More chyle

Well, you might remember that on Saturday they had to change out the chest tube because it was clogged. Well, since then, there had been very little drainage. Again, got my hopes up.

Well, the docs told me this morning (Monday) that her x-rays showed more fluid in her chest than EVER before! So, something was up with the new chest tube. Also, her sats were low, her respirations high, and her heart rate decreased and it was all out of rhythm. So, they ordered more detailed x-rays and an ultrasound. They weren't sure if they were going to need to move the chest tube to a different area or what. Well, I went to a luncheon at CMS (my work buddies) and Tom came to the hospital to sit with Harlie. He said she vomitted a lot and the next thing they knew she started draining. So, she took care of the problem herself. She has been draining steady since and she is so happy about that. Since then she has been more playful than I have ever seen her. And her heart is back in order and beating normally again. Sats are back up and all seems good right now. I guess we will just watch and see how much she drains over the next few weeks and go from there.

Well, thanks for lunch, CMS. It was so great seeing all of you today!!

Take care,
Christy

Today is Tom's Birthday

Well, against my better judgment, I started to get my hopes up. They started the Portagen 2 days ago and she had very little drainage. But her x-rays on Friday showed more effusion, so that meant that her chest tube was probably clogged. So, today (Saturday) they had to change the chest tube and 40 mls drained. I think I heard them say that they are going to give her a week or two and if the drainage doesn't taper off, then her cardiologist is going to go back to her surgeon for more discussion. But, as you might have noticed, things change all the time, so who knows.

Despite the effusion, Friday was a good day for Harlie (and for me). She was so playful and it was so much fun to spend time with her. They let me hold her for 2 wonderful hours. I can't believe how much I missed holding her! I think I turned that emotion off during the past month. You should have seen her face when the nurse handed her to me. She was so excited to be out of bed and hanging out with her mama. It made my day.

Today is Tom's birthday. He is 34. Murphy is at my sister's house and my mom is with Harlie. So, we are going to a neighbor's party tonight. Then tomorrow we are going to have a small celebration here at our house. Well, that's about it for today. Thanks for everything.

Take care,
Christy

Hoping for no more chyle!

So, we heard from her surgeon in DC. Out of the two options we had, he opted for neither. Basically, he said he doesn’t want her on long-term TPN and he doesn’t want to do the plug in her PA. It is his belief that all chylothorax’s will eventually stop.

So, they started the Portagen again last night. It is possible that she was on TPN long enough that the vessels have had a chance to scar down and no more chyle will drain. We are keeping our fingers crossed that that is the case. If chyle does begin to drain again, then we keep on feeding her the Portagen and wait for the draining to stop – however long that may be. Unfortunately, going this way, prevents her from coming home until the draining has stopped completely, and the chest tube is pulled out. So, that’s that.

The central line in her leg started leaking yesterday. So, they had to pull it out. They found a vein in her arm and put in just a regular IV. She is still battling fevers, so they are doing other tests to see what’s going on. Yesterday, we had a surprise lunch visit from some of the girls I used to work with at Parker, Pollard and Brown. Thank you Patty, Pat, Jackie and Sue!!! Well, please continue to keep us in your prayers and pray that there is no drainage so we can get home soon.

Thank you,
Christy

7 Months Old

So, Harlie is 7 months old today. She weighs 11 pounds, 8 ounces. So far, she has spent a total of 3 months in the hospital. This particular stay was 1 month yesterday. I am so ready to get her home so we can be a family again!!

She is cutting her first tooth, so that should be real interesting. I’m really not sure how that works with her underdeveloped jaw. Only time will tell I guess.

I spoke with her cardiologist yesterday and no word from the surgeon yet. They need to put in a permanent line regardless, so we talked about that. Basically, there are no good options. So, they had to weigh all the risk factors and make the decision that way. They decided that they are going to put it in her neck. With her trach ties and trach care, I am a bit nervous about it. But he said that it will go under her skin and it should be fine. I think the access to the line will be behind her ear or something. When I think of how much I am NOT looking forward to this I just remember that at least I will get to bring her home and that helps.

They don’t know when they are going to put in the line. They have to wait 48 hours from the last negative blood culture. So, it could be the end of this week or beginning of next considering she’s had a fever off and on for the past few days.

No word on when they are going to pull the chest tube out. Hopefully soon.She is in good spirits. She’s been more smiley and playful the past few days. Yesterday she sat in her bumbo seat for 1 hour and 20 minutes!! She did great! Her head control is really improving and I will be so happy when we don’t have to worry about that anymore!!!Well, that’s about it for now. Oh, I added some new pictures, so check them out when you get a chance. Well, thanks for checking in. I hope you are all well.

Take care,
Christy

Update

Well, I spoke with her cardiologist today (Monday). There is a theory about Harlie's situation - I'll give you the basics. They are just thinking that they might need to put a plug in her pulmonary artery (it is still there with a band on it). The surgeon was thinking that she would need the extra blood flow. But now they are wondering if it is making the pressure too high, contributing to the chylothorax. Again, this is just a theory. So, her cardiologist here spoke with her cardiologist and surgeon in DC and it will be the surgeon's say as to if that should happen.

If he says yes, then he will also decide if it should happen here in Richmond or in DC. They would put it in through her vein (another heart cath). If he says no plug should be done, then they want her to stay on TPN (fed through her blood) for SIX WEEKS!!! Yes, that's right... six loooong weeks. I will admit that I was not prepared for that number. They will let us take her home on TPN, which is good. That just means that we will have to wait longer for some of that freedom from the IV pole that we were really looking forward to.

If they do the plug, then I think they will keep her on TPN for a little while and then try the Portagen one more time to see if the theory was right. So, the answer from the surgeon makes a big difference on what the next few steps are - and when we can bring her home. Hopefully we will hear something soon.

She has been battling a fever the past few days. They have been taking a lot of blood cultures to try to narrow down the location of the infection. Her chest tube has been in for over 2 weeks now, so that's a very good possibility. And of course, she has the central in her right leg, which has been in for just over one week. The one that was in her left leg was in for one week as well when that got infected. The last I heard, they were thinking it was the line in her leg.

I know that they want to put in a more "permanent" line to reduce the chance of infection. The problem is that there is no good place to put it. With her circulation, it is my understanding that they can't put it in her upper body. And her veins in her legs are so damaged already. So, I don't know what they're going to do. But I think they have to do something pretty quickly before that infection spreads. So, at this point, no one is talking about a discharge date. There are just too many things up in the air. Regardless, we are looking at a couple more weeks at least.

Well, thanks for all your continued prayers and support. I hope you are all well.

Take care,
Christy

More Chyle :(

Wow. Today was a crazy day. First thing this morning, Harlie had another cyanotic episode (turned blue). She had all the same symptoms as weeks ago. One doctor (a new one for us) said that maybe she was just “agitated”. I guess so. You would be too if you couldn’t breathe! I asked if the effusion (chyle) was back and they said it was very unlikely. (Don’t worry, I didn’t buy it.) They took another x-ray and the radiologist said he could not tell if it was edema, effusion or both. So, they did an ultrasound. And guess what... There was an effusion. They thought her chest tube was clogged, so the plan was to go on ahead and pull it out. The estimate for how much chyle was in there was about 20-25 ccs. So they were going to use meds to help dry her out. Normally, this wouldn’t be that big a deal. If one lung can’t function at full capacity, the other one compensates. But, Harlie’s right lung is already compromised with the chest mass. So, when her left one can’t work that well, it really shows.

Well, they had to change her bedding and with all the movement, the chest tube started to drain! Within minutes over 40 ccs drained! Immediately her sats went up, and we were able to turn the O2 down a bit. So, now we have to start all over again. She’s back on TPN for at least 5-6 days, then they will re-introduce the Portagen and see what happens. So, it is safe to assume that we will be here at least another 2 weeks. I am so thankful that we love our nurses! And that we are here instead of DC. Well, I have to run. I hope all is well with you.

As always, thank you for all your support and prayers!
Take care,
Christy

Two steps forward, one step back

Well, after all the excitement about no more O2, Harlie is back on O2. (one of the topics with kids with heart defects is whether or not it is safe for them to ride roller coasters - well, who needs to ride a roller coaster when that is your life???)

Anyway, her hemoglobin was low (the red blood cells that carry oxygen) so they had to give her some blood Monday night. We thought that would help bring her sats back up and I hoped that she would be off O2 by Tuesday morning. But as is the medical juggling act, the added blood meant fluid overload for her body, so she developed pulmonary edema (fluid build up in the lungs). And that certainly doesn't help bring up sats! They gave her lasix through her g-tube, but that didn't work. So they had to give her lasix through her IV (they are absorbed differently). They are working on getting all of her meds to be through her g-tube in preparation for removing the IV soon (which is a good thing). So, unfortunately, she is still on O2. But hopefully once everything gets balanced again, she won't need it.

On Monday, her belly wasn't tolerating the Portagen. They stopped her feeds for most of the day and then started them again at a very slow rate. They also put her on a few meds to help the food empty from her stomach faster (I'll spare you the medical speak on this topic). And 24 hours later it seemed to be working. So, we are keeping our fingers crossed that those meds do the trick. We decided that we would rather not put her through any more tests (gastric motility or modified barium swallow studies) unless absolutely necessary. So, we're trying the meds first and we'll see what happens. She will get another x-ray in the morning and if it does not show any effusion, they are going to pull the chest tube out. (YAY!) Then we can hold her again. We tried a couple of times, but it was just too uncomfortable for her. So, despite a couple of steps back, it is still progress. Hopefully we are in the fine-tuning phase.

As always, thanks for all your support and prayers!

Hope you are all well,
Christy

No more O2

Well, this weekend was very busy for Harlie. The BEST thing about this weekend is that for the first time in Harlie’s life – she is NOT ON OXYGEN!!!

She has gone 2 full days on room air and maintaining her sats in the high 80’s and low 90’s (which is where they want her to be). I know it is hard for most of you to know what I’m talking about here – but this is truly life-changing for us. Of course, as I write this, her alarm is going off because her sats are 76 and her respiratory rate is 120 (which is too high!). Anyway – she was weaned off the vent and she is now back on her trach collar (which is what she has on at home). Hopefully, in the next few months, we can start to wean her off the trach collar when she is awake. Now THAT will be incredibly freeing!!! But, I’m getting ahead of myself.

They had to put in a new central line. The blood cultures showed that the central line in her left leg was infected. So, they had to put a new one in her right leg. The doctor that did it was the same doctor that did her heart cath back in February. When they put the first central line in, it took under 30 minutes. For her second one, it took an hour and a half!!! Her veins have really been through it – causing scarring that makes putting in a line extremely difficult.

They started the Portagen again (special formula). Hopefully there won’t be any more drainage. And hopefully, her belly will tolerate it. At this point, we have to:

1. Wait a few more days for the infection to stop showing up in her blood cultures. (I think they said she has 2 or 3 more days of antibiotics).

2. Make sure that the damaged lymphatic vessels have stopped leaking chyle.

3. Make sure she’s tolerating her feeds.

4. Get a gastric motility or modified barium swallow study done.

Once all that is done, we can take her home!! Overall, we are very pleased with her progress this weekend. We are definitely heading in the right direction. Well, that’s pretty much it for tonight. I hope you are all well. Thank you again for your continued prayers and support.

Take care,
Christy

Infection

Hi. Well, I think Harlie is starting to feel better. She was smiling a lot more yesterday. It is so nice to have her smiling again! Her drainage has really decreased. They changed it to a water seal drain this morning. Then, at some point, whenever they think it’s time, they will go back to Portagen (that special kind of formula) to see what happens. They will watch the drainage to make sure that the chyle doesn’t leak again. If it doesn’t, great. If it does, well then we go back a few steps and try again later.

They are trying to wean her off the vent. So, we’ll see what happens there. Unfortunately, she has developed an infection in her blood. She has been battling a low-grade fever for the past few days. I told them that she usually runs lower than the average baby, so I thought a low-grade fever should be taken a bit more seriously in her case. They went on ahead and cultured her blood – and they found an infection. So, she’s back on big antibiotics through the IV. Hopefully, she’s enough post-op for this to be not that big a deal for her. And hopefully they caught it early enough that it won’t be severe. I haven’t talked to the doctors yet. I’ll let you know what I find out.

That’s about it for now. Thanks again for all your support. And thank you Marcy for your post on the guestbook!!! That is so thoughtful of you!

Take care,
Christy

Just another update...

Well, luckily, all is calm here. Yesterday was pretty uneventful. The chest tube continues to drain. Hopefully that will stop soon - she hasn't had anything in her belly for days now (other than a couple of meds). She is on Fentanyl to keep her comfortable because they say that those chest tubes can be very painful. She hasn't smiled in a few days, which feels like an eternity to me! Yesterday she looked absolutely pitiful. But this morning, she looks a bit better - still not smiling, but for some reason I think she must feel a bit better. She actually put a toy in her mouth this morning (yesterday she wouldn't even grab it). She had a fever this morning, they gave her some Tylenol, which she promptly threw right back up. I was comforted by a little “normalcy” for her. haha

I spoke with her cardiologist this morning. He reminded me that treating chylothorax can be a long process. Having a diagnosis certainly helps, but that doesn’t make the treatment any easier. (Don’t forget that we are still fighting the viral pneumonia as well!) So far, they have tried treatments that work for most patients. As usual, she has to be UNusual. So, they will continue to try other treatment options until one works. This is why it can take a long time to fix. And, in case you are wondering, a long time can mean months. Yes, months. The longest he has seen took 6 months. I would not be surprised if it took Harlie 6 and a half months, just so she could break the record. Hopefully she’s not that competitive (like her Nana). :)

So, basically, we are expecting her to be here for a few more weeks. Hopefully the next week or two will be good ones with a lot of progress. If not, they will have to put in a more permanent IV (a port-a-cath, most commonly used by cancer patients) and a chest tube that is re-inserted into her stomach so it can constantly drain back into her body for re-absorption. Hopefully, those things won’t happen, but if that’s what we’ll need to do to get her back home, then so be it.

Oh, here’s a funny story for you…I spoke with someone about Saturday’s scare. She said that she would look back at the monitor’s history to see what kind of alarm was blaring (was it in panic mode and still being ignored) and how fast her sats dropped, etc. Well, a nurse finally noticed that they had Harlie’s name misspelled in the computer (they had Holter instead of Holton) and on Saturday night, she corrected it – which ERASED all the history. How convenient. I told her it looks like a cover up to me. :)All joking aside, she is handling the matter with all seriousness, which I completely appreciate. No one wants anything like that to happen again, that’s for sure.

Also, I want to take a moment to thank all of you that have taken the time to come and visit us at the hospital, bring us meals and goodies, etc. We have loved and appreciated everything!

So, thank you, thank you, thank you!!!
Love,Christy
PS, Tom added some photos in the Family Album.

Easter Excitement

Happy Easter everyone.

Well, Harlie had a rough day on Saturday. When I got to the hospital, I met one of her cardiologists in the hallway and started discussing her status on our way to her room. As soon as we walked into her room, we could clearly see that she was BLUE and struggling. Her alarms were blaring and her sats were 67!!! No one was in her room – no one was helping her! The doc called for help and 30 to 45 minutes later, all was calm again.

Clearly, I will be talking with the appropriate people about this situation. Enough said about that. After that chaos, there was someone checking on Harlie every few minutes. I have to say that I appreciated all that her nurse said about the situation. She handled it very well and I know that she can't be in two places at the same time. She said that she had just checked on Harlie about 10 minutes before that episode. It goes to show how quickly things can change.

They think that the settings on her vent (which were turned down the night before) just made her too tired to continue at that level and the fluid build up must have pushed her over the edge. In one aspect we got lucky. Somehow they accidentally took two chest x-rays, one at 9am and one at 10:30am. There was such a difference in the amount of fluid build up between the two, that they decided to put a drainage tube in.

So, they put a central line in her leg, gave her some pain meds, and inserted the tube. This tube will stay in her chest until fluid stops draining. After just 24 hours, over 200ccs have drained (that’s almost 7 ounces)! They had her on Portagen, but that might not be working. So, tomorrow she will go on TPN (total parenteral nutrition - which is nutrition given directly into her blood). They are hoping that this will give her time to stop producing this fat and hopefully when they re-introduce the Portagen, there will be no drainage. At this time, I have no idea how long the TPN or the chest tube will stay in place.

Despite the drainage tube, her respirations are still extremely high. The doctors seem stumped by this weekend’s events. Hopefully we will get some answers soon.I hope you all had a nice Easter. Hopefully this week will be a better one for Harlie. Please continue to send her good thoughts and prayers.

Thank you and 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

Tuesday in the PICU

Hi. Well, not much new here. We are trying very hard to not get too frustrated, but it is definitely getting challenging. There is no light at the end of the tunnel yet and that is very hard to live with each day. Today Harlie had a CT scan of her chest. They really wanted to do one with contrast, but Harlie’s veins have had it, and have shut down. They struggled for 2 days to try to get an IV in one of her arms, but no luck. She had an IV in her foot, but with her new circulation, the dye will not make it to her lungs to get a picture. So the dye must be injected in her upper extremities. After the expert from NICU couldn’t get it in, they said they would have to do it without contrast and hope for the best. It wasn’t long after that, that the IV in her foot shut down. Luckily, she was done with her IV antibiotics, so they are going to give her a break and not put in another IV until absolutely necessary.

I am officially tired of seeing them stick her. She has been through enough.Unfortunately, the CT scan wasn’t that helpful without the contrast. She still has a lot of fluid in her left lung and they aren’t sure what it is or where it is coming from. They are trying to up her Lasix to dry her out a bit and if that doesn’t work, then they will have to drain it with a needle.

Clearly, she will not be home by Easter. I am really ready for us to make some good progress. Hopefully something good will happen soon.Well, thank you for your continued prayers and good thoughts. I hope all is well with you.

Take care,
Christy

Sunday night

Well, we are still here in the PICU. I think she looks better tonight than she has in a while. She looks much more like herself and is very smiley again, which is so nice to see.

Our family and Brandy gave us a break this weekend. From Saturday morning to Sunday afternoon, she had someone with her at all times and Tom and I got to spend the weekend at home with Murphy. As much as I enjoyed our time together, it made coming back here that much harder. I really hope that we can have her home by Easter.

As far as a medical update, she is doing well, I think. They have taken x-rays everyday. They have not improved, but they haven't worsened, either, so that's good. I think they are leaning toward viral pneumonia which means it needs to run its course and antibiotics won't help. But I'm not completely sure on that.

They tried taking her off the vent Saturday, I believe, but she didn't do well. So, they are going to give her another day or so and try again. I guess they will keep on trying until she starts to tolerate it. Her respirations are still averaging near 100. I think they want that number to be closer to 50. So, we'll see what happens in the next few days. I know that I will be talking with all of her doctors tomorrow (Monday), so hopefully they'll have some more info to share with me. If anything comes up I will update this entry at the bottom.

Thank you for all your support and prayers. Talk to you soon,
-Christy