Orkambi

As most of the CF world is probably aware of by now, the vertex drug developed for DDF508 has a good chance of becoming available to patients this July. Everyone in the CF community was hopeful this drug targeting DDF508 would be a life changing miracle drug just like Kalydeco was for people with the G551D mutation. Unfortunately, the results of the study quickly dashed many of our dreams that this drug would be our miracle drug.

As much as I am disappointed that Orkambi, which will work on my mutation, is not the life changing and life extending drug that Kalydeco is for a select few with CF. I can say I am beyond excited, despite having all the facts, that we may have another medication that could potentially help slow the progression of my disease. When you live with a progressive disease new treatments are never developed fast enough. In fact, discoveries to treat CF are far and few between. And even when therapies are developed I have found myself allergic and unable to take advantage of many of these medications and treatments. The progression of the disease is always three steps ahead of the treatment and watching your lungs and body deteriorate is heartbreaking at best. It development of treatments are very much a matter life or death for so many with CF. This is why I have to roll my eyes at the naysayers that complain about the humble 3% increase in lung function that were seen in the Orkambi study.

What the naysayers don't realize is that 3% may not seem clinically significant, the truth is that the drug is so much more than 3% increase (on average) in lung function. It is the fact that is can slow the guaranteed progression of this disease. That is something that can not be taken lightly. To me, it  means I may have a few more years added to the end of my life to watch my daughter turn into a woman. It may mean one or two more anniversaries I spend with my husband. It may mean another birthday and a few more grey hairs. So yes, 3% may not seem like a big deal to someone who hasn't had an expiration date stamped to their lungs. It may not seem like 3% could change a life, but pessimists are stuck on a low percentage and not the potential for what slowing, even minutely, can do to a life. How a few years can mean so much to a mother, a wife, a sister, a friend who would do anything to hold on to their life for just a little longer.

So yes, lets celebrate the accomplishment of the researchers, the foundation, the  CF community that we may (as long as the FDA gives the okay this July) have one more tool in our fight again CF, we may have a little more hope for tomorrow, and we may have a little more time on this Earth.

Spring

Spring has arrived gently this year. The heat of May never showed its face and we have been soaking up the cool days and even cooler nights. This spring weather in combination with my behaving lungs has brought a constant flurry of activities. It has been a welcomed change to the hibernation and sickness that winter brought and I feel I emerged this spring as a new person.

Rather than finding comfort and therapy through the keys on my laptop posting to this blog, I am finding therapy in the days I spend splashing in the river with Kaylee, conversation with friends and neighbors, long walks and bike rides, feeding the baby ducks along the canal, and spending entire days tending to the garden.

I am finding my peace watching Kaylee pick handfuls of beans and a fist full of carrots only to flop down in our strawberry patch and have a breakfast straight from the Earth.

I am finding my healing in the mornings that I don't wake up coughing, the laps I can swim at the gym pool, and the roundness of my belly that only comes when my health is doing well.

I am thankful every single day that I wake and feel well, feel like myself, the self I should be. Not the self stifled by this debilitating disease. I know, after 31 years with this disease, that hard days are around the corner, my lungs will struggle to get my through the day, and the hospital will be my temporary home once again, but until then I am basking in the warmth of the sun, fresh air, and health that this beautiful spring has brought along with it.

Guest Blogger- Anika

It has been a while since I have been able to say this so I am extra excited to introduce another mom-to-be with Cystic Fibrosis. Anika is 26 year old and author of the blog, Faux Islander. She is more than half way through her pregnancy at 24 weeks!

 

Everyone that has CF seems to have a very different experience. Share a little about your CF story or how CF effects you.

At six months of age I was diagnosed with cystic fibrosis as I was not gaining weight or thriving as normal babies should. Other than not being able to digest food without taking enzymes, the CF didn’t start to affect me, well my lungs in particular, until I was 22 years old. I started getting reoccurring chest infections and had to be hospitalized for the first time with IV antibiotics at 24 years of age. As my lungs have always been healthy, it’s a bit scary for me now as I get older to see them deteriorate so quickly but I am very diligent in exercising and doing my nebulizers which helps to keep them infection-free. Regardless of the hospital admissions and lower FEV’s, I am very blessed to be as healthy as I am with CF; it is something I do not take for granted.

Deciding to have a child when you have CF is a very difficult decision. Please share how you came to the decision to ttc.

 Having kids and a family is something both my husband and I wanted, it was just the timing we were unsure of as we are newly married and didn’t know if we should work for a few years and then ttc or just go for it! In the end, we decided to just stop using protection and see what happened as I wasn’t even sure if pregnancy was possible for me due to my CF. It was definitely a surprise when, one month later, we found ourselves on the road to parenthood with a positive pregnancy test.  

 

What were some of your biggest fears regarding the pregnancy itself? So far have those fears been warranted?

 

Wondering how my lungs are going to cope is definitely the biggest fear I had, and still have with my pregnancy. I wasn’t sure if getting pregnant would cause my lungs to deteriorate faster and thus leave me worse off afterwards but the doctors and my CF team have told me there is no evidence to believe that getting pregnant takes years off your life in regards to the strain it puts on the lungs etc. I found this very reassuring and was thankful they addressed that fear because my lungs have suffered more infections during pregnancy than what is normal for me and it is easier to cope knowing that exacerbations during pregnancy are normal and will (hopefully) only be temporary. I am 24 weeks and have had IV antibiotics twice as, both times, I got sick with a normal cold and couldn’t get the infections cleared solely with oral antibiotics. I am very diligent in exercising and doing my nebulizer (hypertonic saline) with the hopes that I can prevent more exacerbations, or at least extend the times between them.

 

How has your pregnancy gone so far? What have been some of the biggest challenges in your pregnancy so far?

The biggest challenge for me has been trying to avoid getting sick. Ironically, the actual pregnancy itself has been such a pleasant experience; I’ve had no morning sickness, no nausea, no heartburn, and no food cravings or aversions. Sure I slept a bit more in the first trimester and I have the occasional ligament pain as the uterus expands but from some of the stories my friends have told me of their pregnancy experiences, I know mine has been a cake walk in comparison.

 

How do you feel your pregnancy is different that a non-cf pregnancy?

 I think the biggest difference (as best as I can theorize) is that moms without CF don’t have to worry as much about their own health; their stress and anxiety is aimed mostly towards the well-being of baby where as a mom with CF is hyper-aware of every little difference she feels in her lungs (or body in general) as it is hard to know if it’s just a normal pregnancy symptom or a CF symptom which needs to be dealt with.

 

What are your doctors doing in order to ensure your pregnancy is healthy (more doctor appts, change meds, etc)?

 Anticipating my need for more frequent IV antibiotics, my CF doctor inserted a PICC line in my left arm which will stay in until after I deliver as it allows for quick and easy administration of the meds. Having the PICC has actually been such a blessing by taking away a lot of my stress because I know that if the need arises for IV antibiotics, I can basically get them started instantly which is vital to managing lung infections; the sooner the treatment is started the better the results.

 

What are some of your concerns about mothering and CF?

Initially, I am quite concerned about the first few months of motherhood as the needs of the baby are in high demand which means little sleep for me and, inevitably, result in a weaker immune system to fight off colds. Once the baby sleeps through the night I am anticipating and hoping things will then stabilize and I can just carry on carrying on.
I do think about the future and the scary thought of leaving behind a young family if I die earlier than later but not doing things in life (like not having kids) because of fear or because of my CF is not the way my husband and I have chosen to live our lives. No one can know what the future will bring, so just be thankful for every day the Lord gives you…… and live!  

If you could give advice to a CF woman who is ttc or is pregnant what would you tell them?

 I would say, don’t lose heart or get swallowed up with fear if you are constantly finding yourself fighting off chest infections; frequent exacerbations are normal as making a baby puts huge demands on the body causing even perfectly healthy women to suffer more. Also, try and make sure you have supportive people in your life to encourage you and help you. My husband has done an absolutely amazing job at calming my fears and remaining positive when I hit some of those lows; he is my constant companion and helps out in any way he can. My family has also been a huge support and I am comforted to know they are here to help once when the baby finally arrives.
Pregnancy is hard on the body so take the time to care for yourself, whatever that may be.  

**Anika gave birth to a healthy baby boy July 25, 2015 at 36 weeks gestation. He was 5lbs 15oz ***


To read the stories of other women with CF and their journeys with CF and pregnancy/motherhood please click here.

If you are a CF mother (though adoption, step children, surrogacy, fostering) or are CF and pregnant and want to share your story e-mail me at inhalinghopecf(at)gmail.com

 

Two Months Since Zosyn

It has been two full months since I had my port deaccessed and stopped IV antibiotics. Two full months of feeling like myself. Two full fever free, ache free, sick free months. The people at the gym know me by name and the pool feels a little like my second home. This is the longest I have felt "normal (like CFers ever feel normal) in a really long time. I know better than to get my hopes up, but for now I am enjoying the old me once again!

Toddler Talk Part 6

Language development has been by far my absolute favorite part of watching my daughter grow up. The most fascinating and comical aspect of language development of an almost three year old is how one moment she can use words that seem far beyond her years only to completely botch the very next word that comes out of her mouth. So despite the correct use of words like disgusted, delightful, and homesick she also says the following not so correct sentences/words on a regular basis:

1. "Yummy to my chum chum chummy!"

2. She calls an oven mit a "hand bra"

2. Wrinkly fingers from the bath she calls "crumbly fingers"

3. Excuse me is said, "scoo scream"

4. Whenever she cries and her eyes are full of tears she calls them "blubbly" (like bubble with an extra l)

5. When I got home from the hospital, but was still on IVs we watched the Daniel Tiger where his mom is sick a lot. In the episode Daniel Tiger sings a song, "when you are sick rest is best." Kaylee still loves to sing, "When you're sick rest is rest" which seems a little redundant, but who am I to judge?

Allergic To Life

As a teen my dad would always bring me to my CF appointments with a pen and notebook in hand. He would scribble everything the doctor said and keep track of all my stats from the appointment in that notebook much to my horror. "Dad, you don't need to take notes. This isn't school," I would plead, but my scientist father paid no mind to me and kept those books until I started going to appointments alone. And although the notebook was not my style I learned the value of keeping track of my health and trends over the years. So I started a calendar (visual learner much?) and kept track of my PFTs, O2, clinic appointments, and took careful notes of everything important that took place during my appointments on the calendar.

So when April rolled around, only 6 weeks after IVs, and my lungs felt like they completely closed shop leaving me gasping for air I turned to my 2014 calendar. I was wondering how I felt last April when I was only a few months out of the hospital. Sure enough April was my worst month last year. I wrote that although I didn't FEEL sick I was tight! So tight that my lung function plummeted and I was using my inhaler up to seven times a day. There were nights I woke as if I were suffocating and puffed my inhaler until I felt well enough to sleep again.

Exactly a year later the symptoms were the same! I felt so tight my inhaler was glued to my side and I found myself gasping for air as if I were drowning at the smallest of tasks, but besides being short of breath I didn't feel that bad. After April my health improved a lot which led me to believe allergies may have been the culprit. I went out and bought a new allergy pill (to add to my year round Singulair) and within 24 hours I felt a huge improvement. My 7 puffs of Combivent a day was reduced to three or four and my PFTs continued to climb over the next few days. I am still not at baseline, but I am significantly higher than I was before switching allergy pills. It also confirmed that even though my 14 year old self was ashamed of my nerdy dad taking notes as if he were in a lecture hall rather than a tiny clinic room he really knew what he was doing. Sometimes you have to admit that your parents really were right all along!

Quality Control

I got my first vest at the age of 14. That obnoxiously large Vest shook my chest walls throughout my teens and followed me to multiple dorm rooms, apartments, and even saw the first few years of my marriage. At 28 I finally got an upgraded Vest, not because it was broken (although the timer no longer worked and the peddle sometimes needed a little encouragement to get the thing to start), but because my insurance finally approved it. The new Vest came when I was heavily pregnant, but even with a Vest that couldn't be fully snapped, I was delighted with the much smaller, lighter and programmable Vest.

And then within two years it broke. Just stopped working. So I got a new one. This time with locking hoses (!!!) so that less time was spent popping tubing back into the machine. Although the tubes did wiggle their way out of the machine from time to time I was still excited to get an upgraded version that would make my Vesting experience more enjoyable... err..as enjoyable as sitting in a Vest that violently shakes your chest for 30 minutes can be.

And then with only 200 hours clocked on the machine the new and improved locking hose broke. A little over 6 months and the tube is completely useless. As I wrote "call the Vest company" on my Monday to-do list I couldn't help but think about the old, loud, HEAVY vest that lasted 14 years without a problem which seems to be 12-13 years longer than I can get the newer model to last!