enzymes. She was really happy with my weight gain and told me she wants me to be at about 120 pounds at my next visit. It’s a tall order, but I can do it. Before leaving the room, I showed Rachel a picture of my wedding dress and she also asked to see mine and Jason’s engagement pictures. I LOVE how personal my care team is. It is one of the biggest blessings in my life, and I honestly hope to care for my patients as much as Rachel cares for hers.

Not too long after Rachel left, the CF Research Coordinator (whom I had never met) came to speak with me. She told me about a research study that they were enrolling patients for and she said I might qualify for it. I won’t go into all of the boring details, but basically, I qualified for it because I am taking Orkambi. However, when I told her what my lung function was, she said I probably wouldn’t qualify for it anymore because 98% is too high. That is okay with me!

Unfortunately, Candace came back in later to give me my flu shot. I’m actually against flu shots and I don’t believe that they truly help prevent the flu, but it was necessary for me to have it this year for my internship starting in 2016. Booooo for having to get the flu shot and for my left arm hurting like crazy for the next two days. But also, WOOHOO because I start my internship next  year!

Last but not least, I finally saw Dr. Burk and his PA, Cindy. These two really crack me up just in how they interact with one another. Dr. Burk is an older man with a very dry sense of humor, and Cindy is there to encourage me and keep Dr. Burk in line. No joke.

Okay, so here is where the post gets pretty personal. I’m going to give you some background information so that you understand where the upcoming conversation with Dr. Burk came from.

As I have already mentioned, I am taking a medication called Orkambi that is considered a “gene therapy” med. This is important because it works directly on the gene mutation that I have in order to improve lung function. Now, research has shown that it works best with those who have a poor lung function, or a lung function worse than the one I have. However, I chose to try it and see if it could help me. Honestly, I haven’t seen too much of a difference, and I’ve had this red rash on my arm and neck since starting Orkambi and it’s just really annoying.

I was also told, since I’m getting married next year, that I can only use a copper IUD or condoms as birth control because Orkambi will decrease the effectiveness of any hormonal birth control options. Jason and I had talked about it before I started Orkambi and we were okay with that decision. I was also told I could not take Orkambi while pregnant, but we aren’t planning on having any babies any time soon!

So after being on Orkambi about three months and not seeing much of a change, I decided to talk to Dr. Burk about stopping Orkambi so I could take birth control pills when it is closer to the time of Jason and I getting married. It was just a personal preference for me and something that both my mom and Jason thought would be a good idea. Plus, the less medications that I’m on, the better!

So I told Dr. Burk what I wanted to do and why, and he and Cindy were all for it. Cindy said I was being very proactive by talking about this now since we still have several months before the wedding. Dr. Burk wants me to finish the 3 weeks of Orkambi that I have I left, and then I can go off of it and see how it affects me without being on birth control. If all goes well and I am able to maintain my lung function, I can stay off of Orkambi as long as I don’t need it in the future. I told Cindy, “My mom will be so proud of me!” Dr. Burk laughed at that, and said he thought it was a good decision as well.

We also talked about getting Jason tested to see if he is a carrier of any of the CF genes. With CF being a genetic disorder, there is a possibility that our kids could have CF if I end up passing on both of my genes to them. There is an even higher possibility of passing on CF to our children if Jason is a carrier as well. Of course, having a child with CF is not a deal breaker for either one of us. Children are a blessing and a gift from God- no matter what! I told Jason that it is completely up to him if he wants to get tested-it does not matter to me either way. So we will see what happens! It’s not a priority for us at the moment.

Dr. Burk then listened to my lungs, which sounded great, and I was allowed to leave and go to the lab after my throat was swabbed to see what’s growing in my lungs.

We waited almost an hour before I was finally shown to a room to get my labs done. Throughout my appointment, I drank a gatorade, a bottle of water, and I had eaten a chocolate bar, so I was ready for the blood draw. Not to mention, I had also agitated the veins in my hands quite a bit in the waiting room.

I gave the phlebotomist my spiel about my tiny veins that like to roll and how “you’re only going to get blood from my hands”. She nodded, stuck the needle in my hand, and had all the blood she needed within 30 seconds. I was amazed. Whenever anyone gets my blood that quick AND manages not to leave a bruise or make me cry, I make sure to tell them how great they are at their job. Looking at my hand now, you can’t even tell I had my blood drawn. For my labs this time, they are checking vitamin levels, my liver enzymes (Orkambi could lead to liver damage, so this has to be monitored every time), and my titers for my internship. So lot’s of blood!

After being at Cook’s from 1:30 to almost 5, Jason and I were ready to go home. So we grabbed a burger from In-n-Out and headed back to Lubbock!

It was so great to finally be able to share this experience with the one person I’ve always wanted to share it with. He got to see so much of what I go through each time I go to clinic. I got to talk to him about my concerns-not that I haven’t been able to do this before, but it was just different this time. I just breathe a little easier when I have Jason there with me. I am looking forward to going back with him in March! I am so blessed to have a future husband that cares for me so deeply and will sacrifice a couple of extra hours of sleep just to drive me to a doctor appointment in a different city. I hope every CFer finds someone like my Jason- I thank Jesus for him every day.

P.S. I have asked Jason to do a guest post on my blog talking about his experience at Cook’s with me. So be looking for that coming shortly! 🙂

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