Just some things...about Holland

There is a documentary on TV tomorrow night about a girl with Cystic Fibrosis who is planning her wedding while waiting for a new set of lungs. It airs at 10pm on TLC tomorrow, 2/29. Here's a link to the article about the documentary.

I had a Tupperware fundraiser in February that raised $618.75 for our team this year. Shout out to all who participated, because that is a totally awesome profit!

I'm still pregnant.

We had a little birthday party for Drew & Lily this past weekend. They don't turn 2 until March 14th, but I think I'm going to be either pretty busy or very pregnant then so I had it a couple of weeks early. It was so great to have our friends and family here celebrate these two little wonders in my life, each high maintenance in their own way. You often think of twins as two peas in a pod, but outside of being from the same "pod", these two couldn't be more different. I must say how freaking adorable they both are, and how they both make me smile and laugh all of the time. They definitely know how to work together to make me crazy, but I couldn't love them more. And I can't believe they're already 2!

I've been working on my presentation for CF Education Day at Children's on March 17th. I'm hoping that the baby gets here before then so that I can go. I have so much to say, I seriously could fill up a half an hour but I've been given 5 minutes. I decided to focus my speech on the importance of advocacy. I had written something that I was originally going to read, but then I got the presentation from the National Advocacy Leadership Conference and decided to take a few slides from that and speak about advocacy and what I've done and why its important rather than just reading my thoughts. So I'm combining the two and need to work on it a little bit so that it gets people interested. I have only 5 minutes to get people interested so I'd better come up with something powerful.

I found a story that another CF mom had shared on her Facebook wall a week or so ago and I really loved it so I want to share it on here as well. And that will be all I have to say for this evening!

Welcome To Holland
by
Emily Perl Kingsley

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It’s just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

From a restaurant

Tomorrow we are having a little birthday party for the twins. I cannot believe its been two years! I seriously didn't think we would survive. Or they would survive. Or something. And yet we are all in our routine bopping along, mostly happy, incredibly healthy.

Drew had his surgery a week ago, and it was a long Friday, that's for sure. He didn't wake up from anesthesia well, he didn't want to be anywhere but on my shoulder, and you could just tell he was in pain. Fast forward to Saturday morning, when outside of a little bloody nose, you would have no clue he'd had surgery the day before.

In addition to his sinus surgery, he had a bronhoscopy. I mentioned in my previous post how excited everyone was about how clean and healthy his airways looked. I still was prepared for a call this past week letting us know that the culture they took was growing something that would require IV antibiotics. That call, my friends, never came! Instead, I got both a call from the nurse and an email from his doctor, ecstatic that his culture grew nothing. Not even a little staph. He was completely and totally clean, and this all in the middle of the germiest month of the year! We've had dozens of cultures taken and have never ever had one showing no bacteria. His lungs look and sound great, and he's currently bacteria free!

So the party will go on as planned tomorrow. I dropped Ella off at school this morning with the intention of running to CF Foundation to drop something off before heading to Costco, only to
realize once I left that I had left the things for the CF Foundation on my counter. And Costco doesn't open until 10 so I had an hour to kill. I went and had breakfast by myself. I remember the days of seeing folks alone at a restaurant and feeling bad that they didn't have anyone to dine with. I take that back. It was a glorious (and delicious) hour of peace and quiet. I drank my coffee and ate my French toast. I didn't have to wipe any hands or butts or settle any disputes. Just me, at a table, wishing the serenity could last all day. I gotta do this more often!

I ordered our Great Strides T-shirts last night. They will be on sale for $5 with 100% going to the CF Foundation thanks to a generous sponsor picking up the bill. You'll have to wait to see them, but the quote on the back this year is a good one: Stop being afraid of what could go wrong and think of all that could go right!




- Posted using BlogPress from my iPhone

Post Surgery Update

Drew had surgery yesterday on his sinuses and a bronchoscopy to check the state of his lungs. Thankfully, we were cleared on Thursday by his pulmonologist and were ready to go for Friday. Most of the time when I have to schedule a procedure that would require anesthesia, I do it for the first available appointment in the morning because the kid can't eat anything for like 6 hours prior to the procedure. Yesterdays appointment was at 2pm. He couldn't have anything but clear liquids after 5am. He had apple juice until 9:30 and then had to fast until the surgery. He actually did remarkably well, not crying or complaining once about wanting something to eat or drink.

Things were right on schedule with them taking him back at just before 2. The plan was for Pulmonary to do his bronchoscopy and get a good look at his lungs and take a sample of the mucus from down there to test for bacteria, and then the Ear, Nose & Throat (ENT) docs to put him on a ventilator and do his sinus surgery. The bronch only takes 15-30 mins, so shortly after we got back into the surgery waiting room they called us back to talk to the doctors. The first thing he said when he walked into the room was "I don't know what you've been doing with that boy, but I am astounded at how good his lungs look!". WINNING!! He said that never in a million years would he have guessed, based on the way his lungs looked a year ago at his last bronch, that he would have no mucus and big, clear, open airways. The tracheomalacia that we've been dealing with since birth has mostly gone away, and the crazy abnormalities that they've never seen before (coming from a man whose done over 40,000 bronchoscopies) were starting to wide. He said they still look weird, but whatever their shape, they seem to be working for him just fine. This was such awesome news for us to hear. We're managing to keep him healthy! They did "rinse" his lungs so that they could get a good sample to test for bacteria. While we haven't cultured pseudomonas for over 6mo, I'm prepared to hear that he's got it again. I'm starting to believe that it doesn't ever really go away, but more is kept at bay for significant periods of time by the IV antibiotics we use. Either way, as long as its not causing problems for him, I'm okay with it.

So we went back to surgery waiting room and about an hour later got called back into the consult room to talk to the ENT doctor. He said that he had removed a significant number of polyps from his nose, and then cleared out his sinuses which were compacted with puss and mucus. I have pictures of all of this, but I'll spare you. He said this is something that Drew will have to have done on a pretty regular basis, based on the significance of his sinus disease. We're hoping that the drug that will cure him of his CF will remove all of the funk thats clogging his sinuses and we won't have to go through this every year. I guess we'll see.

After we talked to the doc we got to go back and see Drew. That poor thing was so out of it and in so much pain I was sure they were going to keep us overnight. It was already 5:00 at this point and I just didn't see him overcoming this in enough time to get home for the night. He needed a little oxygen which he was waking up, but some tylenol and mommy's shoulder helped to calm him down. He just had a glazed look about him. He didn't know who we were or where he was. He could hardly open his eyes. We got him to take a few sips of juice but he was just so out of it. He fell asleep for about a hour and when he woke up he was breathing a lot better. He still wasn't with it at all, but since his vitals all looked good and his pain seemed to be managed, they said we could go if we felt comfortable. So we brought him home! He laid on my lap from about 7-10pm, then earned himself a place in bed next to me. He pretty much slept all night long, not needing tylenol again. At about 7:45, he woke up, asked for dada, and walked himself downstairs. He played, ate breakfast, did all of his breathing treatments and hasn't looked back. We gave him a little tylenol late this morning when he seemed a little bit uncomfortable again. Outside of a gunky, bloody nose that leaks from time to time and a slightly swollen face, he seems fine. Little kids are remarkable in how resilient they are.

Thanks to everyone for the thoughts, prayers and well wishes. Hopefully in just a few days when the wounds heal, Drew will be able to smell again and his appetite will return and he will be good as new! And if you want to make a donation to the cause, you can click on THIS LINK. The sooner a cure is found, the better his chances are of not having to go through this again!

Let's work together

Its been another crazy week here in the Moore house! We still have lots of coughing and runny noses from all 3 kids. All are now on antibiotics with Lily's bronchitis diagnosis today. Hopefully we will be sleeping all night again soon because mama needs some rest.

Drew's sinus surgery is scheduled for next Friday. I had planned a little birthday party for the twins for next Saturday before we knew when the surgery was going to be, and then had to move the party. I just don't know how great he's going to be feeling the morning after his surgery. I hope he's already feeling better but I didn't want to roll the dice and have a house full of people if he just wanted to lay around. The ENT doctor called me yesterday to tell me that anesthesia said they wouldn't do his surgery given his recent lung infection. I about had the baby at that very moment. He's got CF! He ALWAYS has a lung infection! He had an increased cough and is on antibiotics for some staph that they found on his culture, and guess what - he's sounding much better. Anesthesia said they wanted to wait until after March 5 to schedule the surgery and I said that just straight up wasn't going to work. I assured the woman that he's had far more complicated procedures done under far worse circumstances, and if I had to put him under myself, we were having this done next Friday. To some of you I may sound totally unreasonable, and being 35 weeks pregnant automatically makes me a little unreasonable, but seriously, this poor kid has had a chronic stuffy/runny nose since about October. He can't taste, he doesn't want to eat anything, and the junk that's been running out of his nose for so long has his poor nose raw. I want him to get better. Waiting longer means staying away from other kids or other public places where he could pick up more germs because another cold means the surgery would be pushed back even longer. I'm not being totally selfish in wanting this done before the new baby comes. I just want him better. Honestly and truly.

So the ENT nurse said that she would talk to anesthesia and call me back today. I hadn't heard back from her and around 2 I decided to call. She hadn't heard any updates from anyway so I took matters into my own hands. I called his pulmonary nurse who contacted his doctor, who immediately called anesthesia to get this all sorted out. Anesthesia agree to keep the surgery as scheduled if pulmonary would see him the day before and clear him for the procedure, since they know him best. Of course there is always a chance that he's sick or doesn't sound great and they don't want to do it, and I'm perfectly okay with that.

I hate the social limitations of CF. I hate having to ask everyone that comes to my house if they are healthy. I hate being stuck in the house for big parts of the cold and flu season. I hate passing on birthday parties because I can't control whose going to be there and don't know what kind of germs we could pick up. I hate wiping down my 3yr old before she gets into the car after preschool. I hate having Clorox wipes in my diaper bag and cleaning off high chairs and tables and anything else within reach when we go out to eat. I really do try to stay as "normal" as possible, participating in as much as we can and just being careful to wash hands and wipe off what we need to, but there is that fear in the back of my head that trying to be "normal" socially will land us with some funky bacteria and more trouble than its worth. And not more trouble as in a pain in the ass for me, but more trouble as in a serious lung infection and time in the hospital and decreased lung function and all of that stuff. I hate that we can't plan anything, or if we do plan stuff we have to be ready to cancel. I hate the disappointment that that brings to everyone, self included.

Switching gears just a bit, I'm a little disappointed about the way my fundraising has started this year. Last year, once the emails were sent and the video was posted, donations came flying in and walkers were signing up left and right. This year it all seems to have come to a screeching halt. People are always asking me what they can do to help and this is what you can do. You can sign up to walk with us. You can help us to raise awareness and money. I know fundraising can be awkward and uncomfortable. Share our video and our story. Here is the link to an inspiring story about a father who fund raises for his son who lost his battle with CF when he was 10. Another mom whose blog I read has grown her team in the past two years since she lost her 7 year old. Another mother posted the eulogy she read at her son's funeral when he passed away in January. He was 2. I know the statistics say that Drew has a 50% chance of living until he's 37, but precious children continue to lose this battle everyday. The thought of not doing all that I can to save him pains me. If we don't already have a team in the city you live in, you can create one for us! You can share our story and spread awareness and help us to raise the money we need to cure Drew. So many people I've talked to say that they don't have time. I don't have time not to. I will do all of the work for you. I will give you a letter to send out. I will share our video with you to post on Facebook. I will give you suggestions for different fundraisers you can hold. I will help you if you will help me.

Donate now by clicking here!

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Theres an App for That

Sickness has plagued our family. It started last weekend with me getting a sinus infection. It had me down and out for most of Saturday and Sunday, but by Monday we were back in business. Monday afternoon Drew developed a fever and started coughing quite a bit more than normal. By Wednesday, Ella had a fever as high as 103 at times, and wasn't feeling well. We missed dance on Thursday and school on Friday. Drew saw the doctor on Friday and got some antibiotics for a sinus infection and a steroid to reduce some of the inflammation that was causing some of his coughing. Both Ella & Drew's fevers would go down with Tylenol, but they were just hanging on for days and days. Saturday morning, Lily woke up barking like a seal and I knew that a croup diagnosis was in our future. I took Ella & Lily to the doc Saturday morning. The gave Lily a steroid for croup, and thought that since Ella's fever wasn't getting much above 100 anymore that she just had a virus that needed to work its way out. Saturday was a rough day, with everyone on some kind of medication and overall just whiny and tired. Saturday night Ella came in around 1:30 am with a fever of 103.5. The doctor had told us that if her fever went back up that we would need to go back in. She had some Tylenol and went back to bed. She woke up this morning with another 103 fever so we called the doc again. This morning when they saw us, they heard some crackling and told us it was pneumonia. Poor girl is so darn sick, just laying around and sleeping and crying inbetween doses of Tylenol, Ibuprofen and an antibiotic. Her fever was really high all day today, and I'm hoping that the two doses of antibiotics she got today will start to fight this and that tomorrow she will start feeling better. I am drained. I'm not convinced we are in the clear yet, but let it be known that I am ready to be.

So when I went to the Pulmonary doc with Drew on Friday, I was talking to his doctor for a few minutes about the approval of Kalydeco. Anyone whose been reading this blog with any frequency knows of my hope for this drug to work for him. Since it was just approved on Jan.31, it was my first opportunity to talk realistically about the possibility of getting him on it. She said that she really didn't expect that it would work for him because his mutations we're "gating" mutations. Wait, what? I thought that R560T was a Class 3 mutation, just like G551D which was tested with Kalydeco and showed remarkable results. She said that she had an app from Vertex Pharmaceuticals that allows you to look up mutations and learn a little bit more about what class they fall into and what kind of problems they cause. HELLO! (The iPhone app is called CF Gene, for anyone who cares). So she pulls up R560T and it shows it as a Class 4 or 5 mutation, just like F508del. This is contrary to everything I've ever read or seen about his R560T mutation, and I told her that. The great thing about our CF doctors is that they want to work with you and learn and grow in their understanding with you. It has never been, for us anyway, a I'm the doctor so I'm right kind of relationship. So she asked me to send her what I had seen about R560T being a class 3 mutation that could benefit from the new drug. I was able to send her several articles, including some from the National Institute of Health and other medical papers from renown research hospitals. She emailed me back today saying this:

There seems to be conflicting information because I see where they call R560T a class 3 mutation and another place where they refer to a gating defect, but I also found an article where they stained the R560T region and did not find the protein on the cell surface. This suggests that it is a class 4 or 5 mutation. I'll check with Dr. Clancy to see if he knows which source is most reliable. I know you will be disappointed if Kalydeco does not work for Drew. I think Kalydeco is just the tip of the iceburg. We are getting closer to having drugs for other mutations and Kalydeco's success will help push research for other drugs along.

We didn't know if Kalydeco would actually work for Drew or not anyway, and she's right that I will be disappointed if it doesn't work for him, but I'm really curious now to see what class his mutation falls into. I hope that the app is wrong and I am right because I like to be right because I'd love to have a "gating" mutation that might benefit from a drug that is now available. If it turns out that its not, the drug that targets Drew's other mutation, VX809, is expected to be available to patients within the next 18mo. So I guess we just have to wait and see. Even if it happens that I am correct and Drew does have a gating mutation, we will have to get into a research study that tests the drug on gating mutations other than the one it was approved for because right now its only available to a small, select group of CF patients. Fingers are crossed :)