Tuesday, August 31, 2010

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.

1 comment:

  1. Hi, Erin! I saw your comment on the CFF facebook site and came to read your blog. I also have a son with CF. He'll be 21 in November and we've also been through some out of the ordinary things. Nothing like what your sweet baby is going through, though. So sorry to read about all this. I would just recommend one thing as a mom who has spent a LOT of time with her kid in the hospital. If he's having trouble breathing and getting blue, I would opt for staying in the hospital where you are surrounded by people who would know what to do if things got drastically worse very quickly. We've almost lost our son a few times and in 2001, the docs didn't think he'd last another year, but here we are 9 years later! We have a different "normal" than most families, he's been on IV meds at home for the past 9 years and he's been in the hospital a LOT! But he's an amazing young man and it's because of the things he's gone through.

    I never could have gone through half of what we've faced if I didn't have a firm faith in God, knowing that He loves this boy even more than I do and He has His hand on him and knows the number of his days. We appreciate the gift of each and every day and never take anything for granted. I will be praying for your family and for the doctors who will be treating your son.

    If you ever need a shoulder or an ear, please don't hesitate to email me at Junes_Chocolate@yahoo.com