Post-Op Day 5 - last update

Today we had a visitor - my friend Kim. She definitely made the day so much better. And her daughters (Katie and Sadie) made these totally awesome get well cards for Harlie. I'm so mad I didn't take a picture today, so I could show you. I will do that tomorrow, though so you can see. Katie just finished up Kindergarten, and she did such a great job on her card. It is so cute!

Unfortunately, it's been another day, and no improvement. And as I left her tonight and watched her breathe, I would have to say that not only is her respiratory status not improving, it is declining.

Not only did they have to raise her settings on CPAP, they couldn't do another trial to get her off. And they said they were actually going to put her on a rate tonight. Which I believe (I don't have any real experience with ventilation as her time on it as always been so brief) means that she's actually being ventilated tonight (the machine will breathe for her). Again, not the direction in which we want to go.

I spoke with the doctor today about her x-rays. He tried to show me that they weren't that bad. And that there was no evidence of fluid in her chest cavity. Of course, I had to tell him that gives me no comfort. Never in all my experience with Harlie - after HUNDREDS of x-rays - has an x-ray actually diagnosed anything. The x-ray might push you in a direction - but it's the x-ray, coupled with other symptoms and further tests, that actually gets us anywhere.

Anyway, while her x-rays aren't that bad, she's getting worse (again, how much can I believe the x-rays?). Here's a quick video I took of her sleeping today. Notice how long her expiratory length is (especially about 20 seconds into it). And this is on the higher settings of the CPAP.



She didn't sleep that well today. But it is hard to sleep when you're working so hard to breathe.

The last time I saw her breathe like this was when she had a chylothorax (fluid in the chest cavity surrounding the lungs making it very hard to breathe). While I'm terrified of that - I would rather it be that than something worse. I know what the treatment is for that. And while lengthy, it is completely recoverable. As long as you know it is there. Thinking about her declining and not knowing what's going on is WAY too scary - and I just don't want to go there. Period.

Oh, I forgot to mention in my last update that they took some respiratory cultures yesterday and it appears to be growing something. So, instead of waiting to see exactly what it is (might take several days) they went on ahead and started her on an IV antibiotic. While she felt very warm to me, I don't think she had a fever today.

I had to help the nurse change her arterial line dressing today. She said it looked terrible and was a breeding ground for an infection. It was not fun. And, just for the record, the smell of blood is NOT pleasant. The nurse we had yesterday said that she heard that the arterial lines can be very painful. They actually cut down into your wrist and, I guess you could say surgically place the line in your artery. Then they stitch it in place to help keep it from coming out. It was yucky and I had to hold down her arm so she wouldn't go flinging about while the nurse was messing with it. Oh, the things I hate doing to my sweet little girl. I just hope that one day she doesn't hate me for it. I often wonder if she's thinking, "why are you letting them do this to me?" And it kills me.

Oh, I saw Harlie's very first nurse - ever - today. When I was wheeled over (in a wheelchair) the afternoon Harlie was born, Sarah was her nurse. She was her nurse that day shift, and the next. And she remembered us, too. It was so good to talk to her and to talk about those first days. Oh, they were so hard. I feel so lucky that we had her to help us during that time. What a difference she made! Thanks Sarah!

I know I sound like a broken record - BUT - hopefully tomorrow will be better. Hopefully we'll start to see things turn around - for good.

And Kim, thank you so much for coming to visit us. I know it's not convenient in any way, shape or form. So, please know you helped a lot today!

I'll update again after rounds in the am!

Thank you,
Christy

Post-Op Day 5 - 1st update

Well, I was hoping I could start off this post with good news. But, she's pretty much the same as yesterday. She remained on CPAP all night long. During rounds they said that they wanted to start some timed trials to see if she can come off today. The goal was to let her breathe completely on her own for four hours. I think she only made it to three hours before they had to put her back on. And she is still working so hard to breathe! It seems like it's harder for her to exhale than to inhale.

Her oxygen saturation levels are low. Consistently lower than yesterday. Right now she is hovering in the mid-60s! Ugh. That is SO low!!! (a normal persons is close to 100). And when she turns on her left side (the side she prefers of course) she desats down to the low 60s - like 61!!! It's starting to kinda freak me out. I'm wondering if they need to turn up the settings on the CPAP so that it does more work for her. I don't know. I think the doc is going to come in and listen to her again because her nurse said she sounds really congested.

Ugh. I am just praying that a chylothorax isn't brewing.

Well, the doc just came in and they are turning up the settings on the CPAP. Not the direction in which we want to go.

She's miserable. And I am pretty convinced that one of the most depressing places ever is a children's hospital on the weekend. Minimal food is available, the halls are deserted and those of us that are here look sad.

Hopefully things will turn around soon and I'll have a better report tonight!

On a side note, Tom is thrilled that the Pittsburgh Penguins won the Stanley Cup last night. As for me, I am thrilled that the whole hockey playoffs (which started in 2007 I believe) is finally over. Geez! Every time I turned around there was a playoff game on that he HAD to go watch.

Oh, and after he watched the game he ended up getting to meet Jerome Bettis and Hines Ward from the Pittsburgh Steelers. All this while I sat here in the lap of luxury at Children's National Medical Center. Ahhh, life is good!

Ahhhh...Post-Op Day 2

She is finally resting comfortably. It was so wonderful to walk in the door and see her like this...



Of course, when they did rounds they said she was resting TOO comfortably. Go figure. They have weaned her from most of the drugs except the new one they gave her last night (which obviously did the trick). The new drug is also called Precedex, which is a lot easier to spell and pronounce than Dexmedetomidine. Whew. Anyway, she can't leave the ICU until she's off the Precedex and she doesn't have an arterial line anymore. If that arterial line comes out, she could bleed WAY too much, so she needs to have a nurse in the room all the time to make sure that doesn't happen. That was why yesterday was so awful for her nurse. She was very concerned that would happen with all her thrashing about.

They've turned off her pacer for now and she seems to be doing okay. They are leaving it hooked up, just in case, but hopefully she won't need it again. I've asked her cardiologist why we don't just hook up the darn thing for once and for all so we don't have to worry about it anymore, but I can't remember what she said. We had that conversation back in April. I guess I'll be asking her that again soon. Oh, and she can't leave the ICU with the pacer hooked up, either.

The team that rounded said that she drained 120 mls of blood in the past 24 hours (30 mls since 7am today). That's over their 100 mls limit, so I thought for sure they would say they need to stay in. But, they said they were going to take them out. I was happy about that. But, then they said they needed to get the surgeon's blessing and he said no. So, the chest tubes will stay in for at least another 24 hours. I'm not really that disappointed because I would rather be absolutely certain that it's safe. The last thing we need is to have to put them back in later!

Plus, her history of a chylothorax is still in the back of my mind. She had it after her last heart surgery when she was six months old. They said that complication is unusual after the Glenn (the procedure she had at that time). And it showed up three weeks post-op (which is also unusual) and caused her to go into respiratory distress (the chest cavity fills up with chyle making it difficult for the lungs to inflate, which makes it difficult to breathe, of course). We just happened to be on the way to the pediatrician at that time and her doc had to call 911 when we got there. It was very scary. And she ended up in the hospital for eight more weeks waiting for things to heal.

Anyway, while they say a chylothorax is unlikely after this particular surgery (DKS), I'm not completely comforted. And it would be nice if they could start her feedings while she still has the chest tubes in to see if they drain milk. Now the last time it took weeks to show up, so I know it wouldn't be that easy. But still, if she has the "world's worst scarring" maybe it would be different this time around.

She hasn't had anything in her belly since 10:30pm on SUNDAY night. And today is Wednesday! They said that we could start to give her some Pedialyte slowly, then gradually go to her normal feeding schedule. But, it takes forever for them to put in the orders and get things moving.

Oh, and she earned 26 more Beads of Courage from Friday to Tuesday! I will try to take a good picture of them when we get back to the Ronald McDonald House tonight, so you can see them. I love the Beads of Courage and am so glad that they started the program here. One day it will be so great to explain them to her and see how she likes them.

Well, that's it for my update for now. I'll update again tonight. Thank you for all your wonderful messages. I know it must be hard to think of something to say, but just knowing that you're thinking of her, and us, makes all the difference. Sometimes when I get overwhelmed with all of her complexities, I want the world to stop. It is hard to think that everyone else goes on about their daily lives. I know that's silly and juvenile (and impossible, duh!) but that's the way it feels to have this kind of life. I don't feel this way when we're home and she's playing. So, even though you can't stop everything, just knowing that she's in your thoughts makes me feel less alone somehow. So, thank you for that.

Surgery Consult

Yesterday, we had our consultation with Dr. Jonas, Harlie's heart surgeon. I'm afraid that there's no way I can explain properly what's going on and what's going to happen. I would need pictures.

Basically, the subaortic stenosis must be corrected, or alleviated in some way. This obstruction is tissue that has grown over the entrance to her aorta and is making it difficult for blood to leave her heart to go to her body.

The cath doc told us that the surgeon would most likely cut away the tissue that was causing the obstruction, and that it could grow back in time.

When we spoke with her surgeon, he had a different plan. Instead of removing the obstruction, he is going to create a whole new path for the blood to leave her heart and get to the aorta to go to her body.

With her main heart defect (cc-TGA), the electric rhythms of her heart can be unstable. And a huge risk with cutting the obstruction away would be that it could damage her electric connection between the ventricles and atrium, making her pacemaker dependant. This we clearly do not want to do. It is one thing to have a pacemaker that helps your heart beat on occasion, another thing entirely to have the pacemaker telling your heart to beat all the time. And it is possible that the obstruction would just grow back, and that would mean another open heart surgery.

So, his first preference to fix the problem is to create a whole new connection from her left ventricle to her aorta (to give the blood another way out). That procedure is called the Damus-Kaye-Stansel procedure or DKS. He will use donated human tissue to make the passage way (thanks to all those wonderful people who donate their organs!) If that isn't available then he will have to use Gore-Tex. Real tissue is so much better because it closes up better around needle holes. Nice thought, huh?

I have to admit that I am not happy about this obstruction and the extra procedure he has to do. And I will be even more unhappy if he can't do the DKS for some reason. I really don't want her to be pacemaker dependant.

While signing the consent form for the surgery, he told us that a normal Fontan operation is a fairly routine surgery (it is one of his most common surgical procedures) but that adding the DKS makes it complicated. He said that the normal risk of brain damage or death is about 3%, but by adding the DKS, and the fact that Harlie has more issues, it is slightly higher than that - closer to 5%. Before Harlie came along, numbers brought me comfort. You have to go with the odds. But, prenatally, Harlie was given a 5% chance of living, and she did - so I don't consider 5% that small anymore.

He did give me a glimmer of hope that maybe she won't develop a chylothorax (like she did after her 2nd heart surgery, the Glenn). If you click on chylothorax, it will take you to one of my blog entries where I explain what it is - just scroll down a ways and you'll see it. Hopefully, relieving the pressure from obstruction below her aorta will lessen the chances of it happening. If it does not happen, then we could potentially be home in 2 weeks!

Stress

I think I'm suffering from pre and post traumatic stress disorders.

Ever since her heart cath on Tuesday - when I knew that her surgery date would be April 16th (barring any unforeseen obstacles) I have started to lose my mind.

Looking back on the last two and a half to three years, we've really been through the ringer. And I am pretty proud of the way we've handled things, all things considered. For the most part, I've managed to stay positive and not let things get me too stressed out. But I gotta admit, I'm stressed. REALLY stressed. And mad. Well, not mad at this exact moment. But I was. Most of last week. I was mad. At everything. And at nothing. But, I'll have to talk about that when I'm mad again. It's hard to talk about being mad when you're not currently mad. Well, at least for me it is. And most of the time I try not to show that side of me on here. But I have a feeling that's going to change in the next few weeks...

Anyway, I'll talk about being stressed. Because right about now, that's a given. And I'm guessing I'm going to stay that way for a while. I'm thinking that if I just accept it as a fact, and not try to fight it or hide it, I'll be better off.

Here's what I'm stressed out about (in no particular order):

1. The long days at the hospital. The fluorescent lights. The people. The food. The restrooms. The beeping. The #$!%$#@$! beeping. The same questions over and over. The same answers over and over.

2. The 3am blood draws (yes, she's sleeping soundly, and then BAM - bright lights right in her eyes) the tourniquet, the holding her down and then the sticks. Oh, the sticks!

3. Chest tubes. Everywhere.

4. Not being able to hold her or comfort her. At least for a while anyway.

5. Restless sleep - for her and for me.

6. Missing my family. Missing a "first" of Cooper's. Missing my friends. Missing my life at home.

7. Not knowing how long we'll be there.

8. Tom's birthday (April 28th). Murphy's birthday (May 11th).

9. The sticks. Did I mention the sticks?

10. Not being able to leave her side - comfortably. Not being able to run (although I'm going to try).

I could go on, but I won't.

Her surgery is on April 16th. I'm guessing the average length of stay for this surgery is about 2 weeks. However, based on Harlie's history, I cannot assume that the average would apply to her.

In order to truly understand why I say I'm suffering from pre and post traumatic stress disorder you have to know a little bit of history:

Her 2nd heart surgery was the Glenn, when she was 6 months old. She was in the hospital for 10 days. She was home for 10 days then went into respiratory distress and 911 was called from her pediatrician's office. She stayed in the hospital for an additional 8 WEEKS. That's a grand total of 3 months!

The reason she went into respiratory distress is because she developed a chylothorax due to the surgery. During surgery, her lymphatic vessels (carries the fat from your diet into your blood stream) were damaged, which caused fat to leak into her chest cavity, making it difficult for her lungs to inflate. A drain tube was placed in her chest to allow the fat to drain out and we put her on a no fat diet and waited for the vessels to scar down, heal, and stop leaking. The healing time is different for every patient, and there is no way to predict how long the healing process will take. It could take anywhere from days to months. Really.

It is my understanding that if a chylothorax developed after the Glenn, there is a good chance that it will develop after the Fontan. During the Fontan, more "work" is done in the area of the lymphatics, increasing the chances of damage.

Since they didn't know she had this complication after the Glenn, we got to bring her home. So, she was here in Richmond for the 8 week hospital stay. However, since they leave the drain tube in to watch for a chylothorax, if it does develop, we will be in DC. So, we will have to see how the team there deals with this complication. And see if we can get her transferred here to MCV while she's healing.

I know it must sound as if I'm being negative. But, I am simply preparing for the worst, and hoping for the best. And I'm stressed. Who wouldn't be?

If I had known at the beginning of her last heart surgery what was in store for us, I never would have believed we could do it. It would have been too overwhelming. But with each new day, there was a new opportunity for the healing to happen that day. Every day, we started out the day by looking at how much drained during the night. Hopeful that it was less than the night before. And it really is a true belief - there have been studies - about children suffering from PTSD after having spent a substantial amount of time in an intensive care unit.

She has no idea what's in store for her. I suppose that's a good thing. But it still makes me hurt inside. I know things will be better for her after this surgery. And I know that miracles happen, and perhaps no complications will occur. But, I am still not looking forward the experience.

Another trip to the ER...

So, on Friday, we go to Harlie's follow-up appointment with her pediatrician. She is now on O2 continuously. When we put her on the scale to weigh her, we have to remove the mask (just for a second). Her color immediately changes. He sees her, doesn't like her color at all (even though her sats are in the 80s). He calls her cardiologist, they say come to the ER.

The plan is to admit her, tweak her meds, see what happens. So, off we go, back to the ER. Bummer.We get there and check in. There is a girl there who looks in the stroller. She says, "oh, she's got a trach?" That's interesting. No one ever says that. We said yes. She tells us that she had one when she was a baby - of course we both look at her neck and notice some scarring - but not from the stoma (hole). She said she was born without an esophagus. Wow. Then she said, "but I'm here!". How great is that?

Well, they take x-rays. Now she has pulmonary edema, which is fluid IN her lungs (whereas the chylothorax was fluid AROUND her lungs). Personally, I'm thrilled. That's understandable, really and an easy fix. A little over a week ago we stopped her diuretics (which helps your body get rid of extra fluid). They were concerned that she was getting dehydrated (because of the C-Diff), which would be very bad for her blood pressure, since her blood flow is passive.

Then, on Wednesday, when we went to the ER, since they were thinking dehydration, they gave her fluids through her IV. The fluids proved too much for her and now we have to put her back on the diuretics. A constant balancing act.

They wanted to give her the meds through an IV, since that works better. But, no such luck. Her veins are spent. They wanted to admit her and watch her for 24-48 hours after starting the meds again. But, I pleaded my case - saying that there is nothing that they will do at the hospital that I can't do at home. I will be able to watch for all the same things and will monitor her respirations, temperature, O2 requirement, etc. So... they let us take her home! YAY!

So, at midnight we get home with a very sleepy baby. I put her in the crib and next thing I know she is dancing! Both her arms are just flapping away, both legs are going crazy and she is smiling and her eyes are bright as day. You can not tell me that she isn't fully aware of what is going on! She was so happy to be home!

Then, she slept through the night just fine. I've given her 2 doses of her meds, and I haven't really noticed an improvement yet. Hopefully, we will soon. I am just so thankful that we aren't at the hospital!

Well, that's it for today. I hope you are all well. Talk to you later!

Take care,
Christy

Chylothorax lesson

Hi everybody,

Well, Harlie is doing much better, I think. She was so active today, moving her arms and legs all over the place. She is such a little charmer, flirting with anyone that gives her a glance. It has been really funny to watch. She has had a lot of repeat nurses and they tell me that they are requesting her - which completely warms my heart!!

They weighed and measured her today. On March 5th – exactly one month ago today, she weighed 11 pounds, 1 ounce at her pre-op appointment. So far, she has been gaining about 1 pound a month. Today she weighed 12 pounds, 1 ounce!!! I am so pleased! She still gained 1 pound, despite the fact that she has spent 3 weeks out of the past month in the hospital AND had major open heart surgery!!!

As far as a medical update goes…I got my lesson on chylothorax (chyle) today. Here goes:

There are long chain, medium chain and short chain fatty acids. The long chain fatty acids travel up your lymphatic system to a vessel near the heart and get dumped into the bloodstream. Most likely, during her heart surgery, that tiny vessel (about the size of a strand of hair) was nicked and damaged. This allowed the fat to leak into her chest cavity instead of into her bloodstream.

What that means is that as her lungs were trying to inflate with her breaths, they were fighting the fluid IN the chest cavity, surrounding her lungs, making breathing more and more difficult. And, since she doesn't have adequate lungs anyway, she has no reserve. Her lungs are fighting an uphill battle.

So, we will give her formula made of medium chain fatty acids (Portagen – special order through a pharmacy).

The medium chain fatty acids are absorbed into the body differently, bypassing that damaged vessel. So, that way the vessel will not leak, giving it time to heal on its own. Hopefully in 3 months, when we re-introduce regular formula, we will see no build up of fluid in her chest cavity and then we will know that it has repaired.

Until then, we watch closely and if too much fluid builds up again, then they will drain it again. If that continues to happen, they will surgically insert a chest tube that has a little suction to it, to help get the fluid out - 24/7. If that happens, we can't leave until they pull the chest tube out.

They are thinking that she has a combination of viral pneumonia and chylothorax. Hopefully now we are well on our way to recovery. Of course, this hospital stay has put a kink in our surgery plans for the future. So, everything will be delayed a bit. They want 6-8 weeks from RECOVERED to her next surgery. And frankly, I’ll need the break, too!

Well, that’s about it for today. Thanks for your support and prayers!!!

Take care,
Christy

Drained the fluid!

Well, I think today was a pretty good day. After the increase in Lasix didn’t help, they decided to pull the fluid out with a needle. It took all day for this to be put into action. This meant that she couldn’t eat all day – but she didn’t seem to notice or mind. I think she was in her best mood since her admittance. She was all smiles to everyone. She has been getting chest x-rays every day and they started to show a pleural effusion. A pleural effusion is an accumulation of fluid between the layers of the membrane that lines the lungs and chest cavity. The main goal was to find out what the fluid was. There were several possibilities. It turned out to be chylothorax (milk).

I only know the basics now, but will learn more tomorrow. It has something to do with having cardiac surgery and the re-introduction of milk afterwards. Something about the fat from the milk leaking from the lymphatic system. Since there were several possibilities, we didn’t go into much detail prior to knowing what it was. Now that we know, we can concentrate on that.

I feel better just knowing – that is so much better than watching everyone scratch their heads. They tried again to get an IV in – but all her limbs are done with that. So, they ended up having to put one in her scalp. Anyway, the amazing thing is that they pulled 93 ccs out of her! That’s about 3.25 ounces – which is a lot for her little chest!!! She should feel so much better now. I think they said it will most likely return. But now that we know what it is, we can start to work on that. I think we will have to change her diet (take the fat out maybe?). But, I’m getting ahead of myself. They will explain it to me tomorrow.

She is snoozing comfortably now. So, more stuff to learn tomorrow. Thank you for all your prayers!

Take care,
Christy