Where do I even start

Well, we got another cold...or something like a cold. On about Tuesday of last week Drew started with this new cough. He had no runny nose, no other signs on being sick, just this strange new cough. I called his doctor who said that she would see him on Thursday. On Thursday morning, I called again and said that I didn't really think he was sick so I wasn't sure what she was going to be able to do for him, he just had this terrible cough and seemed to be working a little harder to breathe. They said that they were fine with waiting until Tuesday (today) to see him as we already had an appointment scheduled for a checkup. They gave us a new nebulizer med called Atrovent to try to open his airways a little to help with coughing up whatever he had going on.

Well, as it always goes, the next day he was worse. He was almost completely voiceless (as in hoarse, lost his voice) and coughing up a storm both while awake and while sleeping. I called again and they said to try the Atrovent for a few days as it should really help. Saturday came and he was worse yet; struggling to breathe and coughing nonstop. It was so sad to hear his little cry without a voice - screaming at the top of his lungs but not a sound coming out. He was coughing so hard on Saturday that he threw up a couple of his bottles. By about 8pm he was working so hard to breathe that I called the Pulmonologist on call and we decided he needed to go to the ER.

The rushed him right back and put him on oxygen. He oxygen was low but not alarmingly low. They did a chest and abdomen x-ray which showed nothing. They concluded that this was just a virus (read: cold) that was being made to sound worse than it is because of all the abnormalities with his large airways (trachea). They decided to give him some steroids to try to relax and open the airways which might help to calm the cough and ease the breathing, and they sent us home.

They did ask 100 times if I was comfortable with the decision to discharge him, and I was, I guess. The reason I was okay with it is because there isn't a whole lot they could do for him in the hospital other than observe him. A virus can't be killed with antibiotics. They can't give him any breathing treatments in the hospital different than I'm giving him at home. They just don't know what to do for this poor kid who was practically turning blue gasping for air on Saturday night. Imagine breathing through a coffee straw...all the time. That's Drew. Now imagine it being bent or compressed in some fashion. Yes, you can see where this is going.

He seemed a little better on Sunday, at least the breathing part. The cough is still atrocious but at least he was able to breathe. Hes not sleeping well because hes coughing so much. He actually slept with us last night because I was worried about him. He had a CF appointment this morning and they told us to come prepared to have him admitted. UGH!!! The doctors all took a listen and his lungs do not sound clogged. This has been the same since day 1, which is what led them to the whole airway issue. You see, we see the CF doctor because, obviously, he has CF, but thus far the CF has not been the problem. It certainly complicates the problem, but its not the problem. His trachea abnormalities are the problem. The compression of his aorta on his trachea is a problem. The cartilage rings around his trachea that are misshaped are a problem. This is whats causing all of the coughing and breathing trouble, not the CF. The CF just causing the mucus and secretions to be abnormally thick making them extra hard to clear. When you or I have a cold, we cough and the phlegm comes up and we spit it out or do whatever you do with gross phlegm. Drew can't get it up. Its just to thick (from the CF), and his small, compressed airways aren't allowing wide enough openings for it to come up. Trapped mucus in his lungs = breeding ground for bacteria = really bad news in the CF world. The silver lining is that there isn't (or hasn't been) much mucus, or bad mucus anyway. With all thats going on, he really hasn't been terribly affected by the CF, at least not yet anyway.

So we talked with the doctor. We talked and talked and talked. The best doctors from around the world have seen Drew's bronchoscopy and seen his abnormal airways and no one really knows what to do. They are reasonably confident that this current cough is just another trachea related issue and not a horrible CF infection. The first abnormality, the aorta compressing the trachea, can be fixed. There is a surgery that isn't uncommon that can repair that, lifting the artery off of the trachea thus allowing it to be open to its full, round, normal size. If that were the only issue he had going on, it would be a no-brainer to go in there and fix that. But its not the only issue. When he takes a deep breath in, instead of everything expanding, parts expand while other contract, causing the airways to not be nearly as open as they need to be. No one has ever seen this before. No one has seen super small airways like his and frankly no one can believe that he breathes and acts like he does, given whats going on inside. And they don't know how to fix it. Conversations have gone something like this: "Well we could try this and see if that helps" or "We could maybe try something like this", all conversations that I'm glad are being had, but at the same time wish they were going differently. I wish there was an answer, an easy way to fix this, even if it were a surgical answer.

They think that fixing the aorta compression issue will help with his coughing and breathing, but they just aren't sure if thats the main problem. It won't hurt at all to do that surgery, they just don't know if it will help the way that we need it to help. They think that this new, terrible cough that he has is his bodys way of telling us that something needs to happen. I think the way we're (docs and us) are leaning is to do the surgery to move the aorta, and while they have him open, really observe whats going on with the lower airways and decide what, if any, intervention is necessary. This unfortunately may mean more than 1 more surgery for the little man. The ideas of putting a stent in his airways to open it up was one thing that was thrown out. A trache tube was another, with a sort of custom tube long enough to reach down into his lower airways and hold them open until they get strong enough to open appropriately on their own. I don't want him to have a trache.

They aren't terribly settled or completely comfortable with moving forward with any of these options just yet. They want to keep talking and truly figure out what is the best thing for Drew. That said, they are nervous about the upcoming cold and flu season and how it will affect him. Hes getting (I hope, as long as insurance isn't a huge pain in my a$$) a special monthly vaccine shot that will protect him from RSV which would be devastating for him to get, but that will only protect against RSV. We still have colds and flu's to worry about. He'll get a flu shot for sure, and there will be incessant hand washing and sanitizing going on here. But kids get sick. Its just so so bad if he does. I hope its not always the case. I hope we get this figured out and can go on leading our lives, dealing with CF (until new meds are available or a cure is found). But right now things just aren't good.

Please please please use your best judgement when coming to my house and if you have or have been around someone with a cold, please don't come. Please wash your hand when you come in, regardless of whether or not you'll be toughing the babies, and please don't touch Drew without first using sanitizer. Ella is starting preschool in a week and will inevitably bring home germs. I do understand that. I will do everything in my power to keep her healthy, but it do know that she will get sick. Just becasue my kid might bring germs into my house doesn't mean I can afford to have your kid bring them in as well. But please don't be scared of me either. I want friends (I need friends) and so does Ella. Please don't take offense to this. I'm just doing what I need to do to protect my family. I'm trying so hard to find balance. I'm just doing my best.

The doctor today prescribed some inhaled steroids that he will take once or twice a day until we get this airway issue resolved. Steroids have worked the past few times we had to give them to him. Here's hoping it makes things better this time until we get a better plan in place.

A Few CF Fundraisers

The end of Cincinnati's Finest Young Professionals Fundraiser is near and the candidates could still use your support. The Finest Finale is on September 10 at the Redmoor in Mt. Lookout Square in Cincinnati. Tickets are on sale for $65 - includes 2 drink tickets, valet parking, by-the-bite foods, live music from Stays in Vegas, silent auction, raffle & more. Price goes up to $75 if you purchase at the door- if there area any tickets remaining!

There is a wine and beer tasting event called Let it Breathe on Thursday, September 16th at 6:30pm(I'm going!). The Cystic Fibrosis Foundation Let It Breathe event gathers over 250 wine and beer lovers of all experience levels! This casual, fun atmosphere offers guests an opportunity to SIP and SAVOR superb wines and local microbrews alongside delicious food pairings prepared by the Greater Cincinnati Independents (GCI) local chefs and restaurants. Bid on a variety of silent auction items and mingle with friends and fellow foodies in a lively social networking atmosphere.

Finally, these bracelets will only be available for a limited time. They were created as a part of the Finest Young Professionals fundraiser and are not yet available through the Cystic Fibrosis Foundation. Please help to support the CFF and purchase your bracelet today!

8:57

That's what time I went to bed last night. Kids went to bed. I got a shower. Then I went to sleep.

This was my first week without a mom in town to help me and I think it went exceptionally well! All kids are still alive and the house is still standing. I consider that a success. It was a lot of work and I was exhausted by Friday (hence the 8:57 bedtime) but I think we're gonna be just fine. Now, my house probably won't win "cleanest house on the block" anytime soon, but I'm okay with that.

Drew's got something new going on. I don't know what it is, but naturally, it worries me. He got this new, deep cough on like Tuesday or Wednesday. I called the doc and they decided to try him on a new medicine called Atrovent to see if it would help to open his airways and help him clear whatever is causing this new cough. So we tried it for a few days and now he has completely lost his voice. Its so sad to hear when he cries, this very faint little squeak coming from him. I called the Pulmonary doctor on call at the hospital to talk to him about it. He said that it sounds like hes got some swelling thats causing the hoarseness, probably from all the coughing. He doesn't think that the Atrovent would cause hoarseness but he said if it wasn't helping that we should discontinue. We gave him some Motrin to try to help with the inflammation/swelling and are supposed to call back tomorrow and give an update. He just seems to be breathing a little heavy and struggling to catch his breath. Its hard to watch a baby in distress. But his demeanor remains calm and happy. So we shall see.

Ella is scheduled to get her cast off on Friday! They will take it off and xray to see if its healed (please dear God let it be healed!). If shes better then we are done. If not, we get a new cast for 4 more weeks. Unfortunately she can't start preschool until she can walk so I'm really really hoping that shes better.

Drew and Lily are 5.5mo and are starting to sit. I love this age because they do really cute, fun things but they still can't move. Baby food starts soon. I'm going to make it myself this time for 2 reasons. The first is that Drew will need higher calorie than they sell in the store, so rather than buying that and modifying it I can just beef up the calorie content of the stuff I make. And second is that it will be cheaper. One healthy baby alone costs a lot of money. Two is just unbelievable; the number of diapers, the canisters of formula, the extra everything (high chair, carseat, etc, etc, etc) and pile on top of that copays and medications. Sheesh!

Hopefully this new cough turns out to be nothing but I'm not optimistic. I just wish we could catch a break (not literally!) and have a healthy baby boy.

Still No Computer

Its been about 2 weeks now since our computer bit it and I'm about to lose my mind! I am a total computer junkie and surfing from my phone only does so much. Its also near impossible (though there have been a few posts written on the Droid ) to blog with my mini touchscreen keyboard. Typos abound and options are limited. I don't have anything terribly worthwhile to blog about, but with the option gone its all I want to do. Hopefully we will be getting it back either today or tomorrow, so until then...

A few random ramblings

There's a website that a friend of mine has used for a while now that I just recently started to utilize and its called ebates and if you shop online then its totally for you.

Ebates is a website that rewards you with cash-back for shopping through their website. Its kind of like credit card points, only its cash. There is really no catch (honest!). There are over 1200 reputable retailers (Gap, Babies-R-Us, Diapers.com, Home Depot, some airlines, etc etc etc). If you shop online, you simply go to ebates.com and search for the retailer you are looking for. For example, the other day I wanted to buy diapers. I went to ebates.com and I looked for Diapers.com on ebates site and once I found it its listed that they offer up to 5% cash back for shopping through their site. So from the ebates site I clicked on the link for Diapers.com and it took me to diapers.com where I made my purchase. A percentage of my purchase is then credited back to my ebates account automatically. Every 4 months I get a check in the mail for the total of my rewards. It is that simple.

If you buy wedding or shower gifts online, you can do it through ebates. If you buy airline tickets, you can do it through ebates. If you buy diapers, you can do it through ebates. Pretty much all major retailers are listed on ebates and you get cash back for shopping them. And the best part is there is absolutely no work from you necessary. If you shop online, you simply start at ebates.com and the rest is automatic. They don't take your credit card info. They don't charge you anything. They are simply a vehicle for shopping your favorite stores and they pay you for using them. Its real, its legit, and I just wanted to share.

To sign up or just check it out, follow this link- http://www.ebates.com/rf.do?referrerid=mKvKuCJB%2BtTPnv49oCTpTQ%3D%3D - its my referral link. If you sign up and you actually use ebates 1 time to make a purchase, you get $5 put into your account and I get $5 put into mine. Once a quarter you will get a check (like a for real legit check) in the mail. Just saying, if you shop online it really seems silly not to use this site.

Next, I miss my mom.

Next, I seriously cannot believe how fast these kids are growing up. Last night was our first night sans Miracle Blanket (if you don't know about it, you should) and it went surprisingly well. Laid back Drew just stretched out on his back and fell fast asleep. Lily fussed a little bit here and there, mostly when trying to fall asleep, but they slept all night so I consider it a success. Both kids eat rice cereal now - Lily better than Drew. The both roll and are working on sitting. The both talk and talk and talk. They are seriously both absolutely adorable (i'll post some new pix soon - our computer died so I'm using a makeshift).

Ella doesn't look like a baby anymore. Shes getting so tall and is so funny to listen to. She tells us the funniest things. For example, yesterday we went to visit Martin at work for lunch. On the way home, in an attempt to keep her awake, we were talking away. She asked if we could go back and visit daddy sometime. I told her that sometime we could but that daddy is busy at work and I asked her what she thinks he does all day. She thought about it for a minute and then said "Probably draws rainbows all day". I said probably.

Next, I hope this fall I can do some of the things that I've been missing...such as having friends over for dinner or drinks, making it to Gourmet Club, getting back to the east coast for my cousin's wedding and to visit our families and friends back east. I really hope we can do some of this.

Finally, I miss my mom.

That's all that's on my mind right now. More later.

I just haven't met you yet

I'm not a huge John Mayer fan. There's some music I like and some I don't. I was driving home from someplace the other day and everyone was asleep in the car and one of his songs came on the radio - "Half of my hearts got a grip on the situation...". Its really pretty much exactly how I feel. Sometimes I feel like I'm under control, like I'm understanding and accepting and going to be okay with all of this. Then other times I feel like I'm about to spiral out of control. The "why me" and "its not fair" and "I don't understand"'s start flying through my head. I really feel like half of my hearts got a grip on the situation.

This past week I did a lot of waiting. I wasn't sure when or if the doctors were going to call with the plan but I waited and waited. I didn't necessarily sit around, but when I was home I ran to the phone everytime it rang. When I wasn't home I was hoping they wouldn't call because its a nightmare to try to return a call from a doctor. On Thursday the phone rang and it was Dr. Chini (Drew's CF doc). She wanted to let us know they were still talking about Drew but that's as far as they were. Talking about him? Tell me more! Tell me what you're talking about!!! She said that they just didn't know what to do. See, there are a few issues, one of which has never been observed before...ever. Its not good to be the different one. Especially when you've got CF.

The discussion with Dr. Chini continued and she said that there are a number of doctors getting involved. Since they haven't ever seen anything like this before, they are having a tough time deciding what to do and want as many thoughts, ideas, opinions as they can possibly get (fine by me!). The facts are as follows:

• Drew has stridor (a sound babies make when there is floppiness or an obstruction in his airways, but no actual difficulty breathing (although it sure sounds like it).
  
• Drew has CF
• A critical element of CF airway clearance, which is inhibited by his floppy airways

Dr. Chini said that there are few lines of thinking going on. First and foremost from a CF perspective, we need to get those airways working just the way we're supposed to so that his health is maintained. Other pulmonary doctors think that perhaps, as is the case with other kids who have this one problem with the innominate artery compressing the trachea, as they grow and the cartilidge in their trachea gets stronger, it will sort of move the artery on its own, thus solving the problem. The concern with waiting to see is what, if any, lung damage will occur because of the CF and the inability to clear his airways the way he needs to. Third, some ENT docs and a cardiothoracic surgeon wonder if doing the surgery to move that artery will even solve the problem, since the artery isn't the only problem (remember the prior post on his lower trachea moving in unison?) They wonder if that issue, which is the one they've never seen before (like ever) is the cause of or the affect of the innominate artery issue...or if they two are completely independent of one another.

So they're talking. I'm glad they're talking, trying to figure it all out. Its definitely a little scary that they just don't know what to do, but I'm glad they're not just sitting around. The video and pictures of his bronchoscopy as all over the hospital and are "different" (I believe thats the word thats been most used) than anything they've ever seen. Dr. Wood, a world renowned Pulmonologist whose performed more bronchoscopys than any other doctor in the world, has never seen anything like this. Whether its a bad thing for Drew has yet to be determined. As of this far, he hasn't been affected - no breathing issues, no bacteria in his lungs, no trouble. But they just don't know.

I talked to Dr. Chini for over half an hour. I asked her a billion questions - could this get worse? what does waiting mean? A few answers were comforting. She said that she doesn't know if this could get worse. The innominant artery part of the problem (again, read prior post for the scoop on that) doesn't usually get worse as kids get bigger, it tends to heal itself in about 75% of the cases. The other 25% have it fixed surgically and the problem is solved. The real puzzle and therefore concern is the funny motion of the lower trachea (parts contract while others expand all at the same time - not normal, never been seen before). And shes not sure if they can expect that to get better, get worse, or stay the same and not affect Drew. Waiting, meaning no surgery right now, simply means more trips to see Dr. Chini for regular, probably monthly, tests and cultures to make sure that the sound is still just a sound and not nasty bacteria festering in his lungs. It means more bronchoscopys to look down his throat and see if anything has changed.

I'm okay with the waiting option...I guess. I mean I wouldn't want to see him have to go through another surgery if this is something that he could potentially grow out of. At the same time, if waiting means life with "floppy" airways and ineffective airways clearance and potentially irreversible lung damage, then I don't want to wait. But what if we do the surgery and it doesn't fix anything? And what if we wait and he gets worse? Half of my hearts got a grip on the situation.

Dr. Chini said that shes sorry they didn't have a better answer for me or a plan in place yet, but to know that all of the moving parts were working together and I would be the first to know of any discussions or decisions or what have you. So Friday morning around 7:45 the phone rings and its a nurse calling to let us know that they have scheduled Drew for another bronchoscopy at the end of October, like the 25th I think. I guess they were able to talk and decided that in the short term, waiting and re-scoping him would be the best option! To take a look about 3 months after the last scope and see if anything had changed. I guess we will see if things have improved or remained somewhat the same, and hopefully haven't gotten any worse, and take it from there. My hope is that they have improved, even if just marginally, and we can continue to wait another three months, and then another 3 months. As much as I hate the tests they have to do to him, if we can spare him a major surgery then I'm all for it.

This may all sound like such a jumbled, disorganized mess. You might wonder why we can't get an easy answer to this, that or the other thing. And I wonder the same thing. But I'm not frustrated with the doctors or the hospital. I'm confident that they are working on it, that they are doing their best, that they are doing everything they can. They aren't just doctors, they really do care. Maybe with other problems or issues they are just doctors writing out a prescription or putting on a cast and handing out an order to come see them again in a few weeks. But with CF I have found that they have such a deep concern for and dedication to the search for the cure. There just aren't that many of people with CF. The clinic in Cincinnati only has 200 patients. There are only about 30,000 in the US. They are doing everything they can to ensure his quality of life, both now and in 50 years. They do care about him and they want to make the right decision as much as I want them to make the right decision. It just means having some patience, a virtue that I was never blessed with, but I'm working on it.

What I'm frustrated with is CF. I'm frustrated with people asking well why not this, or asking if i've thought about that or the other thing. I shouldn't be. I know people just want to know. And I would probably be frustrated too if no one ever asked. I so appreciate the notes I've gotten from people letting me know how much they appreciate my blog, or "stalk" my blog or admire me for what I've been dealing with. I do really appreciate it. Its good to know that someone is reading this all, that someone wants to know how I'm feeling or whats been going on. But forgive me if I don't respond. I do find it easier to post my feelings on here out in the open than to talk about them verbally.

I'm optimistic. I woke up this morning, like most mornings, and was happy to see my 3 smiling kids. I know a cure will be found. Its right around the corner, we're just waiting. And while we wait we continue to do what we have to do. I enjoy the 30 minute sessions several times a day that we get to hold Drew to do his treatments. Hes so mellow and its nice to just sit with him and hold him. I do it for him.

I'm so pessimistic. I worry everyday that hes going to get sick and that I won't have him forever. I wonder how I would possibly go on without him, or without any of my kids. They are my world. I get mad that all of these other issues keep arising. I get mad at insurance and I hate the fight. But I would gladly take the fight over not having him.

I feel like I just haven't met him yet. I feel like poor Drew is just waiting to emerge and all of these stupid problems keep popping up and I just haven't met him yet. No one has met him yet! He hasn't been out much, except to the hospital one to many times where he melts the hearts of all the young nurses every time they seem him. I wonder when we will meet him. I hope soon. I hope he will be "normal" just like your kids. There's another song, this one by Michael Buble, called "I just haven't met you yet" (can you tell I'm listening to Pandora this morning?). It probably wasn't written to be understood the way I listen to it, but I like it and it goes like this:

I'm not surprised, not everything lasts. I've broken my heart so many times I've stopped keeping track. Talk myself in, I talk myself out. I get all worked up and I let myself down. I tried so very hard not to lose it. I came up with a million excuses. I thought I'd thought of every possibility. And I know someday it will all turn out. You'll make me work so we can work to work it out. And I promise you kid that I give so much more than I get. I just haven't met you yet.
I might have to wait. I'll never give up. I guess its half timing and the other half's luck. Wherever you are, whenever its right, you'll come out of nowhere and into my life. And I know that we can be so amazing. And baby you're love is gonna change me. And now I can see every possibility and somehow I know it will all work out. You'll make me work so we can work to work it out. And I promise you kid that I give so much more than I get. I just haven't met you yet...

Things Could Be Worse

A few weeks ago, my dad took one of our cars home and left his here (they'd been putting a TON of miles on their car driving back and forth all the time). While he had it, he cleaned it! And while cleaning it, he found under the seat a little book (maybe 2x4 inches) that on the front says "To help you keep looking on the bright side, here's a little book of some things that could be worse...". He had figured that Martin had given it to me, or me to him, but neither of us has ANY idea where it came from. Its a little worn, appearing as though it'd been there for a while. But seriously, where did it come from? And could it have fallen upon us at a more perfect time? Its simply designed to make you laugh a little but its cute. Here's some of the "things that could be worse"...

• Your butt could fall off (not sure why I found that so funny but I laughed out loud)
  
• You could permanently itch
• You could have no friends, no bladder control and no sense of humor

It was a nice little gift from wherever it came from and it came at just the right time. The stress of Drew's impending procedure is weighing on me. I'm tired, just really really tired. Tired of waiting. Tired of worrying. Tired of wondering when life is going to be normal for him. I'm hopeful that it will come. I'm hopeful that once all of these other stupid little problems are fixed he will be just the normal little boy we hope for him to be. I try to be optimistic, but its hard when it seems all you hear is bad news all the time. I just hope we can get this all sorted out.

My mom is leaving on Friday. I (and Martin sincerely agrees) would move her in permanently if I could. Ella adores her and she has been so wonderful to have around all summer. Ella breaking her leg kept her around for an extra week, but shes got to go back to school next Wednesday so we've kept her as long as we could. I wish we lived closer. I wish we lived closer to both families. Their help and support has been invaluable over the past 5 months. I've written about this before as its such a struggle between being so close to such world-class care for Drew and being so far away from family. I'm hoping that we can get this nonsense with Drew under control enough that we can move at some point. The reality is that we will go and do whatever we have to do, wherever we have to, for as long as we have to, to keep our kids as healthy and happy as possible.

I asked Ella what she wanted to do with Gaga before she left and she said make cookies, go to the zoo, get ice cream, play with guys, play play-doh (and pretty much everything else that we've done all summer long). She has no idea what it means that Gaga is leaving. I'm pretty sure she thinks she lives here. When Martin's mom was here, she thought she lived upstairs and just came down to do dishes. Its funny how kids perceive things. She's going to miss her though, that I know. I am too.

I'm off to do a few things with my freedom and free, trustworthy babysitter for these last few days before she leaves (doctor appointments, haircuts, quick trips and errands). After Friday and until this cast comes off of Ella's leg, the 4 of us will be stuck in the house until daddy gets home from work. I had bought a Joovy stroller for 3 a couple of weeks ago but hated it so much (it weighed 1000lbs and was impossible to maneuver) so I sold it...the day before Ella broke her leg. So I have no practical way to transport all 3, so until Sept.3 (day the cast comes off and hopefully stays off), you can come visit me, but you probably won't catch me out and about (not that I've been out and about for the past several months!).

Things could be worse, just remember things could be worse :)

Pictoral

I'm going to attempt to describe through pictures the issue we're currently dealing with. First, here is a picture of the procedure that was done on Tuesday. They stuck a tube down Drew's throat and took pictures the whole way down through his trachea (windpipe) and into his lungs to see what the problem was. This is how they discovered the problem.


Your trachea is more or less round. Its a long tube leading from your throat all the way down into your lungs. Below is the shape of a normal trachea. If you pictures it upright, the rounded part (blue) faces out toward your chest behind your sternum and the flat part (brown) faces your back.
The picture below is an actual image taken during a bronchoscopy of a compressed trachea. So if you use the image above to visualize, the picture below is the same orientation. The arrows point to the back of the trachea (the brown part in the picture above, the part that faces your back). The black opening is the airway, and you can see its not round, its sort of squished. The shadow around the bottom of the picture is the area being compressed by something and making the airway the shape that it is rather than being open and round. Remember, in this picture you're looking down into the airway, so you're standing in front of someone looking straight down their throat. The bottom part of the picture is the front of the airway near the chest. That black hole should be round, not oval. If you can picture the shadowed area below the black hole being black as well, that would represent a normal trachea without the compression.

Drew's is even more closed. Frankly, they said they aren't sure how he's breathing the way he is given the condition of his trachea.So the final picture I found is of the whole anatomy of the chest. It shows how the arteries and trachea wave around one another. The esophagus is closer to your back and the trachea is closer to your front. The right common carotid artery wraps around the front of the trachea, and in Drew's case its a little too close and therefore pressing down on the trachea causing the compression. What the surgery he needs entails is lifting that artery off of the trachea and attaching it to the sternum with a stitch to hold it off of the trachea.


The other issues he has of the whole trachea not moving in unison when he inhales or exhales is a whole new can of worms. They aren't sure whats causing that and if it poses a serious problem. The plan is to take care of the compressed trachea and when hes open to take a closer look at the weird rhythm of the trachea and the "weird lungs". Hopefully they are not an issue, and this surgery will fix his sound and allow him to clear his airways more effectively.

Ask me questions if you have them. If I don't know the answer I will probably want to learn it so I'll find out. Hope this helps to clear things up some!

Here's the Scoop

I'll start by saying that this post is just going to be a high level overview of what's been going on. I'm hoping to find some time this weekend to sit down and find pictures and links and thoroughly explain what is happening. But for now, just the basics.

Drew got a cold about 2mo ago. When his cough and froggy voice persisted, he was put on oral antibiotics as the thinking was that he managed to grow some bacteria that needed to be eradicated. Several days later and no better, we were admitted to the hospital for IV antibiotics. Still sounding the same after another week, it was concluded that something other than bacteria was causing this sound. That was good from a CF perspective, but raised some other concerns as to what the problem is.

On Tuesday, we were back at Childrens for a Bronchoscopy and Laryngoscopy (english: they stick tiny cameras down his throat and into his lungs to take some pictures). The were thinking they would discover that he simply had floppy airways (malacia) but found something very different. First, toward the top of his trachea or airway, they noticed that something seemed to be compressing it. Its supposed to be a big round opening and Drews is squished near flat in one area. Once you pass that spot it opens back to to normal size. However, the behavior of the trachea was un like anything they had ever seen. Normally when we breathe in our airways expand and when we breathe out our aIrways contract, in unison. Portions of Drews trachea open up when he breathes in but other portions compress.

They decided to admit us based on their findings and try to further investigatge to bigger issue of what is compressing his trachea. They did a CT scan and found that the aortic artery crosses his trachea a little low and is compressing. Its not an uncommon thing, but it does require surgery to fix. A chest surgeon will open him up, lift the artery off of his airway, and attach it with a stitch to his sternum. Whiles he's open, a pulmonologist and an ENT will be present to watch him breathe and see if they can determine the cause of or the solution for the second issue. (I know its hard to understand which is why I will do another post with pictures and all).

So what does this all mean for Drew? It means he's got shitty shitty luck. This in no way has anything to do with the CF but because of the CF its a big problem. These problems are prohibiting him from effectively clearing his airways, a critical element of staying healthy for CFers. The aortic valve problem isn't totally uncommon, and many times its not an issue that needs surgical intervention. However, hecause of the CF it needs to be fixed.

I requested that the doctors and surgeons and everyone from every specialty involved get together and talk about this so we are sure this is the best option. They are also going to help facilitate a second opinion. We should hear from them next week or so with the game plan, but its looking like somethig they would like to see happen in the next couple weeks.

It sucks, I'm sad and worried, but we need to do what's best.

More to come.

(no comment)

I don't think I made a ton of sense in my ramblings yesterday. A lot more has changed. I don't have the energy to write about it tonight but I will soon, and hopefully with more clarity. He needs surgery again.

What we have here is a failure to communicate

We saw and ENT Specialist and 2 CF doctors before we decided/agreed to the procedure that Drew is having done today. We asked all 3 of them, as well as the folks who called to pre-register us and our nurse who called on Friday to confirm everything if we would have to stay overnight. The answer was a unanimous no. They why, you may ask, are we staying overnight? Apparently it was overlooked that he isn't 60 weeks old (born at 36 + 20 wks since he's 5mo.) and anesthesia needs to monitor him overnight. I wouldn't care about staying here had we planned for it! We have no meds, no food, no clothes, nothing. We were planning on being home before dinner. So my mom has Lily and Ella with her broken leg, I'm here at the hospital, and Martin is at work. He'll come here after work and ill go home, returning tomorrow to sit with him. My mom will bring the girls over around lunchtime as I have to take Ella to get her cast on at 1. Hopefully that will go quick because Drew has a CT scan scheduled for 3:30...

Which brings me to how today went. Not as planned. They knocked him out and looked down his throat to see his trachea and his bronchi (lower airways/tiny lungs) and its not really malacia. They said that it looks like maybe there is an artery compressing his trachea which would likely need to be surgically addressed. They're doing the CT scan to see if they can determine what's causing the compression since its not malacia like they had suspected. Then they looked into the lungs and the doctor said they looked different, like "nothing he had ever seen before". He said that he's been doing this for 30 yrs and had never seen lungs that looked like Drew's look. It wasn't terribly concerning in the near term, as his oxygen levels don't seem to be affected by this. However this doctor didn't know how it would affect his ability to clear his airways, a critical element of stayig health with cystic fibrosis. Keeping the airways clear of mucus and thus bacteria is the best thing for CF. With him being so small and so far unaffected by any bacteria, its hard to say whether or not his abnormal lungs could be causing any harm. There wasn't really any conclusion on that conversation. Just that he was going to have to talk it over with some colleagues and figure out what they wanted to do.

I've got a couple of thoughts or feelings on all of this. First, this sucks. I wanted normal, common malacia. I don't want it to be an artery pressing on his airways, or anything pressing on his airways that would need surgical intervention. Hasn't the poor kid been through enough? I'm scared of what they're going to find or what they might have to do. Absolutely none of this has gone according to plan. I'm scared that the doctor hasn't ever seen lungs like his before. What does that mean? For now? For his future? Why is this all happening?????

I'm selfishly thinking about how I'm going to do this by myself. Martin has to work. Our families have things of their own going on. They can't stay with us forever. I know they will be here as much as they can, they already have! I need to learn how to have 3 kids. But what if he has to go back into the hospital? I guess we will all just sit in here with him. We can pack a bag of toys and movies and all hang out here for some time. By that's not fair to Lily or Ella. But its not fair to Drew to not have me here. I can't move back to Philadelphia when Cincinnati Childrens Hospital has the number 1 CF Center in the country and perhaps the #1 pediatric respiratory team in the world (second to London).

I guess we will see what the CT scan shows and take it from there. We will get a second opinion and maybe even a third. It will be a pain in my ass, having to either find someone to watch the kids or packing them all up and bringing them along , but its his life and we owe him everything we've got. I wish I could put all of his doctors (CF and all other pulmonary docs) into a room together and listen to them debate it and then make a decision. I understand that there are different opinions and potentially different options but I just want to understand and see different perspectives and make our decision.

This is so not how I thought things would go. No humor here today, just a worried mom with a heavy heart.

The Tips

A few days ago, I wrote about how I wanted people to ask me to questions...and I got no questions. I did get a nice note from a woman named Heather whose life was also affected by CF. She has 2 sibling with CF - a sister who passed away at age 26 and a brother who is still alive at 48. Heather wrote a book to honor her sister. I haven't read it but I'd like to. She said that it will help others to understand. I don't know if anyone will really ever understand, but anything that would help to add perspective for others is worth a shot. If you're interested in taking a look you can visit Sixty Five Roses: a Sisters Memoir

Another thing that Heather shared with me was 15 Tips that her mother, a mother of 4, 2 of which had CF, put together to share with other families dealing with a disability or an illness. I read it and I felt justified in my feelings. I don't like them all, but I feel them all. I don't like that our lives are so different than they were just 4 short months ago, but I wouldn't trade it. Its just taking some getting use to. If it weren't hard enough dealing with this all - the emotions, the stress, the exhaustion, the doctors, etc, etc, etc, - its hard to see how our friends and relatives take it and I wonder and worry what they think and how this all affects them and us and our relationships. Its hard.

Here are the 15 tips:

These tips are based on the experience of Doug and Donna Summerhayes, founders of the Canadian Cystic Fibrosis Foundation and parents of four children, two with Cystic Fibrosis. Their daughter Pam died from CF at age 26. They are still involved in caring for their son Jeff, who at 48 is one of the oldest Cystics in North America. Their well son Gregg is a police officer and married father of four. Their daughter Heather is the author of “Sixtyfive Roses: A Sister’s Memoir,” which tells the story of how this family fought for their children’s lives.

· Share "important" information and understanding regarding care programs with your little family and your extended family and close friends.

· Do not try to satisfy the extended families "standards" or expectations. You must do what is right for your family and not what extended family or friends expect. Remember that you will never "be the same as everyone else," so don't try.

· Remember that you are husband and wife first. How the marriage is nurtured will preserve your union and reflect on the care for both the disabled child and your well children.

· Consciously develop your sense of humour.

· Make sure to get adequate exercise and rest so that you don't develop your own health problems. Try not to over schedule yourself. Organize some care relief if possible, even if it’s just an hour or so a couple of days a week, so you can nap or take a walk.

· Remember that you may not be able to do everything alone. Swallow your pride and ask for help. Make your specific needs known. Graciously accept the help others are willing or able to provide, remembering that everyone has their limitations. When help arrives, don’t spend all your time and energy hovering or directing them – go do what you need to do!

· Encourage your disabled child to do as much as he/she is able, just as you would your well children. Dwell in possibility. Include them in all family functions and outings.

· Set aside special, individual time for your well children – even if it’s just a once a month parent/child date. Make that date sacrosanct, and if it has to be cancelled for a medical emergency, make it up asap. By the way, cleaning the garage together is not a date. Let the housework suffer and create some fun instead.

· Just as you may sometimes suffer resentment and guilt about your disabled child, remember that your well children feel the same. This is natural. Acknowledge and validate those feelings, and work with your well children to find appropriate outlets for them.

· Get family counselling. Think of it this way: if you’ve never gone mountain climbing, you wouldn’t just go to Mount Everest and start up - you would seek instruction. Each stage ofraising a child with a disability is something you’ve never done before. Take some “mountain climbing” lessons. Counselling will also give you a safe place and space to voice your biggest fear, your deepest anger, and your most profound grief.

· When you feel overwhelmed with the demands of treatments, care, housework, cleaning, outside commitments and pressures, STOP (Sit, Think, Observe, Plan) and regroup. This may take several days but it will pay off in clearer thinking and happiness.

· Recognize that you can’t control life by being afraid of it. So often we make our decisions from a place of fear. Fear separates you from your abilities. It does not protect you, nor will it alter an outcome. Instead, focus on your power to choose your response to any given situation – you can choose faith over fear, hope over despair, and allow yourself moments of joy even in the midst of sorrow.

· One of the good things about dealing with illness or disability is that it forces you to prioritize. That being said, don’t burden yourself with a lot of “shoulds” that you will just end up feeling guilty about. Know your limitations, and focus on your strengths. Take it one day, one hour, or one minute at a time.

· Make a plan for your disabled child to be cared for in the event of your own illness, injury or death. Don’t just leave it to the well-siblings.

· Make forgiveness a practice in your home – starting with yourselves.

Big Day Tomorrow...and the next day

Drew has his bronchoscopy tomorrow. We need to be at the hospital by 9 for the procedure at 10. They will knock him out and intubate. I'm just so ready for a break. For some weeks or months without a trip to the doctor or a hospital stay. We're hoping that hes got malacia. We will know when the surgery is over whether or not thats what it is. If its not malacia then there is some other underlying problem likely somehow related to the CF that is causing him make the atrocious sound he makes (imagine having a terrible cold and a giant frog in your throat....an not being able to clear it. Thats what he sounds like). I'm not going to speculate. I'm just hoping for malacia. I'll update tomorrow.

In other news, Ella is being a champ with her broken leg. We get the cast on Wednesday. I'll be happy once thats on and the poor little leg is secure in a hard place, and the fear of knocking it into a gate or bumping it when shes sitting in the high chair (it sticks mostly straight out when shes sitting) is lessened. She has her moments of frustration, as any 2 yr old whose lost all of her freedom to roam would. I only have experience with 1 two year old, but she never really sat and played with one thing or in one spot for any real length of time, maybe 15min max. So now, the situation is pretty much the same, only when shes done in the spot shes in someone has to move her. And when her snack or baby doll or whatever falls off of the couch she calls for help. Or when shes coloring and drops a marker, she calls for help. Its taxing! I can't be mad at her though. Its not her fault. I joined Netflix to get some new movies to watch (we saw 15min. of Finding Nemo today). I'm just hoping when we see the Orthopedic doc to get the cast on Wednesday he tells us that its only a couple more weeks. Lord, if its gonna be 6mo. I might have 2 more on the move before she gets the cast off!

I Could Write a Book

Ella broke her leg. Yup! Non-weight bearing, foot to hip cast for 4-6 weeks. What's more fun than 2 babies who can't walk? Three! Especially when one of them is a two year old.

It was a simple freak accident. She was at the splash park and slipped and fell down and screamed bloody murder. I really thought she had just twisted it, but after a few hours and her unwillingness to put any weight on it, we decided a trip to urgent care was probably our best option. Poor girl was so tired and in so much pain. We had to restrain her for some x-rays but as soon as they took the first of 4 and then told us they didn't need any more, we knew.

They called it a "Toddler Fracture" and said its incredibly common. Toddlers bones aren't as hard as adults, and the doctor said that this happens to tons of kids, typically from jumping off a couch or a bed or something...or apparently slipping at the splash park. They put her in a splint for a day or two until the swelling goes down, then they will replace it with a cast. It will, in all likelihood, be one that can't get wet, though they did say there's a chance. It was a "clean" break so there's no need for manipulation to get the bone back in place - its already in place, just needs to grow back together.

Martin and I had decided yesterday morning that we were going to go out for dinner since we hadn't been since the twins were born, and my mom is only here for another week. That plan seemed unlikely to materialize as we sat in urgent care. I have to give props to the Urgent Care facility, as we arrived just after 6:30 and were back home by 8. So we got Ella all setlled and put to bed, did Drew's breathing treatment and got him into bed, and we decided that we were still going to go out. We only made it to a near-by chain (Outback), but we had some beers and some food and a good laugh about our life before we came home and crashed.

Ella woke up at quarter of 6 this morning. She can't walk. She can't jump out of bed to get a book to read until we come to get her. She can't get her toys to play with. We're gonna spend a lot of time watching movies or just sitting in one spot and playing. The nurse asked, before we left, if we had any questions and I said "What the hell do you do with a 2yr old who can't move for 6 weeks and she said you get creative. I went in to see her and she said "Ella can't move. Ella fell down and leg is broken". Poor thing. (and yes, she always talks in the 3rd person).

You just can't make this stuff up. It seems that once we finally have things under control, we take 2 steps back. I must have done something really terrible at some point in my life that I can't remember that I'm now paying for. Oh well, I love it all and wouldn't trade it for the world. We just have to keep moving forward.

Q&A

Over the past several weeks I've come to realize how little everyone knows or understand about what we've got going on. Heck, I would be clueless to if we weren't in the middle of it! Just questions that we've been asked or assumptions on different things have we have seen made makes me realize how very little is truly understood. And I want people to understand. I want people to know what we're dealing with. I want people to realize that I'm [usually] not being an asshole, i'm just living and managing my life the way I need to now.

So I want to do a blog post to simply answer questions. Nothing is off limits. My email address is e.keeleymoore@gmail.com. Email your questions and I will post them all along with answers. Ask me whatever you don't understand, whatever you want to know - about how we do something, how we feel about something, how we manage something - and I will answer them. I won't put names down so no one will know whose asking. I'll simply post the question and the answer. I know you've got them, so go ahead and ask. I won't judge, I promise. I'll just answer :)

Exhale

I can finally exhale. The crazy weekend is over and we're back to reality. We had Ella's second birthday this weekend [even though her birthday was a month ago] and the twins baptism as well. About 25 family members from out of town were here for the festivities, along with about 25 friends for the birthday party. I feel like I hardly got to visit with anyone! There was so much planning and organizing and managing and worrying that I feel like the weekend just slipped by. I think its fair to say that good time was had by all. And frankly I think I did a great job, on a number of fronts! :)

I was a nervous wreck going into the weekend. Having that many people at our house threw me into a tizzy of worry about Drew. The biggest crowd he'd been around since birth was about 6 people, and now we were multiplying that 9 times. We sent out this note to family the week before the party:

As you all know, he was back in the hospital a couple weeks ago and thankfully is doing fine now. That said, it has highlighted how important it is for us to help him from getting sick to the extent possible. Therefore, if you have, or feel you are getting a cold, please let us know. We have masks that you can wear if necessary, and you'll need to keep your distance (doctors recommend ~6 feet) from Drew. I know that's sort of weird, but it is truly an important aspect of his long-term health. Also, please understand that we're going to avoid passing him around, particularly during the birthday party. Kids are dirty and there are going to be a lot of little ones running around touching things and each other. Other times throughout the weekend, you'll be welcome to hold him and play with him as you wish, but we'll require that you clean and sanitize your hands first - by necessity this is now our standard operating procedure. Please don't be offended if we repeatedly ask you if you have or if you will wash your hands. While it's sort of become second nature to us, it takes some getting used to to remember that anytime you are going to touch him, you need to sanitize first so we'll only be doing it to remind you. Rest assured, he is now a very stout kid who loves to be held and played with and there are no real restrictions on any of that!

I'm glad we sent it. People were great about washing and sanitizing before touching him, but I felt like all weekend I was a nervous wreck if he wasn't in my sight (he was for most of it). But there was still a lot of passing around and it all still made me nervous. I tried to act "normal" but in reality I wanted to keep him all to myself, guarded and protected (maybe I should get a tatoo like crazy Casey of a heart covered with a shield "i'm here to guard and protect your heart" HA!). I'm just struggling so much with that right now. I want to treat him like we treated Ella when she was that age! I want to show him (them) off and let people hold him and love him, but at the same time the fears I have of him getting sick are so overwhelming I feel like I'm gonna go crazy.

You just don't understand. You can't possibly understand unless you have a child who takes one step backwards every time he gets sick. Getting a cold for most kids means a runny nose, maybe being a little cranky. Getting a cold for Drew means a trip to the hospital. It means mental, physical, emotional, financial strain for us. It means less time with our whole family together as we are split spending our time between Ella & Lily at home and Drew in the hospital. It means the beginnings of chronic lung disease. It means CF is winning.

After the parties, we (Meme and Grandpa and Gaga) Clorox wiped every square inch of the playroom (and there are a LOT of toys in the playroom). We wiped down doorknobs and spigots and phones and remotes and tables and chairs. We disinfected everything. We have to. I'm not naive, I know colds and other illnesses are in our future, but we've spent 6 weeks of the last 4 months in and out of the hospital, and to the extent possible, I'm going to prevent another stay. And I still feel guilty for not protecting him better. Hes not sick...yet. But I feel like I could have (should have) done a better job. And at the same time I thought I did a good job letting go a little and letting our families hold him.

I just don't know how I'm feeling. I'm all over the map. It's just different now. Things are just going to be different. And I have to stop caring what other people think. I have to stop worrying about being judged for the way I handle this, that or the other thing. You can judge me when you walk a mile in my shoes. No one has said anything to me at all, I just feel like sometimes I'm being judged and I hate it. Things are just different now.