Wednesday, May 20, 2015

Stay tuned for whats to come!

I use to love writing on this blog. I still do love doing it but just can't seem to find the time. I want to commit to doing it more often, but it might have to wait a couple more weeks until school is out and we can finally set a new schedule that builds in time to do just that. I'm currently enjoying some R&R in a Bed & Breakfast, by myself, in Sonoma, and oh my is it glorious. A much needed escape from the chaotic everyday.  Yesterday I did a little touring and meandering around town before turning in early for a good night sleep. Today, I woke up late, chatted with a friend, and pulled out my computer with one thing in mind, writing on this blog. Later today I will be heading down to San Francisco for a meeting at Genentech before heading back to my family on Friday. I'll be in town Saturday and Sunday, and then I'm off to DC for the second design meeting at the CFF where we are imagining the CF Care model of the future, and then building it. More on that in a moment!

First, an update on Drew. He is well! HE IS WELL! We aren't sure if he was ever really having an exacerbation or what, but through some digging into my self tracking data I found that we had stopped Prevacid around the time that his cough developed, and when we restarted it, after 10 days on oral antibiotics, 14 days on IV antibiotics and 5 days on a steroid, his cough finally went away, and stayed away. It makes so much sense how his cough was random and sporadic, his appetite was down and his BMI was falling. Was it acid reflux that this kid was experiencing? When we added Prevacid and his cough disappeared and his appetite and BMI improved it sure made it seem like that was the culprit. I'm just so glad that he is better. The inexplicable in CF is often the hardest pill to swallow. I'm hoping some of the stuff I'm working on will help to decrease the amount of inexplicable by organizing what we all have and know just a little bit better than it currently is.

I have shared many times in the past about this C3N work that I've gotten involved with at Cincinnati Children's and the CF Foundation. Over the past 6 months I've been working with a couple dozen other folks who share my desire to see big change in CF care to dream up what the perfect system could and should look like. This isn't just about patients. Its about patients and clinicians and researchers all working together, finding ways to collaborate, do our jobs better, improve health and care. We've come up with some design targets; the new system for CF care must do things like build a culture of generosity, shared commitment and personal agency to improve health outcomes; it must foster strong care partnerships with mutual trust, collaboration and empathy; it must enable easy peer-to-peer support and information/relevant research; allow everyone to get care that they need, regardless of their ability to pay for it; it must encourage everyone to bring their ideas to the table for continuous learning and quality improvement; create joyful work conditions for those providing care to people with CF; and these are just some of the design targets! This is no small scale change that's happening here! We have spent months deconstructing the current system for CF care - what works, what doesn't work, what good ideas do we have for improvement that are either novel or stolen shamelessly from other disease communities doing this successfully. We have interviewed and researched and discovered different programs and tools that can potentially help us to meet some of our design targets, and have brainstormed ways to measure their effectiveness.

Next week, on Tuesday and Wednesday, the team will get back together at a meeting in person at the CFF in Bethesda, to shake out how we can pull together the key components of this new system and test whether or not some of the proposed pieces will be an improvement! What you probably don't know is that YOU are part of the team. If you have CF or love someone who has CF or care for some who has CF or research CF or fund raise for CF, you're part of the team. We all have good ideas to share, and this system will be a dynamic one that allows for anyone to contribute and test out ideas for improvement. It sounds a little abstract right now, but I promise you, it's going to bring transformation change to our community. The success of this model is dependent upon your commitment and contribution. Join Twitter and follow the hashtag #CFBigIdea. Write a blog about what you know and what you love and hate about CF life and care. Start a podcast (I did, you can search CF Matters on iTunes!). Tell the community what you're clinic is doing, and what ideas you have for improvement. Ask questions. And stay tuned for whats to come!!

Thursday, April 16, 2015


It has been a whirlwind month for us! As I mentioned in my last post, Drew had been struggling with an increased cough for several weeks prior to the decision on March 24 to start him on a course of IV antibiotics. The goal was to try to kick whatever is going on with this increased cough, lack of appetite and subsequent weight loss. We started his IVs on the 24th and by last Tuesday, things weren't much different. He's not presenting with a typical CF exacerbation type cough that is constant and relentless, but rather is having irregular coughing fits periodically throughout the day or week to the point where he is gagging and throwing up. He can go 2 or 3 days without a cough, and then out of the blue will wake up in a coughing fit, or have one in the middle of the afternoon at school with little warning.
Last Tuesday was the day we were going to pull the PICC line but decided to keep it in in case we needed to try different antibiotics to see if he might respond differently. We went back to the doctor and together decided to try an oral steroid for 5 days as he sometimes responds to that better than an antibiotic because of his airway abnormalities and trachealmalacia. He had another coughing fit on Wednesday evening, and then was cough free through Sunday but work up on Monday morning having another coughing it. After some emails back and forth with his doctor, we decided to wait a few more days to see if the steroid had actually worked and or if it would be necessary to restart a different IV antibiotic. He hasn't really coughed much over the past three days, maybe a little more than his baseline, but nothing too significant. 
This is the really hard thing about CF, for me anyway. It's never really being sure if we're out of the woods, in the clear, well. Is this slightly changed baseline his new baseline? Does the benefit of trying more medications and other treatments to try to get rid of symptoms outweigh the risks of those harsh drugs and the time that it takes us to implement? Without having a CT scan, which comes with its own risks, we aren't really sure what kind of lung damage is taking place, and even if we knew, there really isn't much that we could do differently. I suppose we could be even more aggressive, whatever that means. Just the thought of having more to do makes my head hurt. There seems to be such a lack of evidence on the "best" treatment or combination of treatments for this disease. We are always making our best educated guess, with the guidance and support of our care team, but we just never know. It's disheartening. 
We decided this morning that we will pull his PICC. He no longer has symptoms that we would necessarily treat with IVs, and there are risks associated with having a PICC in (bloodstream infection, for example). He's going back to the doctor on Tuesday of next week, exactly 4 weeks after our special #CF1Day event took place and this whole series of events started. We really have come full circle. If you have a positive thought to throw our way next Tuesday, we will take all the support we can get! 

Tuesday, March 31, 2015

Dear everyone,

It has been a rough week. At this time last week we started to have the conversation with Drew's doctor about whether IV antibiotics would be necessary to get him well again. I say it was out of the blue, but maybe deep down I knew, or feared, it was coming. It sort of felt a little like everyone on the healthcare team had had this conversation without me - if his cough still isn't improving then we need to talk to mom about getting him on IVs. Well, his cough wasn't improving, his BMI was down along with his appetite, and his lungs didn't sound great. I sort of wish I had been included in the conversation, if there was one, about this plan, because I feel like I was blindsided a bit. It sounds strange to even write that because this isn't our first rodeo. There is always a chance that we will need a hospital admission or a course of IVs. He looks well, everyone says, and while I agree, I knew that he wasn't. The cough had been going on for more than a month, and it was time. I just wasn't ready for the conversation, and that sucked.

One of the hardest parts of the conversation about starting him on IV's was which antibiotics to use. The problem that we have is that Drew has a multi-drug resistant bacteria in his lungs called achromobacter. In CF patients, often times treatment is based on symptoms, and this bacteria seems to have permanently increased his cough and taken away his appetite. He's had this bacteria in his lungs for over two years, and there is little hope that we will get rid of it. Drew spends 3 hours a day inhaling a series of medications to try to keep this bacteria suppressed so that he can live and breathe well. This bacteria has one antibiotic that seems to be effective in helping him to stay well, and that's a medication called Ceftazadime. The concern that we have is that, like anyone else and any other antibiotic, if will eventually lose its effectiveness, and that's really scary for me. To know that he is 5 and only has 1 antibiotic that works is scary. Does this mean he's going to die? Not tomorrow. Probably not this year or next or the year after. Hopefully, dear God hopefully, he will be with us forever. Everyone tells me we will cross that bridge when we come to it, but in the meantime I'm searching desperately for anything that might offer a new hope. I cannot wait until we get to that bridge to figure out how we will cross it.

So when we were deciding which antibiotics to treat him with, we decided to use two medications that his bacteria doesn't usually respond to well. Why, you ask? When done via IV, the hope is that they will work together to attack and suppress the bacteria and keep it from causing problems. We decided to do this because his body needs a break from Ceftazadime so that it remains an effective treatment option for us. The concern with using the other two medications is that they won't pack the punch that we know the one that works usually does. So we risk being on IVs for a longer period of time if we run into the need switch the medications because he's not getting better.

The good news is he seems to be improving. His cough is down from what it had been earlier this month, and his appetite is like I've never seen it. The homecare nurses say that his lungs sound clear. He's just not back to 100%. I worry that we will get him to 80% and end up on IVs again before we want to be. We are going to hang on and keep trying, hoping that this works, and make a decision on whether he needs to try something new later this week.

Life in our house when Drew is on IVs is sort of miserable. The positive spin is that at least we aren't in the hospital. However, we have nurses coming two or three times a week, sometimes twice a day, to draw labs to make sure that the medication he is on is potent enough to kill his bacteria without being too potent to kill his kidneys. He needs regular dressing changes on the PICC, which are awful because its like an incredibly well stuck band-aid that's twice the size and stuck on to an open wound. He hates it, and so do I. If you added up the time that I have to spend on the phone with insurance, homecare nurses, homecare pharmacy, and his medical team, I would estimate 2+hours a day. They're all just a quick 10-15min call, or series of calls when we have to leave a message and then find time to call back when they return your message with a message. That's all on top of our 3 hours of regular maintenance treatments per day. Our schedule is completely screwed up because of the times of day that we need to give the medications - 7:30am 8:30am, 3:30pm, 11:30pm - and the times that we must be available for nurses to come and draw labs, usually two and again 6 hours after we dose one of the medications. Homecare delivery drives are knocking on our door with supplies from 8am until 9pm, which I am so grateful for, but I'm overwhelmed. And the exhaustion isn't helping that any.

Drew asked us, jokingly but nonetheless, if the PICC was going to make him die. No sweet boy, the PICC is our hope for keeping you very much alive. It highlighted that we haven't had time to help him or any of the kids really understand what is going on, both with this acute illness but also overall with regard to CF. We don't keep any secrets, but we answer questions like you would answer questions related to anything a 6yr old or 4yr old might ask, by addressing what they asked and not going to far off track so as to confuse or scare them. I've found that just listening to them provides far greater insight than asking does. Its so hard at the end of a long day to find the time and patience to to listen to them at bedtime, but they need it. It's also hard that the listening is often interrupted by one of those phone calls that I have to take or knocks on the door delivering medication that I must sign for.

I need to publicly thank our family and friends for showing up when we need them. From delivering dinner to facilitating work phone calls that I cannot attend to sending coffee and coming by to say "you need a drink and a distraction", to just listening to me complain, and worry, and is all so appreciated. I may not have time to write you a thank you note, or the energy to even tell you in person, but we appreciate all of it and all of you.

You may have seen something that was recently shared on Twitter about a day that I spent partnered with Susannah Fox in an empathy building exercise organized by Smart Patients. The project was called "Just for a Day", and I spent the day sharing all of the details of what its like when cystic fibrosis is a part of your life. You can read the first part of the story that Susannah captured by clicking here . The outpouring of love and compassion and empathy has been overwhelming, in a mostly good way. It's hard to do this CF mom job, and so many of us do it quietly, trying to fit in with the rest of the world, when really our lives are nothing like those in the rest of the world. The congnitive burden is heavy, and we are forced to make a ton of decisions  many times a day. We weigh benefits against risks daily, hourly, talking with care teams and others who have walked in our shoes. While all advice sent and recommendations made for ways that we could better care for Drew or keep him healthy are recognized as well intended, we are already struggling with the weight of the decisions that we have to make when things don't go as planned. And it's often not because we did or did not do something, but because the complexities of this disease and the path it takes regardless of effort. Please know and trust that we are trying the best that we can, and please keep sending your support and advice and evidence, but kindly leave your judgement at the door. We are trying.

The next time you see me or text me or call me and I don't answer or seem flip or look tired, don't take it personally. I am totally exhausted. I'm physically tired from running this life. I'm mentally tired from having to make at least a dozen serious decisions a day, and manage 4 different people and a disease that has a life of its own. I'm emotionally tired from the worry about the decisions that I make, related to and not related to CF. I know that this, too, shall pass, but right now I feel like we are in the trenches and it is exhausting.

Thats all for now,

Tuesday, March 24, 2015

What we need most

I'm sure many of you heard that Drew had a rough day today. A routine clinic visit didn't go as planned and he ended up in the hospital for the day getting a PICC line to do 2 weeks of IV antibiotics for a bacteria infection in his lungs that he can't kick. This means strong mediciations administered through a IV in his arm 4 times per day. It means no gymnastics, no swim lessons, no baths. It means hours per day spent sitting and watching TV or playing on the iPad during infusions while his brother and sisters and friends play. It means painful dressing changes and distraction from the simple pleasures of everyday life
Coming home from the Cystic Fibrosis Foundation​ Volunteer Leadership Conference that I attended last week, I believe that a treatment for him is on the way, but we continue to need the support of you all to help us get there. Tomorrow night I am having a Matilda Jane fundraiser at our house from 7-9pm that anyone and everyone is invited to attend. A percentage of all purchases will go to the Cystic Fibrosis Foundation. If you cannot attend but wish to place an order you can view the catalog online at, add items to a Wish List, and then choose the option "Send to Trunk Keeper" and enter Tomorrow is the last day that you can order as the party will close at the end of the night.

What we need most right now is your support. Join us in this fight by making a donation today!

More on today's clinic visit coming soon!

Saturday, March 14, 2015

He's FIVE (and he's still alive!)

I have so many thoughts to share on this special birthday, but for now I will ask for your support. I want more birthdays for Drew, and the only way that he will get there is with your kindness and support. Thank you - for supporting us through the years, for being our friends, for loving Drew like we do.

Donate Today by clicking HERE!

Saturday, February 28, 2015

Rare Disease Day 2015

A few years ago, I wrote this post about what a typical day looks like for us. What I wasn't able to 
articulate at that time, whether it was because I was so deep in the motions of everyday life or the true burden of this disease hadn't yet full sunk in, was how it makes me feel. So today, on Rare Disease Day, I want to share what a day really feels like for me.

I wake up in the morning and I don’t want to get out of bed. Like ever. I'm tired. Always. I'm happy to grab a kid and pull them in to snuggle, but I’m so tired of this routine that I just don’t even want to get out of bed and start another day.

I finally pull myself together and head downstairs. I pour a couple of Ensure Plus into a cup with a straw, hoping he will drink them, knowing that he needs them, worrying that they won’t be enough. I make breakfast for the other three, and lunch for my first grader, while everyone complains that i’m making something they don’t like or that I’m not paying attention to whatever they’re telling me. Its true, I’m not. I want to be paying attention, but I can’t. I’m not sure why. I’m thinking about the coughing that I hear. Its usually there when he wakes up, some days worse than others, most days subsiding after the morning treatment. I have to decide if its enough to stop everything else and start the treatment or if it can wait the 15min until two of the 6 are out the door. I’m yelling out spelling words and only half listening to hear if she spells them correctly because the coughing interrupts her answers.

While they’re eating breakfast, I pull together the medications and equipment that we need to start our day. Theres always moaning and groaning when I announce that its time for “breathies”, and I wish he could really feel me when I say “I so hear you bud, I don’t want to do this either”, but I’m committed. He begs for the “short” treatment, the one thats only 24 minutes instead of 30. He can tell the difference.

Once we agree on a show to watch, we settle in for 30 minutes of vigorous shaking and various medications. We always sit with him, usually me, so that he doesn’t have to do it alone. The weight of that is heavy, as sitting with him means time away from the other two still at home. They’re use to this. They shouldn’t have to be. It doesn’t make it any better.

Truthfully I don’t know if any of them really know what CF is. I don’t know if they should? I wonder if I’m keeping too much from them? I also wonder if I’ve given them too much. Are the behavioral issues because he’s 4? Does she act out because I unfairly dedicate more of my time to him? Are they because she doesn’t understand or she’s curious but doesn’t know how to ask? Do they have questions that they’re not asking me because I’ve shown them that I don’t have time to listen? I know that really, I don’t have time not to.

I’m scared when our secondary insurance decides to drop us and we now need a few extra hundred dollars a month to pay for the Ensure, that is the only reason he is gaining weight, on top of all of our medications. I keep meaning to schedule an appointment with the behavioral psychologist about his eating habits but I hate that I need someone to help me parent. It feels like a failure. When dinnertime becomes a nightmare, when he won’t eat any solid food, and when has to whisper to me that he doesn’t want to eat when other people are looking because he has realized and I have failed to realize that he’s aware that others are aware that there’s a difference between whats on their plate on whats on his, I concede and schedule an appointment. It feels awful.

When we go to the doctor and we only get more bad news - more to do, more to schedule, more to track - if feels like I’m going to drown, but I have no choice but to just keep swimming. And I have to keep a smile on my face because I am trying with all my might to convince him that he’s no different than anyone else, and that this disease is just something that runs in the background. He doesn’t need to know all that it takes, but he does. He’s smarter than most people I know, and he’s not even 5 yet.

None of the tasks that I get to do on a daily basis are a heavy lift for us - by most standards a well-to-do family, reasonably well educated and financially secure - but the combination of small tasks that I have to perform, the cognitive burden of this disease is sometimes more than I can bear. I wonder what the real impact is of all of these medications and treatments and appointments. I wonder what’s actually necessary, and if I’ll ever actually know whats really necessary. Is it okay that I skip an afternoon treatment now and then for a trip to the park? I think his mental and emotional well being are of equal importance to his physical health. I think the doctors agree, but they only ever seem to offer more to do. I do believe they have his best interest at heart, but not like a mother does. Or maybe they do have his and I’m being selfish in thinking they perhaps they should also have mine? Do they understand how guilty I feel when I have to say no to school or the day of a big party because the teacher has been kind enough to let me know that there are a number of kids there with colds? They can’t possibly feel the ache in my heart when school calls to tell me he won’t come out of the bathroom because he has trouble with his bowels and wiping by himself. When the family wants to take a vacation to a lake, something that we just can’t do because of his health, or have a fire in their fireplace, the little things that we just can’t partake in, and trying to minimize them so it seems like no big deal that we have to miss out, when really I desperately want all of my kids to partake in lifes simple pleasures. Is it right that I keep all of them from these things so that he doesn’t feel even more left out? Should they have to suffer from this disease too?

There is always a fear that you will lose your baby. It’s not all encompassing or debilitating, but its ever present and its completely out of our control. I could do everything right - by the books, medically speaking, everything right - and we could still lose this fight. It happens every day. We see it on Facebook, on blogs, among friends. I think about that.

I also think about how lucky we are and how good we have it. I cry for the families that lose their children to CF because they were born into a different socioeconomic class than I was. I feel an obligation to use what I have and what I know to help them too. And then I feel guilty for dedicating my time to someone other than my family. I wonder if all the work that I do is really hurting my family more that its helping us. I think that a balance is good, teaching my kids the importance of helping others and also finding time for yourself. I don’t think I’m doing a very good job of it, but I’m trying.

That’s what I think about in a day, every day. What I what you to know is that we are trying. The people like me, and the people nothing like me; we are trying. We are trying the best that we know how. We have to perform medical procedures, without any professional medical training - correcting a rectal prolapse, cleaning a stoma, changing a trache. We compound medication in our kitchens, monitor oxygen saturations at 2 in the morning, sterilize medical equipment the best way that we know how, all with knowledge that we’ve put together from the expertise of our healthcare team and also from outside of the institution, from our peers. Our medical care does not happen 4x per year when we go to see the doctor. It happens 24hrs a day, 365 days per year.

We aren’t mad at doctors. We are desperate for them to ask the questions that will allow us to tell them all of this. This isn’t easy to say, and we often don’t even know how to put into words what our priority is. We want the same thing that you want for us - good health. But to minimize the impact of this disease in our lives - for a day, an hour, just a moment - would mean the world.

I often go to bed at night feeling defeated. The kids are not happy. The house is not clean. The disease is not cured. I know whats on the other end of a good nights sleep. But I remain hopeful.