I admit it, I'm tired

I've gotta be honest, i'm exhausted. This whole back to school thing is kicking my butt. It's not just the back to school, it's also the extra hour and a half of treatments that we now need to find time for in a day, and the swim lessons, and the dance lessons, and our upcoming fundrasier (which will be awesome). This is how yesterday went:
Alarm goes off at 6:45. I know that's not early for many people, but the Moore family rarely sees the 7am sun. We didn't get out of bed before 8am but a few times all summer. Now school starts at 7:45. So the alarm goes off and I go to wake my daughter for school. We are dressed and downstairs by 7. I simultaneously make her breakfast, make myself coffee, and pack her lunch. By 7:30, my husband is downstairs, as are the other 3 kids who all want food, drinks, and a different cartoon on TV. I spend about 30 minutes pacifying that bunch while #1 heads off to school with dad. By 8am, I start to get Drews medications ready, checking the calendar to make sure I'm not giving the Tuesday-Thursday-Saturday pill on a Monday, Wednesday or Friday. The liquid antibiotic that he's on for inflammation, not antibiotic properties, is only given on Monday-Wednesday-Friday, but while a small bottle yields a little more than 12ml, it's only good for 7 days after mixing, and at 3.6mls three times per week, I need to try to recall when this bottle was mixed as there seems to be enough left for another dose, but it might not be good anymore. I dispense all other meds and assemble nebs, mixing up our inhaled antibiotic that literally smells like cat urine. It goes in a different kind of neb cup, but when Drew sees it he  freaks because 1) a mediction that smells like cat urine is misted into a mask strapped to his face and he knows how awful it smells, and 2) he knows that when he sees that neb, he has to sit for twice as long as a regular treatment beause it takes nearly 30 min to nebulize that medication. So I mix the medicine, hide the neb, and tell him its time to start treatments. It's now close to 8:30. This is problematic for a couple of reasons. First, this treatment will last around an hour. He is supposed to get the inhaled antibiotic 3x a day, spaced every 8 hours. There aren't enough hours spent awake for him to accomplish this, so we settle on trying to evenly space them. The other problem is that swim lessons start at 10am, which means that between the time we finish at 9:30 and 9:45 when we need to leave, I need to get two 3yr olds into swim suits and shoes, find towels, buckle in three kids, grab something to entertain the baby, and back out of the driveway so that we arrive with enough time to use the restooms before getting into the pool. It may seem silly that we do swim lessons...or dance class...or tball, what with everything else we have going on, but staying active plays a big role in staying healthy with Cystic Fibrosis. And while there may be no scientific proof to this, since we started swimming, the severe sinus disease has all but disappeared. I wouldnt want to be the guy swimming next to my little man, cleansing those funked up sinuses, but if a little chlorine can do the trick and keep us from another sinus surgery, then we will be efficient with that 15 min window that we have to get our act together. Move it or lose it people.
Swim lessons are only 30min long, thank God, because we need to get home to eat an early lunch because, you guessed it, we have another treatment to squeeze in before we leave for school at 12:15. Three year olds move at warp speed in almost all areas of their life, except for changing out of a wet swimsuit. This is also when the independance that I pushed them so hard toward comes back to bite me - they do not need my help, they can do it themselves. I try to be patient and watch and wait but we're on a tight schedule and FOR THE LOVE OF GOD why is it taking you so long to put on underwear!?! Focus people!
We manage to eat a quick lunch and get in a treatment before its time to buckle everyone in, again. Drop off is at 12:30 and we are all ready for it. Now its just the baby and I, with a quick stop at the post office to make before heading home. I imagined the post office to be quick and easy with just 1 kid in tow. I have a wild imagination. If I could send a package via carrier pigeon from my back porch, I'd buy a damn pigeon.
We're home before 1 and we're both ready for a nap. He gets one, I get to do the dishes, the laundry, the prescription reordering, the insurance claim negotiting, the [brief] treadmill walking, the dinner making and then the baby waking because at 3pm we need to pick up #1 from kindergarten. Back into the car we go at 2:45 and head off to school. #1 is tired but happy to see us and have some mostly uninterrupted attention while we wait for 2 & 3 to get done preschool.
At approximately 3:30 pm the fighting commences - because it really does matter who gets to the car first, who gets buckeled first, who had the best day, who made the first song request, who asked to go the back way home, and who isn't being treated fairly, by me, on our 5 minute drive home.
After we get in the front door and clothes are changed and drinks are distributed, there is a consensus to go outside to play. It's really not fun at all, by anyones standards. Exhaustion, in my house, shines through in the form of fighting, whining and crying. We remain outside until dad drives up, home early so that I don't pull out all of my hair. I warm up the dinner that I made earlier in the day, everyone complains that they don't like it, and I decide I'm not making anything else. Aftter all, if they starve to death there will be fewer mouths to feed!
At 6:30, its time to start the third and final treatment of the day. The kid are filthy, so we divide and conquer, one of us mixing and organizing medicines and devices, the other dealing with tired, dirty kids. The whole evening comes to a close shortly before 8pm when teeth have been brushed and stories have been read and four little people who I love more than anything in the world are ready for sleep.
We spend the next hour cleaning up our house. If someone had come to rob us, they would swear our house had already been hit. Baskets of clothes emptied to be used as cages, food and drinks scattered about, "artwork" taped to any and all open wall space. By 9pm, life seems to be in order. I want a drink, and I'd have one but being anything less than 100% the next day leaves me at an even greater disadantage than I'm already at. And lets be honest, I need more than just A drink. For an hour or so, I feed my Twitter addiction in between ordering school photos, registering for the PTO, sending out sad emails begging our families to purchase something from the magazine drive so that #1 can dress down one day next week, and checking the calendar to figure out how tomorrow needs to run.
I finally get to bed a little after 11pm, but, SURPRISE! The day isn't over yet! At 3am we are awoken by a screaming, bloody 5yr old who fell out of bed and split her forehead open. If we had a suture kit in the house, I would have considered sewing her up myself because 1) who wants to run to the ER at 3am and 2) we were literally just there two weeks ago getting staples in someone elses head for a trampoline injury and I'm not in the mood to hear the lecture on keeping my house safe for my kids. One of these days they'll call Child Protective Services and I'll contimplate the relief it would be if they took them away for just a few hours until they read my blog and realize that I'm just a mom trying to do the best that I can.

Not out of obligation, but out of love

Drew still has achromobacter. We just got the test results a few hours ago. We'd spent more or less all summer on a series of inhaled antibiotics, spending 3-4 hours every day, at home and on vacations, to try to eradicate this antibiotic resistant beast, but to no avail. I guess I had accepted that we probably we never really going to get rid of it, but I had still hoped that it would happen. We have another clinic appointment tomorrow morning to figure out what's next, but I think [read: know] that he will be considered colonized with Achromobacter and we will start to cycle inhaled antibiotics. If we have to live with this, I hope that we can at least keep it suppressed to the point that it's not causing any problems, seen or unseen.

Preschool starts on Wednesday. We're going in "healthy" and I'm not looking forward to the chaos that early fall will bring with exposure to 18 three year olds and all the germs that accompany them. I met with Drew's teachers last week and am happy that we have chosen to send him where we did. I am hopeful that they will do everything that they can to keep this disease invisible to him and help him to learn and grow the way that I hope for him to. I think he's going to love preschool, and if he gets sick and has to stay home, I think it's going to be a major bummer. There isn't much that we keep Drew from doing, not much that the CF prevents him from participating in. But man, those times when he's got his heart set on something, even something simple or insignificant, and we have to stop him because he needs a treatment, or needs to be hooked up to an IV, he gets so bummed. Those are the times that my heart hurts. But then I curse CF and get all fiery and go trying to change the world (it's happening, more on that later).

The other [selfish] reason that I'm hoping Drew's health remains good is because I'm attending two conferences this fall and leaving the family in the good care of my husband. Not that he wouldn't be capable of taking care of Drew if he were to get sick, but Drew getting sick turns this family on it's head. With 4 young kids, some in school, some in activities, all needing care at all times, it's a nightmare trying to keep things in check when one of us has to be in the hospital with Drew or even at home managing administration of IV medications on top of all that we already manage in a day. 

Speaking of conferences, I was invited to speak at Stanford Medicine X ! I'm going to be on a panel about what it means to be a "patient" in 2013. I don't know if I ever announced it on here, but the CF Foundation is moving forward with a partnership with the C3N project at Cincinnati Childrens. (Yay!) I talked to people and shared my story and shared my vision and started tracking and started engaging in Drew's health at a different level and the results have been outstanding, and some prominent people in the healthcare world have caught wind of this and that's how I got the invite. The stuff that I'm working on at Children's isn't rocket science. It's engaging healthcare professionals in new ways, and sharing ideas about things that could improve health and care, not just in Cystic Fibrosis, but in chronic diseases all around. I'm showing people that everyone has expertise, that I can learn from them and that they can learn from me. And at Stanford at the end of September, I'm going to learn and to share with a ton of great people. I'm honored, excited, and just a little bit nervous to be in the presence of such an incredible group of people. 

The other conference I'm going to is the North American CF Conference in Salt Lake City in October. I'm totally pumped about that one too. While many of the other participants there (docs, other clinicians, researchers) might not feel the same way that I do, I'm there to learn but also to share and hope that they will be able to learn something from me too. I won't have a podium or an audience at that conference (not yet!), but I will talk to people about my vision for a better future and how we can all work together to get there. 

Another exciting thing that happened this summer is that I was interviewed for a piece on NPR about the "Quantified Self" movement (you can listen by clicking here - the whole thing is really awesome and I highly recommend listening if you have the time, but my interview starts around the 24 min mark). 

On Friday night, we went to a CF Fundraiser that another family that has a child with CF was hosting in Northern Kentucky. I'd never met them before, just heard about their event through Facebook and knew of another family that might be attending. We decided to go, both to support the family and the CF Foundation through the fundraiser, but also to show them that we care, we get it, we've been in their shoes, we know how it feels day in and day out, always hoping for the best, sometimes dealing with the worst. It ended up being a really great night, in terms of money raised for CF and the company that we had for the evening. 

Our Doin' It For Drew Oktoberfest Fundraiser is coming up on September 21 (all are invited, bring your friends!) and as we go into another fundraiser, I trudge through realizing the importance of these funds that we raise, but hesitant to ask the same people for another dollar, more of their time, more of their energy. At the event on Saturday night, they had programs and I have to share the message that they wrote on the back because I couldn't have said it better myself. It may not have been written by me, but the message that I share below comes from my heart. 

"There's little we can say to show our gratitude for your generosity & support. There have even been moments we've felt guilt for all that has come our way; that we were asking too much or putting those around us in a place of obligation. But then we realized something that deep sown we had known all along. That God had been preparing us for this our entire lives, and that through our [son] we would learn of you, our "silent army" that has been surrounding us all along". 

Thanks to all of you, our family, our friends, complete strangers who we've been touched by our story, we've raised $25,000. We hope and pray that you will continue to fight with us until CF is a thing of the past!

Unaccountable

I've been reading this great book called Unaccountable: What Hospitals Won't Tell You and How Transparency Can Revolutionize Health Care. It's really good, and a lot of what's in it is stuff I've been working to change. One of my favorite quotes from the book is this: "In short, data transparency, properly weighted, would empower patients to make informed decisions about where they should spend their health care dollar. If we had more of it, the accountability visited on hospitals would revolutionize the quality of medical care in every city in America, dramatically reshaping our health care landscape." I do believe that to be true. 

We just spent a week in Michigan with my husbands family. It was so nice to get away for a few days. Drew has been doing pretty good lately, knock on wood. We're about to finish up 60 days of inhaled antibiotics. I have little hope that the achromobacter will ever be eradicated, but it's been nice to be cough free and generally healthy. What I suspect will happen is that he will continue to culture the achromobacter and we will cycle 30 days on/off of an inhaled antibiotic. I just hope, so so much, that the 30 days off won't be symptomatic and causing him harm. I guess only time will tell. We finish up this Friday and will do a culture next week. He sees the doctor again two days before he starts school so hopefully we will at least get a clean bill of health to start out the year.

School is definitely been on my mind. I'm trying not to worry to much. I know that he will be great, that he will love it and enjoy being around others and learning and playing and just being away from me for a bit, but I worry that he's going to get sick. No one takes care of him like I do. No one follows him around with hand sanitizer to make sure his hands are clean before he eats and drinks or after he's been outside playing like I do. No but me makes sure that the kid next to us at storytime isn't coughing and sneezing up a storm. We have never missed enzymes at any meal or snack or drink ever. And now I have to give some of that responsibility over to Drew and to his teachers, and I'm definitely stressin'. I know it will all work out, but it's just a change and sometimes change can be hard.

I wish I had more time to write on the blog. Summer has been great, but very very busy. When school starts in just a couple of weeks, I'm hopeful that I can find some more time to jot down more of the things I'm thinking about and working on!

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.