Friday, April 29, 2016

Toddler Talk Part 9

Kaylee loves rhyming. Sometimes she rhymes real words and other times made up words or a combination of the two. In the morning she sniffed my coffee and quickly pulled away saying, "Yuck-o fuck-o!" (it took everything for my husband not to laugh).

I was telling Kaylee how much I loved that her eyes get lighter blue as they approach her pupils. She gazed into my eyes and said, "I love how the white of your eyes have red cracks in them"

Kaylee and I were getting the mail when we noticed a neighbor left their keys in the keyhole of their box. So Kaylee and I walked over to their house to return the keys. As we were leaving Kaylee turned to me and said, "You are a wonderful mommy to those people."

A few days ago I was in a really bad mood. I wasn't even sure why I was so grumpy, but I could not shake my foul mood. I told Kaylee that I was sorry I was so grumpy that evening, but that I was sure I would wake up in the morning in a much better mood. She reassured me by saying, "Even when you are grumpy, you are still the best mom ever."

I have at least a dozen nick names I give to Kaylee, often they are names I just make up on a whim. The other day my husband and I were snuggling on the couch and Kaylee started running at us full blast while shouting, "Here comes love muffin!"

Saturday, April 9, 2016

Three and three quarters

I am a little late on this update, but you have hit two huge milestones (?) since you were three and a half and thought it would be appropriate to have it in writing somewhere.

You officially have your first real crush. You have been going to story time at our local library for years, but about a year ago the story time teacher left and a new young male, Mr. Nate, took her place. For about a year you would participate in class, but whenever Mr Nate came your way you would clam up and refuse to look at him directly. This was especially true during arts and crafts immediately following story time. You would flat out ignore his presence if he came over to look at your art or try to make any conversation with you. You still wanted to attend every week so I brushed off your behavior as being shy.

The past few months however, you seem to have grown more than a little fond of Mr. Nate. When we visit the library for any reason you demand on knowing his where abouts. A few weeks ago we went to "art hour" at the library where Mr. Nate often helps out and you spent most of the hour keeping tabs on your beloved, "Mr. Nate is going in the back room, Mr. Nate is talking to that boy, WHERE is Mr Nate??" This was a turning point and after this day you started actually acknowledging Mr Nate to his face. Eventually you built up to initiating conversation and will even give him a high five.

You recently decided you wanted mommy and daddy to go out of town so Mr. Nate could babysit and you were confident that after spending the day with you he would love you too much and would have to move in. The love affair has grown and the other night when I went to kiss you goodnight you were pretending to sleep. With your eyes sealed shut you whispered, "Shh, I am with Mr Nate." You may be delusional when it comes to love, but at least you think of yourself as a great catch!

The other huge milestone was one mommy wasn't totally ready for. Last week you went to your parent and child Spanish class (after taking several months off) and during class you were sitting still, watching the teacher, answering all her questions in clear Spanish. It was clear all the content she was covering you already understood and that colors, numbers, and the alphabet in Spanish was just too easy for you. It wasn't just that the content was easy, but you seemed like a real student rather than a little kid in a toddler class. You were just so much older than you were just a few months ago. At the end of class your teacher said she thought you were ready to join the 4-6 year old class. The big difference between the 4-6 class was that you would be attending alone, as in without me! I agreed with the teacher and said it was up to you. I explained that you would be in class with other kids and that I would be waiting outside for you. Your teacher even said you could try the 4-6 year old class that day (as in 15 minutes from when she told me about the level change). You were so excited you could hardly stand it and eagerly agreed.

I was excited and proud and felt like I wanted to cry all at once. This was your first time at "school" and I was not completely mentally prepared (you are starting preschool in the fall) for this major step. Sure, we had been apart from one another. You have had many sleepovers with your grandparents and my last hospital stay was 8 days. This hour and a half was nothing in comparison. However, it still felt monumental in your growth and development. You were so ready to take this next step in a school setting and I was overwhelmingly proud of your confidence in yourself. I was also feeling a slight loss of the baby that used to need me so much while navigating through this world. Needless to say, you absolutely loved class and you ask daily if it is the day you go to Spanish class.

Monday, March 28, 2016

Guest Blogger- Sara

I am thrilled introduce to everyone to another CF mommy! Sara is 35 years old and a mother to Wiley and Gunnar, ages 8 and 6. Sara, her husband Bill and kids are an outdoorsy family (just like mine!!) and they enjoy hiking, skiing, and going to the ocean! She is a fellow double delta F508 with a baseline of 75%. She stays healthy by doing the Vest and hypersonic saline 4x a day as well as running up to 4 times a week.

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

When I was born in 1980, I had a meconium ileus. I was being prepped for surgery when I passed it on my own, luckily! The doctors suspected I had CF. But didn't test until I was a month old. With a positive sweat test at that point, my parents were told that my health was in their hands. They began doing manual CPT twice a day and giving me enzymes. I was followed at a CF center that was 3.5 hours from my home, so that I could receive the best care possible. My FEV1 remained well above 100% until after I graduated college at 23 years. CF was more of a nuisance, requiring CPT, nebulizers, pills, visits to the doctor, and occasional 2 week stays at the hospital.  I was always full of energy and never felt sick. My parents and doctors were very proactive and aggressive with treatments. Unfortunately, during the transitional years where my care was turned over to me, it became  reactive and lazy. And my lungs paid the price. Although I had played  division III lacrosse all through college, I did not do much else to maintain my lung health during those 4 years or the 2years after. , and as a result I had my first case of pneumonia upon graduation and in the months after that I lost a lot of lung function. CF had little impact on my health or life until that time. I spent minimal time doing treatments and only thought about CF on the 2 days a year that I went to clinic. Now it seems I think about it everyday, and I spend hours doing treatments and trying to stay ahead of the germs my two sons are constantly bringing home to me. Although I have maintained my lung function at 75% for the past 10 years, I have had to work harder everyday for that 75% than I ever did for my 120%.
Deciding to have children when you have CF is not an easy decision. How did you and your husband come to the decision that having children was right for you?
We didn't really give it much thought. I always wanted to have kids, and I was going to do that. My husband had a genetic test done, and he was not a CF carrier, so we went for it.

How did CF play a role in your pregnancies with your sons? Were both pregnancies similar or were they different?

My first pregnancy had a rough start. I had a miscarriage just 2 weeks before I conceived my first son. When I was first pregnant I didn't know it. I suspected I might be, but didn't want to get my hopes up. I wasn't going to test until after I had gone past the point where  I miscarried my previous  pregnancy. However, I caught a nasty virus that had me coughing so hard I was vomiting, and delirious with a fever. I ended up in the hospital for an urgent clean out. At the time of admission, they did a pregnancy test and confirmed I was pregnant. I had lost a good amount of weight from the virus, so I had to play catch up all through the pregnancy, but I managed to gain about 40 lbs by the end. I did not need IVs again for 7 years.

Life is harder with CF than without. It was a drag  going to so many extra doctor’s visits. And the tone of the doctors detracted from the joy of being pregnant, particularly in my first pregnancy when I was a first time mom and was unsure of what it would be like. Rather than reassure me, they fed into my insecurities. The second time around, after having a successful  and uneventful first pregnancy, I had confidence and enjoyed the experience 100% more. Both of my babies were full term, big (8lb1oz, 8lb 2oz) and healthy, and I gained weight easily with both. The best part about my pregnancies was that my lung function actually improved throughout them. In the 8th month of my second pregnancy I hit an FEV1 of 88% !

While CF did not have much impact on my pregnancies, I would say pregnancy had a huge impact on my CF, in a good way. I think the combination of wanting my babies to be as healthy as possible as well as sharing life with a healthy being in my body are the two things that helped me gain health throughout both pregnancies. I controlled my blood sugars so tightly that the doctors told me to loosen up a bit. Or maybe I was just so full of happiness it literally brought me health.

Please share a little bit about the birth of both your children. Did CF play a role in the delivery or recovery?

Both of my babies were full term. They were both delivered vaginally. But other than that, I had two very different birth experiences. The first was induced, I had an epideral, and I had a lot of tearing requiring stitches. I pushed for a few hours on my back, at the doctors command. Recovery was hard because of the tearing and because I got very engorged when my milk came in. It took a few weeks to get everything under control.  The second time I mostly labored at home, and delivered him 16 minutes after arriving at the hospital. I pushed on my hands and knees for 30 seconds.  I  had no IV, no epideral, not even a heart monitor on my belly, and very minimal tearing, if at all.  Recovery was much easier the second time as well. I didn't have the tearing, and my milk came in just right, I never got engorged. Although learning to be a mom of 2 was challenging.

I didn't feel that I was limited at all from CF. But I did have the usual extra stuff to do to care for myself in addition to laboring, recovering, and caring for a newborn. I did my vest and nebs during labor both times. And continued to check my sugar levels, give insulin, eat and so on. Doing the vest when I was engorged actually didn't hurt because it promoted the milk to let down, and afterwards it was like I  had just pumped (but it was messy). I nursed my first son until he was 17 months old, and I was 3 months into my second pregnancy. I nursed my second son until he was 2.5 years old. And I still haven't lost all of my “baby weight”.

Caring for another person day in and out regardless of health challenges can be extraordinarily challenging at times. What aspect of CF and motherhood do you find the most challenging?

 I feel like I just don't have enough time or energy to be the kind of mom I thought I would be. So the hard part is being the best mom I can be, and trusting that is enough. I'm tired all the time, but I am very careful not to let myself get so run down that I get sick. Because being sick makes everything harder. So it seems like I am constantly choosing what is most important to do each day with the limited energy I have. I can't do everything that other moms do, and I feel like I am always letting someone down. Also, when I am feeling extra run down from fighting off a cold, or taking home IVs the whole family’s day needs to revolve around me. That makes me feel guilty, and inadequate as a wife and a mother. Thankfully my mom has stepped in whenever I need help. She will stay with us and do everything I normally do with housework, and watching the kids, as well as take care of me. But it is hard knowing I need help like that. Also, having my mom live with us for 2 weeks at a time has it's own set of challenges!

Kaylee is only 3 1/2 and is already aware that I do things that most moms do not have to do such as treatments, taking enzymes, etch. How do you explain your CF or tackle health questions your boys may ask?

From day one, my goal has been to include my boys in my CF care and decisions as much as possible. My goal was to normalize CF. And I realized that CF would be to them, whatever it is to me. So I had to decide what it means to me, then make sure my actions and words back it up. I brought them to all of my clinic appointments until they started school. They would sit at the doctors office with me for 2-3 hours 4 times a year. When they were small enough I would roll them in in the stroller and there they would sit, and take it all in. When they got older, they would walk in and sit in the chairs or my lap, and continued to be a part of my appointments. Sometimes they would ask what we were doing, or what something was. They asked small, simple questions, and I gave small, simple answers.

At some point, I brought up the fact that other people’s moms don't do the vest or have a port, or drink “milkshakes” (boost plus). We have participated in a large fundraiser for CF for as long as they can remember, and that has been a way to bring up CF as well. Through the event we have met other healthy Cfers who are thriving as well. We’ve had conversations about why it is important for me to do all my treatments everyday. And why I decided to have sinus surgery and go in the hospital for a clean out. The answer is simply because I have CF my body needs different things to keep it as healthy as possible. My sinuses and lungs were getting too filled up with germs, and I'm  going to clean them out so I feel better. To them, CF is just another part of who I am. I'm different from Dad because I have long hair, CF and a vagina. They ask all kinds of questions about all kinds of things, and CF is no different.

What advice would you give to other mothers that are juggling both CF and motherhood?

I think that growing up with a mom who has CF can be a great thing for a child. They get an excellent role model for overcoming adversity, demonstrating work ethic, and living a life with intention. Everyday I work for my health. Sometimes I feel lazy and start wishing I didn't have to do so much, but I never give in. They see that.  Sometimes I'm sick and I have to take IVs at home. I schedule my days in a way to get all my treatments in, get enough rest and still be emotionally present for them. I struggle, and I mess up sometimes, but I try again the next day. They learn that. They love me unconditionally, and I didn't even know what that meant before I had them. It’s their love that led me to love myself just as I am. I have a peace in my soul I didn't have before. I'm so grateful to have them on this journey with me. At the end of the day, life is the connections we have with those we love. Everything else is just trivial distractions.

It is our job as parents to teach our kids about life, and how to live. I like to include death in those discussions as well, because it is a universal truth that everyone is going to die. Death is not something to fight or avoid, but to embrace. No one knows when they will die, but when it is time, it happens. Until then, we live! We take care of the bodies we live in, we connect with our world, we laugh, we cry, we love, and we talk about death.

There is a parenting style that embodies the quote “never do for a child that which he can do for himself”. As well as “if he can walk, he can work”. I don't have the energy to parent any other way, but even if I did, I'd still parent this way. My kids are confident and capable little people. They are tuned in to what is happening around them, and excited to do their part for our family. You can learn more about it from Vicki Hoefle, and Parenting on Track. As well as her two books “ duct Tape Parenting” and “Growing a Grown up, the Straight Talk on Parenting”




Thursday, March 10, 2016

Spring In My Garden

Spring has arrived in the part of this world I call home. The first sign of spring arrived several weeks ago. Yes, there were buds bursting from the silence of winter, flowers unfolding in the warmth of the sun, but the first sign of spring for me is the feeling of my lungs clamping down and the chronic wheeze that comes buzzing from these clunky old lungs. Spring allergy induced asthma has always been much more predictable than any weather man I have ever encountered. And despite spring being the absolute hardest season for me to breath in I still hold a special place for spring in my heart. There is no other time of year that I feel so much hope and anticipation for what is ahead. Let me show you what I mean:


Strawberry flower

Echinacea just starting to open
Yellow Plum Tree

Ornamental Plum Tree
So much hope and promise is held in these few short months. As I walk around my garden I can't help, but feel overwhelmed at the potential of the things to come. Even when my breaths are frustratingly loud and feel as if my lungs are hardly inflating with each breath I take, I still can't help, but feel excited for tomorrow.

Saturday, March 5, 2016

24 Weeks of Orkambi

Two years ago I tried to get in the Orkambi trials, but after going through all the pre-trial testing I was denied. When I finally received the medication Sept 2015 I was curious as to how my life may have differed today if I had been the trial two years ago (and had the same dose medication that was approved by FDA which would not have necessarily been the case). Would I have been healthier with better lung function? Would my two years of decline and instability have been less "eventful"? There is no way of knowing how things would have been different had I started the trial 2 years ago, but I was still curious as to what my experience on the trial would have been like. So after starting my Orkambi I marked my calendar for 24 weeks (the duration of the Vertex trials) and waited.

I blogged about the first couple of months here if you want more detail as to the rough start I had with Orkambi. The biggest changes from the last 24 weeks of Orkambi:

1. Plugs!
I coughed out my first plug about 2 months into Orkambi and I had no idea at the time, but they would continue to come out at an alarming rate peaking at 4 months. There were days I would cough out up to four or five plugs in a single day. Around 4 months I started to lose sinus plugs at an equally alarming rate. There were weeks that I got either a lung or sinus plug and often multiple times a day! 
**For reference, prior to Orkambi the only time I would cough out a plug was during a round of IVs and these plugs were usually "newer" plugs that were more gelatinous and lighter in color. Orkambi plugs were dark and were so foul I often gagged as they came out sometimes verging on throwing up. They were clearly bacteria ridden and extraordinarily old. Good riddance!**

2. Sickness
I have had a rough couple of years. There was an entire year that every single time I started to feel better I would instantly catch a cold and be incredibly sick again. It seemed that once I felt well for a few days I would wake up the next day feeling a little off only to be sick with high fevers or completely bed ridden by afternoon. Sickness came quickly and often. I hardly left the house and bathed in hand sanitizer and was still constantly sick. I also ended up with reoccurring infections because my lungs couldn't handle the stress and mucus overload.

Since starting Orkambi I have had the first winter in a very long time that I was NOT SICK AT ALL! (Yes, I shouted that). What is even more amazing is that Kaylee and I have not restricted ourselves due to risk of getting sick like we have in the past. We continued storytime at the library, had regular playdates, and generally acted like normal human beings. And somehow I did not get sick! The most insane part is that I never even touched a bottle of hand sanitizer this winter. I ran out and never replaced it. Clearly, my body working more properly has allowed my immune system to function again (years ago I had a pretty amazing immune system that was so taxed the past few years it seemed to completely stop working).

There were 3 times over winter that I thought I was getting sick (the achy tired feeling is always a red flag that sickness is approaching). All three times I mentally prepared for a hospital stay because the last few years sickness always ended with IVs because my lungs just couldn't handle the overload. But unlike pre-Orkambi somehow I got over whatever was making me feel off. As in, I never actually got sick. This was such a sharp contrast from the past few years that the first sign of ache always left me feverishly shaking in bed within 24 hours.

3. FEV
So unfortunately, I have not seen an increase in FEV1. In fact, my last appointment I was down a little. However we are in the throws of allergy season at the moment which is my hardest season. My allergies present themselves as asthma and my lungs often feel like they completely shut down during allergy season. At this point I do not believe I will be one of those people with CF that see a positive change on FEV1. I won't lie I would absolutely love extra lung function and really wish I would have seen some improvement in that regard.

I am a skeptic when it comes to... well most things. I did not assume Orkambi would be a miracle and I did not assume every positive (or negative) change in my body would be from Orkambi. Our bodies are complicated and CF can be so unpredictable that it makes distinguishing correlation vs causation hard to separate. However, the hundreds of plugs I have coughed out in the past 6 months and the fact that my completely unstable health (especially in winter that usually left me a hermit for months on end) seemed to stabilize (for now) is enough to make this skeptical person confident that Orkambi has greatly improved my quality of life and I am so thankful it came into my life when it did.

Friday, February 26, 2016

Toddler Talk Part 8

Things Kaylee has said recently that I find amusing:

While kissing Kaylee goodnight
K: "Mommy, am I your son?"
Me: "No, you're my daughter because you are a girl. If you were a boy like your cousin, you would be my son"
K: "But I am your son sometimes! I am your daughter and sometimes your son."
I was not following her logic, but was tired, "Okay, you can be both."
K: "Remember, you call me your sunshine sometimes so I am your son too."

After seeing her dinner plate Kaylee said disappointedly, "That was not the rice I was respecting (expecting)"

A few days before her cousin was born.
K: "What do you think my baby cousin is going to look like?"
Me: "Hmm, I am not sure. Maybe he will look like his big brother."
Kay: After a few minutes of silence, "Well, I think the baby will be smaller than his brother."

Squirrels love to dig in our newly seeded beds to hide their acorns so I often have to cover the seedlings with bird netting. Kay was watching this process and proclaims, "MOM! I have an idea so we don't need to use netting anymore. We can just post a sign on the fence that says, No Squirrels Allowed In The Garden Please."

Funny words she still says just because I never want to forget:

Children - childlen
Mother- mudder
Scrambled eggs - scrumbled eggs

Monday, February 15, 2016


I remember tucking Kaylee, when she was still a chubby baby, into her stroller on a particularly rainy day several years ago. We wanted to take advantage of the sun between the cycles of rainfall to get a quick walk and a break from the four walls that made up my living room. She was mesmerized by the world around her glistening from being bathed in raindrops, making the mundane and familiar walk seem mysterious and wondrous.

That very same earth soaked walk I took with Kaylee so many years ago was one of the last walks I would take before my health plummeted and my world was forever changed. I had achieved my biggest dream and felt as if life in that rain soaked afternoon had been almost too good to me and gave me more than I deserved. I had no idea how quickly things could change.

The following year, the smell of the earth freshly cleansed by raindrops seemed to be a distant memory, just as that toothless, chubby cheeked baby smile seemed as if it were a muddled long ago dream. We were in the midst of a drought and the sun bathed winter days seemed odd and out of place. As the months and then the year passed there was murmurs of worry. These murmurs turned into loud voices, limiting household water usage. Those voices turned stern as people were told to let their lawns turn brown, throwing around slogans that "gold" was the new green. And soon those voices were thunderous and forests and towns all around the state were going up in flames, destroying all living things in its wake. And we waited and hoped. We helplessly waited for the clouds to roll in and the sky to turn dark and wash away the drought that plagued us for years.

My health tumbled quickly after contracting the flu, and although I was worried I knew that the next set of IVs, the next round of oral, the next month would bring better health. My murmurs of fear that my illness was intent on killing me turned louder as months passed and my health had not returned. My fear became louder as yet another round of IVs made no difference in my health, which was something that seemed foreign to me prior to contracting the flu. As the years passed and my health plummeted lower and lower despite IVs and hospitals and steroids my fear became so fierce it often clouded my mind. I wrote a letter to my family on the wishes I had for my daughter as she grew older, most likely without me. My belief in a future with my family grew dim.

And then there was a glimmer of hope. The news kept announcing that El Nino was coming, which has something to do with abnormal ocean temperatures which meant the only thing we so desperately needed, rain! There were days we went outside and were reminded of the familiar smell of the sky on the cusp of rainfall. There were swollen dark clouds that rolled in heavy with raindrops waiting to tumble to our dry and cracked land. And soon the lakes and rivers started to fill, the land turned green and lush like it was always meant to be.

Around this same time there was a glimmer of hope for me as well. I made it two months without getting sick. Two months turned into three. My port went unused and I didn't see the hospital walls 8 whole months. I started to lose the paranoia of germs and sickness and death that so often haunted the deepest darkest corners of my mind. I started to think that maybe I would live long enough for my daughter to have memories of my existence outside of pictures and stories told by family members.

We are still in need of rain to fill our reservoirs and snow pack in the mountains. We are still restricted on our water usage and we know that we are teetering between being okay and not having nearly enough water to sustain our land and our people's need for water. And yet, we got a small reprieve from the four rainless years and the fear of running out of water has quieted even if just a little.

My health will never be the same as it was so many years ago. So much of my lungs are now damaged and scarred, damage that will never be healed. I still find myself gasping after carrying a heavy basket of laundry or bending over too long. I get short of breath from gardening and seemingly simple tasks which can be frustrating and saddening at the same time. And yet, I am so far from the place I used to be. I no longer wonder when I go to bed at night if I will be able to crawl out of bed in the morning. I no longer fear the grocery store because pushing a cart is something I am once again confident I can do. I no longer need a nap after even the most mundane outing. I have a life again. I know I am always one bad bug away from not being okay, but for now, I am thankful for my small reprieve from the all consuming sickness that plagued me for so many years.