I'm tired of being the lone nut

Take a quick moment and watch this video - 3mins, worth your time - https://www.youtube.com/watch?v=V74AxCqOTvg

I am the lone nut, doing what often feels like a drunken dance in the rain. My drunken dance is one to say that what we have today isn't good enough, and we, the CF Community, know more and can do more and can do better, but we have got to do it differently. When I'm refusing to settle for the status quo, to accept that change happens slowly, being reprimanded for disrespecting and hurting people's feelings when I speak what I believe to be so true, I am the lone nut. When I'm pushing against the inertia of the current healthcare system, one that is failing us all in a big way, people stop and stare but they don't understand, and I'm still the lone nut. But this is about all of us, the cf community! The stakes are too high here, you see. Coming home from NACFC where I realize once again just how little we know about the best treatment options for any given bacteria or fungus or patient, where the clinical recommendations for cleaning equipment vary center to center and contradict the recommendations of the CFF guidelines (if they exist) and the manufacturer recommendations. What do we do?Continue blindly into the next phase? Or must we say STOP! Enough!! Fucking enough! This isn't a game, this is my kids life! 
I had the opportunity to listen in on a call today about the new CF Experience of Care Survey that will be going out to all care centers so that the CFF can collect more data on patients and try to better understand our experience of care. But the experience of care is not one sided - its me and my doctor or care team - yet we aren't surveying them. What if both parties involved in this relationship were surveyed and the improvement efforts involved closing the gap between the two? While I understand the intention, its misguided. Patients, the most overworked and under appreciated member of the healthcare team, are once again being asked to do more, to give more, so that someone else can learn. Its my damn data, and I want to see the results of it. I don't want my care team to be the gatekeeper of it. In fact, care teams are being incentiveized to get patients to fill out this survey twice a year, and the patients still gets nothing more than more to do. If information is generated about me, I deserve access to it, and I want to be part of the improvement efforts to make right whatever is wrong. Can we please put the patient at the center of something? 
I've heard from so many people over even just the last 6mo about their frustrations with the status quo in CF Care. These are people who have great ideas and great desperation as their lives or the lives of their children or grandchildren are at stake. I'm telling you that I cannot be the lone nut anymore.  I'm begging you, you cf mamas with passion burning deep for your kids, you cf dads who are using their brains and the love of their kids to hack the system and are desperately trying to find ways to share your brilliant solutions. You sisters and sister in laws who are fundraising, advocating, praying for the cure. The grandparents who can't bear to see their own children suffer through the physical and emotional exhausting of parenting a chronically ill child. We need to stand together and say ENOUGH!! We need to do something about this! This applies across conditions, across generations.  We cannot continue to do more of the same and expect somethings different. We should not be the advisors in healthcare, we are the leaders. We are not fitting into this broken system so let's turn it around and make the healthcare system fit into our lives. Let's share what we know, with pride, and cure these diseases. I'm starting a movement, but I can't do it alone. 

If you're curious and you want better than what we have today, join the convo on Smart Patients where we are talking about what really matters in our lives, and where major decision makers are listening (www.smartpatients.com/cf). 

Watch these videos that show how patient voices are making advances in health care: https://www.youtube.com/watch?v=Zsn_9EplBz8&feature=youtu.be

Here is video showing how parent voices in the IBD disease community are making a difference, and is a model for the work that we are doing in CF: https://www.youtube.com/watch?v=mljKTqHcdiA

Sign up to learn more about your job in this movement; we all have one, and we need you all  https://www.surveymonkey.com/r/CFCareModelDesignProject

Drew Update

Last Friday, Drew had a bronchoscopy so that we could see what his lungs actually look like in real life (vs on a CT scan or MRI) and so that a good sputum sample could be collected from the depths of his airways. They always add a little sterile water into his lungs to wash things around and then suck it out to test to see what kind of bacteria it grows so that we can target our treatment. Different bacteria responds to different antibiotics, and viruses & fungus are a different game altogether, so its good for us to know what he has going on so that we can treat it appropriately.
I was prepared for the anxiety filled wait for results, when on Sunday I got a call from his doctor. My mind always assumes the worst when the hospital calls when I'm not expecting them too. She was calling to tell me that they had found a fungus called histoplasmosis, a fungus that is common in our part of the country, but that there was little data available on for treating in CF. She had called me on a Sunday knowing that she had a busy week ahead and wouldn't likely find time to talk, and wanted to walk through some options. When a healthy person has histoplasmosis in their lungs, you don't even know they have it. It sometimes shows us as a calcified lymph node in an x-ray many years after they've had this, but it typically isn't something that's treated  because its something that typically isn't even known about. Now, in CF its a different story. As it goes with most of the things we find in Drews airways, we treat based on symptoms. But the million dollar questions is does he have symptoms. He coughs a bit from time to time - I don't think hes coughing more than normal - but his latest CT scan showed some evidence of bronchiectasis and we were wondering if perhaps the inflammation caused by the histo was setting off a cycle of infection and causing lung damage. There isn't really a good way to figure this out, except trying to treat the fungus and repeat the CT scan to see if we notice a difference. There's all sorts of other complicating factors, like the fact that the typical treatment for this fungus cannot be taken with a PPI (Prevacid), something that we identified he needs to be on, so we need to consider other treatment options.
ANYWAY, because the lab report wasn't final and fungus is a slow growing beast, it not looks like it wasn't histo down there, but rather another fungus called Candida, something which we also know little about in CF. Drew great that once before and we didn't treat it and it went away, but we don't know if it caused lung damage or if the achromobacter that he's had for a long time is causing some silent damage. So we know nothing really, still waiting on more tests, not changing the current treatment plan for now, waiting to see what else we can learn before making a plan to move forward.
Its really frustrating knowing how little information there is on so many of these different things, something that was really highlighted to me at NACFC. There were tons of excellent presentations on research being done that gave no conclusion or recommendation, just the facts that we just really don't know. I think that we (CF community) need to get smarter about how we are collecting and organizing data that we gather on our own about ourselves. Aggregating this type of information can lead us in new directions, help us to put together this puzzle and really start to move things farther faster. We are getting there, but its never fast enough.

A story from the trenches (2015 NACFC - Infection Control Guidelines Session)

I remember the day a nurse walked into my sons room in the NICU in a gown and gloves, before we ever knew what cystic fibrosis was, before our official diagnosis. I remember feeling like something must be wrong with him if the nurses wouldn’t care for him without wearing a gown, gloves and a mask. I came to learn that those measures were precautionary, meant to protect him.  I came to wonder so innocently what exactly it was that we were protecting him from. Shortly thereafter, I learned the harsh reality of this disease.

Drew is 5 now, and I want to share with you what it feels like to live with CF and the ever-present threat of a new infection. The anxiety of bringing my son into clinic is great. We put on a mask before we exit our car in the parking garage and we push the buttons on the elevator with our elbows. My mind is racing as we stand in line at registration between two families that I know from the CF community. Immediately after we sign in, we are roomed for infection control protection. Its such a relief to be in there. I trust that the room that we are in has been cleaned before we are in there because I trust my care team, though no one explicitly tells me what has been done to ensure my sons safety. As the mother of a young child, I wonder if they’ve wiped down every button that he finds, the floor that he will inevitably be on at some point because he is a 5yo boy.  I worry about how the acquisition of a simple, easy to treat bacteria will change our treatment plan, how it will impact our schedule. I wonder about how we would handle the news that it wasn't a simple, easy to treat bacteria. I wonder what it would feel like for something to just remain the same. Where do I get to share my concerns? How can I join the conversation on how to make things better?

I want to tell you about my struggle with this as a mom, a CF mom. I'm always in a state of wonder about whether the choices I'm making are the right ones, for my son with CF, for my other kids, for my marriage, for our life. The information that I receive from my clinic is just one piece of what I use to shape my opinions and make my decisions. Patients don’t often see the slides that Adrienne just shared, the profound impact that infection control measures can have on outcomes. Patients don’t always know that a special negative pressure air filtration system has been put into place in their clinic, and if they do, the clinical benefit that is being recognized. They see more limits placed upon them, more boundaries that they are forced to work within. How might we change that? If we can both contribute, we both learn more.

When I Google Infection Control in CF, my options are to read an 80+ page document from the CFF, some outdated journal articles,  or a handful of blogs with information varying from totally in line with to totally against the CFF recommendations. To be clear, i’m totally in line with the recommendations. Knowing that we have data on what can help to reduce the spread of bacteria which ultimately translates to a higher quality of life for my son is good. Understanding how it’s being implemented and learning together with my care team where we can improve would be better.

For our family, we've identified priorities and built life plans and work plans and treatment plans around them.  And with an upcoming appointment or new test results hanging in the balance,  I wonder how our priorities will have to shift, who will be disappointed, how we go forward.

It could be something small, easily treatable, that he acquires next. My heart aches when I have to break the news that we have a new medication. I'm feeling guilt for adding to an already overflowing schedule. I'm thinking about the 5 phone calls over two days it will take to coordinate the arrival of the medication to treat it, and how I will have to put the daily school report from my kids on hold due to an untimely callback despite my best efforts to coordinate this while they are at school.  I feel shame from having to cancel volunteering in my daughter's classroom or miss a field trip. I'm so disappointed that he is so disappointed that I have to change our sport schedule so that we can add just one more thing to our day. And I immediately wonder what I could have done differently. While I know its almost impossible to ever know how a bacteria was acquired, I wonder. What if I had made a better effort, done something differently. These are the things that are going through my head when we go in for an x-ray and the technician isn't wearing her contact precaution gear. This is what I worry about when we have to go into the PFT lab, having read the research available on infection control that seems to indicate that most outbreaks of infection have happened inside of that PFT lab. These guidelines certainly limit the spread of infection, but they don’t eliminate. I don’t want my son to be part of the next outbreak that we are reacting to.

I have to carry the weight of the risk, even if its a low risk, against the impact on my life. This is the constant struggle of a CF mom, rebalancing of priorities, trying to normalize our life thats far from normal. There is almost always more to do and never more time to do it.

If we have information available on how to limit the spread of infection in our clinics then we must take action to implement. Where you live and what you know should not determine whether or not you survive. We have an obligation - you in this room, and me, and the folks who might be watching this livestream - we have an obligation to do all that we can, because my sons life depends on it.

When I recently returned home from short trip away from my family, it was the middle of the night and I was exhausted. As I always do, I went in to the rooms of my sleeping children to kiss them goodnight before going to bed myself. As I pulled up the covers and touched his sweet forehead with my lips, I could taste cystic fibrosis on my son. He was sweating in his Toy Story pajamas, and a little crust of salt had formed around his hairline. Its this. Its the simple kiss on his forehead that reminds me that he might not be here forever. Its the cabinets full of medical equipment and the medication nestled between the wine and chocolate syrup in my fridge that serve as a constant reminder that despite my best efforts CF is not something that I can control. I implore you to do what you can with what we know. Lets take the evidence that we have now try to get ahead of whatever might be coming next.