At least we have a plan!

I think that our baseline is changing. Every time Drew gets sick, with some combination of antibiotics and therapies we seem to be able to get him back to his baseline, which is cough free. In CF, this is important, because one of the most recognized causes of lung damage is exacerbation, and symptoms typically mean that something is going on. We've been off of IV antibiotics for as long as we have ever been - last time was January! - but I think that it's time to start them again. I had a long talk with his doctor yesterday about new symptoms that I have noticed. He is not sick right now, but I have noticed an infrequent cough  happening, just a few times a day, but it's been around for the past 2-3 weeks. I have wondered if it is seasonal allergies, or perhaps a touch of asthma that could be treated with an inhaled steroid, but with some small changes the cough seems to remain.

So what do we do? Some people would suggest that his baseline has changed and he now has a little cough as his baseline. I'm not "some people" and I'm not ready to settle for that until we have exhausted our options in an attempt to return him to his cough-free state. We could try oral antibiotics, or even inhaled, but the bacteria that Drew has in his lungs (achromobacter), is highly resistant to just about all antibiotics. IVs are stronger than oral, and if we want to give it our best shot, we feel that this might be the way to go.

You might ask why we treat when he's not sick. Here's the best way to explain it. Imagine a barrel that we will pretend is your lungs, full of water that we will pretend is bacteria. The barrel can only hold so much before it begins to overflow. Usually, overflow is point where we would treat because we see symptoms. Antibiotics would reduce the amount of bacteria in the barrel and take it down to a level that is no longer overflowing (let's say half full since CF lungs usually have some kind of funk in them causing silent damage). So now imagine that we are in that silent damage phase, perhaps more than half full but not overflowing. And now if we treat with antibiotics, we might be able to lower the level even below the half full point and help to keep those symtoms or overflow from coming back for a longer period of time. Of course there is the possibility that this won't work and he will just have a cough from now on, but there's a reasonable chance that it will and that's what we're going with. I talked to his doctor about this for a long time yesterday, and we decided that being aggressive is in his best interest. It's certainly not what every doctor or patient would choose, but we're going to give it a try.

We hope to go in for our PICC on Friday, November 7. I am going to be out of town most of next week, and running IVs from home is both mentally and physically exhausting. It's definitely not something that one parent who is in charge of 3 other little kids in addition to the one on IVs should have to do alone, so we will wait until I get back. I don't know yet what antibiotics we will be on, but have another post coming on quality improvement type stuff where I will talk a little bit about that. I hope that they're every 12 hours because those every 8hr treatments are so draining. In an effort to avoid even more antibiotic resistance, I think we are going to try inhaled Tobi or Cayston for the week leading up and the two weeks following so that he doesn't get 6 weeks straight of Ceftazadime, the one antibiotic that this miserable bacteria still seems to respond to.

This sucks, but I feel like going into this with him well, and having a week to mentally prepare for a PICC and our crazy hectic life when Drew is on IVs is a nice way to do it. When he is sick and we get thrown into a tailspin, it is much harder to find our footing and regain composure. I don't know if this all will work or not, but I'm hopeful that it will, and at least we have a plan!

A day off

I have today off. Well, mostly off. I have set a regular work schedule for myself in an attempt to accomplish all that I want to while maintaining the much needed balance in my life to feel at ease. I have a nanny who comes on Mondays and Wednesdays and I've been trying to schedule most of my meetings and work during the times that she is there. I have some afternoons as well, when most of the kids are at school and the ones who aren't are sleeping, that I'm happy to take calls and roll up my sleeves. The problem for me with all this work that I've been doing (which I must add that I do love) is that I'm a stay at home mom. I already had a full time job when I slowly acquired a second one. I found myself working around the clock, quite literally, answering emails and taking calls, and my kids noticed. Patience was low, stress levels were high, and time-outs were reaching epic levels. That's when I realized that too many time-outs were the result of not enough time in. All these kids want is my time and attention, and if I didn't figure out a way to balance all of my new work with all of my current work, we weren't going to be a happy bunch.

The kids were off school on Friday, so we packed up the car around 9am and headed to the Children's Museum, followed by lunch and an afternoon trip to the Nature Center. The weather was beautiful and we just wanted to be outside, with no agenda. That night, there was a fall carnival at their school. We had dinner there, and then  played enough games to fill us up with carload of junk for the ride home. We had friends over to play on Saturday, and a birthday party with more friends on Sunday. We all spent 3 days together. I'm not going to lie and say that it was all fun and smiles, but I noticed an improvement in attitudes and a drop in our time-out rate. If I could put this into a pretty little chart, the Quality Improvement folks that I work with would be proud.

I hadn't opened my computer once since Thursday afternoon until last night, and even then, it was only briefly. So when this morning rolled around and our nanny knocked on the door at 9am, I had plans to find a quite place to sit and get back to business. But my calendar was free! I had nothing scheduled today, and it has turned out to be just what I needed. I ran a few errands, I did some things just for me, I spent the last hour sitting in Starbucks plugging away at the blog, and it feels so good. I am refreshed. I am not short on time today. I'm not rushing anywhere, and I think the kids will notice. When I pick them up from school in 30 minutes, I won't be fresh off a conference call or trying to tie up loose ends while they vie for my attention to tell me about the picture they drew or the game they played or the homework they have. We have a birthday party for a stuffed animal taking place today at 4pm, and I want to be there, in body and mind.

I feel guilty about working, about being away from them, even though I absolutely love what I do. They aren't going to be little forever, and they need my time and attention and I want to give it to them. In talking to a friend a couple of weeks ago, she used the term "mission path" to describe the way that she remains focused on the work that she wants to do. I've thought a lot about that, and have tried to focus in on what it is that I want to do. In work and in life, if I'm asked to participate in something that is outside of my mission path, I think seriously about the implications of choosing whether or not to participate. I don't think my mission path is well defined just yet, but I think I'm getting there. I actually said no to a couple of really great opportunities in November because my family is my priority, and I think that's a step in the right direction.

I am going to schedule a day off from time to time, and I'm going to appreciate how it refreshes me.

A Fun Announcement - Data4Health

I have been named to the Robert Wood Johnson Foundation’s Data for Health advisory committee. The Data for Health initiative will explore how health information can be better harnessed to improve people’s lives. The initiative is led by a high-profile advisory committee, co-chaired by David Ross, ScD, director of the Public Health Informatics Institute, and Ivor Horn, MD, M.PH, pediatrician and health services researcher at Children’s National Medical Center. Data for Health will be hosting a series of events in five cities across the country with participation from the U.S. Department of Health and Human Services. Committee members will hear from local leaders, consumers, and professionals from a wide range of sectors on what data is important to them, and how they might use it to help people lead healthier lives. The advisory committee will issue a report and recommendations in early 2015. Find out more: http://bit.ly/Data4Health

NACFC

It's been more than a week since I returned from the North American CF Conference (NACFC) and I finally have the chance to sit down and write something about it. It was nothing short of amazing, once again. The best way for me to recap what I learned is by Storify-ing my Twitter feed from the meeting. I take notes in Twitter form and then go back to read them and dig further into the points that I captured. I'll share the link to my talk up here at the beginning if you're not interested in reading through the whole Storify. I was the last speaker during this session titled The E-Patient Experience, and I realized that while the other clinicians were presenting before me (I was the only "patient") that the way they were using E was for "electronic", not e-Patient in its traditional and appropriate sense. Below is the link to my session. My part starts at the 1hr 26min mark. They don't make it easy to access this either - you might have to create a free username and password and login and all that fun stuff, but I think it's worth it :). Goodluck!  http://arc.nacfconference.org/cff/live/player/74


Here's my conference experience:

Don't. Tell. Anyone.

I have a great idea. Let's have a meeting. Let's invite all of the brilliant minds in CF - the scientists, the care providers, the people who dedicate their lives trying to understand the science to make this life better. Let's bring them all together, all roughly 4000 of them. Let's ask them to present what they know, to talk to one another, to create posters that display all of their amazing and literally life-saving work. And let's not tell anyone else about it. If you want to know about it, you have to have a medical degree. And even then, don't talk about it. Don't take pictures of what you're learning. Don't tweet what you hear. This is a closed meeting.

How will we learn more? How can we grow? How can we collaborate with the real experts, the patients and families who have their heads in the clouds and their feet on the ground! They're the ones willing to do anything! When they do get their hands on a medical journal, it sits at their bedside where they read it and re-read it and then read it again. They're the ones who have funded this research. They should have a right to it! They should get to decide what everyone gets to see and how everyone can share it. Even the work funded by drug companies! I am an investor. I need to take control of my kids outcomes. I have a right to this information. If anyone is going to accelerate the pace of this, it's us! Show us the damn data. I cringed when I saw a "no photos disclaimer" on the app for the upcoming meeting I'm attending. I wish it also said what would happen if you were caught breaking this rule so that I could make a educated decision on whether it was worth it.

Non-Compliance Anonymous

My name is Erin, and it's been 4 and a half months since our last clinic appointment. Why haven't we been to clinic? It depends who you ask. When patients aren't doing what the doctor tells them to do, it's usually identified as a lack of understanding, or a time or money issue.  For us, it's not any of those reasons. I think it's fear, mostly. Fear of what else we could catch in the hospital.
I meant to make an appointment for August, but it wasn't on the top of my "To-Do List" because Drew had been well. Then he got sick, but we handled it remotely - I emailed the doctor, we went back and forth a few times, sent a video, decided on a treatment plan, Drew got better, no need to go into the office. Then I actually scheduled an appointment for October. Next week in fact. But I don't know yet if we will make it or not. I have a babysitter lined up for the other kids. I think about going frequently. I guess I'm just afraid. I'm afraid to go and I'm afraid not to go, paralyzed in between putting his health at risk either way.
I've spoken with his doctor about this many times, with her explaining that she needs to know him when he is well so that she can better assess and treat him when he is sick. She tells me about the value of good infection control practices and how the risks have been lowered even further by moving patients at our clinic under age 5 to a separate floor where they are taken directly back to a room to avoid other CFers. She tells me about the CFF guidelines, and how being seen quarterly is known to be associated with better health outcomes. I know all of this. No one has really validated my concerns and offered to work with me on a solution that meets my needs.
I don't think that I'm better than anyone else, and that by working at the hospital I should have special privileges, not at all. In fact, I very much try step out of my role on the team when I am talking with my sons healthcare providers about an issue pertaining to his well being. That's tricky though, because the very expertise that I have as a mom is what helped to develop the role of Family Partner, and the entire premise of the position is one of patient & family real-life experiences and interactions with the healthcare system. I think that we're just in a different place than we were 10 or even 5 years ago, when a face-to-face clinical encounter or a brief phone call was the only way to communicate with your care team.
What I'm struggling with is the idea of non-compliance. I'm labeled by the CF clinic as non-compliant because they cannot check the box that confirms that I have been in to see them in the last 3 months. The box was created by the CFF, and is associated with better health outcomes. That's the part I'm pushing back on. There is no doubt that staying connected with your care team will help you manage your disease and achieve more of what you want to in your life. I just don't think that it always has to happen in real life in the clinic, especially given the new infection control guidelines.
I would bet that my sons doctors know more about him than many of the other patients in that clinic, regardless of whether they are seen quarterly. I have put a lot of time and effort into making sure of this. I track everything about him. I weigh him weekly. I contact them regularly with updates and questions and concerns. I use the tools that they have empowered patients to use to identify things like an exacerbation, and I work with them very closely, as his primary caregiver, to do what we need to do to keep him well.
They tell me that the risk of him catching something in clinic is low, but no one appreciates the impact that taking that risk, and failing, has on my life. He has complicated airways that may or may not make him more susceptible to pathogen acquisition. I believe that those complicated airways are directly associated with the complexity of treatment and eradication. It's flu season now, and we just got him over the 40lb mark. Excuse my language, but that was fucking hard work. Constantly thinking about, worrying about how to get more calories in, invisibly, and sticker and star charts and frustration and praise. If he gets the flu, we're taking two steps backward. I'm already operating at capacity, and my reserves are bone dry. And this new enterovirus 68 that's floating around everywhere, making some kids really, really sick. Not only do I fear for his life, quite literally, if he were to acquire that, but the quality of life for the rest of the people in this house that need me when I have to spend more time than I already do caring for my son if he should get sick.
I think that there is merit to my fear, data that shows how these harmful bacteria have been known to travel indirectly from patient to patient. Unlikely to happen with good infection control precautions, but this whole risk assessment thing is where I'm stuck. If your kids life were on the line, what kind of risks would you, should you take? Is the first step here admitting that I have a problem? Or is it identifying that there is a problem with the way that we think about compliance. I need to find a way to break the stigma associated with non-compliance, and I don't know that being compliant, taking part in something that I don't necessarily agree to be the best option for us, is the right way to go about that. I just don't know what is. Thanks for listening.