"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

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!

Finding a cure

"Walk with the dreamers, the believers, the courageous, the cheerful, the planners, the doers, the successful people with their heads in the clouds and their feet on the ground. Let their spirit ignite a fire within you to leave this world better than when you found it"
The other day I found my self daydreaming about what this life would be like if CF weren't a part of it. I was imagining playing after dinner instead of setting up for treatments, or waking up and taking a day trip without time constraints. I imagined making the kids all the same thing for lunch, not spending time pouring vegetable oil onto fruit and trying to mentally calculate how many grams of fat he would be consuming to appropriately distribute enzymes. I imagine what I would do with the time that would become available, previously spent refilling prescriptions, fighting with insurance companies over why we need a certain medication. I imagine counting weeks and months as weeks and months instead of 15 and 30 day on-again off-again antibiotic cycles. Maybe the constant fear and worry and anxiety that I have and try my hardest to suppress would melt away, or maybe I'm confusing the emotions of parenting with the emotions of parenting a child with a chronic medical condition. I really don't think the days that we're experiencing are all that bad, but I was dreaming of better. 
I keep myself pretty busy these days and a lot of it centers around Cystic Fibrosis. The C3N work that I'm doing is amazing. I am involved with different groups and committees at the hospital and at the CF Foundation, related to everything from adherence to advocacy. I am a caregiver, so I order meds, administer meds, clean medical devices, manage insurance issues, track symptoms, and the list goes on. However, the list of things that I do that aren't related to CF is longer. CF isn't our life, it's just a part of it.
We are exactly one year out from the first time I approached the CF Foundation about embracing the value of collaborative care - care involving patients and clinicians and researchers all working together to test and improve tools and systems to better manage health and care. Next week, we are going to Bethesda, MD to the headquarters of the CF Foundation to discuss the scope of work that we are about to partner on and I could not be more excited. How did all of this happen? What did I do to get here? Could my dream of collaboration for a cure really be coming true? 
I'm often asked what I did to get here, or what I even still do. I talk about it. I talk to everyone I meet and tell everything I know to everyone I know. I gather information and I curate information and I share information. I ask a lot of questions (I mean a LOT of questions). I identify problems in the current system and share opportunities for improvement. I pay my own way to conferences and am forward in suggesting that others pay my way to more. I'm energetic, passionate, and persistent because it is in my nature to be so, but also because I don't have a choice not to be. He is my child and my job is to keep him alive and well, and to guide him in the directions that his life takes him. It is my job to protect him at all costs, so I take the risks that I probably wouldn't otherwise take with the hope that I will find the missing puzzle piece that will cure this disease. One of these ideas will work, and I will keep trying until I find it. 
I'm in disbelief that it's all happening, honestly. I heard about it and I asked questions and I shared what I learned and I nudged and nudged some more, and at times I pushed and at others I pulled, but we're getting there and I can't believe it. I go to bed at night sometimes annoyed by my own optimism and excitement. It's a sheer and giddy joy knowing that this is all happening because I had the courage, and the responsibility as the parent of a chronically ill child, to speak up and encourage the re-design of a system that has the potential to save my sons life. I'm no longer imagining that potential because I'm working with other patients and clinicians and researchers and all stakeholders and I'm living this change. 
I cannot wait to share about all of the awesome things that will come out of our collaboration with CFF next week when we visit their headquarters in Bethesda! 
"Never underestimate the power of a small group of committed people to change the world. In fact, it is the only thing that ever has." -Margaret Mead

Every day is a long day

We just found out this week that Drew's secondary insurance decided not to renew us for the upcoming year like they have every year for the past 3. Instead, we have to save receipts and prove to them that we spend a certain amount out of pocket on our medical expenses on a monthly basis and then fill out a gazillion and ten forms and send it back to them for review to decide if they will cover us. It's as though they think that this disease gets cheaper to manage as time goes on, when the exact opposite is true. There are more medicines and treatments, taking more time and costing more money, and none of it keeps him from getting any sicker. The co-pay on Drew's monthly medicines is around $6,000, monthly, so hopefully we will be able to get their decision changed fairly quickly, but it's just something else that's been added to my plate, something else that I have to fight for, and I hate fighting all the time.

We're into our 15 day on/off cycles of inhaled Ceftazadime. When we finished our first cycle, Drew's cough was nearly gone and his appetite back up. We've been off for a week now, and just this morning he decided he didn't want to eat again. My gut (and the data that I have been collecting on his behaviors) tell me that that means he's getting sick. I hate that he's always sick. I'm always fighting with him to eat, to drink, to take his meds, wash his hands, use the bathroom. He takes a multivitamin that he recently started refusing because they no longer make the kind that he'd been on his whole life. They changed the flavor to orange and now he won't eat it. My husband tried it and said that it tastes like vomit with a little citrus flavor, so I can't say that I blame him, but he needs it and so I fight with him to take it. I fight with insurance over whether or not certain medications need prior autohrization and then fight with them again when I don't get what I need in time because there are so many stupid policies and procedures in place, that,  I might add, are direct barriers to adherence. 

Patients and caregivers dealing with chronic conditions need a break! If it weren't for our fight, nothing would ever get done. Yet we are the ones with the least time and the least energy left at the end of a long day, and every day is a long day. I'm tired and I'm annoyed. I'll be appealing the decision but it won't be without a fight. 

Stream of Consciousness on the NACFC, Patient Engagement and Hope

Last week I attended the North American CF Conference in Salt Lake City, Utah. After I'd gone last year, I made it my mission to attend every year until the value of having patients present was fully appreciated and I'm happy to report that we're getting there, but we still have a long long way to go. There were actually a few sessions this year specific to the value of including patients and families as a part of the care team, partnering together to improve outcomes and changing lives. My goal, leaving the conference this year, is to find some ways to quantify the value that patients/parents bring to the table and share with all 4,000 attendees next year how I believe that the missing puzzle piece in this quest to cure cystic fibrosis is us, the very people living every day so desperate for a cure. 

On my way to the conference I started to read a brilliant book called "Critical Decisions" by Peter Ubel. It is a passionate plea for patient empowerment with quotes like "The new revolution in medicine needs to be less about power and more about partnership. The best decisions are often shared." and "We won't achieve true shared decision making until we prepare physicians to interact with prepared patients." and "Modern patients have a duty to be informed in health care decisions." That last one really got me thinking. You see, the NACFC isn't open to just anyone. It's a conference held for 4000 or so doctors and researchers and other clinicians, but not specifically for patients or families. We're not totally excluded but it certainly isn't encouraged and definitely not made easy if you decide you want to go. There are some hoops that you can jump through to get yourself there, if you have the $575 to pay your way or are lucky enough to have someone offer to send you. 

The purpose of the conference is to share the latest and greatest in scientific research with others in the field to make better informed decisions regarding the health of CF patients and ultimately find control of and a cure for this disease. There's no doubt in my mind that that is going to happen, but without the participation of the ultimate stakeholder, the patient or the people that are fighting for them (families), it's going to take a lot longer.

"Modern patients have a duty to be informed in health care decisions." There was so so so so much valuable information that I came across at this conference that isn't available for others in the CF community, unless of course they paid their way and got the book of abstracts and sat through endless talks full of medical jargon that had even the most brilliant physicians googling terms. It's not fair that I, having fought and pushed and proved my value to our clinical team to the point where they sent me, am the only one who knows all of this stuff that could be life changing for so many. Stuff like the re-introduction of Atalauren to the drawing board for nonsense mutations. That, my friends, is hope for some people in some hopeless situations. And hope can carry you a long way. But no one knows about this but me and a handful of people who follow me on Twitter. Sure, many clinicians now know too, but there has to be an appropriate context for them to bring this up with their patients, all the while balancing the plethora of other valuable information that they think is important for their patients to know.

I learned about the dangers that inhaled corticose steroids pose to CF patients, increasing the risk of culturing non-tuberculous mycobacterium 5 fold, while really showing no signs of reducing inflammation in the CF airways. That's important, and perhaps even life changing for some. But unless you were a doctor or one of the few parents or patients privy to that information shared during one of the sessions at the NACFC, you are still in the dark, and that's not acceptable.

Doctors cannot know it all. Patients have a duty to be informed in their health care decisions, but as ePatient Dave says it best, "It's perverse to keep people in the dark and then call them ignorant." It is the patients themselves that have contributed to and participated in the research that these brilliant doctors and scientists are doing, but yet we do not have access to it. Bob Beall gave a heartfelt "thank you" to patients and families, for without them we wouldn't be where we are today. I propose a "thank you" and a program next year, sharing with them the very research that they enabled. The research will go farther, the puzzle one step closer to completion, if you embrace patients as partners.

I can almost guarantee you that I was one of the few who read nearly all of the 600 or so abstracts combined into the program that was the 2013 NACFC. Doctors don't have time to do that nor should they be expected to. Researchers don't have specific interest to do that, looking at the things that are relative or important to them and then moving on. But I, the parent of a patient with an incurable, life shorting disease, I don't have time not to. My ears listen carefully to every option, every alternative, every mild suggestion that there might be hope. My exhausted being and worn out kid try novel approaches and treatment options, in clinical trials and independent of them. Our doctors are deciding what information to share with us, some more than others, some better than others. Some share all of the knowledge and options that they know of, but it's impossible for them to know it all and it is our duty as patients to seek out what we need and work together with our clinicians to help ourselves and to help others.

I overheard a conversation between two (i'm assuming) doctors that was narrow minded and disheartening. During a presentation on the role of viruses in a pulmonary exacerbation, the speaker, who probably had a degree in some sort of medicine that I'm not even able to appropriately pronounce, was getting slightly deep into molecular biology and from the tone of the conversation in front of me, somewhat off topic. The one doctor said to the other, "This guy needs to move on. If people want to learn about this they can find it in the journals." But we can't! We can't find in in the journals because we don't always know what we're looking for. And with all of the information being shared here, these doctors and researchers aren't going to have time to do it, if they even remember to! And "the journals" cost money, something like $15/article. It's like our co-pays, no big deal to most families just needing to refill a prescription here and there, but our co-pays are over $6,000 every month, so yes, I will find ways to alleviate the burden and get us access to what we need because not doing anything is simply not an option. Putting this incredible information into journals and then storing them in a vault is not doing us any good.

All of this work that I'm doing to design a collaborative chronic care network for Cystic Fibrosis is for reasons like these. We "patients" are finding ways to get what we need. We are organizing ourselves and talking to each other and we are asking clinicians and researchers and other stakeholders to join our conversation since we haven't been invited to theirs. Everyone is so afraid of taking that risk, worrying about patients taking something out of context or their identity or reputation being tarnished because of something going wrong online. I can appreciate those fears, but I implore you, take the risk. Dr. Wendy Sue Swanson said it best at MedX during a Masterclass on engaging through social media - If you're a moron in real life than you're going to be moron online. Don't be a moron. Why am I the only person who doesn't seem to be afraid of things going wrong, but rather looking toward the possibility of all that could go right. What if my patient reported data could be appended to some highly clinical research to bridge that gap and complete that understanding, leading to the development of new treatments and therapies and ultimately curing this disease. Again, I implore you, take the risk!

I overheard another clinician speaking about how it's their (clinicians) responsibility to educate patients on how to do different treatments and that if they're not doing them appropriately then it's a failure on the clinicians part to appropriately educate. What if the patient understands perfectly well all about the treatment but just simply doesn't want to do it? I will agree with that doctor that it is a failure, but not a failure to educate. Rather, it is a failure to engage and empower and embrace that patient as a partner in their own care so that you together understand the relationship and how you can work together to preserve health.

If I create data and you create data and we share that data with one another and then we are able to identify things in our data, and then we get his data and her data and add it to our data, before we know it we have our own little research going on. The value of that data shouldn't be diminished. We control our research because it's our data. The data that researchers have is technically our data too, but somehow they own it and we can't always get it back. So we (patients) started collecting data on our own and learning things about ourselves, individually and collectively. Since we can't access all of the brilliance that already exists, we are accessing what we can and are making informed medical decisions with it. Just imagine how much father we could go if we put this all together. It is not rocket science, but rather an activity taught even to my preschoolers and it's called teamwork.

Toward the end of the conference I was speaking with some folks from a variety of positions of power and influence in the CF community, both within and outside of the CF Foundation. Both asked the same question - what are we supposed to be doing? How do we engage patients in ways that are appropriate and useful? How do we curate information for our patients and families? How can we be truly helpful? My answer to them was simple: Take a risk. Join us. Participate in our conversations. Listen to our feedback. Embrace us as partners, not recipients of some service or tool that you offer. Show us that we are truly in this together by holding our hand and combining our efforts and working together to create and test and implement tools and solutions that will aid in the development of a cure for this disease. I hope that they took me seriously because I meant it.

There's so much more that went on at the conference of value to me and to patients and their families that I don't even know how to put it all down on [virtual] paper. But I'm putting out there right now that I want to help you. I have the resources from the conference. I would love to help whoever needs my help by searching through the abstracts and posters to find any information that might be of value to you. Email me, tweet me, Facebook message me. I will try to put as much important information out there as I can, but if there is something that you're interested in or scared about or just wondering if there was any discussion around, please ask me. I want to help. I believe that we are the key to the cure.

Small Victory

Drew has been culturing Achromobacter, an antibiotic resistant bacteria, in his lungs since January. We have had several hospital stays, a couple of procedures, endless antibiotics and even more cultures done in our quest to tame this beast. It's not causing any terrible harm, as far as we can tell, yet. His CT scans don't look any different than they did two years ago and he is growing well. It just seems to cause this little, annoying, lingering cough to pop up from time to time and it won't go away without antibiotics. And when it comes, he doesn't want to eat. But he needs to eat, and a lot. Drew has been off of antibiotics for no period of time longer than 2 weeks since last January and that's terrifying. You see, he's only three years old, and if we pull out the big guns now to fight this, I'm afraid we won't have anything left in our arsenal when we really need it. There is an abstract in the 2013 North American CF Conference Program that states, " Once colonized, achromobacter appears to lead to a significant decrease in FEV1 and increase in hospitalization rate. An average decrease of 16% of FEV1 over an average of a 48-month period was observed." He's too young to do PFTs to determine his FEV1, but let's suppose he's at 100% today. By age 7, his lung function would be at 84%. By age 11, down to 68%. Before he is 20 years old, if Drew continues to grow Achromobacter, his lung function would, theoretically, be down to 36%, low enough to place him on the transplant list.
As I've spoken about many times on here, I track everything about Drew in an attempt to learn more about him and about his CF and how his CF compares with the CF that other people have. It's such a unique disease in that everyone is affected differently. I've been tracking using PersonalExperiments since early this spring, and have gotten some very useful data. This data, in fact, even helped to prevent an admission at CHOP this summer. I scour the internet and medical books looking for answers that could help to explain different aspects of this disease that I don't always understand or don't agree with. I look for novel treatment options and I take what I find to his doctor. When I ask her questions, she does her best to answer, and always offers to help me to find the answers that she doesn't have. I go to conferences, I talk to everyone I meet, desperately hoping that someone will mention something that brings me to my "Ah-ha!" moment and I can solve this mystery and put CF behind us. I push our team at Cincinnati Children's Hospital to move faster and work harder to try new techniques that, for me, have provided insights that are changing things for us. If they work for us, they can work for others, and the new learning that will pour forth could be eye opening to even the most distinguished doctor or scientist. I'm talking about insights that I, the patient, bring to the equation.
When Drew first grew Achromobacter last winter, we immediately tried IV antibiotics. Even though this bacteria is antibiotic resistant, sometimes some antibiotics work. For Drew, the IV's didn't. After the IVs we tried an inhaled antibiotic for 30 days, to no avail. From there we repeated our course of IVs with different antibiotics followed by a bronchoscopy, a CT scan, and more IV antibiotics. The achromobacter remained. At this point we were resigned to the fact that Drew was colonized with this bacteria and it was unlikely to ever leave him. That did not mean that we would stop our fight against it. We decided to start cycling him 30 days on/off an inhaled antibiotic, as many with CF who are colonized with a bacteria do. The idea is that if we can't get rid of it, we try out best to suppress it and keep it from causing more damage.
When Drew was on an antibiotic he was symptomless, but once we came off, the symptoms quickly returned. During one of our early 30 days cycles, we noticed that his symptoms would go away for about 15 days and then start to return, even as we remained on our antibiotic. It was easy to keep track of this with the tools I was using. We decided to try another angle, 15 days on one inhaled antibiotic and then switch to another for the next 15 days. That technique managed to suppress symptoms for almost 30 days, but the cultures were still showing that achromobacter was there.
At this point, we were no longer really trying to eradicate, but rather trying keep Drew symptom free. The 30 day cycles split between two antibiotics seemed to be our best bet, but after the 30 days was up the symptoms would immediately return. We took another angle and thought that maybe because of the severity of his tracheomalacia and the propensity of these inhaled antibiotics to cause bronchospasms and a degree of airway reactivity that he wasn't actually inhaling as much of these medications as we thought or as necessary to control this bacteria infection. We decided to do 90 days on inhaled antibiotics, alternating between two different drugs every 15 days. For 90 days, Drew was symptom free (except for a brief stint during the summer when he got rhinovirus). When we cultured in early September, the achromobacter was still there. We decided that it was time to just run with the idea that we would keep this bacteria suppressed as best we could by cycling 15 days on/off of an antibiotic that seemed to really best control his symptoms, despite his achromobacter being totally resistant to it in the lab.
We began our first 15 day cycle in early September and Drew was happy and symptom free. He started school without incident and continued happily about his days with his cough nearly non-existent. On the last day of our 15 day "on" cycle, I happened to be looking over the data that I had been collecting on him and realized that the only combination or course of inhaled antibiotics that we hadn't tried was this one particular drug for a straight 30 day course. We had done it on and off for 90 days in 15 day increments with a second antibiotic mixed in. We had tried other antibiotics for 30 days straight. But the use of this particular drug for a full 30 day course hadn't been tried, and I, with agreement from his doctor, decided that we needed to at least try. I was the one who had decided to cycle him 15 days on/off because there was evidence in the data that I collect on him that his symptoms seem to return after 15 days. I was also the one who, desperate to try anything that we hadn't already tried, decided at the last possible second, to continue our first 15 day "on" cycle and turn it into a 30 day "on" cycle. I had agreed with the doctor that if this didn't produce different results that we would have to turn to either 30 days on/off or 15 days on/off, that he couldn't simply remain on antibiotics nonstop, forever.
So we pushed through, for 30 days, 3 times a day for 30 minutes on top of the 3 times of day for 30 minutes that we were already doing for normal, routine, maintenance medications. We were spending nearly 3 hours a day every day doing treatments. Drew hated it. We hated it. But we saw a clean culture!
We got a clean culture, a culture that showed no growth of achromobacter, our first since last January.We weren't going to announce this initially. We thought we would keep this information to ourselves until we had a second confirmed culture free of achromobacter.  But in the world of chronic disease, we celebrate the small things.
The next day Drew got the cold that everyone else in this house had been fighting and the achromobacter came back. I know it wasn't really gone, but it was suppresed, and the extra mucus from his cold just stirred everything up and the bacteria is back and causing problems once again. We're back on the antibiotics, going to cycle now 15days on/off indefinitely. Because we were able to suppress it to the point that it wasn't showing up on a culture, albeit for short period of time, I will push forward on my mission. I will continue to try in whatever ways reasonable to keep it from causing him any harm. I will not accept a lung function decline of 16% every four years. But a clean culture is a clean culture, right? A small win, even for just a very brief period of time, is still a win. We will fight on!

Patients Included: But Are We Respected?

"Any man or institution that tries to rob me of my dignity will lose because I will not part with it at any price or under any pressure." - Nelson Mandela, Long Walk to Freedom

A few weeks ago, I was asked to be a part of a Cystic Fibrosis work group at a hospital on the east coast under a grant from the Patient Centered Outcomes Research institute, or PCORI. A colleague had recommended me for the position, knowing my passion for collaborative efforts in improving outcomes and the value of the patient voice in research.  Below is an excerpt from the email that I received:

PCORI was founded under the ACA to fund comparative effectiveness research that improves the information available to patients in a meaningful way. Specifically, PCORI has two goals: further engage patients in research design, and reduce the gap between research results and patient knowledge. The CDRN opportunity grants institutions money to develop a large multi-million patient data warehouse for researchers (from around the country) to conduct research - both prospectively and retrospectively. PCORI will fund seven of these networks around the country to form one national network. If awarded the grant, our team would like to offer you a role as a Cystic Fibrosis Community Workgroup member.

My interest peaked, I wrote back and a day later had an interview and was submitting my biosketch and a letter of support. I made them aware of the other work I'm currently involved with to ensure there would be no conflict of interest, and expressed the need to weigh the time commitment and opportunity for compensation with these other projects. Then, as if to define how they value my knowledge, experience, and expertise, I received this reply:

While this particular position will likely be unpaid, this is an incredible opportunity to directly influence the research efforts around CF and will provide you with access to cutting edge researchers in our city.

Is my time and participation not valuable enough to compensate?

Later that week, I packed my bags and headed to MedicineX in Stanford, California, a conference that positions itself as a catalyst for new ideas about the future of medicine and emerging technologies. At this conference, there were patients and clinicians, caregivers and technologists, entrepreneurs and researchers and others from the industry. It’s difficult to explain the experience of being at a conference so full of energy, excitement and hope for better care of rare diseases and chronic conditions, better interactions with the healthcare system, and how the only way to improve is collaboration and teamwork, valuing and trusting one another, in every single possible way.

During the first day of this conference, PCORI -- the organization offering the grant mentioned above -- was presenting a panel on the importance of involving patients in research. I wondered about the offer I had received: Was it the institution that could not offer to compensate me for my time? Or PCORI that isn't accommodating patients who participate in the research by appropriately and fairly compensating them? Or maybe just not all patients? Some view the amount that I work to improve health and care in Cystic Fibrosis as remarkable. I view it as necessary. If I had endless capacity, I would do it all. But remember, I have a seriously and chronically ill child that I am caring for, in addition to 3 others, and a husband, and a couple of fish. Unlike many researchers, I don’t have an institution or grants paying me a full time salary to participate in such research.

My reaction: Don't take advantage of the knowledge and experience that I have that you want and need to create a system that works for me. Appreciate me, and embrace me just as you would any other member of your team, and just watch how far we can go together.

A huge barrier to true patient-centered care is the failure to see patients as equals to the other contributors in this system. Let's not forget than in medicine, the patient is the only one who is expert in her experience. Should the hospital that offered me the position in their workgroup receive the grant from PCORI at the end of the year, I will participate and my participation will not be in vain. I said yes, regardless of compensation status, because I know and appreciate the value of this project. I have a unique perspective to bring that I believe will add value to this research and benefit others in the community. I said yes, because so many other people who have expertise and passion and value to add don't have the time or the voice or the energy to do it. I believe that our story, in the end, will be one of success. But to quote an incredible patient and advocate that I had the great pleasure to meet in person this weekend, "Not every medicine story can have a happy ending. It's not the ending that makes it a success."

How much farther and faster could this patient-centered research go if it weren't so hard to be a part of it?

A Collaborative Chronic Care Network (C3N) for Cystic Fibrosis

What is a Collaborative Chronic Care Network? I asked the same question about two years ago when I first heard the term mentioned. In short, it's a platform for bringing together patients, clinicians and researchers to work together to create better ways to manage health and care. We all have knowledge and expertise, and by working together we can understand the tools and systems needed to improve outcomes and save lives. We're creating a C3N for Cystic Fibrosis. Get excited people!

Last week, we had the opportunity to introduce the C3N work that we are doing at Cincinnati Children's to some influential members to the CF Community. We had, I think, 19 people on the call and got nothing but positive feedback. The questions that were asked ranged from how we engage others to participate to how this project will be funded. We discussed data privacy, research, and a few of the specific interventions that have been created and tested in other chronic conditions and how they have transformed care. We did have a few technical difficulties and weren't able to have as much of the open conversation as we had hoped too, but questions that were typed in and feedback that we received after the call seemed to indicate that we generated a fair amount of interest and excitement as well as a desire from some to collaborate and get these tools out for use by patients. I was very pleased with the way that the call went and would love to be able to continue these conversations.

Now I want to share it with you! Again, we had some technical difficulties during the presentation and it would seem that because of the way it was recorded, you may need to go through the GoTo meeting link and register to view the recording. The GTM link is: https://www1.gotomeeting.com/register/352665816 After entering in an e-mail address, you should be prompted the option to download the GTM codec. PC Users may first need to install the GoToMeeting codec.

If you're at all interested in this super cool work that we're doing, watch the webinar and then take a look at the questions below. We've tried to address any currently outstanding questions from the webinar below, but know that there are always new questions and ideas and I'd love to help address any that come up. And even more than that, I'd love to hear what you think about all of this! This is going to change the way we manage our health in CF and I couldn't be more delighted to be a part of it!

How well does a C3N work with more passive individuals who don't have great advocacy skills?

Within the scope of the C3N project, there is a role for everyone. There are people who will simply be aware of the project. They will be aware that there are tools at their disposal, that their doctors and nurses are using to manage different aspects of CF health and care. There are people who will participate, who will ask questions, be a part of a pilot, read our blog and follow us on Twitter. There are people who will be contributors. They will give us feedback, join a QI team at their CF Care center, post to the blog. And finally there are people who will be owners, who will lead teams and run QI projects, create tools and organize events. Our goal is to make everyone aware. It would be nice to have 90% of the community participate. A very smaller percentage of people will contribute, and we would be happy if 1% actually take ownership roles. It’s important to remember that this framework is for the C3N project, not for the implementation of different interventions into the CF community. We are using this platform to test and organize new ways for doctors, patients and researchers to transform care. Once we can validate the usefulness of different tools, we hope to work with the CF Foundation on implementation. If you consider things like a quarterly visits for example, they were something that was researched and deemed valuable and the CF Foundation helped to implement it across its centers as a standard of care. Everyone will get to benefit from the outcomes of work that the active and engaged contributors and owners produce.

Have you begun the creation of a C3N for CF? What are the first steps? What do you need from us? How do we see it to fruition?

Cincinnati Children’s has already started work on the creation of a C3N for Cystic Fibrosis. The hospital has provided some funding to begin work on a few interventions over the next year, and we have also received some other grant money for the work that we are doing. The best way to get funding is to raise awareness and to show the value. We are working with the CF Foundation to show how using the C3N platform can change the way that we manage a complex chronic disease like cystic fibrosis. Even though several of these interventions are in pilot phase in Cincinnati we still need broad collaboration from all stakeholders to create a system that will be useful and beneficial to everyone.

Is Ginger.io app available to general users now?

Yes and no. Ginger.io is currently being used in the Diabetes community and is being tested out on patients with Crohns and Colitis. Within the scope of the C3N Project for the IBD community, Cincinnati Children's and Ginger.io are hoping to map the “behavioral genome” of a patient living with IBD. Ginger.io is not yet being used with CF Patients. For the cystic fibrosis community, we imagine using Ginger.io as a way for clinicians to monitor patients between visits and for patients to gain insights into behavioral patters that could affect their health. By creating an automated system that outlines the “norm” for a given patient, both the patient and their clinicians can be alerted when they fall outside of that range. Using a tool like this in collaboration with some of the data that I had shared through PersonalExperiments, we can dig deeper into what is going on before it becomes a big problem. You can learn more about Ginger.io on their website atwww.ginger.io or by watching this 60 second video that does a great job of explaining how they work http://seanduran.com/clients/gingerio/Gingerio_1080p_v4.mp4

How do you plan to initiate and facilitate conversations within the platform (peer to peer, peer to provider, peer to researcher, provider to research, provider to provider) and how will all of those conversations be cataloged? Will they be accessible and open to everyone?

We are still testing the peer to peer communication platform on a small scale, but the idea is that this will be a forum in which conversations are discoverable (by hash tags and other search methods) and available to everyone in the community. Part of the idea is to spark the conversations, but another big part is curating these conversations so that they are useful to people who come after.

What about privacy issues, how is the data protected? If you are collecting all of this data from various stakeholders, is there an opportunity to analyze that data?

Privacy and data ownership are important issues. We start with the principle that patients/parents own their data and should be the ones to decide what their data are used for. Many patients/parents want their data shared more broadly, especially in a de-identified format. We are working closely with key stakeholders to craft policies that balance privacy and sharing.

Again, please do not hesitate to contact us if you have other questions to ask, ideas to share, or are just simply wondering what's going on! You can email us at C3NCF@CCHMC.ORG, visit us on Twitter @CF_BigIdea or on Facebook at The C3N Project

We're having a party

I hope that my last post wasn't perceived as a complaint. While there may have been the undertone of "get me out of this hell", I do love this crazy life. I could do without the CF and all of the whining and fighting, but I bet that one day, sooner than I'd like, the neediness will be gone and I will long for it. And not all days are like the day I described. We are busy, no doubt, but I do find time to do some things that I really enjoy. I've been taking a lot of pictures again lately and that makes me happy. I've also had the opportunity to set aside some time to do more work with Cincinnati Children's Hospital on the C3N Project that we're creating for Cystic Fibrosis. We have a webinar on Wednesday to introduce it to some influential members of the CF community and I'm both anxious and excited.

It's an amazing thing that we're working on, but it's a hard concept to wrap your head around. We've sought out people who are already very engaged in their health and care at a number of different levels, people who would naturally fit into this new model of health management. I just hope we don't overwhelm or scare people away by the novelty of the idea. It's understandable though, when we say we're going to change the way people manage health and care and are shifting the culture to be one of active participation by all parties - doctors, patients, researchers - for people to think to themselves that it's a cool idea but we haven't a chance of actually doing it. But we do my friends. I do believe that we can, and the CFF believes that we can, and it's happening, starting on Wednesday!

I had a really great call this weekend with the other e-patients that I'll be sharing the stage with at Stanford MedX in two weeks. We had the chance to get to know each other a little bit and talked a little about what being a "patient" in 2013 means, how does the role of social media affect that, how did we become empowered and how do we empower others. I've gotta say that i'm most excited about the questions from the audience and from Twitter. I've never been on stage in front of a lot of people, and I'm a little nervous that I don't actually know anyone else that will be there, except virtually through Twitter, but I'm comfortable with what I know and what I do and I'm confident in my thoughts and beliefs about health and care. I think this conference is going to be amazing and I'm very much looking forward to it. I'll be tweeting at the conference (I'm @ekeeleymoore) or if you just wanted to hear all of the amazing people tweeting about the conference, you can follow hashtag #medX. The other folks I'll be talking with are Chris Snyder @iam_spartacus,  Emily Bradley @chroniccurve ,  Jody Schoger @jodyms  and Joe Riffe @dirtemedic. These are empowered patients with valuable perspective to provide, you should check them out!

Schools gotten off to a pretty good start for Drew. I've been overly impressed with the engagement of the school - calling me at snack time to find out how many enzymes he should have for a given snack, wanting to know if he can have different things to drink when I don't pack him a drink, and even a call from the teacher to tell me she has a bit of a cold and asking me how we should handle it. When I asked if she could just be diligent about keeping his hands clean and as much as possibly keeping a little physical distance until she was better, and that we would hope for the best, she responded with "Whatever it takes, we are in this together.' I almost cried. It's so comforting to know that we made the right choice. 

Despite his colonization with achromobacter, he's really been doing well lately (knock on wood). We were able to get his weight up and he's now in the 71st percentile for BMI (the goal in CF is usually to be above 50th). He's not a great eater but he will drink PediSure  morning, noon and night, and that in combination with the little that he does eat has him doing alright. He's wise beyond his years, and a little gentleman already, holding doors and always saying please and thank you. I hate that he has to have CF, but he's doing alright.

If you live under a rock and haven't heard about our Oktoberfest: Drinking for Drew fundraiser coming up on Sept. 21, you're invited. Everyone is invited. We have live music, tons of food, delicious beers, kids activities, vendors and a silent auction. You should come, and bring some friends. It's $40 per family or $25 for an individual. It's gonna be awesome!! (Click here to RSVP)

I admit it, I'm tired

I've gotta be honest, i'm exhausted. This whole back to school thing is kicking my butt. It's not just the back to school, it's also the extra hour and a half of treatments that we now need to find time for in a day, and the swim lessons, and the dance lessons, and our upcoming fundrasier (which will be awesome). This is how yesterday went:
Alarm goes off at 6:45. I know that's not early for many people, but the Moore family rarely sees the 7am sun. We didn't get out of bed before 8am but a few times all summer. Now school starts at 7:45. So the alarm goes off and I go to wake my daughter for school. We are dressed and downstairs by 7. I simultaneously make her breakfast, make myself coffee, and pack her lunch. By 7:30, my husband is downstairs, as are the other 3 kids who all want food, drinks, and a different cartoon on TV. I spend about 30 minutes pacifying that bunch while #1 heads off to school with dad. By 8am, I start to get Drews medications ready, checking the calendar to make sure I'm not giving the Tuesday-Thursday-Saturday pill on a Monday, Wednesday or Friday. The liquid antibiotic that he's on for inflammation, not antibiotic properties, is only given on Monday-Wednesday-Friday, but while a small bottle yields a little more than 12ml, it's only good for 7 days after mixing, and at 3.6mls three times per week, I need to try to recall when this bottle was mixed as there seems to be enough left for another dose, but it might not be good anymore. I dispense all other meds and assemble nebs, mixing up our inhaled antibiotic that literally smells like cat urine. It goes in a different kind of neb cup, but when Drew sees it he  freaks because 1) a mediction that smells like cat urine is misted into a mask strapped to his face and he knows how awful it smells, and 2) he knows that when he sees that neb, he has to sit for twice as long as a regular treatment beause it takes nearly 30 min to nebulize that medication. So I mix the medicine, hide the neb, and tell him its time to start treatments. It's now close to 8:30. This is problematic for a couple of reasons. First, this treatment will last around an hour. He is supposed to get the inhaled antibiotic 3x a day, spaced every 8 hours. There aren't enough hours spent awake for him to accomplish this, so we settle on trying to evenly space them. The other problem is that swim lessons start at 10am, which means that between the time we finish at 9:30 and 9:45 when we need to leave, I need to get two 3yr olds into swim suits and shoes, find towels, buckle in three kids, grab something to entertain the baby, and back out of the driveway so that we arrive with enough time to use the restooms before getting into the pool. It may seem silly that we do swim lessons...or dance class...or tball, what with everything else we have going on, but staying active plays a big role in staying healthy with Cystic Fibrosis. And while there may be no scientific proof to this, since we started swimming, the severe sinus disease has all but disappeared. I wouldnt want to be the guy swimming next to my little man, cleansing those funked up sinuses, but if a little chlorine can do the trick and keep us from another sinus surgery, then we will be efficient with that 15 min window that we have to get our act together. Move it or lose it people.
Swim lessons are only 30min long, thank God, because we need to get home to eat an early lunch because, you guessed it, we have another treatment to squeeze in before we leave for school at 12:15. Three year olds move at warp speed in almost all areas of their life, except for changing out of a wet swimsuit. This is also when the independance that I pushed them so hard toward comes back to bite me - they do not need my help, they can do it themselves. I try to be patient and watch and wait but we're on a tight schedule and FOR THE LOVE OF GOD why is it taking you so long to put on underwear!?! Focus people!
We manage to eat a quick lunch and get in a treatment before its time to buckle everyone in, again. Drop off is at 12:30 and we are all ready for it. Now its just the baby and I, with a quick stop at the post office to make before heading home. I imagined the post office to be quick and easy with just 1 kid in tow. I have a wild imagination. If I could send a package via carrier pigeon from my back porch, I'd buy a damn pigeon.
We're home before 1 and we're both ready for a nap. He gets one, I get to do the dishes, the laundry, the prescription reordering, the insurance claim negotiting, the [brief] treadmill walking, the dinner making and then the baby waking because at 3pm we need to pick up #1 from kindergarten. Back into the car we go at 2:45 and head off to school. #1 is tired but happy to see us and have some mostly uninterrupted attention while we wait for 2 & 3 to get done preschool.
At approximately 3:30 pm the fighting commences - because it really does matter who gets to the car first, who gets buckeled first, who had the best day, who made the first song request, who asked to go the back way home, and who isn't being treated fairly, by me, on our 5 minute drive home.
After we get in the front door and clothes are changed and drinks are distributed, there is a consensus to go outside to play. It's really not fun at all, by anyones standards. Exhaustion, in my house, shines through in the form of fighting, whining and crying. We remain outside until dad drives up, home early so that I don't pull out all of my hair. I warm up the dinner that I made earlier in the day, everyone complains that they don't like it, and I decide I'm not making anything else. Aftter all, if they starve to death there will be fewer mouths to feed!
At 6:30, its time to start the third and final treatment of the day. The kid are filthy, so we divide and conquer, one of us mixing and organizing medicines and devices, the other dealing with tired, dirty kids. The whole evening comes to a close shortly before 8pm when teeth have been brushed and stories have been read and four little people who I love more than anything in the world are ready for sleep.
We spend the next hour cleaning up our house. If someone had come to rob us, they would swear our house had already been hit. Baskets of clothes emptied to be used as cages, food and drinks scattered about, "artwork" taped to any and all open wall space. By 9pm, life seems to be in order. I want a drink, and I'd have one but being anything less than 100% the next day leaves me at an even greater disadantage than I'm already at. And lets be honest, I need more than just A drink. For an hour or so, I feed my Twitter addiction in between ordering school photos, registering for the PTO, sending out sad emails begging our families to purchase something from the magazine drive so that #1 can dress down one day next week, and checking the calendar to figure out how tomorrow needs to run.
I finally get to bed a little after 11pm, but, SURPRISE! The day isn't over yet! At 3am we are awoken by a screaming, bloody 5yr old who fell out of bed and split her forehead open. If we had a suture kit in the house, I would have considered sewing her up myself because 1) who wants to run to the ER at 3am and 2) we were literally just there two weeks ago getting staples in someone elses head for a trampoline injury and I'm not in the mood to hear the lecture on keeping my house safe for my kids. One of these days they'll call Child Protective Services and I'll contimplate the relief it would be if they took them away for just a few hours until they read my blog and realize that I'm just a mom trying to do the best that I can.

Not out of obligation, but out of love

Drew still has achromobacter. We just got the test results a few hours ago. We'd spent more or less all summer on a series of inhaled antibiotics, spending 3-4 hours every day, at home and on vacations, to try to eradicate this antibiotic resistant beast, but to no avail. I guess I had accepted that we probably we never really going to get rid of it, but I had still hoped that it would happen. We have another clinic appointment tomorrow morning to figure out what's next, but I think [read: know] that he will be considered colonized with Achromobacter and we will start to cycle inhaled antibiotics. If we have to live with this, I hope that we can at least keep it suppressed to the point that it's not causing any problems, seen or unseen.

Preschool starts on Wednesday. We're going in "healthy" and I'm not looking forward to the chaos that early fall will bring with exposure to 18 three year olds and all the germs that accompany them. I met with Drew's teachers last week and am happy that we have chosen to send him where we did. I am hopeful that they will do everything that they can to keep this disease invisible to him and help him to learn and grow the way that I hope for him to. I think he's going to love preschool, and if he gets sick and has to stay home, I think it's going to be a major bummer. There isn't much that we keep Drew from doing, not much that the CF prevents him from participating in. But man, those times when he's got his heart set on something, even something simple or insignificant, and we have to stop him because he needs a treatment, or needs to be hooked up to an IV, he gets so bummed. Those are the times that my heart hurts. But then I curse CF and get all fiery and go trying to change the world (it's happening, more on that later).

The other [selfish] reason that I'm hoping Drew's health remains good is because I'm attending two conferences this fall and leaving the family in the good care of my husband. Not that he wouldn't be capable of taking care of Drew if he were to get sick, but Drew getting sick turns this family on it's head. With 4 young kids, some in school, some in activities, all needing care at all times, it's a nightmare trying to keep things in check when one of us has to be in the hospital with Drew or even at home managing administration of IV medications on top of all that we already manage in a day. 

Speaking of conferences, I was invited to speak at Stanford Medicine X ! I'm going to be on a panel about what it means to be a "patient" in 2013. I don't know if I ever announced it on here, but the CF Foundation is moving forward with a partnership with the C3N project at Cincinnati Childrens. (Yay!) I talked to people and shared my story and shared my vision and started tracking and started engaging in Drew's health at a different level and the results have been outstanding, and some prominent people in the healthcare world have caught wind of this and that's how I got the invite. The stuff that I'm working on at Children's isn't rocket science. It's engaging healthcare professionals in new ways, and sharing ideas about things that could improve health and care, not just in Cystic Fibrosis, but in chronic diseases all around. I'm showing people that everyone has expertise, that I can learn from them and that they can learn from me. And at Stanford at the end of September, I'm going to learn and to share with a ton of great people. I'm honored, excited, and just a little bit nervous to be in the presence of such an incredible group of people. 

The other conference I'm going to is the North American CF Conference in Salt Lake City in October. I'm totally pumped about that one too. While many of the other participants there (docs, other clinicians, researchers) might not feel the same way that I do, I'm there to learn but also to share and hope that they will be able to learn something from me too. I won't have a podium or an audience at that conference (not yet!), but I will talk to people about my vision for a better future and how we can all work together to get there. 

Another exciting thing that happened this summer is that I was interviewed for a piece on NPR about the "Quantified Self" movement (you can listen by clicking here - the whole thing is really awesome and I highly recommend listening if you have the time, but my interview starts around the 24 min mark). 

On Friday night, we went to a CF Fundraiser that another family that has a child with CF was hosting in Northern Kentucky. I'd never met them before, just heard about their event through Facebook and knew of another family that might be attending. We decided to go, both to support the family and the CF Foundation through the fundraiser, but also to show them that we care, we get it, we've been in their shoes, we know how it feels day in and day out, always hoping for the best, sometimes dealing with the worst. It ended up being a really great night, in terms of money raised for CF and the company that we had for the evening. 

Our Doin' It For Drew Oktoberfest Fundraiser is coming up on September 21 (all are invited, bring your friends!) and as we go into another fundraiser, I trudge through realizing the importance of these funds that we raise, but hesitant to ask the same people for another dollar, more of their time, more of their energy. At the event on Saturday night, they had programs and I have to share the message that they wrote on the back because I couldn't have said it better myself. It may not have been written by me, but the message that I share below comes from my heart. 

"There's little we can say to show our gratitude for your generosity & support. There have even been moments we've felt guilt for all that has come our way; that we were asking too much or putting those around us in a place of obligation. But then we realized something that deep sown we had known all along. That God had been preparing us for this our entire lives, and that through our [son] we would learn of you, our "silent army" that has been surrounding us all along". 

Thanks to all of you, our family, our friends, complete strangers who we've been touched by our story, we've raised $25,000. We hope and pray that you will continue to fight with us until CF is a thing of the past!

Unaccountable

I've been reading this great book called Unaccountable: What Hospitals Won't Tell You and How Transparency Can Revolutionize Health Care. It's really good, and a lot of what's in it is stuff I've been working to change. One of my favorite quotes from the book is this: "In short, data transparency, properly weighted, would empower patients to make informed decisions about where they should spend their health care dollar. If we had more of it, the accountability visited on hospitals would revolutionize the quality of medical care in every city in America, dramatically reshaping our health care landscape." I do believe that to be true. 

We just spent a week in Michigan with my husbands family. It was so nice to get away for a few days. Drew has been doing pretty good lately, knock on wood. We're about to finish up 60 days of inhaled antibiotics. I have little hope that the achromobacter will ever be eradicated, but it's been nice to be cough free and generally healthy. What I suspect will happen is that he will continue to culture the achromobacter and we will cycle 30 days on/off of an inhaled antibiotic. I just hope, so so much, that the 30 days off won't be symptomatic and causing him harm. I guess only time will tell. We finish up this Friday and will do a culture next week. He sees the doctor again two days before he starts school so hopefully we will at least get a clean bill of health to start out the year.

School is definitely been on my mind. I'm trying not to worry to much. I know that he will be great, that he will love it and enjoy being around others and learning and playing and just being away from me for a bit, but I worry that he's going to get sick. No one takes care of him like I do. No one follows him around with hand sanitizer to make sure his hands are clean before he eats and drinks or after he's been outside playing like I do. No but me makes sure that the kid next to us at storytime isn't coughing and sneezing up a storm. We have never missed enzymes at any meal or snack or drink ever. And now I have to give some of that responsibility over to Drew and to his teachers, and I'm definitely stressin'. I know it will all work out, but it's just a change and sometimes change can be hard.

I wish I had more time to write on the blog. Summer has been great, but very very busy. When school starts in just a couple of weeks, I'm hopeful that I can find some more time to jot down more of the things I'm thinking about and working on!

If we all work together, everyone wins.

If you're unfamiliar with my blog, I have a 3yo son who was diagnosed at birth with Cystic Fibrosis, a progressive and fatal genetic disease for which there is no cure. On this journey, we have the support of many family members and friends, an outstanding clinical staff, and a National Cystic Fibrosis Foundation Center that is transparent with its outcome data in an effort to promote awareness and research and fund a cure. We're getting there, but it can't happen soon enough. I spend a lot of my free time trying to think of or find ways to make life easier or do things better, things that could have an impact for my son and for other people in the CF community, and ultimately change outcomes. From medication management to insurance headaches and everything in between, I have an image of what life with this disease could look like. It would involve seamless tracking of symptoms and medications and treatments. It would include the opportunity to communicate with your doctor in between visits in a way that didn't involve phone tag. It would allow transparency in all data, allowing me to see the results of my tests and the notes that the doctors have on how I'm doing, eliminating duplication and error. I would more easily be able to find information on my disease that is relevant to me without having to stumble upon it on Facebook. And as for the things that I don't know I don't know? My doctor could be proactive in providing those things to me through the collection and organization of information that is already being shared in chat rooms and on social media. All of these things would be organized and tested and implemented so that I'm not the only person benefiting from what I want or what I want to know. 

I started tracking Drew's health a couple of months ago. I record things like his weight and cough frequency as well as his appetite, stooling patterns, oxygen saturation and daily calorie intake. I've been able to share this information with his doctors to give them a more complete picture of his health in between visits, them learning about obstacles that we face in adherence to daily care regimens and me getting suggestions for ways to overcome some of those obstacles. We've worked together on additional measures to track and have discussed many ways to make life with this disease better while we wait of a cure. Being an active and engaged parent in a complicated chronic disease, what I want most is something to help alleviate the burden of care - the time it takes, the money it costs, the energy is soaks up. Enter Ginger.io.

Ginger.io is a behavioral analytics platform that turns smartphone data into health insights. I think we can all agree that people behave differently when they aren't feeling well. By collecting your behavior patterns - how often you text, the times of day you make phone calls, different movement patterns - Ginger.io aims to map behavioral patterns to create insights that, long term, have the potential to predict and prevent future problems. It's still being tested out, but I hope the Cystic Fibrosis Community and all chronic conditions will be able to benefit from a tool like Ginger.io. 

Cincinnati Childrens has teamed up with Ginger.io to develop a research study for Inflammatory Bowel Disease (IBD) patients to track their symptoms. As part of this study, patients can even get paid for tracking their data.  What if finding a way to treat one chronic illness like Crohns Disease, with something as simple as a mobile app, could help to find a way to treat another chronic illness like Cystic Fibrosis or Sickle Cell Disease. If you know anyone with Crohns or Ulcerative Colitis between the ages of 13-25, have them check out this study. If we all work together, everyone wins. http://ginger.io/join/c3n/

Good things keep happening!

Lots has been going on around here lately. In addition to it being a packed summer full of swim lessons and play dates and vacations, the CF Foundation has decided to move forward with a partnership with the C3N project! YEAH! We were able to share with them the value that we see if collecting and sharing new ideas and ways of managing health in CF and how the C3N project is just the place to do that. I've been keeping busy as we start to kick this thing off, creating a brand for it and figuring out a communications plan and implementations strategy. Late this week, I'm having a meeting with some folks from the foundation to share the data I collect on Drew and discuss other things that we could measure and what we could learn from the stuff we are tracking. We are also working with the UC Design School to figure out what this project needs to look and feel like so that it will work for everyone.

Drew is doing well. We had a regular clinic visit about 2 weeks ago right after we returned home from Philadelphia and our little adventure downtown for a night. His weight was down a little, which wasn't surprising given that he doesn't eat anything. We decided to start tracking his calorie intake and charting it in my PersonalExperiments with the rest of the data I collect to see what he is actually getting in and where there's room for improvement. We also started giving him Ensure Plus instead of just Ensure. I've been so focused on fat, so we decided to shift that focus to calories for a while and see what it gets us. After just two weeks of tracking, we are getting in over 2,000 calories most day, and his weight has gone up .2kg each week over the past two weeks! While keeping a food diary certainly takes some time, MyFitnessPal has made it incredibly easy for me to quickly track both what I'm giving him and then by adjusting serving size I can track what he actually takes in. The dietician can log in to see his data and his calorie distribution throughout the day, and offer suggestions based on what he seems to enjoy or what meals he seems to eat the most at. I take the total calories in a day number from MyFitnessPal and put it into the PersonalExperiments and then I can see how his stool output or cough frequency relates, if at all, to his appetite and calorie intake.

The other exciting thing that happened is that we found an electronic stethoscope company who is interested in partnering with the C3N project! They are going to let me test out the tool and we will work together to see how we can quantify breath sounds and record them and append them to the data I already collect to see what value that can bring to care. I don't have it yet but I'll definitely be updating the world once I get it and start using it.

That's all the updates I have for now. Trying to keep cool and busy this summer, and next week we head to Michigan for a little vacation. More updates as they break!

I lived my "What If..."

A few months ago, I shared a story with the CF Foundation about my vision for the future of chronic care management. If you missed it, you can read it here. Last week, I lived my "what if". Let me explain.

About two weeks ago, we packed up shop and headed east for our summer visit to the grandparents house just outside of Philadelphia. About a week into the trip, Drew developed a cough. He'd been doing pretty well and so this cough was new but not anything that I was terribly worried about. Two of his siblings had runny noses and coughs and I'd figured he had just caught the virus that they had. After a few days, the cough frequency was on the rise and his oxygen saturations were on the decline. I track many of his symptoms/behaviors with a tool called PersonalExperiments so I could see from his data that this was the case. I put in a call to his Pulmonologist back in Cincinnati suggesting we start an oral antibiotic and after some discussion she agreed that that might be the best next step.

Later that night, Drew went into some serious coughing fits like I had never heard before. He would cough and gag for hours on end, getting little to no relief from breathing treatments or airway clearance. He finally fell asleep around 2am, but woke up worn our and not much better. The next day he had another one of these intense coughing spells, and his doctor and I felt that he might benefit from an oral steroid as his symptoms seemed to indicate that there may be some serious inflammation causing the intense coughing fits. That evening, we started both the steroid and oral antibiotic and he went to bed. Then, at 11pm, 2am, 4:30am and 6:30am he was up again, coughing and gagging, still getting little relief from breathing treatments, so we decided that it was time to head to the ER.

We arrived at CHOP around 11:30am and were immediately taken back into a room. The doctors and nurses listened to my whole story and I shared with them the data I'd been collecting so they were quickly able to see what had been going on. I also gave them access to his EHR through MyChart, where they saw his bacteria sensitivities from his most recent culture and decided which antibiotics would be most effective in treating his exacerbation. We had checked in, gotten an x-ray, done a viral panel and an epiglottic culture and were admitted and moved up to a room in under 2 hours. If you've never had the opportunity to visit the ER, that quick of a conversion from arrival to admission or discharge is unheard of.

Once we were up in our room, we were greeted by a resident before we had even been screened by our nurse. She asked to see the data that I had been sharing in the ED (good news spreads quickly!). I shared my data with her and expressed my apprehensions about this being an exacerbation. An exacerbation is pretty much defined simply as a temporary worsening of the lung function due to an infection or inflammation. Although no formal definition exists, an exacerbation is generally characterized by the following symptoms:
1. Shortness of breath
2. Fatigue
3. Increased cough
4. More productive cough
5. Drop in FEV1 or other markers of the pulmonary function tests

The protocol at many hospitals is to admit and treat with IV antibiotics if a patient is exhibiting these symptoms, as the assumption is made that they are suffering a pulmonary exacerbation. I did not believe Drew was having an exacerbation. While I haven't been collection data for long enough to have caught an exacerbation, I recall him being symptomatic 24/7, and Drew's serious symptoms seemed somewhat isolated to sleeping or laying down. I had notes in my tracking that called out the times that these things were happening, and at 3pm he was running around the hospital room and playing without a care in the world - asymptomatic. I thought that Drew had caught the cold that his siblings had and while he was sleeping/laying, the post nasal drip was throwing him into these coughing fits. His symptoms just didn't fit the bill of his "typical" exacerbation.

Based on the data I shared from his EHR, the attending doctor came in to discuss the IV antibiotics that they wanted to put him on. If I was going to agree to IV's, they chose the two that I would have also picked based on the positive response he had to them in the past, but I wasn't ready for IVs. Once you start two weeks of IV antibiotics, it isn't common to stop them until they're complete. I once again pulled out my data and had a discussion with the doctor about waiting to see if the steroid and/or the oral antibiotic that he is on would kick in. Since we were already in the hospital, he agreed to monitor Drew overnight before starting IV's on two conditions: I would agree to start the IV's if his oxygen dropped any lower (he was at 92) or if he had any more coughing spells overnight. He had started the steroid the night before so had only had 1 dose of both that and the oral antibiotic, usually not quite enough time for Drew to respond. He was also on inhaled Colistin as we work to eradicate his Achromobacter. The benefit of Colistin is that is covers pseudomonas and the Cipro he was on covers a number of other bacteria that he has grown in the past. It just seemed to me unlikely that he was having a true exacerbation. 

So we hooked him up to a Pulse Oximeter and put him to bed. Moments later, a doctor stopped in to tell me that his culture came back positive for Rhinovirus, otherwise known as the common cold. It is known to cause a lot of inflammation and it's not unheard of for kids to have lots of coughing with this virus. Kids who have tracheomalacia, Drew, often do end up in the hospital because their airways are under attack more than they can handle on their own, and many times need a steroid to help reduce the inflammation and help them to get over the hump. For the first night in 3 nights, he slept all night long without coughing. His oxygen levels gradually started to go up on their own overnight. He woke up in the morning refreshed and looking/acting well again. I needed to take him to CHOP that morning because I was starting to reach the point of uncomfortable managing it from home in the case that things would get worse before they got better. But had I given it one more night, one more chance for the steroid to kick in and do it's job, we probably could have avoided the hospital altogether. 

We were discharged at 10:30am the next morning, just under 24hrs after we had checked into the ER. Because I had been tracking his health so closely, because they were able to access his previous test results, because I felt empowered enough to speak up and express my perspective and desired course of action, and because I had given them the evidence to trust me, we found a mutually agreed upon solution that saved us two weeks in the hospital. It saved Drew the stress of being in the hospital for 2 weeks. It saved me the trouble of trying to arrange my life for a 2 week hospital stay. It saved the doctors and the hospital time and money. My insurance company wasn't being billed for unnecessary tests and an extended hospital stay. 

I have data on Drew. I know the signs and symptoms of a pulmonary exacerbation. I know the difference in his cough frequency or appetite that is associated with the onset of symptoms. I know what has worked for him and what hasn't. Having that data and sharing it with anyone and everyone who might be able to better help him or benefit from it personally is why I track it. I lived my "what if" and I couldn't be more pleased with the way it turned out.