Monday, January 27, 2014

"Not everything that is faced can be changed, but nothing can be changed until it is faced."

Tomorrow is our last day on IV's. Can I get a HELL YEAH!?! Our original 14-day plan didn't quite cut it, and with little improvement and a hospital stay under our belt we decided to forge ahead with another 14-days. I am happy to report that he is well. I will also hypothesize that by the end of the week he will no longer be well as the rest of this house is working on a nasty cold virus. We're washing hands, covering mouths, drinking our fluids and hoping for the best.
This month has been a blur. Between sick days, snow days, and school holidays, we've had our hands full. I've been doing a lot of work again on the C3N for CF. I've connected with a number of patients and families from other disease networks to see how we can work together to facilitate cross-disease learning; no need to recreate the wheel! I will soon need the help of a few CF patients or caregivers to participate in testing out a new learning network platform (think Facebook-esque but WAY better) that holds the opportunity  to connect patients and clinicians and share best practices and learn from one another. I know that these conversations are already happening on places like Facebook and Cystic Life, and I think the CFF is aware that they are happening, but I don't think they have any idea of how often or to what extent. While many of the conversations are useful, I think that we could make them more valuable by organizing them and using them to guide future discussion, or to teach others, patients or clinicians, about what's important to patients, and get feedback that includes recommendations from both physicians and even the CFF. Anyway, I have more info to share, but we're not totally ready to go 100% public with it yet, so if you're interested in learning more and possibly joining this little test group, shoot me an email - erin (dot) moore (at) cchmc (dot) org. You won't be disappointed!
I was invited by the Agency for Healthcare Research and Quality (AHRQ) and the Center for Education on Research and Therapeutics (CERT) to "share my unique perspective" at an upcoming conference that they are having, focused on Medication Management. I don't totally know what that means, and to be perfectly honest, I don't think they do either. If their goal is to improve medication adherence, they've got the wrong goal. If their goal is to use new and collaborative strategies to improve outcome for patients with chronic conditions, and finding ways to better medication management is identified as a part of that, then we're moving in the right direction. I feel like so many people are missing what their looking for on their road to improving "compliance" or "adherence" or whatever you want to call it. If you can find ways to meaningfully and appropriately engage patients in the creation and use of solutions that work for them, adherence will follow. As humans, we are so much more likely to continue doing something that we want to do, versus something that we have to do or are told to do. If all of these wonderful clinicians continue to put together ideas and papers on how patients can manage medication, at the end of the day they will have a stack of papers on how to manage medication and no one will be getting any healthier. Giving me more information to educate me on something indicates both that you don't believe that I know what I'm doing or don't understand and that by saying it another way will make me more likely to comply with your wishes. False. It is likely to annoy me and perhaps make me even less "compliant". If you ask me what I think, or how I would do it, or simply listen to me to gain a better understanding of what my barriers to adherence are, you will learn so much more. And the opportunity for us to work together on a solution that works for me will open doors for other patients to see that they too can participate in the design of a plan that works for them. It's quite simple really. A perspective to share I certainly do have, but I assure you that it is not unique. Ask most patients or caregivers living with a chronic condition and I think you will find a lot of similar thinking. I can't wait for the opportunity to share this perspective with ears who are looking for answers and willing to listen.
I need to put together a slide or two to tell people about myself and share my story. I want it to have an impact. I want them to really hear what I have to say. And I'm giving some thought into the best way to do that. I'll definitely be sharing it on here as well and welcome any thoughts on the best way to engage this influential audience of physicians, insurers, pharma reps, policy makers, and other thought leaders in the wonderful world of healthcare.
I just registered for this years March on the Hill, the CFF's advocacy campaign on Capitol Hill where State Advocacy Chairs meet with members of Congress on Capitol Hill to share the policy agenda of the Foundation. Last year's campaign happened during the "great snow-quester of '13", otherwise known as a warm and sunny afternoon in DC, so we were unable to meet with actual senators and congresspeople, but instead their staffers. Hopefully this year we will get the opportunity to share our stories directly with Ohio's decision makers! Following the March on the Hill is the CFF's annual Volunteer Leadership Conference. Talk about an amazing weekend, this event brings together the best of the best engaged and energetic advocates and fundraisers to share stories and encourage one another to continue to fight. Last year was our first time going, and I cannot wait to go back. 
We are also about to start our 2014 fundraising campaign for Great Strides. I need to put together a new video, but I'll leave you with last years. This has indeed been a long year for us health wise, but we will continue to hold onto hope.

Friday, January 17, 2014

An update from the homefront

We're home! We got discharged from the hospital Wednesday evening and made in home in time for evening breathing treatments and the chaos that typically ensues with bedtime. Everyone has been out of sorts for the past few days - not feeling well, not sleeping well, not knowing what's next - so it will be nice to rediscover our groove. All of the tests that they ran on Drew came back negative. Their first suspicion was that he had a bloodstream infection from his PICC. When he wasn't showing signs of sepsis, they started to test for the flu. When we went into the ER he only had a fever and had turned a pale-ish blue color, despite his oxygen saturation being at a totally reasonable level. He had no signs or symptoms of the flu, though I learned that sometimes symptoms follow. Regardless, the flu test came back negative as well. We spent most of our time in the hospital just waiting for results while hooked up to meds. Everyone suspected that he had some sort of viral infection, but when the viral panel that they ran came back negative and he got better, they decided that it would be okay to send him home. It probably was a virus that caused the fever, but not a typical one that shows up on the standard viral panel. Having a virus can cause the body to produce more mucus and inflammation, and in CF that can stir up bacteria and cause trouble. That is the best guess of what happened.

We are home now on two more weeks of IV med. Prior to this admission, we had spent the past two weeks on one IV antibiotic + an oral antibiotic + an inhaled antibiotic. That combo didn't seem to have any impact on his health, leaving him with the same lingering cough that we had been working so hard to suppress. This time we are trying two - IV ceftazadime and IV cipro. We were so ready to be done with IVs because they must be run every 8 hours around the clock for 2 weeks, but it's what he needs so we will do it.

A big thank you to everyone who brought us coffee, sent us dinner, drove our kids to or from school. We don't always know how to ask for help, but every kind gesture is greatly appreciated!

Tuesday, January 14, 2014

10pm Update

When I ranted and raved this afternoon about my frustration with Drew still needing 2 weeks on IVs, I had no idea what our evening held. Drew got home from school and was playing and having fun, acting just like he always does. Around dinner time he wasn't looking like himself - a little pale, tired, not at all interested in eating dinner. He felt warm to me so I took his temp and it was 102.1. When you have a PICC line and you get a fever you go to the ER as there could be a bloodstream infection.
So to the ER we headed where they began to treat him for sepsis. They also drew a lot of blood to run labs to see if there was an infection in his bloodstream or if he has a virus or what is going on. The tests thus far seem to indicate that he doesn't have sepsis or any blood stream infection though we will have final results tomorrow. They also did a viral panel to determine if he has the flu and have begun treating him with Tamiflu. We will know tomorrow if he has the flu and if we will continue that or figure out what is going on. He is sleeping now, finally, though they will wake him at some point soon for a dose of Tamiflu. The poor kid needs some sleep so that sucks. I will try to update the Doin' It For Drew Facebook page as I get any new info. We appreciate all the thoughts and prayers and hope to be out of here and well again soon!

- Posted using BlogPress from my iPhone

Monday, January 13, 2014

Deep breath. Exhale. Carry on.

We are 13 days into our current treatment plan which is IV, oral and inhaled antibiotics around the clock. Sadly, there has been not improvement in symptoms. He's not "sick" like most people would think of sick. He's coughing a little more than he normally does, but probably not anything anyone but me would notice. In the past, two days on an inhaled or IV antibiotic seem to make a difference. I thought he might need some extra time since a bronchoscopy seems to cause some irritation and stirs up all the mucus and bacteria just a little. I talked to his doctors last week and we all decided to ride it out. Today we decided to change course. He should be getting better and he's not.
I feel like CF is a crap shoot. We do these epiglottic cultures, like a strep test, where he coughs onto a swab and we catch a little mucus and we put that into a dish for a week to see what kind of bacteria grows. Older kids can cough up a sample but we aren't there quite yet. If he has symptoms - increased cough, decreased appetite, dropping oxygen saturation, lethargy - we treat him with an antibiotic that his current bacteria is susceptible to. However, just because a bacteria responds or doesn't respond to an antibiotic in a petri dish doesn't always mean it will react the same way inside of the human body. The bacteria that we are fighting is called achromobacter and it is almost completely antibiotic resistant. It's sort of like having MRSA in your lungs, only worse (and not the same clinically, for any doctors reading this, I know the differences, the comparison is for the non-medical folks, two antibiotic resistant bacteria, that's all). His culture reports tell us that it is susceptible to a drug called Colistin. We can't (or haven't yet) use that on Drew because it will wreak havoc on his other organs and he's not really big enough to tell us neurologically if he's experiencing symptoms. We've tried to use the inhaled version, which you can think of as topical rather than systemic because it goes right to his lungs to kill the bacteria when he inhales is, vs an IV sending the drugs into his bloodstream to disseminate. Inhaled doesn't improve his symptoms. There has only been one inhaled drug that improves his symptoms and it's an antibiotic called Ceftazadime (it's the one that smells like cat pee - I know, gross). He currently cycles 14 days on/off with inhaled Ceftaz and we see 100% improvement where he is back to his baseline, healthy and well. The problem is that when he's on an off cycle, on no medication, he's getting sick. So we're going to see if giving him Ceftazadime intravenously helps to suppress the bacteria further than the inhaled antibiotics seem to. Fingers are crossed. They always throw in an extra IV antibiotic for good measure so that the bacteria don't go getting all clever and become resistant. We will start those on Wednesday.
Of course we can't just order two new medications and continue on the path we've been traveling, that would be far too easy. The cap on his PICC line is stuck on there and it was supposed to be changed last week. They usually change the cap every 4 days, and whey they'd discovered that it was stuck he had just been changed 3 days prior and only had a week left to go before we all thought that the PICC was being pulled. Well now that we're going to keep that PICC line in for two more weeks the cap will need to come off and if they can't get it off we will need to change the PICC. Joy! They also always administer the first dose of any IV antibiotic in the hospital where he could get immediate medical care if there were any sort of reaction. I appreciate this. My schedule does not. The hospital is formulating a plan as I type this so we will see what happens.
I have a good feeling that this will eliminate his symptoms while he is taking the medicine. I'm anxious to see if his symptoms will stay gone after the medication is finished. I am also anxious to see if I can really survive for 4 weeks of staying up until 1am to administer medication.
I have another post brewing about how much better this could all be going if only things were better organized and documented. If I could reference something that told me how other people managed their symptoms when they had achromobacter, or how their kids reacted to certain antibiotics or antibiotic combinations. Right now, today, I can get the most valuable information to answer those questions on Facebook. That's right, Facebook (don't knock it till you've tried it!). There's a better way folks and I'm working on it. I just need it here now. Deep breath. Exhale. Carry on.

Saturday, January 4, 2014

8 hours

It's midnight on January 3rd and I'm awake waiting for an IV infusion of the antibiotic Zosyn to finish. Drew went into the hospital on New Years Eve for a bronchoscopy and to get a PICC line so that he could get IV medication; a "tune-up" if you will. We decided that we weren't okay with our current regimen, cycling inhaled ceftazadime 14 days on/14 days off, since he could hardly make it from the end of an on cycle to the beginning of the next without needing some kind of medical intervention. He's got a viscous bug deep in his lungs called achromobacter that is resistant to [most] antibiotics, and its wreaking havoc. Havoc must not know me and my relentless pursuit to save my sons life.
Being in the hospital for anything is unpleasant. Surgery is the worst. I'm certain that there are varying degrees of this agony that is handing your baby off to total strangers and literally placing his life in their hands. Whether it be for a life-saving surgery or a 15-minute "quick look" at his airways, it sucks for me just the same. I try to trust the people that I hand him off to, but I don't know them and they don't love him like I do. He cried this time. In the past he's been so little, so oblivious. This time he was too, until we walked into an OR full of doctors and nurses in gowns and gloves and masks. He cried and clung onto me, silently begging me not to leave him. They tried to ease his anxiety by drawing a silly face on the balloon that fills with "sleepy air" and challenging him to blow it over with each exhalation. He zoned out, went limp, and then we were escorted out. The very kind nurse suggested that we grab a bite to eat while we waited. The procedure was scheduled for 92 minutes, though having been through this more times that I can count I knew that it wouldn't be more than an hour.
We checked into the Same Day Surgery waiting room, an unpleasant room that is smells like a combination of bad coffee and tired people, the air almost visibly thick with anxiety. We gave our cell phone numbers because we were going to be leaving the waiting room to meet the Homecare nurses to be trained, for the 4th or 5th time, on the administration of IV antibiotics from home. I cried on our walk from the third floor to the first. I hate to leave him, especially knowing that he was so scared. I was less worried about the approx. 92 minute procedure than I was about the results that we have yet to learn.
We met the homecare nurse on the first floor and told her that we've been through this all before. I've never met a more efficiently run organization than homecare. They make tough situations run smoother than you can imagine. They show up when they say they will. They always, always, send you exactly what you need and often times even extras or "just-in-case" items. They call to remind you and they haven't yet messed up (for us anyway!). They make my life easier, and for that I love them. We needed to show the nurse our competence in caring for our son at home while he has a central line to his heart through which we administer a strong intravenous antibiotic. I've probably done it 100 times, but I simply could not focus on the task at hand. My baby was just put to sleep, and I didn't know what the outcome would be. I know that I need to wash my hands and put on gloves. I know the steps: clean the tray with 2 alcohol wipes, get out two saline syringes & 1 heparin, 1 tubing, one alcohol wipe, one swab cap. I know to check the label and prep the tube. I know to flush his line before I hook him up, and to always turn it on with the alarm in case there is an occlusion. I promise you, I have never been more diligent about a procedure in my life than one where I could literally take his life if I do it wrong. Yet in my worried, anxious state, I stumbled through it, making mistakes all along the way. She understood.
After only 30 minutes, my cell phone rang. It was the Same Day Surgery receptionist letting us know that the procedure was done and that the doctor was ready to talk to us. The results were good. His lungs looked better than they have almost ever before. He has severe tracheomalacia and bronchial malacia and for the first time since he had his very first bronch, very very early in life, there were signs of improvement. There was no mucus in his nasal cavity. His trachea was open, though there was still a near complete collapse when he would cough. There were areas of mucus, typical in a CF patient, that were washed and extracted so that they could be cultured. Overall his airways looked great. The opportunity to look at the physical shape of his lungs was a secondary benefit though. What we were doing the procedure for was to collect mucus for a culture, to see what kind of bacteria is living in his airways and causing him so much trouble. Would it still be just the achromobacter? I sort of hope so. Would there be something else, something that if treated differently would not be causing him so many problems? Would there be something untreatable? Dear God I hope not. But we don't know. We don't get that information for a week.
So we are home now, on an IV, an oral and an inhaled antibiotic combination therapy. It was recommended by an infectious disease doctor who has had some experience dealing with achromobacter. It is a long shot that we would ever be able to get rid of this bacteria, but so help me God I will try. It is exhausting running IV meds from home. They need to be done very specifically 8 hours apart so as to not hurt his kidneys. We also need to give him his oral antibiotic every 8 hours, so we wake him up every so slightly right as we finish up the midnight IV to get him to take it. The inhaled is just twice a day, so we do that with our morning and evening breathing treatments. Our cabinets are filled to capacity with medication, and he's not even "really sick" yet. I hope he's never "really sick". I hope that his cure is coming soon. Until then, we will continue our fight.
It's been 35 minutes. It's time to unhook the IV and go to bed. We will do this all over again in just 8 hours.