Monday, December 16, 2013

"Those who move forward with a happy spirit will find that things always work out."

Drew has been having a lot of trouble staying healthy during his off cycles of inhaled antibiotics and it is concerning. He should be able to go much much longer than 2 weeks without needing an antibiotic to suppress a bacteria to keep it from causing problems. The good news is that the antibiotics continue to work, bringing him back to normal. However, we need to find a way to keep him healthier for longer periods to avoid the inevitable lung damage, so we are going to try another round of IVs. We are actually going to triple hit the bacteria, with an IV, and oral and an inhaled antibiotic to see if we can regain control and keep the poor kid feeling good. I wish he was old enough to do PFTs so that we could measure his lung function and determine what kind of toll this is really taking on him. I also wish I had a logistics manager living in my house.

Drew will be going into the hospital on new years eve for a bronchoscopy and PICC line and then will come home to do the IVs from here for two weeks. I joked with his doctor that when they offer him something to make him a little fuzzy before they knock him out for surgery that they should also offer that to parents to take the edge off. And it's not just the edge of sitting in the "Same Day Surgery" waiting room while you're baby is put to sleep and poked and prodded inside of an OR without you there to see him, but also for the waiting of test results post bronchoscopy. It takes about a week for bacteria to grow when they take a culture, and so we will treat while we wait but we will still wait and worry and wonder what we will find and what we will be able to do about it. A part of me still hopes that it's just this antibiotic resistant bacteria, achromobacter, because we know what it is even though it's not responding to anything. It sounds sick to hope for that, but things could be worse. A little part of me hopes that it's a different bacteria that isn't horrible, but that we've just been treating with an antibiotic that doesn't cover it, and therefore if we can treat it with something that it's sensitive to it could help to make things better. I think that scenario is probably the most unlikely. We could also find other horrible bacteria or fungus, stuff that doesn't respond to antibiotics or anything and causes rapid lung function decline. This, too, is perhaps unlikely though certainly a possibility. There is just no way to tell but to wait.

It almost seems as if there is no good scenario here. We are going to get test results that will tell us what bacteria he has growing in his lungs and hopefully what antibiotics, if any, it can be treated with. I might have an ulcer waiting for these test results. Drew looks and sounds and acts great 95% of the time, so it's really hard for people to understand the complexity of this disease and how sick he really is. Even if the bacteria that he is growing is antibiotic resistant, we will continue to treat him with a number of different antibiotics from all different classes to try to find something that will get and keep things under control. 

And then I look at him and I think no good scenario? He was swimming this morning, and well! Dance lessons are his favorite hour of the week. He goes to school 3 days a week, and runs and paints and plays with other little boys and girls who is no different than. He watches an inordinate amount of Toy Story. He tells jokes and wrestles with his brother. He loves the Children's Museum and going to the zoo. His a wizard on the iPad. He is so gentle and loving and kind, a true mamas boy. He cares so much for other people, offering a hug to a crying sibling or helping us to clean up a mess around the house. He holds my hand through every. single. treatment., just taking it all in stride. I'm living a good scenario and I don't take that for granted. 

I really can't believe how wonderful this year has been. It may seem like a strange transition from my downer health update, but it has truly been an amazing year. The opportunities that have come my way are beyond words. The connections that I have made and the work that I am doing and the people that are all on my team fighting for a better way, a faster way, surely don't realize how much I value their camaraderie on my mission. It's every time I speak up, every tweet that I send, every time someone offers me a glimmer of hope that we can do this, we are so close, I can't even express my gratitude. Emails from people around the world let me know that while others don't have the courage or even just capacity to do all that I do, they appreciate it. I forge ahead at 110mph hoping and praying that we get this disease figured out in time and that the people who I annoy along the way realize that I do it all with the very best intentions. I am trying to save a life, and live a life and raise 4 little lives and be a friend and have some fun and change the world all at the same time. I have so much hope but I am terrified, so please stick with me. 

I couldn't be more grateful for all of the ears that have listened to me over the past few years, but this year in particular. The CF Foundation is on my team now, creating a Collaborative Chronic Care Network for CF. They realize the value of all working together for better outcomes and a faster cure. My heart belongs to Cincinnati Children's Hospital for embracing me as a "family partner" on their team, interjecting my thoughts into their well planned everything, sending me to conferences to learn more and share back what I learn, listening to me express both hope and fear, and encouraging me to keep going. 

I read a quote today from John Wayne that struck a chord: "Courage is being scared to death and saddling up anyway." There are so many more opportunities awaiting in 2014, so I will roll up my sleeves, drink my Starbucks, and continue to do the best that I can, or saddle up, if you will.  But I can't do it alone. If you're reading this, I need your help. I need friends and family to fund raise and advocate with me. I need fellow CF mamas to test out these systems and tools with me, and bring new ideas to the table. I need patients to push the limits of their doctor-patient relationships to show the world how much better things go when we work together. 

So thank you, to everyone in my world, for an absolutely beyond words amazing year. Cheers to another great one in 2014. Courage

Thursday, December 5, 2013

My Energy Reserve

Drew is getting sick again. Well, not really again as much as he's not ever really getting better. It's a little hard to explain, but I'll give it a try. Last March, Drew started to culture an antibiotic resistant bacteria in his lungs called Achromobacter. While a bacteria in a petri dish may or may not respond to different antibiotics, the reaction of the bacteria to the antibiotics inside the body isn't always the same. The achromobacter that Drew cultured appeared to be resistant to most antibiotics in a petri dish but we tried anyway and Drew had a positive response to a few of them. His symptoms would disappear for a period of time but would always return and we continued to culture achromobacter. After months of trying to suppress symptoms and eradicate this beast, we had to move on. There are literally no other options suitable for Drew to try to get rid of this bacteria, and trust me I have searched the globe. A common practice in CF, to manage symptoms and keep bacteria suppressed to a point where it's not symptom causing, is cycling inhaled antibiotics on and off for different periods of time. It's a reasonable way to manage these different and persistent bacteria and prevent them from casing lung damage by keeping the bacteria suppressed to a sort of non-damage causing degree. Anyway, we've been cycling Drew on an inhaled antibiotic called Ceftazadime since this summer, 15 days on and then 15 days off. The problem is that he hardly makes from the end of an "on" period to the beginning of the next without. About a week after he finishes his 15 day on cycle, he starts coughing quite a bit, his oxygen saturations start to decline, his energy level bottoms out and we end up starting the antibiotics again to help bring him back down. As time has gone on, these episodes that occur during the off cycles seem to be getting worse, and today we decided that he needs a bronch, a tune up with IV antibiotics, and then a CT scan to determine if all of these exacerbations are causing lung damage. Lung damage is inevitable, but it hasn't happened yet and I am not ready to see it. Many people with CF start to develop lung damage or bronchiectasis early, so it wouldn't be abnormal if we saw it on a CT scan, but it still sucks.
Right now we are in a bit of a waiting period. His doctor is going to get a bronchoscopy scheduled, and since they have to sedate him for the procedure, they will place a PICC line at that time to start the IV antibiotics. Drew started the inhaled antibiotics last night, so he's had several doses now as we do them 3x a day, but his oxygen levels haven't yet gone back up to where they should be. If they don't start to get back toward normal tomorrow then they will admit him to the hospital where the bronchoscopy procedure and PICC line can be expedited. It would mean spending a few days in the hospital, but after the procedure we could probably go home and finish the IVs at home. He will probably respond to the inhaled antibiotics at home like he usually does, but the IVs will hopefully work a little better and give him more and longer periods of time without this bacteria causing a problem.
I know this all sounds crazy and complicated, and it sort of is, but it's also par for the course. Tune ups are commonplace, done in an effort to return patients to their baseline, but it doesn't make it any less stressful or scary. I hate to see Drew not feeling well. It's mentally, emotionally, and physically exhausting trying to figure out what's going on, what's causing it, what our options are for fixing it. The rest of my crew is out of sorts as we spend hours doing treatments and on the phone with doctors and schedulers and concerned family and friends, missing lessons and appointments but needing to prioritize. I will not lie, it is not easy. We are stressed and tired and guilty about who we should be spending time with and what we could have done differently to prevent the need for this even though we know we are doing the best that we can.
At times like these, I wish that I would have appreciated those down times a little more, the opportunity to bank a little bit of energy. I wish I would have gone to bed earlier, said no to a meeting or a dinner, and just taken a deep breath and relaxed. I don't know that it would make a difference, but during times like these I need to dip into my energy reserve, and it is dangerously low. Patience is shot, tempers are short and I am tired.
We should have more information by the end of the day tomorrow. I'm hopeful that his oxygen will start to go back up and that his doctor can get him scheduled for what he needs to have done sometime in the next week or so. I will try to keep the blog updated with how he's doing, though i've been noticeably absent from the blog for the past few weeks. I have so much fantastic stuff going on, stuff that will change the face of this stupid disease, and I will blog about it soon!