Tuesday, May 28, 2013

Personal Experiments

We're wrapping up 30 days on inhaled Colisitin and I think its fair to say that everyone in this house is anxious to regain that hour of our day. I don't know if it's working or not. Drew has seemed to be doing alright the past couple of weeks, but just over the past couple of days seems to have something going on again - kind of tired, coughing more than normal, less interest in food. I've been emailing his doc and we're just keeping an eye on things for a couple of days. I hope its just a virus and he gets over it.

We've been talking about the plan for if the Achromobacter isn't eradicated and it seems like there's a decent chance that that will be the case. Right now, our plan is to cycle Colistin like many people cycle Tobi for pseudomonas. We will do 30 days on and 30 days off indefinitely if he grows Achromobacter during his next culture to keep the bacteria from causing problems. It's so hard to eradicate some of these pesky bacteria. We just have to do what we have to do to keep the bacteria from acting up and causing breathing difficulty and lung damage. I really hope it's gone but I'm okay if its not. We'll deal with what we have to deal with.

We had two great fundraisers the past couple of weekends. The first was a Doin' It For Drew BBQ and the second was our 3rd Annual Garage Sale for a Cure. We raised about $700 at our BBQ and almost $1100 at our yard sale. Let me just tell you that the haggling that goes on with the supremely cheapest of cheap people at a yard sale means that that $1100 was hard earned.

I'm still working hard at CCHMC on bringing a C3N to the CF Community and it's all about to happen. I've started working with a PhD student from MIT who created something called Personal Experiments where I can use mobile technology to track a whole slue of things - weight, appetite, stool, medications, you get the idea. You can use that data to create personal experiments. So for example, one thing I've always thought is that Drew's appetite decreases in the days leading up to him getting sick. I know that there's some correlation, but now that i'm actually tracking his cough frequency and his oxygen saturation and his appetite, I have actual data to show me if there is a relationship there. I've only been at this for a couple of weeks so I don't have enough data yet to make any real observations, but it's a start. Hopefully the CFF and Cincinnati Children's will be reaching an agreement here any day now to get some feet on the ground running with this project. I'm already running so they're all going to have to catch up!

We will re-culture Drew in about 2 weeks so cross your fingers and say your prayers that we get results that we like!

Sunday, May 19, 2013

Are Patients a Distraction? I think not...

There was a healthcare conference last fall that I desperately wanted to go to. The conference was for healthcare professionals - doctors and researchers and other clinicians - to hear the latest and greatest about what's going on around this particular disease, my sons disease. I had to email half a dozen people to find the right one who could tell me what I had to do to gain access to the conference so that I could register and attend. I had a special form that had to be filled out by the director of the clinic that my son attends and then it had to be sent back to the organization hosting the conference and then they had to provide me with login information so that I could register. I saved money for a year. I drove my whole family, including 4 children ages 4 and under, 1500 miles so that I could go to this conference. I finally got the green light to go and I was pumped.

The conference was targeted toward healthcare professionals and much of the information presented was above our heads. I'd just relearned the Punnet Square  2 years prior and they were talking DNA sequencing or the particle sizes of different nebulized medications. Surprisingly though, I understood a lot more than I had anticipated. I was in awe for most of the three days that I was there, reviewing posters on everything from infection control to quality improvement and everything in between. My eyes were opened and I finally had a deeper understanding of why many things are done the way that they are. I asked for clarification when I didn't understand something. I had paid the fee to be there and had just as much a right to ask the questions that I had as anyone else there. By the end of the conference, I had a book of notes and questions, things I couldn't wait to talk to our doctor about, a stack of business cards from admirable folks who I had had the opportunity to cross paths with, and a catalog that included every talk and  poster and presentation on the disease that my son has. The book, to this day, sits on the end table in my family room and I reference it very regularly.

Toward the end of the three days, I found a member of the leadership team that was putting on the conference and I asked him why parents and patients weren't invited to attend. He responded, without hesitation, that parents and patients at a conference like this are a distraction. He was serious.

I think patients and parents are the focus, or should be if they're not already. We are focused more than any scientist on finding a cure. We are better investigators of options and alternatives than any doctor ever could be. We create focus when we need another treatment option or need a referral or need to find a better way to perform a process or save time or money. We keep notes and make phone calls and insist on perfection. We are the energy behind QI projects, and often times even the ideas for them. We bring such a unique perspective to the healthcare team, yet most of the time we're not a part of it. We are on the receiving end of care, and to make the healthcare system better we cannot be viewed as a distraction but rather as the focus. Listen to us. Make your decisions around what we want. Ask us what we want. Make us a equal part of your team.

I've been fortunate enough to stumble upon a group at Cincinnati Children's whose focus is just what it should be - patients. People still think that I speak up because I like the attention or because I'm trying to prove a point or get my way. All of those things are true, but the real reason that I do it is to save my sons life. If I don't fight for him, who will? There are ways to make the healthcare system better, and I speak up about them. I interject my ideas and provide feedback when I think something may or may not go over so well with others. It's so interesting to see how different perspectives can be when I'm meeting with clinicians. Most of them do truly have the patients best interest at heart. But more times than I can count, ideas and priorities do not align. We both want to get to the same end point, the change for the better, but the way that we might go about it could be so wildly different. Instead of working in two paths that separate and then come back together, let's walk together along the same path and learn from one another. We can save time and money while improving the care if we could just work together.

I'm not just a "patient". I am a mother, a wife, a daughter, a sister, a niece, an aunt, a neighbor, 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 everything in between. I am a volunteer, a blogger, an advocate. I am a chronic pain sufferer, a Starbucks lover, and allergic to many animals. My point is that I have life experience and so does every other person who has ever been a patient. 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 to find ways to keep their children healthy and alive, what we have to say is what should create the focus.

When I was told that patients are a distraction, I was still relatively new in the world of the empowered patient. Today, if I crossed paths with a highly respected healthcare provider who shared the sentiment that patients are a distraction, they should expect an earful and leave our conversation realizing how much they wish they had me on their team fighting for their cause, because I am anything but distracting when it comes to fighting for my sons life.

Monday, May 6, 2013

Just a little while longer

Everyone wants to know how long my son will live. That's what they mean when they say, "So what does this mean?". I want to know too. But I don't and I never will.

It's hard when you're first diagnosed with a "progressive and fatal genetic disease". You worry and wonder and savor every moment not knowing which one will be your last. And this beast is like a light switch; one minute everything is going fine and then out of the blue the switch flips and everything is not fine. In fact, nothing is fine. We're scheduled to go to the park for a t-ball game and instead we're at Children's Hospital getting a PICC line placed to receive IV antibiotics. Or we're running all over town to find a GI Specialist who can see him on short notice. His weight is up and his weight is down. He's never had an appetite. The cough that they treated as a pulmonary exacerbation for the first six months of his life turned out to be tracheomalacia, which was good in the sense that it wasn't a horrible infection causing the issues but rather a floppy windpipe. But it was not good in that he can't afford to have a floppy windpipe. He needs a strong windpipe to be able to cough out all of the infection, not one that collapses when he coughs trapping the bad things inside. He continues to acquire new and different dangerous bacteria. Some of it is treatable, other isn't.

Let him just be a kid. Make sure you wash his hands good after he's outside playing. Be careful in soil or sitting water. Some pets carry bacteria that can be harmful so just be cautious. He needs a high calorie diet. He needs another test. He needs this medication at 11pm. No hot tubs. Try to stay away from anyone who might be sick. Don't be around other people with CF. We need to see you every 3 months when he's healthy. Keep him hydrated. Don't give him apple juice because the sugar is causing a problem with his stool. Take these pills. Add this treatment. Drink this half n half.  Add more airway clearance. We're so close to a cure. He needs to be admitted.

I understand the importance of doing everything I listed above but it is a lot and it is constant. The physical demands are a lot, for him and for us. The mental and emotional are more. First and foremost, I am his mother. I want him to run and jump and play and get dirty and watch movies and eat snacks and sleep well. I just want him to be, just like the other kids. He gets treats and he gets punished just like everyone else. I don't want him to have CF. It's not fair that he has to sit for hours every day taking medications and doing treatments. He doesn't want to do it and I don't want him to have to do it. So what can I do.

I have this feeling in my gut that the place that we go to keep him well is the place that is making him sick. I don't have proof of this, other than the research that came out showing that CF patients are sometimes passing these dangerous pathogens to and from one another in the clinic and the hospital. That was enough to both scare me and validate my fear. Maybe its his vulnerable trachea that's trapping things in even when we're doing our best to prevent that from happening. Maybe its just his CF. Maybe he was just going to pick up these bugs regardless of what we did or how his windpipe looked. Its impossible to know all of that, but it has created a worry and a fear in me that I've gotta find a way to deal with.

Drew was supposed to go to clinic this morning. Yesterday afternoon I cancelled. Again, I am 100% clear on the need for his doctor and other clinicians to see him on a regular basis. They need to monitor his health closely, to see what he looks like when he's good so that they can appropriately treat him and understand the what's going on when he's not good. I get that. But I am fearing that the bacteria that he has now and that he has grown in the past has come from the clinic, and I am scared to death to put him at risk of acquiring something new. The doctors there get it. They don't know the extent to which this passing of bacteria is happening, but they acknowledge that its happening. They gown and glove and mask in between patients. They're continually working on ways to improve outcomes and avoid any direct or indirect patient to patient contact. But while they work on that and try new things, Drew continues to acquire new pathogens and his lungs, over the long term, can't handle that. He's not necessarily getting more than other kids or worse ones than other kids, he's just getting things and I'm starting to believe that putting him at any risk isn't worth the benefits of seeing his team. But I'm torn because I know that better outcomes are achieved by regular visits with the clinical team. A better outcome for him will be achieved by keeping his lungs free and clear of any and all bacteria, and how we do that is where I'm stuck.

While we wait for our cure, a cure that is so close I can almost taste it, I need to keep him healthy. I cannot afford lung function decline in a kid who hasn't even achieved peak lung function yet. I don't think that my concerns are irrational as they've been validated by research data. Perhaps not to the extent that I'm going with him having acquired everything from the clinic, but it's definitely happening. I try my best to let him be a kid in the midst of all that he has to do all day everyday. He doesn't know any other way. His instincts tell him that it shouldn't be this way, but his mama insists upon it as a part of our daily routine and so he does it. I will take him back to the clinic, and soon. I just needed a little more time to sort out all of these fears and find a way to keep Drew safe and healthy, just for a little while longer.

Sunday, May 5, 2013

Off of IV's and onto inhaled

The IV antibiotics that Drew was on for the past two weeks did not get rid of the bacteria that he is growing in his lungs. We knew there was only a chance that it would work, as the bacteria is antibiotic resistant to just about everything, but we had to try anyway. We found out last weekend that it didn't work and decided to move on to an inhaled antibiotic that is more or less the only thing that this bacteria isn't resistant to. You may be wondering why we didn't start there, and the answer is because of the reaction that Drew has had to inhaled antiboitics in the past. Because of his tracheomalacia, his already floppy airway is just made floppier with an inhaled antibiotic and it seems to cause a degree of collapse in his airways. Luckily (knock on wood), he seems to be tolerating this drug just fine. I'm not sure if its just the time that has passed that has allowed his airways time to grow and strengthen or the fact that its a different antibiotic, but regardless, he's tolerating it so we'll take it.

I don't know if he will ever get rid of achromobacter. Even with these inhaled antibiotics going right to the source, he may simply just not ever be able to get rid of it completely. Our CF docs (and I think most CF docs) treat based on both what people are growing and their symptoms. Sometimes, despite their best efforts, stubborn and clever bacteria find places to hide in the lower airways and just never go away. They might not cause any problems most of the time, but every now and then they will start to grow and thrive and wreak havoc on the lungs causing a pulmonary exacerbation and needing attention and treatment to get it back at bay.

And the big question on everyones mind is "What does this mean?. I don't know really. It's not going to kill him tomorrow, or even later this year. It's unlikely that it will kill him in the next 5 years. And frankly he may live with this bacteria being a pain in my ass here and there for the next 60 years. I just don't know, and neither does anyone else. Here's what we know:
-Achromobacter is a multi-drug resistant bacteria
-It is known to cause inflammation
-It may be a contributor to lung function decline in people with CF, but not enough people have it for researchers to be able to reliably report its short and long term effect.

So there you have it. I don't know what it means if we don't eradicate it. I would love to have it not show up on a culture ever again, or at least for a while, but I don't know how likely that is. For now, we will continue the inhaled antibiotic and see where that takes us. Thank you for the continued thoughts and prayers. We'll keep you posted!