Cardiology appointment

So, we had her cardiology appointment today. I definitely feel better about my understanding of everything now. As far as her lung surgery goes, there is definite promise that it will greatly improve her life. Once she isn’t working so hard to breathe, she should be more comfortable in general, she should sweat a lot less, she should be able to start to gain some real weight and with any luck, we should be able to get her off the O2! So, we are certainly excited about all of those things.

I still haven’t heard from the surgeon, and I think he is leaving tomorrow for vacation for 2 weeks.

Now, as far as her heart goes… I understand what the argument is there. In the most basic terms, the group wants her to have a procedure called the Damus. Her surgeon does not want to do this. He feels that it is his experience that when this is done, it causes valves to leak and you end up right back where you started, but with a more scarred heart. Makes sense to me. And it means no extra heart surgery, at least for now anyway. So, I’m fine with that. Plus, we can do this lung surgery and give her some time to heal and gain some weight, and then do more testing to see how things are functioning. I think it is possible that the relief of the lung pressure could help the heart. The idea is that he’s going to have to go back in there in the next year anyway, and that surgery might take care of it as well.

One new development… I mentioned to her cardiologist that since we left the hospital two weeks ago, her heart rate has been continuously low. We knew she had heart block (which is why they installed the pacemaker wires when she was just 4 days old), but so far, it hasn’t been that much of an issue, so they haven’t hooked it up to a battery yet. Anyway, I was thinking that the monitor we have just couldn’t keep up with the heart block, so it was showing 50 beats per minute, when in reality it was more. So, he felt her pulse and thought the monitor was correct. So, they put a Holter monitor on her to record her heart rhythms. I will turn the device in tomorrow for them to analyze. Her doctor said that if her heart rate is actually that low, they will need to hook up her pacemaker asap. Well, that’s about it for tonight.

Take care,
Christy

Stuff...

Well, we had another helmet fitting today. Last week the pressure points started to break her skin, so I had to take the helmet off for a while. She made some adjustments but I can’t put it back on Harlie until her skin is all healed. Hopefully I’ll have her back in it very soon. Even with the broken skin, she didn’t cry while wearing it. She’s something.

I spoke with a scheduler from Children’s in DC about Harlie’s lung surgery. Unfortunately the surgeon will be on vacation the first two weeks of July. I keep forgetting that July 4th is coming up. Anyway, so it looks like it will be the third or fourth week of July. The surgeon is supposed to be calling us to talk to us. I really don’t want to wait 4 weeks for this. Hopefully he doesn’t either.

We meet with her cardiologist tomorrow. Oh, I forgot to mention that Murphy’s 3-year checkup went well. It is so funny how different the appointments are. Talk about both extremes. I was just reading a post on my trach board about helping our kids deal with the anxiety of so many drs. visits. One girl who had been through it herself as a child said that it is so much better if you are honest and tell the child that it will hurt, etc. So, I thought I would try it out on Murphy. I told Murphy that the nurse was going to give him a shot and it was going to hurt, but only for a minute. I told him as the nurse was coming in so he didn’t have to think about it for too long. Well, I really think it helped. He handled it great. Well, that’s about it for tonight.

Talk to you later.
Christy

Lung News and Hannah

So, I heard from Harlie's cardiologist today. Here's what we talked about...

The chest mass is not a chest mass - it is Congenital Lobar Emphysema (CLE). It is a rare (shocking, I know) chronic disease that causes respiratory distress in infants. Air enters the lungs but cannot leave easily. The lungs become over-inflated, causing respiratory function to decrease and air to leak out into the space around the lungs. CLE is more common in boys than in girls. It is often mis-diagnosed in utero because there is no air in their lungs at that time.

Your right lung has 3 lobes, your left has 2 lobes. Congenital lobar emphysema usually affects only one lobe, and this is usually an upper lobe. It occurs most frequently in the left upper lobe, followed by the right middle lobe. Leave it to Harlie to be a-typical.

Her CLE is in her upper right AND lower right lobes. Her cardiothoracic surgeon will not operate on her lung and heart at the same time. Since her lung is deteriorating more quickly, that surgery will be first. Her surgeon said he will not do this operation. So, the head of general surgery will be doing it. I think he will go in through her back or her side. My poor babe will have scars on every area of her body!!!

Anyway, I am still waiting on a surgery date. Although I have been told that it will be in July. He said that the surgeon thinks that he should be able to remove the upper and lower lobes, and leave the middle lobe to expand some. The idea is that the pressure will be relieved, thus enabling the middle lobe to expand and grow along with her. They are thinking that we should see her breathing easier almost immediately. Ahhh! What a relief that will be!

As far as her heart goes - the jury is still out on what to do with that situation. Evidently, her surgeon is the lone ranger and doesn't want to do it. I tried so hard to follow what her cardiologist was telling me, but I simply couldn't. I just don't understand. But we will be seeing him on Thursday and Tom will go with me so hopefully when he explains it again, I'll get it.

I'm not beating myself up about the fact that I can't begin to understand everything. They have spent many years learning and gaining experience in this. I have spent many years doing, uh, other things. Like having fun and NOT learning how a heart functions, or doesn't function properly. All joking aside, it is very scary. Since they do not agree, I feel like I should have a say and be the deciding factor. But, I can't even begin to have an opinion. Another surgeon in DC said he would perform the surgery if her surgeon won't. I am not comfortable with that. So, we'll just see what happens over the next several days after everyone's had a little while to think about it.

Now, on a different note, I would like to ask you for some prayers for a friend of mine. Her name is Katie and I met her when Harlie was about to be discharged from MCV in May. Her and her daughter, Hannah, were right beside Harlie and I. Hannah also had a trach and a facial syndrome so Katie and I had a lot in common and we really enjoyed finding each other. Hannah had Moebius Syndrome, which is facial paralysis, so she could not smile or make any other facial expressions. She was so cute and full of personality! Well, Katie called me today to tell me that she lost her beautiful Hannah. She was 20 months old. Somehow her trach came out and she went into cardiac arrest. Tom and I went to the funeral home tonight. I just want to ask you to pray for Katie. We fear what she is going through right now every day. With children like Hannah and Harlie, we know all too well that things can go from great to tragic in a matter of minutes. For the past 20 months, Katie has worked non-stop to prevent that from happening. My heart just breaks for her.

Thank you for your thoughts and prayers.
Christy

Ortho appointment

Well, we went to Harlie's orthopedic appointment on Friday. I say that because I was SO tempted to cancel it after the ENT experience on Thursday. To be honest, I didn't want to hear anything else bad. But, avoiding doesn't mean it isn't true - so I decided to keep the appointment. I figured that the knowing is better than not knowing and I knew that I would regret it later if we didn't go. So, we went.

When I made the appointment, the nurse told me to come prepared to wait. So, I called ahead and asked if I had to wait, can't we just come later? I told her that Harlie was on oxygen. She said that she would make sure we didn't have to wait. So, we got there about 15 minutes late (not bad by our standards!) and we were OUT THE DOOR IN LESS THAN ONE HOUR!!! Can you believe it? They pushed us right through and I felt like there was a red carpet underneath us.

Harlie had x-rays and the doc told me that yes, she will need surgery to help her - but not now. I said, "well that's a good thing, because I don't think I could work you in." He said that she clearly has more important issues right now. A brace will not help. They will have to fuse her vertebrae together to keep them from bending anymore. We will go back in 6 months. He told me for the time being - don't worry about her back. Wohoo!!! And he said that the helmet is fine and won't hurt her neck and physical therapy is fine, too. So, a bunch of worries off my mind for now. So, that's a good thing. I'm really glad we went. I'm not glad that she will need more surgery, but I knew that already.

This week I take Murphy to the pediatrician for his 3 year check up. Yes, I know. He turned 3 in May. I have one kid I can't keep out of the doctor's office and one I can't get there! Then we have another helmet fitting and a cardiology appointment. Hopefully the DC team will be reviewing all of Harlie's info tomorrow and we will hear soon what the plan is. UGH... the waiting. Hate it.

Okay, well it is late. I hope you are all doing well and I will update you again soon.

Take care,
Christy

ENT news

So, we went to Harlie’s ENT appointment today. I went hoping to hear that Harlie could be upsized to a pediatric sized trach (she’s still in a neonatal size). The reason why I wanted that is because then you can start to try a Passy Muir Valve (PMV). It is a one-way valve that allows air in, but doesn’t allow air out. The trach tube is smaller than the trachea, so air should be able to go past the tube and past the vocal cords, thus making sound. But, unfortunately, we learned more bad news. She has tracheomalacia.

Tracheomalacia is a weakness and floppiness of the walls of the trachea. The walls basically collapse, which doesn’t allow air to pass through. Typically, they outgrow this condition between 18-24 months of age. So, until she outgrows this, a passy muir valve is not an option. This appointment was not fun. At one point I had to turn away – I just couldn’t watch Harlie struggle to breathe while the doctor did her stuff. I guess this makes her jaw reconstruction less of a priority. This whole time we were thinking that if her jaw was fixed, we could take the trach out. Now that that’s not the case, I guess we can relax on that.

I have to say I was bummed to find out that she has TWO reasons that are RARE to need a trach. Anyway, tomorrow we have an orthopedics appointment (for her spine issues). Hopefully, it will go okay.

Take care,
Christy

Quick update.

Harlie seems to be getting back to her usual, happy self. She was very smiley today. She is doing great wearing her helmet. She is wearing it the full 23 hours a day now. And she really doesn’t seem to mind at all. We take it off to give her a bath and I will take it off during tummy time and to let her roll over.

She had physical therapy today and we left it on during the whole session. Hopefully the weight of the helmet is helping her gain some strength. Her physical therapist said she is doing really well. Harlie even sat up by herself for a few seconds. Hopefully once she learns how to balance and realizes she can use her arms and legs, she’ll really take off.

We went to see the GI doctor and nutritionist yesterday. Nothing too eventful there. She has C-Diff again. That’s a bummer. She had to have Vancomycin again in the hospital, so that’s not surprising. Plus, she’s on an antibiotic for her ear infection. She’s now on 7 medications. Ugh. We were down to 3 at one point.

We see a new ENT doc tomorrow (here in Richmond). The only ENT we’ve seen is one in Dr. Magee’s practice in Norfolk (other than hers in DC). Oh, you should see Harlie when Murphy walks in the room – she goes crazy! Her arms start flapping and she kicks her legs and laughs. It is so funny! Well, just a quick one tonight. I hope you are all well.

Take care,
Christy

Frustrating Day

So, we went to the pediatrician today. She weighs 13.7 pounds. So, at least we’re starting to make some progress. I know it is hard to tell in her photos, but she is TINY. She needs some fat in a bad way.

She has her first ear infection. I am very surprised that we’ve made it this far without one to tell you the truth. She has a very small ear canal, so I really thought they would be more of a problem before now. We are going to go on ahead and when she has her tracheostomy re-done after the next heart surgery we are going to have them put a tube in her ear just to be on the safe side. (They have to remove the trach and put a bandage over the stoma for infection control during heart surgery – then they re-do the tracheostomy.) Since she only has hearing in one ear, we need to do everything we can to keep it good and healthy.

I am really starting to like this pediatrician. After talking about the recent developments, he clearly understands how hard this is for me as a mom. Most doctors just look at surgeries without any emotion. He was very sympathetic and clearly understood how discouraging this news has been for us. No word on her surgery date. Not that I was expecting one by now anyway. I am really hoping that we will find out soon. Watching her work so hard to breathe is starting to take its toll on me.

Oh, one good thing, I must have misunderstood her doctor when he told me that they don’t have any history of a person with one lung and her heart surgeries. I’ve been told that there are kids out there that have just one lung and have had the Fontan (the last surgery). Maybe he was talking about her particular lung mass. At any rate, it gives me some hope.

So, I had a very frustrating experience today. I was getting ready to take her for another helmet fitting (we forgot to drill holes in it and it was still a little too close to her left eye). I started loading the car and organizing myself to get out the door well in advance of when I had to leave. Got in the car, Harlie had to be suctioned, I get out of the car, run around and suction her. Get back in the car and realize that someone is backing in my driveway (it was Harlie’s equipment and supply company). I get out of the car and the man tells me he has an oxygen concentrator he needs to switch out (I had NO idea they were coming). I kindly explain that he cannot do that right now, I have my daughter in the car and we are on our way to an appointment and I have to leave or we’re going to be late. There was NO way I was leaving her in the car unattended and absolutely NO way I was going to take her out! And I didn't have the time to spare. So, he says, “hold on, I have to call someone.” WHAT?

So, he brings me his cell phone and puts me on the phone. I tell that guy (with less kindness) that I must leave now, I’ve got my daughter in the car, etc. Now, I am having to constantly open and close the doors and she is getting hot and sweating all over – which makes her cough and gag and vomit. So, that guy says, “okay, well, hold on, let me get my boss.” Are you kidding me???? I am STEAMING mad by this time. The last guy gets on the phone and says, “what’s the problem ma’am?” Oh boy. I explain it to him with NO kindness and tell him that I don’t have time for this, I have to go and tell your guy to get out of my driveway! Finally, he says okay can we come back tomorrow? I say yes – but it has to be before 11:30. So, he says, “okay we’ll be there at 11:30”. Duh. I said BEFORE mister – “we have to leave the house at 11:30 – and we aren’t doing this again!”

Clearly, this guy does not have a medically fragile child. Harlie can be fine, and you change temperatures, and it can take her 30 minutes to recover. And who is the one that is working like crazy to help her – and is in agony over watching her retch and cough and gag and try to catch her breath? ME! It is during times like these I wish I had some way to make people understand. This guy didn’t want to be inconvenienced by having to drive all the way back out to my house tomorrow. And I know he didn’t know it, but he was essentially making my child suffer. He probably just thinks I am some kind of crazy woman. And yes, just in case you were wondering, I was late for our appointment.

Ahhh, another day, another adventure. I can only wonder what tomorrow will bring.

Take care,
Christy

Helmet appointment.

Well, I think Harlie is starting to feel a bit better. This afternoon, she was much more herself and tolerated sitting up in the high chair for a while. She started this funny thing today. When you smile at her, she smiles back and tilts her head to one side and scrunches up her shoulders. It was hysterical. It's like she's flirting. It is so nice when she is interactive like that.

On Friday, we went to get her helmet adjusted. I was alone that day and getting her out the door proved to be especially challenging. She has a feeding tube that is connected to a backpack that I wear, a pulse ox monitor hooked up to her foot, and a tube from her trach collar to the oxygen tank. So, I have to carry her, a monitor, a backpack, an oxygen tank, a suction machine, and a diaper bag. Yeah, it's something.

So, I hooked her up to our handy-dandy traveling oxygen tank and got her ready to take downstairs. I turned on the tank and heard it come on, put it on her and started to gather everything in my arms. But, her alarm was going off and she was not looking good and started to turn color. I checked everything and realize that the tank has gone empty! So, I grab another tank (larger and not so travel friendly). Hook that up and hear it come on. Carry everything downstairs. She's not looking good again. Alarm starts sounds. I realize that tank has gone empty, too! So, I run in the garage and grab my last tank (only one "emergency" tank left in the car). I transfer the regulator onto the new tank and realize that I don't have the key to open it. So, I run upstairs to grab the key from the first tank. Run back downstairs, open the tank and she's like, "ahhhh". And so am I.

I thought about calling and canceling the appointment, but darn it, we need to make some progress somewhere! So, I loaded up the car away we went. Luckily, Christina only had to make a few minor adjustments, so it was a quick one. Harlie slept the rest of the day! And that night! I know she doesn't get good sleep at the hospital, so I guess she needed to get caught up.

Well, she has been doing great wearing the helmet. I can't believe how well she tolerates it. If it weren't so hot, it would be a snap. I took her to the neighbor's this weekend, but didn't make her wear it outside. WAY too hot for that.

Tomorrow we have an appointment with her pediatrician; Tuesday, GI; Wednesday, PT; Thursday, ENT; Friday, PT and Ortho. So, another busy week ahead. Oh, and I think we're supposed to see cardiology again, but we don't have an appointment yet. Hopefully, we'll hear something soon from DC. Well, that's pretty much it. I hope you all had a nice weekend.

Take care,
Christy

Home again.

Well, we are home. Harlie is still not herself. But hopefully a good night’s sleep in her own bed will help.

As far as what we learned today…I spoke with the pulmonologist and he showed me her CT scans. The good lung is in the middle and it is a small patch. He is guessing that's about 20% of that lung. Unfortunately, when comparing the two CT scans, it is clear that the mass has gotten larger since her birth. It is in her lower and upper lobes, which is not characteristic of a CCAM (which is what was diagnosed a year ago). The other unusual thing is that CCAMs either stay the same size or shrink. They typically do not grow after birth.

So, what is it you ask? They don’t know. So, they are definitely going to have to remove the mass. Then they will biopsy it to find out what it is. The doctor said that normally you can remove one lobe at a time since there is a clear separation. However, there is a chance that the mass cannot be “neatly” separated from the good lung. Which means that they might have to remove the entire lung.

The team in DC will be receiving all the information in the next few days, but one of the main docs is on vacation next week. So, they are thinking that they will not be able to have any answers to us until the last week in June. Basically, all we are waiting on now is when the surgeries will be. Dr. Jonas is her heart surgeon and he will also be removing the mass.

Now that I've had a little time to begin to accept where we are, I am ready to move on and get this behind us. So, hopefully we won't have to wait too long.

At this point, Harlie is back on O2 – full time. The second you take away the oxygen mask, her sats drop and she turns color (blue). Her x-rays after the heart cath were great. So, no major problems in her left lung as far as fluid build-up or anything. Hopefully, we’ll find the answer to this soon. The required O2 is definitely a downer. Makes her way less portable among other things.

We got her new helmet today. It just needs a minor adjustment and we are scheduled to have that done tomorrow. So, hopefully we’ll be able to make some headway on that now. hehe, pardon the pun.

Tomorrow will make one year since we learned of Harlie's heart defects. It has been the longest year of my life. But when she smiles at me, it all seems to melt away.

Well, that’s it for tonight. Thanks for all your support.
Take care,
Christy

Heart Cath Results

So, it was a busy day indeed. And tomorrow will be as well. She did great during the heart cath and she is resting soundly now.

They tried to get an IV one more time this morning. They went straight for her scalp since they knew there were no other options. It didn’t work. So, they applied some numbing cream to her groin area and neck in preparation for lines. Then at noon, they gave her a shot to sedate her. Then at 1pm, she went in for the heart cath. My friend, Melissa, came up for the day, so we went to lunch and then went back to sit in the waiting room until it was over.

I don’t know if I’ve ever explained what a cath is. Basically, they put a line into her vein, which goes into her heart. Then, they inject dye through the line and take x-rays and films of the dye moving through her heart and lungs.

Dr. Moskowitz tried to get the cath in through her legs – neither worked. So, he had to go through a vein in her neck. Boy, she is going to be one sore, bruised, little babe. After the cath, she went straight to get the CT scan. They wanted it with contrast (when they inject dye), but they said that would have taken her over the limit of radiation exposure in a day. So, they did it without the dye. I had copies of the CT scan taken up in DC when she was only a few days old, so they will have that to compare. Hopefully, that will help.

At 4:30, Dr. Moskowitz came to let us know what he learned from the cath. Harlie is so incredibly complicated. Well, that he knew, but now she’s even more so.

1. She has only a middle patch of working lung tissue in her right lung.

The chest mass is taking up the upper and lower lobes and is squishing the center lobe and remaining lung tissue. The CT scan will help us determine what to do. We will discuss the results of that study tomorrow.

2. She has developed subaortic stenosis in her heart (a narrowing of the area below the aortic valve).

This is a new development since February’s cath, and was a complete surprise. It must be fixed within the next couple of months. Unfortunately, this means another open heart surgery soon. The team here is sending all the info up to the team in DC and they will go over what all this means and gather a plan.

3. The pressures are good in her heart and lungs right now.

This is very important as to how good of a candidate she will be to undergo the Fontan (the last heart surgery to finish the “repair” that has been started). The Glenn (which was done in March) re-routed the blood from her upper body straight to her lungs. The Fontan will re-route the blood from the rest of her body straight to her lungs. The harder everything has to work – the higher the pressures get. This is what makes someone a good candidate (less risky for the patient and better success) for the Fontan.

Some ideas to help make her a better candidate for the Fontan were to 1) remove the mass in the right lung, with the hopes that the remaining lung tissue grow and get bigger, and 2) perform the Fontan sooner than normal so the pressures don’t have the chance to get too high. They normally like to wait one year after the Glenn to perform the Fontan. So, I don’t know what they would need to do in Harlie’s case. That will be up to her surgeon and his team in DC.

I think if I had to break it down, the scariest thing for us, at this time, is that the Fontan is not a guarantee. I had no idea that this would be an issue. Once you start this transformation to a single ventricle heart, I didn’t think you could stop without finishing. I asked Dr. Moskowitz what happens if the Fontan cannot be performed and he said he didn’t know. He does not know of any history or cases of these surgeries being performed on a patient with only one lung.

Tomorrow, the pulmonologist will come and talk to me about the CT scan, and what we need to do about her lungs. I am assuming that if they want to perform surgery, it will be soon and it will be done in DC. Dr. Moskowitz was thinking that maybe they could do it at the same time, but I don’t know how the heart surgeon will feel about that.

Well, I know that is a lot of information and if you are still reading this, you are a good person. Thank you very much for hanging in there with me on this one. Harlie is now resting comfortably and because she has the line in her neck, they moved us back into the PICU – our old home away from home. I know most everyone over here (more importantly, they know Harlie) and the care is so much better, so I am able to relax a little. Hopefully, they will remove the line tomorrow, resume regular feeds, and with any luck, we’ll be able to go home on Friday.

Well, please keep Harlie (and us) in your prayers.

Thanks,
Christy

Busy day coming up.

Well, this is just going to be a quick one tonight. I'm sure you're happy about that! haha

Nothing happened today - no new lines - no tests. So, it was a good lazy day for us. Tomorrow is another story...

Harlie's heart cath and CT scan is tomorrow. I am glad because it means less sticking and consolidating sedations.

On a good note - Harlie's reflux seems to be under control! YAY! In the past 2 days she hasn't thrown up ONCE! What a welcome change for us! And her C-Diff seems to be under control, too. So, hopefully, she will finally start to gain some good weight. I long to see some fat on her.

I'll let you know what happens tomorrow. Please continue to keep her in your prayers. Thank you so much for all your support!

Take care,
Christy

More stuff..

Okay, where to start...The cardiologist has a couple of theories. One is that her pulmonary edema is being caused by too much blood flow to her lungs. He thinks this because the past several days of diuretics should have helped by now if it was just an isolated thing, caused by the IV fluids given on Wednesday. This leads him to the next theory. Remember the CCAM (chest mass) that was diagnosed at the very beginning of this crazy life of hers? Well, prenatally, it appeared that it took up all of her right lung (which consists of 3 lobes) and squished all of her other organs (left lung and heart). After she was born, she had so many other issues, all they said was that it was "insignificant" at that time. He thinks that the CCAM never allowed her right lung to develop properly (something we feared prenatally). So, all this blood flow going to her lungs... if she has little to no lung tissue in her right lung, then it all goes to her left lung (which consists of 2 lobes). Clearly, this is overloading it. So, she needs to have a contrast CT scan of her chest. They tried today - but the IV stopped working. They put the IV in at 1pm and by 4pm it wasn't working. This was the 3rd IV in 24 hours! Having to stand by while they work to find a vein, and then try to stick it, is absolute agony for me. It makes me want to scoop her up and take her home AMA (against medical advice). So, no CT done. They need the CT information to help them determine what to do. And to make matters worse, they need an IV to give her Vancomycin just in case the positive blood culture is for real. Her next dose was due at 6pm tonight. I told one of her cardiologists that I just couldn't take them sticking her again tonight. Oh, and I forgot to mention that they also have to do another heart cath to see if putting in a plug to reduce the blood flow to her lungs helps. When they do that, they have to use a central line, which stays in better and longer. My suggestion was to wait until Wednesday (when the heart cath is scheduled) and do the CT at the same time. Especially since they said that they forgot to mention that she needs to be sedated for the CT scan. But, with her blood flow, the dye has to be injected in the upper body and the central line is usually done in the groin area. Oh, and they need the CT info before doing the heart cath. So, since it was the evening, no central line can be done tonight. So, luckily, the docs agreed that she will just have to skip the Vancomycin for tonight until we can figure this out tomorrow. Now, to add to the agony (which I am sure is what you feel after reading this), in order to put in a central line, she has to be sedated. In order to sedate her, they need an IV. Do you see how frustrating this is? Are you feeling my pain yet? I think they are going to have to put an IV in her scalp again. That worked last time and although the IV didn't stay in for long - it was in long enough to sedate her. Well, clearly, no plastic surgery appointment tomorrow. We are here for the week. I had to call and reschedule it. And she was supposed to get her helmet on Wednesday. Luckily, Christina is so good to us. She is going to bring it to the hospital, mark it up and then take it back to her office to cut it. So, hopefully we'll make some progress on that. Well, I gotta go. Please keep our sweet babe in your prayers. She is really something special. After all the sticking, she still smiles. Take care,Christy

Say it isn't so!

So, lazy Sunday, both Tom and I are feeling awful (colds, sore throats, etc.) when the phone rings. It's the doctor from MCV. He tells us that the blood culture they took during our Friday ER visit came back positive for Staph aureus (type of infection in the blood). I'm betting the culture was contaminated.

Although she's still tired a lot, and still requires O2, she has NOT had a fever. But, he said that we had to bring her back. And this time she would definitely be admitted (no talking my way out of this one) until another culture came back negative. And they have to give her Vancomycin (IV antibiotics) to be on the safe side. UGH!

Sometimes I feel like I'm going in circles!!!! Vancomycin is what probably led to her C-Diff. Which is probably what led to us being here - again - for the 3rd time in 5 days! I really am getting to know too many people here. It's scary. Nurses that had not had Harlie before already knew her history.

So, now we are back up in the Progressive Care Unit. But this time, we have a private room - the isolation room. So, that makes it better. I spoke to one of her cardiologists and told him I would REALLY like to be outta here by Tuesday morning. She has her appointment with Dr. Magee at 11am in Norfolk and I really don't want her to miss it. Especially since she's seeing ENT and I have a ton of questions for him. I know that wanting to see her craniofacial plastic surgeon might sound superficial, but it isn't. He is the one that will do the repair that will get her trach out. And after Tuesday's craziness - we REALLY want that trach out!!!

Oh, and on Thursday night she pulled her trach out for Tom when I was out. He got it back in, though, without too much trouble thank God.

So, hopefully, her blood culture from today will be negative and we will be able to leave here and still make it to her appointment in Norfolk on Tuesday. And she gets her helmet on Wednesday. So, we have a busy week and cannot be hanging out here at MCV! So, please keep your fingers crossed that everything is okay with her and that there isn't a more serious underlying issue they haven't found yet. Hopefully, this is just another speed bump and we will be back to making progress soon.

Thanks for your continued support. We really appreciate it.

Take care,
Christy

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

Quick trip to the ER

So, another crazy day in the Holton household.

This morning, I got Harlie up and brought her downstairs. She was sleepy again in no time. She slept a lot yesterday, too. So, I took her back upstairs for a nap. I hooked up the monitor, and her sats were in the high 60s. Definitely low for her. She's usually in the 80s.

So, I wait, just to make sure it's reading correctly. Then I turn on the oxygen and they go back up. She takes a nap and a while later I get her up for physical therapy. Our physical therapist brought over a piece of equipment to help her stand so she can learn to put weight on her legs. She also brought me some special foam to cut and put in her high chair to replace the towel that I was using to get her higher than the tray. By the time we got that all set up, we ran out of time for PT. Which was fine, I thought, because she was already tired again (after less than an hour). So, I take her back upstairs and check her sats again. Still 60s. So, I go on ahead and call her pediatrician. Who calls her cardiologist, who tells her to tell me to bring Harlie to the ER at MCV. UGH!

Of course, Brandy was off today. And Tom had to go to Charlottesville after work for a meeting. So I call my brother and arrange for him to pick up Murphy. Then, I get Harlie all loaded up. Hook the oxygen up in the car, and head on down there. We get all settled in, catch up with each other (it's pretty darn nice to know everyone there) and they take x-rays. The x-rays look better than they did a month ago - so that's good. They think maybe she was dehydrated, so they put in a line and gave her some fluid. Again, we had to go through finding a vein - so much scarring already.

Since the x-rays were so good, they think maybe she is getting a cold (something we suspected a few days ago). Unfortunately, because her right lung is compromised (chest mass), the slightest thing offsets her balance. She just has no reserve.

So, we get discharged and I sit in traffic for over an hour. I decided to stop at the grocery store for dinner on my way to get Murphy. It was the first time I've been in a grocery store with Harlie. And it went fine. I only had to suction her once.

Well, it is late. Talk to you later.

Take care,
Christy

Accidental Decan.

I guess Harlie just doesn’t want me to relax. Today, the respiratory therapist (RT) came to do her monthly assessment of Harlie’s medical status and check the equipment. As I am talking with her, I hear normal Harlie sounds over the monitor (she was napping).

Brandy goes upstairs to check on her and then I hear Brandy say calmly, but seriously, “Christy, come up here.” So, I run upstairs and as soon as I get to the top, Brandy tells me that Harlie pulled her trach out and she can’t get it back in.

So, I try to get it in. No way. Not going. We tried repositioning her. Brandy put her head back to expose her stoma, and I tried again. No way. At this point, Harlie is definitely struggling and turning blue.

So, I go and get another trach, put the obturator in (a guide that’s firm to help the trach go in, because the trach tube itself is kinda floppy). I can’t remember if I tried again or not, but decided that she needed to be on the changing table where I normally do her trach changes. I thought maybe some normalcy would help. And that would certainly put me in a better position to know that I'm at the right angle, etc.

Well, Brandy starts to bring her over, but then we realize that she’s still connected to the feeding pump, which clearly, will not reach. So, the RT (who followed me upstairs) turns off the pump and we disconnect her, then Brandy puts her on the changing table and I try again. I literally had to force it, and thankfully, it FINALLY went in. Whew!

Then the RT held the trach in place while Brandy put new ties on. I suctioned her, and there was just a little bit of blood, which I was very surprised about. I really had to force it, so I expected more. We put her back on the trach collar with a lot of oxygen (the RT had gone straight to the concentrator as soon as she came upstairs which was really smart, I think) and she pinked back up. And then she smiled at us. That little stinker.

I think the whole episode lasted less than a minute. And let me tell you, A LOT can go through your mind in a minute. But, thank God, none of that happened and she is fine now. As the RT was leaving, she told me that I did everything right, which was really nice to hear. And I think Brandy and I worked very well together in this stressful situation, which is very comforting.

So, that was our excitement for the day. For the week, month, year, I hope!!! As luck would have it (hey, I’ll take what I can get), we see ENT one week from today (in Norfolk), so I will run it past him and have him check her out extra carefully to make sure she’s okay. Oh, I can see that little girl is going to keep me on my toes!

Take care,
Christy

Good things.

On Sunday, my family went out to dinner for a surprise retirement dinner for my mom. It was so wonderful to load up my family of four and go out to eat. It was the FIRST time the four of us have been out to dinner together. Can you believe it? Harlie is 8 months old, and it was the first time!!! It is amazing how something that most people do without even thinking about, is such an accomplishment for us. Of course, there were 18 of us and Murphy was down at the kid’s end of the table, but Harlie was in between Tom and I and, technically, we were out together. So, I think it counts.

I also think it was only the second time the four of us have been in the car at the same time. Once, we dropped Murphy off at school on our way to a doctor’s appointment. But that was a long time ago. Anyway, I am very thankful for those moments. I truly do believe that things are getting better for us. And that is such a good feeling.

Oh, and to further prove that point, I forgot to get the mail on Saturday. So, Sunday night I grabbed it and there was a notice from Dr. Magee’s office (Harlie’s craniofacial plastic surgeon) letting us know that she has an appointment to see him on June 12th!!! Yes, NEXT Tuesday!!! How great is that?!? I had him on my list to call and schedule something – fully prepared to wait months to get her in. And here, the appointment just comes to me. AND, to make it even better, we didn’t have anything already scheduled on that day!!! Seriously folks, the stars are aligned!

While there, we will see Dr. Magee (of course), someone from neurosurgery, and ENT. I am really hoping that Dr. Magee will have a plan for her jaw reconstruction and maybe, just maybe, we can set a tentative date? I guess I won’t get my hopes up that high. But, he did say he could do it after she was cleared by her cardiologists, and she is! So, please, please, keep your fingers crossed that good news is coming our way! I can’t even allow myself to think about life without the trach. When those thoughts enter my mind, I have to shake them away. To be able to hear Harlie laugh, or burp, or whatever, will be better than I could ever describe. Heck, it is so great to hear her play with toys. I LOVE it! I can only imagine how great it will be to actually hear HER!

Well, that’s it for tonight. As always, thanks for checking on us. I hope all is well with you.

Take care,
Christy

C-Diff and rambling...

So, the C-Diff (short for Clostridium difficile) test came back positive. So, now she’s on antibiotics to treat that. Here is a little explanation that I found:

C. difficile bacteria are everywhere — in soil, air, water, human and animal feces, and on most surfaces. The bacteria don't create problems until they grow in abnormally large numbers in the intestinal tract of people taking antibiotics or other antimicrobial drugs. Then, C. difficile can cause symptoms ranging from diarrhea to life-threatening inflammations of the colon.

It went on to say that it is most often spread while in the hospital. And to add to the risk, she was on so many antibiotics during all her line infections, including Vancomycin, which evidently is pretty powerful. She started showing symptoms while in the hospital, but everyone attributed them to her formula change. I am so glad that her nutritionist suggested the test. Hopefully, once we get this under control, she will start to gain weight again.

On Thursday, Harlie got another mold of her head made for her helmet. It should be in the second week of June. And hopefully she’ll be able to wear it with no problems so we can get her head fixed. I know it is hard to tell in the photos I post. I will take a picture from the back so you can see, too. My only fear of her being able to tolerate it is that it is so darn hot, and it is summer. She will have to wear it 23 hours a day for several months. So, we’ll just have to keep her in the a/c.

After that, we went to see her pediatrician. Nothing major happened there. Just him getting to know her and to talk about her upcoming appointments.

On Friday, her physical therapist came to see her for the first time since March. She was very pleased at how well she has done despite being in the hospital for the last 2 and a half months. She is going to come at least twice a week for the next couple months. Hopefully, between that and me working with her the rest of the week, Harlie will be sitting up on her own very soon. She has so little fat on her bum, that her tailbone is incredibly sharp. I am a little worried that sitting up for an extended period of time might hurt her. But, hopefully with the new antibiotics, we’ll start to see a faster weight gain. And maybe by the time her balance and strength can handle sitting up, she’ll be fatter.

Oh, on Thursday night I went to see Martina McBride, Rodney Atkins and Little Big Town at the coliseum with Nancy and some of her friends. I wasn’t really a Martina fan before, I was just going to go out with the girls, but seeing her live gave me a new respect for her voice. Rodney Atkins opened for her and he sings a song called If you’re going through hell. It came out last summer and the first time I heard it – it spoke to me. Of course, I was pregnant with Harlie and we knew about her chest mass and heart defects. Tom and I said that was our theme song. For those of you that don’t know the song, here are some of the words:

Well you know those times
When you feel like there's a sign there on your back
Says I don't mind if you kick me
Seems like everybody has
Things go from bad to worse
You'd think they can't get worse than that
And then they do

If you're going through hell
Keep on going, don't slow down
If you're scared, don't show it
You might get out
Before the devil even knows you're there

Anyway, it is funny how you think some songs were written for you. I think it still applies. Monday is the last day to get your Pampered Chef orders in. Well that’s it. I think you are all caught up. I hope you are all well. Talk to you soon.

Take care,
Christy