Thursday, December 29, 2011

Out with the old, in with the new

I don't think I properly expressed how excited I was about Drew's clean culture. This is the first culture he's ever had, EVER. He had his first culture in the NICU shortly after he was born and was already growing staph in his lungs. Since then we've had everything from staph to pseudomonas and everything in between. Just when we get rid of something, something new grows and it seems to be an endless cycle of antibiotics and doctors visits. But not this time. This time, we went two whole months without a call or visit to the doctor and the outcome was a culture free of any and all bacteria. There wasn't even some of the less harmful, more common bacteria in there. There was nothing. I know it doesn't mean there will be nothing forever, but for now, what a great feeling. And I mean we are smack in the middle of cold and flu season. If we can just make it to April bacteria free when the new CF drug will hopefully receive approval from the FDA and we can try it and it will hopefully work for us, we might never have to deal with bacteria in the lungs again. A new year is right around the corner and I'm hoping it means a world of change for us, in a good way of course.

Our Christmas was good. Naturally, we overdid it. The kids got tired of opening gifts about halfway through and just wanted to play with what they had opened. We ended up putting about a third of the gifts back into the closet to be pulled out here and there over the next couple weeks of winter while we're stuck inside. The hit of the holiday was a trampoline in the basement. It definitely helps them to work out their energy during the day and sleep well at night. And its in the comfort of our basement so I get to relax on the couch while they jump away.

Martin and I got out to dinner while my folks were in town. It was almost comical trying to figure out where to go when given the opportunity. I guess that shows just how little we get out these days. It was nice though!

Quatro is growing nicely. We have about 12 more weeks to go and preparations are underway. I'm hoping to transition Drew and Lily to beds by the beginning of Feb so that they have a couple of weeks to adjust before #4 gets here. Their new room is yet to be painted and we have some rearranging to do, as their new room use to be our guest/storage room. What we're learning is that we have way to much crap.

I'm gearing up to start our Great Strides fundraising for 2012. The past couple of months have been spent brainstorming, trying to find the best ways to raise the most money for CF. This is a huge year for the CF Foundation, with them operating in deficit due to all the funds they put into the development of these new drugs that just very well might change the world. I don't say that loosely either. These drugs truly might change the entire world of genetic diseases, not just Cystic Fibrosis. We can only wait and see right now, but I will do everything I can to try to help facilitate that change.

I wanted to thank everyone who reads this for your kind words and support that you've shown us over the past year and a half. We're really hoping that 2012 is a great year for us and for you as well!!

Friday, December 23, 2011

Great Early Christmas Gift

Everytime we go to the clinic, the swab his throat to get a culture of the bacteria that he may be growing in his airways. It is always an anxiety filled week waiting for the results, especially this time since the results were due back today, December 23, just two days before Christmas. Well I am happy to report that there is no staph and no other really bad bacteria currently causing trouble in his airways! His culture came back mostly clean, with a little staph which we always seem to have. He is already on an antibiotic for this sinus thing he has going on, which I believe will cover the staph, but nothing is causing any coughing or difficulty breathing so no other treatment is necessary! Such great news for our little man. We can celebrate Christmas without an IV antibiotic schedule :)

Wednesday, December 21, 2011

Making a difference

I don't know if I mentioned on here the family that I met several months ago in the NICU at Childrens who had a son born with renal failure. He was too small for a transplant or dialysis and so they were basically waiting for him to die. There was almost no chance that he would survive long enough to get a transplant, and a miracle was about all that was going to help him. Well that miracle came! I saw the family tonight and the little boys kidneys just started working. I don't know how or why, but they were elated and are hoping to be going home sometime in January. I've thought about them a lot over the past several months and I'm just so happy for them.

There were generally fewer people than normal there when I was there tonight. I think that's awesome, given that Christmas is just 4 days away. Most of the folks that I had known all went home on good terms, and I can only hope that the ones that I didn't know so well also left on a high note. We spent one Easter in the NICU and it was definitely a bummer holiday, so I can't imagine having to be in there for Christmas. But I know that the kids in there need to be in there and are getting the best care they can.

We heard some great news from the Cystic Fibrosis Foundation again today. I got the following email from one of the Public Policy Directors:
I’ve got some great news to share. As you may know, late last week Congress passed the final FY 2012 Appropriations bill - or “omnibus” - at long last completing the budget process for the current fiscal year. Thanks to your hard work, the CF Foundation has achieved three of our main policy priorities in the budget, no small feat considering what a contentious year this was on Capitol Hill.

1) As we told you a few weeks ago, funding for the Food and Drug Administration was increased by $49 million in the 2012 budget. This boost will help reviewers to more quickly move important treatments from the lab to the patients who need them.


2) I am happy to report that the National Institutes of Health (NIH) will receive a $300 million funding increase in Fiscal Year 2012, providing resources to advance innovative biomedical research for diseases like cystic fibrosis.


3) Congress established the National Center for Advancing Translational Sciences (NCATS) at NIH in the final FY 2012 budget, one of the Foundation’s biggest priorities. The Foundation has been a strong supporter of this center and believes it will have a real impact on the advancement of drugs for serious diseases.


These victories show just how profound an influence citizens like you have on their members of Congress. In no small part because of your tens of thousands of calls, emails, and meetings, the federal government will have the tools to help us move closer to the cures of tomorrow. There is no doubt that your persistence helped make this possible, and you should be proud of all that you do.

Thank you for being such an integral part of Team Public Policy in 2011, and we look forward to an even better 2012! Happy holidays to all of you and your families.
That really is all great news for CF. I wanted to thank everyone who participated with me in clicking on links I provided through my blog and contacting decision makers to let them know how important all of this stuff is to us. We can and did make a different and I know that I will certainly continue my efforts going forward knowing that!


Tuesday, December 20, 2011

Clinic Visit

We had a clinic visit yesterday. I felt like we hadn't been there in ages, when in reality it was just over two months. I hadn't had to call or make one visit there since our last appointment because Drew's health has been so good. It was pretty remarkable that he went that long without getting sick or having something happen that would at least merit a call to the clinic, but it happened.

The doctor thinks he looks and sounds great. The head cold that he's had for 3+ weeks now was diagnosed as a sinus problem. Apparently most people with CF end up with some degree of sinus disease. Just how a cold that gets into the lungs has a hard time getting out, so does a cold that sets up shop in the sinuses of a CF kid. What for Ella or Lily would have just run its course as a runny nose, for Drew turned into inflammation of the sinuses and extra mucus production creating an infection that he wasn't able to clear on his own. We ended up on a nasal steroid and some oral antibiotics for 14 days to get that cleared up. Very luckily, nothing had moved to his lungs at all and hope is that remains the case. They did do a throat swab to check for bacteria in his lungs, and we will have those results back by the end of the week. I asked what our course of action would be if he was growing pseudomonas or something again, and the doctor said that based on his awesome clinical appearance, nothing would need to be done until after Christmas. What a relief to not have to worry about squeezing in extra treatments over the holiday weekend!

I talked to the doctor also about the hunger strike that Drew is on. It was a damn miracle that the boy gained any weight between the last visit and this one because his diet consists of whole milk and an occasional corndog. He refuses almost everything, and yet somehow he continues to gain weight. Because weight is such a huge issue for many CF kids, it causes a lot of anxiety for me when he won't eat. I fear that he will end up with a g-tube feeding him overnight just to get in enough calories. Its not the end of the world if that happens as its the reality for many folks with CF, but with the good weight that he's always been able to show us, I would just hate for things to turn that direction. The doctor and the dietician both think that his weight is great and I shouldn't worry. He's in the 90th percentile and the goal for CF babies is to make it to the 50th. If he loses a couple of pounds we'd still be in good shape, but I just feel like if we allow this sort of behavior now that it could eventually turn into a problem. They recommended that we meet with a behavioral psychologist who specializes in eating issues in kids with CF. I spoke with her for a few minutes before setting up an appointment to come back, and she suggested that for kids his ages, its simply about control. They can't control much, but he knows hes got my attention with his mealtime antics, and she can offer some suggestions for making meals a better experience for all involved. We'll see what happens.

The other thing that Drew's doctor mentioned was the feedback that they had received on the presentation that was done on him at the NACFC. Here is the link to it - http://nacfcdl.cff.org/Documents/Wyatt.pdf . She said that the presentation provoked a great deal of conversation, and that a group of doctors and researchers wanted to further expand on this presentation with more studies and research. How cool that Drew started a conversation about malacia and airway abnormalities in CF that could lead to more research on the disease?!

Finally, some more great news out of the CF world just posted today. Kalydeco, the drug that's currently going through the approval process in the FDA, is now available at participating clinical sites throughout the country for people with the G551D mutation who have highly limited lung function and may benefit from treatment. The drug that may just change the game for us as we too have a class three mutation (though not the specific one tested) is already being used to change the lives of people who are critically ill with CF. What a great Christmas gift for the people who will benefit from this drug!! Hopefully it works for them and will work for us when it becomes available soon.

Thursday, December 15, 2011

FDA Grants Six-Month Priority Review of Kalydeco (VX-770) — First Potential Drug to Target Underlying Cause of Cystic Fibrosis

Just a few days ago, I wrote a post about my annoyance with the FDA drug approval process when I heard that a "hangover pill" had made it through the pipeline while life saving medications like VX-770 sit in the queue. Today, my attitude toward them is changed. Today, Vertex Pharmaceuticals announced that the FDA has granted a six-month priority review for Kalydeco (vx-770)!!! The application for approval of the drug was made in October, which means that by April a decision will be made on the approval of this drug. By April, this drug could be available to us and begin correcting the problems that CF is causing at the cellular level. This is so incredible and awesome for so many reasons. We don't know if the drug will work for us, but we're going to give it a shot. If this drug works for Drew and he can get it before he has any progression of lung disease, his life expectancy will be the same as that of you or I. This drug will help his body to work normally, and prevent him from catching those life threatening lung infections that we deal with all to often. I'm so excited by this news and I hope that we can try it as soon as its approved...and that it works for us!!!
Here's the press release from the the Cystic Fibrosis Foundation:

FDA Grants Six-Month Priority Review of Kalydeco (VX-770) — First Potential Drug to Target Underlying Cause of Cystic Fibrosis

December 15, 2011

Vertex Pharmaceuticals, Inc., announced today that the U.S. Food and Drug Administration (FDA) has granted a request for a six-month priority review of a potential new CF therapy, Kalydeco™ (VX-770).

The company is seeking approval of the drug for people ages 6 and older with the G551D mutation of CF.

The expedited review sets a target date of April 18, 2012, for the FDA’s approval decision, four months earlier than the standard review time of 10 months.

If approved, Kalydeco (kuh-LYE-deh-koh) will be the first drug available that targets the underlying cause of CF. The FDA grants priority review for several reasons, including situations where a potential drug offers a major advance in treatment.

Kalydeco was discovered in a collaboration between Vertex and the Cystic Fibrosis Foundation, which provided substantial scientific, financial and clinical support throughout the development process.

Vertex’s application for approval of Kalydeco, submitted to the FDA in October 2011, included results from Phase 3 clinical trials of the drug in people ages 6 and older with the G551D mutation of CF. The results showed that those receiving the drug had remarkable and sustained improvements in lung function and other key symptoms of the disease, compared with those on the placebo.

Additional Resources

Tuesday, December 13, 2011

Hold on to your valuables!

Twelve days before Christmas and all through the house, everyone was grumpy and I had a sore back. I'm not about to break into poem here, just spreading some more holiday cheer. We are ready for a break, a change, something. I am so darn tired with being pregnant and chasing around three lively children all the time. Its way to cold to go outside, and I'm way to tired to try to get them all dressed and bundled to go anywhere. So we play with the same toys everyday. We watch lots of movies. We've tried to be festive with cookie baking and decorating. Our Elf on the Shelf has only been out since Saturday and we've already forgotten to move him...twice. He isn't having the affect that I would like him to be having. I need to make a secret trip to see Santa and give him the skinny on what my kids need to hear from him before I take them there for a visit.

Today I purged in the playroom. Three trashbags full of toys and toy parts and junk and garbage. I was so sick of our house being a constant mess of stuff that I eliminated things they don't play with, things that are missing critical parts, things that aren't toys (my tupperware bowls). I put puzzles back together with their appropriate pieces. The play food is with the play plate and play forks and knives. The trucks are in the truck bin. The guys are in the guy bin. For the first time in a long time there is a sense of organization and it feels good. I don't though. I am tired and I am sore. Its a trade-off I supposed. This was a good trade-off.

The basement has seen better days, but its the basement. We need to get it cleaned up before Christmas but I have, what, 12 days left to do that? No sweat. Yesterday we baked and decorated cookies all afternoon with some friends from school. It was really a fun afternoon. We're giving the preschool teachers some cookies and other little gifts that the girls made. It was a fun afternoon, but by about 7pm I was seriously beat. I laid down at about 9pm and slept until 8am. I wasn't doing any kind of extra hard work, but it seems that anything extra these days takes it out of me. Only 14 more weeks and for some reason in my delusional mind I think thinks will get better. Whats one more kid, right? At least I won't be pregnancy exhausted! Again, I acknowledge my delusional thinking.

I'm going to try to go through some Christmas presents to see what all we have and who we have it for. I think we're done shopping but I need to inventory. Its time for this Santa to get wrapping! Hope everyone is staying jolly jolly!

Sunday, December 11, 2011

Christmas Cheer

My kids are all in bed already, though none of them are asleep yet. I'm exhausted. I have a hundred and ten things I both need and want to get done in the next two weeks before Christmas and I have no clue when or how I'm going to accomplish most of them. I've been trying to keep in the holiday spirit, baking cookies with the kids, taking the whole gang to cut down a tree, decorating said tree as a family. Most of these events end in sheer disaster, with me pissed off and the kids all screaming and crying. Yet I push on. Tomorrow we are doing holiday crafts and baking cookies with a few of Ella's friends from school. I volunteered my house because the twins can sleep while we all work, though the thought of the mess that will be our house at the end of the day makes me want to cry. I'm so tired of cleaning, and I don't even really clean (I pay someone to do that for me, judge away). I'm tired of little people pulling out parts to 100 different toys and leaving them all over the house. I'm tired of never having all of the parts to any given toy or any game. I blame myself. They are still so young. That doesn't excuse them from picking up their toys, but lack or organizational skills leaves us with lots of pieces but no puzzle. I'm determined to clean out the playroom before Christmas. I want to put parts of toys together with their counterparts so that when someone gets out something to play they can enjoy the whole toy and [hopefully] not get frustrated by the missing parts and walk away. I'm just so darn tired! By 8:00 when everyone is in bed, I want to go to bed. I have been choosing bed over any of the other tasks I want to complete more often than not, and its showing in this house. Maybe its a bit of nesting setting in, but we are down to 14 weeks until this family of 5 becomes a family of 6 and we don't have anywhere to put Quatro. The guest room is becoming the twins new room and the baby will go into the nursery. That's the current plan. Only the guest room is currently our storage facility for all outgrown clothes and pretty much everything else in the house that doesn't have a home. We will have to find a new storage facility...within the house of course.

The one thing I have going for me is Drew's health. He still has the gunky nose I spoke of last week, but it has yet to hit his chest. Its been a real blessing not having to find time to talk to doctors and nurses and run into the office so they can check him out. I've spent more time at Children's Hospital in the past 3 months without him than have I have with him, and that's awesome. He has his next visit on the 19th and I hope that they're as happy with his health as I am. And of course I hope that we get a good culture report. It will suck if he's growing bacteria because he's been sounding so healthy. There is almost no cough anymore and his noisy breathing is essentially gone. The worst part about having a culture taken on the 19th is that we will get any bad results on the 23rd. I don't want to have to scramble the day before Christmas Eve to get him on whatever medication he might need. I don't want to have to have a schedule on Christmas weekend to administer antibiotics. I just want to have a stress-free, healthy holiday. Is that to much to ask?

I have to voice a complaint about one thing while I'm going. In the news this week, the FDA approved an over the counter drug that claims to cure a hangover. Perhaps a few years ago I would have thought that this was a great thing. However, when I see things like this in the news and think about how the drug that could save Drew's life is also at the FDA and just waiting in line for approval, it makes me furious that we are waiting behind drugs like these. I know that everyone has their cause and their issue that they're dealing with, but in my mind the system is seriously flawed when drugs that "cure a hangover" are placed in the same waiting line with drugs that "cure a genetic disease". I'm sorry, but people who take things to grow their eyelashes thicker or get Botox injections to "cure" a wrinkle have no clue what a real problem is. To spend hours every day doing breathing treatments to "hopefully" keep oneself from getting sick with irreversible lung damage or to take dozens of medications because your body doesn't produce the enzymes it needs to break down food, those are real problems. I wish the biggest problem we had was dealing with a self-induced hangover. So that's my beef. How can these things even be on the same playing field?! We need a new system because our system is currently broken. And i'll shout it from the rooftops, letting any and all decision makers know how I feel about it. One person can make a difference, and I'll prove it.

Finally, if you're in the market for Christmas movies this holiday season, consider buying them from any CVS, where $5 from every DVD sale will go to the Cystic Fibrosis Foundation.

Cheers!

Tuesday, December 6, 2011

Touring the CF Facilities at Cincinnati Childrens

Dr. Clancy is one of the top CF researchers in the country, and also a practicing pulmonologist at Cincinnati Children's CF Clinic. He's been a part some of the biggest new drug studies, including the one for the drug VX-770 which is currently at the FDA awaiting approval for use in patients. If you hadn't seen my post on huge this drug is to the CF world, click here. It essentially corrects the basic defect of CF in patients carrying a certain CF mutation. Its a remarkable medical breakthrough for the entire genetic disease community, not just CF.

So back to Dr. Clancy. He had arranged with the CF Foundation a tour of the Clinic and inpatient care facilities, as well as the CF research center. It was open to anyone - patients and families, large donors, people who were just interested in learning more about CF. Today was the tour and I had the honor of attending. It started with about an hour long chat with Dr. Clancy telling us all about the great stuff that's currently going on in the research world, including current studies going on and drugs that are coming down the pipeline. Never has anyone involved with any genetic disease ever been so close to finding the cure as the CF researchers are to curing Cystic Fibrosis. Dr. Clancy talked a lot about the different drugs that help any number of the 1800 different mutations. With so many different mutations, they are grouped into 6 classes based on their defect and how they affect the person. The drug that the FDA currently has, VX-770 or Kalydeco, was created and essentially corrects the problem called by 1 specific mutation called G551D. I asked Dr. Clancy today if that drug was designed to just help that specific mutation, or if it would be beneficial to anyone who had a mutation in the same class as the mutation that G551D falls into. He said that there has been some testing done recently on the other mutations within Class 3 (that's the class that G551D falls into) and the drug seemed to have the same positive affect on the other mutations tested. And one of Drew's mutation, R560T is a class 3 mutation! We won't know for sure until the drug is available to try it if it will help him. The tricky thing with CF is that with two mutations, both falling into different classes, its hard to say if fixing just one of them will correct your problem. In some people it certainly seems to, but in others they improve but not enough to be symptom free. The good news is that the drug that would correct Drew's other mutation, F508del, is in the works (VX809 - you can read about it by clicking on VX809 and then finding it in the pipeline (near the top) and clicking on the hyperlink). Hopefully within the next 3-5 years, this drug will also improve and make it to the FDA for approval and then use in patients. Taking these two drugs together have the potenial to "cure" CF for Drew and thousands of other people. Obviously I'm hoping that the 770 does the trick because that will be available, with any luck, in the next year. The longer we go without a cure, the more risk of irreversible lung damage we have. Of course we will take a cure whenever we can get one, but the sooner the better.

So the tour and the talk today were fabulous and left me feeling so good and hopeful. I could listen to Dr. Clancy talk for hours on end. If you ever get the chance to hear him speak I highly recommend taking it. He's a brilliant man who has his hand in so many different things, and yet he can still relate to you on a very easy to understand level. He's a really nice guy and I think he's a big part of the cure.

I know this post seemed to ramble on and on. I couldn't contain my excitement and now anticipation of the approval of this new drug. I know I am constantly harping on how important funding is, but without donations and support of our wonderful family and friends, these drugs wouldn't be what they are today. In this season of giving, the CF Foundation would so greatly appreciate your support through a donation. And so would I. We're going to find a cure for this disease and soon, I just know it!


Monday, December 5, 2011

A lingering junky nose

The lack of posting on here has been both due in part to a busy busy schedule and a lack of Cystic Fibrosis related issues going on in our life. For us to not have any health issues to discuss is pretty huge. Our next clinic visit is coming up on the 19th, and for the first time ever, we haven't had to call or visit the clinic for any health concerns since our last visit, over 2 months ago! That's HUGE!
I'm a little worried that they may have forgotten who we are, given that for the past 20 months I've spent a significant amount of time on the phone and in the office talking with a doctor or nurse or someone about something concerning to us on a weekly, if not sometimes daily, basis. I am happy though! I'm not sure if Drew is finally outgrowing the malacia and that's allowing him to clear his secretions better, or if the hypertonic saline that he now gets twice a day is really helping to replace that lost surface liquid and allowing him to function like someone without CF, or a combination of the two. All I know is that I'm please with the way things have been going and hope that they continue that way.
As I type this, Drew is screaming bloody murder at my feet because I won't let him play with his inhaler. There are certainly worse things in the world. He's had a funky nose for a couple of weeks now. He usually wakes up with it being all crusty, and then throughout the day its a combination of running down his face and crusted shut. The crazy thing is that it hasn't moved down to his chest at all. I'm a little concerned about the nose given the amount of time its been going on, but I try to think of him like the I do with the other ones, and if they just have a nose issue I'm not running them to the doctor. I'll let it go another couple of days before I call someone to see if we need to take action.
Tomorrow is the day I get to tour the CF Research facility at Cincinnati Childrens. I was supposed to go last week but the back injury prevented me from attending. Its 2 hours long, and even if they just tell me stuff I already know, I'm always interested in hearing the information they have. I'll definitely post about that shortly after it happens!
Here's to continued good health!

Monday, November 28, 2011

The posts on here have been few and far between. I figured I'd try to squeeze in a quick one before I drift off to dreamland assisted by some muscle relaxers. I threw out my back on Friday and haven't been able to get out of bed since. I went to the hospital and had an MRI and luckily there wasn't any major break or tear or anything like that. I either pulled a muscle real good or pinched a nerve, but man does it hurt something fierce. Quite unfortunately, tomorrow is the day I was scheduled to tour the CF Research facilities at Cincinnati Children's. Luckily, there is another date that I can do it. I would have been royally bummed to have missed that opportunity.

I haven't had much to write about lately. Health has been good, and I put a lot of that on the second hypertonic we are giving Drew each day. He's gotten a few colds and gotten over them without the help of antibiotics. Lately when he has sounded junky in his head it hasn't moved down to set up in his chest, so that's been pretty awesome. Maybe it's some immunity built up from last year or maybe it really is the hypertonic helping to keep that mucus thin and slippery the way it's supposed to be. We have an appointment at the clinic on Dec. 12. I haven't had to call the doc one time (knock on wood) since his last appointment, which up until this point has been unheard of. This is also the longest that he's ever gone without antibiotics. I couldn't be happier with his current health, and hope it continues this way!

No news with Quatro. I was going to talk to my OB about the DHA and GHS supplements that I was considering taking after having talked with Dr. Clancy but now this back problem has taken priority so we will wait until that problem is solved before we move on to the next thing. I still haven't really made up my mind on whether I want to bother taking them or not.

That's about all I've got for now! Hopefully I'll have more to share about good health and other positives soon.

Wednesday, November 23, 2011

Be Thankful

Be thankful that you don't already have everything you desire.
If you did, what would there be to look forward to?
Be thankful when you don't know something,
for it gives you the opportunity to learn.

Be thankful for the difficult times. During those times you grow.
Be thankful for your limitations,
because they give you opportunities for improvement.
Be thankful for each new challenge,
because it will build your strength and character.

Be thankful for your mistakes. They will teach you valuable lessons.
Be thankful when you're tired and weary,
because it means you've made a difference.

I'm thankful for all that has happened to us this year. I'm thankful for good friends and family, good health, great opportunities and countless other blessings. I hope that everyone can enjoy their Thanksgiving Day by being thankful for what they have and giving what they can.

Saturday, November 19, 2011

Celebrity Waiters 2011

This past Thursday I had the honor of attending the Celebrity Waiters Fundraiser benefiting the Cystic Fibrosis Foundation. I had agreed to volunteer at the event...before I even knew that the Lachey brothers would be attending! I must say that having Nick & Drew Lachey at the fundraiser was the hit of the event! It was the 25th anniversary of the event and was moved this year from a luncheon to a dinner gala. There were about 250 guests there to honor the woman who started the event 25 years ago and to raise money for a good cause. The folks who attend the event are mostly local business owners or otherwise independently wealthy folks who have some money to throw around. I wish more CF families could attend, but honestly we couldn't even afford to go. I guess it doesn't really matter whose there, as long as they're giving their money to the CFF!

The night started with some speeches and then turned to the live auction. This is where Nick Lachey made a BIG impact. He made some big bids on some not so big items. He also agreed to golf with the Bengals head coach and two guests of your choice, and if the bidding hit $2000 he agreed to double it if someone would match the bid. And they did! I think that close to $20,000 was raised during the live auction alone. The silent auction and the balloon auction were also both hugely successful. I can't wait to hear how much was actually raised all told, but I know it was significant.

I had the chance to talk to both Drew and Nick and tell them about my Drew. I thanked them for being at the event and let them know how much I appreciated their support for this cause. Here are a couple of my favorite pictures from the night:


There were a handful of doctors and other caregivers from the CF Clinic at the event as well. Its always amazing to see the effort that they put forth to support their patients and their families far beyond the doctors office. I spoke for a while with Drew's old doctor. She was talking about how they had been discussing getting a few parents on board with them as part of a process improvement team. I LOVE the idea and want nothing more than to be a part of it. It sounds like something that they will be trying to get kicked off at the beginning of the new year. My philosophy on the whole world of CF has always remained the same - the more I know, the better off we are. Any doctor who knows my name or any advocate who gets to hear my message or any parent or friend or whoever shares information with me or I with them, the more I know and am known, the better off we all are.

I'm participating in tour of the Cincinnati Children's CF Research Facilities led by the one and only Dr. Clancy on the 29th and I couldn't be more excited. I can't wait to see firsthand whats going on behind the scenes and hear from one of the top CF researchers in the country where we are and where we're going. I will definitely be blogging about that after it happens.

Some of my husbands family will be coming to town this week to join us for Thanksgiving. Its nice to be able to stay at home and have family around to celebrate with. I'm looking forward to a relaxing week with lots of food and maybe a little shopping. I hope everyone has a wonderful Thanksgiving and I'll be back soon!

Saturday, November 12, 2011

A lot of info

We have had a FULL schedule the past week and a half and things finally seem to be slowing down. Between doctors appointments, meetings, play dates and other commitments, we haven't had a minute of downtime.

This past Thursday was the kickoff for Cincinnati's Finest Young Professionals, a fundraiser for the Cystic Fibrosis Foundation. Two of my friends were nominated this year and I'm excited to work with them to help raise money for the CFF. At that event, I had the opportunity to talk with Dr. Clancy for a few minutes! I think that I had posted a couple of weeks ago about how I had asked Drew's doctor if she had any knowledge of taking DHA and GHS while pregnant with as a CF carrier to prevent meconium ileus. Her response was that no, she hadn't really heard of it, though if there weren't really any risks involved, while she hadn't known of a proven benefit, it couldn't hurt. So I checked with my OB, and her response was similar. To actually have human data to look at, there would have to be a sample of people who knew that they were pregnant with a CF baby which, in itself, is rare. Then, since meconium ileus only happens seemingly randomly in babies who have a mutation that carries pancreatic insufficiency, it would really be hard to isolate how truly effective taking those medications are in preventing meconium ileus since you couldn't be sure the kid was going to be born with it in the first place. That said, my OB said that she didn't really see any harm in taking those medications but suggested that I also check with Dr. Clancy to see if he had any knowledge of research done on these medications for the purposes I was asking about.

So Thursday night came and I had the chance to talk to Dr. Clancy. He said that there were actually some current studies being done of supplementing newborns with DHA and GSH and how they help them since CF kids produce insufficient amounts of these on their own. He also said that he didn't see any real harm in taking them while pregnant, as long as it was in a reasonable dose. It would be unclear how much would actually get passed along to the baby, who may or may not have CF (obviously we're hoping not) and also unclear would be if the baby were born with CF and without meconium ileus if it would be because of my having taking those medications. That said, he said that if it would help me sleep at night thinking that I was doing something proactive and there was no risk to the baby either way, he didn't see anything wrong with it. So I may. I haven't decided yet.

The North American CF Conference was last week and I so so wish that I had been able to attend. Some of the materials have been made available online (CLICK HERE). There's a really interesting one on Animal Models in CF, and if you read through it and see some of the Pig Model (which has only been around for 3 or 4 years), there are crazy similarities between the symptoms of the disease that they display and the ones that Drew has. In talking with Dr. Clancy on Thursday, he said that hes sorry for all that Drew has had to go through the past 18mo, but that they have learned a TON through him. Prior to Drew, the Cincinnati CF clinic hadn't seen airway abnormalities and tracheomalacia in CF kids like Drew had. They had assumed that they two were unrelated and that he was just unlucky in having smaller, disfigured airways on top of his challenge to clear secretions. But with the research that has come on on the pig model in the past year or two and seeing the airway abnormalities of the pigs, it would seem that its more than a coincidence but rather a clinical manifestation for the mutations that Drew has.

So with the knowledge that his mutations seem to have caused most of his problems, I was doing a little more research on the mutations that he has. He's got f508del and r560t. Delta F 508 is the most common CF mutation with over 70% of the CF population carrying at least one copy of this mutation. This mutation falls into Class 2 of CF mutations (see HERE for more on mutation classes). His other mutation, r560t, falls into Class 3 (stay with me, I have a point). You may have read recently some of the news for the new drug called VX-770 or Kalydeco that just went to the FDA for approval. It treats one of the basic defects of CF....namely, the defect of Class 3 mutations! It wasn't specifically tested on his mutation but rather a mutation called G551D. But that mutation falls into Class 3. Here is some what was written on the CFF website about VX-770:

"...Some mutations result in CFTR proteins at the cell surface that do not function properly. They function like a locked door. VX-770 unlocks the door and allows these proteins to function correctly, improving the flow of salt and fluids in and out of the cell.

VX-770 is designed to increase the function of the defective protein at the cell surface in people with the G551D mutation. The defective protein functions like a locked door.

VX-770 helps unlock the door at the cell surface, enabling chloride to pass through, as it would in healthy cells. This restores a proper flow of salt and fluids on the surface of the lung. For these reasons, people carrying the G551D mutation appear to have a strong potential to benefit from VX-770.

It is possible that the results of the Phase 3 trials will provide a basis for the use of VX-770 to treat other similar mutations...."

So that gives me some hope (okay, a lot of hope) that this new drug, which will hopefully be made available in 2012, will remove the symptoms of CF in Drew and for everyone with Class 3 mutations and not just those with the G551D mutation. And if it doesn't, there is another drug that targets delta F508 that should be available in the next few years. I also found this posted on a CF Forum website: "The thing that gives me the most hope for Class III mutations is this quote from an abstract of a presentation made by Vertex, where they tested VX-770 in-vitro (in a test-tube): Class III mutations
demonstrated the largest VX-770 response, with all 8 class III mutations tested (e.g.,G178R, G551D, G551S, G1349D) showing a >10-fold increase in Cl? transport compared to baseline". So I guess we'll just have to wait and see what happens!

The other thing that I've been quite happy about recently (knock on wood) is that Drew got his first cold that he got over on his own without antibiotics! I'm not sure if its his prior year exposure that has his immunity built up a little or his stronger airways now that hes a little older allowing him to cough things out a little better or what, but I'm happy about it. We've been on hypertonic saline for a while now, but we'd only been using it once a day. I had read some research suggesting its benefits to be more profound if it was administered twice a day. However, it can't be given at the same time as Pulmozyme, so that allowed us to use it with his morning treatment but not his evening treatment when we give him his Pulmozyme. But I decided that we would see if it really helped him, so I added in a third treatment during the day, right after he wakes up from his nap. And wouldn't you know that having been observing this now for probably a good two months, there is little to no coughing, colds seem to be a runny nose that lasts for a few days and then disappears, and overall he just seems better. So we will continue with the hypertonic twice a day...until that little blue pill becomes available to us and we "cure" him! Fingers crossed people!

Friday, November 4, 2011

Something great that you have to see

I so wish I was there

The North American CF Conference started yesterday and I'm so bummed that I can't be there this year. I'm already working on plans to be there next year. I wanted to share an article and video that the CF Foundation posted on their website about the conference. There is so much hope.

First Day of North American CF Conference Features Breakthroughs in Research and Inspiration from People with CF\

November 4, 2011

The North American Cystic Fibrosis Conference (NACFC) kicked off its 25th anniversary on Nov. 3 with exciting reports from the CF drug development front and powerful messages of hope from people with CF and their families.

Nearly 4,000 medical professionals, researchers and caregivers from around the world convened in Anaheim, Calif., to discuss the latest results of CF research studies and exchange ideas about ways to improve the quality of life for people with CF.

In his opening remarks at the conference’s first plenary session, Robert J. Beall, Ph.D., president and CEO of the Cystic Fibrosis Foundation, described the remarkable progress of potential drugs targeting the underlying cause of CF.

“We are on the cusp of the greatest breakthrough in the history of CF,” he said, “and it would not have been possible without teamwork — from around the world and across disciplines.”

In a video presentation, Lauren Brenneman, 33 — an adult with CF and mother of 4-year-old Isaac, who also has CF — shared her inspiring story of participating in a clinical study of VX-770, a potential therapy under review by the U.S. Food and Drug Administration to treat the basic defect in some people with CF.

Marcus A. Mall, M.D., of the University of Heidelberg, Germany, capped off the first day with a stimulating overview of milestones in understanding and treating the underlying cause of CF since the first NACFC 25 years ago and the lessons that can be applied to development of the “next generation” of therapies and used as a model for other rare diseases.

Watch Lauren Brenneman and her son Isaac's video presentation


Thursday, November 3, 2011

About preventing a bowel blockage

So, as we all know by now, being a CF carrier married to another CF carrier gives any of our offspring a 25% chance of having CF. That means a 75% chance that they won't. We had discussed this all at length before deciding to have another kid after Drew's diagnosis, and also decided that we wouldn't be finding out before birth if the baby does or does not have CF. There is more risk than benefit involved with finding out, as there is nothing that can be done for the kid ahead of time.

Having had Drew born with meconium ileus, I had some questions for both his doctor and mine about the likelihood of CF manifesting itself in that form again. I learned that about 85% of people with CF are pancreatic insufficient (which is why Drew takes enzymes every time he eats to help him digest the food and hold onto the fat) and that of that 85% about 20% are born with an intestinal blockage. Having had 1 baby with meconium ileus doesn't make me any more or less likely to have it happen again, but with the two mutations that any of our children would have if that had CF, they would be pancreatic insufficient and therefore put them into the 20% that could be born with a blockage.

I had asked Drew's doctor if she knew anything about stuff a mother could do to prevent such a blockage from happenings, such as taking a laxative or stool softener. The answer was no, because drugs like those don't enter your blood stream, they just go right to your gut. If it doesn't enter your bloodstream, it doesn't get passed on to baby through the placenta, therefore rendering it useless to baby.

So I did a little research online and I found an interesting article:

If You are a Carrier Pregnant with a CF Child

.... Either way, a woman pregnant with a fetus they either know or suspect has CF will wonder if there is anything they can do to lessen the chance that the baby will suffer complications in utero.

Until very recently, the answer was no. All that could be suggested was ultrasounds in the third trimester to look for signs of incipient meconium ileus (severe bowel blockage) that might necessitate emergency Caesarean delivery and immediate surgery for the newborn. However, some very recent research argues that supplementation with two nutritional supplements - DHA and GSH - may lessen or even prevent the manifestations of the disease that begin even in utero.

DHA is docosahexaenoic acid. Recent research has shown that CF persons suffer from a 3-fold decrease in the amount of this lipid present in the cell membrane. (Please see section on DHA for more information and scientific references.) The researchers argue that this lipid imbalance plays a role in the development of inspissated plugs in the pancreas and other organs - which plugging begins in utero. The researchers suggest that supplementation of the mother with DHA will tend to rectify the lipid imbalance in her fetus, thus preventing the development of such plugs even in utero. Their theory suggests that the chance of the fetus developing potentially life-threatening meconium ileus can be drastically reduced if the mother's diet is supplemented with DHA. (It is important that the supplement taken contain only DHA, and not any other fatty acids such as EPA or linoleic acid, which compete with DHA for placement in the cell membrane.)

GSH is reduced glutathione. GSH is the most important antioxidant in the body for neturalizing water-soluble oxidants. It is also a powerful mucolytic. For our purposes here, it is glutathione's third property as an important regulator of inflammation that we will discuss in this context. Researchers have shown that the chronic and excessive inflammation that characterizes CF begins in utero. This inflammatory state directly damages the tissues of the body, which in turn primes the body for bacterial colonization as well as eventual immunodeficiency. If one could lessen or even shut off the very start of that inflammation which begins in utero, the CF infant should have a better start in life. In 1998, researchers first noticed that the CFTR channel, which channel is missing or defective in CF persons, is the main efflux route of cellularly-produced GSH (see section on Glutathione for more information and scientific references). This is a very important finding, as the redox state of GSH in immune system cells is the primary trigger of inflammation in the body. If GSH becomes depleted in immune system cells, inflammation begins. This is precisely what begins to happen in the CF body, and this is what is hypothesized to be happening in utero. This theory suggests that supplementation of the mother with GSH may tend to lessen any GSH deficit that may start to develop in the immune system cells of her fetus. This should serve to lessen or even shut off the origin of fetal inflammation.

Thus, in addition to all of the usual vitamins and minerals a pregnant woman is asked to take, a woman who is pregnant with a fetus that she knows or suspects to have CF might also consider supplementation with both DHA and GSH. DHA and GSH are both nutritional supplements available in health food stores without any prescription. And, given that the woman herself is a CF carrier, and therefore suffering to a lesser degree from the CF mutation, the additional DHA and GSH might do her own body good as well! (Please also see our link on the importance of a postpartum Vitamin K shot for a CF newborn.)

One last piece of research will also be useful to the woman: pregnant CF carriers tend to develop gestational diabetes during pregnancy. Alert your obstetrician or midwife to that fact, so that precautions can be taken. You may need to avoid certain types of food, such as sucrose, during your pregnancy. You may also gain more weight than the average pregnant woman because of the gestational diabetes. One good thing is that unlike non-CF carriers that develop gestational diabetes, you are unlikely to develop potentially dangerous hypertension. The reason for this is that CF carriers, like CF persons, tend to have lower-than-average blood pressure to begin with. This is one of the few blessings of having a CF mutation! However, you cannot count on this as a certainty. We have heard from one mother, a carrier of the delF508 mutation, who did have high blood pressure while pregnant. Another observation has been that the placentas created by the bodies of CF carriers tend to become quite calcified - almost to the same extent as if the mother was a heavy smoker. It is theorized that this has to do with the impaired absorption of minerals by the CF carrier's body. This may also be important for your doctor or midwife to understand.

I sent it to our doctor at the CF Clinic and this is what she had to say:
I have seen a review article that describes the fatty acid alterations in blood and tissue of CF patients. There have been studies looking at supplementation in CF patients which show an increase in the fatty acid composition but no change in clinical outcomes. They conclude that larger studies are needed and more clinical outcomes need to be evaluated to see what impact supplementation may have. I have not seen anything about fatty acid alterations in CF carriers or supplementation during pregnancy. However, it seems as though DHA and GSH supplementation would not cause any harm and therefore may be worth a try. You might want to talk to your OB.
So I emailed my OB today and asked her to take a look into this and see if there are any risks associated with taking DHA and GSH while pregnant and when I have my appointment next week, we can discuss and perhaps I can start taking these supplements. They very well may be all for naught, as there is a 75% chance that this baby will not have CF. But if we got unlucky and potentially help to removed the chance of a blockage, i'll do whatever necessary.

In my searching, I found this interesting website. It had a few things on it that I found interesting to read about, like the pseudomonas vaccine and some other interesting therapies. I thought I'd share the link. It is in no way associated with the CF Foundation, but interesting research nonetheless. http://cystic-l.org/handbook/

Sunday, October 30, 2011

Happy Halloween

I know Halloween isn't until tomorrow, but I had a few minutes to sit down and share some photos from Ella's Halloween party at school. Ella is dressed up as Jessie the Cowgirl from Toystory 2 and the twins are garden gnomes. I seriously couldn't stop laughing at how ridiculous (and cute) they all looked in their costumes.




We were all set for an uneventful, relaxing weekend when Lily decided Saturday morning to drink half a small bottle of hand sanitizer. I immediately called poison control who said she needed to be taken to the hospital to be monitored, since its 62% alcohol. I was also advised that she might start to stumble, slur her speech and potentially pass out (none of which ever happened, thankfully). Martin took her to the ER where they drew blood and kept an eye on her for a few hours. Her blood alcohol level came back normal so either she didn't actually eat as much as we thought she had, or she's been up to this before and has built up a tolerance (kidding!). Very luckily, she was fine and we were able to carry on with our weekend.

In other very good and exciting news, Drew's latest culture came back NEGATIVE for pseudomonas! Hopefully it remains that way for a while. He did have some Haemophilus but they are going to treat that unless he shows symptoms of being sick, which he is not. Hopefully he can cough that out on his own without antibiotics.

Tomorrow night we will all bundle up and brave the cold for some trick or treating. I don't suspect it will last long but hopefully everyone will enjoy themselves for the short time we spend outside!

Thursday, October 27, 2011

a little bird told me...


Life is a trip with a perfect itinerary that we cannot see. Imagine it this way -- that there really IS an itinerary, but you just can't see it. There is an amazing destination, but you just don't know where the stops along the way will happen where you will have to refuel, where your best memories and your greatest lessons will happen.

We don't know that what may seem like useless detours are where we might meet the most amazing people of our lives, or enjoy the best scenery, or learn the most important things.

Sometimes we fail to enjoy the trip because we are so hung up on wanting to see the itinerary. You will have the best times of your life when you just TRUST that you are exactly where you are supposed to be, doing exactly what you are meant to be doing, and that tomorrow you will be lead to the next destination.

You always have been, you always will be.

Remember to travel light, only take what you need with you. It will make things so much easier.

ENJOY YOUR FLIGHT, little birdie!

Wednesday, October 26, 2011

Clinic Visit

Not much going on around here these days. The weather has been really nice and we've been spending tons of time outside. Its supposed to get cold now, just in time for Halloween. I'll definitely have to post some pictures after that because the kids costumes are CUTE!

We had a clinic visit on Monday and are hoping that we get a good culture back later this week. Hopefully our pseudomonas is gone again and will stay gone this time. Drew's overall health looks good. I got to see his CT scan that the doctor still says shes very happily shocked about. Everyone kind of just expected things to not look good with all the trouble that he had over the past year, but alas, it looked just fine. They said that sometimes in younger kids there is some evidence of bronchiectasis in the upper lobes but he doesn't seem to have any signs of that whatsoever. There wasn't any signs of lung damage and everything looks just the way its supposed to. I'd like to keep it that way.

Drew is currently on a hunger strike. I haven't figured out what he's trying to prove, but the boy won't eat. Ok let me correct that - he won't eat anything but fruit and an occasional corndog. The boy who use to love peanut butter now steers clear of it. He won't eat chicken nuggets, corn dogs, hamburgers, pasta, nothing. His weight isn't down (thank God) but i'm afraid we're headed there. I hear that toddlers go through these stages. Its just a little more challenging for a toddler who needs fat to thrive. We might be turning to smoothies soon.

I'll post an update when we get Drew's culture back, probably on Friday. Until then, I hope that our lives remain uneventful in a good way!

Friday, October 21, 2011

What If

A message from the Public Policy Department of the CFF



What if many people with cystic fibrosis lost the ability to go to their care center? What if they could no longer afford their treatments? We need your help to make sure that the thousands of people with CF who rely on Medicaid never have to answer these questions.

Twelve members of Congress could limit the ability of many in the Medicaid program to access their care. Your voice can help ensure that doesn’t happen.

Many families in the CF community would struggle to afford care without Medicaid protections. Speak out now!

This summer, Congress created a new deficit reduction “supercommittee.” Its 12 members were given broad powers to change the Medicaid program.

Medicaid provides health coverage for children and adults with CF who cannot afford other types of insurance. It is often the last resort to make sure they are able go to a CF care center, see a doctor with expertise in CF and afford their inhaled antibiotics, nebulizers, enzymes and other treatments.

Supercommittee members need to cut the country’s budget deficit, but it’s up to us to make sure they protect access to the specialized care people with CF need to stay healthy.

Help spread the word:

Are you on Twitter?
Please retweet the CF Foundation’s messages in the coming weeks to each member of the supercommittee.

Not on Twitter? No problem!
Send a message to all 12 members of the supercommittee now.

Thank you for all that you do! Together, we are making a difference in the lives of people with CF.

Wednesday, October 19, 2011

Great news for the CF Comminuty

Vertex Submits Application to FDA for Approval of VX-770 – First Potential Drug to Target Underlying Cause of Cystic Fibrosis

October 19, 2011

Vertex Pharmaceuticals, Inc., announced today it has submitted an application to the U.S. Food and Drug Administration for a potential new CF therapy, VX-770 — under its new proposed trade name, KALYDECO™.

If approved, it will be the first drug on the market that targets the underlying cause of cystic fibrosis. Therapies available to people with CF to date only treat symptoms of the disease.

The company is seeking approval for the drug in people with cystic fibrosis age 6 and older who carry at least one copy of the G551D mutation of cystic fibrosis.

KALYDECO (kuh-LYE-deh-koh) was discovered in a collaboration between Vertex and the Cystic Fibrosis Foundation, which provided substantial scientific, financial and clinical support throughout the development process.

“The CF Foundation is thrilled that KALYDECO is on track for possible FDA approval in 2012,” said Robert J. Beall, Ph.D., President and CEO of the CF Foundation. “This is a significant step forward in our collaboration with Vertex and is further validation of the CF Foundation’s drug development strategy. We remain committed to accelerating the development of similar targeted medicines that will benefit all people with cystic fibrosis.”

Vertex has asked the FDA for priority review of the potential drug, which, if granted, could shorten the review from 10 to 6 months. The FDA grants priority review status for several reasons, including in situations where a potential drug is considered a major treatment advance.

Results released earlier this year from Phase 3 clinical trials of KALYDECO in people with the G551D mutation of CF showed that those receiving the drug had remarkable and sustained improvements in lung function and other key symptoms of the disease, compared with those on placebo.

As FDA review of the potential drug gets underway, Vertex has set up a program to provide KALYDECO to people age 6 and older with the G551D mutation who are in critical medical need and could benefit from the treatment prior to potential approval.

The expanded access program is designed for people with CF who have highly limited lung function and meet other criteria. (Information about the program is available at CF Foundation-accredited care centers.)

KALYDECO is currently being evaluated in combination with another oral drug in development, VX-809, in people with the most common mutation of CF, Delta F508.

Vertex plans to begin the second part of the Phase 2 KALYDECO and VX-809 clinical trial this month and will evaluate the two drugs over a longer period of time.

Additional Resources

Tuesday, October 18, 2011

Clarity

After I slept on it, I had some more thoughts on last nights post. First, our situation is not the same as the woman who wrote that article. My heart breaks for her. While I have shared many of her feelings and emotions, we do have hope. We have treatments and therapies that will hopefully prolong Drew's life. We have a future planned. We have plans and ideas and goals, and hopefully will be able to fulfill them all. There is no cure for CF, but there is hope. I hope that Drew lives to be 95 before it "gets him". Its scary and its hard some days, but I try to focus on the positive. We do try to cherish every moment and every memory, but I did that before I ever had Drew too. Our house is a mess. There are usually dishes on the counter and mounds of laundry all over the house. There are also usually 3 kids running around, sometimes naked, squealing with delight and making a giant mess. I try not to let the little, insignificant stuff bother me.

I feel like sometimes people are a little afraid of me. I've had people comment that they shouldn't even talk about their crazy day because its probably nothing compared to mine. We all have our own crazy. People either don't want to talk about CF because they don't understand, or because they're afraid they have to hop on an emotional roller coaster named Erin if they bring it up on the wrong day. I don't always want to talk about it. I deal with it every day, some days better than others. Some days I don't mind when people ask me questions about it and about things. So few people really understand and I get frustrated because I feel like they haven't taken the time to understand. Other friends have distanced themselves. They have no clue what goes on. I don't know why they've left us, but they have and I've become okay with it. I use to get mad when people didn't "get it", but with time I've come to realize that its not their life to get, its mine. As friends, I thought that they would share this with us, but whether they are scared or confused or simply don't understand our situation, they have chosen distance as their mechanism, and I'm okay with that. I've got who I need in my life.

I think that mostly I've settled into our life the way that it is. I only get worked up when I hear a sad story or hear bad news or just have a bad day...just like the rest of us. I do have lots and lots of hope, but it doesn't mean I'm not scared sometimes. I'm not naive to what this disease can and will do to a person, but I'm optimistic. The perspective that the woman who wrote "Notes From a Dragon Mom" provided in her article was emotional, eye opening and inspiring. This is what I felt I shared with her, as the mom of a kid with a disease with no cure:
"Our goals are simple and terrible: to help our children live with minimal discomfort and maximum dignity. We will not launch our children into a bright and promising future, but see them into early graves. We will prepare to lose them and then, impossibly, to live on after that gutting loss. This requires a new ferocity, a new way of thinking, a new animal. We are dragon parents: fierce and loyal and loving as hell. Our experiences have taught us how to parent for the here and now, for the sake of parenting, for the humanity implicit in the act itself, though this runs counter to traditional wisdom and advice."

Different from the author, I have not abandoned the future. I am hopeful for Drew. I'm just a mom who is truly, madly and deeply in love with my son who fights a disease for which there is no cure every day. But I'm hopeful. That's why I fight for him. I'm hopeful.

Monday, October 17, 2011

A sad story

I wanted to share an article that a friend recently posted on Facebook. While I read it, sobbing, I could relate. It's sad - consider yourself warned. I have felt many of the same feelings and emotions. I hope and pray everyday that they find a cure for Drew's terminal illness.

Click here to read Notes From a Dragon Mom, and say a prayer for her tonight. And one for Drew too if you have one to spare.

My problem child

Its been a long time since I've been on the blog. Mostly because I've been otherwise occupied or sleeping. The other reason is that health here has been good, so no drama to blog about other than Lily trying my patience these days. We had to call Poison Control when she got a hold of a tube of toothpaste and consumed an undetermined amount. She plays in the toilet regularly, and when she gets caught she has usually unrolled half a roll of toilet paper while she's at it. We bought cream colored couches long before kids were even a twinkle in our eye. To our surprise, they had remained pretty clean...until Lily discovered she can open the drawer where the crayons are. There is pen and crayon all over many different parts of the house. I like to think it adds to the eclectic feel of the house, but who am I kidding. Its really a problem that she has got the most beautiful blue eyes, and a smile and hug that will melt your heart, because she is just so so bad. You may think I sit around with my feet up allowing her to do all of this, but truth be told, when the kids outnumber the caregivers, its hard to keep tabs on everyone at every moment. Things should get even more interesting in March when Quatro arrives. But man, she's really freakin' cute, and I just love her to pieces.


There are a couple of big CF fundraisers coming up, and we're looking for auction items. If anyone who reads this provides some type of service (photography for example) or makes something (art, clothing, toys) and wants to donate something for any of these events, we'd gladly accept. Gift certificates are always awesome, or other fun products from small companies always make great gifts too (Tupperware, Pampered Chef, Stella & Dot, Tastefully Simple, etc). Shoot me an email if you have something to offer. We need things no later than Nov.1 to get into the Celebrity Waiters fundraiser. Anything received after that can be used for other fundraising events.

If you're in Cincinnati and care to attend Run Like Hell on Oct. 28th or Celebrity waiters on Nov.17, CLICK HERE for more info.

Drew has a clinic visit next week, so we'll have more to write about on his health. I'll hopefully get to see his wonderful CT scan, and I'm sure we'll discuss what to do if he needs inhaled antibiotics again since he doesn't seem to tolerate them. We'll also get a culture. Please cross your fingers that the pseudomonas is gone [and that it never comes back]. I'm off to spend some time outdoors in this fabulous fall weather!

Wednesday, October 12, 2011

What an honor

We spent the end of last week and last weekend in PA for a visit with my family and a good friend's wedding. I have spent the last 3 days trying to catch up from our trip. but it seems that every time I try to sit down, something else needs to happen. I just wanted to share the sweetest thing that our friends did for us at their wedding.


What a totally awesome thing for our friends to do for us! I will post more about our trip and about our latest goings on with CF and what have you the second I have a chance to. Today's field trip to the pumpkin patch took it out of me and I'm going to bed.

Thursday, October 6, 2011

Advocacy Newsletter

I was asked to write a little something for our local CFF chapter newsletter about some of the advocacy work that I've been doing as the State Advocacy Chair, and I thought I'd share that letter on here as well. Toward the end of the note, there is a very important action item that I have asked all readers to help with, and I'd like to ask the same of you. And if you have a blog or a Facebook page, share the link with your friends and family too. The more people who participate, the more influence we can have. Happy reading!

My name is Erin Moore, and I have an 18mo old son who has Cystic Fibrosis. Several months ago, I volunteered to work as the State Advocacy Chair, helping to raise awareness of the disease and lead the Foundation’s efforts by building relationships with targeted Congressional offices in order to increase support for the Foundation’s policy agenda. I have asked my friends and neighbors to advocate with me, and today I'm asking for your support as well.

A few weeks ago, I had the opportunity to meet with Congresswoman Jean Schmidt to discuss some of the things going on in the Cystic Fibrosis Community and how she can have a positive impact for the many people living with it. First on my list was to ask for her support in opposing cuts to the Food and Drug Administration (FDA) and the National Institute of Health (NIH). The House’s 2012 Agriculture, Food and Drug Administration Appropriations bill cuts funding for the human drugs section of the FDA by $61 million. Cuts in funding for drug evaluation in the FDA could mean fewer, more overburdened reviewers, slowing the review process. There are 8 promising CF treatments heading to the FDA for review in the next couple of years, and those with cystic fibrosis and other rare diseases can’t afford to wait for urgently needed new medications. The National Institute of Health provides funding for the research that is going on at places like Cincinnati Children's Hospital. Cuts to this funding could mean that this research must be put on hold, and we don't have time to wait.

Another item on the Policy Agenda of the Cystic Fibrosis Foundation that I was able to discuss with Congresswoman Schmidt was the importance of assuring that people with cystic fibrosis receive access to the care and treatment they need to help them live longer and healthier lives. More specifically, I was asking for Congress to help people with CF to have access to high-quality health care that adheres to the current standards recommended by CF treatment and research experts and to protect the ability of cystic fibrosis patients to get the medical care they need by reducing the increasingly prohibitive cost-share burden of this expensive disease.

Congress is currently working on the 2012 budget and many parts of that budget will impact the CF community. Congress has signaled that they are looking to reduce spending. Spending cuts that affect the Food & Drug Administration (FDA), the National Institute of Healthy (NIH) or Medicaid could be harmful to those of us dealing with CF. We need to be the voice that collectively tells Congress why preserving strong funding to these programs is so vital to the health of our community.

WHAT YOU CAN DO TO HELP!
In October, the Joint Select Committee on Deficit Reduction will be responsible for finding $1.5 trillion in savings from the Federal Budget. Even though everything in the budget (including FDA and NIH funding) will be on the table in their discussions, conversations with leaders on Capitol Hill have led us to believe that Medicaid is in the greatest danger of significant changes that could leave thousands of people with CF without vital health coverage. As a result, we think our collective voices will be most effective if we focus exclusively on Medicaid in our messages to this committee.

Contacting the members of this specific committee will be critical over the next several months if we want them to consider the very serious implications that cuts to Medicaid will have to people living with Cystic Fibrosis. Please take just a moment to click on this link to let them know that you oppose these cuts - http://bit.ly/ngfEuP

Additionally, advocacy will continue to be an important part of preserving the quality of life for Cystic Fibrosis patients. Sign up to be an advocate and let your voice be heard in Congress for yourself or your loved ones living with Cystic Fibrosis. It takes no more than a few minutes a month but has an immeasurable impact. Visit http://www.cff.org/GetInvolved/Advocate/ to sign up today.


- Posted using BlogPress from my iPad

Monday, October 3, 2011

And we're off Cayston

That lasted all of 3 days. We switched to Cayston because Drew's doctors felt that having him on inhaled antibiotic would help us to continue the pseudomonas eradication process. Tobi didn't work for us, as it threw him into bronchospasms. Cayston was worse. We almost ended up in the ER today and if I'm being honest, I could have predicted that when we started the medication on Saturday afternoon.

Saturday morning we got a huge package in the mail that included an entirely new and different nebulizer machine, a giant box of medication, a sterilizer machine and, thankfully, a DVD explaining what to do with all of it. When we got it all figured out and got Drew hooked up to it, we thought we were home free as this machine administers the medication in just about 3 minutes. Well, that first 3 minutes was the longest and close to the worst 3 minutes of my life. The mist is so thick and dense that comes out of this machine that Drew was literally acting like he was being suffocated. Martin and I were both holding him and bracing him and he was fighting for his life, thrashing about, turning purple, doing everything in his power to get that mask off of his face. We felt it necessary to try to get the antibiotic into him, especially since a 28 day supply cost just over $6,000. Waste not want not!

The next dose wasn't quite as bad, but we were noticing shortly after giving it to him a tightness in his chest. He was doing a lot of coughing but it was all a very dry cough. His breathing was very labored and noisy. We were using a lot of Atrovent to try to open him up on Saturday and Sunday, and then today it finally all came to a head. He again fought for his little life as we tried to complete this 3 minute therapy this morning. By lunchtime, he had used his inhaler several time (prescribed to be used no more than every 4 hours). I had to do the medication again right after lunch and he simply couldn't handle it. I am not exaggerating when I tell I felt as though he was being suffocated by this medication. He got it off a couple of times and I finally gave up. I put him down for a nap and he cried and coughed and gasped for air for 45 minutes before I gave him a double dose of his Atrovent. I tried again for a nap but he couldn't pull it together. He was beat red, retracting around his ribs, and so unsettled.

I called his doctor and they said to stop the medication. The prescribed a steroid to try to relax some of the inflammation and bring him back to even. They also said that if he was needing Atrovent more than every 4 hours then he needed to be brought into the hospital for observation. My hesitation with going to the hospital is that 1) he ALWAYS picks something up when we are there and 2) if you're not going to do anything different than I'm doing here, why do I need to be there. They "monitor" him. The only thing they would do for him that I'm not is giving him oxygen if necessary. They say that they can administer the Atrovent more frequently if necessary (which I don't know why I can't do at home).

I was worried about him all day. It may seem silly that I didn't take him in, but being in the hospital is no walk in the park. I was close today, but with the help of his doctor and nurse, and my wonderful husband keeping me calm and reasonable, we got him to a comfortable level of of breathing both for him and for me and got him his steroids and got him to sleep. I'm hopeful that without that inhaled antibiotic (without any inhaled antibiotic) for a day or two, his airways will recover from whatever distress they were being caused and he will be back to himself. My fear is how we will deal with things like pseudomonas if it continues to stick around, because its most effectively treated with inhaled antibiotics. I will try not to worry and cross that bridge when we come to it. Maybe he will be older and better able to tolerate them by then. Maybe the pseudomonas will never come back. Maybe they'll find the cure before he ever needs to worry about this again. A girl can dream...