To be fair. I didn’t know what CF was when my son was first diagnosed. And now, nearly nine years later, it has become such a huge part of our lives that I forget that others have not a clue. They attempt to relate (as the title suggests and¬†no lie, this was said to me this week) but fail miserably. Bless ’em.¬†I have heard some good ones, but currently this is my favorite. I just smiled and hoped that she would investigate CF and find pie on her face. She better. She is my son’s teacher this year. It’s gonna be great!

I try not to be so frustrated with ignorance, but no need to one-up me here. This isn’t a game show where the¬†most troubled wins some home make-over.¬†And, I’m not telling you to make you feel sorry for him (or me). Generally, I do not share it unless there is some situation that warrants you needing that information. I, otherwise, do my best to pretend we are completely “normal.”

Normal. Now there’s a word that plagues us and the CF community as a whole, right? There is no such thing! Although, I have gotten good, really good, at pretending. When asked by other parents during our “tune-ups” at the hospital why I am there, “My son just has CF.” So, I probably just perpetuate the ignorance, I know. I just try¬†so hard to rest on the positive aspects of our path and smooth out the bumps. Eternal optimist, I claim¬†to be.

I digress. For those who are all too familiar with Cystic Fibrosis, you understand my plight. For those who have no idea what CF is and looking for answers and/or direction. Welcome and buckle up!

……

Our doctors did a great job in the beginning; informing and comforting us. Consultations, social workers and literature happily provided¬†to help us understand. They cautioned, however, on what books and information existed on the internet. That not everything was current or factual. The only book they suggested I read (and acknowledge) is Cystic Fibrosis: A Guide for Patient and Family¬†by David M. Orenstein. It still is a great resource and remains on point with all of the medical information that you should need and are able to comprehend. However, I found little out there that truly captured the essence of CF life, as a patient or as a parent. In the years since I have had my son, I have learned that maybe that doesn’t exist because each CF’er has a different path and no two are alike. There is no benchmark, there is no compass.

So, there in lies the problem.

“Cystic Fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States¬†(70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus tha…..”

OMG. If I see that explanation another time I’m going to scream! It just doesn’t make any sense and it is so political and uninformative, to me.¬†I explain CF a bit differently, with a little¬†more emotion and a lot less¬†medical jargon…..

“Cystic Fibrosis¬†is¬†a genetic disease, passed down from the mother and the father. It¬†is coded in your DNA and cannot ‘get better’. There is no cure and, statistically speaking, averages a life expectancy of around 37. It affects three¬†major areas of the body: the digestive system, the lungs, and the¬†reproductive system. The stomach doesn’t digest food¬†at all or on its own,¬†a simple cough can become a big deal because the body works against itself to fight it off and for boys, they are generally impotent.¬†My child is¬†still¬†able to learn, play and jump around just like others. His quality of life is quite good, actually. There are a lot of things that I have to think about and watch and even more medicines to administer, but otherwise, he is a wonderfully healthy and happy little boy.” There. Do you feel informed? Did I capture some of the good and some of the bad? I realize it doesn’t put it into perspective, yet, but this is just my opening statement.

About 1 in every 31 Americans are carriers of Cystic Fibrosis. That means that there are over 10 million carriers in the U.S. alone. If you go to a party, there will be at least 1 carrier present. It gets more¬†mind-boggling when you think about procreating with that ONE other person in the room and [possibly] creating CF as a result. The chances are slim, but there. I’ll tell you one thing, it¬†will swiftly make you question every decision you ever made.¬†The personal guilt that goes along¬†with the genetic part of this disease is intense. I procreated (after marrying) that one other person, three times and I got one¬†child with CF, one a carrier like me and one is completely clear. Those are your three¬†paths when all the stars line up for you. A 50% chance to create another carrier, 25% chance for no CF, and a 25% chance for CF. Roll the dice, I did.

CF has not been a death sentence for us and I refuse to ever allow my self to feel that way. We do our best to take it in stride, but when the road tries to change and throws in some curves, I definitely start to waiver on my¬†optimism. To me, that’s the most difficult part of this disease. It is ever-changing and has no respect for how well you have¬†settled into a pattern. There is a cadence to our days;¬†breathing treatments, medicine regimens, eating healthy and staying hydrated. Exhausting some days, but good¬†maintenance is key and our rituals¬†bring some comfort. Even when we are admitted for our “tune-ups”, I find comfort in knowing what to expect. I have never enjoyed a pity party (even if it was for me), but I do hate the unknown. When my son’s “normal” starts to change or he gets the first clear of the throat, I know it’s time to suit up and get ready to battle. I’ll be damned if I let it get the best of me.

So, in all of this, maybe you can find some insight if you are one of the mis- and/or uninformed. If you know all too well, I hope you can empathize and find comfort in hearing another’s journey. I won’t be the one updating every day on lung functions or doctor’s visits, but I do want to share the “meat” of CF. The wide spectrum of ups and downs and how we cope with them. I want to inform¬†and connect¬†without¬†the bore of¬†the mundane and trivial¬†(no promises).

It has been difficult to connect with other CF families and learn more myself about the disease. There are some seriously strict contact precautions between CF’ers and families. I have reached out on Facebook to no avail, FB is all about “me” these days. There is no other mom in clinic as they are so careful to move us swiftly out of the waiting area and no support groups for CF in our area….another harsh reality of CF…..it’s a lonely path, physically and intellectually.

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Our ClanThe greatest job in the world….and the most difficult one. There are ups, way-ups and downs, waaaay-downs. I have experienced many a roller coaster and learn with each corner and roll. I hope to find others that can relate, connect and enjoy. Laughs, tears and the hopes of motherhood…….

Hold on, little one.My son had a rough start into this world. He was over 6 weeks early and, as we would later learn, in distress. We lived amongst the halls of Childrens’ Hospital in the crisp fall months of 2002. I won’t go too deep into details this go-around but I will attempt a visual of my experience…..

Imagine…..

For many years you have imagined visiting Hawaii. The breath-taking landscapes, the crystal clear waters and feeling the sun warm your cheeks. You start planning and saving for
this tropical paradise, dreaming of what you will do and the people you will meet. Finally, you have pinched enough pennies to afford a trip. Elated, you shop and pack and pack some more. How many bathing suits? How many bottles of sunscreen? Do I need a new pair of sunglasses? And so on and so on. Until your day of departure has finally arrived. You are glowing. You can hardly contain your excitement!

The airport is miraculously a breeze. Check-in is quick, the lines seem to speed along and even security is pleasant as you make your way to your gate. You settle in after boarding, resting your head on the back of your seat, close your eyes and anxiously await your paradise. You gently nod off in your daydreams, a gentle smile graces your face.

“Please return your trays to their upright positions and buckle your seatbelt. The captain has notified us that we should be landing shortly.” Hawaii! Oh, how exhilarating! It’s time! You have waited so long! I bet it simply just smells different! Is it going to be like the movies and a woman is awaiting every passenger’s exit, placing a lei over their head as they exit?

You joyfully grab your carry-on and await your turn to exit the plane. Slowly you move towards the door and attempt to peek around heads and shoulders hoping to get just one tiny glimpse. Your counting down now. Only five people ahead of me, now four, now three…

And finally, you have arrived to the exit of the plane. You stand in the doorway wanting to breathe it all in at once, bask in all it’s glory. You stand there, eyes closed, waiting. A breeze lifts your hair and kisses your cheeks, but strangely it doesn’t feel warm and welcoming as you had imagined. It was a chilly breeze. One that forces you to wince in shock and discomfort. As you slowly peel your eyes open they resist at the brightness, not of the glowing sun and blue skies but of white, crisp snow and overcast clouds glaring above you.

How could this be? What happened? Am I dreaming? Some one has made a mistake! This can’t be right! You are greeted by name and welcomed to Siberia…wait, what?! You protest. You pull out your ticket, yes, see, Hawaii.

“No ma’am. There has been a correction, this is your final destination and no return flights are offered. No doubts and no refunds. Buh-bye.”

Packed and dressed for the hot summer weather, you shake in the chill. You stand, stunned and confused. In your daze, your luggage is tossed from the plane and closes its doors and begins to taxi away. Leaving you. Stranded and alone. The other passengers have dispersed. While they seem just as confused as you, they pay you no attention and scramble to find shelter and comfort from the cold. It’s just you and this suitcase of swimsuits and sun-hats, all of no use in this unfamiliar and desolate place.

In the days and weeks and months to come, you learn how to manage your new residence and way of survival. You have clawed, scrounged and fought through it all and now even seem to have found a rhythm and an acceptance of your fate. You still daydream and wonder about Hawaii. What if I had made it there? What would I be doing? How different things would be….

And so, this has been my path. Or, at least, the beginning of it. The harsh reality and eventual acceptance and ownership of a plan gone wrong, a destination never reached. Within hours of my son’s birth he was wisked to emergency surgery; and after a long recovery and many tests, my first born child was diagnosed with Cystic Fibrosis. I would later learn through consultations and reading material what this meant for him, for me and for our family……Welcome to Siberia.

Posted: July 29, 2011 in Cystic Fibrosis
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