Wednesday, March 30, 2011

The return of Pseudomonas - boo!

We just got a call from the CF clinic that unfortunately Drew's culture from Monday grew Pseudomonas again. UGH! Just when we were hopeful and optimistic that we were done with Tobi for the time being, it rears its ugly head once again. We'll go on Tobi for 28 days and also take Cipro orally. We're still going for eradication vs. just trying to keep it at bay. The doctors aren't convinced that he's colonized with pseudomonas, but unless the drugs get every last little bit of it, it will come back and continue to grow. I guess we should just be thankful that they found it and we can treat it, vs having not found it and let it grow in his lungs for some period of time.

Here's a link I found that helps to explain the bacterial infections in CF patients, including pseudomonas - If you read the page, you can click on the tabs on the top of the picture to see CF infections described visually.

If at the end of this round of antibiotics he is still growing pseudomonas, we will likely get put on IV antibiotics. I expressed my desire to do that from home vs. being put in the hospital. Its every 8 hours for 2 week and would need a PICC line put in (sort of like a port), but at least we could manage it from home. The purpose of this would again be to try to eradicate. You can compare pseudomonas to something like smoking, as far as what it does to your lungs and how quickly. Its a nasty and resistant bacteria that over time causes decreased lung function and ultimately lung disease, so we need it gone today. You smoke a cigarette today, not much is going to happen to you. But smoke one tomorrow too, and the next day, and so on and so forth and your lungs can take off their sunglasses because their future ain't so bright :)

Please hope and pray that we can get rid of it with the Tobi & Cipro. And just as a little FYI, the CF Foundation, who you so kindly donate to by supporting team Doin' It For Drew, is responsible for all of the research and science that went into creating Tobi, the first new oral antibiotic created to treat pseudomonas in over a decade. Just sayin' :)

Other bloggers

There are a few blogs that I read very regularly. Some of them are CF related; others are not. I've talked about some of them on here before - Enjoying the Small Things, The Breathing Room, MckMama - But over the past few days, I've read some things on a few peoples blogs that brought up a lot of thoughts and feelings. First, a few on CF.

There is a woman, whom I have never met, who lives in Florida and has an almost 4yr old daughter who has CF. The other day, she posted on her blog about decision making that involves CF. I swear it felt like she wrote the exact words that I often think. Everyone involved with CF can understand how all encompassing this disease can be. And how much you just truly hate every part of it deep down, knowing that it can steal a life at any moment. And how much you struggle to keep your life as normal as possible, both for the person with CF and for the other family members, and frankly for your own personal sanity too. But its hard. Its really really hard. When Ella started pre-school this fall, I was a wreck the first time she came home with a cold and then Drew got the cold and I though I really should pull her out of school because its just to detrimental to Drew's health to have her bringing all of these germs inside. But the more I thought about it, I realized that some day she was going to go to school. Sure I could have prolonged it, but it was going to happen eventually. And the same for Drew. He will go to preschool and to 1st grade and to college and hopefully do all of the other things that any non-CFer would do. But my goodness is it terrifying every time they get a cold. That first call that he had pseudomonas - devastating. You kind of know and expect that at some point in time it will happen, but you just think and hope that it won't happen to your CF kid.

There was one paragragh that my blogger friend wrote that really hit home with me. (if you're reading, I hope you don't mind me sharing!). She wrote,
" I have such a social little girl on my hands, whom I don't want to ever have to tell " I chose ___________________ because you have C.F." I don't want her illness to define her, or have her life filled with choices and options that were tailored to C.F. I want her to do things that everyone else said she couldn't, I want her to build lasting relationships with people who support her and lift her up while she is hurting.
Is that not the truth or what! I am going to try to live by that. If I have to make a decision regarding Drew or my girls, or anything in general that may affect our family, I'm going to first ask myself if I will have to explain it in terms of CF. I refuse to let this get the best of me. I didn't invite CF into my life, and I'm certainly not giving it the power to make decisions for us!

I don't want Drew to miss out on things that Ella enjoyed when she was his age. I loved taking Ella to story time and on playdates, but this winter we rarely left the house. When I pick Ella up from school, she gets a thorough wipe-down with antibacterial wipes. Friends who I regularly had over previously haven't been inside our front door in months. We don't go to the museum, or when we do, the twins stay in the stroller. We don't go to storytime or music class. I made excuses for getting together with friends so that their healthy kids didn't bring any weird germs into my house. And still, we had a sick sick winter. Drew was on different antibiotics from October through the first part of March. Every time he got a cold, his lungs created the perfect environment for bacteria to grow because he can't cough and clear a cold like you or I can. And its terrifying.

Another blogger, who unfortunately lost her 7yr old son to CF about a year ago, wrote on her blog retrospectively about how as much as she'd gone on and on about how CF didn't define her, it really did. And to be honest with you, its hard for it not to! When you have to get up extra early to get in all of the breathing treatments and medications, and you can't go places in the evenings because of the airway clearance regimen that has to happen. And the doctors appointments, for well and for sick checks, that happen oh so frequently. I find myself always telling Ella to wait just 1 minute, either because we need to finish up Drew's breathing treamtents or because we need to get him his enzymes before he eats. We forgot his enzymes on a recent trip to the zoo, and I did everything in my power to not ruin the afternoon for all. I gave him the only foods I could find without fat in them, the only things he can eat without enzymes, while the girls ate pizza and goldfish and cheese, all foods he loves. He gets sick if he eats without his enzymes, but if I needed to get his enzymes, that meant that the fun at the zoo had to come to an end...because of CF.

I try hard not to let it define us. I want him to live a normal life, but that fear of him catching something un-treatable, or getting sick with something that would require a hospital stay and send our happy little family into even more of a tailspin, all of that stuff forces us to be extra careful. Lunch out is a freakin' workout, let me tell you. It takes me 10 minutes just to get the restaurant high chairs disinfected. Then making sure that the table and anything within his reach is also cleaned. Any food or cups that hit the floor are history. I seriously look like a crazed woman, but I have to do it. And I do it happily, and will for the rest of my life if it will keep my baby boy healthy.

The median life-expectancy for someone with CF is 37. Thats the middle. Its not the average. Half of the people in the world with CF don't make it to 37. Out of the 30,000 people in the country suffering from Cystic Fibrosis, 15,000 will die before they are 37 years old. I try not to think numbers. I try very hard to live in the moment and enjoy our life and our family. But the devil in the back of my head is constantly shoving CF down my throat in the form of a neighbor sneezing on our wagon or a stranger reaching out to touch a smiling baby. Maybe I need anti-anxiety medicine. The mother who posted on her look back at how all encompassing CF became in her life said she would have just let it all happen, let her son live his life. I don't know that I believe her 100%. I believe that she wishes she would have, or could have. But I think its natural instinct to try to protect, and I'm doing that the best way that I know how.

We won't be hermits forever. Once the germy season is over and we can get outside I will try to ease up. Once the babies get a little older and Drew doesn't put everything and anything in his mouth all of the time I might be able to ease up a little bit. I know that people don't understand, and thats okay. Its not your situation, its mine, and I'm dealing with it the best way I know how. Its unfortunate that its alienated some friends, but I've made lots of new ones. Don't stop asking me to hang out. Don't get offended if I say no 99% of the time. Please respect my family and the illness we are dealing with by not brining germs around us. I know we can't live in a bubble, and I don't think we do or want to, but we need to be cautious and careful so that we can get the best of Drew and CF doesn't get the best of us.

In another note, I want to go public with my decision to get rid of the negative in my life. I have enough negative that I have to deal with regularly, and I don't need unnecessary negativity bringing me down. I'm going to stop reading blogs that I think aren't nice or I simply don't enjoy. I can use my time for better. I'm not going to waste my time with people who I simply don't enjoy being around. I know that I am a good person and I try to do as much good for as many others as I possibly can. I'm sorry if I offend you in any way, but I'm just living my life the best way that I can for my family and for my future. If you think thats selfish, well, so be it. You're not gonna get me down!

Tuesday, March 29, 2011

Anna's Army

Here's a story that was on the news tonight of a little girl who lost her battle with CF two years ago today. I don't think anyone realizes the severity of this disease...but you can see and hear just how bad it is as this mom tells it and this precious little 3rd grader gives it her last fighting breath.
Do you have $5 today to help support our cause? Can you help us find the cure for Drew?

Monday, March 28, 2011

Anniversary Visit

We had Drew's 1yr visit at the CF Clinic today and it went really well! All of his bloodwork came back good - liver is working good, kidneys doing what they should, vitamin levels are all where they should be. It was pretty uneventful actually, which I'll take any day!

We're supposed to re-start the Tobi on Saturday. Because he looks and sounds so good, there is some hesitation to treat him. There is concern that even with a negative culture, Pseudomonas might still be growing down in his lower airways that we wouldn't catch without a bronch. Treating him with Tobi would be to be on the safe side and get the medicine in there to kill off the bacteria that may exist lower than a throat swab could detect. On the other hand, we don't necessarily want to treat him with an antibiotic that he doesn't need lest he build up a resistance to it, though I learned today that inhaled antibiotics work a little differently than oral or IV and don't usually become ineffective even if given over long periods of time. So the downside of not treating him is that we miss bacteria that might be down in his lower airways. Anyway, they did a throat culture today and we're going to wait to see how that turns out. Obviously if it shows pseudomonas then we will start again with the Tobi and Cipro combo again this Saturday (the start of our 28 day cycle) and if its negative I think we're going to wait until we get another positive culture, either orally or from a bronch. Obviously my hope is that we never get another positive culture...ever. We'll see what happens.

We're trying a new method for calculating enzyme dosage. Drew's poop has been on the rather intense side, and we need to get his enzymes in order. Not many people in the US actually calculate out enzymes per grams of fat, but its a common practice in Australia and New Zealand. If it helps with the pooping problem then I'll do it! The good news is that his weight isn't suffering so we have some space and time to get it all sorted out before it would become a problem.
I also talked with our fellow (who will become a full fledged member of the faculty at Children's in July and likely our primary CF doc) about the mutations and the class of mutations and she had the chance to talk to J.P. Clancy again (lead CF researchers on the CFTR mutation) and after some thought and research he said it was probably unlikely that the new drug coming out will be affective on the mutation Drew has. BUMMER!! The drugs don't work on "classes" of mutations, but rather specific cells, and the drug that is on its way to FDA for approval doesn't target the cellular issue that Drew's got. Research has come so far, but there is still so much more that needs to be learned and understood. The good news is that there are a ton of other drugs coming down the pike that will target the mutations that Drew has. I've got a lot of faith in Dr. Clancy and I haven't even met him yet!

Sunday, March 27, 2011


We finally have our design and supplier for our 2011 Great Strides T-Shirts!! I love them. We were all over the place trying to decide if we wanted something serious or humorous or with a lot of info on it or what, but in the end, the it turned out to be just what I was looking for. If you haven't already registered to walk with us and are planning on joining one of our walks, please go online and register or I cannot guarantee that there will be a t-shirt available for you. All walkers who are registered to walk with us and who are raising money will receive a t-shirt compliments of us. If you want to walk but do not want to raise money, you can purchase a t-shirt for $12. If you simply want to buy a t-shirt, they are available for purchase on the left-hand side of this page. Hope many of you will join us as we walk on May 21st here in Cincinnati!

Friday, March 25, 2011

Featured Corporate Partner of the Cystic Fibrosis Foundation

Just thought I'd share this nice little story about Genetech that was featured on the CFF Website. The more sponsors that we can get to do stuff like Genetech is doing, the faster we will reach our cure. If you or someone you know works for a company who might be interested in partnering with the CFF during this exciting time when we are so close to a cure, please don't hesitate to contact me or anyone at your local CFF office.

Genentech: Making CF History

Genentech has been a generous corporate partner of the Cystic Fibrosis Foundation for more than a decade, and today, its employees are more committed than ever to making CF a thing of the past.

“Participating is important to Genentech because we know that the money raised goes back to funding the research we’re so passionate about,” says Sheri Morin, senior director of sales and marketing. “It only makes sense for us to be involved!”

Genentech is both a national sponsor of Great Strides and a supporter of local walks in Northern California, where the biotech company is headquartered. During Great Strides season, Genentech encourages its 11,000 employees to join a team or make a donation.

Caring for the CF Community

Team Genentech at the Great Strides walk in San Francisco, California.
Team Genentech at the Great Strides walk in San Francisco, California.
In addition to their passionate support of Great Strides, Genentech employees contribute to a variety of regional and local events year-round to raise funds for CF research and medical programs.

Genentech is also committed to ensuring all people living with CF and their families have access to the information and treatment support they need to stay as healthy as possible.

Many of the educational resources the CF Foundation provides to the community are made possible with significant support from Genentech.

For example, in 2010, with an unrestricted educational grant from Genentech, the Foundation established a CF education webcast series to bring the cystic fibrosis community together in a virtual forum to learn from CF experts nationwide.

In addition, the company supports research that aims to identify new ways that doctors can help patients stick to their prescribed treatment regimens.

In 2009, Genetech contributed a total of nearly $700,000 to the support the search for a cure.

“Genentech is a company that has long demonstrated it cares about the CF community,” says C. Richard Mattingly, executive vice president and chief operating officer of the CF Foundation. “We are so grateful for everything they do to support better health and a brighter future for people with cystic fibrosis.”

Wednesday, March 23, 2011

I've been invited!!

UPDATE!!! I got a call from the CFF office yesterday, saying that they had been invited to attend the symposium and were asked to bring a few parents & caregivers with them to listen in for the day...and they wanted to know if I was interested in attending!! So I've officially been invited, and I can't wait to go!

Okay, well technically not really. But I can go if I want! To what you ask? To the Thomas Boat Research Symposium on Cystic Fibrosis. I have a dear friend who works at Children's and she forwarded a note to me that she received about this event. Here's what the body of the email said:
Please join the Division of Pulmonary Medicine for a day of scientific discovery and celebration. The symposium will feature scientific breakthroughs in the field of Cystic Fibrosis led by researchers throughout the country and at Cincinnati Children’s. We will also celebrate the establishment of the Thomas Boat Chair in Pulmonary Medicine and the appointment of Dr. John Clancy to the chair.
Now let me explain what that all means. These doctors - Thomas Boat and John Clancy - are some big time Cystic Fibrosis doctors and researchers. Dr. Boat, though I've never met him, has a reputation as a pioneer in the care for and treatment of cystic fibrosis patients. Dr. Clancy has been called the lead Cystic Fibrosis researcher in the country. AND THEY ARE BOTH IN CINCINNATI! So I get this email from my friend and I think about how awesome it would be to meet them and hear them speak. And I open the email attachment and I see that its more than just those two amazing doctors that are going to be there, but rather the best of the best from around the country!! All of these doctors and researchers, all in one place, and I have access?! Get out of town! Here's a peek at the agenda:
10:00 am Welcome and Introduction
Raouf S. Amin, MD
Director, Division of Pulmonary Medicine
10:15 am Cystic Fibrosis Science at Cincinnati Children’s
Arnold Strauss, MD
Director, Cincinnati Children’s Research Foundation

10:25 am Cystic Fibrosis Progress
Thomas Boat, MD
Executive Associate Dean, University of Cincinnati College of Medicine
CEO, University of Cincinnati Physicians

10:55 am The Network Development of Cystic Fibrosis Basic and
Clinic Science: The Role of the Foundation
Robert J. Beall, PhD
President and CEO, Cystic Fibrosis Foundation

11:35 am Breakthrough in Animal Models of Cystic Fibrosis Disease
Michael Welsh, MD
Director, University of Iowa Cystic Fibrosis Research Center

1:20 pm New Paradigms of Inflammation in Cystic Fibrosis
Christopher Karp, MD, PhD
Director, Cincinnati Children’s Cystic Fibrosis Foundation

2:00 pm Bringing Quality Improvement to Cystic Fibrosis Clinical
Care and Applying to Clinical Research
Gerald T. O’Connor, PhD, ScD
Professor, Medicine and Health Policy and Clinical Practice at
Dartmouth University

2:50 pm The Realities of Cystic Fibrosis Disease, Current and Future Targets
Michael Konstan, MD
Professor and Chairman of Pediatrics, Case Western Reserve University
School of Medicine, Rainbow Babies and Children’s Hospital

3:30 pm Cystic Fibrosis Modulators – Are They Enough?
John Paul Clancy, MD
Director, Clinical and Translational Research, Cincinnati Children’s
I emailed one of our doctors, Lisa Burns. She's a fellow and she is awesome. After I saw the agenda I thought to myself, "I wonder if this is for parents" as I hadn't received an invite, I had been forwarded an invite...from a clinician at Children's. Lisa emailed me back shortly thereafter and said that she would check and see. Dr. Amin (our August 2010 hero of the month for the discovery of the aorta compressing the trachea which lead to Drew's heart surgery that helped him so very much) responded that patients and families were not invited to the symposium because it was directed more towards clinicians and scientists, but he also said that I welcome to come if I wanted. YES! I want to! There is my official invitation!

Martin thinks its weird. I guess it might be a little weird if I'm the only non-doctor/nurse/dietician/specialist there, but to be perfectly honest with you, I am a specialist on the subject as I deal with Cystic Fibrosis 24 hours a day, 7 days a week. These doctors get to go home at the end of their patient-checking, researching days. I have to sit at dinner and administer enzymes before Drew eats. After dinner, while the girls get to go play with my husband, I have to sit with Drew to give him his Pulmozyme and Pulmicort and Tobi while his Vest shakes the crap out him...all while watching the same episode of Sesame Street that I've seen 3 times a day for the last 6 months...not that i'm bitter. I have to spend my "free time" in the afternoons charting what he's eaten so I can consult the dietician on the frequency and texture of his poop. I swear, I don't mind doing it, I'm just ready for the cure to be found. I'm mad that CF has this kind of control over me. I'm an expert and I should have gotten invited to this symposium.

I understand why I wasn't though. Not all CF Parents are like me. Many are. Many many are. But in the past, when conferences have been organized and held to education and inform CF families, attendance has been low. And I understand why. Its hard to find the extra time when your child needs so much care and attention...and you have life going on in the background. Its the same thing with fundraising for Great Strides or the other CF Foundation events. Its hard to find that extra time and that extra money when medical bills mount and your time is spent between enjoying games of catch and building block towers in the living room to fielding calls from insurance and the pharmacy telling you that its not yet time to re-order the drug that you just ran out of.

But I'll be damned if its gonna get me down. I'll find the time. I'll make the time. I want to be in the know. Knowledge is power. CF is not our life. Its a very large part of our life and I do my best to keep it running the background. For me, thats the blessing and the curse of this disease. Everyone sees a healthy happy kid. But you're not here for the phone calls and the treatments and the doctors visits. And I'm happy about that because you all treat him the way I want you to treat him - just like anyone else. But I also want everyone to be able to see what really goes on, how really horrible this disease is. Not for my sake, but for Drew's. For all of those kids out there suffering from it. I want to go and listen to these doctors talk and ask my questions from an experts perspective. Sure, they might know more about the science and technology, but I will always know more about my son.

I'll ask Lisa when I see her in person next week if she thinks its weird that I want to go. If she says yes, I will wait for the next opportunity. Martin thinks its weird. I don't so much care if people think i'm weird. I don't want to overstep boundaries, but I'm curious. And I feel like if they really didn't want me there, they would have stopped with "parents aren't invited". We'll see what ends up happening.

I also learned today that the cousin of a friend of a friend (i know its a stretch) works for Gilead, the company responsible for Cayston! They are a sponsor of the Great Strides walk in Philadelphia. We haven't been on Cayston yet, and God-willing won't have to be, but it never hurts to have a connection in the business!

Monday, March 21, 2011

Please understand where I'm coming from

I've been struggling lately with my feelings on my fundraising efforts. I want to raise more. I want to do more. I owe Drew all that I've got. But I don't want to get on your nerves; that's not what its all about. Here's where I'm coming from...
The science for a cure is there. There are people bright enough and smart enough that have figured out exactly what Cystic Fibrosis is and does and what exactly needs to happen to cure it, or at least stop it in its tracks. So many conditions and diseases receive support from our government. Cystic Fibrosis does not. Its my job, as his mom, to raise that money. Here's the problem - when people look at Drew, they see a happy & healthy little boy. He looks healthy on the outside, but he is fighting a fatal and progressive disease that affects his lungs and digestive system. He is not healthy. Everyday we spend hours giving him breathing treatments so that he can breathe like you and me. Everyday we spend hours clearing his airways by hooking him up to a machine that compresses and vibrates against his chest to shake loose the mucus. Drew cannot have a sippy cup of milk without first taking a special medication that helps with his digestion. Every time he eats or drinks anything, he must first have his enzymes. He is on 6 different medications that he takes multiple times a day. We see his pulmonary doctors every month, sometimes more. He's been in the operating room more times than I can count, with 4 major surgeries under his belt and at least half a dozen bronchoscopies.
How can I not want something different for my son? How can I not ask you to donate to our cause? How can I not invite you to participate in every event that comes down that pike benefiting the Cystic Fibrosis Foundation? I know that not everyone will be able to participate in everything that I post on Facebook or email you about. I do understand that people have other causes that they support. But that doesn't mean that I won't ask. I have to ask. You have to understand. I don't want my friends to think I'm being pushy. I promise you that I do understand if you are unable to help us. All I ask is for your consideration. I'm in a race against time, against Cystic Fibrosis, to save my son's life. If it were your son I'm sure that you'd do the same.
Help us if you can.
  • Spread the word!! Share Drew's video. Here's the link:
  • Donate to our Great Strides team by clicking HERE. If you can only spare $5, we will appreciate your $5. If you can spare $500, we will appreciate that too.
  • WALK WITH US!! Click on that link above that says HERE and join our team
  • Hold a fundraiser for us! I know lots of creative people. Surely some of you can come up with some creative way to raise some money to help us.
  • Sign up to walk with us in a city near you. Here is the link to a list of cities that Team Doin' It For Drew is walking in, as well as a link to find a walk in a city near you:
  • Ask your company if they are willing to support Cystic Fibrosis by offering matching gifts on donations, or by making a corporate donation. You never know how many people you can reach until you ask. The worst they can say is no.
  • Become an Advocate for CF. It takes but a moment of your time to send a pre-drafted letter to your congressman/woman, petitioning them to get federal support for Cystic Fibrosis. Imagine if we had government funding for research instead of just the money that I'm able to raise!! Click here to read more about becoming an advocate: BECOME AN ADVOCATE
  • Say a prayer for little Drew. He needs it. And if you have one to spare, I could probably use one too.
And I'm sorry if I'm bugging you. Please just try to understand where I'm coming from. And Thank You!!

Friday, March 18, 2011

Great Strides T-Shirts and Other Official Business

We're going to order our Great Strides T-shirts soon and I'm so excited. Last year was such a whirlwind, as the twins were only a few weeks old at the time of the walk and we were still learning all about this thing called CF. I'm trying to figure out how many shirts to order, and here's where I need your help. We have decided that everyone who is signed up to walk and is raising money for us will get a complimentary t-shirt . We would also like to offer t-shirts to your family members or friends if they will be walking with you so that we can be identified as one unified team for Drew. We understand that not everyone is able to raise money, so we are going to offer the t-shirts to walkers who do not raise any money for a small fee (probably about $10 to cover the cost of the shirt) and all kids will get a shirt for free (while supplies last).

I am trying to figure out how many shirts I need to order, so I'm asking that you register yourself and your spouse/children/neighbors/friends or whoever will be attending the walk, regardless of if they plan to raise $ or not, so that I know how many shirts I will need. It would mean the world to us to have the support of all of our family and friends and neighbors and friends of friends and everyone who cares about us and about Drew, to have you there with us the day of the walk. For all of my Philadelphia friends & family, the other benefit to registering to walk is that all walkers registered by April 1 will receive free admission to the zoo the day of the walk (the walk actually takes place inside the zoo). If you do not register by April 1, you will have to pay $15 admission to the zoo for the day. This goes for all of your family members, including all children over the age of 1. So it might seem silly, but please register anyone who will be joining you on walk day so that you can get into the zoo for free and you can get a team t-shirt from us.
Here are the links to the cities we are walking in. There is no obligation to raise money (although we'd love for you to!) if you officially register as a walker. It will simply help me to gauge how many t-shirts I need to order. I'm ordering on April 1, so please register before then.

Next on the list, we're going back to Tobi. It was our decision and I think we made the right one since there's really no downside. Tobi only treats pseudomonas, and the last culture that was done on Drew was taken while he was still on the Tobi, but it came back negative for pseudomonas. That was good news, sort of, because it told us the Tobi was working to get rid of that bacteria. However, pseudomonas apparently is really good at hiding in your lower airways and doesn't always show up on oral cultures. The docs and us were both a little concerned that we would stop the Tobi and not cycle it (its usually given on a 28 day on/off cycle on an ongoing basis for people who are colonized with the bacteria) and the bacteria would be secretly living way deep down in his lower airways doing the damage that pseudomonas does. So we decided to do a second cycle of Tobi which will start on April 2, twenty-eight days after we ended the last 28 days run, and will end a couple of weeks before his next bronchoscopy. This is probably a lot of confusing jib-jab, but basically we're going to err on the side of caution and treat aggressively trying to eradicate the miserableness that is pseudomonas, lest it haunt us on an ongoing basis. I also should note that its not terribly common for CF kids to get as many bronchoscopy's as Drew gets. He mostly gets them to see how his airway abnormalities (tracheal malacia and bronchial malacia) are changing and hopefully improving, and how much they are really hurting his ability to appropriately clear his airways. Hopefully, as he gets older and outgrows these malacia problems, the bronch's will become less and less. By then though there will hopefully be a cure, which leads me right into my next topic...

There was another article giving some more media coverage of the new CF drug which will hopefully help Drew and thousands of other people. Here's what Business Week had to say about VX-770:

Vertex's Treatment for Cystic Fibrosis

Its VX-770 is the first pill designed to repair an inherited flaw based on the discovery of the gene mutation that causes the disease

Then in December 2009, Schaller started taking VX-770, an experimental drug from Vertex Pharmaceuticals (VRTX). Now the self-described exercise enthusiast says she can run 10 to 12 miles with barely any breathing problems. "This past year has been the coolest of my life," says Schaller, who runs the Rock CF Foundation, a nonprofit near Detroit that helps fund research on the disease, which afflicts 30,000 Americans. "It was a whole new feeling that I hadn't felt since I was a little kid."For the better part of two decades, Emily Schaller could barely take a deep breath without lapsing into coughing fits. At the age of 18 months, Schaller was diagnosed with cystic fibrosis, a hereditary disease that chokes the airways with sticky mucus, making victims susceptible to infections and other problems that often kill them before they turn 40. Schaller, now 29, says doctors told her parents, "Enjoy her, because she probably won't live long enough to graduate from high school." By age 13, she was suffering the full effects of the disease, which required her to take various medications and wear a vest that vibrates to loosen her congested lungs.

Vertex's drug is in the third and final stage of testing required for U.S. Food and Drug Administration approval. In a study released in February, Vertex reported that the drug increased breathing function by 10.5 percent over a year, reduced patients' need for antibiotics, and led to an average weight gain of seven pounds. "We were euphoric," says Robert Beall, chief executive officer of the Cystic Fibrosis Foundation in Bethesda, Md., which helped fund development of the Vertex drug. The results "suggest we've already added five years of life to these patients."

The CF gene, discovered in 1989, was one of the first found using new gene-hunting techniques that have led to better understanding of the biology of dozens of illnesses. The genetic research is helping scientists close in on treatments for cancer, diabetes, and other disorders that target glitches that occur during an individual's lifetime. VX-770 is the first pill designed to repair an inherited flaw based on the discovery of the mutation that causes the disease.

Cystic fibrosis is caused by mutations to a gene that, when working properly, creates proteins to help transport fluid in and out of cells. When the gene is defective and the proteins don't work, chloride, a component of salt, can't travel freely across cell membranes. That limits the flow of fluid outside the cell, causing mucus there to dry up and clog airways, allowing bacteria to gather.

VX-770, designed for a mutation carried by 4 to 5 percent of CF patients, enhances the function of the protein, creating a gateway that allows chloride to pass through the cell wall. About 90 percent of people with the disease, though, also suffer from a thornier problem: The protein never makes it to the cell wall. So Vertex is developing a second drug—VX-809—that helps transport the protein to the membrane. "To have optimal benefit, you need two compounds," says Eric Olson, head of Vertex's cystic fibrosis unit. "One to get the protein to the surface, and one to boost its activity once it's out there."

VX-809 is still in a so-called Phase II test as is the combined treatment. While VX-770 could win approval as early as 2012, any formula with both drugs likely won't be available for several years. "The combination therapy down the line is going to be the real driver," says Brian Skorney, an analyst with brokerage ThinkEquity in New York. Such a drug, he says, could eventually generate $1 billion a year in sales. On Feb. 23, when Vertex reported the VX-770 data, the company's Nasdaq-traded shares jumped 15 percent.

The medicines are the fruit of more than a decade of research. After the CF gene was discovered, scientists initially pursued gene therapy, trying to introduce a normal gene into cells to replace the faulty one. When that didn't work, the focus shifted to medicines such as VX-770 that could simply help defective proteins function properly. The Cystic Fibrosis Foundation asked drugmakers if they would screen compounds in their chemical libraries to determine whether one might affect the protein that helps move fluids in and out of cells.

Aurora Biosciences in San Diego agreed to test thousands of chemicals, eventually yielding compounds that led to the Vertex therapies. Vertex bought Aurora in 2001 for about $600 million and maintained the relationship with the CF Foundation, which supplied $75 million in funding for the program. "Discovering the genetic cause of a disease can lead you toward a therapy you never would have thought of otherwise," says Francis Collins, one of the CF gene's discoverers and now director of the National Institutes of Health.

The cystic fibrosis program, once an uncertain part of Vertex's future after the Aurora acquisition, has transformed the company into a diversified drugmaker, says Phil Nadeau, an analyst with Cowen & Co. in New York. Vertex is awaiting regulatory approval for Telaprevir, a medicine for hepatitis C that Nadeau estimates could draw more than $4 billion in annual revenue. "Now people see [Vertex] as more diversified," Nadeau says. "VX-770 is a second pillar the company can stand on."

The bottom line: If approved, Vertex's new cystic fibrosis pill will be the first treatment developed from genetic research for an inherited mutation.

If you missed my post on why this drug is good news for us, CLICK HERE to check it out.

That's about all the energy I have for this evening. It was a great week in our house. The nice weather allowed us to get outside for many hours on many occasions this week which was literally a breath of fresh air. Hope you'll sign up to walk with us!

Tuesday, March 15, 2011

A little bird told me...

Monday, March 14, 2011

Happy Birthday To You!

This picture was taken one year ago today. I remember it like it was yesterday. It sounds so cliche, but I swear a more true statement has never been spoken. In so many ways, it has been a long year. In so many ways, it has been a wonderful year. In more ways than I can express, it has been the best year of our lives. How can you not love these two? I just can't express on paper (or blog) the feelings I have for them, about them. I'm never at a loss for words, but today I can't think of the right ones to describe our perfect world. They have changed me in the best way possible.

Here's a few pictures from our year

We have a lot to celebrate this year. Do you want to help us? Can you spare $5 to honor Drew on his birthday? Even if you've already donated, or haven't yet but plan to later. Can you spare $5 to help him today? I want to celebrate his 2nd and 3rd and 60th birthday with him. Please help us. Happy Birthday Lily Grace & Drewboy. I love you!

Thursday, March 10, 2011

What on earth do ΔF508 & R560T mean??

I was watching a Great Strides video the other day about a little girl named Lilly who has CF. Her mom used the song "Just Breathe" by Pearl Jam in her video, and I loved it. You can click below to play it while you read whats on my mind this morning.

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I have been doing a little research on Drew's mutations, mostly because I haven't in a while and I just wanted to make sure that I understand what he's got going on. I'll take a step back first with a little bit of the biology on CF mutations. This info is probably going to take you all the way back to middle school, so bear with me :) Remember those genetics charts - aka the Punnett Square? Here's a picture to refresh your memory

That's how the genetics of Cystic Fibrosis work. Martin and I are carriers. If you think of the big G as the dominant trait and the little g as the recessive trait or the "mutation" in the gene causing it to not work properly, we each have one big G and one little g. Together, a big and a little do not equal the disease. Two big G's would mean that you got two of the dominant genes and therefore none of the "mutations". One big and one little would make you a carrier - like Martin and I are. We each got 1 dominant and one recessive gene from our parents. To get the disease you would need two little g's. Drew got my little g and Martin's little g. Make sense?

So now the crazy part of CF. There are thousands of kinds of little g's. (And for the record, the G's are demonstration purposes only, absolutely nothing to do with CF whatsoever). The two that Drew has are called ΔF508 (or Delta F 508) and R560T. ΔF508 is the most common CF mutation while R560T is more rare. Not all CF mutations cause the same problems, which is one of the challenges that doctors and researchers deal with in their search for treatments and a cure. All of these thousands of kinds of mutations are grouped into 5 classes based on what complications they cause. To make things even crazier, everyone with CF has TWO mutations (or they wouldn't have the disease) and the combinations of mutations from different classes makes it near impossible to pinpoint how a person is going to be affected.

We all know (or will after I type this) that the cells in our bodies (yes, yours and mine) all transport chloride as part of their job. The main problem with the mutant CF genes is that the cells in a persons body have trouble regulating the chloride that moves in and out of a cell. Without this chloride working properly, the mucus that is in our bodies gets too thick causes buildups, causing problems where mucus is critical - our lungs, pancreas, and intestines. You may not think of mucus as critical to these areas of your body, but it is, and very. You just don't recognize it as "mucus" in its typical form, because you don't see it. Its just a slippery substance thats in there helping everything to move along just as it should. In CF, this is not the case because the mucus is like mucus as you think of it when you have a terrible cold and are all snotty and gross. The mucus in Drew's lungs and gut are all just thick and sticky and don't allow things to move the way they need to, leading to life threatening infections.

The thousands of different CF mutations have been put into 5 classes. I promise I'm going somewhere with all of this so bear with me. Take a look at this chart below.
Here's the quick and dirty on what that chart means in picture form. This is from Johns Hopkins. Its a little tricky to understand, but try to follow the picture. In normal cells, the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) protein acts as a channel that transports chloride into and out of cells, and regulates the passage of salts. The CFTR can also transport other ions and chemicals such as bicarbonate.
The first picture is a normal cell. If you can follow, the chloride is the little red/purple dots. They move normally in and out of the cells as they should. Now look at the picture below to understand some of the different classes of CF mutations. All work a little differently, but basically prevent chloride from being processed appropriately in some way or another.

Class 1 mutations

Defective protein production with premature termination of CFTR production. Class 1 mutations produce few or no functioning CFTR chloride channels.

Class 2 mutations
Defective trafficking of CFTR so that it does not reach the apical surface membrane where it can function.

Class 3 mutations
Defective regulation of CFTR even though it is able to reach the apical cell surface.

Class 4 mutations
CFTR reaches the apical surface but chloride transport through the channel is defective.

Class 5 mutations
Reduced production of functional CFTR. A small amount of functional CFTR may reach the surface.

If you've been able to keep up thus far, you might now be starting to see how some of this gets complicated. Because everyone has TWO mutations in order to actually have CF, those mutations often time, as in Drew's case, fall into different classes. This poses a problem for researchers and doctors as they search for the cure. Uncertain of which mutation to treat or how to treat the many many combination to get the cells working properly. A lot has been done, thanks to your donations over the years, to figure out how to correct these problems. There is a cure for CF, but the research has not yet been able to determine how to make it stick inside of a person. That's how close we are.

So a couple of weeks ago I posted this article about a new and exciting drug which corrects the basic defect of one of the mutations of CF. The study was done on a mutation known as G551D. Well, mutation G551D falls into Class 3 of the mutation, the ones that have trouble regulating chloride. The new drug that should hit the market in later 2011 or early 2012 corrects this regulation problem, getting chloride in and out of the cells the way a normal persons would. Mutation R560T, one of Drew's mutation, also falls into Class 3. (I'll give you a moment to get excited if you can see where i'm going with this).....

So I emailed one of Drew's doctors this question - So the new drug that's going to the FDA for approval was tested on people with the G551D mutation. Drew has f508 and r560t and the r560t is in the same class of mutations as g551d. Do you know (or think) that that drug might be affective on all mutations in that class since it seems to correct the protein regulation? Thoughts? I know that no one really knows anything for sure, but does it seem like it might be a reasonable assumption to make?
This is the answer I got in two parts.
Part 1 - I looked into the question about VX-770 (the new drug coming to a pharmacy near you in 2011-2012) because I could not remember all of the details. You are correct that the G551D is a mutation that causes the CFTR (chloride channel) to get to the cell membrane but it does not pump chloride appropriately which is similar to Drew's R560T mutation. VX-770 "potentiates" this defective CFTR and allows more chloride to be pumped out of the cell. I do not know how the drug is able to potentiate this channel so I am not sure if VX-770 will be able to work on Drew's mutation. Luckily we have one of the lead researchers on CFTR mutations and drug development here at Cincinnati Children's, Dr. J.P. Clancy. I sent him an email and will let you know what he thinks. Of course as you said we will not know for sure unless we do studies on the other mutations as well. However, if the drug works to fix the channel that is independent of the specific mutation, then it may be a reasonable assumption that the drug will work for Drew as well. I believe the drug had the most effect on cells with the G551D mutation, which is why they studied it further in children/adults with this mutation.

Part 2 - Dr. Clancy got back to me about the possibility of VX-770 working on the R560T mutation. He said that the drug appears to work on multiple CFTR mutations and there is currently a project going on to look at its effect on the top 200 mutations. He seemed to think that if Drew's R560T abnormal chloride channel could get to the membrane of the cell, then there is a chance that the drug could help the channel function. As opposed to his DF508 abnormal channel which has a problem getting to the membrane of the cell. There is another drug being investigated to try to help the DF508 (Drew's other mutation) abnormal channel to get to the membrane. I tried to find out more information about the R560T mutation but I could not figure out if it gets to the membrane. If it is in the same class as G551D I would expect that it is able to get to the membrane, but I can't say for sure.

I'll take that! If the lead researchers on the basic defect of CF thinks there's a chance, I'm willing to put some hope out there for it too. An outstanding question that we asked a few months ago that no one really has the answer to is whether treating one of the mutated genes would correct the problems that CF causes. It would make sense, in my head, because if you think about it, I have one mutated gene and Martin has one mutated gene - thats how Drew got CF - but neither Martin or I has any of the effects of CF. So since Drew has two bad mutations, theoretically, if one of them were fixed and he just had 1 of the genes not working right, he would be cured, theoretically. We wouldn't need hours of airway clearance and dozens of medications to keep his body functioning "normally". I'm hoping that the time is near when we just have to take a pill everyday to right all of the wrongs that these two tiny mutations cause.

So that's what I have to say about that today, just 3 days shy of their first birthday. I really cannot believe that on Monday my babies will be 1. I'll write more about it Monday. Until then, I will live high on the hope that a cure is imminent, with the help of your donations (heres the link!) and I will enjoy my weekend with family and friends celebrating the two joys that came into our life one year ago :)

Sunday, March 6, 2011

Who doesn't love (essentially) free stuff!

March is Drew's birthday month, and I want to give away a present. I have some delicious Tastefully Simple products, totaling over $130, and you have the chance to win it. Everyone who donates $5 or more in the month of March will be entered into a drawing to win this basket. Donate $10, get two entries (and so on and so forth). Its that simple. Donate to me or donate to any of my walkers - we've got a lot of them! CLICK HERE to donate on my page. The winner will be announced at the beginning of April! And while you're here, check out some of my sponsors on the lefthand side of the page, all donating a portion of their sales to Drew and his fight against CF. Easter is coming up, and then Mother's Day. You can find lots of great gifts in those links!

Saturday, March 5, 2011

We need your help

I just can't help but tear up every time I watch the Great Strides videos being made by so many families affected by CF right now. I wanted to share a few. The first is a blogger friend. We've never met but we share a common bond - her daughter has CF. The next is a friend of a friend who also has a daughter with CF. These kids make you smile, but if you saw what was happening on the inside, if you saw what their daily routine looked like to keep them looking the way that they do on the outside, maybe you could start to understand. Live a day in my shoes for a moment. The median age of survival is 37. That's the median age. The average. Unfortunately that means that there are way to many young lives being lost to this disease that's dragging that average down. Is Drew going to be one of them? I met a woman today who is friends with a 18year old girl waiting for a double lung transplant instead of heading off to college with her friends. It breaks my heart.
A huge study just came out with fantastic results on a new drug that targets the basic defect of Cystic Fibrosis. Will it get through FDA approval in time? How much longer will these children have to suffer? Please help us. Please donate. To Drew, to Ayla, to Lilly, to the thousands of other kids living with CF. They need your help so badly.

And incase you haven't seen ours yet, here it is:

Friday, March 4, 2011

The end of the Pseudomonas fight...for now and hopefully forever

Tomorrow I will regain an hour+ of my days back. Tomorrow morning is our last dose of Tobi...for at least 28 days. You may recall the post about Pseudomonas. Well, we're hopefully nearing the end of treatment for that. The Tobi that Drew had to inhale twice daily for 30-40 minutes for the last 28 days is finished tomorrow. He will be off of it for at least 28 days...hopefully more. It depends on how his next culture looks. If hes growing Pseudomonas again then he will pretty much be on Tobi full time on a 28-day on 28-day off cycle. If he gets 3 negative cultures in a row over the next 3 months, then he will be considered Pseudomonas free and hopefully can remain that was for some time. Its almost inevitable that he will get it again at some point, and in all likelihood become colonized with it as a large percentage of people with CF are, but the longer he goes without Pseudomonas the better his lungs will be for the long term.

The outpouring of support from friends and friends of friends and neighbors and people I've never even met contributing to our Great Strides campaign has been HUGE. I sent a mailing out to our neighborhood - 163 letters to be exact - on Tuesday. I have received over $300 in donations from neighbors whom I have never met. Its incredible what people are doing to help my little boy.

I know that this fundraising stuff gets annoying. Dozens of people ask us every year to donate to their cause or help support a foundation near and dear to their heart. One can only give so much of their time and money, I do understand that. But you have to see where I'm coming from. I'm literally raising money to save my son's life. Seriously. The money that I raise goes 100% directly to research the cure for Cystic Fibrosis. I have to exhaust every avenue of possible funds to be raised. I will ask everyone. I will knock on doors and hang my flyers until a cure is found. I understand that not everyone can help me, but I can't understand not asking. The worst people can say is no, and I do understand.

I have one more sponsor who jumped on board today. Kathleen Groeschen, of Creative Memories, has joined me to help raise awareness and funds for the Cystic Fibrosis Foundation. All orders placed through her website will count towards my fundraising efforts. Kathleen has agreed to donate 15% of the sales towards the cause. Creative Memories is for EVERYONE who takes pictures and is looking for a solution to getting those pictures into their life so they can be viewed and enjoyed. Whether you are looking just for a way to organize your printed or digital images or you are trying to put the pictures into an album, there is a solution for you. (picfolio albums, traditional scrapbooking, quick kits, and paper albums). For those of you that work with a digital camera, Creative Memories offers two award winning software programs: Memory Manager helps you get your images sorted/organized/edited while Storybook Creator Plus helps you create beautiful digital photo books, posters, cards and prints quickly and easily! (All software purchases include a free one-hour consultantion). Kathleen also offers her services as a Personal Photo Organizer to do the work for you ( organized printed/digital images, convert slides/negative to a digital format, create traditional and digital photobooks for you!) Please check out Kathleen’s website at to place your order. When you check out, you will be asked to associate the sale with a hostess – be sure to use my name – Erin Moore. If you have any questions, feel free to contact Kathleen directly at / 513-459-2820 and she can advise you on the products or services that would best suit your needs.

I know that not everyone is going to be able to buy something from all of my sponsors, but I do think that there is something for everyone. A lot of the sponsors have products that make great gifts (something to keep in mind if you're not finding anything you need right now!). Any and all support is so greatly appreciated!