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.

Thursday, February 5, 2015

That time I went to the White House (part 2)

On Friday, January 30, 2015, I arrived at the White House (yes, the one the President lives in) at 9:30am. I found some of our CF Foundation Leadership already waiting at the gate along with Bill Elder, an adult with cystic fibrosis who benefits from Kalydeco, the life changing drug that essentially cures cystic fibrosis in the 4% of CF patients who have the specific genetic mutation that it treats. I was introduced (or in many cases introduced myself) to anyone who looked even mildly familiar. I met a Nobel Laureate from MIT, the founder of a startup that is finding new ways to use "big data", and the current director of the National Institute of Health and discoverer of the CF gene in 1989, Francis Collins. He was in line behind me at going through White House Security, and when I saw him, after I did a double take, I introduced myself and asked him if he would mind taking a picture. What an incredible honor and privilege to be at an event, at the White House, with the man who made one of the first and certainly most remarkable discoveries affecting the CF community.

This is the post about the details, and I'm going to give you the nitty gritty, because if I hadn't been there but I knew someone who had, I'd want to know.

You would think that they would have a lovely holding area for White House guests, perhaps something more than your run-of-the-mill tent equipped with a space heater and a few secret service agents. Alas, they do not. They do however have multiple tents, where they perform the exact same security measures multiple times.

If you make it through the tents, you enter a small building with signage that indicates that there are dogs inside. Behind a large screen that you cannot see through, you walk past one or many K-9's toward a metal detector and an X-ray machine for your personal belongings. This is no nonsense. They're not smiling or asking about your day. If push comes to shove, these folks will give you the shakedown.

And then you're in.
Francis Collins, Director of the National Institute of Health and Scientist responsible for the discovery of the CF Gene

Me with Preston Campbell, SVP of Medical Affairs at the CFF and Bill Elder, an adult with CF

When I walked into the White House (THE WHITE HOUSE!!!!!!) I saw the President's dogs, Bo and I have no idea what the other ones name is. I'm not a dog person, but still, I saw the dogs.

There were Marines around every corner donning their dress blues and referring to everyone as sir or ma'am. There was a coat check further down the hallway where we saw the dogs. Everything was so historic and pristine! We made our way upstairs to the reception area where they had coffee (White House coffee!) and water. I passed on the drinks for fear of spilling on my outfit. The was a marine playing a beautiful historical looking piano as people milled about, introducing themselves to one another and sharing their excitement for being invited to such a cool event.

After about an hour, another set of doors was opened leading to the room where the announcement was to be made. We filed in and found our seats. The only seats that were assigned were the guests of honor, those patients and families who had attended the State of the Union the week before as guest of the First Lady. They go the front row. Let me just repeat that for a second - PATIENTS and FAMILIES sat in the FRONT ROW. Not the second row. Not mixed in with the crowd. The very front row. That in itself spoke volumes. There were probably 6 rows in the front section followed by an open aisle and then the remainder of the seating. I was in the first row of that second section. Here was my view, panoramic style:

There was an announcement made to silence all devices, and a hush fell over the crowd. As we anxiously looked around the room, I caught the eye of one of the marines who whispered to me that as long as the piano was playing the President would not be coming out. I felt cool for being in the know.

A few minutes later, the piano stopped, the patient/family guests of honor were ushered in, and an announcer said, "Ladies and Gentlemen, The President of the United States of America."

The President was introduced by Elana Simon, a 19 year old student who’s high school science fair project was about finding a treatment for her own specific type of liver cancer. In other words, the President of the United States was introduced by an ePatient. 
The teleprompter that read Remarks of President Obama on Precision Medicine, The White House, January 30, 2015.

To borrow a nice summary from the S4PM website, "The initiative begins with a budget request for $215 Million in new funds. The funds will be used, mainly at the NIH, to:
  • Develop a national cohort of a million volunteers who will donate their health-related data for open research.
  • Provide increased funding for research to identify genetic drivers of cancer.
  • Help the FDA speed up the regulatory process
  • Fund the development of data interoperability standards

Because Cystic Fibrosis is such an incredible example of the value of personalized medicine, treatments that target specific genetic mutations, one of the VIPs was an adult with CF, Bill Elder. Bill was a guest of the First Lady at the State of the Union, and was invited to attend this event as well. President Obama highlighted the success of Kalydeco by sharing Bill's story. I was able to get a video of it so check it out:

I may not be Mr. Obama's biggest fan. However, this announcement and the proposed commitment to advancing this initiative is the first step toward progress in curing a disease like CF. Congress must still approve this budget. We will need to learn more specifics around the proposed initiatives. We will have to get a better understanding of the resources available to push this forward. But it's a start, and it's further than we have ever before been with support for addressing the extremely personalized needs of patients with specific genetic mutations in CF and other rare and orphan diseases. Here is a fact sheet that the White House released post announcement:

Best parts: 
  • “Patients and advocates won’t be on the sideline. They’ll help us design this ” -Obama
  • "Precision Medicine is delivering the right treatments to the right person at the right time every time” - Obama
  • "The patients here are living proof that the new dawn of precision medicine is upon us." - Obama
The talk was about 10min long. At its conclusion, there were a few handshakes and pictures with the folks in the front row before the President was gone. We came, we saw, we left. Let me tell you those marines mean business when it comes to ushering you in and out of the White House! 

Following the announcement, I was invited to a luncheon with a handful of other patients and patient advocates pretty close to the White House, and it was equally as amazing. I met some seriously incredible people, including a guy who found his own brain tumor by accident and had a 3D printout of it in his pocket, a woman who created a remarkable registry for a rare disease that her children have, the always amazing Emily of Emily's Entourage (who just recently did THIS) and many others. We talked with some of the folks who work at the White House Office of Science & Technology Policy about what we thought some of the major hurdles might be. Chief among them were privacy issues and the need for updated IRB protocol. The whole thing was like one big highlight in my life, but one of my favorite parts was definitely getting to spend some more time with Francis Collins. It was such an intimate group of people at this lunch (maybe 25-30 people?). Francis Collins had us go around the room and introduce ourselves and say 3 words related to how we were feeling about the announcement that we had just been a part of. There were lots of "hopefuls" and "readys", a few "desperates" and even a handful of concerns related to the funding and the resources available to do this. When it came back to Francis Collins, this is what he had to say: "I've been dreaming about a day like this. We are all in this together." He went on to ask us to work with them, to share with them all the work that we are doing so that they can learn from us, saving time and money by not having to recreate the wheel. He asked us to speak up and let them know when they're doing something wrong, and then work with them to make it right. "We are all in this together." Indeed we are. 

I'm hopeful. I know that there is a long way to go, but we finally have a stake in the ground, a starting point. This experience definitely ranks near the top of my list of coolest things I've ever done, and I do hope that this really is just the beginning!