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

Anticipation

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.

Friday, June 12, 2015

3 Years Old

My Sweet Baby Girl,
You are three years old today! Although when people ask how old you are you are adamant you're still two. You have grown fond of being two and it may take a while to accept the fact that you are officially three, but that is okay you have a whole year to get used to the idea.

When I look back over the years I am amazed at how much has changed, but I am even more amazed at how much has stayed the same. So much of who you were as an infant is still exactly who you are as a toddler.

You still are extremely expressive and it is almost impossible to get a picture of you without some strange or goofy expression. When you were an infant I was envious of all my friend's who had sweet smiling pictures of their sweet babies when all of my pictures of you were with a furrowed brow, or a shocked expression, mouth agape. Now as a three year old your expressions are just as wacky, but I no longer long for sweet smiling pics because baby girl you have too much personality to contain and it shows with those wild pictures.

You have always been an artistic spirit even from a very young age. The arts move your soul and fill you with joy. I have set up an art station in our home where you frequent up to a dozen times a day. You spent the majority of the day coloring, drawing, cutting with your kid scissors. I have never seen a child so passionate about being creative. When we blew up balloons for your party you asked if you could draw on them to make them more special, when you are in the bath you spend your time with bath crayons coloring the walls, outside you spend most of your time drawing with chalk. You even started bringing a notepad and colored pencils in the car so you can draw while I drive. All the practice has made for some pretty impressive art and a huge mess in my house! Oh, but to watch you create brings me as much joy as it brings you to be the one creating and so I don't mind the mess and I love the creations.

You were an early and constant talker. The amount of talking has only increased and it is rarely quiet in our home. You always have something to say and you often get mistaken for much older because of your ability to talk, and talk, and talk! Your understanding of Spanish is continuing to develop, but at a much slower pace than English. You understand everything that your daddy and some of our Spanish speaking friends say, but you still prefer to answer in English or Spanglish.

When you were a baby I often felt sadness when I thought of the days passing and you growing older. I wanted to keep you as my little baby snuggled in my arms forever. But as you grow older I get more excited for the future seeing glimpses who you will become. Our mommy daughter dates are full of conversation across the dinner table (although that of a three year old variety) and we are starting to have many of the same interests which makes for such a beautiful relationship. I like to think that we will grow closer and closer as we both age and that our mother daughter relationship will someday be that of two best friends, drinking coffee in the early evenings, eating brunch on Sunday mornings, chatting on the phone and enjoying all the beauty life has to offer with one another.

Our future looks bright together, kiddo.

Tuesday, June 9, 2015

Public Pool Humiliation Again Again

Yes, I used again twice in my title. Yes, I know that doesn't make a whole lot of sense, but I never claimed to be a real writer and it is the internet... they let anyone post anything. There are no rules.

My favorite exercise over the years has always been swimming. Yes, I have dabbled in other forms of exercise like jogging and zumba, but I always find my way back to swimming because we have history together. And as the title implies our history has not always been drama free. In fact, nowhere else in my entire life have I experienced humiliation like I have at a swimming pool. And although it has been years since I had an episode such as explosions of mucus or a nip slip complete flashing fiasco I have once again found myself humiliated while donning a bathing suit.

This evening when I arrived at my gym I frantically shoved my belongings into the locker slammed it shut, programmed my locker with my secret code and half walked/ran to the pool. I have this irrational fear that if I don't get to the pool as quickly as possible after arriving at the gym someone will take the last lane and I will have to wait for someone to leave so I can workout. It makes no logical sense, but there you have it.

So I have a decent workout and go to redeem my items from my locker. I punch in my lock code and nothing happens. Oops, wrong locker. I try a a different locker, nothing. I decide to shower and try again afterwards, as if that would change something. To my surprise it did change something. Instead of desperately trying to open my locker in a bathing suit I was now trying to open my locker while holding a towel around my naked self. And as I try over and over again the locker room starts to fill up with women getting changed for water aerobics. The more time I take trying to open this locker the more women start pouring in. Realizing it was a futile task I ask a woman to go to the counter and find someone to help me.

The gym employee comes in and asks for my secret code and starts punching the code in the keypad. I have been at the gym for over a year so I am pretty confident I know how to punch numbers on a keypad, but who was I, naked and cold, to judge. She then starts questioning me, "are you sure this is your locker?", "Are you sure it was on this wall?", Are you sure it was on the top row?" At this point I don't know anything anymore except that my naked confused self is providing a lot of entertainment to the other naked women in the locker room.

Finally, after I promised ten times and swore up and down that locker 122 was indeed the locker that contained my clothes (even though I had NO clue if that was true) she punched in her master code and opened the locker. Low and behold, it was my locker. Half the women cheered, it was that big of a deal. A couple murmured that was their worst fear, and eventually everyone left to their class.

And so now I have learned several lessons over the years: Don't try to retrieve mucus out of a pool, frumpy bathing suits are the way to go, and pay attention when locking up your belongings.

Wednesday, June 3, 2015

Orkambi

As most of the CF world is probably aware of by now, the vertex drug developed for DDF508 has a good chance of becoming available to patients this July. Everyone in the CF community was hopeful this drug targeting DDF508 would be a life changing miracle drug just like Kalydeco was for people with the G551D mutation. Unfortunately, the results of the study quickly dashed many of our dreams that this drug would be our miracle drug.

As much as I am disappointed that Orkambi, which will work on my mutation, is not the life changing and life extending drug that Kalydeco is for a select few with CF. I can say I am beyond excited, despite having all the facts, that we may have another medication that could potentially help slow the progression of my disease. When you live with a progressive disease new treatments are never developed fast enough. In fact, discoveries to treat CF are far and few between. And even when therapies are developed I have found myself allergic and unable to take advantage of many of these medications and treatments. The progression of the disease is always three steps ahead of the treatment and watching your lungs and body deteriorate is heartbreaking at best. It development of treatments are very much a matter life or death for so many with CF. This is why I have to roll my eyes at the naysayers that complain about the humble 3% increase in lung function that were seen in the Orkambi study.

What the naysayers don't realize is that 3% may not seem clinically significant, the truth is that the drug is so much more than 3% increase (on average) in lung function. It is the fact that is can slow the guaranteed progression of this disease. That is something that can not be taken lightly. To me, it  means I may have a few more years added to the end of my life to watch my daughter turn into a woman. It may mean one or two more anniversaries I spend with my husband. It may mean another birthday and a few more grey hairs. So yes, 3% may not seem like a big deal to someone who hasn't had an expiration date stamped to their lungs. It may not seem like 3% could change a life, but pessimists are stuck on a low percentage and not the potential for what slowing, even minutely, can do to a life. How a few years can mean so much to a mother, a wife, a sister, a friend who would do anything to hold on to their life for just a little longer.

So yes, lets celebrate the accomplishment of the researchers, the foundation, the  CF community that we may (as long as the FDA gives the okay this July) have one more tool in our fight again CF, we may have a little more hope for tomorrow, and we may have a little more time on this Earth.

Sunday, May 31, 2015

Spring

Spring has arrived gently this year. The heat of May never showed its face and we have been soaking up the cool days and even cooler nights. This spring weather in combination with my behaving lungs has brought a constant flurry of activities. It has been a welcomed change to the hibernation and sickness that winter brought and I feel I emerged this spring as a new person.

Rather than finding comfort and therapy through the keys on my laptop posting to this blog, I am finding therapy in the days I spend splashing in the river with Kaylee, conversation with friends and neighbors, long walks and bike rides, feeding the baby ducks along the canal, and spending entire days tending to the garden.

I am finding my peace watching Kaylee pick handfuls of beans and a fist full of carrots only to flop down in our strawberry patch and have a breakfast straight from the Earth.

I am finding my healing in the mornings that I don't wake up coughing, the laps I can swim at the gym pool, and the roundness of my belly that only comes when my health is doing well.

I am thankful every single day that I wake and feel well, feel like myself, the self I should be. Not the self stifled by this debilitating disease. I know, after 31 years with this disease, that hard days are around the corner, my lungs will struggle to get my through the day, and the hospital will be my temporary home once again, but until then I am basking in the warmth of the sun, fresh air, and health that this beautiful spring has brought along with it.