Living Life Well-One Breath at a Time

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Yesterday was my most recent clinic visit at Cook Children’s in Fort Worth. I wasn’t nervous going into this appointment at all, which is rare for me. If you read my last blog post, you know that I will now have labs drawn every 3-4 months (every visit) to make sure my liver is not damaged due to the VFEND medication I am taking for the mold I am growing in my lungs. You also know that I have done a lot of research on tube feeds (g-tubes/buttons specifically) because I know that I have not been gaining weight even though I have tried so hard the past three months. But I always like to give the full picture, so here is how my day went yesterday!

I woke up early in order to due my breathing treatments, take my antibiotics, and eat a good breakfast so I could have a good blood draw (and maybe magically gain a couple of pounds). We left for Cooks in order to have a DEXA scan done at 11 am. This is done about every 5 years to check my bone density. CF patients are more at risk for things like osteoporosis and osteopenia due to the malabsorption of vitamin D that we so commonly have . This took about 15 minutes, so we headed out to find some lunch before my clinic appointment at 1 pm.

We ended up eating lunch on the patio of a Potbelly sandwich shop in downtown Fort Worth. Sure, it was 102 degrees, but it was so beautiful (and what a great way to get some vitamin D!). I enjoyed eating a good lunch with my mom and sister before jamming to Taylor Swift’s “1989” CD (helps me breathe better when I sing) on our way back to the hospital.

The check-in procedure was a little different this time as they had me fill out a 3-page questionnaire on how I’ve been doing, medications I’m on and why, allergies, stools, and several other items. Now, this may be too much information, but I’ve always said I’m going to be very real with y’all. So you might want to skip this part, but it doesn’t bother me.

When I started the VFEND mid-June, I became constipated. I had a CAT scan done a week and half or so after being on it and it showed that I was as well. So that has continued even though I eat plenty of fiber and have been drinking more water and fluids more than I normally do. So that was a concern I had. (Not so bad, right?) I also mentioned in the questionnaire that I wanted to talk to the dietitian about a g-tube and probiotics for my gut health.

Pretty soon I was called back. First thing’s first, vitals. My oxygen was 98%, no fever, blood pressure and heart rate were normal and unfortunately I hadn’t gotten any taller. I also weighed about 100.75 when I had weighed 102, almost 103 pounds at my previous visit. So I knew how the tube feeding conversation would go at that point. At least I was prepared!

I was then shown to my room where my new nurse swabbed my throat for my sputum culture test. (She turned out to be a fellow Red Raider Alumn so we had a good conversation topic!) Not long after, I was taken to do my pulmonary lung function test (PFT). I was feeling pretty good about it, and sure enough, my highest was 94% when my it was 95% at my last visit. This was really good because lung function and weight normally go hand-in-hand. Therefore, since I had lost weight you would expect my lung function to have declined markedly as well. So I was very happy with this!

After waiting for almost an hour, Rachel, my dietitian (who was super sweet enough to help me with my case study mentioned in my previous post), came in to see me. She immediately asked if I was done with summer classes yet and if I had done well on my case study. I told her I’m finishing class this week and I’m still waiting on a grade for the case study! We then jumped into what my number one concern was-the g-tube. I told her I had been doing everything possible to add more calories to my meals, eating breakfast regularly, drinking a carnation instant breakfast supplement once daily, and taking my enzymes/fat soluble vitamins more regularly-yet, I had still lost weight. I told her how frustrating this was for me and that I had done so much research about g-tubes, especially with my case study, so I was rethinking how I felt about them. I also told her that with this next year and a half coming up, it will be filled with intense grad school classes, my teaching assistant position, as well as a stressful internship-not to mention just trying to have a life. The last thing I need to be worrying out is how I’m going to get enough calories in the day. I know myself, and I will push myself in order to be the best in whatever I do-no matter what.

At this point, my mom walked into the room. Normally I go into appointments by myself now since I’m an adult, but my nurse went to get her because she thought she should be involved in the conversation about the g-tube. I was thankful 🙂

Anyway, my dietitian said she could see how hard I push myself and she thought having a g-tube was the right decision and thought I was being smart and proactive. I would hate to start my internship and get too sick to finish it because I was pushing myself too hard and not eating enough because I would be stressed out all of the time! Of course, having one doesn’t mean I can slack off from eating, it just means less pressure to eat so much during the day.

Rachel mentioned that Dr. Burke (my doctor) preferred to have his patients on J-tubes instead of G-tubes. This means the tube would go into the jejunum (the lower part of the small intestine), instead of the stomach. This would mean less risk of acid reflux, which is apparently common in CF patients. I told her I haven’t ever taken any kind of Proton Pump Inhibitor (medication for reflux) and she was surprised. So she thinks I would be able to handle the G-tube well and it wouldn’t be a problem, I would just have to tell Dr. Burke what I wanted.

Source: medicalhomeportal.org

She continued on to ask about what I normally eat, and when I told her, she complimented me on eating a lot of fattier (yet healthier) items. I told her about my stools and she said she would talk to Cindy, my PA, about that being a side effect of the VFEND. She prescribed that I take a generic probiotic for gut health and said that would be fine. She left saying that if I ever had any more questions about school then I was more than welcome to call her again. I hope to be as great of a dietitian as she is to me 🙂

Carrie, yet another nurse came and asked if we knew where my genetic testing was done when I was first diagnosed with CF at 11 months. My mom and I both looked at each other, because, that was a long time ago. Apparently, the results never made it to Cook’s, even when I was going there when I was younger. It hasn’t been an issue, but with the release of the new drug Orkambi, insurance companies are requiring to see proof that a patient has the double delta F508 mutation in order to qualify to take the gene-therapy drug. So our job is to hopefully find that document somewhere so that I do not have to get the test again.

Soon after, Cindy, my PA, entered the room with Johnathan, a PA that was joining the team and just shadowing her for a while. She immediately commented that I hadn’t been sick at all on VFEND and that my PFT had stayed relatively the same even though my weight had decreased. So I’m staying on the VFEND because it seems to be doing a great deal of good for me! Normal treatment is 3-6 months so I will finish that up.

We then talked about the g-tube placement and kind of went into detail on that. Dr. Burke was out of the office for the week, so she will talk to him in the early part of next week and talk him into letting me have the G-tube that I want instead of the J-tube. She told me not to worry about it, so I won’t. She mentioned that they have a great gastroenterologist at Cook’s that they like and trust, but that they have never put a button (what I want) in an adult patient before-it’s always been pedi patients because the adults with tube feeds are normally older patients who get an actual tube, which sticks out, while a button does not. The older patients normally do not care that they have the tube, but for someone younger and more active, I definitely care! So that’s why she is recommending I find a pedi gastroenterologist to do the placement. Currently, we are searching for one in Lubbock so I can be closer to home, friends, and family. As soon as everything is approved by Dr. Burke and the insurance company, I will be getting the button placed and can start gaining age appropriate weight!

The last thing Cindy and I talked about was the new Orkambi drug that works specifically on my double gene mutation. She told me that it has been shown to have the most improvement on patients with a lung function of 35-90% and frequent lung exacerbations. So even though I have had some frequent exacerbations, my lung function has stayed above 90%, so she doesn’t think it would help me very much. Plus, it’s an almost $300,000 drug (my jaw dropped when she said this) and I always get the weirdest side effects from any changes in my treatment regimen. She also mentioned that I cannot take Orkambi while I’m on VFEND, so it looks like Orkambi will have to wait and we can revisit it in the future if needed!

And then it was time for my blood draw. I checked out and made an appointment with the clinic in 6 weeks to check my liver function again and see if the VFEND needed to be discontinued or not. Then I headed to the lab!

I drank a water bottle, a bottle of gatorade, and ate a Take 5 bar during my appointment so that my blood would flow well. Before they called my name, I was agitating the veins in my hand to get them to perk up. The phlebotomist actually listened to me when I told her I was a very hard stick my veins like to roll, and they always get blood from my hands and never my arms. So she agitated my veins a little more, found a vein, stuck me once, and I was done in 5 minutes. It was a miracle and I walked out of that room praising Jesus! I made sure to tell the phlebotomist that she was really good at her job. Then I was on my way back to Lubbock!

Overall, I feel really good about this appointment. I got a lot of good results and everyone is on board with me getting a g-tube. I am so thankful that I had the opportunity to research CF patients with g-tubes before going into this appointment so that I was prepared and ready for what was to come. It still feels a little surreal because it’s a couple of weeks down the road most likely. I’m excited, but I’m also really scared. I’ve never done this before and it’s going to be hard getting used to it. Other CFers say the first two weeks are the worst because it hurts to cough, laugh, or sneeze until the site heals. I will need to rest a lot, I’m sure. The Lord gave me so much peace about this decision, even before today, so I know this is the right thing for me. But as you think about it, please pray for me. Pray that my fears and anxieties would be put to rest and that we would find a pediatric gastroenterologist willing to place a g-tube in Lubbock. Also, please pray that our insurance will not be difficult about this because they have a tendency to be. Pray that placement will go well, whenever it comes, and that the feeds will be tolerated. Pray that the knowledge that I have from my studies will be put to good use and that I will be able to learn from this experience in more ways than one! The Lord is so good to me, and I know that having this feeding tube will be a huge blessing. I’m living in anticipation of what is to come!

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