Saturday, August 6, 2016

Research is Hope

“Hope” is the thing with feathers - 
That perches in the soul - 
And sings the tune without the words - 
And never stops - at all"

I am vulnerable. I have a child who is sick, like really sick.  I go to the doctor with the desperate hope that someone - anyone - will be able to help me to keep him well, to save him. And in that vulnerability, I am asked to do things I often don't fully understand. 

I had to give my baby to strangers when he was two days old, signing papers to waive liability if something happened to him when he was under the care of someone I had never even met.  This is the story of most chronic illness caregivers.  As time goes on, our comfort level grows, relationships build with our doctors and care teams.  But with chronic illness, or CF, anyway, in our case, there is always something new, always something scary, always something we don't understand. Then researchers come in - knocking on the door after a long 3 hr appointment, or while we are in the surgery holding area waiting to go back and wondering how the results of whatever we're having done will forever change our reality, yet holding it together for our kid who is equally scared - trying to explain what the researcher whose interested in a sample of our tissue or some of our thoughts (neither of which we're giving with a clear mind) is trying to do, what they're hoping to learn. But in that instant it sounds promising so we go with it, signing their paper since the risks seem low, collecting our gift card for participation, or just their thanks, in many cases, and the promise of learning or better connection with our care team, whatever it is that their trying to do. Hope is really what their offering us, and we sign up. 

Sometimes there's further interaction, but many times not. Then months later, long after we've signed their papers, something hits us and we remember that time we signed up for that thing, and a bit of us wonders what ever came of it. And, if we search just a little bit, we find out that the study didn't recruit, or that it didn't meet the appropriate end points, or that the institution supporting it didn't see the value in it and it's done. Those researchers move on to their next theory to test and our contribution has been tossed aside. Or maybe it went on and that researcher learned many great things that they wrote about and got credit for and published their findings in some fancy journal that we don't have access to because, after all, we're just the patient. Forget about what the patient thought, what they learned, that brief glimmer of hope. 

It feels abusive, really, to approach the sick and offer them hope - to take something from them, physical or otherwise, something that's so sacred - and then to just end it because it didn't meet your goals. When do my goals matter?

Why are we allowed to just throw that all away? Why is that ok? Shouldn't there be, if nothing else, a moral or ethical obligation to say, "I'm sorry, I tried."  Shouldn't we be giving patients the opportunity to take what's been learned from the work they contributed to - the work that couldn't have been done without them - and start from a new baseline of learning? Why aren't we sharing what we are learning in research, successful or failed, in a way that allows folks to build upon it? This is among the greatest failures of modern medicine. 

I want to ask researchers what they're actually trying to do. If the answer isn't that you are trying to, wholeheartedly, help patients, then please stop wasting our time. 

A research project I had been a part of recently shut down and I'm so...I guess sad is the right word. I wasn't a participant, but rather a member of the study team so I can't help but feel a bit of responsibility. My frustrations are for those families who joined this project, sharing with us their precious time, the intimate details of their health and lives. People who were given hope only to be shown another dead end, and for no good reason. My participation and contributions felt uncomfortable at times. It was frustrating to have my ideas and suggestions for a more patient-centric approach limited by both institutional research regulations, and the discomfort or fear or I'm not exactly sure what of trying to do something different than it had ever been done previously. I should have pushed harder. 

Helping patients means doing things differently than they're being done right now.  I do believe researchers are  trying to learn, to help us. But to be honest, it's moving at a devastatingly slow pace and it's too often letting us down. 

Give me back what you've taken from me and encourage me to share it, to build upon it, to figure out what else we can learn together. My participation in research and the view I have of the participation of my peers has left me feeling exposed and uncomfortable, not wanting to say yes to future engagement. This is a problem that we need to fix! This is how we will really start to learn and grow and change and improve. Stop picking on the weaker man. 

Research is Hope

“Hope” is the thing with feathers - 
That perches in the soul - 
And sings the tune without the words - 

And never stops - at all"

I am vulnerable. I have a child who is sick, like really sick.  I go to the doctor with the desperate hope that someone - anyone - will be able to help me to keep him well, to save him. And in that vulnerability, I am asked to do things I often don't fully understand. 

I had to give my baby to strangers when he was two days old, signing papers to waive liability if something happened to him when he was under the care of someone I had never even met.  This is the story of most chronic illness caregivers.  As time goes on, our comfort level grows, relationships build with our doctors and care teams.  But with chronic illness, or CF, anyway, in our case, there is always something new, always something scary, always something we don't understand. Then researchers come in - knocking on the door after a long 3 hr appointment, or while we are in the surgery holding area waiting to go back and wondering how the results of whatever we're having done will forever change our reality, yet holding it together for our kid who is equally scared - trying to explain what the researcher whose interested in a sample of our tissue or some of our thoughts (neither of which we're giving with a clear mind) is trying to do, what they're hoping to learn. But in that instant it sounds promising so we go with it, signing their paper since the risks seem low, collecting our gift card for participation, or just their thanks, in many cases, and the promise of learning or better connection with our care team, whatever it is that their trying to do. Hope is really what their offering us, and we sign up. 

Sometimes there's further interaction, but many times not. Then months later, long after we've signed their papers, something hits us and we remember that time we signed up for that thing, and a bit of us wonders what ever came of it. And, if we search just a little bit, we find out that the study didn't recruit, or that it didn't meet the appropriate end points, or that the institution supporting it didn't see the value in it and it's done. Those researchers move on to their next theory to test and our contribution has been tossed aside. Or maybe it went on and that researcher learned many great things that they wrote about and got credit for and published their findings in some fancy journal that we don't have access to because, after all, we're just the patient. Forget about what the patient thought, what they learned, that brief glimmer of hope. 

It feels abusive, really, to approach the sick and offer them hope - to take something from them, physical or otherwise, something that's so sacred - and then to just end it because it didn't meet your goals. When do my goals matter?

Why are we allowed to just throw that all away? Why is that ok? Shouldn't there be, if nothing else, a moral or ethical obligation to say, "I'm sorry, I tried."  Shouldn't we be giving patients the opportunity to take what's been learned from the work they contributed to - the work that couldn't have been done without them - and start from a new baseline of learning? Why aren't we sharing what we are learning in research, successful or failed, in a way that allows folks to build upon it? This is among the greatest failures of modern medicine. 

I want to ask researchers what they're actually trying to do. If the answer isn't that you are trying to, wholeheartedly, help patients, then please stop wasting our time. 

A research project I had been a part of recently shut down and I'm so...I guess sad is the right word. I wasn't a participant, but rather a member of the study team so I can't help but feel a bit of responsibility. My frustrations are for those families who joined this project, sharing with us their precious time, the intimate details of their health and lives. People who were given hope only to be shown another dead end, and for no good reason. My participation and contributions felt uncomfortable at times. It was frustrating to have my ideas and suggestions for a more patient-centric approach limited by both institutional research regulations, and the discomfort or fear or I'm not exactly sure what of trying to do something different than it had ever been done previously. I should have pushed harder. 

Helping patients means doing things differently than they're being done right now.  I do believe researchers are  trying to learn, to help us. But to be honest, it's moving at a devastatingly slow pace and it's too often letting us down. 

Give me back what you've taken from me and encourage me to share it, to build upon it, to figure out what else we can learn together. My participation in research and the view I have of the participation of my peers has left me feeling exposed and uncomfortable, not wanting to say yes to future engagement. This is a problem that we need to fix! This is how we will really start to learn and grow and change and improve. Stop picking on the weaker man. 

Friday, July 29, 2016

Vacation FTW!

This vacation.... You guys, this vacation was the most vacation-y vacation I think I've ever been on. Yes, the kids were there, and yes it was hotter than just about anywhere we've ever been, but the "wake up when you want" and the "walk to the beach in 5" and the "getting in the pool a moment after someone's says 'Hey does anyone wanna go in the pool'"....and it being 85ish in there when you jump in. And I haven't even mentioned the shrimp and grits yet, or the oysters and fried chicken. A day at the spa and having someone rub my feet for an hour. RUB MY FEET FOR AN HOUR!! We're doing puzzles and playing euchre, coloring and crafting and moving at a pace that everyone should try. Clear minds here. There's not a schedule. If you wanna go sightseeing you just do it. And if you wanna go out for breakfast you just go. There's no "we need to get home for..." and there's no answering emails or solving problems because there aren't any problems. I'm reading a book instead of a Facebook feed and checking for the best spot to set up on the beach more than I'm checking email. This is freaking vacation, and I needed this from the bottom of those rubbed feet to the tip of my soul. I'm tan! Like not tan mom tan, but I've definitely been whiter, so this counts. This is cornier than I get, but the refreshment that this week away is bringing to our family after a month of problem solving and worrying and making excuses and trying and hoping - the kind of stress that exacerbates back pain and turns a typically happy family into a tired and confused family - the relief of this vacation has alleviated all of that. We didn't just pack up that worry and stick it on our doorstep to wait for our return, we let it go. We got our answers before we left and we decided to forget about life for a while. There's still treatments and medicines down here, but there's comfort in knowing that his lungs are ok. He's doing ok and we're doing ok. Actually we are doing freaking awesome. Charleston for the win!

That dropping FEV1 is just a number - as he proclaimed with frustration over the weeks of medications and appointments and attempts to get that number to stop dropping and start improving prior to our departure for this magical vacation - and  when we got the results from the bronch and found no bacteria - not even the achromobacter that we've been treating for the past few years - we moved to the other side of worry. As predicted, fungus seems to be the culprit, and an oral anti-fungal medication for the next few months will hopefully help to shift that dial back toward a higher number, but he is well. When we return we will talk to our doctors and make a plan and keep on swimming. For now, I'm putting in a vote for more vacation.

Thursday, July 28, 2016

Your vote counts

This post is an ask for your vote for me, as President of the....wait, wrong topic.

I'm darn proud of the work that I do and I'm even prouder to share that I've been nominated for it as a leader in the Health Activist category by Health 2.0 Conference, the leading showcase of cutting edge innovation that's transforming health and healthcare. I'm listed among many of great ePatient leaders that I'm also proud to call my friends and know that whoever is chosen to represent the collective ePatient voice will do us proud. I'd also feel quite proud if I was chosen as that leader. Voting ends this Sunday, so if you have a moment to spare, your vote for me, Erin Moore, under the Patient Activist category, would be most appreciated.

Vote Here!

Wednesday, July 20, 2016

74

I figured out what happened with the Ativan that we were trying to get for Drew's PICC placement last week. His doctor had tried to place the order for it through EPIC for me to pick up at the local pharmacy but because its a controlled substance, you need to physically pick up the prescription from the doctors office and hand the paper to the pharmacist in order to have it filled. Keep in mind that I was getting .25ml, a quarter of a milliliter of Ativan, to help a kid relax. When I had her paged from the pharmacy at the hospital, after searching  high and low for the prescription she told me she had called in, to send a new prescription down while she was in the middle of a clinic, she didn't have the option to send, but only to print. Even within the hospital, she couldn't send an order for .25ml of Ativan to the pharmacy for me to pick up to give to him. You may wonder why they didn't just have her order it and have someone from the hospital administer the medication while he was in the OR for the PICC? Well, there isn't a doctor or nurse whose care hes under unless he's admitted to the hospital. So for an outpatient procedure like a PICC placement, the only way we can swing it is for me to get the medicine and take responsibility for him when I give it to him. If only the different parts that make this whole work would talk to one another before making arbitrary rules. This system is so f'd up. I digress.

As I've blogged about before, we've survived a week on IVs. That means dosing at 7am, 8am, 3pm and 11pm - cleaning hands, putting on gloves, cleaning trays, scrubbing the PICC, counting supplies, hooking up the medication, letting it run for an hour, cleaning the PICC again and then tucking it away until the next dose. Add to that mix phone calls from the pharmacy and nurses about levels of medication in his bloodwork, schedules for deliver of new medication and supplies, nurse visits to change the dressing on the PICC and draw labs, and the other interruptions of everyday life. I shouldn't complain about being tired because he sits through all of this and takes it like a champ. There is no swimming, no running around, no sweating. We play games or watch movies, but its a whole lot of inactivity and boredom for a 6yr old.

The hardest part this time around is trying to convince him to take one more medicine, to do one more treatment, interrupting his play to sit through one more dose of IVs when he's feeling absolutely fine. We try to explain that we are doing this to try to get his lungs healthy, to try to get that number back up, and he cries and tells us its not gonna work, mostly because all we've done so far hasn't worked. Maybe he can feel that nothing is changing, or maybe he's just frustrated like us. Either way, its hard to get him to do this all when he doesn't feel like anything is wrong.

What's most disappointing here, and alarming, is that his numbers continue to drop. His normal lung function is in the mid 90's. In the middle of June it was down to 87. Then we tried an oral antibiotic and rechecked and it had gone down to 79. We tried a week on a steroid and it went down to 78. The we went in for the kill with the IV's and after a week, I'm sad to report that he's now down to 74. He's down 20 points in 4 weeks and that's not cool. Just for reference, typically around 30% lung function is when people are discussing the options for transplant.

It's so hard in CF to determine the cause of the problem, like a constant crap shoot. I usually know the symptoms that merit an intervention, whether its a steroid or some IV's, and he hasn't shown signs of needing either of these things this time. We know his lungs are colonized with a drug resistant bacteria called Achromombacter but that doesn't seem to be whats currently causing the problem, otherwise the many different stabs we've taken at this problem would have likely caused the decline to stop, and hopefully turn the other direction. I've had a suspicion from the start that there could be a fungus causing this problem. The last time he had a bronchoscopy, he cultured a lot of fungus in his lungs. People with CF get fungus in their lungs sometimes, and we got it. Was it because he's always on an inhaled antibiotic that he needs to treat symptoms? Did it come from the inhaled steroids he's been on to help him go longer stretches with good health? Is it just something in the environment that he caught because that's how unfortunate having CF can sometimes be? We have no idea. We treated it with 3 months of an anti-fungal medication called fluconazole and it seemed to help his ever so slight increase in cough go away. The thing was that there were no symptoms that alerted us to the fact that he had this problem in his lungs. Last year he wasn't yet doing pulmonary function tests to tell his how his lungs were functioning because he was too young. But could this again be the culprit? Could the fungus we treated have not really actually gone away and now be causing this rapid decline in his lung function? It's our next best guess but there's only one way to find out - time for another bronchscopy.

When Drew learns to cough out the mucus his body produces, we will be able to take a sample of that to the lab to get tested to see specifically what type of bacteria and fungus grows in his airways and more specifically target therapies to help him. Until he is able to do that, he has this procedure done called a bronchoscopy where they put him to sleep, and they squirt saline liquid into his lungs to "wash" them and then suck out the water and test it to see what kind of bacteria or fungus they've found. We can't do this when he's on IV medication or an anti-fungal or the medication will skew the results of the test. So effective today, we stopped all antibiotics and will take him in for a bronchoscopy on Friday afternoon to see if we can figure out what else could be in his lungs that we are missing, and then hopefully figure out how to treat it.

I spent two hours with his doctor yesterday talking through all the options and symptoms and possible outcomes, and I requested that we start him on this anti-fungal drug and think about the bronch when we return from vacation, something we are scheduled to leave for this coming Saturday. She didn't want to start him on something for fear that when we return and do the bronch it wouldn't give us a true picture of what's really going on because of the medication in his system. She also didn't want to wait much longer to try to get to the bottom of this because losing lung function this quickly just isn't good. She also put him back on the steroid with the hope that we can knock out some of the inflammation before the bronch to try to get a really good wash of his lungs, stating that there was little risk [except to his life as me or one of the other kids might strangle the devil child he becomes when he's on a steroid] associated to trying the steroid for another short period of time.

In the meantime, Drew is acting perfectly fine. You would have no idea that anything was going on with him, as he's skipping around here just happy as can be, a little annoyed and sad that he's gotta do so many treatments and take so many pills right now, but otherwise with his normally cheerful and passive attitude about life. I'm hopeful that we will figure this out and  be able to regain much of the lost lung function with the right treatment. While we won't have results on Friday since it takes time for a culture to grow, we will have a different treatment option to try and will have answers (hopefully) the following week. Regardless of whether they are good or bad, at least we should have a better idea of what we are dealing with. Thanks for all the thoughts and prayers. While we can't answer everyone individually, know that we appreciate the support and well wishes and will try to keep you posted as we know more!

This video was taken 15min before I wrote this post - as you can see he seems to be feeling just fine!
video

Tuesday, July 12, 2016

Crazytown

I'm hoping that this brain dump will alleviate my headache. God its been a long couple of days. We went back to Children's yesterday for a PFT to check Drew's lung function post-steroid and to determine if he would need IV antibiotics. I knew that the steroids weren't working, but held out hope that we wouldn't need IV's. He blew a 77 this time, down again. We went from 92 in March, to 85 in early June, to 79 two weeks ago and we're now down to 77. The mystery of it all is that he remains symptom-less. I've been wracking my brain trying to figure out what could possibly be wrong since this is completely uncharacteristic of an "exacerbation" for Drew. I have an exacerbation score sheet for godsake and it's telling me that he is not having an exacerbation, despite lung function that continues to drop lower and lower. All of this thought and wonder and worry culminated in the parking lot of Home Depot on Sunday where I sat crying because I couldn't find a paint color for my living room. My cure for grief is action, and so despite being mentally and physically exhausted, I decided that we needed to paint the house. So we did. (I finally found the right paint color and we got that all taken care of on Sunday, in case you were wondering).

We had talked to Drew's doctor over the weekend about our plan for if his numbers were still low, and had scheduled an appointment to have a PICC placed at 10am on Monday, something that we could easily cancel if his numbers were miraculously up. They weren't, and so at 7:45 on Monday morning we knew our plan. We had discussed what medications we would use, the dosing schedule, the plan for the PICC placement - no sedation, just a small dose of Ativan to keep him calm (next time i'm asking for a dose for me too) - and we were set. A wonderful friend who is going to make a wonderful doctor someday soon met us at the hospital to keep us company and better understand the patient and family experience of care through this process. I was so grateful to have her with us when the first hiccup of the day happened.

Drew was scheduled for his PICC at 10am, and I was to pick up his dose of Ativan at the hospital pharmacy that morning. When we arrived at 7:30, I realized that that pharmacy didn't open until 9am, no problem. After we got the results of the PFT and knew that the PICC was a go, we went down to radiology to register for the PICC procedure and were quickly ushered back to an isolation waiting room, something I request the moment we step foot into the hospital. For me, there is always a fear of a[nother] hospital acquired drug resistant infection, so I take extra steps to ensure his safety and my comfort with it. The issue I have with these so-called "isolation rooms" is that there is no indication of when it's been cleaned. I don't know who was in it before me, whether it's been cleaned, if it will be cleaned when I leave it, or anything at all about its cleanliness. I do not trust blindly, especially when it comes to Drew and germs. Can't we put a sign on the door to indicate that the room is clean? Anyway, we got called right up to interventional radiology so they could use an ultrasound machine to identify a good place in his arm to put the PICC and then mark it and add numbing cream. The nurse then took us back to the isolation room - the one we had been in, the one that I had closed the door to before we went upstairs for a moment but when we returned the door had been opened, the one I had no indication of having remained ours and no one elses while we were gone for 20 minutes - to wait for the pharmacy to open so we could get his dose of Ativan before starting the procedure. She told me to have the receptionist page her when we got the medication and were ready, as it looked likely that they could take us early.

So at 9am, I left my friend with Drew in the isolation room to run over to the pharmacy to pick up the medication, only they didn't have it. Did I screw up? Was I supposed to have gotten it from CVS on my way in? I had her call CVS to see if they had it and could transfer the prescription, but they didn't have it either. His doctor had emailed me yesterday to say she had called it in, and I was able to pick of the other anti-nausea medicine, but the Ativan was nowhere to be found. I called up to clinic and had the receptionist page the nurse who was covering for our nurse who is on vacation. She told me she would page Drew's doctor, who is in clinic seeing patients, to have her call down a new prescription as soon as possible. At 9:40, forty minutes after having arrived in the pharmacy to pick up this order and delaying the process of getting a PICC, something that was suggested by my husband that perhaps I try without the Ativan so as to not delay the placement of the PICC any longer, the pharmacy got the order and gave me the med. I was livid. I was livid not because of the mix up, though I don't know what happened to the first order, but because I was already exhausted - mentally and emotionally, trying to prepare myself and Drew and our family for what life will be like for the next two weeks with running IVs from home, with no swimming, with no sweating, with lots of interruptions from pharmacies and home care nurses and extra appointments and a rigid schedule of medication infusions - and now I had to become the detective and problem solver of a problem that I had no part in creating. Sure, I could have walked away and we could have done the PICC without the Ativan, but to help him, if even just a little, relax and feel a tiny bit more at ease about the whole process was worth the work. The longer it took to get the medicine, the more my stress levels rose. He was waiting, nervously, without me in a room with a friend of mine he only kind of knows while I asked and waited and tried to decide which direction to go. Thankfully I stayed and got the medicine, because the PICC placement didn't go as planned - 6 sticks, two blown PICCs and finally a successful placement later and we were all set. Without the Ativan, this would have been suffering. Avoidable, minimizable suffering that has a lasting impact on a kid who already has more to deal with than most adults will in their lifetime. I will always request Ativan, not because he needs it, but because he deserves it.

Following the PICC placement procedure, we went back up to clinic to meet with the homecare nurse for the first doses of the medication. We always do this at the hospital in case there is a reaction. IV's are no joke, and its better to be safe than sorry. Our nurse was lovely and we chatted while I answered a flurry of text messages from concerned friends and family members. Drew happily colored and played on his iPod until the first two infusions were done, and we were on our way home by 3pm. To think of this as a short day was a bit ironic given that we had arrived at 7:30am, but sometimes these days can last for 12 or 15 hours, so this felt short. It felt short anyway until we got home. Then it hit me like a brick wall. We had made it, I could exhale, and the stress of the unknown from the past several weeks started to show up in my horrible back pain, throbbing headache, and exhausted mind. I laid on the couch for the next many hours, my husband putting the kids to bed, and then finally went to bed myself after hooking him up to his nighttime dose of Meropenum. I woke up today feeling awful. My head hurts. My everything hurts. I don't know if its harder this time because he's more aware of what's happening and in addition to managing my own stress and anxiety about everything I'm also trying to minimize his? Is it because the rest of the family sees this as unfair and I feel responsibility to help manage expectation and disappointments and frustrations? I don't know. I do know that in a day or two we will find our groove, everyone will have accepted our temporary routine. Forgive our messy house and scattered minds and shitty attitudes for the next two weeks. It's sort of like survival mode over here, for all of us. We so greatly appreciate all of the thoughts and prayers, and will keep you updated on all things happening here in crazytown.