Change

The last few months I wasn't able to blog much due to a rather ancient computer that tired out and left me hanging high and dry. I am back online and hopefully this computer will stick around for a while. This past year seems to have been a blur of sickness, meds, and doctor appointments. As the new year approaches I can't help, but silently hope and wish for a healthier more stable 2015. And yet, in the back of my mind I hear the words I spoke at the end of 2013, that I hoped for a healthier and more stable 2014. I have been thinking a lot, often while sitting in the doctor's waiting room, about my health and the lack of control I have over everything. How despite being compliant and aggressive with treatments my health seems to yo-yo in ways I never imagined possible.

In recent months I have seen gains I only dreamed of in the height of sickness last April and yet just as quickly as those gains come, they seem to disappear and my health tumbles to new lows. This taste of healthier days, filled with more energy, more endurance, the ability to exercise tease and taunt my very soul, "Look how wonderful it is to be able to jog on the treadmill and feel your legs tire below you and how amazing it feels to gulp that oxygen into your open lungs. Feel how liberating it is to walk all afternoon without that all too familiar feeling of drowning. Notice what it feels like to be everybody else. Now just wait, because tomorrow you will open your eyes and for no reason your lungs will burn, your breath will be shallow, you will struggle to walk to the bathroom and you will question your sanity that this amazing day with a chest full of healing oxygen and a body that seemed to function just as it should even existed."

This has replayed over and over the past several months. Making me crazy with anticipation for when my health would return and fearful of every minute that passed that I felt well knowing all too well it would disappear with a blink of an eye.

And so I am learning to be grateful despite the endless sickness, the tiredness I can't shake and the infections looming around the corner. I need to learn to be grateful or go crazy with the instability, lack of control, and constant fear. I am adjusting my expectations and learning to enjoy the good even if there seems to be so much more bad. The days I find my breath shallow and labored I remind myself to be thankful that the fevers have stayed away for weeks. On the days I can exercise well I try not to think what tomorrow will bring and enjoy that I was able to exercise at all. I try not to look into the future, but rather stay in the moment because a good morning does not guarantee a good afternoon and a healthy few days does not mean that this disease is backing off.

The chronic, never ending feeling this disease brings can be exhausting. Thinking of my future, the slow decline, what my life will be like at 35 or 40 can feel overwhelming. I can't even begin to picture myself 15 years from now. And so for now I will take it day by day, moment by moment. I would love to wish for a healthier 2015, but instead I will hope for a better perspective, a healthier outlook on life. I will always hope for healing and stability, but knowing CF is chronic and ultimately fatal I will not hold too tightly to that hope. Instead I hope to enjoy each day that comes and find the good in every day regardless of my state of health. So much of our lives are so completely out of our control and often the only aspect we can control is our attitude. So my lung function may or may not be higher in 2015, my lungs may or may not struggle to breath more often than not in 2015, and my bacteria may or may not continue to win over precious real estate in my lungs, but I do know that I will enjoy 2015, CF be damned!

Two and a Half

Dear Takey,
You are hurling towards three and I am just trying to keep up with how fast you are growing! You spend your days trying to be  as independent of me as possible only to run back in my arms and snuggle close asking me to carry you "like a tiny baby". You are discovering who you are and who you want to be testing your wings (and at times testing me!)

A trait you have had since you learned to talk was bossiness! You also are a rule follower and you like to enforce those rules (hence the bossiness). On playdates you are quick to tell your friends what isn't acceptable, "No climbing on the table!" You shout to squirrels or cats that may wander in the road ensuring they understand the rule is, "You have to hold hands when crossing the street!" You also are wildly bossy (in a loving way) to your cousin. Recently, at my parents the two of you went outside and when your cousin ran to the (locked) pool gate you ran after him.
"Stop! Stop! Look me in my eyes. Do NOT go in the pool!!" Needless to say we are working on respectfully telling others the rules.

Your imagination is bigger than life! You pass in and out of pretend and reality that i often confuse which is which. Like when you shouted there was a black widow in your cup. I ran with the speed only a worried mother (with crap lungs) could run only to watch you coo into your cup at the imaginary black widow mommy and baby pair. There was also the time just after you turned two (before i realized how much time you would spend in an imaginary world) that you shouted to me that it was too dark to see. We were in the sun filled living room and I had slight concern for your eyesight. When I inquired you explained, "I pushed this green button on the wall and now it is dark!" and then just to be sure I understood you assured me, "Mama, it's just pretend".

You are wildly independent and refuse help when going to the bathroom. You use a chair to turn on the light, hoist yourself on the adult toilet, you flush and even turn off the light yourself. Other times you ask me to cuddle on the couch and pretend you are a tiny baby. It this dance of running from me to be independent only to run back for reassurance. It is wonderful watching you grow and change, but I am glad my arms are still your favorite place to be.

Where We Started

I havent updated in a few months. I am currently without a computer and trying to type without a keyboard is challenging to say the least. However, I do want to update.

My last post life was turning around after a grueling 18 months of endless setbacks. I felt I was finally getting a hint of my old life back and started, just started, to feel some hope for the future with my husband and daughter. Oh, but life is cruel and less than 2 months later I am back to my lowest lung function, 30%.

About two weeks ago after finishing another round of antibiotics I started waking in the morning with extreme lung spasms. The spasms were so bad that I would be on the floor, coughing, choking, and throwing up unable to function until my lumgs calmed down again up to an hour later. I knew it wasnt an infection because once ,y spasms would stop I would be fine the rest of the day. I went to the doctor and he believes I was experiencing acid reflux at night that was actually going into my lungs causing the extreme discomfort.

The good news is after one dose of antacids my horrific spasms ceased. However, according to my home FEV meter my lung function hasnt changed. You win some you lose some, I guess.

No More Cough Cough

I have been doing really well keeping up with my exercise routine to the dismay of my daughter. She started crying whenever she saw me put on my exercise clothes. The other day I sat her down and explained that exercise helps mommy's lungs so that I don't cough so much. She seemed to understand and whenever she starts to get upset at the prospet of me leaving to the gym we talk about why I go to the gym and she usually calms down.

Last night, I had a horrible night cough and spent most of the night awake hacking away. Around 5am I found little Kaylee at my bedside where she whispered, "Kaylee exercise with mama so no more cough cough."  She doesn't actually know what exercise is, but she knows it helps me stop coughing. Despite waking her up two hours early because of my cough she came in my room full of love and a solution. I love this kid more every single day.

6 Weeks Later

About a month and a half after my first positive appointment in about 18 months I had a follow up appointment. To say I was nervous is an understatement. I felt much better health wise than when I was at my worse, but wasn't so sure I was any better than my previous appointment 6 weeks ago. When my health improves or declines slowly I seem to adjust with the change and it can be hard to measure the differences I feel. "Did I have this much energy two weeks ago?" "Was I coughing more than this after swimming last weekend?" can be impossible questions to answer because these changes can be so subtle.

So Monday morning as I waited for my PFTs I kept reassuring myself that as long as I didn't lose lung function I was okay. I had energy again, I was swimming again, I maintained my weight without supplements (I stopped Megace and due to an insurance issue didn't have Ensure). So although I wanted 35% (the goal I set for myself) I couldn't be too upset as long as I blew a 33.

2 months ago my PFTs were in the high 20s. 6 weeks ago I hit 33%. Last Monday I surpassed my goal of 35% and saw a beautiful 39% flashing on the screen!!! My highest PFT since getting the flu including my PFTs after all my IV abx and hospital stay.

And so this is officially my come back! My weight is stable despite being off Megace, my PFTs are climbing, and my monthly clinic visits were changed back to the typical once every 3 months. Watch out 40s here I come.

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..."

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)."

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%!!!***

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.

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.   

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

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!

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.

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.

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!

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!

The Right Direction?

I am not superstitious, I really am not. And yet, I feel nervous writing this in case I jinx myself. In case, by writing these words for others to read it will make me unworthy of what is happening and everything will vanish as mysteriously as it came. But as much as I am currently doubting it, I am in fact, not superstitious so here it goes.

There has been a shift. A subtle shift. It is not that my breathing is necessarily any easier and I am not sure if my lung function increased at all, but there seems to be a difference in the way I feel each day. I am noticing I am doing so much more throughout the day and not feeling like I want to fall into bed long before my daughter is tucked into bed.

I can function like a human being again. I can make it through more than one outing without feeling like all of my energy is completely drained. I feel like I am slowly crawling away from deaths door, because really I was hardly living a life anymore. Not that I was deathly ill all the time, but it seemed that when I was "better" I wasn't my old self and it would only last a week at most before I felt horrific, completely useless.

Today for example, I ended up taking a nap for about 40 minutes. However, this nap allowed me to have enough energy to work in the garden, make homemade pesto and homemade ice tea (using herbs from our garden for both), and homemade mounds bars for dessert. I then did the dishes, washed the windows, vacuumed the whole house, wrote this blog and did my nails. And you know what? I am still standing and don't feel exhausted yet. A month ago, I would need a nap and still not be feeling well enough to do much all day besides caring for my little one.

I am also realizing why I may have been so sick. I feel like we, the doctors and I, were baffled at why I couldn't get better. I think there were some obvious issues like my immune system and body still recovering from the flu which resulted in continuous colds and illness. My body was repeatedly getting knocked down. However, I am almost positive I had an infection deep in a pocket of my lungs where mucus is completely trapped. The fevers were an obvious sign of infection, but oddly enough my mucus was thin and light in color so I never felt like I actually had a respiratory infection. I would feel better after abx (although my mucus already looked "good" so it never seemed to change much), but I would seem to fall sick again almost immediately with a cold that would result in a fever. I think I wasn't being treated long enough for how bad the hidden infection was, but since I had none of my usual obvious signs of respiratory infection we never thought to keep extending the abx. My increased airway clearance and nebs weren't touching the area of my lungs that were infected so they weren't making a difference.

This last appointment, I asked to stay on my meds (Levaqui and Bactrim) for 3 weeks instead of my usual two since it seemed that I would just start to feel "better" when my meds would end. I also started to worry about the lung pain I kept feeling on my left side which seemed to be a sign of infection. I think the 3 weeks of antibiotics plus the added weight gain is what may have finally given my body a fighting chance.

Like I said, I can tell my lungs aren't even close to where they used to be and I know it will be a long road ahead, but at least for now I feel like I can live my life the way I am supposed to with my amazing little girl and my wonderful husband. I can enjoy the daily aspects of life again and what could be better than that!

Homemade Larabar Recipe

I am venturing out to try something new for my blog. Today I am sharing... a recipe!! I am sharing this recipe because it is delicious, high fat, high calorie, delicious, easy to throw in your bag or purse, my entire family loves them, oh and did I mention delicious? Now if you Google Larabars you will find tons and tons of recipes, BUT there is actually a technique to them and it took me a while to perfect the Larabar so I decided to share my recipe that has been tweeked many times in order to make the perfect Larabar!

Homemade Cherry Pie Larabar
1c raw unsalted almonds
1 c dried unsweetened cherries
1/2 c pitted Majool dates
1T water

You will also need the "s" blade on your food processor.
I always double the recipe because they disappear so quickly in my house AND they freeze really well. In fact, I freeze them immediately after making them and take them out as I want them. They don't freeze solid so you can munch on them straight from the freezer if you choose. 

Put 1T water in your food processor (don't soak fruit like some recipes recommend because the texture will be super icky!) and slowly add cherries and dates. Keep pulsing until the cherry/date mixture sticks together and forms a sticky ball.

See how the cherry/date mixture is clumped together? This lets you know it is time to take the sticky mixture out.

Remove cherry/date mixture and set aside.

Cherry/date mixture waiting to be used.

 Place almonds in the empty food processor and pulse till nuts are at a rough chop (processing the nuts too much will release their oils and give you oily bars). 

Rough chop of the almonds

Slowly add the cherry/date mixture to the nut mixture. (This step is crucial because if you try to dump all the cherries at once it will burn out the motor on your food processor. Happened to me more than once, yikes!)

Almost done!

Remove nut and fruit mixture and place on wax paper.

It will seem crumbly at first, but as you work it the mixture will become stickier and hold its form

Use the wax paper to form it into a ball (it will be sticky so the wax paper keeps your hands cleaner!)

Flatten ball with your hands. Cover flattened ball of fruit/nut mixture with wax paper and use rolling pin to get desired shape and thickness.

Use a sharp knife to cut into squares or rectangles. My double batch makes 12, but I like to cut them big.

K and I always share the scraps from making the edges straight. It is our reward for all our hard work.

Wrap in wax paper, stick in ziplock bag and freeze (or refrigerate) and you are done! Make sure you enjoy one (or two or three) before you freeze them though!

All ready for the freezer!

This snack is healthy enough for anyone in the family to eat, but full of good fats which is great for us CFers. It is easy to bring along as a quick snack on the go and they freeze really well so you don't have to worry about them spoiling (not that they will last long anyway). Even my hubby who is not a snacker can't refuse a homemade cherry larabar and don't even mention them around me toddler, she is obsessed!

Enjoy!

When Running Is No Longer an Option

Part of my current goal for my health is to gain weight, but more importantly to gain muscle. I am on an appetite stimulant and am quickly gaining the fat I lost through being so sick, but now I have the job of using that energy and calories and gaining as much muscle as possible, since it is a better weight to have. Muscle is much harder to lose than fat so I want to gain weight, but mostly in the form of muscle. The question is how to gain muscle and build my lungs when my lung function is so very low. I took for granted how easy it was to work out with 50% lung function. It seems like an unbelievably daunting, almost impossible task, when your lung function is so very low.

While discussing working out with my doctor last clinic he told me with the most empathetic tone that he doesn't think I should jog anymore. He feels it is too much of a strain for my body and I need to try things that build muscle, but are low stress on the body such as yoga, pilates, weight training on weight machines, etc. He was so sweet in his delivery, worried I would be upset being told I could no longer run, but in my head all I could think was, "YESSSSSSSSSSSS!" 

I started running so I could quit the gym because we needed to save money. Being sick is expensive! Co-pays, prescriptions and my hubby has had to take more time off work than he has sick days for because of my hospital stays and frequent sickness so we have been extra tight when it comes to finances lately. But in all honesty, I hate hate hate running.

So as my doctor requested I rejoined the gym. I had already started going to yoga again a few months back. I have to say being back at the gym and being back in my yoga studio makes me feel so much healthier. I know it hasn't been long enough to actually make much of an improvement, but being in the places I used to go to when I was my healthiest (right before I had K) makes me feel like I am that person again, the "healthy" person, the strong person, the fit person. I feel a new sense of hope, being in these places knowing that each day I am slowly, but surely making my body, my lungs, my spirit just a little bit stronger. Maybe, just maybe someday I can be that healthy strong person again!

Megace

I mentioned in a previous post that along with my lung function plummeting so was my weight. My BMI was getting close to the "underweight" category and the doctors thought that bringing my weight back up would help with my overall health and therefore help with my lung function. I can eat fine (about 3000 calories a day) when I feel well, but when I am sick, which is happening frequently now, I have absolutely no desire to eat. I also often didn't have the energy to really cook or make myself anything to eat when I was feeling feverish or ill. So I asked the doctors for an appetite stimulant to help me get out of my rut. Oh my goodness, I had no idea what I was in for.

The first day I took 2 of my 4 doses since I didn't receive it until late afternoon. That very same day I started to feel a little hungry. Within two days I felt like I always wanted a snack. I started opening the fridge so many times that Kaylee must have thought I had gone crazy. I was eating nonstop and still woke up starving in the morning. I think it helped that I was coming off of three weeks of antibiotics so I felt well. The combination of feeling well and having an appetite stimulant made me a ravenous pig!

I kept asking my husband if I looked fatter, a loaded question in most relationships, but I was hoping I looked a little more roundy. He kept laughing at me telling me it couldn't work that fast. Oh, but darling it did! I was 110 pounds (I am 5'4" for reference) last Monday when I started Megace. I am currently 117lbs only one week later! That is a pound per day! I actually was checking in the mirror today for stretch marks because I was worried I was gaining weight too quickly, but luckily found none. I only want to gain about 8 more pounds, but at this rate I will be there by next week!

I feel like I look better, I felt skeletal and weak when I was 110 and even the seven pounds has made a difference in my appearance. I also feel so much better. I am not sure if it is psychological because I look healthier so I feel healthier, but I do feel less weak and frail. Having an appetite makes me feel more normal as well. I may need to pick up a part time job to pay for all this food I am eating though!

23 Months

Dear K,
You are 23 months old today. Only one month before you, my baby, turn two! I am so proud of the little girl you are growing up to be. You really are turning into a little girl. The toddler in you is starting to fade and although I sometimes wish you wouldn't grow so fast, I love learning about the person you are becoming each and every day.

You recently learned to tell me your emotions, sad, happy, mad, and we are working on frustrated. This has made both our lives so much easier. When you have the words to tell me how you feel you don't need to act them out so much and we can work through them together.

Things You Like: You like manners. I know that sounds strange, but you expect people to use their manners around you. You are good at remembering to say please and thank you. You also say, "salude!" when someone sneezes and sorry if you accidentally bump into someone. You also like formal greetings and I often hear you making your stuffed animals ask, "Como estas?" and the response is always the same, "Muy bien, gracias" You also expect others to use those same manners with you, as you should. The other day while distracted I asked you to hand me something. You did and then standing at waist height your blue blue eyes looked at me with a little disappointment and you said, "mama, gracias?" Sometimes mamas need reminders too.

Things You dislike: The sun. Well, you actually like the sun, announcing each morning that the sun (solecito) is awake as if there was any doubt the sun would rise, you get excited when the sun dries your clothes, and when the sun shines through your prism in your window creating rainbows on the wall. What you despise is the sun in your eyes. You have little sunglasses that we have to carry with us wherever you go just in case the sun decides to shine too bright. You look like a rock star in those glasses so I am grateful for sunny days.

New Words: You have almost mastered complete sentences. Well, you do speak in complete sentences, but they often have errors and you drop a lot of the smaller words. You also add "the" often when it isn't needed such as, "Kaylee is on the dada" We all let our voices get a little higher at the end of a sentence when we ask questions, it is just part of speech, but baby girl, your little voice gets so high pitched when you ask a question it is hard not to laugh. Sometimes I act like I couldn't hear you just to make you ask the question again. Too stinkin cute!

Your Spanish is improving and you seem to be figuring out when to use Spanish and when to use English, You will often use the English version of a word with me and the Spanish version with your dada or when in Spanish class. There are still a lot of words you only know in Spanish such as colors, numbers, the alphabet, animals, etc but you are very aware that most words come in two version, English and Spanish.

What I Like About This Age: I am in LOVE with this age. I told your dada the other day I wanted to have another baby, but give birth to them when they are 20 months old. The past 3 months have been my favorite. You are so easy to please (for the most part), a great helper with chores and cooking (although it does take twice as long with my little helper), easy to entertain, extremely independent, and a great little talker. I am a little nervous for the "terrible twos" to approach, but I am sure we will figure it out as we go!

I do miss naps, but I am adjusting to your nap free lifestyle and my lack of breaks.

Port Flushes

I am not going to lie, sometimes having a port is a pain in the butt. Mainly trying to coordinate port flushes when you have a toddler, a husband with an insane work schedule, and life in general. This past month was the worst as schedules kept changing and I had to call and reschedule my appointment twice only to have my husband tell me that the final time I decided on didn't work either. I was approaching 6 weeks without a flush and decided that I could not risk another week and brought Kaylee with me. I couldn't get anyone to watch her and she really is such a mellow kid I figured it wouldn't be a problem.

Poor little Kaylee fell asleep in the car. I had to pull her sleeping body out of the car into the bright sunshine and carry her to the somewhat busy cancer center. She was awoken by the shuffle of the center and was groggy and disoriented when I signed in. The receptionist looked at Kaylee and asked if I had someone to watch her. When I told her I couldn't get childcare she explained that they don't allow children under 13 back where chemo is administered and a nurse would have to come and watch her while I got my flush. My daughter is social and quite charming, but she is not the type of child that will gladly leave mama to go to a stranger. In fact, I was positive hysterics, especially given her sleepy state, would ensue.

And just like that, I cried. Not in front of the receptionist, but in the waiting area. I am really not an emotional person so I surprised even myself, but there I was silently crying in the waiting area. I just felt bad. I felt bad for making my baby miss her nap, I felt bad for dragging her to so many appointments, I felt bad some stranger was going to take her from me, I felt bad that I have been so sick for so long which effects my energy level and my mood with her, I felt bad that CF already inconveniences her life so very much. I guess it all built up and poured over in that stupid cancer center waiting room. Luckily, only one person witnessed my moment of weakness and he uncomfortably avoided eye contact with me. I guess if you have to cry a cancer center is a pretty common place to do it.

By some act of kindness the nurse assigned to me snuck Kaylee back with me and we sat side by side in the infusion chair. Kaylee read her alphabet book, telling both the nurse and I what each letter was and what picture was on each page. At one moment she looked over at the nurse working on my port and said in a matter of fact tone, "clean, clean mama port!" She went on "reading" to us until my labs were done and port was "clean clean".

Kaylee reminds me over and over again that even when I feel bad for putting her through so many things most *almost* 2 year olds never experience that she is just happy to be with mama. She finds joy in life whether in an infusion chair or a busy doctor's office. She reminds me small children are resilient and can easily find joy and excitement even in the most boring situations. She is helping me to notice the beauty in all of life's experiences both good and bad. Everyone knows a mother spends her life teaching her children, but children teach their mothers the most important life lessons.

Toddler Tak Part 2

K does really understand what the word dry means, but uses it often to mean something that is not hot. K and I went on a date for coffee (for me) and croissants. So here was this little girl, not yet two, sitting in a coffee shot biting a croissants saying, Oh, this dry!!" She seemed like quite the snob!!

Every time she poops in her potty she always says, 

"Yay, poopoo! No caracol (snail in Spanish), poo poo." 

Thanks, for the clarification, Kaylee!

My husband and I were holding each other close dancing around the living room. After a few minutes we stopped to get on with our lives. 

K started shouting, "Boob boob on!"

We couldnt figure out what you wanted (especially since you are now weaned) until you pushed us together chest to chest (boob to boob) and said, boob boob on. I guess you liked our dance moves.

K was showing her dada President Lincoln and said, "Lincoln!"

My husband: "Yes, that is Lincoln." 

My husband has an accent and so the way he says Lincoln isn't really the same was I do. 

K upon hearing him say Lincoln her brows furrowed, "No, Lincoln!" 

Hubby, "Yes, Lincoln" (with his accent obviously). 

Kaylee, upset, "LINCOLN!" 

Me: "Just say it like a white person" 

He exaggerated an American accent, "Lincoln" 

Kay smiled, "Lincoln! No, Lincoln (with his accent) Dada!" 

Totally rude had it been an adult, but coming from an innocent kid it was really funny.

Happy

Riding along the river, stopping to feed the baby ducks, watching the turtles sunbathe, and looking for fish under the greenish blue water the sun is shining bright. It is a beautiful Spring day as I ride my bike down the path homebound after a full morning of exploring all the river has to offer. Kaylee is the trailer behind me I peek back to see her lounging, feet propped up on the side of the trailer, sunglasses on and a water bottle in her hand.

"Mama!" Kaylee calls to me.

"What baby girl?" I ask turning my head slightly in her direction.

"Mama, Taytee happy!"

"You know what baby girl? Mama is happy too!"

And we were, happy in each others presence, happy in the sunshine, happy with the wind in our hair, happy we have one another to enjoy our days together. Days like these are what motherhood is all about. 

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.

Guest Blogger- Beth

We have another CF mommy-to-be to introduce today! Beth is from the blog, The Overdeeps, where she chronicles her pregnancy journey. She discussed everything from dealing with CFRD while pregnant to nursery preparations. She has tons of cute pictures too!


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

I was diagnosed when I was 4, after my sister and I had been sick for years. She was 6 at the time of our diagnoses. My lungs were in pretty decent shape but I suffered greatly from being pancreatic insufficient. 

I managed to stay relatively healthy and avoid the hospital until high school. I started culturing MRSA when I was 18 and saw my health really take a hit. It took moving to a new state & clinic to really gain motivation to kick my sick butt into gear and I managed to get my health back up.

I continued to improve, especially since starting Kalydeco. I'm now the healthiest I've been since I can remember (we're talking numbers I haven't seen since probably I was 10 or 12) which is a good thing, since I'm pregnant!

Deciding to have a child when you have CF is a very difficult decision. Please share how you came to the decision to try to conceive.
I've always wanted children. From a small age I was always enthralled with babies. I started babysitting and nannying in high school, and I ran a few children programs out of the public library. I was just good at taking care of children, it was very natural for me. Having children of my own was not really an option in my mind; it was going to happen.

I met my husband in high school-- we started dating our freshman and sophomore years, but I dumped him a few times along the way and we ultimately got back together once we headed off to college. He was and is, my best friend. He always knew my strong desires to have a baby - but he is a very logical, sensible person and always reigned my "I want it now" madness in.

We had to compromise on our timelines. He wanted to graduate, get a PhD and work for a few years before having children -- with his timeline we were looking at early 30's. My timeline was "As soon as you graduate undergrad, knock me up!" Early twenties. Needless to say we both agreed that waiting so long was much riskier, but we needed to be very comfortable in a few areas before getting 'knocked up.'

We decided that once he had graduated undergrad and had a better idea of what our future held, we would talk about trying for babies. He ended up getting into a PHD program and I had our years mapped out to a T. That of course, never happens the way you plan, so my 1 year and then a baby plan turned into a 2.5 year plan. I worked as a nanny in the new city for 2 years and we saved every penny I earned. I concentrated on getting my health up, and with the help of Kalydeco I started seeing numbers I hadn't seen in over a decade. Things were looking good! In the end our decision to have children came down to a few major factors: 

1. Would we be settled for the next couple of years in one spot? (Passing a PhD qualifying exam was the dealbreaker for this question. He passed, so we knew we would be in one spot for at least 3 more years).
2. Did we have enough comfortably saved up to live off of for 3+ years while we cut our income from two down to one and upped our household from 2 to 3. -By saving my entire salary for 2 years, and being good savers from the start of our relationship, we felt we had a comfortable savings cushion to answer yes.
3. Was my health in stable condition? I had gone the longest without needing IV's since we moved and since starting Kalydeco. I went 18 months. I was averaging 6-9 months previously. We decided that yes, with my lung function as high, my weight was stable and I was the strongest physically that I had been in years we thought my health was spot on to support a pregnancy. The doctors I see at clinic were fully supportive and had no concerns about my falling pregnant and carrying a pregnancy to term.

This led us to getting pregnant at 24, and we'll be parents at 25. Mid-twenties: compromise!

Trying to conceive can be a difficult journey for some CF women. How was your TTC journey?

Our trying to conceive journey involved me being absolutely crazy. I started preparing WAY before we threw the condoms away. We're talking years of prep. I focused on getting my lungs healthy, my weight up and putting on muscle mass.

Once we knew we could answer yes to all 3 major factors of having a baby, I started charting my cycle. I did temperature charts noting my cervical mucus and body signs. I was able to track my ovulation within a few days, give or take. The month we decided to go ahead and throw away the condoms I added even more craziness! I took Mucinex to help thin out my cervical mucus, I drank more water to also aid in the thinning of mucus (and just because it's better for you!). I bought some ovulation prediction kits and a bunch of pregnancy tests. I was in it for the long haul!

We had sex every other day after my period ended, and every day the week around ovulation. I ended up feeling sick shortly there after so we stopped having sex. We figured we maxed out our chances and I was certainly not in the mood.

I got all the signs of a lung infection (and hadn't had IV's in almost a year) so I scheduled a tune-up because I knew I couldn't wait. I was in the two week waiting period when you're not sure if you're pregnant or not.

I figured a tune-up now would be good if I was pregnant since I wasn't yet sharing a blood supply and baby wouldn't have hunkered into my uterus yet. Or, if we weren't pregnant I'd be in tip top shape for next month of trying!

I was giving a pregnancy test the first day I was there, only to have it be negative. We treated my infection as if I wasn't pregnant and decided if my period was late we'd retest and reevaluate based on those findings. Lo and behold my period due date came and went and my charting temperature still hadn't dropped. I demanded a blood test (they also did a urine test) and it came back positive!

After one cycle of TTC we were successful. To say my husband and I were surprised is an understatement. We both were mentally prepared for MONTHS of trying, and here we were a mere 3 weeks later... pregnant.

To summarize, I had quite the easy experience getting pregnant and I can't begin to tell you how thankful we are that it worked out the way it did. We contribute a lot of the ease with Kalydeco since it helped regulate my cycles and thin out my regular cervical mucus. My husband however, likes to brag that he's just got good swimmers. ;-)

I think being at peak health- for both of us, and at an age where you're naturally more fertile really helped tip the scales in our favor.

What were some of your biggest fears regarding pregnancy itself? So far have those fears been warranted? / What are some of the biggest challenges of pregnancy for you so far?

Some of my biggest fears for the pregnancy had to be my health issue. Was I going to get morning sickness and lose all the weight I had put on? Was my CFRD going to be crazy and get so complicated? Was I going to need IV's frequently, are there any IV's I can have? Can I stay on Kalydeco? What if my health drops drastically and I miscarry? Etc. ETc.

I think being pregnant for the first time you naturally have a lot of fears and concerns. I mean, come on they tell you not to eat deli meat and sleep on your back! And all sorts of crazy things!! Now add ontop of that the fact that you take 15 different medications to control your CF and you have an issue with diabetes already... The fears were plentiful.

I think excitement really took over and helped me through the first trimester. I had stopped working, which was great, because my biggest symptom was exhaustion. I slept almost all day and all night. I lost some weight since I had no energy to exercise, my muscle mass that I had worked so hard to pack on was slowly decreasing.

I also was newly pregnant in the start of the flu season! Talk about paranoia! I hardly left the house, and one fateful day, when I ventured out, I brought home the flu (even after getting my flu vaccine).

The flu really did me in. Luckily I was out of the first trimester at that point, so a lot of the drugs that aren't safe were usable. The flu festered into a lung infection and I've been sick ever since. I've had another round of IV's and just having come off them 2 weeks ago I'm starting to come down with another infection.

I think overall it's been easier than I anticipated, but in some ways harder. I wasn't prepared for something like catching the flu -- it could have happened to anyone-- and then having that turn into this long drawn out battle of infection. My blood sugars have been a bit of struggle to figure out and tame, but so far so good. I think the hardest part so far has been mentally. I'm mentally exhausted by over analyzing and worrying. "I haven't gained enough weight, maybe if I had done extra airway clearance I wouldn't be sick right now, I'm not eating enough, I'm not exercising enough, what if all my coughing causes early contractions, how am I going to handle a newborn when I'm SO SICK?!"

Having a great husband has been my saving grace. He talks me down, reassures me, and gives me countless backrubs.

So while my second trimester has had a lot of bumps along the way and may be harder than I anticipated, my first trimester was SO easy that I think we're averaging out to be about what we expected or even easier than expected.

I'm currently only 2/3 of the way done baking my baby, so you may have to check back with me once I've finished the journey!

How do you feel your pregnancy is different than a non-CF pregnancy?

I think my pregnancy is different than a normal pregnancy (thanks to CF) for the following reasons:

1. Catching the flu turns into a massive infection!
2. Being exhausted in the first trimester was much more damaging than I would anticipate a normal pregnancy just because we already use more energy just doing simple tasks, add in not exercising and it really affects your lungs and muscle tone which overall affects your general health.
3. Diabetes is strange. I'm not following any sort of pattern; not CFRD not GD, just strangeness. My OB nutritionist and CF nutritionist work as a team so they've been amazing!
4. APPOINTMENTS! My schedule is crazy filled with appointments.
5. This last one may be controversial, but I feel very judged. I feel since falling pregnant (and now looking pregnant) I've been judged. By hospital staff to complete strangers, to even family. I think that was something unexpected and has been hard for me. I'm not sure how a normal pregnant woman would feel - I'm sure we all feel judged at one point "Why yes she did just order a regular coffee and she's pregnant! GASP" but I think having CF really amplifies those feelings for me.

What are your doctors doing to ensure a safe and healthy pregnancy?

My doctors are great. I see a high risk OB practice (who are SO much more relaxed than a regular OB) and I see my CF team regularly. In the first trimester since I wasn't having any problems I saw everyone at normal intervals.

Since catching the flu and then harboring a lung infection, I've seen everyone much more frequently. I'd say my CF team has been way more valuable than my OB team. In fact, I tend to call them first to see if it warrants a call to the OB. My clinic is pretty large (around 400-500 adults) so the fact that I feel like I get extremely personal care, even more so now that I'm pregnant is so reassuring. Now, if only they could deliver my baby too! haha

Overall, not many of my meds were stopped. I stopped a few things for the first trimester: pulmozyme, advair, flonase. We stopped an alternating month of TOBI, but continued all my other meds. Their philosophy is 'healthy mom, healthy baby.' So far it's been working well, baby is looking great and is right on track!

As I progress to the third trimester I'll see my OB clinic every 2 weeks, then every week, along with more frequent growth scans for baby. I'll see my CF team once a month and then every 2 weeks in the final two months. OR, if things get a little haywire I'll be seeing them as frequently as needed.

What are some of your concerns about mothering with CF?
Oh man, where to start! Concerns about mothering with CF. Being a nanny for so long (and working with plenty of newborns and infants) I know what's coming. Only this time, I won't be able to hand baby back and say "goodnight!" and go home to a baby free house. My main concern is sleep! As a CFer my mood and health truly depend on getting enough sleep. Infants and sleep don't really mix, so I'm most concerned with that.

On a larger, much longer timeline/scale I'm worried about how we'll handle hospital stays and getting sick. I'm nervous about taking care of someone other than myself on top of taking care of myself.... all the time! Our nearest family member is about 2 hours away and then the rest of our family lives about 7 hours away.

I'm nervous about not being able to provide adequate attention when I'm getting sick, and I'm afraid I'll be getting sick more frequently. And is it just me, or do we all get snappy and crabby when we're not feeling well? The thought of being snappy to my little girl is terrifying and something that constantly worries me.

Even though we did an amplified CF screening on my husband I'm terrified that our daughter will have CF. I'm not sure I could mentally deal with the fact that we were responsible for putting someone through a life that I wouldn't wish on my worst enemy- never mind our own child!

I have plenty more concerns but those seem to be the biggest ones so far for me: lack of sleep, running myself down, caring for someone while sick, and having a child with CF.

If you could give advice to a CF women TTC, what would it be?
My advice would be to plan, and talk! It's so key to be as prepared as you can, but also flexible in the fact that things don't always go to plan. I think talking is also very very important. Talking to your partner, your parents, your support system, your doctors... talk, talk, talk! The more information you have the less scary it seems. Good luck ladies (and men) it can be a daunting journey, but I'm sure the end result is so worth it. =) 

Beth gave birth to a healthy baby girl on June 18th weighing in at 6lbs 5 Oz.