Tuesday, March 24, 2015

What we need most

I'm sure many of you heard that Drew had a rough day today. A routine clinic visit didn't go as planned and he ended up in the hospital for the day getting a PICC line to do 2 weeks of IV antibiotics for a bacteria infection in his lungs that he can't kick. This means strong mediciations administered through a IV in his arm 4 times per day. It means no gymnastics, no swim lessons, no baths. It means hours per day spent sitting and watching TV or playing on the iPad during infusions while his brother and sisters and friends play. It means painful dressing changes and distraction from the simple pleasures of everyday life
Coming home from the Cystic Fibrosis Foundation​ Volunteer Leadership Conference that I attended last week, I believe that a treatment for him is on the way, but we continue to need the support of you all to help us get there. Tomorrow night I am having a Matilda Jane fundraiser at our house from 7-9pm that anyone and everyone is invited to attend. A percentage of all purchases will go to the Cystic Fibrosis Foundation. If you cannot attend but wish to place an order you can view the catalog online at https://www.matildajaneclothing.com/, add items to a Wish List, and then choose the option "Send to Trunk Keeper" and enter jennybostater@matildajaneclothing.com. Tomorrow is the last day that you can order as the party will close at the end of the night.

What we need most right now is your support. Join us in this fight by making a donation today!

More on today's clinic visit coming soon!

Saturday, March 14, 2015

He's FIVE (and he's still alive!)

I have so many thoughts to share on this special birthday, but for now I will ask for your support. I want more birthdays for Drew, and the only way that he will get there is with your kindness and support. Thank you - for supporting us through the years, for being our friends, for loving Drew like we do.

Donate Today by clicking HERE!


Saturday, February 28, 2015

Rare Disease Day 2015

A few years ago, I wrote this post about what a typical day looks like for us. What I wasn't able to 
articulate at that time, whether it was because I was so deep in the motions of everyday life or the true burden of this disease hadn't yet full sunk in, was how it makes me feel. So today, on Rare Disease Day, I want to share what a day really feels like for me.

I wake up in the morning and I don’t want to get out of bed. Like ever. I'm tired. Always. I'm happy to grab a kid and pull them in to snuggle, but I’m so tired of this routine that I just don’t even want to get out of bed and start another day.

I finally pull myself together and head downstairs. I pour a couple of Ensure Plus into a cup with a straw, hoping he will drink them, knowing that he needs them, worrying that they won’t be enough. I make breakfast for the other three, and lunch for my first grader, while everyone complains that i’m making something they don’t like or that I’m not paying attention to whatever they’re telling me. Its true, I’m not. I want to be paying attention, but I can’t. I’m not sure why. I’m thinking about the coughing that I hear. Its usually there when he wakes up, some days worse than others, most days subsiding after the morning treatment. I have to decide if its enough to stop everything else and start the treatment or if it can wait the 15min until two of the 6 are out the door. I’m yelling out spelling words and only half listening to hear if she spells them correctly because the coughing interrupts her answers.

While they’re eating breakfast, I pull together the medications and equipment that we need to start our day. Theres always moaning and groaning when I announce that its time for “breathies”, and I wish he could really feel me when I say “I so hear you bud, I don’t want to do this either”, but I’m committed. He begs for the “short” treatment, the one thats only 24 minutes instead of 30. He can tell the difference.

Once we agree on a show to watch, we settle in for 30 minutes of vigorous shaking and various medications. We always sit with him, usually me, so that he doesn’t have to do it alone. The weight of that is heavy, as sitting with him means time away from the other two still at home. They’re use to this. They shouldn’t have to be. It doesn’t make it any better.

Truthfully I don’t know if any of them really know what CF is. I don’t know if they should? I wonder if I’m keeping too much from them? I also wonder if I’ve given them too much. Are the behavioral issues because he’s 4? Does she act out because I unfairly dedicate more of my time to him? Are they because she doesn’t understand or she’s curious but doesn’t know how to ask? Do they have questions that they’re not asking me because I’ve shown them that I don’t have time to listen? I know that really, I don’t have time not to.

I’m scared when our secondary insurance decides to drop us and we now need a few extra hundred dollars a month to pay for the Ensure, that is the only reason he is gaining weight, on top of all of our medications. I keep meaning to schedule an appointment with the behavioral psychologist about his eating habits but I hate that I need someone to help me parent. It feels like a failure. When dinnertime becomes a nightmare, when he won’t eat any solid food, and when has to whisper to me that he doesn’t want to eat when other people are looking because he has realized and I have failed to realize that he’s aware that others are aware that there’s a difference between whats on their plate on whats on his, I concede and schedule an appointment. It feels awful.

When we go to the doctor and we only get more bad news - more to do, more to schedule, more to track - if feels like I’m going to drown, but I have no choice but to just keep swimming. And I have to keep a smile on my face because I am trying with all my might to convince him that he’s no different than anyone else, and that this disease is just something that runs in the background. He doesn’t need to know all that it takes, but he does. He’s smarter than most people I know, and he’s not even 5 yet.

None of the tasks that I get to do on a daily basis are a heavy lift for us - by most standards a well-to-do family, reasonably well educated and financially secure - but the combination of small tasks that I have to perform, the cognitive burden of this disease is sometimes more than I can bear. I wonder what the real impact is of all of these medications and treatments and appointments. I wonder what’s actually necessary, and if I’ll ever actually know whats really necessary. Is it okay that I skip an afternoon treatment now and then for a trip to the park? I think his mental and emotional well being are of equal importance to his physical health. I think the doctors agree, but they only ever seem to offer more to do. I do believe they have his best interest at heart, but not like a mother does. Or maybe they do have his and I’m being selfish in thinking they perhaps they should also have mine? Do they understand how guilty I feel when I have to say no to school or the day of a big party because the teacher has been kind enough to let me know that there are a number of kids there with colds? They can’t possibly feel the ache in my heart when school calls to tell me he won’t come out of the bathroom because he has trouble with his bowels and wiping by himself. When the family wants to take a vacation to a lake, something that we just can’t do because of his health, or have a fire in their fireplace, the little things that we just can’t partake in, and trying to minimize them so it seems like no big deal that we have to miss out, when really I desperately want all of my kids to partake in lifes simple pleasures. Is it right that I keep all of them from these things so that he doesn’t feel even more left out? Should they have to suffer from this disease too?

There is always a fear that you will lose your baby. It’s not all encompassing or debilitating, but its ever present and its completely out of our control. I could do everything right - by the books, medically speaking, everything right - and we could still lose this fight. It happens every day. We see it on Facebook, on blogs, among friends. I think about that.

I also think about how lucky we are and how good we have it. I cry for the families that lose their children to CF because they were born into a different socioeconomic class than I was. I feel an obligation to use what I have and what I know to help them too. And then I feel guilty for dedicating my time to someone other than my family. I wonder if all the work that I do is really hurting my family more that its helping us. I think that a balance is good, teaching my kids the importance of helping others and also finding time for yourself. I don’t think I’m doing a very good job of it, but I’m trying.

That’s what I think about in a day, every day. What I what you to know is that we are trying. The people like me, and the people nothing like me; we are trying. We are trying the best that we know how. We have to perform medical procedures, without any professional medical training - correcting a rectal prolapse, cleaning a stoma, changing a trache. We compound medication in our kitchens, monitor oxygen saturations at 2 in the morning, sterilize medical equipment the best way that we know how, all with knowledge that we’ve put together from the expertise of our healthcare team and also from outside of the institution, from our peers. Our medical care does not happen 4x per year when we go to see the doctor. It happens 24hrs a day, 365 days per year.

We aren’t mad at doctors. We are desperate for them to ask the questions that will allow us to tell them all of this. This isn’t easy to say, and we often don’t even know how to put into words what our priority is. We want the same thing that you want for us - good health. But to minimize the impact of this disease in our lives - for a day, an hour, just a moment - would mean the world.

I often go to bed at night feeling defeated. The kids are not happy. The house is not clean. The disease is not cured. I know whats on the other end of a good nights sleep. But I remain hopeful.

Thursday, February 5, 2015

That time I went to the White House (part 2)

On Friday, January 30, 2015, I arrived at the White House (yes, the one the President lives in) at 9:30am. I found some of our CF Foundation Leadership already waiting at the gate along with Bill Elder, an adult with cystic fibrosis who benefits from Kalydeco, the life changing drug that essentially cures cystic fibrosis in the 4% of CF patients who have the specific genetic mutation that it treats. I was introduced (or in many cases introduced myself) to anyone who looked even mildly familiar. I met a Nobel Laureate from MIT, the founder of a startup that is finding new ways to use "big data", and the current director of the National Institute of Health and discoverer of the CF gene in 1989, Francis Collins. He was in line behind me at going through White House Security, and when I saw him, after I did a double take, I introduced myself and asked him if he would mind taking a picture. What an incredible honor and privilege to be at an event, at the White House, with the man who made one of the first and certainly most remarkable discoveries affecting the CF community.

This is the post about the details, and I'm going to give you the nitty gritty, because if I hadn't been there but I knew someone who had, I'd want to know.

You would think that they would have a lovely holding area for White House guests, perhaps something more than your run-of-the-mill tent equipped with a space heater and a few secret service agents. Alas, they do not. They do however have multiple tents, where they perform the exact same security measures multiple times.

If you make it through the tents, you enter a small building with signage that indicates that there are dogs inside. Behind a large screen that you cannot see through, you walk past one or many K-9's toward a metal detector and an X-ray machine for your personal belongings. This is no nonsense. They're not smiling or asking about your day. If push comes to shove, these folks will give you the shakedown.

And then you're in.
Francis Collins, Director of the National Institute of Health and Scientist responsible for the discovery of the CF Gene

Me with Preston Campbell, SVP of Medical Affairs at the CFF and Bill Elder, an adult with CF

When I walked into the White House (THE WHITE HOUSE!!!!!!) I saw the President's dogs, Bo and I have no idea what the other ones name is. I'm not a dog person, but still, I saw the dogs.

There were Marines around every corner donning their dress blues and referring to everyone as sir or ma'am. There was a coat check further down the hallway where we saw the dogs. Everything was so historic and pristine! We made our way upstairs to the reception area where they had coffee (White House coffee!) and water. I passed on the drinks for fear of spilling on my outfit. The was a marine playing a beautiful historical looking piano as people milled about, introducing themselves to one another and sharing their excitement for being invited to such a cool event.

After about an hour, another set of doors was opened leading to the room where the announcement was to be made. We filed in and found our seats. The only seats that were assigned were the guests of honor, those patients and families who had attended the State of the Union the week before as guest of the First Lady. They go the front row. Let me just repeat that for a second - PATIENTS and FAMILIES sat in the FRONT ROW. Not the second row. Not mixed in with the crowd. The very front row. That in itself spoke volumes. There were probably 6 rows in the front section followed by an open aisle and then the remainder of the seating. I was in the first row of that second section. Here was my view, panoramic style:

There was an announcement made to silence all devices, and a hush fell over the crowd. As we anxiously looked around the room, I caught the eye of one of the marines who whispered to me that as long as the piano was playing the President would not be coming out. I felt cool for being in the know.

A few minutes later, the piano stopped, the patient/family guests of honor were ushered in, and an announcer said, "Ladies and Gentlemen, The President of the United States of America."

The President was introduced by Elana Simon, a 19 year old student who’s high school science fair project was about finding a treatment for her own specific type of liver cancer. In other words, the President of the United States was introduced by an ePatient. 
The teleprompter that read Remarks of President Obama on Precision Medicine, The White House, January 30, 2015.

To borrow a nice summary from the S4PM website, "The initiative begins with a budget request for $215 Million in new funds. The funds will be used, mainly at the NIH, to:
  • Develop a national cohort of a million volunteers who will donate their health-related data for open research.
  • Provide increased funding for research to identify genetic drivers of cancer.
  • Help the FDA speed up the regulatory process
  • Fund the development of data interoperability standards

Because Cystic Fibrosis is such an incredible example of the value of personalized medicine, treatments that target specific genetic mutations, one of the VIPs was an adult with CF, Bill Elder. Bill was a guest of the First Lady at the State of the Union, and was invited to attend this event as well. President Obama highlighted the success of Kalydeco by sharing Bill's story. I was able to get a video of it so check it out:

I may not be Mr. Obama's biggest fan. However, this announcement and the proposed commitment to advancing this initiative is the first step toward progress in curing a disease like CF. Congress must still approve this budget. We will need to learn more specifics around the proposed initiatives. We will have to get a better understanding of the resources available to push this forward. But it's a start, and it's further than we have ever before been with support for addressing the extremely personalized needs of patients with specific genetic mutations in CF and other rare and orphan diseases. Here is a fact sheet that the White House released post announcement: http://t.co/bOoYqBzhrr.

Best parts: 
  • “Patients and advocates won’t be on the sideline. They’ll help us design this ” -Obama
  • "Precision Medicine is delivering the right treatments to the right person at the right time every time” - Obama
  • "The patients here are living proof that the new dawn of precision medicine is upon us." - Obama
The talk was about 10min long. At its conclusion, there were a few handshakes and pictures with the folks in the front row before the President was gone. We came, we saw, we left. Let me tell you those marines mean business when it comes to ushering you in and out of the White House! 

Following the announcement, I was invited to a luncheon with a handful of other patients and patient advocates pretty close to the White House, and it was equally as amazing. I met some seriously incredible people, including a guy who found his own brain tumor by accident and had a 3D printout of it in his pocket, a woman who created a remarkable registry for a rare disease that her children have, the always amazing Emily of Emily's Entourage (who just recently did THIS) and many others. We talked with some of the folks who work at the White House Office of Science & Technology Policy about what we thought some of the major hurdles might be. Chief among them were privacy issues and the need for updated IRB protocol. The whole thing was like one big highlight in my life, but one of my favorite parts was definitely getting to spend some more time with Francis Collins. It was such an intimate group of people at this lunch (maybe 25-30 people?). Francis Collins had us go around the room and introduce ourselves and say 3 words related to how we were feeling about the announcement that we had just been a part of. There were lots of "hopefuls" and "readys", a few "desperates" and even a handful of concerns related to the funding and the resources available to do this. When it came back to Francis Collins, this is what he had to say: "I've been dreaming about a day like this. We are all in this together." He went on to ask us to work with them, to share with them all the work that we are doing so that they can learn from us, saving time and money by not having to recreate the wheel. He asked us to speak up and let them know when they're doing something wrong, and then work with them to make it right. "We are all in this together." Indeed we are. 

I'm hopeful. I know that there is a long way to go, but we finally have a stake in the ground, a starting point. This experience definitely ranks near the top of my list of coolest things I've ever done, and I do hope that this really is just the beginning!

Saturday, January 31, 2015

That time I got invited to the White House (part 1)

"Our deepest fear is not that we are inadequate. Our deepest fear is that we are powerful beyond measure. It is our light, not our darkness that most frightens us. We ask ourselves who am I to be brilliant, gorgeous, talented, fabulous? Actually, who are you not to be? Your playing small does not serve the world. There is nothing enlightened about you shrinking so that other people won't feel insecure around you. We are all meant to shine like children do. We were born to make manifest the glory that is within us. It's not just in some of us, it's in everyone. And as we let our own light shine we unconsciously give other people permission to do the same. As we are liberated from our own fear, our presence automatically liberates others."

Almost 5 years ago, on March 14, 2010, our lives forever changed as we welcomed two beautiful babies into our family. We found out just a few days later that Drew had cystic fibrosis, a life shortening genetic disease that does not have a cure. I can't even describe the overwhelming sense of fear that accompanies that diagnosis. 

I remember so vividly jumping into fundraising as we were told that our dollars went right to research to cure this disease, and that the Cystic Fibrosis Foundation was our only hope. We walked in our first Great Strides just days after Drew was released from the NICU. 

He came home with an ileostomy and a list of instructions for how to handle his bowls that were protruding from his abdomen. We macgyver our way through daily ostomy bag changes, becoming better at them than the seasoned nurses who provided regular home care. We were given a pricey special pre-digested formula and a print-out of instructions for how to mix it so that it would be higher calorie and meet his nutritional needs. We were given a nebulizer and other equipment to use with the countless new medications that were prescribed. We had a photocopy of a diagram describing how we should beat on our sons back to dislodge the mucus that was already starting to clog his airways. Our tupperware cabinet quickly turned into our medicine cabinet and our home looked like an assisted living facility. We were measuring stool with a syringe and calculating enzyme dosages on our iPhones. We scrambled as we learned what we needed to do and when we needed to do it. We were completely and totally exhausted, and we were never given time to grieve the loss of the life we had expected. We were not trained for this. Elizabeth Scarboro described it so perfectly in her book "My Foreign Cities: A Memoir" when she said of caregiving, "My field was large and nebulous - I had no idea how to do most of the things in it, and all I could do was try anyway." And try we did. 

As time passed, things got easier. Not in the sense of there being less to do, it simply became more of a routine. We continued to be involved with the Cystic Fibrosis Foundation and I found a new role as the State Advocacy Chair. Initially intimidated by the congresspeople that I would meet with, I quickly realized that they are people just like you and I. I was never politically saavy, but I didn't have to be. When I would meet with them, I thought less about what I wanted them to know and more about how I wanted them to feel when I left our meetings. I wanted the world to be on Team Drew, and I found that sharing our story in all its simple yet oh so complicated glory was helping me and it was helping others. They now had a person that they were thinking about when they were voting on things like whether to support the creation of the National Center for Advancing Translation Sciences, a branch of the National Institute of Health that would accelerate drug development for people like Drew who did not have time to wait. 

The more that I worked at this role, the more I learned. I joined the advisory council at our hospital and I refused to settle for the status quo. I wouldn't accept what I believed was anything less than the full potential of that organization. I had to try, to keep trying, for Drew and for the other patients and families who didn't have the time or the voice or the courage to push these issues like I did. I was very fortunate to be among such amazing people Cincinnati Children's Hospital who were receptive to new people and new ideas, and embraced me as a partner on their team. I served, and continue to serve, as a constant reminder of why they are doing the work that they do and I implored, let patient's help. Team Drew was growing. 

I had a vision of a better way and the more I shared my vision, the more people wanted to hear about it. It seemed such an aspirational goal 3 years ago, to have patients working alongside clinicians and researchers, combining the knowledge they had with the molecular biology and genetics expertise of some of the top researchers in the world. I had data, in my head and in my heart, that helped me to manage Drew's health, and I believed that we could get farther together than any of us could get alone. Then in 2012, at the first North American Cystic Fibrosis Conference that I the opportunity to attend, I heard something that would catapult me forward. The scientists and researchers working so tirelessly to cure this disease felt that the missing puzzle piece was understanding what happened to patients between clinic visits. I thought to myself "I know that! I have that data! I live that every day!". I realized that the cure for cystic fibrosis would come through partnership. The efforts that are made on behalf of patients and families cannot go unappreciated, but alone they are not enough. We need to work together through every stage and every step of the way. 

I've had the great fortune of speaking everywhere from San Francisco to Switzerland and many places in between, advocating for patients and families to be included as partners in everything from clinical trial design to helping clinicians accept the idea that some people with CF may prioritize quality of live over longevity. While the cognitive complexity of adding another medication or therapy to our daily regimen may be small, the burden becomes too much to bear. There is incredible value in a humble awareness of a shared humanity. We are all just people striving to do and be well and I view my role as one to ensure that that is realized and appreciated.

On Tuesday evening, January 27th, I received the following email:
INVITATION: White House Event
The Office of Science and Technology Policy is pleased to invite you to an event at The White House in the morning of Friday, January 30.
DATE: Friday, January 30, 2015
TIME: Morning TBD
LOCATION: The White House
Additional logistics will be made available upon receipt of your RSVP
That was the email in its entirety. I wanted to go, but I had no idea what this was about.  Logistically it would be a tough thing to coordinate in 2 days time. I had just earlier that week sat down to define my mission path, to create some structure for all of the different work that I was doing and help to ensure that the opportunities I would choose to pursue going forward would be ones that fed my soul. I had no idea what this "White House Event" was about, but we decided that I should probably go and find out. 

I used sky miles to book a ticket and invited myself to stay with a friend who lives in town. I would leave here on Thursday night and return on Friday afternoon. That same day, two kids got sick, our only 6 person vehicle broke, and the exhaustion that is a Wednesday night in our world started to set in. I said out loud that I should have just said no. There is always another opportunity and if I didn't make it to this one I was confident that another would follow. I was mad at myself for being "wooed" by the subject line of an email from a sender I didn't even know to an event that I knew nothing about. We got the kids to bed and then everything changed. 

At 8pm on Wednesday, January 28th, I received an email that changed everything. "We are pleased to confirm your attendance at an event On the morning of Friday, January 30 with the President highlighting investments to improve health and treat disease." You guys, THE FREAKING PRESIDENT! Of The United States of America!! My advocacy efforts, in just 5 short years, had taken me from being a scared parent to being invited to attend what I hope will become known as historic event for rare and orphan diseases at the White House with the President of the United Freaking States. !!!!!!. 

The very first thought that I had (and no I'm not kidding) was OMG I have nothing to wear! I hopped in the car and hightailed it to Macys, the closest store to our house, and the only one still open at 9:00 at night. I had less than 24hrs to pull this all together before I would board a plane to Washington, DC, and in our world that 24hrs includes multiple school drop offs and pick ups, swim lessons and gymnastics, story reading and lego building and working, and lets not forget those 3 hours of breathing treatments and airway clearance. I FaceTimed with a dear friend who helped me to pick out the perfect outfit via a virtual fashion show, I packed my bags, and I left for DC. 

Tuesday, January 20, 2015

Creating the CF Care Model of the Future (for real, y'all!)

For many, many months now (as in enough months to make up years), I've been talking about how we can improve the current system for cystic fibrosis care. I've been testing and trying new things on my own, and dreaming about what CF life and care could and should look like. Here's a collection of blog posts with my thoughts on the topic (in chronological order) that also help to put into context some of what I'm about to tell you about:

The Difference Between Improving a Process and Creating a New One - http://66roses.blogspot.com/2013/04/the-difference-between-improving.html
A Collaborative Chronic Care Network for CF- http://66roses.blogspot.com/2013/09/a-collaborative-chronic-care-network.html

This is actually happening! Last week, I spent 2 days in Bethesda with 40 other folks - CF parents, patients, clinicians, researchers, industry innovators - all with the same desire: to improve both life and care in the CF community. 

The first step in creating this new system is to deconstruct the current system to better understand the components - whats currently working in CF life and care and what's not. We got (and continue to get) this information from the community - from blogs and Facebook groups and testimonials from the patients and families in the room. Then with all of this knowledge, we imagined what "the perfect system" would look like and collectively came up with our dream list which included things like:
  • 100% of patients and families feel that there is research happening relevant to their needs
  • Everyone has access to own personal data and a personalized treatment plan that is accessible to whomever they choose to share it with
  • There is mutual trust, collaboration and empathy where all patients/ families/ professionals think that their skills and knowledge have contributed to the decisions and actions that matter to them
  • No more waiting rooms
  • Doing your treatments should be like brushing your teeth. It’s a habit and there is no hassle.  It’s convenient and portable.
Over the next couple of months we will figure out if some of the targets we laid out are the right ones that can lead us to better life balance, health management and care coordination by finding or creating different solutions and tools to see how they might help to achieve some of our "perfect system" goals. There are many good parts about the current model of CF care that either aren't known about or aren't implemented properly, so we are going out into the community to learn more about what works, what doesn't, and what we need to do to create the CF Care Model of the Future. 

My goal of 2015 is to blog more, share more of this work that I find so amazing, and connect with people whose perspectives need to be heard. Twitter is an amazing vehicle for sharing, and the project is using the hashtag #CFBigIdea. Join us!! Another platform that we are using (which I will talk about in more detail in another post) is called Smart Patients.  The recent creation of Learn from Smart Patients creates a feedback look where clinicians can learn from the conversations that patients are having with one another, in a safe and privacy respecting way. The real value in this platform versus something like Facebook is that the conversations aren't happening inside of an echo chamber; we're starting to talk with the people that can collaborate with us to help us overcome the barriers we face [that they don't know we are facing if we only talk about them within a closed group]. Don't get me wrong, there is tremendous value in the empathy and support that comes from a group like CF Mamas. I in no way want any of this to replace that.

I want to talk to you, fellow CF mama warriors and amazing patients and patient advocates. The best way to reach me for questions/comments/concerns is through Twitter, I'm @ekeeleymoore (I'm into learning out loud so that others can reap the benefit - chances are someone else is wondering the same thing you are, and we can all learn something through having that conversation together) or via email at erin(dot)moore(at)cchmc(dot)org.