If you read my last blog post you would have read that this week was a busy week. Maria had her appointment Tuesday for her liver doctor. She went to the liver dr (gastroenteroligst) to see why her liver enzymes have been elevated since this past December. Then on Wednesday it was my turn to head up to CF clinic for the lovely long appointment and drive.
Here is how Maria's appointment went. Overall, it was a good dr visit. We met with the dr and he was very nice and explained everything very well. The course of action and some reasons and things to rule out as to why Maria's liver enzymes have been so elevated. In fact, they are 3 times what they should be. I asked if it could have anything to do with not eating enough and breastfeeding or a rapid loss of weight. He said no there is no relationship there. So he went over a few things with us and said he wanted her to have a blood test to check for a couple genetic diseases. They were celiac disease, alpha 1, and lupus. Maria did have about 10 blood tests run since December but he wanted to test fully for these disease to rule them out. Once the blood results come back then she will have an MRI of her liver if the blood results do not show any diseases. Then if the MRI looks good he may recommend a liver biopsy to check for what is called "fatty liver". But Maria does not fit into the "fatty liver" diagnosis as she is not overweight, no diabetes, and does not have high blood pressure. If nothing is revealed from any of these tests she may be in this doctor's category of about 20 patients he has seen that have unexplained elevated liver enzymes. As you know from our past few years we could definitely fit into that 20 patient list because all the rare things happen to this family!!! The doctor did reassure us that he did not think it was anything serious at this time which is really good. So we left the office feeling I would say overall pretty good. We of course shared our story about our fertility process, labor experience, and our handicapped dog, and my CF. Both the doctor and nurse said we have a lot on our plate. Well, welcome to our life we take the challenges and conquer them. That is how life is meant to be lived!
But here is something interesting that Maria and I were talking about today. Remember that clip that was on Maria's fallopian tube? She had it placed there to prevent a future birth as she is a CF carrier if you are new to this blog. The only way we would conceive is through IVF and PGD, again for any new readers. Well during our labor experience that clip was not on the fallopian tube anymore in fact the OBGYN had said it was next to the tube attached to veins. But as we all know you can google anything and read stories. Maria googled about these clips and them floating around in your body if they become detached and read that someone had their clip attach to their lung!!! So we forgot to mention this at the appointment but could it be a possibility that the clip is attached to her liver? That we do not know. I would say most likely no but its worth to question this. Maybe if she does have the MRI it could reveal if the clip is attached to her liver. More to follow on this when Maria talks to the doctor next week.
Here is the summary of my CF appointment. As always these are very long days because I work till about 2 pm on clinic days and then head home to pick up either Maria/my mom or both of them for my 4 pm appointment. Then we make the hour drive to clinic. We got home at 7 pm so it was a VERY LONG day!
I love how I have Maria/my mom or both of them to go with to my appointments as it keeps the anxiety level low for me. The appointment was going to be a long one because as I said I am participating in a research study. To summarize the research study its dealing with my PFT and approximately 10 questionnaires about my health and mental status. They are looking to see if there is any correlation between mental status and how you feel when it comes to PFTs. The research study adds on a good 30 mins to the appointment and I have to do a PFT every time I am at clinic now. There will be times when I only do PFTs every other appointment. In fact today, I found why this is and I never knew the answer. I guess its required that I do one at least once every 3 months. Now it makes sense because I usually go to clinic every 2 months but the time between my last visit and today was 3 months.
As always I try to mentally prepare myself for clinic and I usually over think the appointment because they have usually been really really good appointments. Today, I had another really good appointment. The doctor said that these lungs of mine sounded very clear which he says every time. I was able to cough up some nice mucus for him. In fact, I was worried that I wasn't going to be able cough anything up as my cough was not very productive today. I always want to have mucus they can test in the lab to make sure I am not culturing any new bacteria.
As I mentioned, my cough was not very productive and I actually talked to the doctor about adding hypertonic saline (hts) in the evening. Right now my evening vest treatment is not very productive as I do not cough much mucus up at all and I think I can add some hts to help increase the effectiveness of my evening treatment. I have tried adding the hts 7% in the evening but all that does is make me cough like crazy during my sleep. So I asked about hts 3% as I have read that this is not as potent and is more tolerable than 7%. I love using hts 7% in the morning but I want to move that mucus out in the evening. The doctor said it was fine to try the 3% hts and I will be getting a perscirption for that soon.
Now on to the PFT test to see how my lung function has been. I have to say I made an error in my February CF clinic post about my FEV 1. It was really at 67 and not the 70 that I had blogged. I asked the respiratory tech before we started what my last FEV1 was and she said 67. I thought it was 70 but anyway she looked at my chart and I have been at a baseline of FEV1 of anywhere from 65-74 these past 2 years. I guess I am pretty consistent which is good. The respiratory tech was really nice and she coached me which I love. I love when a woman is screaming and shouting at me who doesn't!!! Haha! But I honestly still like to be told take a deep breath in and blast it out etc etc. For my first attempt I blew a 72 which I was pretty pleased with since three months ago my FEV1 was at 67. Second attempt I blew a 71 and for the third attempt I blew a 72. So today I got credit for a FEV1 of 72 and a FVC of 96. I felt pretty good with those results since they went up by 5 points or almost a 10% change from 67.
So what have I been doing differently these past 3 monts. Well I honestly have to say I think my PFT has went up because of my compliance to my airway treatments. I have also been doing my morning treatment a little longer than usual. I am now going on a nice long streak of NOT missing a single airway treatment for almost 4 months now! Woo hoo!!! But as I always say a PFT is just a number and WE focus way to much on this in the CF community. Yes, its good to know how are lung function is but it is still only one piece of the CF clinic puzzle.
I made sure to ask a few more questions before I left. I always ask what was in my last culture. Doctor told me that staph and acromobacter is living in my lungs. That "A" word bacteria has been in there for a while but seems to not impact me. The biggest thing is that I have been PA free for well over a year now. I believe that the "A" bacteria has taken over and is more potent than PA. So I hope the PA stays away forever!!!!!
Another question that I did ask about was Kalydeco and my gene. As I have blogged about recently, there is a study in Denver that is looking at splicing mutations and various other genes and the impact of Kalydeco. My doctor really had no idea about the study or if my gene is even considerde a splicing mutation. I have 3849+10kb CT mutation and according to Vertex that is a splicing defect gene. But he did mention that our clinic is studying Kalydeco on heteros who have at least one copy of DF508 and other mutations. He did check with the research team to see if I could participate but my gene combo is not on the list BUMMER!!! But I know in my heart and on Facebook that Kalydeco will work for me. It is working for my exact gene combo its only a matter of time before it will be approved for more genes.
Now, to summarize both these dr appointments. Maria's appointment went pretty good. The doctor was very nice and answered all the questions we had and the biggest thing is he has a plan of action. We are just waiting on the blood results before we move to more testing. The MRI and liver biopsy could be down the road. We will see but I am glad we found a doctor who is compassionate and caring. My CF clinic went well. PFTs are up from FEV1 67 to 72. The lungs sounded good and I will most likely give 3% hts a try in the evening soon. So that is a wrap!!!