3 month Kalydeco clinic visit

I had my first post Kalydeco clinic visit last Thursday. I’ve been on it for 3 months now. I was so anxious about the appointment, about doing my PFT’s. I wanted to see higher number so badly, just for further confirmation that it is working. And I’m happy to report I had a great visit!

Dr. D asked me how I was doing and if I noticed any changes. All my changes have been subtle, I never got that “wow” that I’ve heard others describe after they started K. My cough has decreased dramatically however. I cough during my morning treatment the most, then depending on the day I could go without coughing at all or maybe a slight cough during parts of the day. But overall I’m not very productive. I had a cold a while ago which stayed just that, never developed into a chest infection.

Dr. D assured me that these are along the lines of what she has seen with the off-label patients. She expects increased time between exacerbations & less mucus production. Hearing that this is what other off-label patients have experienced reassured me that this isn’t in my head and that K is working how it should be on me.

Then off to the PFT’s…

FEV1% = 74%

Great air intake, no crackling, no rails

That’s 2% higher then 4 months ago in May at my pre Kalydeco visit. Which was also 4 weeks post my IV tune up. Before that tune up my FEV1% was 70%. I haven’t seen a number higher then 72% since right before I got pregnant with my daughter 2 years ago. So I’m now 5 months post IV’s, 3 months post the start of Kalydeco and my lung function has increased slightly. I am so happy!

All of my PFT results since the end of 2006 were sent to her and she wanted to discuss the trend with me. My previous doctor always just told me my baseline was 72% which was my average and honestly I never paid much attention to it. It hasn’t been until recently that I’ve really started taking an active role in my care. So it was quite interesting to see everything. In 2007 (before I was pregnant with the twins) I had FEV1 of 72%, 76%, 72%, 76%.  Then the two years from 2008-2009 I didn’t blow above a 70%, mid 2009 was when I got really sick also, for a year I didn’t get above 60% actually. In early 2010 I started running and things shot up 70%-72% with the exception of two highs of 103% and 80% which was when I was training for the NYC Marathon. I remained consistent at 70% since the birth of my daughter. I had a tune up this past April 2013 and blew a 72% in May at my post IV / pre K appointment. Now I blew a 74%.

So it really showed me just how much exercise played in not only getting my lung function back up, but also in keeping it there. Although I can’t keep up with marathon type training, running and swimming 15 + miles a week is really important to my health. And the Kalydeco is working in its small ways for me, which is all I could have hoped for. To be the same lung function that I was 7 years ago after taking a dip for a few years is amazing to me.

In other news, I’m starting the TOBI Podhaler next month which will save SO much time! Overall great appointment. 

Quick update

I apologize it’s been so long since I last posted. Our days have been jam packed this summer and any free time is spent in the pool so my nights which is when I try to blog are spent cleaning up the house.

Not much to update about either. I’ve been feeling well, no better, no worse than a month ago. This past Saturday was the Boomer Cystic Fibrosis Run to Breathe in Central Park. Due to the weather conditions it was an untimed run, but it was a great run. I’ve decided to push myself hard and signed up for the Bronx 10 mile in September.

I have a CF appointment in early August. Hopefully I will continue to feel well and see some improvement in lung function.

I’ll try to update more often but I’m just having so much fun with my kiddos this summer!

2 weeks of Kalydeco

I’ve been on Kalydeco for 2 weeks now. I was told to give it 2 weeks – 1 month to really judge if it’s making a difference or not. My verdict… Yes, Kalydeco has made a huge difference in my symptoms. I can’t yet give an answer if it is clinically helping me but symptomatically it has improved my life a lot. I never thought I’d be writing a post on all my mucus changes, but in the hopes of helping someone with my mutations or other similar mutations know what they could expect from Kalydeco here goes…

 The past two days my cough has lessened again and it’s become even less productive. I have one “normal person” cough in the morning, just one singular cough, and spit up a small amount of mucus. But that is compared to waking up and having a mild – moderate coughing session for about 5 minutes coughing up thick mucus that collected in my lungs over night.

The past two days while I’ve done my nebs & vest I have not coughed up anything. I try. I still huff and cough and spit but no mucus. Ok, maybe in total I’ll see a dime size amount of pale yellow mixed in with my saliva.

During the day I’ve become much more conscious when I cough. When I do, most of the time it’s this dry cough that irritates my throat. Maybe asthma, maybe allergies, maybe both. But it’s different than my CF cough. It's strange to cough in the car and not need to reach for the box of tissues or put the baby down to sleep and not have to repeat in my mind "don't cough, don't cough" as I sneek out of her room.

I still find I’m having issues with my sinuses. I’m not blowing my nose constantly like I was when I fist started and I’m still doing a nasal wash twice but I’m noticing the mucus I’m clearing from my sinuses is sometimes green. Green = infection as far as I know. I haven’t had any sinus pain or pressure and I haven’t had any major issues with my sinuses since my surgery debacle about 8 years ago. I don’t know if just clearing stuff that was sitting up there for years? I’m going to try to do my TOBI with a mask for the next month and keep up with the sinus rinses and see what happens.  

I should be having a clinic visit around the 6 weeks mark but I’ve thought about buying one of those inexpensive FEV1 meters just so I can see if there are any changes between now and then. I should have done it before I started but I didn’t think of it. I’m curious to know if anyone else has one and if they blow similar scores as they do at clinic.

Surviving the sickness

I’m still feeling the same as I did on day 5. I basically had no cough from days 2-4 but from day 5 through today, day 11, I have been coughing. During those days it hasn’t gotten better or worse, just stayed the same. But compared to the day before I started Kalydeco my cough is significantly less and significantly less productive and the color for the most part is much lighter. Mostly in the morning and following my hypersal / vest treatment.

I was questioning why I stopped coughing and then started up again but after finding out at least one other person experienced the same thing helped ease my mind a bit. I still don’t understand why, maybe my body was just adjusting to the medication but it still was disappointing to have the cough come back, even though it is SO much of a difference. I had imagined my lungs clearing all the mucus out and it being gone forever. Wishful thinking I suppose!

So for now I’m calling it a victory as long as I don’t feel myself going in the opposite direction. I can live with stability, I welcome stability. And so far so good, I don’t think I’ve gotten the bug my kids have. The baby spiked a fever of 103 last night and was up every two hours. She slept with me the whole night and we wound up taking two cool showers because it felt like she was burning up. Logan woke up crying because his throat hurt him so much and croup was making him cough. I hate not being able to fix my children’s pain.

Got in a 2 mile run this morning, but before I got out of the car to start my run I text my ‘bestest’ CF friend “L” who had a super important clinic visit today. She had just found out that she passed the screening tests and was now enrolled in the Vertx Phase III trial. She needed this and prayed for this and it came along at the perfect time. Words can’t explain how happy and excited I am for her. A new beginning.

How we both wound up in this place, together, going through this at virtually the same time is a bittersweet story. I knew of her and she knew of me for a while, but it wasn’t until years later that she found me on facebook thru our mutual friend Nicole. The three of us all went to the same CF center, had the same doctor and were pregnant at the same time. L and I bonded in a way we never imagined - having to mourn the loss of our friend Nicole and her baby. Unthinkable circumstances brought us together. But we understand each other, our fears, our hopes, our ups and downs in a way that no one else can. In a way I am more excited for her to start the meds then I was for myself. I’m crossing my fingers she gets the real thing.

Normal is beautiful

Today was a normal, ordinary Friday.

 Yes, I know it’s Tuesday but I started writing this on Friday. Where does the time go? I feel like I only have about 5 minutes to myself everyday. But after a fun filled morning in the pediatrician’s office we are stuck home with two cases of croup and one viral fever. Prescribed an afternoon of movies and ice pops I’m finally sitting down for a bit before tacking the dishes that piled up over the weekend.

 So back to Friday…the kids and I woke up around 7:30, made a fresh batch of blueberry pancakes with maple syrup and sausage, all while chasing after Kaeli who’s new favorite sport is to climb up everything. And she is quite the climber for a 13 month old! Grandma came up and watched Kaeli, well got to watch Kaeli nap which means Grandma actually folded my laundry that has been piling up (thanks Grandma!) while I drove the boys to school and went for my 30 min swim.

 A lot of people tell me that swimming is boring. I actually find it very peaceful. It’s the exercise where I focus most on my breathing. When running I distract myself with music and concentrate on my pace, but with swimming the only noise I hear is my breathing. Just a steady in and out, in and out. It’s a time where I get to reflect and actually think without numerous distractions. Today I found myself thinking about how much I appreciate being able to have a normal ordinary Friday.

Normal is boring, tedious, and monotonous. Normal is frustrating, demanding, and challenging, But normal is beautiful. Normal is what I cherish most. I really try not to take normal for granted because it’s a joy I’ve seen ripped away from too many people.

 Of course I will remember the big things like the look on my boy’s faces when we walked into Disney World for the first time, the way I felt running over the Verrazano Bridge, holding my babies for the first time. But it’s those everyday, normal, ordinary things that I have a strong emotional attachment to. The simple things, too often taken for granted. It’s easy to lose perspective on the beauty of normal. Like making blueberry pancakes on a Friday morning, nursing and rocking the baby to sleep every night stroking her hair mesmerized by her face. Blasting the radio on a rainy afternoon and dancing around the living room, or all of us climbing into bed on a lazy afternoon and taking a nap together. Those are the things I will miss the most.

Those are the things I am fighting for. To have many more normal ordinary Fridays.

Becoming a mom

No real changes to report. I coughed a bit more than I did yesterday but significantly less than I have been pre-Kalydeco. And the color and quantity of the mucus is definitely lighter. I'm beginning to accept that Kalydeco is going to work slowly for me but that's ok. A lesson is patience. Maybe I won't be one of those people who sees a 30% increase in lung function but that's ok. It has already helped my cough and mucus so will hopefully help my lungs stay infection fee for longer periods of time. And any increase in lung function is welcomed!

So here is a little about me and the birth of my twins and the fight for my health that came shortly after...

I always knew I wanted to me a mom from the time I was a little girl, so when my husband and I got married, deciding to start a family was an easy decision. We did however put a lot of planning into starting a family. Luckily my lung function was stable with (FEV1 in the mid 70%’s) and I am pancreatic sufficient. I knew I wanted to be a stay at home mom so I continued to work for 4 more years (including while I was pregnant) to save up so I could afford to do so and my husband had a genetic test to rule out being a carrier of CF before I got pregnant.

We found out I was pregnant Memorial Day weekend and we were so thrilled. When I saw my obstetrician at around 9 weeks she gave me an ultrasound just so I could get a tiny peak at the baby. Well that ultrasound changed my life forever… I learned that I was carrying twins! I called my husband in total shock and it took about 2 weeks for it to really set in. I transitioned to a peri-natologist (high risk OB) who was affiliated with my CF hospital which also had a level IV NICU so we had all our bases covered. I can't say enough praises for my OB, he was fantastic the entire pregnancy and I owe my sanity through the pregnancy to him.

Despite being classified as a high risk, the pregnancy was textbook and uncomplicated. I loved being pregnant and it agreed with my body – no morning sickness, a 40lbs weight gain, stable lung function (apart from a slight drop at the end due to the lack of space in my lungs). I had one course of IV Ceftaz at 29 weeks after developing a productive cough but managed to continue to work from home until my boys were born.

One Thursday evening, two nights before I reached 32 weeks of pregnancy, I started to get mild but consistent contractions. By the time morning came they had not let up so we hed to the hospital and I was put on a saline drip, two heartbeat monitors and a contraction monitor. The doctors told me that they would not stop labor at this point, they were just going to monitor me and the babies and see how it went.

On Saturday afternoon, almost 48 hours after my contractions, they told me that I needed an emergency C-section because one of my sons was showing signs of distress. The Neonatal team came up to talk to me about the complications that could happen from a 32 week delivery and what I should expect. I’m not going to lie, what they told me scared the daylights out of me but I didn’t have a choice at that point. I was wheeled into the operating room (with about 20 medical staff in the room) and everything went very quickly. Both boys were born, the Neo-natologist teams made sure they were stable, and I got to briefly see them as they were rushed to the NICU.

J was born 4lbs 10oz and was on Bi-PAP for just a few hours. He had no preemie complications other than being jaundice and having to learn how to eat properly (suck, swallow, breathe). He was in the NICU for 4 weeks as a grower and feeder. L (the twin that was showing distress) was born 4lbs and had a few more problems, He was on a ventilator for a few days and Bi-PAP after that, had a PDA (hole in the heart), needed a blood transfusion and also had jaundice. On Christmas Eve, at 11 days old, the boys got to reunite in the same bed for the first time since birth. L was in the NICU for 6 weeks and his PDA closed on its own by the time he was 2 months old.

For me, coughing after my emergency section was rough. Since I didn’t do IV Tobramycin while pregnant I decided to do it while in the hospital after delivery and while the twins were in NICU so I could be in top shape for when they came home. It took me about 3 weeks to feel normal again after the surgery.

The year following the twins’ birth was a rough one, for a multitude of reasons:
1. There was two of them and one of me
2. I was breastfeeding & chose to be off TOBI
3. I had a C Section & was on IV immediately after delivery for 3 weeks
4. We had to limit visitors early on because of their prematurity
5. I wound up with kidney stones
6. I Got the flu in July ’09 for the first time ever – 2 weeks of home IV’s
7. The boys both got RSV Respiratory syncytial virus) in Dec ’09 and I wound up sick – another 2 weeks of home IV’s

I wasn’t sleeping well for a long time. Fitting in two treatments a day was impossible. July ’09 my lung function was the worst I’ve ever seen it – below 50% predicted – but I wasn’t feeling well and thought I would get back to my baseline once the first year was over. I just didn’t have time to think about anything other than the babies at that point in time. I was getting by doing the bare minimum vest & nebulizer treatments. I had very little energy to do anything and looking back I was probably somewhat depressed feeling “trapped” inside my home that first year. It was extremely difficult to go anywhere alone with two babies. It was near impossible to go even food shopping with two infants in car seats. I was very lucky to have my mom with me to help while my husband was at work.

After my 2 weeks of IV’s in December ’09 something clicked inside me and I knew I needed to get my butt in gear somehow. My lungs weren’t magically going to get better on their own without some hard work on my part. For the first time I was actually scared for my future. I was angry at myself for not trying harder, for underestimating what CF can do in such a short amount of time. I want to live, for myself, for my husband, for my children. And not just live, I want to have energy, run with them, dance with them, play with them. I woke up one day and joined a fitness center and began swimming again. For the first time in probably close to 7 years I was exercising again.

The following April, I decided to give myself the biggest push I ever have and signed up to run the New York City Marathon for Team Boomer later that year. On November 7th 2010 I completed 19 miles of the marathon before being forced to stop due to cold and exhaustion. May be if I hadn’t had a chest infection at the time I would have completed it, but from the beginning of this journey it was never about crossing the finish line. It was about challenging myself and pushing myself to be a better me. And I succeeded. I ran 19 miles when just seven months earlier I couldn’t even run a mile without getting out of breath. I was able to increase my lung function by over 50%. I learned so much about myself and I became a stronger person.

Kalydeco: Day 4 & a 5k

I woke up dry again this morning and didn't cough at all. I had to clear my throat of some mucus once but it was clear / light colored. During my vest / nebs I think I only had 2-3 good productive coughs which is different for me. HyperSal usually gets me clearing a decent amount of junk from my lungs in the morning.

Grandma was here to hang out with K while I dropped the boys to pre-k and went for my first outdoor 5K run this year. The weather was amazing. I haven't run (ok maybe 1/2 ran, 1/2 walked) those hills since before K was born. But now I'm pretty confidant that I'll be ready to run the 10K in July. I had a few quick coughs on my run and could feel my asthma making my chest tighter than I would like so I think next time I’m outdoors I will use my albuterol first.

I apologize for the short posts right now. I don’t have too much free time on my hands these days with the kids and hubby’s crazy work hours. I just really want to make sure I write down all the changes I see right now. I’ll work on some more personal posts for the future.

Kalydeco: Day 3

Woke up very dry. Dry mouth, dry nose. Didn't cough too much this morning, less than usual, but while doing HyperSal/Vest I did started to cough up junk. It was still the normal amount / color that I usually cough up when I do HS. Not blowing my nose as much as yesterday but I have a persistent post nasal drip that is causing me to constantly clear my throat that lasted all day and evening.

The boys had their swim lessons toady and I brought K in like I usually do. She is going to be a fish like her mom - she wouldn't stop jumping in the water! No fear of going underwater at all.

Today was probably the most gorgeous day we've had this year, and I would have loved to get out and run but couldn't escape the kiddos today.  Tomorrow I hope to get in my 3 miles while the boys are at school. I have to step it up to get ready for the Boomer 10K in July!

Kalydeco: Day 2

The same.

I think I'm over analyzing and reading too much into every sniffle and every cough! I just wish there was a clear sign that it's working. Sigh.

The only thing I noticed today is that I had been blowing my nose a lot more this afternoon and (TMI alert) it's been thick and greenish. Yuck. I also feel myself clearing my throat during the day and every time I wind up with some mucus in my mouth.

I don't want to 100% say this is from Kalydeco. This is my allergy season and my daughter has a cold and is full of snot herself. That combination could definitely be the cause of my symptoms.

Kalydeco: Day 1 & my pre-K stats

Yesterday I took my first dose of Kalydeco! The bottle has been teasing me for over a month now, sitting on my dresser, and it was a relief to be finally be able to start. My overall description of day 1: Anti-climactic! haha

NO change in symptoms
NO side effects
NOTHING

I don't exactly know what I was expecting, but I did expect something. After waiting for 32 years I'm getting impatient and don't want to have to wait for the medication to start working. Am I being a pessimist because I keep thinking maybe this won't work for me? Maybe I'm putting too much pressure on this little blue pill of "magic". I asked around in the CF community and based on my CF it sounds like I need to give it 2 weeks - 1 month to really judge it. Ok, Kalydeco, I'll be patient but PLEASE PLEASE work for me.

Since I don't have much to report I'll give you a brief rundown of my CF background.
-Mutations: DF508 & 3272-26a>g (class V)
-FEV1: 72% My baseline has remained at 72% for 5 years now although I have seen a low of 49% and a high of 109%. My last FEV1 before starting K was 72%
-I am pancreatic sufficient
-I have never had a problem bringing up mucus and am always told my lungs sound "clear"
-I usually cough first thing in the morning but by the time I finish my morning treatment I don't cough much during the day at all
-I culture staphylococcus aureus and pseudomonas aeruginosa
-Usually have IV's once a year but was able to go two years since being pregnant with my daughter and starting Cayston -Meds: Pulmo, HS, TOBI, Cayston, Advair

Back to Blogging.... (written April 8)

After 2 1/2 years I thing it's time to start blogging again. A lot has happened in those 2 1/2 years and I will recap one day when I have a few moments to myself but first I must confess why I have decided to start up again...

to document my experience on KALYDECO!!

Yea! After a bit of research and a lot of luck I was approved off-label! I was shaking and in tears when Foundation Care told me it was approved. I honestly didn't believe it and still don't. I am now anxiously waiting delivery of my first bottle and sorting out details of sweat tests, blood work and when I will take my first pill. There is still a lot I need & want to get done before that day comes but right now I am just over the moon excited. I feel like I've been awarded a new lease on life.