Wednesday, June 26, 2013

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.

Sunday, June 23, 2013

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!

Sunday, June 16, 2013

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

Friday, June 14, 2013

Clear lungs, full hearts, can't lose!

What a week! We found out on Monday that Drew was still culturing achromobacter. Much to our disappointment, it is looking like we're not going to get rid of this bug. The question turned from how do we get rid of this to how do we suppress this enough that it's not causing a problem. There was a period of time where it looked like he was going to need to be on constant antibiotics to keep this at bay, but this for us was not a good option. He's only 3 and things just shouldn't be this bad yet! So we decided with his doctor to do a high-res chest CT scan to see if it was causing any lung disease. Talk about an anxiety filled couple of days waiting for that to happen, then waiting the few hours for the doctor to call after the procedure for the results. We had met with his doctor on Monday to talk about our options - options for if it looked good and options for it it looked bad, or even really bad. I was so nervous, but after all of that waiting and worrying, his CT came back 100% clear! There are no signs of bronchiectasis or mucus plugging. Age 3 and no lung disease yet.

While that was great news, we're still culturing achromobacter that is causing him to cough more than he should be and from time to time causing his oxygen saturations to drop, so we need to treat it. Wehave a theory (we = us + his doc) that because of Drew's airway abnormalities that he might not be getting as much of the inhaled antibiotics as we think he is, as there is a degree of airway reactivity and inflammation cause by inhaled antibiotics and he already has that stuff going on in the absence of an antibiotic. So we're going to take a shot at this bacteria with 90 days on inhaled antibiotics, alternating between two different kinds every 15 days. Its going to be a lot of extra work, as these inhaled antibiotics typically add about 30 min to each treatment (so an extra hour + a day), but it's a small price to pay for healthy lungs. We need to get this thing under control before it sets up shop in his airways and starts to cause real damage. Hopefully it works, but in the meantime I will continue searching for alternative means to eradicate this beast.

Friday, June 7, 2013

We are equals...and a Drew update :)

About two years ago, I became the State Advocacy Chair for the Ohio chapter of the Cystic Fibrosis I remember the first meeting that I had. It was at Jean Schmidt's office and Drew's doctor came with me. We nervously sat in the lobby discussing out lack of political knowledge and our anxiety over how we would answer certain questions had she asked them. We ended up meeting with Rep. Schmidt's chief of staff, who coincidentally, had a friend whose baby was recently diagnosed with Cystic Fibrosis. The conversation just flowed. That meeting earned us another meeting with Rep. Schmidt  herself which went just as well, if not better, than our first meeting. That year I went on to meet with representatives from Rob Portman's office, John Boehner's office, and Mitch McConnell's office. Not a ton of time was spent prepping for these meetings because I wasn't learning something new that I was sharing with them, but rather sharing my story, the story of Drew and how we fight for him by raising money and awareness of this disease. 

When I look back on those early meetings, I remember feeling almost intimidated by the power that some of these folks held...until I met them. Then I saw us as equals. Elected officials are put into place to represent me. They need to hear about what I believe in and how their decisions effect me. Similarly with healthcare, the doctor can't treat me without knowing anything about it. They need to know how I'm feeling, my history, my symptoms, my priorities. To do their jobs well, I need to be a part of their team. The walls must come down we must be partners, working together for the betterment of all. 

I'm going to a healthcare conference next week called Creating and Sustaining Collaborative Networks in Pediatrics and I've been asked to share some of my thoughts and experiences with such networks as well as areas for improvement. I've learned a little bit about the audience and what some of the other parent speakers are hoping to share, and I'm still struggling a bit with what my message will be. I think the essence of these networks is making patients and families equal players, and for that reason I want to talk about the value of letting patients help. I think a lot of these people get that already though. So what else can I tell them? What can they hear from me that will be eye opening and leave them thinking about me and thinking about taking action, including patients. I've got some thinking to do over the next few days.

I haven't talked much on here about Drew lately and, with it being a blog on him and all, I thought I'd give some updates. Drew, who is 3 now, has had an on again off again cough since about January but wasn't culturing anything. We treated a few times with oral antibiotics and he would seem to improve and then the cough would come back. In early March, he cultured achromobacter. We treated with two weeks of IVs (tobramycin and meropenum) and his cough seemed to improve but unfortunately after completion he was still culturing it. We decided to try 30 days on inhaled Colistin next. For the first 15 days on Colistin, his cough had decreased but then it slowly crept back. He finished Colistin last Wednesday and by that point the cough had come back significantly and he was in clinic for a sick visit the day after completion. We did a culture and put him on Omnicef and there was an immediate improvement, taking his cough back to almost non-existant for 4-5 days, but once again, it has come back. 

I met with his doctor yesterday and we are assuming that he is colonized with achromobacter. It has been hard to get a true culture since he had just gone off the Colistin the day before he was in for a sick visit, and now he is on an antibiotic again. We're going to do a hi-res CT as he's had one of these before and could provide some insight into what kind of damage, if any, is occurring. 

What I'm struggling with is that even if he is colonized with achromobacter, it is not a good situation when he has to cycle inhaled and oral antibiotics constantly, not even 30 on/30 off,  just to get to his baseline. I'm not ready to accept that this increased, junky cough and frequent de-sating is his new baseline. I believe that he will benefit in some capacity from the Kalydeco + 770 combo, but I need to keep him well until that is available. And he looks perfectly fine. To see him, you wouldn't have a clue that anything was even wrong. 

So our next step is to have a high-res CT done to see if this cough is just a cough or if its mucus really causing some lung damage, bronchiectesis. I was hoping to have that done before we head east for a couple of weeks but I don't know if it's actually going to happen or not. I will keep the blog up to date with everything that's going on!

Sunday, June 2, 2013

Rocky week

We had a bit of a rocky week last week but we seem to be back up and running once again (knock on wood).

Drew finished his last dose of Colistin that he had been on for culturing Achromobacter last Wednesday night. There had always been some concern that he would have a reaction to the Colistin because inhaled antibiotics seems to irritate his tracheomalacia and cause bronchospasms (re: his airway collapses when he takes it), but that didn't seem to be the case this time and with this medication and we were relieved. But about a week ago, he started having an increased cough and a decreased appetite and I could just tell that something wasn't right.

I had emailed his docs to let them know that he was coughing more than normal, and just stayed in close touch with them over the holiday weekend in case something were to change. He never got bad, just coughing a lot and tiring easily, so we were doing extra airway clearance and trying to keep him extra hydrated. Last Monday or Tuesday I thought we were getting better and exchanged a few emails with his doc again letting her know that things definitely weren't getting worse and I thought seemed to be getting a little bit better. Then on Thursday morning his oxygen saturations we in the low 90's and he was tired and coughing up a storm. I took him in for a sick visit and they did a chest x-ray. The x-ray looked fine but they heard some crackles in his lower left lobe and decided to try an oral antibiotic until we got the culture report back. If his oxygen went below 90 we agreed that he would need to be admitted and put on some oxygen until we figure out what's going on.

After just 1 day on the oral antibiotic, things improved greatly. His cough had almost gone away and his oxygen was on its way back to normal. We don't have the culture back yet, but I really really really hope that what he had going on was just a virus that was going around that caused some extra mucus and bacteria to flair up in. I guess we will see soon! I'm just hoping that this week goes smoother than last.