Tuesday, May 3, 2011

Guest post: Can you blame a girl for hoping?

Because it gives me a great excuse not to write while I'm struggling to get back into the swing of things post-hospital, and because I think she did a great job of articulating what I so often feel myself, I wanted to share with you what my sister recently posted on her blog. (Normally I'd just link to it, but her blog is private so many of you wouldn't be able to view it.)

Teresa is my older sister who also has CF and just turned 31. (I hope it's okay that I just told everyone that. If not, we can pretend I said 25.) Thanks again, Teresa, for writing this and letting me share it. Love you!

I wrote this post a couple of weeks ago but because the tone of it is quite different from what I normally write on this blog, I hesitated to post it. However, in the interest of May being National Cystic Fibrosis Awareness Month, I am posting it anyway. I apologize if it is a little negative, but it is honest and hopefully gives a little insight to living with cystic fibrosis.

My sister was admitted to the hospital last week for another two week stay to treat her cystic fibrosis. This is her third stay in nine months.

She called me the day before she went in and we talked for quite a while. You know, I really wish Jenny didn't have CF (well, I wish I didn't have it, too, for that matter), but since we both have to deal with it, it's nice that we have each other. It's nice that we can share our thoughts and feelings and know that the other has either felt that way, or at least understands at a more personal level than most other people can understand.

Well, we talked a little about how these hospitalizations have become common place enough that we no longer burst into tears in front of the doctor when he says "well, I think it's probably time to come in for a clean out". There is no bargaining, or begging, or pleading. No "I promise I'll do my treatments 4 times a day. Just don't make me come in the hospital." In fact, usually now, when we feel sick enough and are having a hard enough time breathing we are the ones telling our doctors "I think I need to come in." Our admissions are more matter of fact, a little less dramatic than they used to be.

But there's another side, a more practical side to having to be in the hospital, and it is this side of it that had Jenny stressed out when we talked on the phone. When a mother knows she is leaving her children and husband for two weeks there is plenty of laundry that needs to be washed, a house that needs to be cleaned, and grocery shopping that needs to be done. All the while you want to spend every second possible with the babies and the husband that you will miss and try to alleviate the stress that you know they will feel in your absence. There is a lot of worry for our husbands who have to shoulder jobs, housework, cooking, and children while we are away. It is an all around stressful time for everyone.

When I was born, the average life expectancy for someone with CF was 18 years. So many advancements have been made and that number is continually on the rise. It is not unrealistic to expect to live to 40's, 50's and beyond. And as I said, the life expectancy is always gettting longer. But the fact is, that even if Jenny and I can hope to have near normal life expectancies, these hospitalizations are never going away. CF is a progressive disease, and we have to come to terms with the fact that our time in the hospital will only increase. These lung infections will only get more frequent, and our time spent doing treatments at home will take an increasingly large chunk of our time. I think we both have a pretty optimistic outlook on our lives and our illness, but in the quiet moments, this is the reality that we have to come to terms with.

I just finished participating in a study for a new CF drug, VX-809. It is actually used in combination with another drug VX-770 (770 alone can be used for some people with CF but for those with the most common CF mutation the two drugs must be used together). These drugs are the first that are aimed at correcting CF at the root, the cellular level, rather than treating the symptoms. Clinical trials of the VX-770 have had amazing results. Lung function of study participants increased an average of 10% and they had significantly fewer cf exacerbations. Basically, lung function improves and then remains stable. It is anticipated and hoped that the VX-809 will have equally amazing results.

So,what exactly would this mean for me and Jenny? It would mean our lungs would improve to within normal functioning capacity. It means our trips to the hospital would be few and far between. It means, as my doctor explained to me, that we would "maybe have to do your treatments every once in a while, like if you got a cold or something." It means that we would be "almost normal" (a great post on the topic by my friend, Cindy).

The studies for VX-809 are in the early stages. No one knows if it will work the way we are all hoping, and even if it does, there is no guarantee for me and Jenny that it will work for us. There have been plenty of times in the past when it was thought we were very close to a cure for CF, yet we still haven't quite gotten there. So I understand the people who may be thinking "don't get your hopes up yet." or "don't put all your eggs in one basket". And yet, my hopes are up. My fingers are crossed. I can either make peace with the reality that is currently before me, or I can look optimistically toward my future, believing that "almost normal" is truly within reach. Given my options can you blame a girl for hoping?

Now, on a slightly different topic, a friend posted this analogy and related it to a diagnosis of CF (particularly for parents of children with CF) but I think it applies to me, living with CF, and hopefully can apply to many of you in the various struggles and challenges you face.

Emily Perl Kingsley
c.1987 by Emily Perl Kingsley. All rights reserved.
I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

Happy CF awareness month, everyone!


  1. I absolutely loved this post of T's when I read it. She is able to articulate so well the struggles of a parent with CF. I adore your sis...and you too!

  2. I really like it over at her blog and I really like it here. I frequently wish that she would share more of her thoughts with us, and I told her. I'm glad that you have each other although you have to stay 3 feet apart :)

  3. So very well written and thanks for sharing. Wishing for you and all CF patients these new VX drugs will be the cure you've been waiting for.


  4. Jenny, I hope you're feeling better and settling in back at home as easily as possible. There is ALWAYS an adjustment period after a hospital stay. What a treat to "meet" your sister and read her words. She touched on SO many issues that I deal with: the guilt of leaving husband and child behind to fend for themselves while I take care of the infection, and the knowledge that the hospitalizations will only increase in severity and frequency with age are both so difficult to deal with.

    I always think "Okay, this I can deal with but no more", and yet there is always something more down the road.

    And yet despite the struggles and fears there are so many blessings. So so many. That we even have spouses, children, and households that we must occasionally leave behind!

  5. I knew you had a sister but had no idea she had CF to! I agree having a sibling that has CF really helps cause they know what your going through... my sister and I are really close because of it. My little brother has it also but he has always been really healthy unlike me and my sister right now. This was a great post, you and your sister have such a talent for writing and being able to put feeling to words. Great post by your sister (:

  6. Thanks,everyone, for your comments. It is kind of fun to hear from all of you since I don't get too many readers on my private blog.

    I didn't properly attribute the Holland story, but it came from Stacey's blog (as in, Stacey, who left the first comment on this post). I would definitely recommend reading it in her post on being diagnosed with CF.

    Thanks again, Stacey, for letting me use it.

  7. I loved this blog! I feel all of these things with every hospital stay, and every drug that is so close but not quite there. Hope you are doing well and getting back to your routine, I hate the adjustment period!


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