Symdeko Update

I have been on Symdeko for 10 weeks as of today!! 10 weeks!! I meant to write an update at 4 weeks, but time got away from me as it often does during the summer months.

The background

I feel like I should give some background information that might be important for someone looking for similar experiences. I should mention I was on Orkambi for a few years prior to making the switch. Starting Orkambi was brutal to say the least and it took about 3 months of Orkambi before I felt human again. After I adjusted I feel like I gained stability, but saw no improvement in my pfts. In fact, I felt like my baseline was a bit lower after starting, but because I was more stable I felt like it was worth the tradeoff. At the very end of my Orkambi experience I was hospitalized with my lowest lung function 29% (although I number I had seen before). 

Two weeks after ending IVs I started Symdeko. This made determining how well Symdeko was working tricky because I wasn't sure how much Symdeko was responsible for and how much was the result of being freshly off IVs. 

Starting Symdeko

The first week I noticed I had a bit of a headache, but nothing unmanageable. I also noticed my digestive system was off. I couldn't really pinpoint what was off except I went from being extremely regular and feeling fine as long as I always took my enzymes to having complete inconsistency with my bowel movements. This lasted maybe a week (and potentially could have been related to being off IVs recently. Although that usually makes me have loose stools which wasn't really the issue here).

The First Month

The first month I recorded my PFTs on a calendar (with a home meter) and I watched them steadily climb. 1.13, 1.19, 1.20, 1.25, 1.28. Part of me felt it was too good to be true and was a fluke with my meter and part of me was so hopeful that this was a sign that Symdeko was working. I did have some streaking of blood in my sputum around week 3 which is really rare for me and was somewhat concerning, but it resolved quickly.

First Clinic

My first clinic since Symdeko was around the 8 week mark. This was 10 weeks after finishing IVs so I was no longer "fresh off IVs" and so I felt at this point my pfts would be a pretty good indication of how well Symdeko was treating me vs a temporary high due to IVs. 

Guess what? My PFTs hit 40% for the first time in YEARS! 40% has been my goal for so many years now and I had 100% given up on every hitting the 40s again. But 2 weeks ago at clinic I totally hit 40%!!! It felt so surreal and was all the proof I needed that this drug was working!!

And that isn't even the best news. I was at the park the other day and for the first time in a very long time I had an overwhelming desire to run. RUN? Yes, run!!! Because when your lungs are functioning at 20 or 30% the idea of running or even speed walking feels so impossible that the thought never even crosses your mind. But when your lungs feel amazing and clear and so much more open things that you never even conceived possible actually seem doable and show up on your radar. And so at my suggestion my daughter and I ran across the field at the park and then we ran all the way back. And you should have seen the look on Kaylee's face. This was something we never experienced together before (or at least in her memory) and she was soaking up every minutes with the biggest smile plastered across her face. That night when I tucked her in bed she said, "mom, it was fun running with you today." And it shattered my heart that I spend 6 years unable to run with her in the way a young mother should with her child and yet it filled me with hope that now I am capable of running along side my 6 year old through the park. It gave me hope that I will be around to run with this precious girl for many more years to come. 

Now I feel even more impatient for the triple combination to come out so that all my fellow CFers can experience better numbers and a run through the park. Because we all deserve that at the very least!

Now vs Then

This week my husband and I had a little get away. We went about an hour and a half Northeast to a historic old town to spend some time in nature just the two of us- child free! I was excited to get a little alone time with my husband before school starts and we thought of it as an early anniversary vacation (we have our 7 year wedding anniversary early next month). My only little concern was that we would 2,500 feet higher in elevation than we currently live (which is essentially sea level) and I was a little concerned with how my lung would handle the elevation. We figured we would give it a shot and if it was a little too high our hiking and nature weekend would end up being a laying around and relaxing weekend.

As my GPS notified us that our hotel was quickly approaching I realized that the car was going up a steep hill. As the car drove higher and higher I started to get worried. We live in a valley so our landscape is as flat as a landscape can be and I just wasn't sure if the hills in this town would make walking a challenge. We heard the town we were visiting wasn't known for good parking and that our best bet was to walk into town for restaurants. Would my lungs be able to handle these steep hills at a higher elevation? I figured there was nothing we could do about it at this point so we hoped for the best.

When we checked into our hotel we realized our room was on the second floor up a very steep and curvy set of stairs. Stairs still give me a touch of anxiety because for a long time I just physically could not climb a flight of stairs. When I was sick for a couple years I would have to climb stairs on all fours because I did not have the lung power to walk them like a normal person. Needless to say stairs bring back bad memories. Again, I was wondering how I was going to make it through this vacation.

All of these secret little fears ended up being fruitless. I handled the hills and the stairs like a champ! Sure, I may have been a little more winded than the normal person, but I also only have 1/3 of the lung function that a normal person has. The entire trip I did not need to stop and rest on the hills or the stairs and we walked a ton of hills! And even more amazing I survived a hike that was a straight downward hill that I would old never have even attempted a few years ago in fear that I would never make it back up the hill. Sure we walked back up the hill very slowly. But my husband was so patient and wouldn't let me get frustrated with how often I needed to rest. He even carried me on his back for a little to give me a rest (although I really didn't need the help it was a nice little rest).

As we were driving back home I couldn't help, but be in awe with my body. I was able to do so much and my CF hardly got in the way of our vacation (those darn treatments are always such a pain on vacation because we can't just get up and go) and I could hardly believe I was the same person I was a few years ago. It also made me realize once again lung function is just a number. I have been hovering in the low 30s for a few years now, but what I am able to do now vs a few years ago is extremely different! I have a feeling consistently swimming has helped condition my lungs and body so that I am able to much more with my little 33(ish)% now than I was even a year ago!

I know this newfound amazement may vanish with the next cold or exacerbation, but for now I am reveling in a body that has dealt with so very much and still seems to power through!

Whaaaat??

After three consecutive colds (after a winter of a million viruses) and a fun vacation that ended up leaving me more sleep deprived than a mother of a newborn I finally gave in and called clinic for meds. All the signs of infection were there: night cough, extra reactive airways, way too much mucus for one person to handle. My doctor wanted me to come in for PFTs before prescribing any antibiotics and so the weekend felt very long in the worst way possible.

The past several years I find clinic to be absolutely deflating. As a CFer I am the teacher's pet. I go to all my appointments, I do all my treatments (like seriously, I never miss mostly because if I do I am miserable and it just isn't worth it), I take all my pills 99% of the time (those dang vitamins are the ones that trip me up), I do everything they ask of me and yet I still have really horrible pfts that never seem to improve.

The frustrating aspect is that I often feel like clinic gets the worst of me. The last few times I went to clinic I was just getting over or just starting a cold. Of course, they also get me when I am sick and desperate for medication. The times I feel amazing and everything seems to be going well? Nope, never have a clinic appointment scheduled then. And so sometimes I feel like clinic has a skewed view of my health and here was just another "sick" pft to add to my list of subpar pfts.

When my respiratory therapist showed up, I was not all that excited to see him. He was in a rush and he wasn't his usual talkative self and I wasn't even able to check out my score before he pushed print on the pft machine, handed me the paper and left.

And guess what? Despite feeling bad, my pfts were up! What?! The last several clinics my pfts have gone up 1% each visit. Okay, I know, I know 1% is not clinically significant, but let me tell you something, it is significant to me!! Do you want to know why? My goal in life is to get back to 40% lung function (or as close to it as possible). When I went though a rough patch I fell to the 20s and even though I rebounded (and fell and rebounded a few times) I got stuck at 31% for a very long time. I tried everything to get past 31% and nothing worked, nothing!! Somehow the last few appointments, despite feeling bad, I have been creeping closer and closer to my goal. 31%, 32%, 33%, and today I was 34% even though I was coming in desperate for medication!! See how creeping up 1% at a time without sliding back down can be significant?

Maybe after this tune up I can gain another percentage point or two? Baby steps... baby steps...

Mysterious Exacerbation

For my own record keeping and to potentially help others that may find themselves in the same situation (even if it is just for the comfort in knowing others go through long bouts of struggle with CF too) I really wanted to be better about keeping this last (exhausting) exacerbation well documented. However, living with the complications of CF everyday can be mentally and physically exhausting and despite finding this blog as a way to release some of the frustration and thoughts that surround CF I often find that while I am in the thick of sickness taking up mental space to write about it can feel like too much. And so this is a quick breakdown of this last complicated exacerbation, which I might add, is not over.

 June 13^th: Despite feeling relatively well most of the summer I blew a disappointing 34% at clinic. Was given 2 weeks of Cipro.

July 5^th: Called to ask for stronger meds as Cipro wasn’t enough to make me feel better.

July 13^th: Started IV Zosyn in hopes of it doing the trick.

July 18^th: After 2 weeks of orals and one week of Zosyn my FEV1 dropped to 28%. Decided on doing 3 weeks of Zosyn total to hopefully kill off more pseudomonas.

July 25^th: Feeling better although not great, but my skin started to react to my port dressing. Started Prednisone to contain my severe contact dermatitis due to all adhesives.

July 6th: Went to the ER with a suspected clot. No clot was found!

August 1^st: Felt like death (virus? Cold?) and blew 31% in clinic. Decided that pseudo wasn’t the culprit to my sickness (it is sensitive to both Cipro and Zosyn) and tried treating other bugs that may not show up on my culture.

August 3^rd: Had an allergic reaction to Zosyn and woke up covered in hives.

August 12^th: Started Bactrum and Rifampin for 10 days. No progress

August 27^th: Started Levoquin and Minocycline and am currently crossing my fingers this cocktail works.

 After two months of antibiotics I still looking for the right antibiotics for what is causing my lung function decline, fatigue, and afternoon fevers that do seem to disappear while on antibiotics, but instantly come back when antibiotics end. CF can be such a frustrating disease and I hate when these exacerbations seem to turn into months rather than weeks. I am hoping that I can get things under control before cold and flu season begins. I have already started stocking up on hand sanitizer for this winter.

 

What Is This?

What is this? I had a good Dr appointment for the first time in what feels like forever?? Why yes, yes I did! So my appointment may not have been very good according to most peoples standards, but what can I say? My standards suck. So I am overjoyed to say that I am officially back in the 30s! I am hoping not to see my FEV1 in the 20s for well...forever, but that is probably asking too much so I will settle for 20 years or so. My numbers were barely hanging onto the 30s at 33%, but you know what? 33% is better than 32% and way better than anything in the 20s so I am not going to complain. I am hoping that my numbers will continue to creep up over the next year or so. I go back to the doctor in 6 weeks and my goal is 35%. Baby steps...

And guess what else? My weight, it rocked! Thank you, Megace! We decided I would stay on Megace until I hit 130lbs which will be interesting because besides being pregnant I have never really been heavier than 125. I may need to buy a new wardrobe!

Other good news (yes it just kept coming!!) Vit A, E, K, and D were all good. A1C was awesome! I was totally on a role...well besides the shitty lung function and all. Thanks to my appointment today I am in a pretty good mood tonight so excuse me while I celebrate with a double helping of Ensure Plus with a few tablespoons of coconut oil and 5 zenpep!

Clinic Updates

This is a quick update, but I had my 3 month appointment at the clinic today and wanted to report some good news. Here is the run down:

• weight is up. I am 5'4" and weight 125lbs. I was 123 last time.
• My O2 sats were 98%
• My FVC is now 97%. My FVC is always significantly higher than my FEV1 so I wasn't all that surprised.
• My FEV1 is now at a recent all time high of 57%

Never thought the day that I could even dream about 60%, but now I am not too far off. I really am still in shock. I know this disease has a mind of its own and that we really can't control our numbers, BUT I do believe swimming (and Cayston) is why my numbers have gone up almost 10% in the past 6 months after 6 years of averaging in the 40s.

We discussed baby making with the nutritionist and the Dr so I will update you on everything from the changes in vitamins, what the doc said about pregnancy with FEVs in the 50s, about trying to conceive and the hurtles that come along with CF and ttc. So exciting and intimidating! I am off to my sister's house, but I will fill in all those little details tomorrow.

Also I found my chart with my FEV history so I will be adding that sometime this week...finally!

Hard Work Pays Off

I am on cloud 9 right now! I just had clinic appointment and after increasing my medical routine from about 1 hour to almost 3 hours everyday my FEV1 lung function was...drum roll please... 54% I am seriously beyond words. I really never believed my FEV1 could get up that high after years of hovering around 49%. My FVC was in the 90s (can't remember what the exact number is at the moment) and my O2 was 98%. The most ironic part of it all is I called panicked because I was feeling so crummy and was worried I needed a PICC line. Turns out it is allergies and is not bothering my lungs. I really just want to cry. I feel like my dreams of being a mom someday feel so much more attainable.

The other amazing part is I am scheduled to visit the high risk OBGYN to discuss pregnancy and CF with her so that we can make an informed decision. I feel like it is all coming together...now I just need to cross my fingers that i can get pregnant easily.

What a good day!