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!

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...

Monday Surprises

Two weeks ago I had my CF clinic. I felt pretty confident going in because I had been swimming 5 days a week for 9 weeks at that point. I felt good, energetic, and was still compliant with my treatments. I was excited to tell my doctor I was swimming and because summer is usually my healthy season I was more than ready for a quick and easy clinic visit.

Soon after arrival the RT brought in the pft machine and I did my first blow. My eyes scanned the computer screen waiting for the results. The numbers that flashed on the screen shocked me. My numbers were lower than I had seen in a very long time. I sorta laughed telling my RT that I must have made a mistake because those numbers were ridiculously sad. However, there was a sinking feeling in my heart because I have never actually messed up a pft, after doing them for so many years it is a hard thing to do wrong. Just as I had secretly expected, but hoped to no end was not true, the next two blows were consistently low. I was left dumbfounded. Everyone seemed a little baffled that I was working out, felt great, and had no clue that my numbers had plummeted. My doctor ended up deciding to put me on oral because of a unusual (but not concerning) new bacteria in my culture. I agreed, happy to do something, anything to improve my horrible numbers, but was utterly confused as to how I could have an infection and feel... well perfectly normal.

Fast forward three few days, I was waiting for my meds after a problem with my first pharmacy, when I started to feel the telltale signs of an infection: fevers. The next several days my fevers flared, despite my lungs feeling okay. If it weren't for my low pfts I would have easily blamed the fevers on a different infection because my lungs actually felt fine, but I kept reassuring myself that my low numbers confirm that my lungs were the ones wreaking havoc on my body.

And then one week after my clinic appointment, an entire week after my low numbers I sat up in bed after a good night sleep to start my day only to feel the weight of my lungs, brimming with infection causing them to feel monstrously heavy. So heavy in fact, that I felt they may just fall from my body to the mattress below. My husband saw my grimace, heard the sound that puffed from my chest, and watched my body move in the way it does when your lungs are completely infested with millions of bacteria that are quickly taking over and said, "there it is...". He was right...there is was. The infection had finally reared it's ugly head a week after it tried to warn me.

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!

Down The Rabbit Hole I Go

I have found myself in a place that makes no sense at all. It all seems to get "curiouser and curiouser" in the worst kind of way.

Last Monday, I went to clinic. When it was time to see my doctor (I usually see one of the two) both doctors came in to talk to me. I knew right then that things couldn't be good. You don't get two specialists sitting in your room if things are going well. My numbers dropped again. I am now dancing in the 20s. The 20s are a wonderful number when it refers to your age, not so much when it is your lung function. The problem is nothing makes sense and nobody knows why. Let me show you what I mean:

After getting super sick and losing a ton of lung function from the flu I had several rounds of IVs where my lung function went up slightly, but I could never get them out of the 30s which is far lower than my (old?) baseline of 50.  So after feeling like IVs and increased CPT wasn't getting me anywhere I decided go get more proactive and here are the results:

Hospital stay = 4% drop in PFTs
Increase CPT to 4x/day everyday for a few months (and going) = 1% drop in PFTs
Go on steroids = 4% drop in PFTs

And so the harder I try the further I fall. The doctors said multiple times that with everything I am doing we should see some improvement and at the very least I should be holding steady. I have no new bugs and the ones I do have are not resistant to anything, I am 100% compliant and do CPT 4x/day everyday. I don't have fungus. I am treating my Psuedo with Cayston and currently on oral abx as well.

 We decided to try one last ditch effort. I am going on an appetite stimulant. I keep losing weight with lung function and even though the dietitian was very happy with my food log I was quick to admit that I eat well only when I am well. When I get sick or fevers (at least once a month and often for a week or more at a time) I just can't find the energy to eat enough. We know there is a correlation between weight and lung function. We hope if I can regain my weight my body will be stronger and more able to heal or fight off germs.

We added a few other changes as well, but the main focus is weight gain and exercise (to build muscle and lung strength). It is interesting that in yoga last night the set was dedicated to building courage and facing the impossible. It felt appropriate given the feat I have ahead.

Clinic And Arm Update

Today was supposed to be the day I got my PICC pulled or was given a new set of meds, but of course with the PICC pulling itself out and my skin falling off I went in PICC free and extremely nervous. My arm is looking worse, I didn't get to finish my IVs and I had no idea how my lungs were doing. I also had to go with my zombiesque arm out on the open. I tried to cover it last night for family dinner at my parents house since I didn't think people would want to look at it while eating. I will spare you the gory details, but it was so not a good idea.

Within five minutes my appointment was already looking grim. Somehow I lost 4lbs between last Monday and today. How is it even possible to lose 4lbs in 7 days? I don't usually have an issue gaining or maintaining weight so this baffled my mind. I was eating a lot all week as I was trying to gain weight which makes it so much more frustrating. I am down to a skeletal 114lbs from 125. I knew my doctor would try to convince me to stop nursing, but I really believe that in a few weeks I can gain most of the weight back. Nursing is too important for me not to succeed!

My PFTs were making me the most nervous since I had to stop my IV course five days early. With my left arm covered in open sores and my right arm too much scarred to access I knew a PICC was out and I just didn't know what we would do if my PFTs hadn't improved. Luckily, my PFTs were just 2% under my baseline. Once I can swim again (when my arm heals) I think I can get back to baseline.

Pretty much everyone that saw my arm made the most horrendous face and said something along the lines of, "Oh, that looks horrible!" One of the nurses said, "I can't even look at it!!", but then kept sneaking peeks at it. Thank goodness the doctor knew exactly what it was. He even had another CF doc come in to get a second opinion and they both said the same thing. I was given a prescription for topical and oral steroids. I am already on Bactrim so that will take care of any infection that may be brewing. I go back in two weeks and if it isn't better they are sending me to a wound specialist. How scary is that!? I seriously hope that it improves with the steroids because nursing, sleeping, even carrying my baby is so much more difficult when I can't use my left arm.

So it looks like I am over the worst of my sickness. I now have the aftermath to clean up (get my weight back and heal my arm), but I can finally see the light at the end of the tunnel.

Bad News All Around

I went to clinic yesterday after 10 days of IVs and I sort of wish I didn't bother to go because it was one huge disappointment. My weight is down to 118lbs. At 5'4" this isn't horrendous, but for me it is pretty bad. I usually weight 125lbs so the drop was pretty significant. I will admit that several days went by while I was sick in bed that I didn't eat anything. At all. So I knew my weight would be low, but I still hate seeing any numbers below 120.

My PFTs are down by 7% which is pretty bad after 10 days of IVs and because I don't feel like at this point I have a lot to spare. I never got my PFTs done while in the middle of the flu, but I am curious what they would have been...I have a feeling I would have been horrified if I had seen those numbers. I was pretty terrified that my doc would come in and admit me to the hospital since everything looked so grim. When he came in he asked me how things were going. I explained that I was sick in bed for 2 weeks straight (which he knew). That I hadn't been eating, exercising (not even walking because I had no energy), and felt as if my lungs were full to the brim, BUT after 10 days of IVs my mucus was getting lighter and it was getting much easier to clear out. I also told him that I knew my body wasn't ready to stop IVs based both on my numbers and how I felt. He said that swimming prior to getting the flu probably helped me more than I can know even though I feel disappointed with my numbers and slow recovery. He wants me back to exercising as soon as I can. We decided to do one more week of IVs and then we will reassess. So no hospital admission for me yet which was a huge relief.

I feel a lot of pressure to get my PFTs as high as I can in the next 6 days because I am terrified of what will happen if I don't. I know very little of my recovery is in my control at this point which makes it much harder to deal with. I am doing all my treatments, doing all my meds, and even have IVs so there seems to be very little more I can add on to my daily health care. I feel this intense pressure to do better, get healthier, raise my PFTs, but I also feel like I have no idea how. I need to find something I can do to feel like I can control. I have decided to gain weight since this is somewhat in my control. I know my lung function is always higher when my weight is good. I am also going to try to exercise as much as my low energy will let me. The weather should be beautiful this week, in the high 70's, so I am thinking I can manage a walk everyday for the next 6 days. I find this disease can make you feel defeated very easily. I think back to 3 weeks ago when I swam a mile with ease and came home to take an hour walk with my daughter. Now the thought of walking up the stairs is overwhelming. It is amazing how quickly life can change. I just hope that things can return to how they were 3 weeks ago.

**EDIT: I should add that my flu symptoms are pretty much gone. I no longer get fevers and I actually get up and get dressed and I even managed to put on makeup yesterday. So I guess it is not bad news all around, just bad news mostly around**

2nd Clinic Since Baby and "Dada"

I meant to post this Tuesday, but you know...

I left the clinic yesterday feeling a little beat up. I hate getting bad news. My lung function was down which wasn't surprising as I haven't been feeling that great. My doctor wants me to start Cayston again and call him in 2 weeks to give him an update. He is so hesitant to put me on any orals because of breastfeeding. I appreciate his concern for my daughter, but it seems a lot of CFers get orals while breastfeeding and do fine. I am hoping Cayston and a few extra treatments throughout the day does the trick, but I am not too confidant that it will work.


I expected my lung function would be lower, but had no idea my weight would be down too. I lost 6lbs in the last 3 months since my post pregnancy weight was a little higher. That is a LOT of weight to lose! I guess I got overly confident. I got through the pregnancy and the first few months eating when I was hungry and not worrying about calories. I think the combination of feeding a 17+ pound 5.5 month old and feeling under the weather didn't help in the weight arena. Apparently, I still have a healthy BMI, but I need to make sure that this weight loss isn't part of a continuous decline. I am actually more stressed out about my weight than my lung function because I am so emotionally attached to breastfeeding. I am not sure who needs it more, me or Kaylee. That night as I was nursing her to sleep (it puts us both to sleep) I was thinking that I would have no idea where to begin to wean her. We both find so much comfort, routine, and love with nursing. I have become as dependent on nursing as she has. As long as I don't lose any more weight my doctor is completely fine with me nursing as long as I want and so I am extremely determined to keep the weight on.

In other news, my husband joined me when I went to clinic to keep Kaylee entertained. The appointments are usually very long and boring. My husband spent the majority of the appointment letting Kaylee admire herself in the mirror and trying quietly trying to teach her to say dada. By the end of the appointment Kaylee eas whispering, "Dada" right back at hubby. That evening whenever she was playing she would whisper, Dada Dada Dada". It has been almost a week since she learned it and it only comes out as a whisper. It is amazing how they mimic every single detail.

First Pregnant CF Appointment

So I went to the CF doc yesterday and felt 600lbs lifted off my shoulders.

I need to explain a misconception about pregnancy. You always hear and read that prenatal care is VITAL and you need to call your OB once you become pregnant to ensure that you are taking proper care of yourself and your baby yada, yada, yada. While this is important I learned that this isn't AS important early on. Let me explain. I found out I was pregnant and like anyone that works at a school was trying to call to get my first prenatal appointment during my breaks by sneaking into my car so nobody would overhear. Without fail I would be put on hold, my break would end, and I would hang up without an appointment. By Friday of that first week I was freaking out. I thought I was being a horrible mother by letting the 5th week slip by without so much as an appointment. From what I read through other CF pregnancy blogs, forums, etc people are getting their blood levels (betas?) checked early on to make sure they are increasing at a proper rate, and going into appointments and are being watched very carefully. Well, get this...if you aren't going though an infertility clinic nobody wants to take your blood to make sure your beta levels are okay and nobody really wants to see you till you are 8-11 weeks along. Uhhhh really? That is almost all the way though your 1st trimester. Crazy, but true.

Anyway getting off my tangent, I was relieved to just get a stupid appointment for ANYTHING just to make sure that something in my life was going okay so I was happy when I got into the CF clinic at 6 weeks. Well here is the run down of the day:

• My weight remained the same which at this point is fine. 
• My blood glucose seems fine, a little high on the normal side so gestational diabetes may come into play later, but for now they are pleased with my numbers.
• My lung function went up 1% -YAY!!! 58% is my new high :)
• I got a lot of congrats from everyone on the team.
• I got my flu shot (100% safe during pregnancy) so I should stay flu free this season- yay!
• Looked over my meds (even though I did this a while ago with my OB) and he suggested I get of Zithromax for the first trimester because it probably won't have a major impact in such a short amount of time. Funny thing is I stopped once I got the BFP anyway for that very reason. He also said to use the allergy pills and nasal spray as needed. Other than that all my other meds got the green light.

So my doc was so happy with my numbers! He even told me he is sure I will see 60% some time in my pregnancy!! That would be awesome! As he was leaving I asked him when I should come back next and he paused for a second and said he was so pleased with my numbers and how well I have been doing that I really don't need to come back for 2-3 months unless I start feeling differently. I am so relieved that everything on the CF front is going well and feel so much better knowing my doc is not really too worried about me. 

The Problem With Lung Plugs

Yes, I am talking about plugs again. Seems to be the theme of my life at the moment!

So I forgot to mention something the doctor told me that you may find useful to know (or not). I was telling him about my painful spot and that I hacked out a big, fat, juicy plug that I believed to be the sneaky culprit of my pain. He said that it very well may have been the plug that I was whining about. He wasn't as super excited stoked about my plug as I was, but maybe you have to have CF in order to get excited over those types of things. I was still sitting there super proud of myself feeling like I was his best and favorite CF patient for single handedly coughing up a monster plug when he told me some sad news.

He told me the problem with plugs is (I knew at this point I was not going to be grinning ear to ear for long) they cause problems even when they are no longer in the lung. Well, damn I am definitely not smiling anymore. He explained it like this (he is a very good explainer so I hope I can make as much sense as he did) a plug is the build up of mucus in your lungs and it can get hard and very, well, stuck! Unfortunately, this can cause the airway to get stretched out because it needs to accommodate this fat unwelcomed guest. So when a patient so miraculously (I added that little detail, but I could tell he wanted to say it) coughs out a very large and bothersome plug its damage to the airway is still present. It creates some nooks and crannies if you will. And anyone who has ever eaten an english muffin knows that things (like butter or mucus) will pool in those nooks and crannies. I have never liked English muffins and now I think I know why. So even when the plug is gone that airway is MUCH more susceptible to replugging.

So now I am feeling less thrilled about that plug and even slightly regretful for taking the picture of it (well, maybe not because it was pretty crazy to look at) and a little more stressed out about my little stretched out airway. He told me to think about that as a spot I have to give extra attention. I should also call him if it becomes extra painful again and maybe I will needs orals again. So needless to say the problem with plugs is they are the gift that keeps on giving...or something like that!

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!