Rare Disease Awareness Day

Today is Rare Disease Awareness Day. Affecting only 30,000 people in the US and 70,000 around the world, Cystic Fibrosis is a rare disease that we could use a cure for. Help us to spread the word and raise awareness to cure this disease!

DONATE HERE

Three Year Anniversary

We had our 3 Year Anniversary appointment at the CF clinic on Wednesday morning. Drew had his labs drawn a week before the visit so that we could review them with his docs at the visit. He came through with shining colors! Everything looked great - all of his vitamin levels, all of the organ function tests they do, all around an A+. We didn't do any imaging this time because Drew is about to start a 2 year clinical trial using imaging and blood work to identify bronchiectasis as an early age. They will do a CT scan and an MRI (a super brand new kind involving the use of helium that does something to the protons...or something like that) along with bloodwork to determine if there are markers in the blood that correspond to what they see in a scan. It is a 2 year trial and will hopefully be getting kicked off later this year. The results will be available to us and our doc, and we know the doctor running the study (the one and only JP Clancy) so we will just take a look at them to see how he's doing. I hope everything looks as good as it did last year when we had his CT!

This time we were in clinic, we saw an endocrinologist. For anyone unfamiliar with what they do, basically they deal with all things hormones. So for CF, that could involve insulin production or growth horomone, among other things. Drew is between the 25th and 50th percentile for height and his height prediction based on our heights says that he should be near the 70th percentile. I understand that kids with CF can have trouble with growth given all of their vitamin deficiencies and other abnormalities and I certainly don't think that Drew looks too short or scrawny, but we wanted to keep an eye on things to make sure that he doesn't start to drop off that curve. There is nothing concerning going on right now, but we're going to have some labs done and a growth plate imaging study done (I don't think that's the official name) and just keep an eye on things. I am anxious to learn more about how we will work with the endocrinologist going forward and more importantly how she can help us!

I think I mentioned a couple of weeks ago that Drew had an ongoing cough for a couple of weeks and we ended up putting him on an oral antibiotic. His culture showed some staph, which he grows from time to time, but we really just thought that his cough had gone on for long enough to take action. The antibiotic seemed to clear him up pretty good, but once he was off of it it seemed to come back within a few days. It's not a terrible cough - just more than his baseline and a little junky. We also realized that when we are cleaning out his sinuses a couple times a day that we are getting a lot of stuff out. So yesterday I talked to Drew's doctor again and we decided to put him back on the antibiotic for another week, and to be aggressive with our sinus rinsing. We are starting to think that this is a sinus issue and not a lung infection. This seems pretty similar to what happened last year around this time - a congested nose and a lingering cough led us to ENT where they found significant sinus disease and a couple of weeks later we had surgery. We've actually been doing really good with the sinuses since that surgery, but we might have reached that point again where we need to go back in and take surgical action. I hope not, like really really hope not. I'm hoping that with aggressive rinses and an oral antibiotic and a topical antibiotic drop in the nose that we can shrink some of the polyps back down and avoid having them scraped out. I feel like every time we end up in the hospital for a procedure, no matter how big or small, we end up going home with a new bacteria that we don't need. I know the importance of having some of this stuff done, but whatever I can do from home I would definitely rather. I guess we will see how things go!

Last week I had a conversation with Ginger.io and was asked to be a part of their team!! We're going to connect again next week to work out specifics. It will only be a part time gig and will start out working with the IBD clinic at Cincinnati Children's and then expanding from there. The reason we are starting with IBD is because Ginger.io and the C3N are already integrated with them as they were the pilot disease for these projects. The results being seen from IBD folks utilizing the C3N are kind of fascinating and I am so so very excited to get to be a part of it. First step IBD, next step CF...and then we change the world [of chronic conditions]!

Next week I'm working with the QI team at Children's to create a Facebook page where patients and families can connect with clinicians to share tips and advice. Then next Saturday  morning we are heading east to get ready for the CF Foundations annual advocacy campaign "March on the Hill" followed by the Volunteer Leadership Conference. I'm really excited about both, and will be tweeting throughout both events (@ekeeleymoore). Once we are back in Cincinnati I will definitely find some time to post about it!

2013 Fundraising

Believe it or not, I really don't enjoy fundraising. I view it as a necessary evil. I do it and I do it well because we need the money to cure this disease, but I don't like it. My friends and family are sick of me pushing one idea after another and holding event after event after event. Thank God I picked good friends because most of them bear with me :)

We are always open to straight donations and greatly greatly appreciate any amount you can afford. You can donate to our Great Strides Campaign by clicking HERE.

Just this past winter I was introduced to Usborn Books at a Holiday Bazaar that I attended. Their books are phenomenal. They are so unique, of excellent quality and most importantly my kids love them. Usborn Books is having a fundraiser for us, donating 25% of all sales in the month of Feb. to our Great Strides team. You can check them out by clicking HERE.

The other thing that Usborn Books is doing for us is a Cards for a Cure Fundraiser. They sell boxes of greeting cards - 30 cards for $30. There is a kids box and an all occasion box. Usborn donates $13 for every box sold. Unfortunately you can't purchase these online, but if you're interested in a box (which you should be because they're very cute and a total steal), just email me via the "Contact Me" link on the left side of this page and we can get you what you want.

Finally, I'm having another Tupperware Fundraiser. I started selling Tupperware about a year ago as another way to raise money for the Cystic Fibrosis Foundation. Tupperware has an incredible Fundraising program whereby 40% of all sales made through a fundraiser are donated to your organization. I have a fundraiser set up that you can access by clicking HERE. Even if you already have a ton of Tupperware in your house, there are so many new cool things to check out so take a look!

Additionally, if anyone reading this, local or from afar, is interested in hosting a Tupperware party for me, I will donate 100% of my profits to the Cystic Fibrosis Foundation, either to my team or if you have another team that you are walking with I will donate them to yours. Hosting a Fundraiser earns you 40% of all sales, and hosting a regular party will earn you what I make which is 25% of all sales. If you think you can pass a catalog around the office and collect a few orders to help us out (or help yourself out if you're another team reading this), then drop me a line and we can get something together.

We're hoping to have a big "Girls Night Out' fundraising event in March, and then our walk is in May. I'm always open to new ideas and suggestions for fundraising success so send them my way if you have them!

Tomorrow is Drew's 3 year anniversary visit at the CF Clinic. We had his labs drawn last week and to my untrained eye, everything appears within normal range. We will have to wait to see what the docs say in the morning, but I'm hoping for a clean bill of health. Send some prayers our way if you have them and i'll be back with an update sometime after the appointment!

My Pitch for the C3N for CF

I arrive for our clinic appointment around 7:45am. The last time we were here was 3 months ago. Usually, we are ushered back to a room within 15 minutes of arrival. A nurse greets us shortly thereafter to review our medicine list and address any issues that we've been having. Just the other day he was coughing up a storm but seems to have gotten over it. Should I talk to the dietician about his diet again? His weight is up and his stool seem "normal" but I'm always anxious about his lack of interest in food. I wonder if an RT is available to talk a little bit about his airway clearance. I don't want to be a bother but I sure do think the airway clearance he had in the hospital this summer was more effective. I wish I knew what they were doing differently!  

Next up is the doctor. She asks how things have been going. Today? Great! Last week? I was a little worried, but the cough he had seemed to go away. There was that one day that his stool was a little weird but that got better too, not sure what caused it. And frankly I can't remember back farther than that. She checks him out and wants to review his labs since we are at his anniversary visit. His vitamin D is low, his breathing still sounds a little noisy, and a note that she has from his ENT seems to indicate that he may need another sinus surgery . She feels out whether I'd be open to a bronchoscopy at the same time. Maybe another CT is a better option.  When I talked to the ENT last month it sounded like things were going well? I guess I don't mind if they do a bronchoscopy while he's under for something else, but I remember huge discussion with other CF parents on the Facebook about CT scans and all the negative effects of radiation and I don't know that I want to do that? I wish I could find that conversation! I ask questions about a game plan for if he needs IV antibiotics if we grow pseudomonas again, having read online about all different methods used for eradication but not knowing which is best and why. It's hypothetical at the moment because they haven't even swabbed him yet. I just have sort of a busy life and sometimes having a plan provides a sense of comfort for me. She suggests an action plan and I am mostly on board, except for the azithromycin because I saw a presentation somewhere that seemed to show compelling evidence against its use. I wish I could find that presentation to show her! I trust his doctor and want to follow her recommendations, but I have some reservations. I don't think either of us has time to get into this as I've already taken up more than my fair share of appointment time. We agree to wait for the results of the culture to decide a course of action.

Next up is the dietician. Lucky for me, he has a "weird poop" while we are there so she can look at it and provide her thoughts. This isn't what all of his stool looks like. I don't really know how often his stool looks like this - sometimes once a day, sometimes 4 times a day, sometimes once a week. We talk for a bit about enzyme dosing and the calories he's taking in, her offering suggestions for beefing up his intake and me adding them to the "notes" section in my iPhone, hoping I can get them down elsewhere before one of my kids deletes them by accident.

The social worker pops in to address some questions about starting preschool that I had mentioned to the nurse 2 hours ago. I had met with the Psychologist about a year ago to talk about his lack of interest in food, but never followed up, mostly because of both time constraints and cost. If only she could pop in and give me a couple of quick suggestions. But I have to go, we've already been here for almost 4 hours. While waiting for my discharge paperwork, a research assistant shows up to see if I'd be interested in participating in a study about I'm not sure what because my 3yr old is clambering to get out of that office. I agree anyway, get my discharge paperwork and fly out the door. 

This summer, we were visiting family in Philadelphia and my son got sick. He was admitted to Children's Hospital of Philadelphia for a pulmonary exacerbation. Not a single morsel of information about him and his history with Cystic Fibrosis was available to the doctors at CHOP. Many phone calls were made between fellows from my personal cell phone to understand his medical history and doctors asked me the same questions day after day before we trusted and understood each other and hashed out what was going on in the days leading to our admission. I know that it was in Drew's best interest that we discuss everything about him ad nauseum, but it blew my mind that in this age of technology, there was no electronic medical health record that the doctors in Philadelphia could access to understand Drew to provide the best care for him quickly. 

When Drew was a baby, we kept a diary of his formula intake and the corresponding output. We set up a nice little Excel chart and shared that with our dietician on a very regular basis. I credit that chart for his gained weight and reaching the 50th percentile by the time he was 6 months old and he has been able to maintain that to today when he is almost 3. I know that all patients aren't equal, but wouldn't it be great if that data, if our "patient reported outcomes" were cataloged somewhere so that we could share what we did and how it worked for us? Sure, I could take to the CF Mom's Facebook page and ask about what high calorie snacks work for their 3 year olds and hope that some of those moms are online and eager to provide some feedback. But once that question is asked and answered, it just simply vanishes into cyberspace. I cannot tell you how many times I've said, "I know I've seen that somewhere". Image the power of cataloging that data, those conversations, those findings. It can be used by patients, by doctors, by researchers and teachers. Just thinking about the power of that is what is driving me to stay involved and insist on nothing less. 

I think you're seeing my point, a point that was brought out in almost every session I sat through at the NACFC this year. The information that we currently have on patients in between visits is limited. Filling those gaps would give us a more complete picture of health. It would help doctors to intervene at appropriate times, times when patients might not call because in their mind "its just not that bad yet", but doctors are able to identify a problem or a pattern that lets them know the direction something that's "not that bad" is headed. They would be able to more easily determine if certain therapies were actually making a difference through the combine use of passive behavioral and active patient reported data, looking at a real-time view of what is going on with a patient, not what they remember to tell you when they are in clinic. The registry could be enhanced by identifying day to day trends and commonalities in patients sharing mutations. And all of this data can be used by researchers and scientists to figure out every last detail this disease and find therapies that work for every one of us. From a parent's perspective, the C3N is what we need to make life easier and improve outcomes while we wait for our cure. 

Introducing a C3N for Cystic Fibrosis!!

Friday was the culmination of many months of work to present the C3N to the Cystic Fibrosis Foundation...and they loved it! The meetings went even better than expected and we're going to work on getting a pilot set up to begin implementing this learning health system into the Cystic Fibrosis community. It's really amazing and I can't wait to share more about it.

Things have been going pretty well here. Drew has had a cough for about a month now, but the first culture we did two weeks ago came back negative so we had just planned to take a closer look at it during his anniversary visit coming up next week. There are some asthma markers that the docs can look for in his labs, and we thought maybe he has some component of asthma or perhaps its just this dry weather giving him this nagging cough. Well the cough kind of picked up a little bit last week, and after a few days of extra treatments and frankly just trying to wait it out, I wanted someone to see him. We took him in on Thursday and his sats were fine and his lungs sounded clear, but the doc agreed that the cough had just been going on for a little to long. We decided to put him on an antibiotic and do another culture. We started the antibiotic on Friday and by Saturday the cough was all but gone. The culture results came back this morning and he has a little staph but nothing terribly concerning, and nothing that the antibiotic won't take care of. So that's good news! Also good news is that the pseudomonas that he grew this summer has yet to resurface, meaning that we get the stamp of "eradicated" since its been 6 months. Hopefully its gone forever. If not, at least its gone for now :)

I'm in the process of signing the kids up for school for next year. I really can't believe that I'm going to have only 1 baby at home 3 days a week next year. Ella will be in full day kindergarten and the twins are going 3 afternoons a week. The anxiety that I'm already experiencing about sending Drew to preschool and relinquishing control is intense, and school doesn't even start until the week after LABOR DAY!! That's 7 months from now. I don't know how much info I should put on the sheet where they allow you to list everything from medical conditions to social concerns. I feel like I should be keeping it brief and then speaking directly to his teachers and other team members at the school to get ready for his arrival. I did have the opportunity to meet the 3yr old preschool teacher at an Open House and I fell in love. Just a genuine and sweet woman and I think that she will do her best to keep Drew safe in her class. If this is how I feel now, I don't want to imagine how I will feel come September.

We're heading back to PA in a couple of weeks before we go to the March on the Hill and the Volunteer Leadership Conference. I do love getting out of town every once in a while, and I'm definitely looking forward to both the Advocacy piece as well as the conference. There are so many amazing CF volunteers, truly the heart of the organization, and I'm anxious to meet with some of them to learn more about how they do all that they do and how I can get more involved. I am not looking forward to as many hours in the car as we will spend over the course of a week. Hopefully it will be worth it :)

Over the next couple of days I'm going to work on getting some of our fundraising information together. We currently have a book fundraiser underway Usborn Books (amazing book company) and I will be able to share a link where 25% of your purchase will go to our Great Strides team. I am hoping to have a Tupperware one organized again soon as well. If anyone out there in cyberspace reading this has and great ideas for fundraisers or is able to help in some capacity, I'm definitely interested in talking to you!

That's all I've got for this very cold Feb. afternoon. More to come as it breaks!