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!

a little bird told me...

A Daily Truth from The Brave Girls Club

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!

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!

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.

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

• Read the Vertex press release.
• Read about KALYDECO in these FAQs.
• Learn about the combination trials in these FAQs.

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.

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!

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.

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

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...