Doubtfully Optimistic for a cure

In a previous blog I spoke of CF Advances and my thoughts on it. In that post I stated that I didn't believe there would be a cure found in my lifetime. I still believe this and stated I would explain at another time, I think I'm ready to try and explain.

I want to go into this blog by stating that I completely believe and have faith in the CF Foundation and they do everything in their power for CF patients, new meds and striving for a cure. There is very much evidence to prove this statement, if you do some research you will find that the CF foundation does exactly what they say they do, and the money that is raised through and given to them absolutely goes to new research. I am not a pessimistic person. I am positive almost all the time and am highly annoyed by negative people, whiners, and complainers in general. I am also a realist. I always have faith and give a great deal of my troubles to God. But I also believe in the facts in society.

Disease is big business. Pharmaceutical companies make A LOT of money from drugs to treat disease. Let me give you an example.

Vertex executives said Kalydeco would cost $294,000 for a year's supply, placing it among the most expensive prescription drugs sold in the U.S. Specialty drugmakers are known to charge $300,000 or more for drugs that treat very small groups of patients.
"The drug is priced for the value it will deliver to this very small group of patients," Vertex Executive Vice President Nancy Wysenski told analysts.
Wysenski said Vertex would provide the medicine for free to people with no insurance and household income of $150,000 or less. The company will also cover 30 percent of copay costs for select patients who have insurance.

$294,000 a year, $24,500 a month, $6,125 a week, and $875 a day. Of course as stated they will help families without insurance, and with co-pays. But lets do some math, math was always my weakest subject so forgive me if I am wrong. Kalydeco helps about 5% of the CF population approximately 1,500 persons. 1,500X $294,000=$441,000,000. The reason the drug is so expensive? It only helps a very small group of people, meaning less is being manufactured which cost more. Which ever way you cut it that is a very large amount of money. The CF foundation works in collaboration with Vertex Pharmaceuticals and other companies to do the research and production of these drugs. They do this to expand greatly on the resources pharmaceutical companies have to labs and chemical compounds, improving there chance of getting these drugs developed. Vertex and other companies also invest in the drug research, giving them profitability. Now honestly I have no idea how much money they make from Kalydeco, and I find it somewhat comforting that they invest back into the CF Foundation. The CF foundation also funds programs for patient support and care.

This is only one example of a drug, a ground breaking drug in the CF community. But If you think of all the other diseases and drugs the treat them, there is a lot of money to be made. That is why I don't think there will be cure for CF or a lot of other diseases. What I do believe is at some point there may be a daily drug that will significantly slow or stop CF. That is like a cure, yes, but a cure to me would be something as simple a vaccine, or a simple procedure to fix the defect. Would I be happy with having to take a pill daily and stay healthy forever ABSOLUTELY! Like many do for many diseases. The bottom line to me is it's a business. The CF community always stays at about 30,000 because people are always dying and always being diagnosed. We are never ending money maker for these companies. I don't think they do this out of malice, and I believe their is many in the business to cure disease. There's just a lot of bureaucracy that goes along with it. Someone once told me "We can put a man on the moon, but we can't cure CF?!"

I really hope this doesn't come off as me being pessimistic or from a place of anger. I am not angry about it or nor do I dwell on it, it's just my honest opinion. I will continue to raise awareness and money for the Cystic Fibrosis Foundation and donate to other great causes working towards treatments for Cancer, Huntington's Disease, and many more. I will take as many drugs a day it takes to save/extend my life and raising and giving money to CF Foundation will lead to those drugs. Because although we may never have answers to these diseases there is always medical advances, and that is the next best thing.

Please visit www.cff.org and look through some of the info they have, you can read all their reports and view the drug pipeline showing which drugs are where in trials.

I love my little life!!



My Brother, myself, and Sarah his girlfriend.



Me, Grandma Mable, and Colton

I love my little life with my amazing husband, son, and dogs! I have had the most amazing 2 weeks filled with so much love I could literally feel myself overflowing! If you read my last post you know I celebrated my 30th birthday on Halloween, well I thought I celebrated it! Little did I know, I would really celebrate it the Saturday after. Brad my amazing husband, my Mom and family threw me a surprise party. I was completely clueless, blindsided, and surprised! The emotions I felt when I walked in that room were beyond words, I started crying, I was happy and so overwhelmed by the love I felt from everyone in that room! So many helped to make it perfect and make such amazing foods, all my favorites! I was shaking with excitement. It's so hard to explain how I felt. As I said in my previous blog I never thought I would celebrate this many years of my life. So many in my family have always held me so close because of this fear too, no ones ever came out and said that but I've always just been able to tell. There is so many people that mean so much to me. The obvious ones Brad, Colton, Mom, Dad, Brandon and all the rest of my immediate family, but then there are people who may not think there a big part of my life, that we may just be friends or acquaintances, little do they know they mean the world to me. Every person who comes into my life has an impact, it may be big, it may be small, it may even be bad, but it's all a part of my life making it a part of me. Just like CF. I only hope these people know the appreciation I have for them. The love of my family has no doubt kept me fighting so hard to kick CF booty, especially in the really hard times when I want to just give up. The thought of their love for me and the pain and disappointment it would bring them if I gave up brings me back every time. I love my family so much!! My cousins are more like siblings to me and the love and bond I have with them is really quite special. The younger ones being grown now and bringing significant others into the family that I come to love too! I can't even go into the love between my Grandma and me in this blog, but her impact on my life is beyond words! I have a huge support system and am blessed and very loved.

Horseback riding

Then less than a week later I got to go on an anniversary get away with Brad to celebrate our 9th year of marriage. We went to a beautiful bed and breakfast, Castle in the Country in Allegan MI. It was such a beautiful area and we got to go tour downtown Holland as well. We got massages, relaxed and re-connected! It was beyond amazing. We went horseback riding which I loved, miss riding so much! Ate at some great restaurants and went crazy in a candy store! Who could ask for more?! The whole time I just couldn't stop thinking I love my little life!

Out in front of the Bed and Breakfast

Downtown Holland

Also before my birthday Brad said my gift from him was to get a puppy, I had been looking for a few weeks any ways. So we went to the shelter.I went in thinking I wanted another big dog and a girl, because I'm surrounded by boys! Well I came out with a boy, Frank, who is a Dachshund/Lab mix! If your thinking about getting a puppy PLEASE visit your local shelter! You just will never know who will win your heart, like our little Frank did!


Frankfurt Pickard Purchase

I made it!

Today is my birthday, I am 30! An age I honestly never thought I would reach until recently. As most of us with CF born in that time period, our parents were told we would be lucky if we made it to grade school. But my Mom and Dad never treated me any different, and my Mom says that she never thought I was going to die at a young age. I was always told the truth about CF, always knew the age median of my estimated life expectancy. It never really scared me, but I always felt like I was chasing my own death. I would reach an age and the life expectancy estimate would increase, which is awesome! I want to make it clear that my parents or anyone ever made me feel like I was going to die, in fact the exact opposite. It's just the inner part of me that has always felt/thought like this. A little less then 8 years ago I lost a friend to CF she was 31 and has 2 daughters that were quite young at the time. I was a newly married, new mom. This loss hit me hard, I was terrified I would leave my son at to young of an age where he wouldn't remember me. I remember telling my best friend "I will be happy if I make it to 30, at least then Colton will be old enough to remember me." I am beyond thrilled I have reached this milestone. I have done so many things my parents were told I would never do. I graduated high school, I married, I have a son, and I have even traveled some. I have lived my life to the fullest and experienced amazing things! I lived to see my 30th birthday.  I am over joyed and now that I have made it this far I will not except anything less then at least another 30 years. I also have a heavy heart. The CF community lost 5 lives on Friday to this terrible disease! Two of which were in their 40's which is amazing, but not enough, it's sad that 40 is an amazing age to reach, when in reality it should be the start of a new era in someones life! Sadly the one beautiful young lady was just 22 with her whole life ahead of her. With countless others fighting for their lives. I love my birthday, I always have. It's Halloween which has always been so much fun for a birthday. I cherish my birthdays dearly. But this monumental birthday for me has been by far the most cherished, and mostly because I never thought I would see this day come. Do me a favor and cherish your birthdays too, don't complain about getting another year older, rejoice in the fact that God has given you another year to live on this earth. We always dress up and go out to a costume contest. This year I decided to be a Sugar Skull which is from the Mexican holiday Day of the Dead. It really was very symbolic for me.

Lung Transplant in my future??

There was a time in my life when I was maybe 16 or 17 that I had decided I would never get a lung transplant. I had read stories of them in patients with CF and at that time it seemed like a lot of pain, suffering, and difficulty for a chance to maybe live a few years longer. It didn't seem worth it to me at the time.

Asking myself this question 13 years later and the answer is yes I will get one if my health comes to that point, I am able to be listed and most of all actually get a donor. A lot has changed over the course of those 13 years, not only with me, but in the medical world as well. What you might ask made me change my mind? And no it is not a maturity thing, as a lot of CF'ers I was very mature for my age at that time. I thought way more about my future then most, and didn't think I even had 10 years to live. First and for most though I am a Mom. Being a Mom brings your mind to a whole new setting. That setting for me is do everything in my power to stay alive to be here for my son. I am also a wife and I have the most amazing, and loving husband and cannot give up a chance to spend more of my life with him. I am a Daughter, Sister, Grand Daughter, niece and cousin. It would be selfish of me to not take a chance to live, because I know how much they all love me and I love them. Medical advances are also a huge difference in change of mind. I have spoke with several CF lung transplant recipients and all have promising lives ahead of them because of the chance to live with new lungs. And I would be lying if I told you the idea of breathing with lungs of full capacity or near it was not exciting, or the possibility of running, biking, or hiking without getting short of breath. Heck I'd settle for not getting short of breath folding laundry!

You have to understand a little bit about lung transplant in CF. It is not a cure to CF, it's trading one demanding part of my disease off for another demanding regime, although it's worth the trade when it's successful. You have to be sick enough to need new lungs, but healthy enough to receive them. Which is a fine line to walk. I am "fairly" healthy right now, but that could change at anytime, one infection could tip me into transplant range. They most generally start to look at you for lung transplant when your lung function is in the low 30's and below, there are other factors they look at as well, but that is one of the main ones. Mine have been bouncing around in the 30's for about a year now. When you get to the point of being sick enough it's almost a "what have I got to lose" scenario. You run the risk of going into a transplant and not surviving, but you were facing death soon anyways. It's very hard to explain for me, especially because I'm just learning about it more in depth.

If I could stay where I am at now and live a long life I would be happy to do that, and I will continue to fight to stay and always improve upon where I'm at. But the ugly truth about this disease is I most likely won't stay in this state for a long life. That is why my CF doctor, Dr. Fitch (my hero) wanted me to have a consultation with a transplant clinic, so today I did. My CF clinic is in Grand Rapids MI at Spectrum Butterworth Hospital, they started doing heart transplants about 2 years ago and have been in the process of setting up a lung transplant program this year. They have all the doctors on staff, and are just waiting for their UNOS certificate to start doing lung transplants which they expect to receive in December or January. I had a consultation appointment today with Dr. Girgis to go over my history, and just explain the whole process. It is basically a meet and greet, I will see him every 3 months now along with Dr. Fitch my CF doctor, to establish a relationship and follow me. If I get to the point where they feel I need to be listed I will then start that process, which includes a lot of medical, physical, and psychological testing. They then gather all that info together and decide whether I would be a good candidate to be listed or not.

So is lung transplant in my future maybe. But only God knows his plans for me. But in the mean time there are over 70,000 on the active waiting list for organs, some of which are my friends, so become a donor please. https://services.sos.state.mi.us/OrganDonor/Registry.aspx this is the link for Michigan, if you live in another state just google how to become one in your state. It's easy and takes less then 5 minutes.

DIY Thirty One bag liner (my first DIY blog!)

So I like most every other woman in America am obsessed with Pinterest! I find myself re-pinning everything, wanting to learn to sew, bake, use power tools, and just be creative! I occasionally find something I think I can pull off. Well I found this one a while ago and it only lead to a picture no blog or instructions to go with only a short explanation in the caption. But it was pretty self explanatory anyways.

This is the picture from the pin If you own a Thirty One Utility tote you know it doesn't stand on its own, it slouches down. In the original pin they had used a summer vinyl table cloth so it can be wiped off if needed. I couldn't find one I liked and figured a shower curtain would serve the same purpose, so today when I found a colorful zebra print shower curtain(my favorite) on clearance at Meijer I decided to go for it. I first went to my Thirty One catalog to get the dimensions of the bag, I could have measured, but they already do and I'm not that accurate at measuring. I then went to the garage and used some card board box's from my car parts that luckily had not been recycled yet. I cut 3 pieces the size of the front back and bottom of the bag 11.75"H X 21.5"W which is the same size, and 2 pieces the size of the sides 11.75"H X 10"D. I brought the pieces in to do a fitting before I covered, I did end up having to trim them down even more to fit nicely.

Because the shower curtain I bought was kinda see through I had to paint the card board for a background color, I originally thought black spray paint would work good, but it didn't work very good and didn't set my pattern off very good either. So after looking through our paint collection I decided to use a lime green we had used in Colton's room a few years ago. Now had I used a table cloth or solid shower curtain I could have avoided this step, but it was on sale and I liked it.

So I painted all 5 pieces and let them dry, I could have done 2 coats but one was enough with the print on top to cover any imperfections. I then measured enough of my curtain to cover each piece and cut it up. Then I just used spray adhesive to glue the shower curtain to my card board pieces. I'm not that great at getting things to fold and cover tightly, but the curtain was kind of stretchy so it worked pretty good.

I love the way it turned out and the colors and patterns together! No more slouchy Large Utility Tote for me.

But I don't want too.....(insert 5 year old whiny voice here)

The last time I was in the hospital was in November, it was pretty routine I had a sinus infection and didn't want it to get out of hand before the holidays. (I realize referring to spending weeks in the hospital as "routine" is kind of ridiculous, but that's my world) So anyways at that point in time it had been 4 months since I had been in, which was not bad considering my spring consisted of 2 stays within 2 months. Well I have now gone 8 months without being hospitalized, which is great, it's been a long time since I've gone that long between a stay. You get where this is going don't you?! Yup I think it's time to head in for some drugs. All the tale tell signs are there, having more day's of not feeling well,laundry not getting folded or taken care of, I moan at night in my sleep when I can't breathe good, Brad says that I do this almost always when I need to go in. It's hard to explain sometimes. Do I feel terrible? No. Do I feel great? No. Do I always let myself slowly feel worse until I realize I need it? Yes. This happens because with the way I feel it kind of happens slow so I adjust to it without realizing it's even happening. Then when I go in get meds and the works, I realize how bad I felt, because when I'm done I feel SO much better. Having a tight chest the last few nights, and having to put on my O2 the other night before going to bed (I only wear it when I sleep, unless I need it which is usually only when I'm at this point) just sitting is kinda the wake up call for me. I said to Brad tonight "I need to go in the hospital" his response "Your just now saying it out loud?" Yeah he knew, he knew I knew, but like the good hubby he is lets me do it on my own time. I don't hate going in the hospital, I know that sounds ridiculous. I hate it more now that it affects Colton and Brad, but growing up being there so much I am kinda use to it. But what I do HATE is going in during the summer! That's more time Colton is home, when I am his primary care giver it makes it hard to be away, not to mention I miss him (although I will admit the first couple days away are not to bad! Oh my gosh I'm a horrible mother, I'm suppose to miss my child every minute I'm away! Well sue me then because that's how I feel! lol). Colton had a hard time last year when I was in a lot, so I'm always worried about him. I talked to him tonight explaining I hadn't been feeling good (he can always tell too though) trying to let him down easy, and he says "well maybe you need to go in then" I said "yeah I probably will" he then said "well better for you to be gone for a little bit then forever". This kid is wise beyond his years, and continues to melt my heart and bring me to tears. Now I really do feel bad for enjoying my first few day's "away"! It's sometimes hard for me having to rely on my in laws and my parents to take care of him while Brad's at work. But he's always in good hands, by the way, I have amazing in laws! I'm looking at my calendar thinking of all the stuff I'm gonna miss, pouting like a baby, my favorite band Shinedown concert, just got my car back from restoration and had some car shows in mind to take it too. Brad being that great hubby again, says "Those things don't matter!" and he's right in the grand scheme of my health they don't matter. But I still hate missing things! I am the farthest thing away from a home body, I am social and love to keep busy, "running' the roads" as we call it. But we live in a very rural area with lots of small towns 5, 10, and 15 miles apart, in which during the summer there is a festival of some sort going on almost every weekend with lots of fun, free entertainment. So I miss out a couple weekends, but I don't want too!!!! (insert that whiny voice again)

All things Disney! Warning this is long :)

As I had mentioned before we went on vacation to Disney in March. It was amazing, we stayed 5 days 6 nights, went to all 4 Disney parks, Animal Kingdom twice, and Downtown Disney. It was thoroughly exhausting to say the least but worth every second! I did really good health wise, didn't get to tired during the day and felt great the whole time. Wish I had known the whole time that had I taken my letter from my CF doctor, I could have gotten better access! We found out the last day we were there at Animal Kingdom, which allowed us to use the Fast-pass/Handicap lines, that are most generally shorter. Had I known for the rest of the parks we probably could have seen a lot more, spending less time in lines waiting. Either way we still had a blast and seen a lot of stuff. There is so much to see and do it can be a little overwhelming. I hope to go again before Colton really grows up :)

It's really hard to choose a favorite park, ride or attraction. I loved the Magic Kingdom because of it's Magical feel. You really get the Disney movie feel there. With all the characters, and Princesses and Prince's, and parade.

Epcot had my favorite rides, Test Track, and Soarin'! With a lot of other kinda scientific stuff and evolution of culture's. It has a lake in the middle of it and all around the lake is restaurants, stores, and places representing different country's, we did not get to walk all the way around, but what we did see was pretty neat.

Brad is not into amusement parks or rides and he went on almost all of them with us, and his favorite was Soarin' too. Hollywood Studio's is probably my second favorite. It's just very cool, you feel like your on a movie set the whole time. And Tower of Terror was another favorite ride of mine.

I rode it with Brad's friend's daughter and niece that happen to be on vacation the same time we were. Brad. his friend, friend's wife, and Colton didn't want to ride it. Funny little story. We had to wait in line for quiet a while and it got to be longer because one of the "elevator's" on the ride was not working right. Well we finally got our turn, screamed our heads off had a blast on it. When we got off Colton comes running up to me like a concerned parent "What took you so long? I was worried about you! We have been waiting forever!" Hugging me the whole time, just gives you another idea of Colton's "grown up" personality. The next favorite part of Hollywood Studio's was the Stunt show. It was so cool, they showed you stunt cars, and how stunts are done in the movies. They burn a lot of rubber, and Colton says "I love the smell of burnt rubber in the morning!" LOL It was really the afternoon! The real Lightning McQueen and Mater were there too!

There are lot's of 3D and 4D shows in all the parks too, those are fun too

We went to Animal Kingdom our last two day's. Finally got Colton to ride Expedition Everest with me, it was a blast, and he did get a little scared but was good by the end of it.

The Bug's life that is in the center of the Trunk of the Tree of Life was really neat, it was a 4D show, you get fogged, sprayed, and Bugs running under your butt!