Keeping busy

I've been keeping myself pretty busy the past couple of weeks. We had family in town last week, and now we are in PA for about 10 days just visiting friends and family. Not much is new. The final results on Drew's cultures came back negative for pseudomonas so that was awesome. He did end up on steroids again last weekend for difficulty breathing, but we're pinning that on inflammation and malacia and not on bacteria, based on the good culture results. He recovered just fine.

Ella turns 3 on Wednesday and I just can't believe it. We had a little party for her at home, and we'll have another little one here on Wednesday. The twins had their 15mo. checkup last week and 3 of their 4 ears had infections. The doctor told me that a lot of kids with CF ended up getting tubes at some point because the thick mucus their bodies produces makes it hard for infections to drain appropriately from the sinuses. (that was from the pediatrician, not the CF docs) Drew has had the same infection for about 5 weeks now. It gets better and but never seems to really go away and then comes back with a vengeance a few days after the antibiotic end. We just finished up Azithromyacin and are hoping that we finally got it. I guess we'll see in a week when we go back for a follow up check!



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Parent TOUCH Program

I mentioned a while back that I was going to be volunteering with a new program at Children's Hospital called Parent TOUCH (Together Our Understanding Creates Hope). Well, its about to get started in July and i'm very much looking forward to it. There are currently 4 people who do this program, and then 3 new people joining (I am one of them). Here is a little video put together by Children's about the program.

Inflammation

Drew's sick. Well, he's not breathing the way hes supposed to be. I'm not sure if hes sick or not, but something isn't right.

We had our clinic appointment on Monday and the labs from them came back okay. No pseudomonas (WOOHOO!), no staph, a little Haemophilus grew, but that should be covered by the Omnicef he's on for his ear infection, so we're proceeding as though that's gone now. But this morning he woke up and he just wasn't breathing right. When he's struggling, his nostrils flare, he pull in around his ribs when hes breathing deep, and his breathing is quick. This all comes with a very non-productive cough that's near constant. Think about how you feel when you can't catch your breath - you cough, you take lots of deep breaths, its exhausting. We did his breathing treatments this morning but they didn't seem to change much. This oppressively hot weather doesn't help any.

We had plans for Gymboree this morning. I was nervous about taking Drew there. Its like a little mini gym for toddlers and kids up to probably age 5 I would guess. I knew they would all love it, but my mind sees a germ breeding ground on the mats and balls and all the other stuff all those little sweaty germy kids have their hands and mouths all over. I decided to take them anyway. I'm really trying hard not to let my germ anxiety take over my life. I do my best to keep things like toys and hands out of their mouths, and always have hand-sani in my back pocket. They did have fun. Everyone was laughing and running around and climbing and playing, including Drew. But he still couldn't breathe and it was worse than ever. He had an almost constant cough to the point where I was afraid he was going to throw up and he was just almost panting trying to catch his breath. But he wanted to play. I used his inhaler a few times to no avil. Finally, after about 40 minutes we left. It wasn't an easy 40 minutes either. I got a few looks from other parents, and some compassionate comments about how he must not feel very good, but mostly it was glares and comments under their breath about how sick he sounds. They don't know hes got CF and their kids aren't going to catch what he has, and I didn't want to make it the topic of the day, so I just said hes got something that causes some trouble breathing somethings. Heck, I wouldn't want my healthy kids hanging out with someone who sounded like he did. I can't really blame them, but I still just hate those situations.

When we got home I immediately did another breathing treatment but it didn't change much. I gave everyone lunch, put them all to bed, and called his doc. The initial reaction of the nurse was to suggest that he be put in the hospital to be monitored. I told her how much I didn't want that to happen, and she said she would talk to his doctor and call me back. About 10 minutes later, his doctor called me back. We talked for about 30 minutes, and decided that because of the cultures showing no bacteria in his lungs, that the difficulty breathing and cough was likely a result of inflammation rather than infection. CF people have chronic inflammation in their lungs, but with Drew's malacia complicating things, he gets into cycles where he has more inflammation than normal which causes him to cough which causes more inflammation and on and on the cycle goes. He takes Pulmicort twice a day to help with that inflammation but its not always enough. We are going to put him on steroids for a few days to see if knocking out the inflammation will help to end this cycle and make him feel better. She was comfortable with us doing the extra treatments and stuff from home vs the hospital because we've been down this path so many times before. This is one of the reasons I can't imagine moving away from here, because of the trust and understand and care we get from his doctors that is just invaluable.

The other thing that will help him right now is taking it easy. Ha! Telling a 15mo. old to sit and relax is like I don't even know what. The calmer he is the less hard he will work to breathe allowing some time for the inflammation to go down and get better. Its like a sprained ankle - stay off of it - take it easy - and it will get better. I hope it works. Steroids have worked for us in the past. He's not on them often - a few times over the winter when we had particularly bad infections (with infection comes inflammation) and they seem to get him over the hump. I hope this malacia thing isn't a forever thing for us because it causes unnecessary problems when we already have a big enough problem to deal with. Its hot out today, and I thought we'd go swimming after nap, but I guess we will build a fort and all watch Toy Story 3. There are certainly worse things that could happen.

Clinic Visit

We had our monthly visit yesterday with the CF clinic. Well, it had actually been about 6 weeks, the longest we'd gone without seeing those guys since we met them. Lily's been sick for the past two weeks so whatever she had seemed to be making it's way to Drew, so I was glad that we had the appointment when we did. Unfortunately when I got there I learned that our doc had to leave early for a family emergency, so I was going to see someone random. I'm fine with this if it's for a sick appointment, but for a well appointment, for someone to see him who doesn't know him and know what he sounds like and know and trust my assessment, it's never the most productive visit.

As it turned out, the doctor who I saw was the one who gave us the official diagnosis of CF back in the NICU a little over a year ago. He remembered me. He was a nice enough guy, but it rubbed me the wrong way a little when he was explaining Drew's malacia to me, as if I hadn't been dealing with it since birth and was way more familiar with his sound and anatomical abnormality than he would ever be. He thought everything looked and sounded alright, and decided not to treat things until e get the culture back. I guess we'll see. My fingers are crossed that the pseudomonas is gone, but it wouldn't be a total surprise if it shows up.

We're about to start some summer vacations in the next week or so. My mom is coming out next week for the week to both visit and provide childcare because the Yankees will be in town and we've got tickets to all 3 games. Then she will be driving with me and the kids back to PA until after the 4th. I'm hoping to make some plans to meet friends for lunches or dinners or drinks or whatever. I'm sure the week will just fly by. Martin is flying out at some point to join us and make the drive back home. After that, Ella has a couple of summer camps and then we're driving to Charleston, SC for a Moore family vacation. I hear it's hot in Charleston, yet I picked August to go. Hopefully the company of cousins will keep the kids happy and the pool and the ocean will keep us all cool.

I haven't posted out final numbers yet from the walks because we're not 100% totaled up, but I will soon. It was a great year overall, and I'm already planning to top it next year!

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Location:Presidio Ct,Cincinnati,United States

Another step in the right direction, and our Great Strides dollars hard at work!

Phase 2 Study of Two Potential CF Therapies — VX-770 and VX-809 — Shows Promising Results in Patients with Most Common Mutation

June 9, 2011

Vertex Pharmaceuticals Incorporated and the Cystic Fibrosis Foundation today announced promising results from an ongoing Phase 2 study evaluating combinations of VX-770 and VX-809, potential medicines designed to treat the defective protein that causes cystic fibrosis.

The study enrolled 62 people with two copies of the most common CF mutation, known as Delta F508. The trial lasted three weeks. Participants took VX-809 for two weeks, and VX-809 and VX-770 together for a third week.

Patients who took the drug regimen showed a positive change in sweat chloride levels. Excessive sweat chloride is a key clinical indicator of cystic fibrosis. The findings suggest that VX-809 and VX-770 together improve function of the defective CF protein, known as CFTR.

“The results of this study represent a milestone in our efforts to expand the use of small molecules to attack the root cause of cystic fibrosis in those with the most common defect,” said Robert J. Beall, Ph.D., president and CEO of the CF Foundation. “These data, while early, provide important new information that validates our approach and supports continued study of a combination-therapy approach to treating the basic defect of cystic fibrosis.”

The CF Foundation worked with Vertex to discover VX-770 and VX-809, and has provided substantial scientific, financial and clinical support throughout the development process, including an approximately $75 million investment.

These positive results support further testing of VX-770 and VX-809 as a combination therapy. Vertex plans to initiate the second part of this Phase 2 study in the fourth quarter of 2011.

In people with the Delta F508 mutation, the defective protein does not move to its proper place at the cell surface. VX-809 is designed to help the protein reach the cell surface, while VX-770 aims to help the protein function more normally once it is at the cell surface.

Today I'm just down

I'm in a mood today. I didn't wake up in a mood. The morning was quite nice actually. We slept in until about 8. And then after morning treatments and breakfast, I took the kids to an indoor play place for a few hours and everyone seemed to have a lot of fun. I didn't use to take Drew to many places like that for fear of germs, but now that hes walking and into everything, seeing how much he enjoys it just forced me to let go a little. Not everything goes into the mouth directly anymore, and I have to realize that hes going to be a kid just like every other kid - fingers in the mouth and up the nose from time to time, finding a drink and going for it whether or not its his, sharing toys with other snotty little ones - germs just happen. And all I can do is be reasonable about things by wiping hands and noses after play and before eating, cleaning toys, keeping him away from situations that I know are likely to make him sick. But I think about Ella's little preschool this past year - there wasn't a day that went by that someone in that room wasn't rubbing snot all over something. Thinking of Drew in that situation sends me into anxiety overload. But what am I to do...

Another thing thats been frustrating me lately is the lack of interest and understanding in what CF really is, how bad it really is, and how it is affecting us. Its a balance, I get that. I try to balance. He looks healthy. Hes not. I'm scared for his life. I want him to be treated normal, but I also want people to understand what CF really is and what it really does, and I don't know how to get that across. Would people donate more if he was a frail looking little boy who carried around an oxygen tank? Probably! Thats the sad reality for me. Perception is reality. I should put together a video of what our days look like. If you could see how much time is spent on his care in 1 days, it might change the way you think about it. But I don't know what I want you think about it! I don't want pity. I don't want you to feel sorry for him. I want you to fight with me against CF for a cure. I want our knowledge to be productive. Thats what I really want. I don't want people seeing him and saying "It doesn't look like there's anything wrong with him!". That makes me crazy.

The other thing thats been making me nuts is smokers. F you all. There was a woman ON THE CF WALK who was smoking a cigarette. She hung a left before I had the chance to go off on her, but how dare you smoke on walk raising money to research a disease that affects the lungs. How DARE YOU! How dare you, lady in front of Walmart who was probably in her 50s but looked like she was in her 70's smoking a cigarette, how dare you bitch to the pharmacist about your insurance when my son is fighting lung disease and he's never smoked a goddamn cigarette in his life, yet I spend HOURS on the phone fighting insurance companies who don't want to cover his health care. How dare you.

There is a girl in Cincinnati named Alicia who is 17 years old and is waiting for a double lung transplant because her lungs are completely deteriorated from fighting Cystic Fibrosis. Her insurance company will only cover 80% of the 1.5million dollar procedure. She has to stay in a sterile environment for weeks after having the transplant so that she doesn't catch something that could cost her her life. And this is all if lungs become available before its to late. She is 17. There is another girl, Claire, who just turned 14 years old and is dying from lung disease, but she can't have a double lung transplant because her body would reject new lungs. Having the procedure done would likely kill her. Not having it done will surely kill her. Another little boy who was 7 passed away shortly after Drew was born from Cystic Fibrosis. He was 7! And then THIS article where a professor and psychologist at the University of Wisconsin-Madison School of Nursing stated, “In my opinion, it’s just tragic that parents have to suffer and think about their child having a chronic illness when the child is perfectly healthy.” So sorry for you. My son has a lifetime of suffering, so sorry that you had to go through two weeks of stress because of a false positive. You should be thankful that there is a test that can catch these diseases when they are newborns and save lives.

These are all just kids!! Its just not fair! I know that a lot has happened over the past several decades that has extended the life of many CF patients, but theres still just no telling when you're going to catch something that you just can't shake or when CF is just going to get the best of you. Most days I can wake up and put on my positive, fighting attitude and go out fundraising at every chance I get, but today I'm just down for no particular reason. I'm scared of it. I'm hopeful and I'm optimistic but I'm still scared. I always have a lump in my throat for a week before we go to the clinic because I know they will take a culture and I never know what will come back. I just wish it didn't have to be this way. I'm hopeful about things. I'm hopeful that there will be a cure found in time. Today I'm just down about it and I needed to let it all out.

If you have a moment to help, it could make a big difference

I'm not sure how far this blog reaches, but if you find yourself reading this and wondering how you can make a difference, here's how.

I received an email from Eric Chamberlain, the Public Policy Specialist for the Cystic Fibrosis Foundation, that requires some action from us, the people who want to change the world for our kids and our friends and our families struggling with Cystic Fibrosis. Here's the email:

On June 14^th, National Institutes of Health (NIH) Director Dr. Francis S. Collins will join CF Foundation President and CEO Dr. Bob Beall and Vertex Pharmaceuticals Chairman and CEO Matthew Emmens on Capitol Hill for a roundtable discussion with Members of the House of Representatives to discuss new research initiatives NIH is using to accelerate the development of therapies. The speakers will also highlight how the successful partnership between the Cystic Fibrosis Foundation and Vertex Pharmaceuticals on VX-770 can serve as an example for federal government programs. We need as many Representatives as possible attend this event to enable the CF Foundation and Vertex Pharmaceuticals to increase awareness about cystic fibrosis and share how our successes can be replicated by NIH to help those with other illnesses.

Please call your state’s Representatives today and ask them to attend. Attached, you will find a list of Representatives who are being targeted in particular (along with their DC office phone numbers), but please feel free to contact as many of your state’s Representatives as you can. Contact information for all of your state’s Representatives is available through http://www.cff.org/GetInvolved/Advocate/TakeAction/. Select your state from the drop down list on the right under “Legislator Search”, then scroll down to “Federal House”.

Below, you will find the information you will need for your calls:

· Talking points

· Event details

· An additional copy of the target list

If the Representative’s office would like more information, you can send them the attached flyer announcing the briefing, or tell them to contact Stephanie Krenrich in the Public Policy Department at (301) 907-2512 or skrenrich@cff.org. If you need more information, you should contact Stephanie as well.

With your help, we will make this an event to remember.

The past few decades have brought exciting scientific breakthroughs necessary to understand,

diagnose, and treat many diseases. However, the ability to translate exciting advancements into treatments that can help patients severely lags behind the pace of innovation. On average, it takes 15 years to turn a scientific discovery into a viable therapy. For the millions of Americans who live with chronic and fatal diseases, this is simply too long to wait.

TALKING POINTS

When you call, please ask for the Representative’s legislative assistant for healthcare issues. When you are connected either to the individual or their voicemail, please use these talking points as a guide:

1) As a constituent personally affected by cystic fibrosis, I wanted to invite [Representative x] to a briefing hosted by the Cystic Fibrosis Foundation and Vertex Pharmaceuticals with Dr. Francis Collins, the Director of the National Institutes of Health, on Tuesday, June 14^th at 9am in room 2218 of the Rayburn House Office Building.

2) Dr. Collins will discuss “translational science” programs at NIH, which aid the translation of basic medical research into new treatments for patients

3) These initiatives have the potential to speed the development of much-needed medications for some of our most serious illnesses

4) Speakers will also discuss the successful partnership between the Cystic Fibrosis Foundation and Vertex Pharmaceuticals on new cystic fibrosis therapies as an example of how strategies NIH uses have been successful in the private and non-profit sectors

5) Would you like me to email you some more information? [If yes, ask for email address and follow up with the attached briefing announcement]

-or-

Please call Stephanie Krenrich at the Cystic Fibrosis Foundation at 301- 907-2512 for more information.

6) This briefing promises to be a constructive discussion on the best way to spur the development of new treatments for disease, and we hope that Representative x will attend. Thank you for your consideration.

EVENT DETAILS

Roundtable Discussion with NIH Director Francis S. Collins: Leveraging Federal Investment to Speed the Development of Promising Therapies for Patients

WHEN: Tuesday, June 14, 2011

9:00 am – 10:00 am

WHERE: 2218 Rayburn House Office Building

SPEAKERS:

· Francis S. Collins, M.D., Ph.D., Director, National Institutes of Health

· The Honorable Brian Bilbray, Edward Markey, and Cliff Stearns, Members of Congress

· Robert J. Beall, Ph.D., President and Chief Executive Officer, Cystic Fibrosis Foundation

· Matthew Emmens, Chairman and Chief Executive Officer of Vertex Pharmaceuticals

· Eric Olson, Ph.D., Vice President, Cystic Fibrosis Franchise Lead, Vertex Pharmaceuticals

INVITEES: Representatives (plus 1 staff)

TOPIC: NIH’s translational science programs, including NIH’s proposed National Center for Advancing Translational Sciences (NCATS), which have the potential to accelerate the development of much-needed treatments for some of our most serious illnesses. In addition, the event will feature the successful collaboration between the Cystic Fibrosis Foundation and Vertex Pharmaceuticals on promising new cystic fibrosis therapies, as an example of how the strategies to be employed by NCATS have been successful in similar situations in the private and non-profit sectors.

TARGET LIST

Alaska: Representative Don Young (R-AK-AL) (202) 225-5765

Arizona: Representative Paul Gosar (R-AZ-1) (202) 225-2315

California: Representative Mary Bono Mack (R-CA-45) (202) 225-5330

Representative Lois Capps (D-CA-23) (202) 225-3601

Representative Susan Davis (D-CA-53) (202) 225-2040

Representative Howard “Buck” McKeon (R-CA-25) (202) 225-1956

Representative Lucille Roybal-Allard (D-CA-34) (202) 225-1766

Colorado: Representative Diana DeGette (D-CO-1) (202) 225-4431

Connecticut: Representative Rosa DeLauro (D-CT-3) (202) 225-3661

Florida: Representative Mario Diaz-Balart (R-FL-21) (202) 225-4211

Representative Debbie Wasserman-Schultz (D-FL-20) (202) 225-7931

Representative C.W. Bill Young (R-FL-10) (202) 225-5961

Georgia: Representative Paul Broun (R-GA-10) (202) 225-4101

Representative Phil Gingrey (R-GA-11) (202) 225-2931

Representative Tom Price (R-GA-6) (202) 225-4501

Idaho: Representative Mike Simpson (R-ID-2) (202) 225-5531

Indiana: Representative Larry Bucshon (R-IN-8) (202) 225-4636

Louisiana: Representative Bill Cassidy (R-LA-6) (202) 225-3901

Representative John Fleming (R-LA-4) (202) 225-2777

Maryland: Representative Andy Harris (R-MD-1) (202) 225-5311

Massachusetts: Representative Mike Capuano (D-MA-8) (202) 225-5111

Representative Jim McGovern (D-MA-3) (202) 225-6101

Michigan: Representative Fred Upton (R-MI-6) (202) 225-3761

Representative John Dingell (D-MI-15) (202) 225-4071

Representative Dan Benishek (R-MI-1) (202) 225-4735

Representative Sander Levin (D-MI-12) (202) 225-4961

Minnesota: Representative Betty McCollum (D-MN-4) (202) 225-6631

Montana: Representative Denny Rehberg (R-MT-AL) (202) 225-3211

New Jersey: Representative Leonard Lance (R-NJ-7) (202) 225-5361

Representative Frank Pallone (D-NJ-6) (202) 225-4671

Representative Rush Holt (D-NJ-12) (202) 225-5801

Representative Bill Pascrell (D-NJ-8) (202) 225-5751

Nevada: Representative Joe Heck (R-NV-3) (202) 225-3252

New York: Representative Eliot Engel (D-NY-17) (202) 225-2464

Representative Nita Lowey (D-NY-18) (202) 225-6506

Representative Nan Hayworth (R-NY-19) (202) 225-5441

Representative Joe Crowley (D-NY-7) (202) 225-3965

North Carolina: Representative Sue Myrick (R-NC-9) (202) 225-1976

Pennsylvania: Representative Tom Marino (R-PA-10) (202) 225-3731

Representative Tim Murphy (R-PA-18) (202) 225-2301

Representative Joe Pitts (R-PA-16) (202) 225-2411

Representative Charlie Dent (R-PA-15) (202) 225-6411

Representative Allyson Schwartz (D-PA-13) (202) 225-6111

Tennessee: Representative Marsha Blackburn (R-TN-7) (202) 225-2811

Representative Phil Roe (R-TN-1) (202) 225-6356

Representative Scott DesJarlais (R-TN-4) (202) 225-6831

Texas: Representative Michael Burgess (R-TX-26) (202) 225-7772

Representative Ron Paul (R-TX-14) (202) 225-2831

Washington: Representative Norm Dicks (D-WA-6) (202) 225-5916

Representative Dave Reichert (R-WA-8) (202) 225-7761

Wisconsin: Representative Tammy Baldwin (D-WI-2) (202) 225-2906

Advocacy

There are currently 3 things going on in government that WE have the ability to influence by sending a simple letter to our legislators asking for their support. It honestly takes just a moment to click on the link and get this important message out to our decision makers. People ask me all the time how they can help - this is how. Click here. Sign in. Send the letter. Add a personal note about Drew or about your son or daughter living with CF, or friend, or whatever. Send it. Help me save my baby.

Flash Mob for a Cure

Survivor's Story: Flash Mobs For Claire: MyFoxLA.com

Reds game FAIL

I feel like I haven't had a minute of downtime in the past several months. We were so busy with our fundraising for so long and then the walk just came up so quickly and now that summer has arrived and the kids are itching to get outside, I'm always running in one direction or another and when I do get the chance to sit down at about 9pm I'm just to tired to function. We've been keeping busy with trips to the splash park and other parks, lunches with friends, lots of pool time in the backyard. I might as well tar and feather the kids because that's what it's like when they are all greased up with sunscreen and then stuck outside to roll around in schmuck. Every night is bath night these days.

We took a family trip to the Reds game yesterday. It very well might have been the #1 least pleasurable experience of my life. It was 97 degrees and we had to park blocks away from the stadium. We didn't get there until almost the 5th inning and we were gone by the 7th. We were seated 3 rows from the sun and had only bought 2 tickets. The place was damn near sold out so we were three kids and 2 adults sweating profusely in 2 small stadium seats. We had to take the stroller because there's no other way to transport the twins, but we checked it at guest services. The hike up the the nosebleed section was not easy. We took snacks and drinks with us but it didn't do the trick for anyone. Next time (said loosely) I would definitely buy 5 tickets and wouldn't go anytime after probably early May. I love so much when the 5 of us get out and do things together because it doesn't happen often, but this wasn't exactly the picture of a good time. Oh well, lesson learned.

Drew has another ear infection. I guess the first one never really went away. It takes him longer to clear secretions in general so an ear infection is really no different. We're trying Omnicef now. The girls both have summer colds so I'm hoping that if Drew catches what they've got that he can deal with it just like anyone else deals with a virus because he's already on antibiotics which should eliminate the possibility of the funk turning into bacteria and landing us on even more meds. I guess we'll see.

I'm working on our "plan" for vacation this summer. We are driving to Charleston, South Carolina and I need to make sure that I have all the appropriate meds and equipment and contact information and the scoop on local resources, should we need any of it. We aren't going until August, but it will be Drew's first big trip and time away from home and I wanna make sure that it's enjoyable and easy and if I have a plan and my ducks in a row we should be good.

Thats about all we've got going on around here these days. Nothing to exciting, and I'm okay with that.


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