Archive for the ‘News’ Category

Alright not to brag… Ahh what the hell I’m gonna brag!! It’s one of my dreams come true! You will NEVER guess who emailed little ol me! I always tease Derek he’s my boyfriend… That’s right!! Bob Beall (President and CEO of the Cystic Fibrosis Foundation)! Or as I so fondly refer to him as Bobby! Hehe! I love this man so much because of the work he has done at the foundation and I know the decisions he has made are the reason we are so close to a cure. He’s my hero!

Every time I see someone from our CFF chapter I mention how I love Bobby and if I EVER could be somewhere he is speaking I would just die! When I spoke at our walk last month I said if I ever met Bob I just might kiss that man. Ha! Well I guess it paid off because one of the angels that works for our chapter sent a picture of Ging-a-roo to Bob and he took the time to write me! Here is the email I will cherish forever and I will put in Ginger’s baby book and frame in our house! **On cloud nine!**

Dear Sarah

Wow—is she a beauty—my wonderful friend Helen just sent
me a picture of Virginia Blue—wow what a cutie.  Helen also told me of
your terrific support of the Great Strides effort at the Redlands Great
Strides—what a team.
My pledge to you, is that we will use those dollars
well—-and you can look into those beautiful eyes of Virginia Blue—and
assure her that she is going to have the life that we all want for our children
and our children’s children.  That is my pledge to you and
Virginia—

Lots of exciting things happen and it is people like you,
your husband, and Virginia that invigorate and energize us every day to push
the frontiers of science and medicine

Thank you

BOB

Ahhhh!!!! Squeeeeelll!!!!

My dear sweet friends. I have fallen in love with this brave family (the Jones’) They have a little boy with CF like Ginger. He just turned 7. Today he is being admitted to the hospital again and he is not expected to make it much longer. There is nothing more they can do for him. His family is struggling on every level. They’re heartbreak is inconceivable. They are trying to figure out where they will come up with the money to plan a funeral for their baby. I can’t write these words without sobbing… I got together with Conner’s mommy, her name is also Sarah and we set up a place where donations can be made to help them pay for the funeral. We are half way to our goal. If you can help a tiny bit or spread the word it would mean the world to me. These are my friends and they are living my biggest fear. Please help lift one burden off them. Here is his mommy’s blog (http://www.notsobrightandshiny.blogspot.com/ )

If you would like to help them you can at

http://connerjones.chipin.com/the-conner-jones-fund

“I tell you the truth, whatever you did for one of the least of
these brothers of mine, you did for me.’” Matthew 25:40

I’ve been slacking. So there is so much to catch up on! First off Ginger Blue turned 1 on the 3rd. What a special day! The year went by so fast.

I feel like I was just pregnant and now my baby is walking all over the house. It’s sad but at the same time I feel so blessed it’s hard to describe. I feel blessed that I’m finally a mommy. This journey has brought more heartbreak and tears than I could ever speak of. The miscarriages and the fear we would never have a family was overwhelming. Then our dream came true and we had the most beautiful, precious daughter and to find out she is so very sick. I hear so often, she looks great! Yes she does look perfectly healthy, but it stabs me in the heart to hear these types of comments. It’s just a reminder of what we fight all day long. I think it makes people feel better when they see nothing wrong. IT’S ON THE INSIDE! I want to yell, but I don’t I smile and say thank you. This has been on my mind after another CF mom brought up how it bothers her. I thought I was the only one. I’m not and I learn more and more I’m not alone. Derek and I have endured many heartbreaks in the past 4 years, BUT oh we have had joys no one could describe. Any one that has met Ginger knows she brings a smile to your heart like nothing else on earth. She has completely healed every hurt in me. I thought this was not possible. I use to ache so deep…

I feel blessed beyond words. I know my life is not ideal. I mean I know most don’t want to be in my shoes, but I wouldn’t give anyone my shoes anyway! I ‘m blessed in ways others wont and can’t understand, because they have not gone through what I have. Everyday gets a little brighter and I realize it more because I have my heart open.  God is with us every single day and I feel his presence more now than any other time in my life. I don’t think I would be who I am now without all these experiences. I have more trust in God and the people that love me than I did 5 years ago. I’m proud of who I grew up to be.  I love deep, I give my all, I will protect those I love, and I don’t take health and family for granted. I wouldn’t be who I’m without the hardships. Now why are you wondering I’m I feeling grateful for the hardships even? Ha! I know right! Well it’s because… GOD IS SO GOOD! He is fixing things. I know you’ve read that I have felt for some time that God has been giving me signs that a cure for Ginger and all with CF is coming. Yesterday was another great day. We had clinic yesterday, which always puts me in a tailspin. I get all anxious and depressed for about a week before we go. I always decide they are going to tell us she has to be hospitalized again. (the thought is too much to take) Yesterday was GREAT! Her weight is back up! Her lungs clear! Oxygen 99 to 100% (PERFECT!) Every single thing I fear was perfect!

I asked our head doctor that runs our CF clinic if she had seen the new video the CFF released on Tuesday. (this is it)

I told her this was the first time I heard them say more about the new drugs than the results sound promising… He says it looks like this could control and correct the disease! I asked if I’m just hearing what I want to hear or if that would be a cure? SHE SAID YES IT WOULD!!! I almost fell out of my chair! She said, I’m telling you we will be out of business soon. CF will be a thing of the past like Syphilis. God is GOOD I tell you. CF is the last thing that tortures my soul and it will soon be a thing of the past! My daughter will LIVE! Hallelujah. We are still years away from the FDA approval so us keeping her healthy is vital.  We will earn every dollar we can to keep these drugs moving through the pipeline as quickly as possible to save lives. Our happy ending is on the way!

Not long ago we endured two heart wrenching miscarriages. I never understood what kind of pain a miscarriage could bring. I cried many tears over many months. I remember working at a coffee shop where a customer told me, “Your eyes always look watery.” I smiled and said “really?” I knew I was constantly holding back tears but didn’t know you could see it in my eyes. This went on for years. Derek was strong and supportive, but I could not heal my heart. My jaw would lock half closed from the day we lost our first baby until the day Ginger was born. I know this sounds weird but it’s true. I did physical therapy and nothing seemed to help, until my heart was healed. Our bodies hold on to stress and sadness in such strange ways. When we got pregnant the third time, I would see the doctor who at every appointment would ask, “How many pregnancies?” “3,” I would answer. “And how many births?” he would ask. My heart would drop, “zero…” Well during this very scary time God would give me signs that this time was going to be different. I knew I was having a girl, I knew her name was Virginia, and I knew God had his hand in all that was happening. On March 3, 2009 my biggest fear of losing my life long dream of being a mommy went out the window when I heard that little, but very strong cry. I cried with her. I finally was a mommy.

3 weeks later when we got the news she had CF I felt deceived like I again had to worry about losing my hearts desire, but this time it would be even more painful than I ever experienced before. I struggle with these thoughts, but God continues to give me signs the way he did when I was pregnant. I think it really soaked in yesterday that these signs that promised me a baby are now promising me she will be ok. Our cure, or a treatment that would make this disease controllable like asthma is so close. I feel like us getting to sit with the couple that spoke at CF parents night was one of Godsends. The president of the CFF told them he  is in his 70’s and has a bad heart but he believes there will be cure before he dies. This next example sounds silly but I am part of a website called Cysticlife.org It is a facebook type of site that is for people affected by CF. You can ask questions and things like that. You can go on the site and narrow the search to just mom’s in your age range and state to talk to. I was trying to see if I could narrow the search to people that have the same gene combination as Ginger. I wanted to see how they were doing. Anyways I was going to give up because there was no way to do it and most people didn’t even list their gene mutations. I thought in my head I’ll just click on one profile (out of over 900) and if it’s the same mutation then there will be a cure. I don’t know why I thought this because I don’t think this is the was things work or how God talks to us, BUT I clicked on one and there it was. Her EXACT mutation. I was shocked! I mean floored. Her mutation is not a popular one and there are thousands of different mutations you can carry. I felt like God WAS telling me something. Then yesterday I wrote “Today would be a great day for a miracle” within a matter of minutes 3 different articles came out on the Vertex drugs, and how one just finished phase 2 study. These drugs could be as close to a cure as we could hope for. It would be a once a day pill you take and no longer need the breathing treatments, drugs and vest. The only other thing you would have to take is enzymes because the scaring of the pancreas is not reversible. After the three articles came out another CF mom I’m friends with on facebook wrote that she went and listened to the president of the CFF speak. She wrote this: “I went to hear a lecture on this [Vertex drugs] last wk at Albright College by the president of the CF Foundation. This is the most promising news, I’ve heard in the last 24 yrs.  Let’s hope all goes well with this & that we will ultimately have a cure for this horrible disease, in the near future. I was told by him, hopefully 2011-2012!!!! Prayers, lots of them!!!!!”

I can’t explain it but I KNOW in my heart we are going to have a cure for this disease soon. I even talked to my mom about when it happens maybe we will be able to have one more baby, a sibling for Ginger. This is a thought I hardly ever talk about or let myself think about because I thought we would never be able to do and it makes me  very sad.

I have been on cloud nine since yesterday. I truly KNOW we are going to cure this disease. Our money is more important than ever right now. We can make it happen and more quickly if we have the funds to do it. I’m meeting with someone from the foundation today to talk to her about the paper roses you sell for a dollar at stores. I’m going to get them from her and go to all our local stores and coffee shops and see if I can get them to sell them.

I want to thank you all that love and support us in this fight. We will win! I love you all!

My two favorite angels.

Today is Ginger Blue’s 11 month Birthday! I have more to write on that and this week, but I ‘ll have to do that tonight so check back. Right now I’ll leave you with this article. So wonderful it made me cry. Things are looking up!! God is good!

UPDATE 1-Vertex cystic fibrosis drug shows promise in trial

* Drug was well-tolerated in clinical trial

BOSTON, Feb 3 (Reuters) – Vertex Pharmaceuticals Inc (VRTX.O) said on Wednesday that a mid-stage trial of its experimental cystic fibrosis drug showed promise, and the company will study it further.

Cambridge, Massachusetts-based Vertex said preliminary analysis of data from a 28-day trial of its drug, known as VX-809, in patients with a certain genetic mutation, showed patients experienced a statistically significant decline in sweat chloride.

Sweat chloride becomes elevated in patients with cystic fibrosis and is caused by a defective protein in certain cells in the sweat duct.

Most patients with cystic fibrosis have a genetic mutation known as F508del which causes a protein, known as Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), to be missing from the cell surface.

VX-809 is designed to direct the protein to its proper location.

The company is also developing a drug known as VX-770 which is designed to treat patients who have the protein but find it does not work properly.

Vertex said it plans to begin a combination trial of VX-809 and VX-770 in the second half of 2010 for patients with the F508del mutation. The idea is that VX-809 would direct the CFTR protein to the cell surface, while VX-770 would optimize its effectiveness.

Cystic fibrosis is a life-threatening genetic disease affecting about 30,000 people in the United States and 70,000 worldwide. Defects in the CFTR gene result in poor chloride flow across cell membranes, causing the body to produce abnormally thick, sticky mucus that leads to chronic, life-threatening lung infections.

I LOVE THIS! It is such good news and brought tears to the whole families eyes!

Her potassium is good! Actually everything they tested for was good. When I asked about the hemoglobin they told me they didn’t test that. When I told them I thought it might have been low from all the blood draws in the hospital the nurse on the phone said that it was tested at the beginning of her stay and that it was low then, but then she looked into it and it was only a little low at the beginning of the stay and it got lower throughout the stay. They still want to put her on an iron supplement. I talked to her pediatrician today when she got her shots and she said she would recommend I give her half the iron supplement and make sure I take my prenatal vitamins, because the iron she gets from me is more easily absorbed then a supplement.

Oh and then last night I see there is a recall on infant Tylenol and I look up the lot numbers and what do you know! Ours is on there. I think I should start playing the Lotto. The odds of all the stuff that has happened to us in the past year has to be crazy impossible. The last time I gave the Tylenol to Virginia was with her 4 month shots and I was going to give it to her today for her 6 month shots but luckily it was on the CF site last night. The doctor said since we gave it to her 2 months ago she is not too worried and I guess they have not found any live burkholderia cepacia. I called the CF clinic and I didn’t hear back from them before I had to leave to go to the pediatrician. I’ll call back on Monday, but I don’t have a bad feeling about it since we did the bronchoscopy and nothing grew at all from the sample in her lungs , and that was only a couple weeks ago.

Ok so poor punky Sue had to get four shots and she really cried. It was so heartbreaking. I thought I would do better at just shots after seeing her in the hospital for so many days, but nope. My poor little angel. She was so upset she did that cry where no noise comes out for a long time. Her mouth wide open and her face bright red. Oh… She seems to be feeling fine now though. She’s not acting achy or anything. Hopefully she is feeling good tomorrow.

So we follow the CF news EXTREMELY closely. I check cff.org about 3 times a day. I just keep praying for a miracle to happen. When I say miracle I mean a flashing headline in all bold letters that says WE FOUND A CURE!! Well, as of 8:34 August 5, 2009 that has not happened… But a step in a very encouraging direction are two drugs they are working on, the VX-770 and the VX-809. This could be a once a day pill that could improve several key indicators of cystic fibrosis, including lung function, nasal potential difference measures and sweat chloride levels! I keep trying not to get too excited about this because in the early 1990’s they thought they were just around the corner from a cure with gene therapy. Obviously 20 years later we don’t have a cure and so I try to be realistic. Also the trials being done are on CF patients with the G551D mutation, which Virginia Blue does not have. They just started a trial on the VX-809 that includes people with the double delta mutation. Virginia has a single delta… Anyhow this is a big day because they just entered phase 3 of the trial for the VX-770 today! It sounds too good to be true and I can’t wait to see what happens with this.

Here is the article…

VX-770 Trial Begins in Children as Part of Phase 3 Program

August 5, 2009

Today, the CF Foundation announced the initiation of a clinical trial in children as part of the Phase 3 registration program for VX-770. VX-770 is an investigational oral therapy aimed at treating the underlying cause of cystic fibrosis. The drug is being developed by Vertex Pharmaceuticals with support from the CF Foundation.

This is the second of three clinical trials underway in the Phase 3 registration program for VX-770. The registration program is designed to generate data that the U.S. Food and Drug Administration can use to determine if VX-770 is safe, effective and acceptable for approval.

“This potential drug is one of the most promising therapies in our pipeline that aims to treat the underlying cause of cystic fibrosis,” said Robert J. Beall, Ph.D., president and chief executive officer of the CF Foundation.

“We’re excited that enrollment is open for this second trial. The initiation of this study is an advancement in our efforts to bring a new therapeutic option to CF patients.”

The trial is a two-part study of VX-770 in patients with CF age six to 11 years who have the G551D mutation of CF. It will enroll approximately 30 children with CF.

The first trial, which was initiated in May, is examining VX-770 in people with CF age 12 and older who have the G551D mutation of CF.

Faithfully praying for a cure