A rose, by any other name...

(The fact that this post is in different fonts, font sizes and colors is incredibly annoying. Despite many efforts to change it, Blogger seems to revert to the original post with all the irregularities. Sorry.)

This post is starting out as a bunch of random thoughts that I'm hopeful, when woven together, will make them feel far less random. I've been trying to sort out what happened at Flip the Clinic that caused a flip inside of me, a transformation, my breakthrough moment. It wasn't so much related to the project that we were working on, but rather the way I'm approaching the work that I'm doing in healthcare in general, the values that I hold, and what I view to be my role in this whole crazy system.

The last few posts have been about the process we went through and the steps that we were taking to figure out how to transform the notion of transition through the healthcare system at different points throughout the life cycle of a person living with a chronic illness. We had some amazing moments that gave us the ability to see the world the way we hadn't been seeing it previously, and I think we have the framework for a plan to take that work forward. But this post is going to be a little different. I'm going to start with today and work backward to see if I can figure out what changed inside of me and what it means for me going forward.

This weekend  my kids had to go to the doctor for a flu mist. I scheduled these appointments in September and the earliest that our pediatrician could get them in was yesterday. They called on Friday evening to confirm that they would be flu mists, and the kids anxiously asked, again and again, if they would be getting a shot at the doctor on Saturday morning or the nose spray. We reassured them that they would be receiving the mist. So when the time came on Saturday morning, my husband loaded up three willing kids (Drew had his shot at the pulmonologist in early October) and away they went. They returned a little different. When they arrived at the pediatrician's office, they were informed, not at check in but in the exam room, that they had run out of flu mist so they would be receiving the shot. The kids felt tricked, lied to, hurt. All of them in tears, one tried to take off and the others had to be restrained by several nurses and my husband for a quick and simple stick in the leg. And i'm not even really that mad that they had to get the shot. I'm mad that their trust in their parents, their little 3 and 5 and 7yr old trust in their healthcare providers was broken. I'm mad because we've experienced this shitty healthcare system in this way enough times that I know the implications of that simple and innocent change down the road. Next time they have to go to the doctor, it will be hard to get them to go. The ripples of this interaction with healthcare will not dissipate quickly. As an adult, I'm able to recall interactions I've had with the healthcare system that have had an impact on the ways that I behave and interact with healthcare providers today - not being totally forthcoming with information on how adherent I've been with a certain medication, for example, or not seeing my primary care doctor as often as I perhaps should. It's because it doesn't feel good to do that. 

Before the great flu shot fiasco of 2015, we tried to get Drew to take 3.5ml (thats about half a teaspoon) of a new medication that we are starting to treat a fungus that he has in his lungs. And he broke. We had pushed him too far. We were asking too much. But 3.5ml? Is that really what broke him? I had to reflect on why 3.5ml of a mediation that sort of tastes like oranges was the limit. And as I added up all that we asked this kid to do in a day, combined with a little disappointment he was feeling about a playdate he was excited about falling through, I realized something. A quote from "Being Mortal" struck me in this moment: “The terror of sickness and old age is not merely the terror of the losses one is forced to endure, but also the terror of isolation. As people become aware of the finitude of their life, they do not ask for much; they do not seek more riches; they do not seek more power; they only ask to be permitted, in as much as possible, to keep shaping the story of their life in the world. To make choices and sustain connections to others according to their own priorities. Society has dictated that debility and dependence rule out such autonomy, but it’s still very much possible to attain that.”

This poor kid just wants some control over his life. The way that we have shaped our lives around this disease was according to the priorities of our family, not of Drew specifically. And quite frankly, having never lived with a chronic illness prior to Drew joining our family, we are making up our future out of our very imagination. We don't know what you're suppose to do and how you're suppose to act. I digress. While we need to realize that there is some control that he must relinquish due to the simple nature of cystic fibrosis, we cannot simply keep asking him to do more. We need to appreciate what he has and does and how this fits into the context of the rest of his life. He's being taught at school right now about all of the food and all of the life choices that are bad for your health - watching too much tv, eating too much junk food - important lessons, no doubt. But upon some self reflection, we have to appreciate how that might make him feel, hearing the message that what we've been asking him to do is wrong, and even more how him doing it in front of his peers - eating chips for lunch everyday, drinking gatorade, watching hours of tv as he sits doing breathing treatments - how he might feel. I can only imagine his 5yr old mind struggling to rectify this - how does it make him feel? who does he trust? He needs to be permitted, in as much as possible, to keep shaping the story on his life in the world. To make choices and sustain connections to others according to his own priorities. We recognized this and are going to be talking to his healthcare team about what we can change. 

Then I started to think about this in my own life. Feeling judged or even criticized at times for all that I do or don't do, how I choose to share my perspective and experiences, I realized that as the caregiver, I too was feeling the need to keep shaping the story of my life in the world, as that autonomy is something that chronic illness tries to take from you. I think we can separate it from healthcare even, saying that we all desire that autonomy in our lives. Turning into an advocate and a designer is my way of retaining that autonomy, designing for us the life that we need out of nothing more than my imagination. 

In my first post on Flip the Clinic, I had shared about being asked at the summit what we (individually) need to let go of in order for your team to make a breakthrough" and my response being "The fear that if I stop running (metaphorically) my son will die." On the plane on the way home, I realized that I had actually accepted that long ago. That wasn't what I was holding onto that was preventing our team from making a breakthrough. With the speed (or lack there of) at which change occurs in healthcare, I've accepted that much of what I'm doing in trying to change healthcare likely won't help Drew, and that's okay because Drew is alright. I'm able to do what I need to to help him. I was running for a different reason, for acceptance and understand of the way that I think, for who I am. 

I had shared with a different stranger at the summit how hard and uncomfortable and exhausting the work that I do can feel. And her response was that I was being courageous, and that my work is critical.  So on the way home I googled courage.

Courage (also called bravery and bravado) is the choice and willingness to confront agony, pain, danger, uncertainty or intimidation.Physical courage is courage in the face of physical pain, hardship, death or threat of death, while moral courage is the ability to act rightly in the face of popular opposition, shame, scandal, discouragement, or personal loss.

Channeling my inner Brene Brown, I could finally declare that courage is a value that I hold. It is a choice that I make to show up and be seen, and I do so knowing that the only guarantee is that I will likely get my ass kicked. This is who I want to be. 

As if I hadn't received enough soul-filling inspiration from this summit, someone shared this with me, the recommendation  to watch this TED talk - https://www.youtube.com/watch?v=TaX5DUGC1CU. It's about how we, as a society, as people wanting to help, are trained to look at the problems - the lacks, gaps, needs, wants - the broken stuff. She suggests that perhaps instead of looking at all that people don't have, we should start to look at what they do.  I can give you the example of our doctor's appointment this morning. When we sat down in the office the nurse said, "So we have Andrew here, a 5yr old male with a history of cystic fibrosis, tracheomalacia, pancreatic insufficiency, more bronchoscopy's than I can count, chronic sinusitis, a bowel resection, ileostomy, and airway abnormalities. His medication list is 2 miles long but I've gotta go through it so here we go." My response was, "He's got a lot of really great qualities and successes in his life, too." She chuckled and we moved on. What if Angela Blanchard is exactly right and we can't build on broken. What if the answer lies in our ability as a team - patient and provider - to share what we have, and build what we can to find what we need. What if we can't see the solution by just looking at the problem. It is about partnership, but a different kind. A partnership focused on what we each have rather than what we each need. 

If I was asked about my priority at our visit today, my answer would have been, "To get to school before 11am so that he doesn't miss his favorite part of the day." I'm here for a sinus polyp consult, but my actual priority is to get this disease to a place that doesn't interfere with school, because that's his favorite thing right now and I think that finding a way to include his priorities into the story of his life is importat.  Channeling more from Angela, we've all been in situations, as humans, raw and painful and excruciating moments, and it's hard in those moments to think about asking people what they have, talking about their strengths, what they're good at. But much like so many of us feel compelled to help, these people, the patients/caregivers, we want to contribute too. Our stories matter. We want to tell you what we can do and what we do know and that's the first step on my path to a new story. This gives us hope, meaning, a sense of belonging. Think about how that might contribute to successful transitions, adherence, sustainability, success. We all have the capacity to imagine a future different from the life we live. The job of those who want to help us is stand beside us - not in front of us, but beside us. When chronic illness shows up in our lives and takes away some of our freedom, we truly are left trying to create a life out of only our imagination. It's a little bit like stone soup - if we all give what we can, we all have a little more, not a little less. 

So what does this all mean to our work and the project we were focused on at Flip the Clinic? Well, we discovered that perhaps instead of trying to change the system, we needed to change ourselves and the way we interact with it. Rather than focusing on transition as a point in time and trying to identify which transitions throughout the life cycle of a person with a chronic illness have the greatest impact on the other aspects of life and health, we shifted our focus to the core components of a transition point, any transition point, and the parts that we want to take to 100 and the parts that we want to take to 0 - what do we need to start doing and what do we need to stop doing in order to achieve transformation. In our 100 column we thought about things like partnership, preparedness, empowerment, shares goals and ideal communication. In our 0 column we put things like wasted time, feelings of abandonment, falling through the cracks, preventable illness and "the yuck", defined as anxiety, helplessness, hopelessness. We are living with a reality that will not go away, at least not in our lifetime, but we know that things could be better. So we moved from these temporal transitions into the realization that every interaction is a transition and we need to change the way that we interact with one another. As a team we haven't yet come up with a physical prototype, but we did set goals for ourselves. In a year, we hope to have active experimentation and a way to capture our learning, leading to pattern emergence to identify new and better ways of interacting with one another (patients and clinicians) on a personal level to achieve better health outcomes.  We changed our team name from Lost in Transition to Navigating Transition Together. We have plans to connect with one another soon to map out a path to move forward. We've also nominated one of our team members, a med student with the most beautiful soul you've ever seen, to help write our narrative, organize our learning in the form of a story and help us to share it with the world. I'm really excited to see what we can do!

I took so much more from Flip the Clinic than I had expected to. It went so far beyond the work on our actual transition flip. Some of it can't be shared, not because it's private but it was personal, and it wouldn't mean the same thing to you that it meant to me. Words truly cannot express my gratitude for this gift that was given to me through Flip the Clinic. It was transformative for me to have so many breakthrough moments, to have a better understanding of who I am and what I want and am trying to do in the world. I came home feeling like 1000 bricks had been lifted off my shoulders, with a clear eyes and a full heart for my purpose and passion, and I'm going forward 100mph, only now, in the right direction. 

"...at least he fails while daring greatly"

"Its not the critic who counts; not the man who points out how the strong man stumbles, or where the doer of deeds could have done better. The credit belongs to the man who is actually in the arena, whose face is marred by dust and sweat and blood; who strives valiantly; who errs, who comes short again and again, because there is no effort without error and shortcoming; but who does the actual strive to do the deeds; who knows great enthusiasm and great devotions; who spends himself in a worthy cause; who at the best knows in the end triumph of high achievement, and who at the worst, if he fails, at least he fails while daring greatly." ~Teddy Roosevelt

I received this quote last night from a friend and the only other thing this email said was. "This is you, my friend. Vulnerability is the best measure of courage. Show your vulnerability today." And I needed that more that I realized. When yesterday ended, well, the conference proper, I had a hard night. I had to reflect on the reactions to some of my vulnerability and it hurt, a lot. And through both the receiving of this information and the processing of this information, I was reflecting on 1 thing that was said to me here that has had the biggest impact on my decision to keep pushing the train, and that was "This is critical." Darcy Winslow said it to me and its exactly what I needed to hear that no one else has taken me by the shoulders and stared me in the eye and told me to be the truth. 

I'm constantly considering whether feeling the yuck is worth it in my current role. I know there is a lot of yuck in chronic illness, but that's not the yuck I'm talking about. Its the yuck of frustration, of disappointment, and stress. It feels good to connect with other people like me, to take on fun projects that are short and easy and have tremendous impact, not on my life directly, but so little of what I do actually does. I like that feeling. Who wouldn't? I like seeing what good is going on around me and connecting with it, partnering to learn and grow and take pieces back into my life. But the yuck is necessary, in fact critical. I think need to think less about staying in the yuck or leaving the yuck or changing the yuck, and more about how I can make the yuck feel less yucky. 

Perspective is so critical, because while some see my C3N work as "head in the clouds", that's often the way I feel about the work that's happening inside of the clinic or at the Foundation, so far from the actual reality of my day to day. But we don't know that about one another until we can share that. And that's important information to know, understanding where you're starting from, and then considering how might be find ways to communicate better to allow for this? 

Something so simple has helped me to reframe the way that I need to think about this. I know it's what I need to be doing, even though sometimes it feels so yucky, because it is critical. And I will carry on, daring greatly. 

"What do you need to let go of in order for your team to make a breakthrough"

There is so much new and useful information in my notebook that I wrote in the front of it my name and cell phone number, and then in a big box with stars around it "Reward if Found", just in case I lose it.

Holy shit. My thinking has been bent. I haven't tweeted all day because I could not turn an ear away from the conversation that are happening even for a moment. Our day started with a reflection on what we accomplished yesterday, revisiting our Lego display's and continuing to think about the problem. If you want to create a transformational solution, you have to accept that you cannot see the solution to your problem from where you are starting (depicted beautifully below, where the dot on the left represents the problem and the dot on the right represents the solution(s)). We are in the process of climbing the mountain and are close to reaching the summit.

The other thing we did this morning was start to turn insights into "how might we" style questions. Here's a couple of the things that our group came up with:

We've done a lot of diverging as a group, spending time with other groups and learning from their expertise and then bringing it back to our own group. The session I attended this morning was lead by Thomas Goetz discussing how we translate data into action. Right now there is so much latent data, data that could be useful to patients or clinicians but we can't get it or can't get it in a useful form. It's just stuff that already exists that says something to you (lab results were used as an example - I can see my cholesterol number, but so what, what does that mean to me?) 

So what do we do about this? Well Thomas shared about the 4 phases of data: evidence, relevance, consequence and action. An example of this outside of healthcare could be one of those electronic speed monitoring signs that tells you your speed as you pass by. "Your speed is" is the evidence, it is what is. "You need to slow down" is the relevance. "The decision to take the risk and keep going that speed and perhaps get a ticket, or slow down" is the consequence. And "What I choose to do" is the action. So if we think about this in healthcare, test results are designed for, at best, doctors, and at least for the patient, the person who needs to act upon it. How might a better design enable a build on that, adding to verses taking away (yes, and). 

Also related to data we talked about The Principle of Progressive Disclosure, where you get to see the highest level of the data and then can drill down into whatever level you desire. An example used here was around the weather. I can see what the temperature is today, right now. Then I can drill into that and see hour by hour, the rest of the day. I can go further and see the forecast for the rest of the week. And we started talking about how might we have access to healthcare information in a similar way, where all of the information exists but I'm able to access it at my pace and comfort level. I was a particularly huge fan of this idea and even more the opportunity for folks to self-identify their learning style and start to receive information in the way and at the pace that is most comfortable to them. I think it could have a transformation impact on patient engagement. 

Organization of data is another issue altogether, with the current trend and tendency to be the creation of a dashboard. Dashboards can be counter productive because it can be so pervasive that you can't understand change. We shouldn't give people a dashboard without giving them a steering wheel. 

So this hour (I know!! It was a LOT for 1hr!) was really just getting input and information from someone who has expertise in a different but perhaps complimentary space to help you think about your problem differently and overcome obstacles. We had to take this knowledge (my new knowledge was from the Thomas Goetz group while my teammates all split and went to other sessions) and bring it all back together to think about our prototype for a transition tool/program/system/we don't know. Some of the questions that we had at this point were how will this integrate with all that we are already doing? How do different transition points build on the next transition point? How might we turn a linear transition plan into a circular one, to identify critical relationships and key levers? We decided (or thought about) how if we could pick 1 point that has the biggest leverage then that's where we should start because it could help to carry several things forward and have telescoping impact with one thing building onto the next. We were considering things like diagnosis as a major and important transition point, and transition to school (preschool and/or college), transition from pediatric to adult care, transition from being a CF patient to being a transplant patient, just as some examples. Then we had lunch and everything changed. 

We had lunch with a different adviser named Ed Porter and he challenged us to think about the piece of this that, if removed, our system would crumble. And all of us sort of went "ah-ha!" because we realized that it wasn't a specific transition that we needed to focus on but rather the key components that make up any transition point and came up with a list that included things like it must have information (we haven't defined what kind yet), it must have collaborative communication, it must solicit perspective from both (or all) parties it impacts, it must include experience and support, it must include empathy and it must include mindful presence. We didn't define these things further at this point, but we started to think more about how we could create a new way to communicate (patients and clinicians) through these transitions points that are disease agnostic, and less about which transition is actually the most important. And after we created this list, then we were rethinking which of these is critical to the success of the interaction, which is our key lever. While they all perhaps "lift", which ones lift 25lbs and which lift 250lbs. The advice we got was to start there. To choose the lever that could have the biggest impact and then test it.

So following lunch, we as a team were to present our new thinking - where are we now - to a group of other teams and advisers. We were also asked to share the questions that we still had, and talk about where we were stuck. We got INCREDIBLE feedback from the rest of the group, questions like "What do you envision this idea to actually be? Is is an online tool? A face to face encounter? Both, and? How are you thinking about whose needs must be met? There is more than 1 person in this service relationship so do you need 3 tools (patient, caregiver, provider) rather than 1 tool? And how might the other stakeholders fill the toolboxes of the groups that they're not a part of (how do I (caregiver) give the provider the tools they need to help me transition successfully and how do they fill the patients box?) This specific example was coming from the concept of groups like Sisters by Heart that send a new diagnosis package to parents of kids diagnosed with congenital heart defects to help them get started on this journey. What a crazy good idea!! We were challenged to think about how what we create now has to be responsive to the fact that time is changing simultaneously - is the creation of this going to be ongoing and dynamic, will there be a feedback loop for continual improvement? Are the solutions we are creating going to be additive or integrative - we have to make sure that we're not creating more work for anyone, and perhaps even think about what already exists that we could derive valuable information from, re-purpose something and see it through a different lens. It was suggested that we focus on impact versus intent, that whatever we create doesn't necessarily have to be measurable, but it must be communicable.

I think that one of the greatest questions posed was what does the community already have to solve the issues of that community, and how do you build on those strengths instead of trying to fix problems. (((drops the mic, exits the room)))). Wow. We had to take all of these new great thoughts and new ways of looking at this back to our smaller working group and identify what it is time for right now, thinking about how our world will organize around what we focus on, and how do we change our focus. How do we choose that starting point? We run experiments, trying more than one option so that we have the opportunity to compare and contrast, to learn. How do we stack the deck in our favor without artificially creating an outcome? We talked about these things for a while as a group, and then ended our day by talking about all of the things that we want to take to 100% (partnership, joy, just for example), and all of the things that we want to take to 0 (fear, frustration, etc). Tomorrow we get more specific with our actual plan, it my mind doesn't explode before then!

The part I glazed over in the middle was the mid-afternoon walk where we paired up with someone that we didn't know. We were asked to find someone in the room (approx 100 people) and pair up and take a 30min walk with them, getting to know them a little bit, and to come back prepared to answer the question "What do you need to let go of in order for your team to make a breakthrough". And through the conversation that we've had over the past few days on the importance of appreciating perspective, I recognized what I need to let go of in order to better collaborate with the team and on the project in order for it to be successful is the mindset I have that if I stop running (metaphorically) that my son will die.

More tomorrow on how this all comes together and where we go from here.

Flip the Clinic

Its not really fair that I'm writing about this event that I'm at after just day 1 as there are still 2 days left of this experience, but I'm having a really amazing time here and I needed to share these thoughts so that there was room for more tomorrow.

I'm at the Flip the Clinic Summit in Denver working with a team of patients, caregivers, and clinicians on a Flip called Lost in Transition. The actual problem that we are trying to solve is yet to be fully fleshed out, but basically we are working to design strategies to strengthen communication between health centers and children who have chronic conditions, looking to improve a child's understanding of their chronic condition while they're so young so that they can better manage it when they're on their own. For me its about the power of empowerment. As for Flip the Clinic, it's is a community-driven open experiment to transform the patient-clinician experience (their words that explain it way better than mine ever could). I feel like I need to pinch myself to confirm that this is reality and that I'm actually here!

We started off the day talking about how we would be working together - how most of us know many things but that none of us know as much as all of us know. We talked about the elements of a "flipped clinic" - how it would be joyful, people-centered, nourishing, expansive and transparent. Flip the Clinic wants to be (and is, in my opinion) the Petri dish where new culture grows. I seriously couldn't think of a better analogy if I tried. We were challenged to think, be ambitious, and stretch possibility, to be open and challenge assumptions. Founder of Flip the Clinic, Tom Goetz said that he believes in the capacity of ordinary people to make good decisions, and all day long that's what we were doing.

One of the most interesting parts of the morning for me was a discussion about how we need to evolve the way we communicate. It often feels like we are downloading (just saying the things we always say) and debating. This feels like we are both projecting our opinions onto others and judging. It's very hard to be innovative when things feel confrontational. The opportunity that we have is to move into reflective and generative dialogue, which invites innovation and empathy. In the first two we are focused on the past, and in the latter two we are focused on the possibility.

After lunch we had the great opportunity to work in smaller groups with different influential folks from all different industries, and I chose to join a group lead by Darcy Winslow focused on massive complex change in the corporate world and in smaller organizations. And WOW. My mind was just spinning. I'll give you three key takeaways from our discussion. The first was that if we are serious about making changes in the world, we need to do it first from within. Take a risk. Step outside of your comfort zone. And think about how you show up as a leader. This is critical. (((((long pause so that sinks in)))))) I could have left after that insight alone, but I'm glad I didn't because the next key learning was around how we really need 20% buy in in order to create the momentum to move the change forward and make it unstoppable. I need to think about my 20%. Is it 20% of the pulmonary team? 20% of the CF community? 20% of the hospital? And to buy in to what? What's the goal I'm trying to achieve? And then while I'm thinking really hard about my 20%, we started to talk about getting the right people, and how we identify who those right people are. They're not always the people at the top, but they have to be influencers. The third key thing was the need to be multi-lingual; I need to be able to both understand and speak the language of the people that I want to engage. The fourth key takeaway and  a motto that I will live by is to proceed until apprehended. Thats more than 3, and I could go on and on, but I've more to share. 

The rest of the afternoon involved one of the more creative activities that I"ve been part of. We used Legos to build our own individual representation of part of the current healthcare system. I did our home. Then we had to explain our model to our teammates, and combine our models using the best parts of each to represent the current problem that we are trying to address. It was so fun and insightful to see how we came together to literally build the problem together, with our varying perspectives, out of Legos. 

This is our house with all of the orange representing medication and Drew doing treatments and some kids with him and others protesting in another corner of the house, and the strewn legos were representative of the other chaos in our life. Healthcare doesn't just happen inside of the hospital. 

Then this was what we all put together to represent transition. What you can't see that is one of my favorite parts was at the top of that ivory tower an executive that was representative of hospital administration or pharma or insurance sitting above all of us who are trapped in this disconnected system full of barriers trying to navigate these slippery slopes of transitions throughout the life cycle of a chronic illness. The "rest of my life" is on the periphery with my family and friends, and the folks who don't make it across those bridges don't make it. 

Today was about identifying the problem that we want to solve, not yet creating a solution for it. Tomorrow we are going to roll up our sleeves and get to work on that. And if I have to a chance tomorrow night, or if I'm up again at 4am because time zones are evil, I will continue to reflect out loud because I think that this is both important for others to see and I want your feedback on how we can make it all work better. 

Now I'm off to our progressive dinner! Cheers!

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.

Fight Song

Fight Song from Erin Moore on Vimeo.

Letting Tomorrow Wait

This post originally appeared on the CFF blog

It's 10 a.m. on a Sunday and we have received notification of new test results. My son had the CT scan on Friday and I'm surprised the results are available so quickly. While I'm overly eager to enter my username and password to see what the report says, I'm equally hesitant knowing that these results might change our lives. I worry about how the results will change his treatment plan; how it will impact our schedules; how we will handle one more thing. I wonder what it would feel like for things to just remain the same.

This is my struggle as 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 and for our lives. We've tried to normalize something that's so far from normal -- and while that's perhaps not the right thing to do, it's what we've chosen for our family. We've identified priorities and built life plans and work plans and treatment plans around them. And now with test results hanging in the balance, I wonder how those priorities will have to shift, who will be disappointed and how we will go forward.

The results could show something small and easily treatable. But I'm the one who has to find the time to manage that small thing. I'm the one who has to make five phone calls over two days to coordinate its arrival, keeping me from volunteering in my daughter's classroom. I'm the one who has to change our sports schedule so that we can add just one more thing to our day. I'm the one who has to reorganize carpool when we have to return to the doctor for a follow-up, and then have to pick up dinner on our way home because we just don't have time to do it all. I'm the one who has to answer the tough questions asked by my boy about why he has to do just one more thing.

And that's if the results tell us it's something small and easily treatable.

I'm really not complaining about all I have to do, because I often consider it a privilege to see the world through this lens. I'm simply highlighting my capacity to do so.

My time, energy and emotional capacity are not infinite and the cognitive burden of this disease is great.

It's Sunday, and I'm stuck between knowing and not knowing. While I usually have a "glass half-full" attitude, I've decided to wait until tomorrow for the change that may be coming. Regardless of the results, almost nothing can change today, so I'll live in this life for one more day and take whatever tomorrow brings ... tomorrow.

Curated Content for Your Viewing Pleasure

I've recently read some seriously amazing articles and blog posts from friends that I want to share with folks who read my blog. These people, though they may not have CF or even know what CF is, speak my language. They've found a way to put to words what so many of us, across disease boundaries, experience but simply cannot articulate. They're a bit all over the map but they're all so good. I've tried to include a preview from the posts, to share with you why I love them so much. I hope you'll enjoy them too!


A Mom Advocates for Wholeness in Health Care
“Health is woven into the cloth of life, and to divide it into separate pieces labeled ‘school’ and ‘work’ and ‘family’ unravels the entire fabric,” said Cristin. “Yet the very systems meant to support my son, Gabriel, often tried to do just that. Accessing and coordinating his services became a greater challenge than his actual condition.” "I’m not a doctor or a systems expert or a policymaker. I’m a mom. My only expertise is my experience."

Trapped in the System: A Sick Doctor’s Story
"There is no bad guy here. I love the drug company that created this medication. The price is more than reasonable. I love the doctor who prescribed it to me. My insurance company has never refused to cover my care, and has always been honest with me. The laboratory personnel are professional and competent. It’s the system — the way all these things work, or fail to work, together — that’s the issue."

How can I minimize my chances of having a disabled child?
"I am grateful to my "disabled child" for teaching me one of life's most valuable lessons: the importance of using our hauntingly brief time on this planet to do the things that matter, the things that will make a difference -- the things that are inspired by love and guided by knowledge."

Remember the mothers of sick children
For the rest of us who look at these mothers and think, “I don’t know how she does it,” know this: It’s not their abilities that are superhuman, it’s their love. It is this intense love for their child that pushes them out of bed every morning and forces them to keep going, no matter what odds are stacked against them.

Manifesto of the Brave and Brokenhearted

MANIFESTO OF THE BRAVE AND BROKENHEARTED 

by Brene Brown, Rising Strong

There is no greater threat to the critics and cynics and fearmongers 

Than those of us who are willing to fall 

Because we have learned how to rise 

With skinned knees and bruised hearts; 

We choose owning our stories of struggle, 

Over hiding, over hustling, over pretending. 

When we deny our stories, they define us. 

When we run from struggle, we are never free. 

So we turn toward truth and look it in the eye. 

We will not be characters in our stories. 

Not villains, not victims, not even heroes. 

We are the authors of our lives. 

We write our own daring endings. 

We craft love from heartbreak, 

Compassion from shame, 

Grace from disappointment, 

Courage from failure. 

Showing up is our power. 

Story is our way home. 

Truth is our song. 

We are the brave and brokenhearted. 

We are rising strong.

Hope as a primary motivator

School is finally off the ground and other than one mid-day pick up of a puking kid and a handful of forgotten lunchboxes, we seem to be off to a good start. I've had a lot more consistent time to work and to think about my work and what it is that I'm doing or hoping to do and how I'm hoping to do it.

MedicineX is coming up next week, and along with a panel of colleagues, I've been invited to share of my experiences with the C3N and the opportunity that I think it presents for the future of medicine. Reflecting on this - what is the C3N? What does it mean for me? And for you?  - really got me thinking about the purpose or value of all I'm involved with. What is the power of all of this? Why do I believe in it so strongly? And why haven't I been able to help others see what it is that I see with such clarity? 

So what is a C3N (Collaborative Chronic Care Network)? In my opinion, it is a way to organize new and existing stakeholders and resources in a disease community around a common vision to amplify value and improve outcomes. And what does that mean? That's the tricky part. I first thought that it was about tools; what do I have and use and know about that helps me, and how can I share that so that it can also help you. This is no-doubt valuable, but thinking deeper, how did I learn about those things, and how do you learn about those things. Or finding "the best" tools and resources and making them available in a different way. But much like clinical research, the value is not in the published paper buried inside of some journal or identification of what is "best", but in how that knowledge is applied, modified, learned from and spread. 

Perhaps one of the most valuable statements I've heard in regard to this C3N idea was a diabetes dad [sidebar: who is amazing] who said something to the effect of, "We (patients/parents) always need to know whats next, and no matter how much medical information the medical system gives us, we turn to our peers. We need their experience and their stories to heal us so that we can move forward." And then, I was reading this book by Brene Brown called "Rising Strong" (go buy it right now), and she talks a lot about how hope is or can be a motivator; "I’ve found that moving out of powerlessness, and even despair, requires hope. Hope is not an emotion: It’s a cognitive process—a thought process made up of what researcher C. R. Snyder called the trilogy of “goals, pathways, and agency.” Hope happens when we can set goals, have the tenacity and perseverance to pursue those goals, and believe in our own abilities to act." And I thought YES! Yes! That is it!

For a long time when I was one of the only parents involved in the CF group, we talked about needed more people like me. I remember hearing Paul Batalden at one point say that hope is the primary motivator for co-production and I think I’ve always had a lot of hope. But how do we help others to hope? I don’t think it’s that others don’t have hope and that’s why they’re not involved, but rather they feel powerless. C3N provides an opportunity to move out of that powerlessness, allowing that hope to help us to move forward. This is what I think we need to really pay attention to. If hope is the key, and I believe it is, then goal setting, tenacity perhaps through empowerment, storytelling and people connection,  and belief in our ability to act are where we should be focusing our attention and effort. 

Maybe this is where the power of MedX really lies. Maybe its not the cool innovators and disrupters who fascinate us with the novelty of their thinking, but the connection, the empathy, the shared humanity, the hope. Its the people like me in the trenches with our feet on the ground and our head in the clouds, both patients and clinicians and researchers, all sitting at the same table focused now on what we need to do next, knowing that we'll support one another no matter what and in any way we can. I cannot wait to be among my people next week!! I cannot wait to learn more about what their hope is motivating them to do, and how I can build on their hope in my only life. I cannot wait to laugh and cry with my friends from the internet, the people who I see but once or twice a year, yet feel closer to than some people I see everyday. Everyone deserves this feeling, and I believe everyone is capable of achieving it. We are the hopeful, and we will prevail! Here we go!!!!

Normalcy is my priority

The gang finally started school this week. Well, if you count staggered starts and half days. They're officially all in on Thursday. We survived summer. Praise the Lord. Thanks be to God. And Amen.

Its been an exhausting week. This is the first time we've had to wake up early for treatments on any regular schedule. For preschool, Drew went in the afternoon which gave us time to wake up, eat a nice big breakfast and get our treatments done. Now, we are waking up at 6am everyday, barely getting in an Ensure Plus and a slice of bacon, and have cranky, tired kids by 6pm. My kids are 12hr a night sleepers, so for this to be sustainable we will need to start skipping dinner and going straight to bed after homework. CF be damned.

Last time I blogged, I had mentioned a CT Scan and MRI that Drew was having. Two things came out of that. The first was the idea to make IRB protocols more easily understood by kids, and that's actually happening. I got in contact with Flip the Clinic and we are doing this. More to come on that later, but i'll just say that i'm SO FREAKING EXCITED!

The other thing that came out of the CT scan was shitty looking lungs. Boo. They weren't shitty shitty, but they weren't as clear and healthy looking as they have been in the past. My initial thoughts went to that bastard achromobacter that's invaded Drew's lungs and is nearly completely antibiotic resistant. I set up time to talk with his doc about the results, and while I waited for our meeting I searched online journals, I googled everything I could possibly think of related to achromobacter and eradication, and I turned to my peers. Oh, my beloved peers. The people that I've never actually met that I trust more than some people I've known my whole live. They're the ones in the trenches with me, desperate for answers, sharing what's given them hope. They track their treatments, get into deep and thoughtful conversations about options, sharing the recommendations of their doctors and what's worked and hasn't worked for them. I took all of this learning into a conversation that my husband and I had with Drew's doctor two weeks ago.

She started by saying it wasn't that bad. There was some mild lower lobe bronchiectasis (irreversible lung damage) but that it was oddly in the lower lobes of his lungs, whereas the typical progressing of lung disease that they see in CF starts with the upper lobes. She said that the lower lobe damage looks to be more consistent with something that you might see with aspiration. Hmmmmm. Queue my mind back to our situation this spring with the weird cough that wouldn't quit that turned out to be a reflux issue. Could that have been so bad that it caused lung damage? We won't ever have a definitive answer on that, but it sure does make you wonder. And as a sidebar, it makes me crazy mad (not at anyone in particular, just at the fact) that in 2015 we don't yet have a way to look at patients by ages or genotypes or medications they're taking to identify success rates or complimentary treatment options or symptom checkers that others might have experienced that could help us to quickly identify or eliminate what might be going on. It's on my list of things to do.

Anyway, because we don't know what caused the damage and while its possible that it was from reflux, its also possible that the achromobacter treatment plan that we are currently on isn't working as well as we had hoped. There were a couple of options that we had discussed. One was trying a new combination of inhaled antibiotics. Right now Drew inhales Tobi and Ceftazadime on 14 day cycles. His bacteria is resistant to Tobi but we didn't want to put him on continuous ceftaz due to growing resistance risks. An achromobacter eradication protocol called for continuous inhaled Ceftaz with a strong oral antibiotic continuously for something like 6 months. There are risks associated with that as well; resistance to a whole new class of antibiotics, GI issues associated with an oral antibiotic, and of course the chance that it wouldn't help. Another option, and something far more novel that I had suggested, was the use of Cysteamine (if you want to be nerdy, read this - http://www.sciencedirect.com/science/article/pii/S2352396415301109). It seems to have an incredible capacity to thin mucus, disrupt biofilms and therefore allow antibiotics that were once powerless against these horrible bacteria to once again penetrate them and get rid of resistant bacteria. There hasn't been sufficient testing in people with CF yet, but theres a whole group of folks that I've met who have both use and had success treating their resistant bacteria with this medication. Drew's doctor is trying to learn more about it for us so that we can see if it could be a useful option to try for him. If I'm trusting my mom instinct and backing that up with all of the research I've done on it, I think its probably the best option that we've got at getting rid of this bacteria.

What we settled on was a bronchoscopy. We have been treating the bacteria that Drew grows when they take a culture to see what he has in his lungs. The way they do the culture is sort of like a strep test where they jam the giant q-tip looking apparatus down your through to swab it for bacteria. While this is usually a "good enough" sample, sometimes its useful to get a deeper culture from mucus way down in his lungs. Since he's not yet able to cough up sputum and spit it into a cup, the only way they can get that sample is through a bronchoscopy. The reason that we want to do this is to make sure that we are treating the right bacteria and not missing something else living in his lungs that may be causing damage because of our failure to identify and treat it.

So on Monday, the first day of school, as we were walking out of the building and I was hearing all about who sat with who at lunch and how I packed snacks that they didn't like (you're lucky you even got a snack kids, don't push it!), my phone rang and the number was Cincinnati Children's. It was someone from scheduling calling to get him set up for a bronchoscopy. I told them I was busy and would have to call back.  I know how these calls go - 15 minutes on the phone with a usually very kind woman from customer service who asks me 100 questions that I'm quite frankly shocked that she can't easily get the answers to through our medical record, and then tells me the date that they can do it. I was already not really paying attention to the woman because a ringing phone means nothing to 5yr olds so they carried on with their chatter about their day, and I simply told her I would call back to take care of this later. CF interrupts so many other parts of my life, and I wanted to hear about the first [fake, only half] day of kindergarten, uninterrupted. I didn't want to page back and forth between my calendar and my phone call, quickly and distracted, likely to give her a date that wasn't going to work anyway leading to the need for a follow up and repeat of the first phone call/questionnaire. I want to sit down and think about this, find a time that works well for us. Maybe fall break, or another day off school. Missing a day of school right now will just kill him. He's just getting comfortable going, getting to know his routine, meeting new friends to eat that snack he doesn't like with. Missing a day off to relax might kill him too. I have to weigh these things against the importance of having this done, of knowing whats next on this journey.

I think this is the thing that most infuriates me about the current system for chronic illness care. While i'm trying to make these kinds of decisions, decisions that I have to make in 1000 different ways on nearly every single day, medical professionals are trying to help us understand the importance of things like sleep, exercise and taking your medication on time. I get that that's important, but even for those of us who manage to keep our heads above water, normalcy, above all else and in whatever distorted, backwards, chronic-illness-mom-anything-but-actual-normal way we define it, normalcy is our priority. I wish we could find a way to focus on that.

If he's smart enough to ask the question

It has been a busy summer! Health has been good (knock on wood), weather has been bad, and what I'll say about the kids is that I'm just about ready for school to start again.

Drew has an MRI and CT scan tomorrow. He's not sick, which is really why we are doing it now. He's still growing achromobacter in his lungs, that ugly beast of a bacteria that has set up shop and colonized his airways. I found a protocol for achromobacter eradication that another CF mom had shared with me from the CFRI conference several months ago, but its pretty intense and we are trying to decide if its something we want to do. On one hand, the bacteria  seems to be reasonably well managed with the inhaled antibiotics that we are on (constantly). On the other hand, we worry about the silent damage that may be happening to his airways and if we could take away the chance of that happening by getting rid of the bacteria. The chances of us getting rid of it, truly eradicating it, are equivalent to us finding a pin prick on a tennis court. His lungs are vast, and bacteria is tricky.

Talking with his doctor at our last appointment we decided that it might not be a bad idea to do a CT scan to compare it to his prior CTs scans to see if lung damage is happening. It will inevitably happen in CF, but we hope to prolong the time that we see damage as long as possible (that's the goal of the inhaled antibiotics we are currently on to treat the achromobacter). The protocol that I had suggested was seven months on an oral antibiotic combined with regular inhaled antibiotic....for seven months. Her concerns were both resistance to antibiotics that currently work for him and also the side effects of the prolonged use of antibiotics, like GI issues. We've finally been able to get some weight on Drew and with strong oral antibiotics he sometimes has an upset stomach. I'd hate to wreak havoc on his GI tract to save his lungs; we need both!

So we decided to take a look before we make a decision. We decided that if his lungs look good (which I'm less optimistic about than I've ever been before) then we will stay the course of inhaling Ceftaz for 14 days and Tobi for 14 days, indefinitely. If there is some significant changes, bronchiectasis, then we will reevaluate our plan and try to be more aggressive in treating it.

I found out when we decided to do the CT scan that there is a research study underway trying to understand the value of MRI in measuring lung damage, as the risks of MRI are negligible since they use magnets rather than the radiation that a CT scan uses. I remember a presentation at NACFC a few years ago weighing the pros and cons of a CT scan, with some folks not wanting to expose patients to radiation of a CT scan and other feeling the benefits of knowing whats happening in the lungs giving a better opportunity to treat outweighed the risks of minimal radiation exposure. I'm on the fence. The exposure is minimum, but when you add up all of the different scans and test and everything that a kid with CF has done in a lifetime, the exposure to radiation is not insignificant, and so I appreciate that science brought us the MRI and the thoughtfullness or curiosity or whatever that led researchers to compare its value to the CT scan to understand how one might eventually replace the other. I digress.

I agreed to have the MRI done along with the CT scan as long as we didn't need sedation. After some discussions with the doc and the researchers, everyone agreed to try. With the scan coming up this week, I reviewed the IRB (the document that explains the research, what is happening, the associated risks, etc) and decided to talk to Drew about it. I also offered him $5 to hold still for the test so we can avoid sedation. He asked me why he had to have it done and if it was going to hurt, and I tried my best to explain that to him. After our conversation, I emailed the research coordinator and asked if they had materials to explain the IRB to a 5yr old. While they didn't, she did share some images of the machines that I was able to show Drew to hopefully allay some fears. But it got me thinking about how I wished the IRB was directed to him. I'm fine with a copy for me, explaining things at a different level, but if we could involve patients at a younger age understanding why they participate in this type of thing, what exactly is going to happen, how it will feel and how it will help them, maybe it will give them a deeper understanding of their disease, a deeper confidence in the teams that care for them, a deeper appreciation for the entire system that is in place to keep him well. I felt that if he was smart enough to ask the question then he deserves a smart and honest answer. I would love to work on a project with kids as young as 5 translating overly complicated IRB protocols into common sense, easy to understand documents. Maybe I will :)

Stay tuned for results on the MRI/CT study!

Precision Medicine Champions of Change

On Wednesday, July 8, 2015, I attended an event at the White House honoring 9 Champions of Change in healthcare. I've been thinking for a whole week about how I can share what I experienced there that day, and the best I could come up with was a storify. This captures the essence of the event - patients, parents, dedicated comrades, fighting together for equality of access, freedom of our data, and precision treatment options. This is Precision Medicine. This is what is going to save Drew's life. 

APPROVED!!!

We moved into our house in 2009. We had one daughter who had just turned one year old. Nine months later we welcomed twins, and our son Drew was born with cystic fibrosis. It was at that time that we learned that our neighbor had a 50yr old sister who had cystic fibrosis, the doctor across the street had a business partner whose granddaughter had cystic fibrosis, and another neighbor worked with a man whose grandson had cystic fibrosis. With only 30,000 people in the country with CF, the chances of us living among three other families affected by this disease was incredibly rare. This is the way the cystic fibrosis community is though. We celebrate each others victories and we mourn each others losses.
Today, a new drug called Orkambi was approved for use in patients ages 12+ with two copies the F508del mutation. It works to correct the genetic mutation that causes cystic fibrosis. No only will this add years to the lives of those living with cystic fibrosis, but it has the potential to dramatically improve the quality of their lives.
This is a remarkable day for us, for all of us; for those who will benefit from this medication and for those of us who will not. Our drugs will come, so for now we will celebrate with them because we know that when our drugs come they will be there to celebrate with us.