Here is my update on my recent visit to the CF clinic. If you have ever talked with me on social media or in person you know I have a lot to say and this usually means I talk a lot at CF clinic. I asked tons of questions about what’s going on today with all this Ebola talk, the contaminated Sam’s Club wipes with B.Cepacia, enterovirus, kalydeco and vertex, and of course my health.
Dr. – Ebola is a very serious thing. The media and news is really downplaying how serious this is and can be. He believes that this type of Ebola virus has mutated as it has infected thousands in Africa. The more people the virus infects the more chances that it has mutated along the duration of this outbreak. When a virus mutates it develops different strains. If they develop a vaccination it may be tough to include all the strains. He said the virus is NOT deadly but you can die from it. Oxymoron right! But I guess what he is referring to is that you can survive if you get Ebola hence the American who has survived.
Me – So what happens if a CF patient were to contract Ebola? Dr said we should all pray. Basically, Ebola would be pretty awful for any of us with compromised immune systems. I asked if we should be really scared or anything we should do. He did suggest to stockpile food and water. Uhm what? He said if there was a pandemic it would be good to have on hand. Should we live in a bubble? He really didn’t say much but said basically hibernation is always an option.
Sam’s club wipes & B. Cepacia –
Dr. – B. Cepacia has various strains and NOT all will impact a CF individual meaning cause them to get sick and cause rapid decline. He said he sent an email yesterday to either CDC or some medical professionals close to the recall. He wants to know what strain of B. Cepacia was found so he can advise patients if there is cause for concern. He told me not to wipe my mouth with these wipes. Thankfully, I have never wiped my mouth with a baby wipe but we have purchased these wipes from Sam’s Club.
Me- How long would it take for someone to have B. Cepaciaonce exposed to it? He said it may not show up in a culture until after 18 months of first initial exposure. UHM what the heck!!! Should I be concerned? He said well there really isn’t anything that can be done at this point. Bacteria is every where. B.Cepacia can be found in the soil, countertops, sinks, etc. Should someone with CF get a bronchoscopy if they have used these wipes? He said that would NOT advise doing this. You may have to do a bronchoscopy every week if you really wanted to find B. cepacia after first initial exposure. So basically don’t lose any sleep over this event at this time.
Here is a link about B. Cepacia in CF patients.http://cysticfibrosis.about.com/od/relateddiseases/tp/bcepacia.htm
Dr. – Entervirus has yet to be found in any adults that he knows of. He said he has never seen this type of Enterovirus in his career.
Me- I didn’t really ask any questions on this topic. I am not sure if my CF clinic has seen a CFer with enterovirus this year.
Kalydeco & Vertex –
Dr. – The future is very promising for many individuals who have at least one DF508 gene. Vertex believes they will be able to treat at least everyone who has one copy of DF508 or a residual mutation or gating mutation. He believes that R117H will be added to the kalydeco label very soon.
Me – I told dr about the potential of getting on the kalydeco andvx 661 trial. He said that Vertex has been so successful with getting FDA approval on things because they work very closely with FDA to see what they want as outcome measurements in their studies.
My Health –
It was my first appointment where my clinic moved to electronic medical records and I have to tell you I truly hated the patient doctor interaction. The appointment lasted forever it seemed like. I really feel that patients will be the ones who are ultimately hurt the most by this. I honestly could have filled out the questions at home and then came to the visit. It would have saved about 30 minutes.
My weight is always good and my BMI is in the normal range. Woo hoo. I am so thankful I do not have to worry about the struggles of gaining weight. Many of my CF friends battle this every single day.
My lungs sounded good as usual. I don’t ever get those rattles or crackles at least not at clinic. I will sometimes after airway treatment but its just the junk moving around in my lungs.
My PFTs, well they were not the best but also not the worst. I am at my baseline but lower end of baseline. I blew a FEV1 68 last visit I was at FEV1 73. My baseline is anywhere from high 60s to mid 70s. Normal non CF lungs are 80-120. My doctor really dislikes this test just like me. There is way to much performance skills and anxiety involved in this test. He usually concentrates on FVC number which mine was 91 yesterday. He claims FVC is the measurement of how you actually feel. Anything above 80 is normal. I guess that means I should feel good. I do actually feel pretty good but wish my FEV1 was a little higher. When it comes to PFTs I always struggle with anxiety. But from what my dr tells me they are really looking at using a breath test instead of a PFT to measure lung performance. This breath test is more NON performance based. I have no idea when this will ever come to clinic but I believe Vertex is using breath test for lung performance in some clinical studies.
So what am I going to do to get my PFTs to my upper end of baseline? I experienced my first ever lung bleed about a month ago. It truly was a scary event for me!! I called my dr and we discussed what I have been doing before lung bleed. If anyone has ever read my blog or followed me on Facebook you will recall I am big on using 4 vials of HTS a day at 7%. He suggested I cut back on the usage of my HTS. I dialed it back down to only 2 vials a day but I could tell the difference in my breathing and my lung junk. I just never felt fully clear in the last month doing only 2 vials of HTS a day.
In the past week I have increased back to 4 vials of HTS a day. I know it’s going to take time to get back to where I was before blowing a FEV1 of 74 and feeling clear through the day. But I can tell you I already feel the difference in my lungs. I feel so much more open and less junky all day long. I am just hoping that 4 vials of HTS is not the culprit in my lung bleed. I have decided I am going to give my lungs a break every few days and go to 2 vials to see if that helps at all.
I will also be doing more affloing in the car. Affloing meaning using my Afflovest more! I cut back on that as well during my lung bleed to give my lungs a rest. I have been back to using myafflovest 4 times a day. I LOVE the portability of this device. After a week of doing my afflo 4 times a day I can feel the difference in my lungs. I just love this device over my hil rom.
Overall it was a pretty good visit with my CF clinic. I will be going back to clinic right before Christmas so I hope for an early Christmas gift of increased PFTs.