Tuesday, June 24, 2014

Vertex and CF

So as I am sure you all already know the results from the phase 3 Vertex trial for DDF508 has been released and were positive!!! We are hoping the FDA approves it by end of quarter 2014, come on FDA!!

There seems to be a lot of mixed feeling about these meds which I find bizarre to say the least. Many people, like me, are excited, of course! But there seems to be a very vocal group of CFers that like to point out all the negatives of this potential drug.

Ummmmm, hello!! There are new drugs coming out for CF, how in the world is that a negative thing?? Is this a cure? No way. Will it make our lives normal? Nope. Will we see an increase in our PFTs? Maybe, but no guarantee. Will we get to reduce or stop doing our treatments? Of course not! Is there the potential for negative side effects? Yes, but have you ever taken Prednisone? Most side effects > better than being killed which is what CF does best.

Regardless, we have another tool to fight this awful disease. One more chance to live a longer life. One more defense against lung infections and scarring and permanent damage. It may not work for everyone with DDF508, just like Tobi doesn't work for me, but for those it does work for it is totally worth it.

So today is a game changer! Today I am going to celebrate and be excited about the future for CF patients because we are moving in the right direction and there are other drugs following close behind. We just need to keep hanging on as long as we can so that maybe someday our life expectancy will be virtually normal and we can look back and say, "remember when CF would take so many young lives..."

Saturday, June 21, 2014

Toddler Talk Part 3

Nothing is cuter than toddler talk. Their little made up words, brutal honesty, and adorable way of saying things you would never anticipate. Here are a few of Kaylee's:

K: "Mama going poo poo in your pants? That so yucky, mama!"
M: "I didn't go poo poo in my pants" (btw I didn't even pass gas)
K: "Not yeeeeeeeeet!" in a sing song you just wait little voice.

I threw a big frumpy t-shirt on one morning.
K upon seeing me: "Gasp! Nice dress, mama!!!"

Kaylee and I went to music in the park to get ice cream. As we were leaving:
Kaylee: "I like ice cream!"
Me: "I love ice cream!"
Kaylee: "I love YOU, mama!"
She then proceeded to tell everyone we passed, "I love my mama so much" with a few, "I love Kaylee" thrown in for fun.

Kaylee was on the slide in the backyard while I was watering. My hubby was at work. 
K: "Mama!"
M: "Yes, Kaylee?"
K: "Dada is sooooo whiney!!!" And then she went back to playing.

After singing a little made up song Kaylee says, "Mama, Kay-kee is songing (singing)."

Friday, June 20, 2014

Senior Citizen Status

It is official, I have hit senior citizen status at the ripe old age of 30. Let me back up and explain how this happened. So about 2 months ago I joined the gym as suggested by my doctor. I joined a gym with a pool because I was strongly encouraged to swim since it is a great form of exercise for the lungs. However, for various reasons (including fear of failure) I was avoiding the pool. I went to the gym religiously, but I worked on weights and the elliptical machines, but did not step foot in the pool.

I promised myself last week that this week, the week after my daughters (2nd!!!!) birthday I would start swimming. Monday evening I had childcare for K so I knew my goal was to get in the pool and swim as many laps as possible even if it only meant one. I couldn't find my bathing suit, but since I promised myself that I couldn't come up with excuses I pulled out a tankini I had in the back of my closet and decided to go for it (hoping that nobody would be in the pool to see my beach rather than workout attire).

As I approached the gym I glanced through the fence to the pool hoping to see it vacant. No such luck! In fact, it was really really full. I also quickly noticed that these people in the pool weren't moving, but rather standing around in the water talking. This could only mean one thing- aqua aerobics was about to start. You know, that old lady exercise class that takes over the entire pool. I quickly realized, by looking at the gym class schedule, that the pool would be occupied for the next hour for aqua aerobics which meant I could not do laps. I was standing there in flip flops and my bathing suit and I realized I was left with two options: leave or join the class. I promised myself I would get in the pool so there I was, all 30 years of myself, among 60, 70, 80 year old women.

You know what though? The one thing I did not know about aqua aerobics was that you jump the entire class. Yeah, like jump and clap under your thighs, jump in the splits (HA!), jump to the side, just jump jump jump. And if there is one thing that really shakes up mucus it is jumping. I found myself coughing half way through. I even started coughing to the point that I was starting to get those side glances from the other women and I wanted to shout, "I am not sick, don't worry!!" So despite being 30 years younger than everyone in the class I plan on going next Monday and even the Monday after that!

**Oh and I did get to the pool a few days later and was actually able to get way more laps than I ever imagined I would be able to do after being out of the pool for so long. Take that, 33%!!!***

Monday, June 16, 2014

Guest Blogger- Colleen

Our next mommy-to-be CF blogger is Colleen! She is from the blog, *Live*Laugh*Love*Breathe, where you can follow her as starts her career as an elementary school teacher, starts Kalydeco, and goes through a complicated journey of trying to have a baby and IUI. She is 25 years old and 31 weeks pregnant with a little girl!

Everyone that has CF seems to have a very different experience. Share a little about your CF story or how CF effects you.
I was diagnosed with CF at birth due to a meconium blockage. I had surgery to get it fixed and had to stay in the NICU for about a month until I gained my birth weight back since I had lost about a pound from the surgery. Growing up, thankfully I was very healthy. I rarely did treatments and I felt like CF was kind of pushed to the sidelines and not really addressed in my family/home until I was in middle school & high school when the Vest came out. That’s when my parents tried to get me to do more regular treatments, which was not fun at that age!
I finally took charge of my own health when I went away to college and met my now-husband, Tim. I spent the next eight years doing all of my treatments, eating healthier, and exercising to be the best version of myself for him and our future family.  

Deciding to have a child when you have CF is a very difficult decision. Please share how you came to the decision to ttc.
For Tim and I, it was never a discussion of if, but more of when. After speaking to my CF doctor in college about getting myself healthier, we decided we’d start trying after we got married.

TTC can be a difficult journey for many women with CF, how was the ttc journey for you?
Long! We stopped all birth control and let nature do its thing after we got married and thought I’d be pregnant within a few months. After several months off birth control, I noticed that my periods were still very irregular,  so I talked to my OB who referred me to an infertility specialist. He refused to work with us until we got Tim tested to see if he was a CF carrier, which really made me mad because I felt that was our decision, not his. Either way, we finally got Tim tested and he is not a carrier.Smiling face with smiling eyes

How did you decide you needed intervention? What treatments did you receive?
Shortly after getting Tim tested, we moved three hours south where I got my first teaching job teaching Kindergarten! We started seeing a new fertility doctor, a much nicer and well-informed doctor, referred by a coworker. He did several tests and determined that my Fallopian tubes were filled with an embryo-toxic fluid (hydrosalpinx), that they needed to be removed and that Tim and I were only going to be able to get pregnant through IVF.
I had laproscopic surgery to remove my tubes, but thankfully only one needed to be removed. There was no sign of hydrosalpinx, but a significant amount of scar tissue (most likely from my meconium surgery at birth) around my tubes which damaged the one on my right side. My left tube was perfectly fine which meant we did not have to go through IVF to get pregnant!
After the surgery (February), we tried a few months with Fermara (drug to help me produce mature follicles & ovulate) with no success. Then we did three IUI attempts, and the first one (July) ended in a very early miscarriage, second one (September) didn’t work at all, and the third one (November) worked perfectly…and now I’m 31 weeks pregnant!

What were some of your biggest fears regarding the pregnancy itself? So far, have those fears been warranted?
I was very worried about being able to gain weight during pregnancy since that’s been a difficulty for me my whole life. I was also worried about losing a significant amount of lung function while pregnant. Thankfully, I’ve gained 17lbs so far and my CF doctor and OB are very pleased with that. I have lost just over 10% of my lung function, but surprisingly my lungs have been feeling excellent and I haven't really noticed.

How has your pregnancy gone so far? What have been some of the biggest challenges in your pregnancy so far?
My pregnancy has been absolutely wonderful! I have loved every minute of being pregnant! I didn’t have any morning sickness or nausea which definitely helped me gain weight from the beginning. I really didn’t even feel pregnant until about two weeks ago when I started feeling big and a little uncomfortable. But even now, I still have a good amount of energy and I really can’t complain.
The biggest challenge, for me, has been maintaining my lung function. I was on Kalydeco when I first got pregnant, and my CF doctor and I decided to stop it at 7 weeks, just to be on the safe side. I felt great for about six-seven weeks until I ended up getting sick and my lung function took a dip. My FEV1 went from, low 80s to 69% (lowest I’ve ever seen it), so I started the Kalydeco again (at 14 weeks) and did a round of Tobi, then Cayston which made me feel much, much better. My lung function, as of last week, still hangs in the 70% range, but my CF doctor is happy that I’m maintaining and not losing anymore.

How do you feel your pregnancy is different that a non-cf pregnancy? What are your doctors doing in order to ensure your pregnancy is healthy (more doctor appts, change meds, etc)?
I’m seeing a wonderful pair of High-Risk OBs. I was going every two-four weeks, but now that I’ve hit the 30 week mark, I’m now going weekly until I give birth. They are excellent doctors and are great at taking my CF into account with the pregnancy. They have told me multiple times that I’m the healthiest CF pregnant woman they've seen, and they're so proud of how well I take care of myself. Smiling face with smiling eyes  
I’m also monitoring my blood sugars very closely. I was testing four times a day (fasting & 2 hours post meals), but since I was doing so well, they let me cut back to twice a day. I am also going to see my CF doctor more often now, too. I saw her last week, then I’ll see her next month and possibly two weeks after that. This one's a little harder because she’s three hours away, but thankfully I’m on summer break.

What are some of your concerns about mothering and CF?
To be honest, I’m worried that I won’t be alive to see my child(ren) graduate high school, college, get married, meet my grandchildren, etc. This is something that’s in the back of my mind, but it’s also something I use to motivate myself to keep doing my treatments and take care of myself. For the longest time, my motivation to stay healthy was to get pregnant. Now that I’ve achieved that goal, my motivation is to be the best & healthiest version of myself for my husband and our daughter. 

I’m sure its going to be tough, especially in the beginning, fitting in treatments with caring for a newborn, but my husband understands the importance of my treatments and I know he’ll help me out whenever I need.  

I also don’t want my daughter to be scared of my CF. I wanted her to be well informed and well educated about my disease. I’m sure we’re going to have those difficult questions and discussions, but I plan on being open and honest with her.

If you could give advice to a CF woman who is ttc or is pregnant what would you tell them?
Take care of yourself first! Your (future) child deserves to have the best version of you possible. If you're in the ttc/fertility process, don’t give up! Reach out to anyone of us bloggers, or others in social media, because chances are someone has gone through a very similar experience and will help you not feel so alone. And as hard as it is, be patient... it’ll happen when its supposed to. Of course its easier for me to say that now that I’m pregnant, but trust me, it’ll all work out. And when it does happen, it’ll be SO worth the wait!

Colleen's water broke just 12 hours before her scheduled induction! She gave birth to a healthy baby girl on August 8th weighing in at 6lbs 7 Oz and 19 inches long.

To read the stories of ten 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

Thursday, June 12, 2014

24 Months

My Big Girl,
You are two years old today, my love. My mind struggles to remember the days you were so helpless and small. These days, you have to do everything yourself no matter how long it takes. You spend your days chattering to me and sharing your thoughts. You still find clothes a nuisance and can't figure out why we have to wear them. Of course, sunglasses are a must since you have always been sensitive to the sun shining in your eyes. It isn't unusual to find you parading through the garden completely naked with your sunglasses picking veggies along the way. It is a sight to be seen!

Your favorite activities include helping me do chores and cook or anything that involves copying exactly what I am doing, often making my job a little more difficult, but a little more fun at the same time. You also love taking walks before the heat of the afternoon. Of course, nothing can beat spending all morning and evening in the garden. You make yourself busy digging holes, picking produce (especially tomatoes and strawberries), watering plants, and finding bugs.

Little one, there isn't a single day that passes that I am not eternally grateful to be your mother. To have the chance to watch you grow into the beautiful person you are becoming. I wake up every morning knowing I am the luckiest mother in the world to call you my own!

Monday, June 9, 2014

What It Means To Be Different

The other day I was sitting with a good friend of mine chatting while sipping coffee (hers was decaf- she is trying for baby number 2). We have been friends for years and we used to be so similar, we were always in the same stages of life. We went to college together and spent countless hours studying side by side, got married less than a year apart and shared and planned every little wedding detail together, had our babies 3 months apart and took yoga together, went to the same midwife, and eventually breastfed, burped, and soothed our babies in each other's homes. But somewhere along the line my life stalled out and hers continued forward, I got left behind. I are no longer on the same page. I am not sure I belong in the same book anymore.

You see, when we get together she chats about what most 30-something year olds chat about, things I should easily relate to. She talked about how excited she was that they are trying for baby number two. How she wants a large family- 3 or 4 total. She want a house full of kids!
I kept thinking how I will never be healthy enough to have another baby. My husband and I don't have the luxury to discuss how many kids we want because CF took that choice from us.

She was chatting about how she found the perfect school to send her son to. One that does amazing enrichment programs and has a great student teacher ratio.
I kept wondering if I would live long enough to see Kaylee go to kindergarten. With my lung function so low and the fear of sickness lurking around every corner. I wonder how much longer i will be around to raise my precious little girl. Please, oh please, let me see her go to kindergarten. I want to at least live long enough for her to have memories of me.

She was talking about how her and hubby want to take a little vacation in the mountains, you know to get away from it all.
I was thinking about how my husband absolutely loves going to the mountains, but how I could barely breath in the high altitude when my lung function was in the 50s. I can't fathom the struggle with 30%. My husband's favorite vacation spot is a place we can't go anymore. Maybe after I die he can go with his new wife...

She was talking about how she was a crazy teenager and that as hard as raising a toddler is that raising a teenager will be 1000x harder.
I kept silently praying that I can know what Kaylee will look like as a teenager. I want to know if her hair stays curly, if her eyes shine just as bright. Will she be smart and studious, athletic, artist? What will her friends be like? What class will she hate? Which will she love? Oh. I don't care if she makes me drop her off at the end of the street because she is humiliated to be seen in the car with her mom, I just so desperately want to know if that dimple in her left cheek will still be there when turns 16.

I agree and nod and say all the things I am supposed to say. But I don't understand her world- your world. It is not the same that I live in. I do not speak of my horrid reality, it is one you do not wish to know. Nobody wishes to see the world I live in and so I live in it by myself. I think these thoughts, but they will never escape from my lips. Your world has you too preoccupied with the wonderful possibilities your youth gives you, of the vast and open life that lays ahead. My world is rapidly collapsing on me and I won't be so wretched as to invite you in. Nobody deserves to be here.

For now, I will pretend. Pretend to understand the excitement of being young and free. I will pretend to understand what it feels like to know your life has just started rather than wondering how rapidly it will end. I will pretend my life is like yours when in reality I know nothing of the life you live and in reality you know nothing of mine.

Sunday, June 8, 2014

Early Mornings

My favorite memories are always made in summer. The cool crisp air of mornings in my garden. The chatter of Kaylee talking to lady bugs and dragonflies which frequent our little patch of land. The wide open flowers reaching to the low laying sun, letting me know that a crop will be here shortly. The sleeping bees tucked in the brilliant orange of the zucchini flowers. The basket full of the days vegetables and herbs. The tug of Kaylee's hand asking me to "see ready??" her favorite vegetable of the day. The excitement a ripe tomato or raspberry elicits from her tiny spirit. The feel of bark chips beneath our shoes and soil between our fingers. The warmth of the sun on our backs as the morning continues on. The smell of mint and basil filling the air. My favorite moments are always found in the early morning of summer.

Saturday, June 7, 2014

I Will Miss These days

I was looking at pictures of you that were taken when motherhood was still so very new to me. As I looked through the pictures of the first few months I felt pangs of sadness, of longing. I so desperately wanted to reach into those old photos and pluck that chubby baby from the arms of my former self and cuddle her. I wanted to pinch her little baby thighs and kiss her chubby baby cheeks. How can you miss someone that is still sitting right next to you? I realize there will be a day, not long from now, that I will miss almost-two-year-old Kaylee. I will want to pull that toddler from old photos and cover her entire curious face with kisses. So here are some of the things I wish I could hold on to, but like everything else I know one day these will be a distant memory.

  • How you tell me "do it myself" a million times a day, but somehow it sounds more like "do it applesauce".
  • If you hear me coughing you always stop to ask in the sweetest voice, "pop pop, mama?" (pop pop is CPT) And if I say yes, you diligently pat my back until I stop coughing.
  • When you have an itch you tell me, "Back is itchy" then after you scratch it you shout with the enthusiasm only a toddler can have, "Mano (hand) GOT IT!!!!"
  • That you think the dragonfly that comes to our yard everyday comes to visit you specifically. You remind me everyday that he comes to see you and that he likes you a lot. 
  • Whenever you see me doing something that your aren't allowed to do (cut with knives, use sharp scissors, etc) you always say, "Very careful, Mama."
  • You appreciate my sense of syle. After I spent all day painting our bathroom you came in and exclaimed, "Oh, niiice azule!" or after purchasing a new rug to put by our sliding door you told me, "Nice blanket!" 
  • The compliments you give, often out of the blue, "Mama, soo cute!" or "Mama, soo nice!" or "Mama so funny!"
  • You love me no matter how much of a hot mess I am. After wearing a frumpy t-shirt the other day after rolling out of bed you said, "ooooh, mama! Nice dress!" and you actually meant it.
  • You give me so many kisses a day I think I have a lifetime supply. I cherish each on knowing someday you won't want to kiss me, especially on the mouth, anymore. 
Oh little one, you will never fully understand how much love and joy you bring to my life. I can't wait to see what the future brings with you!

Monday, June 2, 2014

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!