Wednesday, June 30, 2010

Vertex...So Close!


So, we all know that there is no cure for Cystic Fibrosis. I have always known this. No cure. It is the worst. Even though this is true, I, my family and friends, have always hoped that one day some one would find a cure; or something close. Since 2006, Vertex Industries has been working on two amazing drugs that seem to me, unbelievable. When I heard that Vertex was working on medications to target the actual cause of Cystic Fibrosis, my heart literally leapt with joy. I remember reading about VX-770 and VX-809 (the two drugs) for the first time earlier this year. Reading about how these drugs would target the defects in the mutations G551D and DeltaF508. Was I reading it right? Yes, I was! These two drugs deal with my two gene mutations!!! As i kept reading about them I started to cry. Tears of joy rolled down my face. I seriously couldn't believe it. I scrambled calling my momma and reading it to her over the phone. Basically, VX-770, which is in the last phases of clinical trials before getting approved by the FDA, helps the CFTR proteins open up and work properly. VX-809, in phase two trials, corrects the placement of the CFTR proteins, so then VX-770 makes them work. Can you even imagine how smart the people have to be to figure out these messed up cells and fix them must be?! Amazing. Cystic Fibrosis is so much more complicated then people describe it to be, which makes it very hard to even imagine that scientists are making such a breakthrough already. I have talked to people about the VX-770 study. Lung function has improved like crazy, like almost 15% higher. That is a big deal, beyond a big. I have read what someone who is in the clinical trial wrote and said they can't believe they feel HEALTHY. Now, a lot of us with CF will say we feel good, because to us coughing everyday is our normal good. To feel healthy I can't even imagine. This woman said that she has even backed off a little bit on doing nebulizers, and her lung function has still improved. Crazy! If I can stay healthy enough, I hope to become involved in these life changing clinical studies. These drugs could change my life. Yes, I know this is not a cure, but hey, I think its getting pretty damn close! Knowing that these target me specifically (my mutations) it could help me have so many more tomorrows. It gives me hope to continually want to try and get even better so that when these get approved, my lungs will again hit PFT numbers that I never thought I would see again. Please continue to believe, have hope, faith, and pray that these drugs will continue in their incredible movings toward getting approved by the FDA; because when they are, it will be THE best day of my life.



To help you understand what it looks like (not my x-rays):

These are "Normal" Lungs
(notice how clear they are)


These are lungs of Cystic Fibrosis
(the cloudiness is scarring)

Sunday, June 20, 2010

Dear Daddy,

Thank you so much for everything you do for me, it means the world. I feel terrible that I can't drive home for father's day this year; my first father's day away and hopefully that last. You've always been supportive in my dreams, whether it be dancing my heart out, going to college, or more relevant to today making sure I keep my health in check. One memory that sticks out a lot is the dance "Butterfly Kisses" for Kids Unlimited several years ago. It was the father daughter dance that I got to do ballet in and the daddy's got to join us. I remember the practices at I think it was or one of the middles schools, the outfits with light pink, the picture we took in our outfits, and the dance on stage. Crazy how vivid I can remember it, and how fast time flies! I know I'm growing up, but I will always be your little princess. It was fun though. I know we've had our bumping of heads but this past year or so, our relationship has been blessed. I am thankful everyday that we have become so much closer and that our relationship is continually growing everyday. Now, you need to rest up and let that knee heal, so we can go dirt biking! I hate being away from family and holidays; hopefully I will be visiting home soon. Happy Father's Day Dad, I LOVE YOU!

~Sunshine


Friday, June 18, 2010

The Best 3hrs of Breathing


For three hours out of the day (at least), I breathe in what feels like the best thing ever. Waking up in the morning, my lungs crave my nebulizers; they crave the antibiotics. Duoneb, the bronchiodialator, that calms my lungs from spams; trying to help them relax and clear mucus with each breath I breathe in. Next comes the Hypersal. Man does Hypersal kick my butt, but the outcome of it later in the day is well worth it. Hypersal is hypertonic saline 7%; extreme salt water for interpretation. Breathing this in is amazing, as it makes the dry thick mucus in lungs hydrated like it should be, so I can actually get things moving and get it out. It irritates my lungs a little and helps me cough stuff out that I normally never would. While I do this, I strap on my vest. My best friend calls it the shake-n-bake. There is no "bake" part but the shake part is very true, as it shakes me and percussions my lungs to move everything around so mucus doesn't build up in one place causing a lung infection. After thirty minutes of that, I start my next neb of Pulmozyme. This helps loosen up mucus and cause it to become thinner; and this one only takes about ten minutes. Lastly, I start the antibiotic nebulizer. This one takes forever, but is my favorite; for it helps me the most. Tobi targets the bacteria psuedomonas that grows in my lungs that the mucus has trapped and made a nice convenient home for. Gee, I sound like a hazard. Tobi, I do as a 28 day cycle and on my 28 days off I do another antibiotic called Coliston. This thirty minutes of nebulizer fights for me what I can't fight. I finish it up with a quick shot of Advair and meds are complete. When I'm done with everything I feel good, I feel great. This is my everyday, every morning and every night routine. It really is a love hate relationship. I hate it because I HAVE to do it, but I love it because I can breathe.

Wednesday, June 16, 2010

My Job

Growing up is a little difficult. I have a job. I work for my body and Cystic Fibrosis. I've recently learned that CF is a full time job. The moment I wake up it starts by reaching over, grabbing a new nebulizer cup, getting the duoneb med, strapping on my vest, and plugging into my nebulizer; all so I can breahte. Every morning I do this for I'd say at least an hour to an hour and a half. I do that same thing at night. I also do two treatments in the middle of the day when I can fit them in. On top of all that I take many pills every morning, and even more with everything I eat. That's my first job. Of course I also need a job that makes some money.

I finally started working again, since I've moved. I commute about a half an hour away, to work in Fresno at Buckle. Working there so far has been great. All the people I work with are very welcoming and the manager is very nice. I have been put on the team as a part-time employee. I've only ever had a part time job and I have managed it quite well. Keep in mind that my part time jobs have always been at tops around 20 hours a week. When my manager asked me if i wanted some more "full time weeks" thrown into my schedule every now and then I said "Yes," thinking that more hours means more money. Well I got this next weeks schedule and he gave me a full-time week. I'll be working some 8 hour shift. A lot of you might be thinking, "Yea so, not that big of a deal, I work that much everyday." My problem is that I already have one job without Buckle; my CF. So, yes, I am nervous for this up-coming week. I'm resting right now since I have two days off, but I am also praying that my body works with me this next week and doesn't get exhausted, weak, or sick. So, please, pray for strength for me and for my body. Thank you!

~B

Sunday, June 13, 2010

Working Out :)

I'm starting my personal work out today! Exercising is so important for those with Cystic Fibrosis. Cardio workouts are key and keep our lungs in shape and healthy. When I was in high school I was always active everyday with cheerleading and dancing; participating in more than two hours per day. After high school I stopped doing a lot, and I think that has contributed to part of the drop in my lung function in the past couple years. So, to fix this, I plan on signing up for summer dance classes this week, and I can't wait. I have to get some exercise in to get my lung functions up; even if it's one percent at a time. My next doctor's appointment is in a little more than two weeks. I have two weeks to gain some weight and improve my health for this first "check point." Tonight I went on a bike ride for about ten minutes; but I'm going to have to drive my car around to see how far I went. I also did some stretches and a little living room dancing so I can start getting in shape for dance too. I can't wait to build some muscle; and actually have some to flex!!! :)

Saturday, June 12, 2010

Oh, How I WIsh...

I wish i could reassure you. It would be nice to know myself, what I'm going to feel like the next day. Not knowing is the worst part of it all. When someone says "Oh, you will get better," I automatically respond with something positive like "Oh I know, I will." Deep down inside when I am told something to that extent, it kills me to say a lie; because if I couldn't tell a lie I'd probably reply with "I HOPE so, I hope I will be able to get back to where I was before." I wish the consistency in my life wasn't a cough, a hack, or deep breath every once in a while. It kills me that I'm not able to reassure you, honestly, and tell you everything will be okay.

Short, Sweet but full of Love.


Love is not just a rose, a box of chocolates, an anniversary, or a holiday. Love is beyond that; beyond an emotion. It has the power to change anything. I choose to love with all of my heart. I agree with the saying "it is better to have loved and lost, then to have never loved at all." I believe love is strong and can touch people unexpectedly like they never thought it could; whether it be in a smile of a passing stranger, a hug from best friend, the opens arms of a family, a the butterflies from a kiss from your true love, or the unconditional love of God. Being surrounded my love my entire life, it was only natural that I would "pick it up" and pass it on as well. I am very blessed to have so many people in my life that love me; I truly am. It wasn't too long ago that I felt like I was selfish when it came to any form of love. Its a hard thing to explain.

It sucks to say, but CF effects my relationships too, especially through my teenage years. My CF was and is something that is always on my mind, and I believe (or use to believe) was always on my friends, boyfriend, or family members mind. Sometimes I didn't want to have to get so close to another person because I felt that getting close to them was being selfish. See, I told you, hard to explain. You're probably thinking, what? Having Cystic Fibrosis I know that my time here is will be shorter. I mainly went through this difficult subject with a significant other. I didn't want to get so close to someone get married and what not then pass away, cutting them short of something that two people have the joy of sharing throughout an entire "average" lifetime. I hope this is making sense. I felt like me creating relationships or loving someone was just me setting everyone up for eventual heartbreak. I felt like a person deserves to have a full lifetime of love from someone, and not just half. Part of this, was me being afraid; not wanting to have to loose all those who mean so much to me and who I love one day, and not getting to spend more time with them.

It was hard for me to get past this stupid idea that I had in my head, but then I realized that it was ridiculous. We are here to spread love. When I look back in my life right now, the ones that stick out are all of those filled with love, joy, and happiness. Why spend my time worrying, being afraid, and not making people happy? The truth is, is that no one really knows what is going to happen to them the next day. When I do leave (which hopefully won't be for a long time) I want to be remembered for love; and touch as many lives as I can for the better. So, with my knowledge now and how I've grown, even though I know what is "suppose" to kill me, will kill me sooner than those around me, I choose to love; and I hope you will too.

1 Corinthias 13:13 "And now these three remain: faith, hope, and love. But the greatest of these is love."

Saturday, June 5, 2010

My CF Awareness Video

video

Blessed with Strength

Today I've just been browsing CF sites and just relaxing, eating, and doing treatments. Strength has been on my mind.

Being strong is what I believe is an understatement, for those who have Cystic Fibrosis. Every person I know, read about and hear about; they have more strength than anyone I know. Strength: 1. the power or state of being strong; vigor 2. mental power, force, or vigor 3. moral power, firmness or courage. Our bodies are weak. Our bodies are not strong, but we have strength to push through it all. It seems to me that those with CF have more hope. It seems that God blessed us. Giving those who he knew would have the strength to handle this disease; to turn this breath taking disease and see it as a blessing. Someone once posted a question on a CF blog site asking "if you could go back and choose to have CF or not would you?" This is a question that is easier than you might think; and you might get a different answer than you would expect. I would not want to change a thing. True, life would be so much easier, simple, less stressful. If I didn't have CF though, I wouldn't be who I am today. It has helped shaped ME, but it is not all of me. I have been faced with challenges and I have overcome them. I know that there are more battles ahead and it makes me want to continue to be hopeful, have faith, be strong, have courage; know that God does not give us more than we can handle.

Many people pass away from Cystic Fibrosis at a young age. An age that seems unfair. In a way it is, but not entirely. Because I know what could happen, I take a different aspect on life. I know I appreciate every family member, friend, laughter, happy moment, sunny day, and breath so much more than anyone else. I love my life exactly the way it is and wouldn't change a thing because I know I am blessed.

Tuesday, June 1, 2010

Finally Moved!

So for a few months now ive been planning this next chapter in my life. I had been planning and trying to make plans to move so I could go to school and work in Fresno; as well as relax. Looking into different living options, I found and was blessed with the perfect set up! I have family in Chowchilla, so my Uncle David is letting me live in a house he owns. I'm now living with my awesome cousin, Colleen, on the almond ranch.

I just moved in yesterday and I am so excited! It's so nice and I cant wait for more fun to come. I had my meeting with the manager at where I'll be working today; so hopefully I get put on the schedule soon. Other than work and having fun my goal here is to relax but still get some exercise that way I can improve my health.

I had my one month check-up at Stanford last friday. I thought I was feeling good, though I did have a feeling I wasnt feeling better than when I got out because I had starting to cough a little that week. Unfortunately, my numbers went down 5% :( I was very disappointed in myself. On the upside of things I had gained weight, now weighing 105lbs!

This was just a little update...more too come and pics of my new place soon!

~B