Saturday, September 19, 2015

Rain Showers and Orkambi

"Maybe we will see a rainbow" my 3 year old shouted with eyes wide at the thought.
My dad and I exchanged glances. While smirking I replied, "Maybe" not believing it one bit.
It hardly rains in the summer. We haven't seen rain in four months or so, but a recent drizzly day made its way into the end of summer leaving my daughter with the optimistic belief that she would see a rainbow. It was true the rain had stopped, but the sun was still hiding behind dense clouds and evening was quickly approaching. My daughter had a date with my father that evening and I was hoping the excitement from the date would be enough to make her forget the disappointment of a rainbow that would never be.

I started Orkambi today. The day I found out that Orkambi was approved, my application was accepted, and the pharmacy was preparing to ship my pills to me I was filled with a multitude of conflicting emotions. I am an optimist by nature, but a realist by life experience. My heart kept shouting, "This is a dream come true! Your body will be functioning more normally than it ever has before. This is what you have been waiting for" But my brain kept shouting just as loudly, "But the benefits are minimal and your lung function is already so low." My heart would rebuttal, "Improvement is not the point, stability is what matters. " The fight between my jubilant heart and my practical cautious mind roared on throughout the week

This morning I woke excited and anxious for the postal carrier. And once the meds were in my hand around 9am I suddenly felt nervous in a way I haven't felt in so long. This was the moment of truth. All the potential benefits and all the potential let downs were running through my mind. I wanted so badly to let my optimism take over my cautious mind, but I was equally concerned about being disappointed. I sat there for a long time before swallowing my first pill. It is okay to hope for the best, I kept telling myself. It is okay to be optimistic for once and believe that good things, no matter how small, are coming your way.

I could hear the giggles and high pitched voice of my little girl running up the front steps. I greeted both her and my dad at the door with a big smile. Before I could even ask how the date night went my daughter, hardly able to contain her excitement shouted, "We saw a rainbow, mommy! We really did!"

Sunday, September 13, 2015

Forgetting Enzymes

CFers take enzymes every time food passes our lips. It is so engrained in our daily routine that we often take them without much thought. It is so routine that there are times we may wonder, "wait, did I take my enzymes already?" It happens to me more often now that I have a little one that distracts me during meal time, but let's be real, it happened often enough before kids. Recently, my dad found the perfect solution to this annoying problem at the local pharmacy.


His great find is a lid that goes on regular prescription bottles (the translucent orange kind) and keeps track of how much time has passed since the bottle was opened. So let's say you ate breakfast at 7am and you sit down for a snack at 10am, but you can't remember if you already took your enzymes. You can take a peak at the lid and if the bottle says 3 hours have passed since you opened it you know you better get swallowing. And if it says 1 minute, you are good (as long as you didn't accidentally place your napkin on top of your pile of enzymes by accident not that I have ever done this ehem).

The downside is they don't fit on the plastic Zenpep bottles, but if you have CF you probably have a few extra orange bottles on hand (or will once your next shipment of prescriptions arrives) that you can use. These were clearly from Rite Aid, but I assume most major pharmacies have the exact same product. Hopefully this will answer the age old CF question of, "Wait, did anyone see me take my enzymes?"

Wednesday, August 19, 2015

A Green Tomorrow

To plant a garden is to believe in tomorrow.
- Audrey Hepburn

I started my first vegetable garden 5 years ago, in pots that were much too small, outside my apartment's front door in a much too shady and busy walkway. Throughout the day I would move the pots holding my precious tomatoes and peppers to wherever the sun was filtering through the branches of an overgrown pine tree. It was the worst conceivable place to grow a garden, but that little potted garden that gave me a total of three tomatoes and two stunted bell peppers ignited a passion in my soul that I have never been able to shake.

Today, the first sight you will lay your eyes on when you go in my backyard is a large display of raised beds that is home to everything from asparagus to watermelon. My mornings and evening are spent tending to my garden and my kitchen is home to the rewards these plants offer in return for my care and attention. It seems at times my daughter was raised in the vegetable garden and the age of two can rattle off facts about garlic scapes, and the importance of ladybugs, and that melons have both female and male flowers.

I always found it funny that so much of my life is avoiding thoughts of the future, a future that statistics and doctors assure me won't be long. Yet, I spend my time on a hobby, a passion, an obsession that is all about forward thinking and constant planning for the future. The seeds I so careful sow in the ground in spring comes with so much hope of thick green vines heavy with melons that will fill my stomach and soul mid summer. I cover my strawberries today to protect them from tomorrows ravishing birds. Asparagus is planted with the hope that in three years time I will have spears to grace my dinner table.

If you ask me about the future of my garden you will grow bored and restless long before I stop chattering on about expansion, and artichokes, and aphids. If you ask my about my future, the future of my health and my life you will be greeted with a harrowing silence. You will see an empty vastness in my eyes so foreign in a woman still in the prime of her adulthood.

The garden is a way to plan for the future and put my hopes and dreams into a tomorrow that feels so uncertain. Somehow it feels safer to make plans for my plants than for myself. If I never get the harvest of cherries it is much less heartbreaking than never seeing my daughter get ready for prom.

So for now I carefully tuck seeds into the soil so that in two months from now the ground will be stuffed with plump carrots. And as for what lies in the future? The carrots are all I can be certain of.

Wednesday, August 12, 2015

Clean and Care Free

You know that feeling you get after filling your gas tank? The relief when that little voice that keeps nagging, "your tank is getting low" and "don't forget you need to fill your tank soon" is finally quiet? That voice that you didn't even consciously know was bothering you until it is finally gone?

Yeah, I get that same freeing relief times ten when I know all of my nebs are clean and dry!

Saturday, July 25, 2015

Orkambi Troubles

I am not sure how one quantifies the value of a life. Apparently, some people know the secret equation, but I wasn't lucky enough to be one of them. Although, I do not know exactly what my life's value is I do know according to my insurance company my life is not worth $259,000/year. In other words, my insurance is not covering the new drug Orkambi for any of their Cystic Fibrosis sufferers.

I was actually really surprised, naïve as it was, because my insurance gladly covers the $300,000/year Kalydeco for patients with the correct mutation. I thought that if they would pay a higher dollar amount for one groups of CFers they would pay for a less expensive version for another. We all know life isn't fair and insurance companies rarely make sense so I guess thinking my insurance would cover this medication was faulty logic.

The really horrendous part of it is I am not sure I will be eligible for the Vertex patient assistance program for people who have insurance refusing to cover the cost of the drug. This could mean that unless I magically become extraordinary wealthy, an extra $20,000 a month wealthy, I may never have access to this medication.

The only aspect of this whole situation that is giving me hope is that CF doctors in my state are coming together to fight for their patients so that we can all access this medication. The CF community is full of doctors who genuinely care for their patients and for that we are lucky. The only other fiber of hope I have is that potentially my insurance company is negotiating price and may eventually strike a deal with Vertex. The part that is the most frustrating is that I am being left in the dark. The morning after I found out my insurance is refusing to cover Orkambi through another cyster I called my insurance company, my clinic (the CF nurse is on vacation), my pharmacy and vertex. I got zero response and no answers. Now that it is the weekend I have to wait until Monday to hope that someone will answer their phone to provide me with some answers on where we go from here.

Cystic fibrosis patients wake up every morning from the moment they are born until they die ready to fight. It is the only way to ensure we wake up every single day. I am ready to fight my insurance in any way possible, my life is on the line!

Saturday, July 18, 2015


There is a flurry of emotions whirling through my mind. I feel as if I am teeter tottering between every emotion that a human can possible feel throughout each and every day. Why all this emotional instability? The newest Vertex drug, Orkambi, is the culprit of all this emotional confusion.

The two years of utter free falling into a dark abyss from this disease made me wish with every fiber of my being for Orkambi to be approved. Every wish my heart held so dearly revolved around something, anything that could pull me up, even just a little, from this bottomless well. Now that Orakmbi is here I feel such a mix of emotions I feel I may go crazy with uncertainty.

The optimistic side of me is feeling excitement so strongly that I can feel it buzzing in my bones. The optimistic side of me wants to scream and shout and jump up and down that there is something, anything that may help me maintain the last 5 months of stability for the next several years. The optimistic side of me want to believe that this drug will work well for me and I will gain 4% lung function or more! I want to have more energy to chase and swim and bike with my daughter. I want to gain weight easily, and have fewer aches, and have more stability in my day to day life when it comes to health.

And then a voice, just as loud and adamant as my optimistic voice, comes bursting into my mind. The voice is my realistic (pessimistic?) side and it is reminding me that not ALL people experienced increased lung function and there were a few that experienced side effects that required them to stop the medication (mainly elevated liver levels). Some felt nothing and others had symptoms that were troublesome and even a bit scary like extreme shortness of breath.

To add icing to my troublesome cake I feel paralyzed with fear that my insurance, which has been reviewing my case for over a week now, will deny my ability to even try this medication.

So much of life with Cystic Fibrosis is luck. What mutations you have, how the disease presents itself, what bugs you catch and how much havoc they wreak on your body, how well you respond to meds and if you are allergic to them. Here is just another example of luck. I have heard this drug has been a life saving miracle for some people and for others it is a nuisance not worth their time. How I feel about this medication is anyone's guess and sheer luck just as most things with CF. And of course, luck will determine if my insurance will even cover this $259,000 a year medication.

I just hope that this time, lady luck is on my side!

Thursday, June 18, 2015

Never Growing Old

My husband and I went away for several days without the little one. There is something so renewing so refreshing about skipping town with the man you chose to marry all those years ago. The man who made a younger version of yourself nervous and giggly, but now makes you feel safe and content.. The man who in the past would make your face light up when you would talk about his warm eyes, strong arms, and addictive smile, but now fills your heart with a sense of home and belonging as you talk to your friends about the adventures the two of you, husband and wife, have recently experienced. The man that would make your heart flutter and your palms sweat when you saw his name on your caller ID, but is now the very first person you want to call with news, both good and bad.

After three years of parenting together it was nice to be able to focus on just the two of us for more than an overnight here and there. We spent our days hiking through the forest and our evenings laying in the warm sand listening to the ocean waves crash near our feet. We got tipsy at lunch and ran while bursting into fits of giggles because we almost missed our steam train ride back to the hotel. We had good wine, the freshest seafood, and amazing conversation.

The last evening after several days of rekindling the zest for our love that was ignited so many years before, we sat down to a romantic Italian dinner. As we were discussing what our evening plans entailed an elderly man was seated at a table across from us. He had trouble lowering his tired old body into the seat and his hands were shaky as he reached for the menu. He pulled out a magnifying glass to look at the menu and carefully sipped his wine while waiting to eat. My husband kept glancing at the man and would whisper, "that makes me so sad." Although, the man dining alone filled my heart with a sad loneliness as well I was surprised at how fixated my husband was on this gentlemen. As we finished out own meal the elderly man had finished his and left. My husband, now safe to speak freely, looked at me and so quietly so heartbreakingly in a voice barely above a whisper said, "That will be me someday." I instantly had clarity as to why my husband was so transfixed by this older man dining alone. I quickly felt my heart shatter with sadness for the man I love so very much because we both knew that realistically CF will take me long before either of us sees old age. I was reminded again how heartbreaking this disease is and that even though we live each day as if life were normal there is always the looming knowledge, the heavy sadness that this disease is killing me and leaves me little chance of ever growing old with my husband. It reminded me how angry I was at this disease for what it does to me, how it destroys my body and my life, but most of all that it takes all those who I love the most in the world and drags them down with it.

We had a moment of understanding and acknowledgment of how this disease can break our hearts and then in true fashion to my husband he followed it quickly with, "at least I won't have to argue with anyone about what kind of wine to order." And just like that we pushed the fear behind us and moved in with life because we know our days are numbered and our moments precious so we can't dwell in the what ifs and sadness.