[Editor's Note:  Erin Moore is "Doin' it for Drew"!  Drew has Cystic Fibrosis (CF).  CF is a life shortening genetic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide).  It is a chronic illness - meaning until a cure is found, CF is forever.  Erin is collaborating with the C3N Project; exploring the creation of a Collaborative Chronic Care Network for CF.  This post was originally featured on Erin's blog - 66 roses.]

I arrive for our clinic appointment around 7:45am. The last time we were here was 3 months ago. Usually, we are ushered back to a room within 15 minutes of arrival. A nurse greets us shortly thereafter to review our medicine list and address any issues that we’ve been having. Just the other day he was coughing up a storm but seems to have gotten over it. Should I talk to the dietician about his diet again? His weight is up and his stools seem “normal” but I’m always anxious about his lack of interest in food. I wonder if an RT is available to talk a little bit about his airway clearance. I don’t want to be a bother but I sure do think the airway clearance he had in the hospital this summer was more effective. I wish I knew what they were doing differently! 

Next up is the doctor. She asks how things have been going. Today? Great! Last week? I was a little worried, but the cough he had seemed to go away. There was that one day that his stool was a little weird but that got better too, not sure what caused it. And frankly I can’t remember back farther than that. She checks him out and wants to review his labs since we are at his anniversary visit. His vitamin D is low, his breathing still sounds a little noisy, and a note that she has from his ENT seems to indicate that he may need another sinus surgery. She feels out whether I’d be open to a bronchoscopy at the same time. Maybe another CT is a better option.  When I talked to the ENT last month it sounded like things were going well? I guess I don’t mind if they do a bronchoscopy while he’s under for something else, but I remember huge discussion with other CF parents on Facebook about CT scans and all the negative effects of radiation and I don’t know that I want to do that? I wish I could find that conversation! I ask questions about a game plan for if he needs IV antibiotics if we grow pseudomonas again, having read online about all different methods used for eradication but not knowing which is best and why. It’s hypothetical at the moment because they haven’t even swabbed him yet. I just have sort of a busy life and sometimes having a plan provides a sense of comfort for me. She suggests an action plan and I am mostly on board, except for the azithromycin because I saw a presentation somewhere that seemed to show compelling evidence against its use. I wish I could find that presentation to show her! I trust his doctor and want to follow her recommendations, but I have some reservations. I don’t think either of us has time to get into this as I’ve already taken up more than my fair share of appointment time. We agree to wait for the results of the culture to decide a course of action.

Next up is the dietician. Lucky for me, he has a “weird poop” while we are there so she can look at it and provide her thoughts. This isn’t what all of his stool looks like. I don’t really know how often his stool looks like this – sometimes once a day, sometimes 4 times a day, sometimes once a week. We talk for a bit about enzyme dosing and the calories he’s taking in, her offering suggestions for beefing up his intake and me adding them to the “notes” section in my iPhone, hoping I can get them down elsewhere before one of my kids deletes them by accident.

The social worker pops in to address some questions about starting preschool that I had mentioned to the nurse 2 hours ago. I had met with the Psychologist about a year ago to talk about his lack of interest in food, but never followed up, mostly because of both time constraints and cost. If only she could pop in and give me a couple of quick suggestions. But I have to go, we’ve already been here for almost 4 hours. While waiting for my discharge paperwork, a research assistant shows up to see if I’d be interested in participating in a study about I’m not sure what because my 3yr old is clambering to get out of that office. I agree anyway, get my discharge paperwork and fly out the door.

This summer, we were visiting family in Philadelphia and my son got sick. He was admitted to Children’s Hospital of Philadelphia for a pulmonary exacerbation. Not a single morsel of information about him and his history with Cystic Fibrosis was available to the doctors at CHOP. Many phone calls were made between fellows from my personal cell phone to understand his medical history and doctors asked me the same questions day after day before we trusted and understood each other and hashed out what was going on in the days leading to our admission. I know that it was in Drew’s best interest that we discuss everything about him ad nauseum, but it blew my mind that in this age of technology, there was no electronic medical health record that the doctors in Philadelphia could access to understand Drew to provide the best care for him quickly.

When Drew was a baby, we kept a diary of his formula intake and the corresponding output. We set up a nice little Excel chart and shared that with our dietician on a very regular basis. I credit that chart for his gained weight and reaching the 50th percentile by the time he was 6 months old and he has been able to maintain that to today when he is almost 3. I know that all patients aren’t equal, but wouldn’t it be great if that data, if our “patient reported outcomes” were cataloged somewhere so that we could share what we did and how it worked for us? Sure, I could take to the CF Mom’s Facebook page and ask about what high calorie snacks work for their 3 year olds and hope that some of those moms are online and eager to provide some feedback. But once that question is asked and answered, it just simply vanishes into cyberspace. I cannot tell you how many times I’ve said, “I know I’ve seen that somewhere”. Image the power of cataloging that data, those conversations, those findings. It can be used by patients, by doctors, by researchers and teachers. Just thinking about the power of that is what is driving me to stay involved and insist on nothing less.

I think you’re seeing my point, a point that was brought out in almost every session I sat through at the NACFC this year. The information that we currently have on patients in between visits is limited. Filling those gaps would give us a more complete picture of health. It would help doctors to intervene at appropriate times, times when patients might not call because in their mind “its just not that bad yet”, but doctors are able to identify a problem or a pattern that lets them know the direction something that’s “not that bad” is headed. They would be able to more easily determine if certain therapies were actually making a difference through the combine use of passive behavioral and active patient reported data, looking at a real-time view of what is going on with a patient, not what they remember to tell you when they are in clinic. The registry could be enhanced by identifying day to day trends and commonalities in patients sharing mutations. And all of this data can be used by researchers and scientists to figure out every last detail this disease and find therapies that work for every one of us. From a parent’s perspective, the C3N is what we need to make life easier and improve outcomes while we wait for our cure.