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...

We switched to Cayston

A few weeks ago, I posted about Drew's medication plan for getting rid of his pseudomonas. We completed our two weeks of IV antibiotics and started on our 56 days of inhaled Tobi. In the past, Tobi seemed to irritate his airways, making his breathing kind of noisy and causing him to cough a lot. Since it had been 6 months since we were on it, we decided to give it another try. Today was the 10th day we were on it and I finally decided it was time to call the doctor. He would wake up sounding fine, get his regular breathing treatments and airway clearance, and then get his 30 minutes of Tobi and at the end of that 30 minutes would be coughing so hard he was practically gagging. An Atrovent treatment immediately following the Tobi seemed to help open him up and help breathe a little easier, but throughout the day he just wasn't getting back to a normal level.

The doctor described what was happening as a bronchospasm. "A Bronchospasm or a bronchial spasm is a sudden constriction of the muscles in the walls of the bronchioles or smaller airways. The overactivity of the bronchioles' muscle is a result of exposure to a stimulus which under normal circumstances would cause little or no response. The resulting constriction and inflammation causes a narrowing of the airways and an increase in mucus production; this reduces the amount of oxygen that is available to the individual causing breathlessness, coughing and hypoxia"(courtesy Wikipedia). Sounds about right.

You may wonder why we let this go on for 10 days before calling the doc, and there are a couple of reasons. First, we felt that continuing on the Tobi was important in the process of pseudomonas eradication. Second, there was no real fear that he was going to stop breathing. I've seen him in total distress and this wasn't that. It wasn't easy for him to breathe but it wasn't emergency room worthy. We were able to regain control with an Atrovent treatment (a medication that helps to relax and expand the airways). Third, I had hoped that it wasn't really the Tobi causing the problem. The girls have had little colds and I thought that perhaps he was just getting their cold which usually causing him a little bit more difficulty breathing, but after a few days of observation and being able to isolate the increased difficulty to the times when we were doing the Tobi treatments, I realized that it wasn't going to be something that we could continue on.

Drew's doctor does want us to continue on an inhaled antibiotic so that we can still try for pseudomonas eradication, so our next option is to try Cayston. Apparently there is an entirely different nebulizer system used for administering this medication that I should be receiving in the mail tomorrow. The plus side to this medication is that it only takes a few minutes to administer three times a day (like 2-3 minutes). The Tobi took us over 30 minutes twice a day, on top of everything else we were already doing, so this buys back a little bit of our time. Hopefully he won't have such a strong reaction to Cayston like he did to the Tobi, but I guess time will tell. Cross your fingers!

Lovely day for a blog post

I really love the volunteer work that I do at Children's. It has nothing to do with Cystic Fibrosis, so its a welcome break from something that's a part of our lives day in and day out. I love meeting other families and letting them know that I care about them, that I've sat in the chair they're in and paced the hallways they're walking up and down. I've worried and wondered and cried and prayed just like they are for their sick little babies. I've been in their shoes. And when they smile at me and thank me for stopping by to talk to them even just for a few minutes it makes it all worth my while.

I met a family last night who recently received a CF diagnosis. Its not very common, as CF isn't very common, but I was able to say to them, "I've been there" and mean it in the truest sense. They asked a couple of questions, but, like us, you're kind of at a loss at the beginning, not knowing what to ask or expect or tell people or anything. You really just don't know what you don't know. I shared my email address and this blog and I hope that the family will feel comfortable reaching out to me if they need support.

Last night I watched School House Rocks - I'm Just A Bill so that I could understand a little better how Congress works. I really am not very government literate. I feel like the things I know, I know very well, but outside of that I must turn to my trusty husband who can always break it down for me. It was an intimidating part of taking on the role of State Advocacy Chair. I knew that there would be communications with political figures, and I didn't want to seem like an idiot when I was talking to them. I like to think of myself as reasonably well educated and fairly well spoken, but government and politics aren't my specialty. As long as someone can help to explain it to me and I can take the time to learn and understand myself, I'm comfortable presenting it, and I feel like that has taken me pretty far in the Make Every Breath Count Campaign.

In March, there is an event called March on The Hill where the CF Community joins together in DC to tell our story (at least that's my understanding). Unfortunately this year we won't be able to go with Quatro due to make his/her appearance around March 21, but I'll jump on any other opportunity that I have to go and speak my piece to the people making the decisions that will influence Drew's life.

Speaking of Drew, we are about a week in on our Tobi and Cipro antibiotics and all is going well. As has been the case in the past, the inhaled Tobi seems to irritate his airways a little bit more than they're already irritated which means more coughing and more Atrovent treatments to keep him open. On top of all of that, I think the girls finally passed along their colds to him so we've got a noisy breathing, coughing, snotty little machine on our hands. I've braced myself for another long winter but I'm hoping this year isn't as bad as last year was!

I'm high on today

key: FDA = Food and Drug Administration, NIH = National Institute of Health

A few weeks ago, I met with the Deputy District Director for Congresswoman Schmidt to talk about CF Policy and to ask for her support. At the end of that meeting, the District Director Sara, asked if we would still be interested in meeting with the Congresswoman. Of course, I said yes. And today was that meeting.

From the start, it was easy to see that Congresswoman Schmidt was a compassionate woman. She shared that she had grandchildren, and in fact, a personal connection with Cystic Fibrosis having lost a close friend of her daughters to the disease several years ago. I shared our story with her and she had unwavering attention the entire time. When I explained that there are lifesaving drugs right now going through the FDA for approval but could be held up if cuts are made, she replied with something to the affect of "that's simply not acceptable". She cared and she understood and she agreed to fight with us for this. We had a quick discussion on Medicaid [and everything that's wrong with it] but mostly how its working for us and for many other people that need it right now. I told her how much time we spend talking to drug companies and insurance companies and having doctors write letters and set up meetings all to get us access to a drug that we need. She gave me the name and direct phone number of someone in her office and said that if I ever again run across any problems like this, to contact this woman and she would call on behalf of the Congresswoman's office to get us access to what we need. INCREDIBLE! At one point she looked at me and said, "I really like you."

We talked about Drew and our daily lives and Cystic Fibrosis for almost 45 minutes before she had to go to her next meeting. We took a picture together (her office did, my cell phone camera crapped out on me) and just before leaving I reached out to shake her hand and thank her and she said, "Would it be aright if I hug you?" Of course!! She hugged me and said that she would love for us to come and visit her again, either here or in Washington. And if I bring the kids to Washington to be sure to get in touch with her because she would take them onto the floor with her to vote if Congress were in session. She told me that she has so much respect and admiration for what I'm doing and would love to continue hearing about it and supporting us in any way she can. The meeting simply couldn't have gone any better.

High on that meeting, I headed about 30 minutes north to meet with Congressman and Speaker of the House John Boehner. He is the Congressman for the 8th Congressional District of Ohio. We weren't sure if we were going to get him or just someone from his staff and it turned of that we were just able to meet with his Chief of Staff today. I will admit that the meeting didn't start out as I had hoped it would. Maybe it was because when we sat down I asked if Congressman Boehner was in town today. He basically rolled his eyes at me and said, "He's not here", to which I replied, "I didn't ask if he was here, I asked if he was in town." I thought hey, why not give it a shot. Maybe a spot opened up in his schedule and he happened to be free. No harm, no foul.

The man who we met with seemed someone less than excited to meet with us (probably because of the way I started out), seemingly shooting down the request that we made for him to oppose cuts to the FDA and the NIH or dismissing it as though we didn't really know what we were talking about. However, with a little bit more discussion and personal story added to the mix, he opened up a little bit and asked us some good questions. He expressed that he thought it was an excuse that organizations were making by backhandedly asking for funding when they asked for Congress to oppose cuts to the FDA and the NIH. He doesn't see these cuts, that will undoubtedly be happening, affecting things as severely as we seem to imply they will. We both agreed that something with the FDA and the NIH needs to change, when drugs for things like Erectile Disfunction or for cosmetic procedures such as Botox are on a level playing field as drugs for life threatening illnesses like Cystic Fibrosis or Breast Cancer. They all have to go through the same process, which is a long, tedious and costly process. This is when I introduced the National Center for Advancing Translational Sciences (NCATS) which is a proposed organization within the NIH that would speed the development of much needed treatments for those with serious illnesses, especially diseases like Cystic Fibrosis. I know that Congress doesn't have any money to spend right now, but adding this to their consideration for a more efficient process can't hurt.

At the end of this meeting, I expressed my desire to meet with Congressman Boehner himself and his Chief of Staff told me to email him and he would see what he could set up. In my mind, that's one step ahead of going through the district scheduler to get a meeting. I'm hopeful that we will get our meeting.

Overall, today can only be described as incredible. Everything went so well and I feel like I'm making a difference. I hope that I can continue with this momentum and help to improve the quality of life for Drew and others like him. The end!

Lung disease free are we!

I went to a viewing on Wednesday night for the husband of a former co-worker of mine. Her husband passed away in his sleep at an age way to young for something like that to just happen. It was sad. There were so many people there to pay their respect, most of whom I assume never even met the man. They were co-workers of the wife. There were people that I'm sure she'd only ever spoken to on the phone concerning a business matter that showed up because they care about her. I was sad for her. And I wondered, just for a moment, who would come out to a funeral if I lost someone I love. I know its not a good attitude to have, but when your kid has a terminal illness, a disease for which there is no cure, I can't help but have in the back of my mind the fear that I will lose him. My job as a parent is to keep my kids alive - feed them, nourish them, teach them, love them. I do all of that the best that I can, and I still might lose. I don't think that way a lot, but when something so sad as what happened to my former co-worker happens in my world, I wonder and I hope and I pray that I'm not going to be in that same predicament of trying to find comfort in such great pain and sorrow.

Drew had a hi-res CT scan yesterday. There was probably close to a years worth of debate over this with his doctors before we decided to go ahead and do it. They wanted to get a good look at his airways to see if he has any lung disease. Its mostly for benchmarking purposes, to see what it looks like now and then in a year and then in another year. The results weren't going to necessarily change any course of action or medical treatment plan, which was what caused my hesitation to do it in the first place. I get curiosity, I'm a curious person. But when it comes to putting my kid to sleep to satisfy your curiosity, I'm going to draw the line. I know that wasn't the case. I know that having the best possible look at Drew's lungs and understanding what they look like and how they handle or have handled certain treatments and whatnot will aid us in treating him in the future. And when we finally discussed a specific action plan based on the results of the scan, I was on board.

What they were looking for was bronchiectasis, which is destruction and widening of the airways, thus not allowing them to hold air appropriately and making it difficult to breathe. Its basically lung disease, which we all know is the culmination and ultimate cause of death with Cystic Fibrosis. Their hypothesis was that because of Drew's malacia, he isn't able to properly clear the bacteria laden secretions that CF patients have and he would show signs of early lung disease because of these chronic infections. We do his breathing treatments and airway clearance twice every day, but when Drew coughs, instead of being able to bring it up, his airways compress and collapse and trap the mucus in there. It is so critically important that CF patients keep their airways clear of this bacteria because this is what causes lung disease. Our plan was to start him on azithromycin 3 days a week if we saw bronchiectasis based on some newer studies about its benefits in reducing pulmonary exacerbations and preventing bronchiectasis.

Well, guess who was WRONG!!

We had the CT yesterday and the doc was supposed to call today with the results, but instead she called me about an hour after we left the hospital because she was so shocked and thrilled with the way his lungs look. There was no evidence of any bronchiectasis or lung disease, or anything of any real concern. They saw some "streaking" which they've seen in his x-rays in the past, but assume that that's just some mucus, which all CF kids have in their airways all of the time anyway. Nearly 70% of 3rd olds with CF show some lung disease on CT scan. I'm quite delighted to know that at just half that age, my boy is currently free from that. I know it will come. I just hope its slow and mild and that everything we're doing will continue to grant him good health. And I'll keep emailing and visiting our congressmen and women and encouraging them to support us in our fight so that a cure is found before we have to watch CF rear its ugly head at us.

Guess what I got!!!

I have a meeting with the office of Speaker of the House John Boehner next Monday, two hours after I have a meeting with Congresswoman Jean Schmidt (really her this time, not just her staff). I'm so excited! The director of the CF Foundation in Cincinnati is coming with me to both meetings, along with another woman I met who agreed to advocate with me. She has a friend whose daughter has CF and she Cincinnati's Finest Young Professionals award two years ago. She knows John Boehner personally and I contacted her and asked if she would be able to get a meeting set up for us. It is unclear whether or not John will actually be in the meeting yet, but if hes not at this one, Lindsay will continue to work to get a meeting set up with him. Good things are happening! We're spreading the word. This disease isn't going to cure itself :)