If we all work together, everyone wins.

If you're unfamiliar with my blog, I have a 3yo son who was diagnosed at birth with Cystic Fibrosis, a progressive and fatal genetic disease for which there is no cure. On this journey, we have the support of many family members and friends, an outstanding clinical staff, and a National Cystic Fibrosis Foundation Center that is transparent with its outcome data in an effort to promote awareness and research and fund a cure. We're getting there, but it can't happen soon enough. I spend a lot of my free time trying to think of or find ways to make life easier or do things better, things that could have an impact for my son and for other people in the CF community, and ultimately change outcomes. From medication management to insurance headaches and everything in between, I have an image of what life with this disease could look like. It would involve seamless tracking of symptoms and medications and treatments. It would include the opportunity to communicate with your doctor in between visits in a way that didn't involve phone tag. It would allow transparency in all data, allowing me to see the results of my tests and the notes that the doctors have on how I'm doing, eliminating duplication and error. I would more easily be able to find information on my disease that is relevant to me without having to stumble upon it on Facebook. And as for the things that I don't know I don't know? My doctor could be proactive in providing those things to me through the collection and organization of information that is already being shared in chat rooms and on social media. All of these things would be organized and tested and implemented so that I'm not the only person benefiting from what I want or what I want to know. 

I started tracking Drew's health a couple of months ago. I record things like his weight and cough frequency as well as his appetite, stooling patterns, oxygen saturation and daily calorie intake. I've been able to share this information with his doctors to give them a more complete picture of his health in between visits, them learning about obstacles that we face in adherence to daily care regimens and me getting suggestions for ways to overcome some of those obstacles. We've worked together on additional measures to track and have discussed many ways to make life with this disease better while we wait of a cure. Being an active and engaged parent in a complicated chronic disease, what I want most is something to help alleviate the burden of care - the time it takes, the money it costs, the energy is soaks up. Enter Ginger.io.

Ginger.io is a behavioral analytics platform that turns smartphone data into health insights. I think we can all agree that people behave differently when they aren't feeling well. By collecting your behavior patterns - how often you text, the times of day you make phone calls, different movement patterns - Ginger.io aims to map behavioral patterns to create insights that, long term, have the potential to predict and prevent future problems. It's still being tested out, but I hope the Cystic Fibrosis Community and all chronic conditions will be able to benefit from a tool like Ginger.io. 

Cincinnati Childrens has teamed up with Ginger.io to develop a research study for Inflammatory Bowel Disease (IBD) patients to track their symptoms. As part of this study, patients can even get paid for tracking their data.  What if finding a way to treat one chronic illness like Crohns Disease, with something as simple as a mobile app, could help to find a way to treat another chronic illness like Cystic Fibrosis or Sickle Cell Disease. If you know anyone with Crohns or Ulcerative Colitis between the ages of 13-25, have them check out this study. If we all work together, everyone wins. http://ginger.io/join/c3n/

Good things keep happening!

Lots has been going on around here lately. In addition to it being a packed summer full of swim lessons and play dates and vacations, the CF Foundation has decided to move forward with a partnership with the C3N project! YEAH! We were able to share with them the value that we see if collecting and sharing new ideas and ways of managing health in CF and how the C3N project is just the place to do that. I've been keeping busy as we start to kick this thing off, creating a brand for it and figuring out a communications plan and implementations strategy. Late this week, I'm having a meeting with some folks from the foundation to share the data I collect on Drew and discuss other things that we could measure and what we could learn from the stuff we are tracking. We are also working with the UC Design School to figure out what this project needs to look and feel like so that it will work for everyone.

Drew is doing well. We had a regular clinic visit about 2 weeks ago right after we returned home from Philadelphia and our little adventure downtown for a night. His weight was down a little, which wasn't surprising given that he doesn't eat anything. We decided to start tracking his calorie intake and charting it in my PersonalExperiments with the rest of the data I collect to see what he is actually getting in and where there's room for improvement. We also started giving him Ensure Plus instead of just Ensure. I've been so focused on fat, so we decided to shift that focus to calories for a while and see what it gets us. After just two weeks of tracking, we are getting in over 2,000 calories most day, and his weight has gone up .2kg each week over the past two weeks! While keeping a food diary certainly takes some time, MyFitnessPal has made it incredibly easy for me to quickly track both what I'm giving him and then by adjusting serving size I can track what he actually takes in. The dietician can log in to see his data and his calorie distribution throughout the day, and offer suggestions based on what he seems to enjoy or what meals he seems to eat the most at. I take the total calories in a day number from MyFitnessPal and put it into the PersonalExperiments and then I can see how his stool output or cough frequency relates, if at all, to his appetite and calorie intake.

The other exciting thing that happened is that we found an electronic stethoscope company who is interested in partnering with the C3N project! They are going to let me test out the tool and we will work together to see how we can quantify breath sounds and record them and append them to the data I already collect to see what value that can bring to care. I don't have it yet but I'll definitely be updating the world once I get it and start using it.

That's all the updates I have for now. Trying to keep cool and busy this summer, and next week we head to Michigan for a little vacation. More updates as they break!

I lived my "What If..."

A few months ago, I shared a story with the CF Foundation about my vision for the future of chronic care management. If you missed it, you can read it here. Last week, I lived my "what if". Let me explain.

About two weeks ago, we packed up shop and headed east for our summer visit to the grandparents house just outside of Philadelphia. About a week into the trip, Drew developed a cough. He'd been doing pretty well and so this cough was new but not anything that I was terribly worried about. Two of his siblings had runny noses and coughs and I'd figured he had just caught the virus that they had. After a few days, the cough frequency was on the rise and his oxygen saturations were on the decline. I track many of his symptoms/behaviors with a tool called PersonalExperiments so I could see from his data that this was the case. I put in a call to his Pulmonologist back in Cincinnati suggesting we start an oral antibiotic and after some discussion she agreed that that might be the best next step.

Later that night, Drew went into some serious coughing fits like I had never heard before. He would cough and gag for hours on end, getting little to no relief from breathing treatments or airway clearance. He finally fell asleep around 2am, but woke up worn our and not much better. The next day he had another one of these intense coughing spells, and his doctor and I felt that he might benefit from an oral steroid as his symptoms seemed to indicate that there may be some serious inflammation causing the intense coughing fits. That evening, we started both the steroid and oral antibiotic and he went to bed. Then, at 11pm, 2am, 4:30am and 6:30am he was up again, coughing and gagging, still getting little relief from breathing treatments, so we decided that it was time to head to the ER.

We arrived at CHOP around 11:30am and were immediately taken back into a room. The doctors and nurses listened to my whole story and I shared with them the data I'd been collecting so they were quickly able to see what had been going on. I also gave them access to his EHR through MyChart, where they saw his bacteria sensitivities from his most recent culture and decided which antibiotics would be most effective in treating his exacerbation. We had checked in, gotten an x-ray, done a viral panel and an epiglottic culture and were admitted and moved up to a room in under 2 hours. If you've never had the opportunity to visit the ER, that quick of a conversion from arrival to admission or discharge is unheard of.

Once we were up in our room, we were greeted by a resident before we had even been screened by our nurse. She asked to see the data that I had been sharing in the ED (good news spreads quickly!). I shared my data with her and expressed my apprehensions about this being an exacerbation. An exacerbation is pretty much defined simply as a temporary worsening of the lung function due to an infection or inflammation. Although no formal definition exists, an exacerbation is generally characterized by the following symptoms:
1. Shortness of breath
2. Fatigue
3. Increased cough
4. More productive cough
5. Drop in FEV1 or other markers of the pulmonary function tests

The protocol at many hospitals is to admit and treat with IV antibiotics if a patient is exhibiting these symptoms, as the assumption is made that they are suffering a pulmonary exacerbation. I did not believe Drew was having an exacerbation. While I haven't been collection data for long enough to have caught an exacerbation, I recall him being symptomatic 24/7, and Drew's serious symptoms seemed somewhat isolated to sleeping or laying down. I had notes in my tracking that called out the times that these things were happening, and at 3pm he was running around the hospital room and playing without a care in the world - asymptomatic. I thought that Drew had caught the cold that his siblings had and while he was sleeping/laying, the post nasal drip was throwing him into these coughing fits. His symptoms just didn't fit the bill of his "typical" exacerbation.

Based on the data I shared from his EHR, the attending doctor came in to discuss the IV antibiotics that they wanted to put him on. If I was going to agree to IV's, they chose the two that I would have also picked based on the positive response he had to them in the past, but I wasn't ready for IVs. Once you start two weeks of IV antibiotics, it isn't common to stop them until they're complete. I once again pulled out my data and had a discussion with the doctor about waiting to see if the steroid and/or the oral antibiotic that he is on would kick in. Since we were already in the hospital, he agreed to monitor Drew overnight before starting IV's on two conditions: I would agree to start the IV's if his oxygen dropped any lower (he was at 92) or if he had any more coughing spells overnight. He had started the steroid the night before so had only had 1 dose of both that and the oral antibiotic, usually not quite enough time for Drew to respond. He was also on inhaled Colistin as we work to eradicate his Achromobacter. The benefit of Colistin is that is covers pseudomonas and the Cipro he was on covers a number of other bacteria that he has grown in the past. It just seemed to me unlikely that he was having a true exacerbation. 

So we hooked him up to a Pulse Oximeter and put him to bed. Moments later, a doctor stopped in to tell me that his culture came back positive for Rhinovirus, otherwise known as the common cold. It is known to cause a lot of inflammation and it's not unheard of for kids to have lots of coughing with this virus. Kids who have tracheomalacia, Drew, often do end up in the hospital because their airways are under attack more than they can handle on their own, and many times need a steroid to help reduce the inflammation and help them to get over the hump. For the first night in 3 nights, he slept all night long without coughing. His oxygen levels gradually started to go up on their own overnight. He woke up in the morning refreshed and looking/acting well again. I needed to take him to CHOP that morning because I was starting to reach the point of uncomfortable managing it from home in the case that things would get worse before they got better. But had I given it one more night, one more chance for the steroid to kick in and do it's job, we probably could have avoided the hospital altogether. 

We were discharged at 10:30am the next morning, just under 24hrs after we had checked into the ER. Because I had been tracking his health so closely, because they were able to access his previous test results, because I felt empowered enough to speak up and express my perspective and desired course of action, and because I had given them the evidence to trust me, we found a mutually agreed upon solution that saved us two weeks in the hospital. It saved Drew the stress of being in the hospital for 2 weeks. It saved me the trouble of trying to arrange my life for a 2 week hospital stay. It saved the doctors and the hospital time and money. My insurance company wasn't being billed for unnecessary tests and an extended hospital stay. 

I have data on Drew. I know the signs and symptoms of a pulmonary exacerbation. I know the difference in his cough frequency or appetite that is associated with the onset of symptoms. I know what has worked for him and what hasn't. Having that data and sharing it with anyone and everyone who might be able to better help him or benefit from it personally is why I track it. I lived my "what if" and I couldn't be more pleased with the way it turned out. 

If I had a stethoscope and a tank of oxygen...and maybe a medical degree

We were admitted to CHOP this morning. After 5 days of an increased junky cough and then two days of incessant coughing and dropping oxygen levels, I finally threw in the towel and brought him into the hospital. We're visiting family in PA so I've been talking to Drew's doctor back in Cincinnati regularly over the past couple of days. We first added an oral antibiotic to try to kick this little extra whatever you want to call it. Then we added a steroid, which sometimes helps him because of his airway reactivity and tracheomalacia. Then last night, after he'd had breathing treatments and airway clearance at 7pm, he coughed and grunted throughout the night, doing treatments again at 11pm, 2am, 4:30am and 6:30am before I decided that this was simply more than I could manage from home and wanted to get him in before things got any worse.

I have to say that this was by far my most pleasant ER experience...of all time. It was quick and easy and straightforward. The doctors listened to what I had to say, and I credit some of that to the data I was able to share with them both from my PersonalExperiments tracking and from MyChart, the way we access all of Drew test results and medications and medical history, basically his EMR. They were able to quickly see when this started and how it all unfolded, and then see from his latest tests and cultures what his antibiotic sensitivities are rather than having to call his doctor or wait for culture results to determine the best course of action. Within 2 hours, we'd had an x-ray, done a viral panel and an epiglottic culture, and were admitted and transferred up to a room. High five to CHOP's emergency department for a job well done.

We got up to 8 South around 2:30 this afternoon. I was pleased with how quickly we saw a doctor who did a very thorough exam and asked lots of questions and listened to everything I had to say. I was less pleased when I did the same thing the second, then third, then fourth time with different residents, fellows and attendings. We missed his afternoon breathing treatments because no order was ever placed for his inhaled medications, despite the fact that I rattled them off, doses and all, more times than I can count. I even brought a bag of every medication he takes with me in case we couldn't get something in time.

After a few hours, I spoke with the attending doctor who was trying to figure out the best antibiotics to put him on. Their protocol is to,while waiting for the results of a culture which could take up to 5 days to be finalized, treat with IV antibiotics when the symptoms are such to suggest a pulmonary exacerbation. I expressed to them that this wasn't his typical presentation of an exacerbation. He is acting normal. He isn't coughing 24/7. He's not acting sick. But he goes into these fits where his oxygen is dropping and hes coughing so hard that he's gagging, and nothing that I could do at home was helping. It seems more like an asthma thing to me than his normal "sick". Something was going on that was causing more inflammation than normal, and I wasn't convinced that starting him on IVs was the best option. I believed that the steroid plus the oral antibiotic that he just started on would probably get him over this hump, but needed some help watching him or providing oxygen when necessary until we got there. The doctor agreed with me that watching him overnight would be a good option, to see if the oral antibotic and steroid start to kick in, or if something has set up shop in his lungs and needs a serious IV antibiotic to kick it. I appreciated that we were on the same page, and was happy to just be here knowing that we have what we need in the room with us (or just down the hall) if he has another episode.

Around 5:30pm, some respiratory therapists came in to talk about evening treatments, and I mentioned that we still needed the afternoon ones. It took them a little over an hour, but they were able to gather the appropriate supplies to get him done and said they'd be back after 8 for the night time nebs. I mentioned that Drew goes to bed around 7:30, but unfortunately that's rounds, so he would just have to wait. My poor, sick, exhausted boy, has to wait. So we ate dinner and watched a movie and then he got real tired. He is 3 years old. He hasn't slept in two nights because he has been awake coughing and doing breathing treatments. But I have to wait.

At 8:10, two respiratory therapists came into our room and told me that they only had two of his medications. One of the ones they had wasn't even a medication that he uses at night. Two of the more important medications that he's on they didn't have yet. Somehow, during those half-a-dozen conversations that I'd had with seemingly everyone who works here, no one put in an order for Pulmozyme. I insisted that he have it this evening so they had the doc put in an order and then we waited. The other medicine, an inhaled antibiotic that he's on as we try aggressively to eradicate or at least tame the achromobacter problem we've been dealing with since January, wasn't something that they use typically use here I was told. I explained why we are using it and that I's brought his with me, enough to get us through till Friday, but was told that I could not use my own medications. The nurse took one of mine down to their pharmacy for approval and said that what they have is exactly the same as what I have and that we have to use theirs, but first infectious disease has to approve it.

It is now 9pm. Drew is a hot mess of tears and tired energy. I asked how long this approval would take and they told me it could be several hours, but he would almost definitely be able to have it again in the morning. IN THE MORNING!? Did I not just express to you the importance of following this strict regimen to try to eradicate a mostly antibiotic resistant bacteria that is setting up shop in my 3yr olds lungs and starting to cause lung damage?! She explained that it is their policy to have infectious disease approve the use of all inhaled antibiotics because of the risks associated with exposure both to him and to other people who might be exposed to it. I understand that there are policies and procedures in place for many reasons. We are here because you are monitoring him. Right now, the hospital is doing absolutely nothing for him - he is not on antibiotics, he is not on oxygen, he does not have leads on his chest, I have not seen a nurse since she was in here at 9pm. The only thing that is happening right now is that you are denying him access to a medication that he needs, a medication that I have sitting right next to me on a table that I could give to him, but that your policy prohibits me from doing. And no matter who calls or how many times they call to try to expedite this process, we simply have to wait. "We have a policy" is simply not a good enough answer for me.

I appreciate the resident who listened to my concerns and simply asked that if I do decide to use my medicine and do the treatment on my own that I notify the nurse so that no one enters his room for 30 minutes, because that's their policy. I don't want to go against their policy, but I am also not going to skip a dose of this because of it. I was hesitant to accept anything less than an immediate solution, but told them that I would wait until 11pm and then I was going to do it myself, and that I would let the nurse know that I was doing it. He's supposed to get this every 12 hours. He last had it at 7am, and he will get it again tomorrow at 7am. Additionally, the poor thing just needs a good night sleep, and when I hook him up to this it will inevitably wake him up. I am so frustrated, but at the end of the day I'm going to do what's best for my son, even if I end up breaking a policy.

I hate being in the hospital because of things like this. When my son is sick he needs 4 things: medication, nutrition, airway clearance and rest. I can provide all of those things in my home. What I can't do is listen to him with a stethoscope to hear how things are. I also don't have oxygen at home to give him if things start going bad. I suppose I could get both of those things, but while I'm a caregiver (and a pretty good one if I do say so myself), I don't want to be his doctor. I want to be his mother. I want to trust that everyone is doing everything they can to keep him well. Sadly, I care about him a whole lot more than anyone else does. I do believe that his doctors and nurses and respiratory therapists do their best to keep him well while he is in their care, but I still care more. I know him best and I know what's best. It's now 11pm and I'm about to break policy.

Short Summer Update

We're hanging out in Pennsylvania for a little bit, visiting with family and just generally relaxing. Drew started on inhaled Colistin about 10 days ago and once again his cough disappeared. Just about two days ago though he picked up a cold or something and has been coughing up a storm. I don't think this is his stereotypical reoccurring achromobacter cough. Unfortunately, his oxygen sats are dropping a point every day. I'm still hopeful that whatever is going on can & will go away on its own, but if the oxygen keeps heading this direction we're either going to need to find a doctor in town or head back to Cincinnati. Cross your fingers that we can get this sorted out!

Creating and Sustaining Collaborative Networks in Pediatrics

This past Thursday and Friday, I spent time in Alexandria, VA participating in an amazing meeting called “Promoting and Sustaining the Collaborative Network Model in Pediatrics” that was sponsored by the American Board of Pediatrics Foundation (ABPF), the Children’s Hospital Association, the Cincinnati Children’s Hospital Medical Center (CCHMC) Learning Networks Core, and the Pediatric Center for Education and Research on Therapeutics at CCHMC. The purpose of this was to engage experts to address how to sustain multi-site networks that are so essential to translating evidence into practice, building the scientific foundation for discovery and improving child health. We reviewed lessons learned from successful networks that engage a variety of stakeholders, including patients, parents and family and representatives from academia, government, industry, and philanthropy. In attendance, there were patients and parents, clinicians, network leads, and representatives from a variety of organizations: children’s hospitals, public and private payers, federal research agencies, professional organizations, children’s hospitals, and foundations. The aim of the meeting was to create an action plan for change - find ways to create these networks - and I was there to let them know of my experience with networks and why we need to work together to develop the networks that already exist and to use those learnings to create new ones [with specific and selfish interest in creating one for CF].

I was asked to speak before we broke into our working groups, connecting the points discussed throughout the meeting, and here's what I had to say.

Good afternoon!

My name is Erin Moore and I have a 3 year old son who has Cystic Fibrosis. He has a 50% chance of living to age 41, and I am here today because I am determined to beat those odds.

Several years ago, shortly after Drew was born, I was invited to be a part of a Facebook group called CF Mamas. It's a group of over 1000 parents of children with Cystic Fibrosis, sharing everything from recipes to heartache. A few weeks ago, someone had shared that they were anxious about their daughters sinus CT scan later than day because she was going to be sedated. I was quick to comment that my son, at age 2, had undergone a sinus CT scan without sedation at that very same hospital, and that she ought to ask about it. Moments before their scheduled procedure, she asked if they could do the scan without sedation and they agreed to try. Thirty minutes later, that woman posted on Facebook that the scan was complete and that she was on her way home with her daughter - not sitting in recovery waiting for her child to wake up, not taking up a room waiting for a doctor evaluation before discharge, she was already in her car driving home. A simple conversation on Facebook that I just happened to stumble upon at exactly the right time saved that family time and stress. It also saved them and the doctor and the hospital and even the insurance company money by not having to sedate that girl...all because of a conversation that I stumbled upon. This mom, like many moms, didn't even think to ask about this as an option. Or perhaps she did, but the doctor who ordered the test was prompted to schedule time for sedation and everyone just assumed that that's what had to happen. No one expected or even fully appreciated the value derived from that innocent post on Facebook to a group that a mom expected nothing more from than perhaps some empathy. While I'm not suggesting that funding isn't important, I think it's a myth to think that the only solution to improving care is to spend a ton of extra money.

On the other side of this, I have seen how harmful information sharing within this vacuum can be. Information often takes on a life of its own and inaccuracy becomes fact. The medical decisions that some of these parents are making without medical evidence or expertise is scary and dangerous. I have seen medications stopped altogether because another parent shared insights from a non-scientific non-medical personal experiment that they had performed, and the results scared others when they believed and held that data as truth. I'm certainly not opposed to personal experiments, I do many myself, but I share that data with my sons doctor and we work together to find insights and value before taking any kind of action. The lack of clinician involvement in these networks is allowing misinformed parents to decide the value of information they are hearing.

I've had the opportunity to see both the positive and negative side of this organic, unstructured, patient-only network. What it has shown me is an opportunity to improve the value by having this data organized and catalogued for future reference and use. Opening this group to other contributors has value - doctors, patients, researchers - using their collective knowledge to create a learning network full of valid and robust data, where question asking and idea sharing is encouraged, finding out what other people are doing that is working, will truly generate "best practices". Patients are creating these networks with or without clinicians, and we're all here today because we see the opportunity to work together to enhance these networks and create learnings. It is both time and cost saving and ultimately holds the promise of improving outcomes.

I am not just "a patient". I am a mother, a wife, a daughter, a sister, a niece, an aunt, a neighbor and a friend. I have experience as a marketer, a researcher, a photographer. I have sold Tupperware, makeup, knives, grills, linens and Girl Scout cookies. I have served coffee and cosmos and jury duty. I am a volunteer, a blogger and an advocate. I suspect that many of you here today are more than just clinicians. My point is that I have perspective to bring that might not always be viewed as relevant, but the collective knowledge of micro-experts, patients, myself and the many other parents that are fighting so hard to keep their children healthy and alive, what we have to say should be included and valued.

My son, at age 3, has a multi-drug resistant bacteria growing in his lungs that has had us in and out of the hospital since January. What I need is access to real-time, trusted, reliable information on everything from novel approaches to achromobacter eradication to reasons why having a lizard as a pet could be detrimental to his health. I want to know what other parents know and have tried, and I want a doctor to weigh in on why those options may or may not be good for me. I want to hear about what tracking methods people use and what they use to connect with their doctor between visits, but even more, how its improving their care. I want other patients to feel empowered to ask about alternatives and suggest trying something new to their doctor, no matter how alternative it may be.

And before I close, let me ask you this question: Is it more preposterous to think that the most relevant, valid, useful, action-worthy information about a disease could come from outside the establishment? Or that a life-saving treatment option can't be found published in any journal?

I truly believe that this connected technology, these collaborative networks lower barriers to engagement, empowering and enabling all, and I appreciate your dedication to working together to help in creating them.

- Posted using BlogPress from my iPhone