Liam's Exciting Pulmonology Update

Liam went to a routine pulmonology appointment yesterday, and I'll admit that I had high hopes going into it.   Can I just say that every single thing I'd hoped for AND MORE was met at this appointment?!?  It was amazing.  Brief, but wonderful all the same.

First of all, the biggest news. Liam has been declared free of his NEHI symptoms!!!!  He may develop asthma later on down the line as is common with NEHI kids, but I can deal with that. I'm a pro at asthma by now.  He also still has a little trouble coming out of anesthesia, but he goes inpatient for surgeries, so that will be fine as well.  The doctor and I had fully expected him to make many, many visits to doctors and hospitals throughout the winter, but he only went three times all year. One of those times was a follow-up to his sleep study.  I can totally handle that.  We are now down to just once a year to say hello unless something happens and he gets really sick.

I decided to take a risk and talk to the pulmonologist about Liam's cochlear implant surgery. It's been a royal pain getting it all set up, but it looks like we're finally almost there.  The ENT wanted to do one ear now and go back in later for the other ear because each surgery is three hours and he was afraid to have him under for a full six. The recovery is easier with just doing both at once, and it's what I really wanted so I asked the pulmonologist what he thought. He said that the ENT gets the final say because he has to feel comfortable doing it. BUT, he also said he'd call the ENT and recommend doing both ears at once.  This means Liam gets to be free of O2, free of constant doctor appointments, AND has a fighting chance at finding his voice and succeeding in the hearing world.

Practically Perfect Pulmonology Appointment Today

Liam saw the amazing Dr. Rivera-Sanchez today for his scheduled appointment. You have to understand that we LOVE this doctor for him, and really respect her opinion. I give her a hard time, I think, because I am comfortable taking care of him at home when she would sometimes prefer me to let him be admitted for a night to be sure he's doing okay. As much trouble as I give her, I do love what she does for Liam and Drezden. I love that she listens and really seems interested our needs as a family. As much as we love her, we wouldn't mind it if our sweet boys got to a point where they got to see her for things like Christmas get-togethers or in passing at the grocery store.

Today, we got one step closer to that wish. She was SO IMPRESSED with Liam's progress. His retractions are minimal. His room air sats are normal!!! His cough is almost non-existent. His stamina has increased. By all counts, he appears to be a typical 14 month old baby. We still have a few small things we need to work on, but it's all managable. We are so delighted with his progress as is she. (Now we just need to get her happy with Drezden's progress..and we WILL make that happen).

Byron was asking me last week why it is that we have so many of these challenges in our lives. I think it's because the Lord is blessing us with the opportunity to show Him we can and will endure to the end making our best effort and with high hopes and hopefully minimal complaining. Now, we are being blessed with miracles for our efforts. Liam is pretty ahead of the game for NEHI children. Most of them are around 2 years old before they start considering weening from the oxygen. Did I mention that he's 14 months old and only occasionally uses daytime oxygen?

Yeah. It was a practically perfect pulmonology appointment today.

Gotta' Love a Happy Update!

Liam has had quite a week. I'm pleased to say it's been a much better week than last week, too!! First, we visited Dr. Sanchez. Liam is officially off all inhaled medicine except for PRN. Yay, Liam!!

I also discussed the fact that it is a NIGHTMARE keeping the O2 on him. It's like the little stinker stays up late at night thinking of creative ways to take it off or something. The other day, I checked his sats and noticed that he was at 97% on room air. That's very good, so I gave him a break. I did this about hourly throughout the day. He spent EIGHT HOURS off his oxygen, and maintained his sats at 97% or better. Good job, Liam!! As I said, we discussed this with Dr. Sanchez and came up with a compromise. Liam can be without his oxygen on as long as we're home and I'm checking his sats very regularly. They have to be at 94% or better, and he can stay off the canula. The second it dips, or if he's sleeping, the canula needs to be on. I think that's a fair compromise.

We also had a very productive appointment with Ms. Linda. She had Liam practice tracking sounds, and he did pretty great despite being tired. The problem he has is leaving his aids in at all. We get maybe thirty seconds at a time if we're very lucky. Otherwise, we have to put things on his arms to keep him from being able to reach the aids and pull them out. We're trying to work on a plan for dealing with it, but so far, Liam is winning the battle.

While we were with Ms. Linda, I mentioned his eating issues so we worked on some of those. We now have a plan to help him learn to work through having food in his mouth. He is very clearly a hungry little monkey, so this will be a great turn of events.

And, the final piece of great news? Dr. K called last night. His testing shows that he no longer has any C-Diff in his system!!!

Yucky Adhesive

I'm pleased to announce that Little Guy has been home for three full days now, and his sats have remained where they should be. I couldn't be happier about that.

One little problem we keep running into is the adhesive that holds his canula in place. If I use stuff that is really subtle, he pulls it right off and it tears up his skin horribly. The poor kid just bleeds and bleeds. You can see the sore spots on his face in the pictures. The stuff that is more gentle on his skin is just plain ugly. It's just big and clunky and yellowy-looking.

I've included pictures so you can see what I'm talking about. Without adhesive, he takes off his oxygen right away. Surely there is a solution out there somewhere. And, yes. I do realize his canula is a little bit low in these pics. That is yet another battle we fight daily.

Tomorrow, we will go with Ms. Kristi to conquor the challenge of setting his hearing aids to the maximum benefit. It's much easier to do with regular ABR testing. But, since he can't have the sedation for the test, we don't really know how far his hearing loss has progressed.

Such is life, I guess. Two steps forward and one step back. I can't complain. I think I'd be bored any other way.

Home Again

We finally got home yesterday. Just four days in the hospital this time. I'm relieved. I think. Little Guy had some blood work done that was "moderately abnormal" and may indicate some pulmonary hypertension. But, the echocardiogram is okay for now.

We stayed a couple more days after that to try and observe and understand why his sats go lowers. We really have no answers. It does look like maybe he's developing some asthma. It wouldn't be a huge shock since two of his brothers have asthma. But it would be a big frustration. Poor kid.

Sooooo, we are home. And we're thrilled about that. We are cautiously optimistic that all is well. In other very exciting news, all of our kids except Number One got their h1n1 vax's yesterday. Number One has to resolve his egg allergy before he can get the shot, so we're waiting for his blood test to come back.

Ahhhh, the joys of parenthood.

Another Weekend in the Hospital

I went to pick up Little Guy the other day after he'd been playing on the floor. His face looked dirty, so I tried washing it. Nope. Not dirty. Just gray. Yikes! I had the King check all of his tubing to be sure everything was hooked up correctly, and then had him bring me the oximeter. 84%!!! Not so good. We increased his oxygen, and that didn't help so we increased it some more. Finally we got his sats into a normal range and waited for the oncall dr. to call us back.

The doctor who returned our call was a total jerk. No seriously. He was just mean. He told me he had no idea what NEHI was, but that I had to bring Little Guy to him to be seen. I asked him to please consult with a doctor who did know what it was. Nope. He refused. What!??! You have no idea what my kid has, refuse to consult someone who does, but you want me to bring him to you???? I think not, my friend.

I got a hold of our regular doctor the next day just as he was starting to look a little bit gray again. She insisted that we go in right away to the ER and had them ready and waiting for us. Really, she wanted him to come by ambulance, but I just wasn't having it. I knew we'd be sitting I had oxygen with me and that I could get there quickly, so I took him myself. I don't think she liked that I did it that way, but it was what I felt was best. Imagine that! A mom thinking for herself a little bit about what is best for her child. Novel idea, right?

Anyway, we got right in and they observed him for a while. Of course is sats looked great while we were there. That's what always happens, isn't it? They admitted him for observation which made me cry for some reason. I think I was just overwhelmed and thinking it would be like last time when we were there for a full month. Plus, the doctor I understood to be on call was the one I'd spoken with the night before who was a pompass jerk and too full of himself to ask for help. WHY would I leave my baby with him??!?

As it turned out, an hour before he was admitted the shifts changed and the partner to our regular doctor came on call. She spoke to me personally as I tearfully explained my objection to letting him be admitted. Swine Flu all over the hospital for starters, but she told me she'd put him on the pulmonology unit which was much safer. I also told her how I felt about him being with some doctor who might not know what to do. Remember the one who wanted to send him home with me knowing CPR and nothing else? Yeah. She was a gem. What if I got someone like her again. Again, the doctor promised me it would be she herself who would be treating him.

With those promises in place, I consented to let him stay. We only wound up staying about 24 hours, so I made it home in time to trick or treat with my kids. and Little Guy got to show off his Superman costume. Can't complain about that, right?

So, we are home now. He has a new nurse who comes daily to check his vitals and a nurse who will be coming twice a week for eight hours at a time. I think the goal is for him to be sort of homebound now. We'll see how it works, and how long we get to be home for.

We've increased his oxygen to a full liter, and it's still a little bit iffy. *sigh* We shall see...

Lung Biopsy Results are In!

We got lucky, and this was the BEST case scenario we could ask for. This is awesome. We know to expect that he'll be admitted a few times throughout the high cold/flu/respiratory illness months. I can deal with that. We also know he's going to have a low threshold for work that may require added pulmonary support, like running, learning to walk, roll over, etc. But, we can work on helping him work on all those things. Not so bad, right?

The name of the disorder he has is NEHI (neuroendocrine cell hyperplasia in infancy). This is REALLY rare, so it's not 100% surety that he'll outgrow, but most of the known cases of it have indeed outgrown it. So, the odds are very much in Little Guy's favor. That works for me. I consider myself very blessed to know there is a light at the end of this tunnel for him.

We still have to work on the obstructive sleep apnea, which he may also outgrow. He's have a sleep study in September, and we'll go from there. No matter how you slice and dice it, though, he doesn't have the inoperable brain tumor they thought he had, he doesn't have cystic fibrosis, this isn't going to kill him, and more than like he won't have to live with this for the rest of his life. So, yeah. We are pleased. We will embrace what we do have to work with and be grateful for what it is...and what it isn't.