Wednesday, February 22, 2017

Guest Blogger- Kristen

I am so excited to introduce to you another CF mother, Kristen! Kristen has a unique CF story in that she was diagnosed with Cystic Fibrosis at the age of 31. She was already a mother of two children at the time of diagnosis. Six months after her own diagnosis her children were tested positive for Cystic Fibrosis as well. Below is her journey with CF and motherhood.

Like me, you had a late diagnosis compared to most people with CF. Can you explain how you were finally diagnosed with Cystic Fibrosis?

For many years I struggled with GI symptoms, chronic sinus infections and low weight. Doctors were unable to connect pieces until I was 31 and diagnosed with pancreatic insufficiency. After the pancreatic insufficiency results and a borderline sweat test, the pieces were put together.

I am sure getting a CF diagnosis as an adult was life altering. Your children were also diagnosed with CF which I am sure added to the shock. Do you mind sharing a little bit about what it was like receiving three CF diagnoses in one family over a short period of time.  

I was told the chances my children had CF were very slim, due to the genetics of myself and my husband. However, both my children were in fact diagnosed with the disease. It was shocking to say the least, but yet I felt that it immediately encouraged me to stay as positive about my disease because it was my children’s disease as well. And I did not want them to have any negativity associated with CF.

Cystic Fibrosis presents itself so differently in different patients. Can you share your biggest hurtle with CF?

My digestive system. Even with clean eating and digestive enzymes, I struggle with DIOS episodes, lack of absorption, and low body weight. And because of that, I can be weak at times.  I also have chronic pancreatitis which can cause pain many days and my blood sugar to be erratic

What are your children's biggest hurtles when it comes to Cystic Fibrosis?

We are very blessed that at the moment my children are only exhibiting mild CF symptoms. My son is very strong. He runs almost daily to keep lung function up. However, he does struggle with intestinal issues as well. He has had several “back-ups” as they are referred to and needs to be diligent with laxatives and enzymes.

My daughter is very tiny, like myself and struggles with her pancreatic function as well. She is prone to upper respiratory infections. We have had a good 6 months, but the 6 months prior to that, she had several cases of pneumonia.

Being a mother, regardless of health status, can be challenging at times. We all know CF can be extremely demanding and challenging as well. Does CF ever get in the way of the mother you want to be?

YES! There are times I am too sick to go into the schools for this or that for my children. There are times I am too sick to get out of bed and I hate not being present for my children. My own care takes a lot of time and that is time I have to schedule in my day and away from other things.

What advice would you give and adult that is diagnosed with Cystic Fibrosis.

To not let this disease define them. CF will become part of your life, but it does not need to be your whole life or define you negatively.

What advice would you give to a parent whose child recently received a CF diagnosis?

I would tell them to raise their child to be responsible for their disease, but to raise them with HOPE. Allow them to be who they want to be. CF is only one fact about them, it is NOT who they are!

You and I have very similar philosophies when it comes to food: whole foods, healthy sources of fat, and limited junk or processed foods. This kind of eating can be unusual among CF most CF patients who are told to get as much fat as possible regardless of the source. What made you chose this route when deciding what foods to put into your body?

It was as simple as trial and error. The bottom line is that I feel better when I eat clean foods. My weight stays more stable when my fat and calories come from natural sources. When I stray from clean eating, I suffer with pain and weight loss. I also believe that people with CF need to plan to live a long life (we are making strides that is a reality now) and I believe you should protect your heart, etc when caring for your body and meeting your CF needs

Please tell us about your website,, where you share tons of amazing recipes.

A Mind “Full” Mom is a place where I share real recipes made from real foods that meet the needs of real families on real budgets. That breaks down to me sharing recipes that are family-approved, easy to pull off and won’t break the bank. I also share ways to be intentional with life and to not let the craziness of this world impact the memories you make with your family. And of course I share encouragement for staying positive when living with a chronic disease.

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 have CF and are pregnant and want to share your story e-mail me at inhalinghopecf(at)

Monday, February 20, 2017

Polar Bear Plunge

I missed a few days of workouts because the whole fiasco with missing some Orkambi which made me too short of breath to workout. Friday I decided my lungs felt well enough to get back into my usually workout routine. I set my alarm and found myself checking into the gym early morning. To my dismay the girl working the front desk informed me that the pool was closed for maintenance and apologized for any inconvenience. Sigh, I wished I had decided to sleep in that day instead.

Over the weekend, I called to ensure the pool would be ready for Monday morning as I didn't want another early morning without a workout. The gym staff assured me the pool was up and running. So this morning, I found myself back at the gym and more than ready to get back into my workout routine. As hard as it is to get myself out of bed to go to the pool I never feel myself when I skip and so I really do enjoy my morning workouts. After checking in (and yes the pool was open!) I ran through the heavily falling rain to the pool, sat on the edge to adjust my goggles and dipped my feet into the water. Eeek! The water felt like ice! I went to the heat pump to check the water flow to see if the water being pumped into the pool was warm (it always is at this time in the morning) and it was also coming out as ice.

I wasn't about to miss another workout and so in I went for my very own polar bear plunge (although the mornings have been around 50 so I guess I really can't pretend I am as crazy... err... brave as the people that really do the plunge). I will say that being forced to swim in cold water sure encouraged me to swim a lot faster though!

Here is to hoping tomorrow morning that the pool will be open and the water will actually be warm!

Thursday, February 16, 2017

Restarting Orkambi

Due to issues with my pharmacy I did not receive my January shipment of Orkambi on time. Of course, I was upset that due to issues with my pharmacy and their lack of organization and communication was causing me to miss medicine that is beneficial to my body. But the reason I was most upset is that when I started Orkambi I had a lot of issues with side effects specifically chest tightness and shortness of breath. I was very concerned that restarting my Orkambi would bring back some or all of these side effects.

When I finally received my Orkambi I was so relieved that I wouldn't have to fight Accredo anymore. I momentarily forgot my concern with side effects. I was abruptly reminded of this concern a few hours after taking my morning dose. Although the side effects weren't nearly as intense as when I first started Orkambi I did notice a huge difference in my breathing pretty immediately. I often felt as if someone was sitting on my chest. I also had random sporadic bouts of extreme shortness of breath that seemed to come out of nowhere and disappear just as mysteriously. The biggest issue I had was I was too short of breath to exercise efficiently so I felt like my lungs weren't getting cleared properly by swimming which seems to be my best defense against mucus build up.

The side effects only lasted about fours days and yet after they subsided my lungs felt congested and heavy. I currently feel like I am on the brink of an infection. I will never know 100% if stopping and restarting Orkambi caused this infection, but I do think adjusting off and back on was really stressful to my body. I also think missing/more lax workouts didn't help the mucus build up nor did the tightness caused by restarting Orkambi. I seriously hope that I am never forced to go off of Orkambi again unless a new and improved drug comes out.

Monday, February 13, 2017

I Hate Accredo

I am not even sure how to explain how impossibly hard it was dealing with Accredo these past few weeks. I think instead of writing a post that could easily go on for hours I will give you the quick and dirty run down. I think it is important to note that a rep from Vertex as well as my clinic coordinator was also helping me deal with Accredo and I have no doubt it would have taken significant longer to get my meds if I was doing it alone. Which is so beyond ridiculous because it took over two weeks for me to get the medicine as it was.
  • At one time my clinic nurse called and explained in great detail what she discussed with Accredo. I called Accredo and when I told the rep what my clinic nurse just explained the rep told me there is no record of such conversation in their computer system. Huh? What? How do you even respond to that?
  • Every time I called the rep would flip flop what they were saying. Sometimes they would say it wasn't covered by my primary which meant my secondary wouldn't cover it either. Other times my primary would cover it, but my secondary didn't leaving me with a co-pay of $5,300.
  • Two times my Orkambi was scheduled to ship out the next day only to be cancelled at the last minute due to "issues."
  • Between Thursday and Friday I spent FOUR hours on the phone! About 90% of this time was spent on hold with Accredo.
We eventually found out a lot of these issues were because someone opened two accounts under my name and neither had both my insurance plans. So depending on which account the rep opened there was differing insurance information. Hence the reason some reps said that my nurse or Vertex never called in. They had the wrong account open. After this issue was resolved I assumed we would be good to go. Oh, but no that would be much to easy!

  • Accredo kept insisting over and over again that my secondary insurance didn't cover Orkambi although it absolutely DOES cover Orkambi and I was told over and over again that the only way to get my medicine is to fork over $5,300. (The issue was that they were using the wrong authorization number)
  • My case had been escalated a few times to people "higher up" in order to try to expedite (HA!) my shipment. However, there was complete lack of communication between the higher up "center of excellence" and the reps. The center of excellence (I use the term excellence very very loosely) made a plan and was told to me by my Vertex Rep. When I called Accrued explaining the plan I was told by both the rep and her supervisor that, "Nope, it doesn't work that way!" I ended up hanging up and calling back until I found a rep that would do what I said.
  • I was told by a supervisor and someone in center of excellence that they would call me back within a specific time frame. Did it happen ever? Nope. And when I called them, the rep said the person I needed to speak to was nowhere to be found.
  • After about two weeks I talked to a rep that said that maybe I should let me doctors office know I was having trouble getting my medication. Uhhh, if you look at my log they have been calling in for almost two weeks now...I think they know! This seemed to be another common theme... reps that didn't bother to read the notes in my file and wanted to d the bare minimum as far as work goes.
So we finally figured everything out and I was starting to believe I would finally receive my Orkambi. But wait, that would be too easy right?

  • I was scheduled to get my Orkambi and although I had already ran out of my supply and it was coming super late I was beyond relieved to know it was on its way. Just when I thought I was safe my phone rang.... it was Accredo... nothing good comes from those calls...
  • They were calling to say that their inventory was low and it would take 7 days for me to receive my shipment. SEVEN DAYS!!!
  • At this point, I will not mention the things running through my head. However, the rep that delivered the news was one of the few reps that knew how to do her job. She had talked to me several times and I kept telling her if there is ANY inventory ANYWHERE they needed to send it to my pharmacy to get it to me. She told me to hold.... came up with a potential plan and told me she would call back.
  • And guess what? Whoever is in charge of updating their inventory electronically didn't do their job (notice a theme??) and they actually did have some to send me. So I was going to miss another entire week of medicine because someone "forgot" to update their inventory list.
And then I got Orkambi and we celebrated. The whole family. Kaylee wouldn't be neglected anymore while I spent all day on hold. My husband didn't have to put up with my mental breakdowns anymore. And best of all I had my medicine. Except, restarting also meant my body had to readjust to Orkambi which my body doesn't do well...

To be continued...

Saturday, February 11, 2017

A Day In The Life- Preschool

I wrote a blog about our daily routine when  Kaylee was a newborn and again when she was about one. I loved looking back at it and was shocked at how long it took to do my treatments! I forgot that I had to break them up throughout the day because a baby's attention span is so short. I decided I would do "A Day In The Life Preschool" version since I know someday these moments will seem like such a distant memory. So like any "Day In The Life" series this is a rough idea of out day because as we all know children often have their own agenda.

  • My alarm goes off and I quickly get my bathing suit on, pull on sweats drink a glass of milk and drive to the gym.
  • I swim for about 30 minutes, shower at the gym and come home.
  •  I arrive home from the gym and my family is still sleeping. I start my Vest and all my nebs.
  • A very sleepy Kay comes to find me in the living room. (Why are kids so darn cute when they first wake up?) I am usually almost done with treatments, if not completely done. If she gets up before I am done I let her watch Daniel Tiger until I am done.
  • I usually brew a cup of coffee for me and start breakfast. I always let her choose between eggs and oatmeal. I do not have CFRD, but I don't do well with sugar in the morning so breakfast is never something convenient like cereal. Even my (and Kaylee's by default) oatmeal has to be a mix of oats, chia, ground almonds and flax meal because straight oats cause issues in the morning too. The older I get the more complicated my body gets!  
  • I also do morning chores like empty the dishwasher, clean the breakfast dishes, and sometimes throw a load of laundry in the wash. I am so bad at letting laundry pile up.

  • Kaylee gets dressed, we brush teeth, and I always do her hair (having her hair off her face means she touches her face less often which is better for controlling germs even though she has no idea that is why I do it. I am trying to be germ cautious without making her realize it so that she doesn't become a germaphobe). Then we hop in the car and go to preschool.

  • Kaylee likes to be the first person in line so we are usually the first people there and I let her play on the playground until school starts. There are a couple other early birds so I get to chat with some of the other parents before school.

  • Preschool! Kay goes to a parent participation preschool so I am there working twice a month. I am also the class mom so I go to all holiday parties and any big events so I feel like I am there a lot, which I love!
  • If I don't work at the school I usually try to make phone calls or other boring errands that aren't fun for Kaylee. On days my husband is home we sit around and drink coffee.
  • Pick up and come home for lunch.
  • We either go to the park, ride bikes, take a walk or run some errands. I usually do some chores (aren't house chores endless?). Really, we have no consistency during this time. On my Cayston months I do my afternoon nebs.
  • Quiet time! Kaylee plays independently and I get to rest as well. We both really need quiet time or we both get grumpy!
  • Kaylee has a snack, plays and then we get dinner ready (Kaylee usually helps).
  • My husband usually gets home right before we have dinner although his schedule can be all over the place so a few days a week he misses dinner.
  • Kaylee and my husband play and I clean up dinner. I often do treatments during this time or if I join in I will do treatments after she goes to bed. If my husband works late I usually do treatments after Kaylee is asleep so then the two of us play together after I clean up.
  • Kaylee picks out her clothes for the next day and I hang them on her doorknob so she can get herself dressed in the morning.
  • We do a five minute clean to make sure all her toys are cleaned up before bedtime.
  • Kaylee has a small snack before bed.
  • Kaylee gets in her pajamas and brushes teeth.
  • My husband and I read books (mine are in English and his are in Spanish) and then we each tell her a story (also in English and Spanish). Mine usually incorporate something we did that day, after telling stories every.single.night for years it is hard to be creative. Then Kaylee stalls as much as humanly possible.
  • Finally, Kaylee goes to bed!
  • I do treatments (if I hadn't already done them)
  • I set out my gym bag and everything I need in the morning.
  • I take all my pills
  • I eat a snack so I can take Orkambi (my morning dose is with breakfast)
  • I also do the evening chores like run the dishwasher.
  • Bed!!!

Thursday, February 9, 2017

Bliss In Pictures

This past week has tested my patience in more ways than one. I found myself grumpy and irritable and in a funk. I decided to take some time to remember the simple things that bring me joy. I needed to find moments of bliss between making phone calls, sitting on hold and fighting with my pharmacy over and over again. So here are a few pictures of the moments of bliss that saved my sanity this week.

Fresh brewed coffee on a rainy morning

A brand new jar of raw local wildflower honey. The plastic bear has nothing on the real deal!

The first sign of spring on my blueberry bush, no less!
Asparagus season has officially begun. It takes three years after planting before you can harvest asparagus and this year finally marks year number three!

Monday, February 6, 2017

Kaylee Chatter Part 3

Kaylee dumped out a box of small wooden whales and shouted, "Woah, there are whales ga-lord!" (galore)

My husband was doing something that Kaylee was not a fan of (I can't remember what it was) and she was giving him the evil eye and said, "I am judging my father right now!"

Kaylee's grandma on my husband's side always tells her, "suena con los angelitos" meaning "dream with the angels" or basically, sweet dreams. So the other day before bed Kaylee shouted, "Suena
con los burros!" to my husband meaning "dream with the donkeys."

"Mommy, I love you more than anyone in the world. I want to marry you because I couldn't love any wife more than you." I have to write these things down so when she is 16 and finds me the most annoying/humiliating person alive and I can fondly remember the days she was obsessed with me!

Thursday, February 2, 2017

Missed Lessons

I will be posting later about my ongoing struggle with Accredo, but honestly, I am so beyond pissed right now at them that I just can't let myself think about it or I may seriously lose my mind. Can you tell I still haven't had my medication situation figured out yet? Despite endless calls from Vertex, my clinic coordinator and myself we are still in the never ending hell that is called Accredo. Okay, I can feel myself getting fired up again so I am moving on to the real reason I am posting.

After dealing with Accredo for two weeks trying to get Orkambi and still not making much progress (it is one step forward, ten steps back and then an oops we, as in Accredo, messed up and we are now nowhere even close to where we need to be) I was pretty much at my breaking point for the tenth time today. I was venting to my husband that I feel there is a reoccurring theme in my life. I feel like the Universe is trying to teach me to be patient. Over and over I am thrown in these sitatuation that extreme amounts of patience is required and I can't figure out why because I already am a very patient person. Trust me I have a lot to work on in life, but patience is hardly one of them.

My husband looked at me and said, "Maybe the point was not that you needed to learn patience, but that you needed to learn to be more assertive."


Yes, I have patience for days and I also am super laid back so it takes a lot to get me upset. Patience is something I am not lacking. Being assertive on the other hand is not something that comes naturally to me. I always envied people that could be very assertive (while respectful of course) and got their point across firmly and without hesitation. That is not me, but I wish it was. Sure, I was assertive today, but while I was fighting for what I deserve and need I felt like a fish out of water, like I was trying too hard to be something I am not. It did not come naturally. I eventually lost my cool (oops) and I know that is not productive either.

From now on, when my patience is pushed, I am going to be more assertive and COMFORTABLE while doing it. I know the more I practice the less uncomfortable I will be because like anything else it is a skill that can be learned. So in 2017 I am going to work on being assertive and saying exactly what I mean without an ounce of hesitation!