3 months old

Well, we had to go to the pediatrician today. Harlie hasn’t been feeling well since Saturday. She had way more secretions than ever before so we were constantly suctioning her (which makes for a very long day – and night!) Luckily our night nurse is great. She is the one that works at my pediatrician’s office. She offered to come over Christmas night to stay with Harlie so we could get some sleep. After spending all night watching her, she suggested we take her in just to be sure. They cultured her secretions, so we’ll see what comes back. I’m hoping for nothing since I think she’s improved a lot since Saturday. Well, we had to let our day nurse go on Friday. That just wasn’t working out. So, for now we only have our night nurse. Hopefully the agency will send someone soon. Oh, Harlie weighed in at 8 pounds 1 ounce. We are really happy with that because that is 5 ounces in 5 days! And after struggling a little bit this weekend, we increased her intake again. I think because she didn’t feel good this weekend, it took her a couple of days to get adjusted to the increased amount. But it looks like she’s adjusted now, so that’s good. It is so great to see the weight on her. I have been so focused on her medical care that it was hard for me to see the “baby” in her. But I think I’m getting better at seeing her in a different light now, which I am loving. We added some new pictures so you can see the changes in her, too. I hope you like them. Well, I hope you all had a great Christmas. We had my family over and it was really nice to have all that craziness in our home – in a good way, I mean! It made me realize that although Harlie hasn’t been the luckiest baby in town, she sure was lucky to be born into this family of ours. Everyone loves her just the way she is. Okay, talk to you soon! -Christy

Merry Christmas

We had our weekly visit to the pediatrician today. Harlie gained 5 ounces in 7 days. She now weighs 7 pounds, 12 ounces. She will be 3 months old on Christmas. Overall, her health seems good. You can definitely see her weight gain now, which is really nice for us. She is also staying awake for longer periods of time, which is also really nice.

Well, it is late so I have to cut this one short. I hope you all have a Merry Christmas.
-Christy

Cardiology appt.

On Friday we had an appointment with the cardiologist. I think it went well. He is very nice and he spent some extra time teaching me “cardiology 101” to help me understand Harlie’s heart defects. Basically, they didn’t want to do her surgery before she was 2 months old. They wanted her lungs to be fully developed. And they want her to be bigger and stronger. But, they also can’t wait too long. Since her heart is having to work much harder than it should, the longer it goes like that, the more “muscle bound” it becomes, and that will make the surgery and recovery more difficult. I will be really glad when her surgery is behind us. March is really not that far away now. Hopefully we will be able to stop using the pulse ox monitor afterwards. It will be nice to lose that cord. He also told me that he will be surprised if she makes it through the winter without having to go back into the hospital. He told me that even though we are doing everything we can to keep her healthy, with the trach and everything else she has going on, getting sick and not being able to fight it as well is normal. We were glad he told us that. Now we won't freak out - as much. But we're still going to try to keep her home for good, of course.As I was leaving the doctor’s office, I passed a mom of a girl that was in a wheelchair. She saw my notebook. (For those of you that don’t know, we had to put a notebook together with dividers for each of Harlie’s problems. It keeps us very organized. It is a 4” binder now.) Anyway, she saw it and said that she has one, too. Then she touched my arm and told me that it will get better. Here was a complete stranger of a girl that has far more severe and permanent disabilities, and she was comforting me. I thought that was the nicest thing ever. Well, I have to go. I hope you are all well and I’ll talk to you soon.-Christy

She's catching up.

So, we went to see the pediatrician today. She weighed in at 7 pounds, 7 ounces - just one ounce less than our goal. We are very happy with that. Tomorrow we go to the cardiologist. Hopefully, all will go well with that. I am expecting it to be pretty routine.

I just want to say thank you to all of you that have provided meals to us. It has been so great to not have to worry about cooking! If I had to cook dinner every night, we would have starved by now. So, you've helped us a lot and we really appreciate it.

Thank you for everything!
-Christy

First PT appt.

Today we had the Early Intervention Program appointment. A physical therapist, occupational therapist and coordinator came over to evaluate Harlie. After a thorough evaluation, it seems that she is in the 25th percentile as far as her development goes. I was actually pleased with that. Considering she was sedated for the first 3-4 weeks of her life and didn’t get to be held on a regular basis until she was 6 weeks old – I think that’s pretty good.

They put her on her belly for the first time. I was too scared to do it on my own. But, as it turns out, she did really well. They showed me some tricks to make it easier on her with the trach. They are going to come once a week and teach me new exercises to do with her to help her along.

Tomorrow is another pediatrician appointment. We are really hoping that the continuous feeds and Zantac have helped her to gain some substantial weight. They want her to gain an ounce a day. So we’re shooting for 7.8. We’ll see….Well, time to go.

Thanks for checking in. I hope all’s well.
-Christy

Eye Update

Today we went to see Harlie’s eye doctor and eye surgeon. They said that her eye is looking good. It has definitely improved over the last month. She is now able to close it when she cries and yawns. Sometimes it is even closed when she’s sleeping (most of the time it is open when she is really relaxed). The doctor’s concern is exposure and her inability to protect her eye all the time. But so far, so good – no scratches or anything. So, we’ll just continue to put ointment in it and let her grow and see what she can do in time.

The eye surgeon said he will have to remove her dermoid (a growth on her eyeball). He thinks that the dermoid is preventing her bottom lid from coming up. He will close the opening to try to make it even with her other eye, but he will not repair the cleft in her eye lid. He said that could do more harm than good at this point. The best part is that they don’t need to see her again until February. Hopefully, her doctor appointments will start to spread out a little more (this week she has 4 appointments).

We’ve been doing the slow continuous feedings through the night for almost a week now. Overall I think it has been going okay. As much as I hate getting further away from “normal”, we are really appreciating being able to get more sleep. Now I only have to get up once to refill the bag, and to pump (and then suction her as needed throughout the night). Hopefully it is working and putting more weight on her. We’ll find out on Thursday. On Wednesday, the occupational and physical therapists are coming out to see her. I’m anxious to see what they will say and do. Well, that’s it for tonight. Thank you to all the visitors we had last week. It was really great to see you all!
-Christy

THE HARLIE FUND

We have had so many requests for an ongoing method to help Tom and Christy in their care of Harlie that we have set up a legal trust, “The Harlie Fund.” Contributions can be made to the Fund to help with the costs incurred in taking care of Harlie’s current and future needs.

If you wish to donate, checks should be made payable to “The Harlie Fund” and mailed to:

The Harlie Fund
c/o William L. Jeffries, Jr.
Kane, Jeffries, Cooper & Carollo, LLP
1700 Bayberry Court, Suite 103
Henrico, Virginia 23226

We are so appreciative of all the support shown to us all. Tom and Christy have gained strength from your thoughts and prayers. We thank you from the bottoms of our hearts.

Sincerely,
Elaine Staples (Christy’s mom)
and
Sandy Jacobson (Christy’s sister)

Feeding Clinic

So Harlie now weighs 7 pounds. Although it is good that she is gaining weight - the rate is slower than what the doctors would like. We have tried to increase the volume, but it seems that her stomach can’t accommodate that and she spits it back up. So the new plan is to continue the day feedings as usual (2 oz. every 3 hours) and at night, put her on a slow continuous feed over 10 hours (slowly increasing the volume given at night over the next few weeks). The goal is to get her to take in more calories (and keep them) and gain an ounce a day (she’s been gaining about a half an ounce a day). They also put her on reflux medicine to see if that helps. We are really hoping that this new plan works. It is hard having a 10 week old baby that still doesn’t weigh as much as the average newborn (and she wasn’t a preemie). I feel like she’s going to be a newborn forever! I think the magic weight for a baby to be able to sleep through the night without needing to be fed is 12 pounds. At the rate we are currently going, she wouldn’t be 12 pounds until May – and she would be 8 months old! They ordered a barium swallow and upper GI. Unfortunately, the soonest we can get in for that is sometime in January. It is definitely going to be a long process to get her to eat by mouth. One problem will be that eating by mouth will make her burn more calories – so we’ll be fighting that again. What we’ll probably do is feed her a little by mouth and then give the rest through the tube. I really wanted to get rid of the tube – but considering all the surgeries she has ahead – the tube will be essential to her gaining weight. And considering that they want her to be as strong as possible for her heart surgery - eating by mouth is not a priority. Well, it’s time to go. I hope you are all well. Talk to you later,Christy

I'm back...

Hi Everybody,Sorry I haven't updated the site recently. The past week was pretty tiring. Out of the 20 weekdays she has been home, she has had 9 doctor's appointments. Tomorrow will be especially busy with 2 appointments. At our last pediatrician's appointment, our doctor said that Harlie had caught a virus. She's been on antibotics and she has eye drops for the virus in her left eye. I guess it was just a matter of time for that one. Although we have been very pleased to notice that she's able to close her left eye when she's crying and sometimes even when she's sleeping. That is a big improvement. So maybe her muscle tone was just a bit underdeveloped. Tomorrow we go to the Feeding Clinic at the Children's hospital here in Richmond. I think they are going to run some tests to make sure that it is safe to start to try to feed her by mouth. I think they will have to do a barium swallow. Anyway, I know that this process will be a long one - but I can't tell you how wonderful it would be to feed her by mouth. On a fun note, Murphy's school is doing a Christmas "show" tomorrow night. He'll be singing with his class. That should be funny. Our nurse is off tomorrow, but she is rearranging her schedule so Tom and I both can go. I think we might have gotten lucky with our nurse. She is very accommodating and seems to care about Harlie and her well being. Well, it is late. I will update the journal tomorrow night to let you know how the appointments went. I hope you are all well. -Christy

Pulmonary appt.

So, we had the pulmonary appointment today. To be honest – it was a little bit of an emotional day for me. I guess being in that waiting room with other parents with their special needs kids – well I guess it took me back to being at the hospital again. We are part of a group of people that no one wants to be in and that’s just the way it is. I did get to talk to another mom – her 3 year old daughter has a trach. I think of Murphy and all the joy that we get out of hearing him laugh and talk (at 2 years old) and that mom has never heard her daughter do those things. We know what we’re missing, and that just makes us sad sometimes. We changed her trach on Sunday and in the split second the trach was out she made a sound. I had the new trach in before we even realized that she made it. Another mom told me that they would take a few seconds to put the new one in so they could hear their daughter cry. Ugh – the simple things we take for granted…never again!Well, overall, the appointment went fine. He educated us on all this oxygen stuff we’re dealing with. I’ll spare you the details – basically, because of her heart defects, we cannot expect her oxygen saturation levels to be in the high range (like a normal person). Her “normal” is just lower than usual. So, until they repair her heart, she’s going to need supplemental oxygen. And your body needs oxygen to grow – so clearly we don’t want to hold back now! We feel much better now. We were thinking that it was a goal to get her off the oxygen – and we were failing. Now we know better and that makes it much easier for us to handle and accept. Our nurse started on Monday. It was weird to have a complete stranger in my house taking care of my baby. But I think the relief of having some help outweighed the uncomfortableness. She will work M,W,F totaling 40 hours a week. We’ll see how that goes and make adjustments as necessary. Have you seen that commercial where a mom is talking to the babysitter and you think that they are going out to see a movie – but then the mom just walks into the living room to watch the movie on their own TV? For some reason that comes to mind. Well, that’s it for tonight. Hope you are all well.-Christy

2 Months Old

Hi Everybody,

I hope you all had a very Happy Thanksgiving. We had a nice time here at home with family. I was very thankful to have my little family all safe and sound at home.

Well, we went to the pediatrician on Friday. It was a big day for Harlie. She got 6 shots!!! After all she's been through, she took it like a champ. I, however, did not handle it as well. She had the normal 4 immunizations, plus a flu shot and RSV shot. Our doctor was very nice and went on ahead and gave all 4 of us flu shots so Tom and I didn't have to go elsewhere.

Oh, Harlie weighed in at 6 pounds, 9 ounces and she is 20.25 inches long. We are so happy to start to see some weight on her. I notice it most in her head and her fingers.

Well, as pretty much expected, Harlie and Murphy had fevers for the next 2 days. Harlie seems to be getting better. But Murphy is getting worse. Just a typical cold, but I am dreading Harlie getting it. Since the trach stops the air flow and secretions from making it to her mouth and nose - everything will have to come through the trach. Basically, it would be like having to breathe through your stopped up nose - and not through your mouth - when you have a cold. So a typical cold for her could be very bad.

Unfortunately, we are really battling the oxygen. We had to go higher than we ever have this weekend. We are hopeful it was because of her fever and that we will be able to wean her back down soon. We had to up her heart medication and reduce her fluid intake a bit to help her heart not work so hard. Luckily, we have an appointment to see the pulmonologist on Tuesday. Hopefully he will be able to shed some light on what we can expect in the near future.

On the positive side, we have a nurse starting tomorrow! She came over on Wednesday night to meet us. She seems nice, but it is absolutely TERRIFIYING to leave our special little girl with a complete stranger. The nurse will be here 3 days a week and 2 nights a week. I will admit that as terrified as I am, I am really looking forward to getting a break. I am actually looking forward to cleaning the upstairs, doing laundry and being able to freely roam around the house! (because of Harlie's equipment she has to stay in one place and I always have to be very close by).

In time, I am hopeful I will be able to run some errands and maybe do some things with Murphy outside the house. Well, it's time to feed Harlie.

Take care and talk to you soon,

Love,
Christy

Appointments in progress

Well, I have been very busy the past few days setting up appointments. Henrico County has an Early Intervention Program (well every county has one) that provides occupational, physical and speech therapies for county residents. The coordinator came over on Monday to complete the paperwork and get the evaluation underway. They have a month from now to get that done. I guess they will determine what her needs are and set up a plan to help her.

I also spoke with the Feeding Clinic at the Children's Hospital here in Richmond. Evidently their Feeding Clinic is very well known and people come from all over the country for help. She said that she will get us in within the next two weeks. She is going to get us an appointment with a doctor that I called last week and was told the next available appointment was Feb. 27!!! Isn't that something? I'm very glad that we'll get to start that so soon. I am very anxious to see when we can start to feed Harlie by mouth.

I talked with a few nursing agencies. So far, no luck finding someone. I'm thinking that the holidays aren’t the best time to be looking (especially Thanksgiving week!). I've been told that we probably won't be able to find anyone for the night shift. Originally we asked for 3 nights and 3 days, so I asked for 5 days as a backup. Maybe that will help.

It looks like we'll be going to see Dr. Magee (plastic surgeon) in Norfolk in January. I am hoping that he'll be able to give us a good idea of what we can expect in the next few years. I think just hearing what he can fix and what he can’t will help me a lot. Overall, I think Harlie is doing really well. And I think Murphy is doing well with her, too. He doesn't seem to notice her issues. Except tonight, he pointed to one of the scars on her face and said she had a boo boo. He likes to kiss her on her head. Tonight he said, "Night night Harlie, see you later". It was pretty funny.

Well, Tom and I are hosting our first Thanksgiving. Tom will be doing the cooking, of course. (You would only want me to cook if you wanted nachos.) I am really looking forward to it. We do have a lot to be thankful for this year. Even though it breaks my heart to think of all Harlie has to go through - she's here.

Well, that’s all for today. I hope you all have a Happy Thanksgiving.

Talk to you soon,
Christy

Photos are up

As you can see, we added some recent photos. Look in “My Baby Albums” for more.

The clear plastic mask-looking thing you see in the photos stays in front of her trach as much as possible. It is called a trach collar. It heats and moisturizes the air she breathes, since her mouth and nose can’t do that for her. That equipment is not transportable. So, when we go out we have to put an HME (heat moisture exchanger) or “artificial nose” over her trach. You can sort of see one in the baptism photo. That device has some spongy-type material in it that captures the moisture when she exhales and then puts it back in when she inhales. But she can only be off the collar for small periods of time. The dry air can dry her secretions and could plug the trach.

I’m told that when we get through the winter and she’s stronger, we can start to take her off the collar more and for longer periods of time. That will certainly be a welcome change.

So, I finally had a few minutes to spend some time on the computer and do some searching. I know that you are all busy - but just in case you are interested, I posted some links under "My Favorite Links". I found a "Famous People who have had Trachs" page. Pretty funny.

Well, it's time to feed Harlie. Talk to you soon,
Christy

Pampered Chef Fundraiser

There is an online Pampered Chef fund-raiser being held for Harlie that will run until Sunday, December 10th. We want to have a fund-raiser for her and her family because, even with insurance, medical bills add up very quickly. The Pampered Chef donates 15% when a fundraiser goes to $600 or above and I am donating all of my commission from the sales. Thank you in advance for your support!

There are three ways to help Harlie and her family.

#1. Please send this to all of your friends, family, co-workers, and neighbors. You'd be surprised at how many people who might not know Harlie would still be willing to help. We are a kinder society than we get credit for.

#2. Place an order, (instructions follow).

#3. Book a catalog/online show. For every "booking" received, The Pampered Chef donates another $3 to Harlie. I know it doesn't sound like much, but it can add up. If anyone wants to book their own show, I can mail catalogs or the show can be done online, just like this one. This is a great way for you to earn something for free or reduced price that might be a little out of your price range to buy at regular price.

Things to know before placing an order:

We have 60 new products that became available Sept 1st. You can view them now on the site. If you have any questions, e-mail me at ginabarnes@comcast.netHow to order:

1.) Go to my site: (there is a link in "My Favorite Links")www.pamperedchef.biz/ginabwithpc

2.) Click on "Order Products" at the bottom of the page.

3.) Go to option 1. “Already invited to a show” Type "Harlie" in the first yellow block, then hit search.4.) Click on "Harlie/Kristie Christiansen" in blue (Kristie is Harlie’s Aunt).

Start shopping!!

Other information:

Show closes on Dec. 10th so get your orders in now.
You will have your products by Dec. 18th.
Please choose to have your products shipped to your own home (unless you live really close to Aunt Kristie in Erie, PA).
Shipping costs begin at $6 and max out at $14, no matter how high your total gets (combine orders with friends to save on shipping).

This fund-raiser is a great way for you to take care of some of your holiday shopping. This is also a wonderful time to pick up those tools you've been wanting for your own kitchen, or entertaining needs. We have many products priced under $15 that are perfect for hostess gifts, or little thank-yous for the people who have been kind to you all year.When you place your order, you can use your credit card and feel safeknowing it is a totally secure transaction.If anyone reading this is considering becoming a Pampered Chef consultant, I would love to answer any questions you might have. I love the flexibility and FUN in doing this. It works great as a part-time, full-time, or hobby job. If you sign with me, I will give you a present when you qualify. And yes, we can be on the same team from different states. :-)Thank you for your time. Let's all try to help this wonderful family lighten their financial burden. And let's all continue to pray for Harlie.Thank you again!Gina Barnes, Independent Kitchen ConsultantThe Pampered Chefwww.pamperedchef.biz/ginabwithpc(301) 570-0206Knead dough? Talk to me about our part-time, full-time and hobbyopportunities!

Dr. appts.

So, she gained 5 ounces in one week! She's now a whopping 6 pounds, 3 ounces! We are so glad to see that she's gaining weight. We even upped her volume again - to 55 ml every 3 hours. Before each feeding we check her residuals. That means we use a syringe to pull the stuff out of her stomach to see what is left over from the last feeding. Since she can't tell us if she's hungry - or NOT hungry, this is the only way we can make sure that she's digesting all the food that she's been given, and that she has room in her belly for the next feeding. Lately she's had virtually no residuals, which tells us she's ready for more volume per feeding.

We saw the cardiologist today. Everything looks good and on track for her next surgery. We are shooting for March. They want her to be bigger and stronger - and out of the winter season.

As far as the oxygen situation goes, as long as she responds to the oxygen positively, then we know that it is not a heart problem, it is a respiratory problem. If we put her on oxygen, and her numbers don't go up, then we know its a heart problem. So, we were glad to hear that.

As far as the surgery, it looks like they are leaning towards doing a "double switch". I don't know if we've given details of her heart defects... but she has "congenitally corrected transposition of the greater arteries" (her left ventricle is on the right, and her right ventricle is on the left), and "VSD" (which is a hole between the ventricles of the heart). With the transposition (in basic terms), the big ventricle is supplying the blood to her lungs and the small ventricle is supplying the blood to her body. So, the small pump is doing more work than it should, and the large pump is doing too little. That's really all I know and understand right now. I do know that the surgery is a big one, but we can't think about that now.

Well, that's about it for tonight. Talk to you soon!
-Christy

Stuff

So, we had the evaluation today to determine how many hours of nursing care we qualify for. We qualify for 12 hours of nursing care a day. It's pretty weird (and scary) to know that we have a situation that requires that kind of care. Now we just have to figure out what kind of schedule we want. Then we have to find the nurses to fill the positions and hope that we are a right fit for each other. The coordinator said that most of the nurses like babies, so maybe that will make it a little easier (I hope!)

Tomorrow we have another pediatrician appointment. I think we will have those weekly for quite a while. Then in the afternoon we have our first cardiologist appointment here in Richmond. I am anxious to see what he has to say. I am getting a little concerned about the oxygen situation. Now it seems we can't take her off. I hope that doesn't mean something bad is going on with her heart. Hopefully he can reassure us a bit.

I also spoke with the county regarding the occupational, physical and speech therapy program. They are coming out on Monday to start that. I am anxious for that, too, (well, clearly I am anxious for everything) because occupational therapy will help us with trying to feed her by mouth. I am not expecting much, because in order for her to pack on some weight, we need the g-tube - for now anyway. Other than the developmental delays not eating by mouth causes, I would like to have one less tube to have to deal with. It is a pain with her baths (can't get it wet until it completely heals) and it is a little awkward with her clothes. My mom put some button holes in her clothes for the tube to stick out so that should help. Until she did that, Harlie could only wear button-up clothes, which surprisingly, is pretty limiting.

Well I spoke to the mom of the little girl that had a trach today. That was such a great conversation to have! As it turns out, her daughter didn't have to have the expansion surgery as I thought. Just her normal growing opened up her airway enough to remove the trach. That's even better news! There are definite negatives with doing that kind of surgery at such a young age. So, hopefully, it will happen on its own for Harlie, too.

Well, it is getting late and it is time to get Harlie upstairs. Take care and talk to you soon!
-Christy

7 weeks today

So I didn't hear from the person who is supposed to do the evaluation for home nursing. Hopefully I'll hear from her tomorrow. At first I was pretty against home nursing. But, after more than a week at home with Harlie on our own, I can definitely see some benefits. I have a ton of questions that would be best answered if someone could see what I'm talking about. So, we'll see what happens.

My pediatrician gave me the name and number of one of her patients that had a trach till she was two. I will try to call her tomorrow. It would be so nice to talk to someone else who has been through this before. Her daughter didn't have Goldenhar's, but she had the underdeveloped jaw (called Pierre-Robin) and they were able to do a procedure to expand her jaw enough to secure an airway that allowed them to remove the trach. We are hopeful that the same could be done for Harlie. The sooner that trach is out, the better for all of us.

I am told that most of the repair to the jaw is done after permanent teeth come in. As heart breaking as that is, we would much rather do what's best for Harlie long-term. Of course, I have already spoken to Dr. Magee (founder of Operation Smile, where I used to work). We had a good conversation over the phone when Harlie was about 2 weeks old. (For those of you that don't know, he is a cranio-facial reconstructive plastic surgeon.) I was feeling very overwhelmed and incredibly sad at the time and he made me feel better. So, as soon as we are able to make the trip to Norfolk, we'll go and see him and hopefully get some sort of plan.

After spending more than a week with Harlie at home, I can finally say that I'm getting to know her. She makes the funniest facial expressions! She was awake a lot today which was really nice. Well I wanted to post some pictures up, but the night has gotten away from me and now I have to go.

I hope you are all well and I'll talk to you soon!
Love,
Christy

Friday

So we went to see the pediatrician today. I actually feel bad for her (the pediatrician) because she certainly has her work cut out for her with us! I am so glad that her nurse knows so much about trach care, too. Today she gave me a mini-lesson. It is very clear that I definitely need some home nursing care. It would be so nice to ask someone what her noises mean, whether I should suction or not, use saline when I suction, or not, is it normal when she does this or that, etc.

The coordinator is supposed to come to our house on Monday to do an evaluation to see how many hours of home nursing we need. So, we'll see how that goes.

Well, Harlie gained 3 ounces since last Friday. So, that's good. Her doctor increased the volume a bit, so we'll see how she tolerates that. Maybe we'll pass the 6 pound mark by next Friday.

So, a few weeks ago we were having chinese food for dinner. For once I got a good fortune in my cookie, "You will have good luck and overcome many hardships". I thought that was kind of funny. I think it is kind of hard to have good luck AND overcome hardships. Seems to me if you have good luck you shouldn't have to overcome hardships - but I'll take it.

Well, gotta go. Talk to you later,
Christy

Eye Appt.

Well, we went to see the eye doctor today. Overall I think it went well. Unfortunately, we won't be able to do anything to repair her eye till she's at least 6 months old. But, at least now I understand why. And that makes it easier for me to accept.

Her eye lid problems are pretty complicated. We have to see exactly what her muscle ability is before they go and do any kind of surgery. The obvious fix could possibly damage her vision - and it just isn't worth the risk. The good news is that her eye anatomy appears to be good, so it is assumed that she can see fine. So, she will be monitored monthly until surgery.

Well, we are still battling the oxygen thing. She seems to do well off of it for 12 to 16 hours, and then she needs it again. I guess this will be a longer deal than I thought. We will see a doctor about that at the end of the month. Just more stuff to learn I guess.

We see her pediatrician again tomorrow morning. I am very anxious to see how much she weighs. She is such a string bean right now! She really needs to get some fat on those skinny cheeks! Well, I think that's it for today.

I hope you are all well. I don't know if I've said it before - but thank you for all the kind words you have posted in our guestbook. I just can't tell you how much it has meant to us.

Love,
Christy

Settling in

So, things are going well, I think. We are certainly getting the hang of some things - and still trying to figure other things out. I think I finally got the feeding thing sorted out. Getting the milk to go into her stomach at the right rate has been trial and error so far. We tried gravity feeding for a while, but the supplies sent to us weren't really appropriate for that. So, yesterday I tried using what they sent us (bags with a feeding pump). Today, the feeding pump was giving me trouble. It wasn't letting milk get through the tube. So, I fiddled with some things and then all of a sudden - the milk was gone! She got fed in about 5 seconds!! Luckily, she tolerated it and didn't spit it back up. But I still felt terrible. But, now I'm straight. So, that won't happen again, I hope. Other than that, I think things are going better than I thought they would. We took her off oxygen this morning and she seems to be doing okay without it. Hopefully, she'll stay that way. We have an appointment with an eye doctor here in town on Thursday. Her left eye has been the hardest thing for me to get used to. It just looks like it would be bothersome. And I hate to think that she can't protect it. The doctors at Children's say it isn't urgent - but that just doesn't sit well with me. I want a plan. So, we'll see what this doctor has to say about it. Well, it's feeding time, so I gotta go. I hope all is well. Take care,Christy

From the Social Worker

Dear Family and Friends of Tom, Christy, Murphy, and Harlie, None of you know me, but I am the social worker who has been working closely with Christy, Tom, and Harlie at Children’s National Medical Center. It has been a privilege getting to know Christy and Tom, and also to journey with them as they welcomed precious little Harlie into the world. Tom and Christy have shown me that they are loving, caring, devoted, assertive, and wonderful parents. And I have no doubt that Harlie has the best possible parents to be by her side throughout her life’s journey. How fortunate Harlie is to have been born into the family that she was. As you all know, Harlie was born with numerous medical complications. She was born with a series of congenital anomalies (that’s medical speak for things that turned out a little bit different for Harlie) which are part of a syndrome called Goldenhar Syndrome. The doctors are hopeful that all of Harlie’s complications can be repaired. (If you would like to check out a website with some good information you can go to: http://www.goldenharsyndrome.org) Both Christy and Tom encourage you to ask questions if there is something you do not understand or would like to learn more about.Because of Harlie’s syndrome, she has undergone numerous surgeries. She underwent open-heart surgery a few days after birth in order to begin to repair her congenital heart defect (this will require several surgeries during the next year or so). She also had a tracheostomy and a g-tube placed. Harlie needs the “trach” because she has an underdeveloped jaw, which makes her airway small, and unstable. The trach ensures that Harlie will be able to breathe. You will notice that the trach means she has a hole in her throat – below the vocal chords. This means that no air passes through the vocal chords, thus she cannot make sounds like a normal baby. So, when Harlie cries, the noise sounds a little funny. This is normal and you will get used to it. Harlie was given the G-tube, because the doctors expect that feeding will be somewhat of a challenge for Harlie because of her small airway and chin. The g-tube ensures that Harlie will get all the nutrition that she will need, since she may not be able to take a bottle for a while. Over time however, as Harlie gets bigger, she will eventually not need either the trach or the g-tube. Harlie’s most recent surgery was to remove 3 “skin tags” (which are little pieces of extra skin that sometimes form on people, for reasons which doctors don’t understand…these are actually quite common) from her face. These were removed quite successfully.Harlie will be having many doctors’ appointments for the next many years of her life. She will also have a few surgeries in the coming years to help her jaw form correctly, to repair her eye (she has a cleft in her eyelid, and the opening is larger than mine and yours – it also appears that she lacks the muscle tone around her left eye which leaves her unable to blink or shut her eye), and her left ear which is a little wrinkled and misplaced. She will have further tests done on her left ear to see if she has an ear drum (there is no opening to that ear to look inside and the doctors are unsure whether she will have hearing capability in that ear). The journey with Harlie will no doubt be a challenging and busy one, but it will also be filled with lots of joy and love. I want all of you to prepare yourselves to meet a beautiful little girl. Harlie certainly has a few unique features about her that make her look a little different. But she is adorable! She has perfect little hands (with looooong beautiful fingers) and feet, and her big brother’s same gorgeous blue eyes. She is long and skinny and has the same cute little knees that Murphy had, that make Christy laugh every time she looks at them. Harlie is a petite, precious, little bundle of joy! However, at the same time, Christy and Tom both know that Harlie does look different and that she does have significant complications that will be challenging and stressful. It will be most helpful to Christy and Tom if you don’t try to minimize the issues Harlie does have, because this makes parents feel invalidated as if they are “only focusing on the negative.” Christy and Tom are Harlie’s advocates and they have to address her complications realistically and assertively to ensure that the doctor’s do all they can to help Harlie have the best life possible. You will be most helpful to this family by being present with them, loving them and loving Harlie. I know from experience that parents are often very apprehensive to show their child to others when they know that people will look at their child and notice their “differences.” But I challenge you to notice the beautiful, perfect things about Harlie! There is a lot to notice. I know that Christy and Tom are grateful for all of your support. They are very lucky to have each and every one of you. Please take care of yourselves, and take care of this very special family too!Fondly, Kristen Caminiti

Lazy Sunday

Ahh, it's Sunday and we have no where we have to be. What a great feeling - one that we haven't felt in a very long time.

Well, Harlie's first pediatrician's visit went well. She is definitely growing - check out her growth chart. We love her doctor. She has another family that she takes care of that has a daughter with some of the same problems that Harlie has and she had a trach, too. So, that's comforting. And a nurse there is also a home health nurse on the side and she takes care of a girl with a trach, too.

I wish she could be our nurse. Getting a complete stranger to come in and help take care of a special needs baby is pretty scary to me. So far things seem to be going smoothly. We've only had a few minor heart attacks. I suppose we'll get used to that, too! One thing we were supposed to do is contact the power company to let them know that she's on equipment 24/7. Tom's mom asked if we had done that yet and we said no. About 17 minutes later, the power went out. Don't you just love it? That pretty much sums up our last 6 months of life. Luckily, it came back on in a few minutes. Guess we'll be taking care of that first thing tomorrow morning.

Murphy seems to be adjusting well (all things considered) to having his little sister being home. It has got to be hard for him to understand that she gets fed through a feeding tube and she breathes through the trach. But it doesn't seem to bother him.

Well, unfortunately we had to put her back on oxygen this morning. Her numbers have been a little low the last few hours and this morning her alarm kept on going off. We tried a couple different things, but it didn't improve. So, I guess we'll see how it goes and go from there. Well, that's about it. Thanks for checking in and for thinking of us.

Hopefully, we'll get to see everyone soon.
-Christy

Late Halloween Entry

We have been so consumed with Harlie's arrival home that I wasn't able to tell you all about Halloween.

Murphy, Christy, and I went Trick-or-Treating on Tuesday for the 1st time. Murphy hung in there long enough for Christy to get her fill of candy...if that is actually possible!

Personally, I was pleased to see all of my neighbors walking around with beers in their hands...I had a very similar idea.Murphy was dressed as a chicken. Yep, you read that corret, a big white chicken. And apparently he was quite the hit at both daycare and in the neighborhood. Amazing all the fuss a last minute $20 Target costume will cause (it was the last costume in his size). Photos in the album.

Anyway, it was a nice night. Our friends Marcy and Mike came over and gave out candy at the house so Christy and I could both go with Murphy. Mike cooked up a batch of New Orleans Etoufee that was of course awesome. Murphy and Christy had a small candy buffet.

Take care,
Tom

Harlie's Home!

Well, my family of four now officially lives in the same house! On Wednesday morning, all the supplies and equipment was delivered. It was crazy. It took us a long time to get everything organized. I think we were trained on all the equipment in a few hours. They had to go and get oxygen because the hospital called to say that she was back on oxygen. Luckily, they seemed to wean her off the oxygen by Thursday. Hopefully, we won't need to put her back on. On Wednesday night we roomed in at the NICU. It was crazy. Their monitors were going off every two seconds, keeping us from ever getting any sleep. Harlie was fine, their monitors are just hyper-sensitive. They gave her the hour car seat test and she passed with flying colors. Then on Thursday we started all the discharge stuff, CPR, paperwork, last minute questions, etc. We had the best nurse ever and she hooked us up and kept things streamlined so we could get on the road before traffic. We definitely did not want to have her in the car for 3+ hours. We had her baptized at 1pm. And then we said our goodbyes to everyone and were in the car at 2pm. It was weird to leave. On one hand it was so nice to know that I didn't have to drive back anytime soon. And it is not easy to be at a children's hospital everyday. The things you see are not good. It is very hard to see so many children not healthy - and to see their parents. I think we all have the same look to us. But then on the other hand, it was hard to leave the shelter of the hospital. Everyone there has seen far worse and Harlie's issues are nothing to them. She's just another baby that they need to help. Now, we enter the real world with people that have never seen a baby with a trache (just like we were) or one that looks like her. And that is pretty scary. Last night went well. She slept soundly between feedings. It takes 2 of us to take care of her right now. Hopefully, soon we get good enough to do some of her stuff solo so Tom can sleep since he has to go to work. I'll just have to learn to take naps during the day when she sleeps. That shouldn't be that hard - haha. Well, we have our first outing in a few hours - to the pediatrician's office. We'll see how that goes. It is safe to say that we will not be traveling light for quite a while. I am definitely not going anywhere alone with Harlie for a while! Well, we have to get going. Thanks for checking in and thanks for thinking of us. I'll have more medical info for you soon (hopefully by Monday morning). Talk to you soon,Christy

Welcome to Holland

As Thursday approaches us with much anticipation, I cannot help but feel a flood of emotions ranging from complete joy - to absolute fear.

It was 6 months ago that we learned about Harlie's chest mass. Since that time, the story has grown much longer and continues to change everyday. We have been on a roller coaster ride since that day and I am absolutely sure this is just the beginning of what will be the ride of our lives.

A very good friend said something to me today that I really appreciated. He reminded me to try and take a moment (during these next 48 hours) and think about what it means to be bringing home our little girl for the first time. It has been so easy to be distracted with the medicine, with the logistics, with the traveling, and with the constant fear of what the future holds for us. So for now, I am going to try and push those things aside and enjoy some time with my daughter.

Tom

ps. I apologize I have forgotten who, but someone during those first few days told us about this letter. I want to share it with you all.

Welcome to Holland

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel.

It's like this......When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.After months of eager anticipation, the day finally arrives. You pack your bags and off you go.

Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland.""Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.
by Emily Perl Kingsley

Home Stretch

Harlie was awake a lot today. I am anxious to see her act more like a normal baby. I spoke to the nutritionist today and they are upping the concentration of her milk from 24 calories per ounce to 27. Hopefully that will help her put on some weight. I also found out that we can start to try to feed her by mouth this week. We had to wait because one of the things that goes with what she has is a T-E Fistula. That is when there is a connection or bridge between the esophagus and trachea. And when you swallow, it can go into the trachea, which is bad. But I was told that she does not have one, which is great. I am really hoping that she can eat by mouth without any problems. I know that it will be a gradual change - I don't think she has the energy to feed normally yet. But, it would be really nice to remove the feeding tube soon - anything to make things more normal. Plus, they can develop oral aversions when they don't eat by mouth.

So, we'll see how that goes.I must say that I cannot wait to be able to get up and not have to drive 2+ hours to see Harlie. I am SO over this commute. The mornings aren't so bad - it is the afternoons that kill.

Well, tomorrow night we are taking Murphy trick or treating for the first time. I hope he likes it. I'm sure that once he realizes he gets candy, he'll be all over it (just like his mama). Well, it's getting late. Have a Happy Halloween!!!

Wendy

Today was another good day with Harlie. This was the only time I was able to go see her this week. Christy needed the break and I was able to take the time off.

In short, next Thursday looks good for bringing her home. The basic plan is for us to get the equipment delivered on Wednesday morning and then get some training on it. Christy and I will then leave for DC for the night. We will be "rooming in" at the NICU. This will allow us to care for her throughout the night. Christy is getting better and better are caring for her. I am watching her for lessons. On Thursday we will be getting her ready to go to Richmond and gathering the 100's of pages discharge paperwork. Just before we leave for Richmond, the plan is to have Harlie baptized by the hospital chaplain in their chapel. That's about it for next week.

Christy will be continue to get trained next week and hopefully I'll catch on quickly. It looks like we will have a few days of private nursing initially when we get home so that should help on those first few days home.

On a very sad note, we had to leave the hospital Friday night and go to the vet for our cat Wendy. Unfortunately, Wendy had liver disease that got bad real quick. Unfortunately, we had to make the decision to put her to sleep. May she rest in eternal peace.For those of you who never met Wendy, I have a few recent photos of her in the album (putting up with Murphy's love...). She was a great cat who put up with alot of grief from her sister Annie and more recently Murphy. Wendy was always known as the fat one and she was as lovable as she was large. Christy has had Wendy for 13 years. She named her Wendy after "Wendy the Whiner" as all the kitten did when Christy met her was follow her around and whine and whine til she found herself a home. We will miss her dearly.

Take care and thanks for checking in.
Tom

Short one

Well, Harlie had a good day. She seemed very comfortable and slept a lot. Regarding her weight...I was told that because of her heart defects, her heart is having to work harder than it should. Which means that she burns more calories. That explains why she was so tiny and they told me to pretty much expect that she will be a more petite baby - at least until her heart surgeries are behind her.

But, they are going to get us a growth chart plan or something for her so we know what to expect. And they might look at other options to get her more calories. So, we'll know more later.

Well, to add to our currently hectic lives...I had to take Wendy (my fat cat) to the emergency vet tonight. She is pretty sick, and, technically not fat anymore. Her back legs gave out on her and she couldn't walk. The vet said that she is jaundiced and dehydrated. So, they are keeping her tonight and running some tests. Hopefully they will be able to let me know something by morning.

Well, it is late and Tom and I need to get on the road early tomorrow, so I need to cut this one short. I should have more info in the next few days.

Talk to you soon,
Christy

One month old!

So, one month has gone by. I'm not sure if I think it has gone by really fast, or really slow. I held her for a while yesterday and she was awake most of the time. It is really nice to see both her eyes open and alert. I am a little disappointed at her weight gain. She needs some fat on those bones! She seems to go up and down a few ounces, bouncing between 5.3 and 5.6. This morning's weight is 5.3, her birth weight.

They have a nutritionist who monitors that and they are fortifying her milk to make it have more calories. So, hopefully we'll start to see a steady rise soon.I spoke to the case manager yesterday. I think since we can't get her transferred closer to Richmond, we are going to try rooming in. They have a room right there in the NICU that is all set up for parents to sleep there and practice with their baby with the comfort of having nurses on hand. So, I think we are going to try to do that and then take her home as soon as we can (maybe next week). A lot depends on the equipment delivery, obviously. She still has some tests to go through, too. So, hopefully they will get them scheduled soon.

Well, I guess that's it for now. Talk to you soon,
Christy

My first trach change

Well, I had a big accomplishment today. I changed her trach.

I am very proud of myself. It wasn't as bad as I thought. Now don't get me wrong - it is definitely unsettling - but I learned that we can do this.

For those of you that don't know what I mean by "changed her trach"... it means I took out the tube that allows her to breathe from the hole in her throat, and put a new one in. It is kinda scary. So, for me, today was a big day.

I think I have now done everything at least once. No news on a discharge date or discharge plans. I think they are still working on getting all the equipment straight. We need to get the equipment and supplies delivered, set up in her room, and get trained on it all before we can bring her home. So, I don't see it happening this week. Maybe next.

Well, today was a long day and it is late, so I have to make this one short. I hope you are all well. Talk to you soon,
Christy

Aunt Mimi (Mandy) met Harlie today

What a difference a few weeks make! Grandma, Pap-Pap and Aunt Mimi went to DC today to visit with Harlie. We had her all to ourselves because Mommy and Daddy needed a day of rest!! I am happy to report that Harlie looks incredibly strong and rested. Continuing all her recovery like a little trooper.

She had an absolute wonderful nurse taking care of her today, who shared with us portions of her care and help us to understand what will be needed when Harlie goes home. Of course, let me tell you that everyone at the Children's Hospital have been great.Today was the first time Aunt Mimi met her new niece and like the rest of us, is extremely proud of our little girl. Just for everyone's information...Harlie likes her pacifier and hates poo-poo and pee-pee diapers. Such a little princess already!!Our hope is that soon Harlie will be home with Mommy, Daddy and Murphy. Plans are being made for her discharge as soon as all can be arranged. Her room at home is all ready for our "princess" to arrive and we thank God everyday for allowing that to happen. Again, thank you for you support and prayers.
Grandma Bowser

Now What?

Well, it appears that Richmond's Children's Hospital is full. That is, the Transitional Care Unit is full. We haven't had a chance to discuss any other options as of yet, I have only heard this via email from our social worker at CNMC. I guess we will discuss that some time on Friday. On the Harlie front, she continues to recover from her little surgery on wednesday. Christy was able to help the nurses do a few of her care items. Some are simple, some are intimidating. Bottom line is we are responsible for her airway and that is a little daunting.

Gonna get ready for work now.
Tom

Change of Plans - Maybe?

Well, Harlie had her surgery today and that went well. So, that looks like that might be it until her big heart surgery at about 6 months. So, instead of us just bringing her home from DC, they are looking into transferring her to Children's Hospital here in Richmond first. They call it transitional care or something like that. Basically, it will allow us more time to become comfortable with her day to day care that will be necessary. And allow us to get fully set up at home with equipment/supplies/etc.

The Case Nurse in DC will be working on that tomorrow. If Richmond's Children's has a spot open, then it could be within the next 4 or so days and she'll be here in Richmond (only 15 minutes away...in traffic!) That's a big deal to us, can you tell?

Well, that's about all the news from today. We/them started working on transferring some of her follow-ups to Richmond Doc's. That sure will help with the traveling that is hard enough on us, yet alone Harlie.

Thanks again for your continued support.
Tom & Christy

We can hold her!

Well, yesterday I finally got to hold Harlie. It is still a little intimidating because she is still connected to everything - but it was wonderful anyway! They did the first trach change yesterday, too. So, that seems to be healing well. I learned how to suction the trach and have done that twice now. A little scary, but I can see that we'll get used to it with time and practice.

She has another small surgery tomorrow morning. And we're thinking that once she recovers from that, we'll be able to bring her home (maybe next week). They have started to get all our stuff in order in preparation for discharge, so I'm thinking that's a good sign.

Today she took a pacifier for the first time. It was wonderful to see her doing something a normal baby should do. It was actually quite a big deal to us - given that she's not had the opportunity to suck since she was born. Feeding her by the G-tube can have negative impacts on the development of her mouth motor skills. So we are hoping that if she will take a pacifier, that might help. Well, it is getting late. Talk to you later.

Back On-Line

Well it turns out that Babysites.com was doing system maintenance all weekend so we weren't able to update Harlie site. Anyway, as far as Harlie goes she is doing well. Assuming her last weigh-in was correct she is on the upswing with her weight. I visited on Saturday with Scott and I was able to play dad...change two diapers. Never thought I'd look forward to that...

This week should begin the training of Christy and I learning to take care of her needs at home. Too me it doesnt seem too scary, but i suspect that may change one we actually have her at home. But, I know we will do just fine. All in all, I think the end of the month still looks pretty realistic for having her home.

On a different note, Murphy and I went to the pumpkin patch on Sunday. Photos in the album at the front of the site.Oh yea, and the Steelers finally won!

Take Care,
Tom

2nd Post-Op

Harlie is recovering well from her surgeries yesterday. She's still on the ventilator, but hopefully they will be able to wean her off tomorrow. It will be one less thing to have her hooked up to.

Unfortunately, the ENT docs don't want her being moved around until after they do the first trach change, which should be Monday. So, that means that we'll have to wait a little longer to hold her. Evidently, things could come "disconnected" until it heals in place.

Then next week, the training begins. We'll learn how to change her trach, which will be done once a week, and of course, learn how to work her feeding tube. Right now, it's pretty scary, but I know that with some time and practice, we'll get used to it.

Well, Murphy needs a bath, so I have to go. Talk to you later.

The Day of Days

Well today became the day. Harlie had her 2nd and 3rd operations today. The trach and the G-tube are in. The operations went well with no problems thus far. The Doc's say she is doing fine and should be fully recovered by Mon/Tues next week. That's all great news. We should have her home sooner than originally expected.

I say this was the day of days as it was a pretty rough day for both of us. Without explaining the entire past 12 hours, lets just say the operations weren't scheduled for today as of last night. But this morning they were...And need me to remind the Doc's the importance of telling the parents that the procedure is complete before transfering the little one back to the PICU..1.5 hours later only to find her sleeping off the anesthesia 1 floor up.

And of course what day would be complete without a nail in the back tire!

Good Night and Good Day!
Tom

ps. we really are very happy the operations went smoothly and that our little Harlie continues to improve with every passing day. and of course, thanks for your continued support.

Just waiting...

Sorry I haven't updated the journal in a couple of days. The commute and days at the hospital have made me pretty worn out. So, I had to take today off to catch up on some rest. I felt really guilty about not going up there today, but the nurses told me that I won't be any good to her if I wear myself out. And they said that I have the best babysitters in the world right now, so I should take advantage of it and not worry. She is doing well. Monday was another good, quiet day. Today they moved her back to PICU. They are starting to prepare her for surgery again. I spoke to the ENT doctor today and they are trying to schedule her tracheostomy for Thursday, Friday or Monday. They are trying to combine a few procedures to lessen the number of surgeries. But getting 4 surgeon's schedules to coordinate is proving very difficult. We're crossing our fingers that it will work out.As much as I hate the thought of her having more surgeries, I know that it gets us closer to being able to hold her and bring her home. I am trying not to be too nervous about her trach (taking care of it, I mean). They said that a nurse will come to our house for so many hours per week to help us. Hopefully we'll learn quickly. Well, I suppose that's it for tonight. Thanks for checking in, and thanks for your continued love and support.

She's awake!

Another quiet day, and she just keeps improving. The nurse let me change her diaper, which was really nice. I know that may sound weird, but it was really nice to do something to take care of her. They have taken her off the meds that have kept her sedated so far. She still slept most of the day, but at the end of the day, she woke up. I mean, she opened her eyes and really looked at me. It was such a great feeling! Everytime she's opened her eyes before, it was just a drug-induced stare. But this time, she really looked at me for the first time.

I spend so much time worrying about everything and being scared, that when she looked at me - I just forgot about all that stuff and thought that everything is going to be okay. I wanted to pick her up and take her home right then!!! But I have to be a bit more patient. It sounds strange, but I can't wait till my little girl gets her trach. Hopefully we'll know the date soon. More updates later!

Saturday at the Hospital

Hello Friends and Family:I spent time with the little gal today. Both Grandma and I had a nice time with her. She was wide awake when we arrived and spent about a hour just looking around and checking out gramdma and dad. It was great to see those eyes moving around taking in the whole scene. As mentioned before, it's mostly quiet during the weekends. Nothing new to report although she is now eating like we do. She gets larger meals spaced out in lieu of a continous slow feed...at which point she fell promptly asleep...just like her old man!Big week next week. A few procedures to get her home and then the real fun begins."Say Goodnight and Good News."Tom

Grandma'a Days with Harlie

I am so happy to inform all you avid readers to Harlie's journal, that I Grandma Bowser was able to spend the last 2 days with our little darling! Each day Harlie is getting bigger and stronger. She only has an IV in for her medications, of course her breathing tube is still there (only for precautions-she is breathing all by herself) and getting more of mommy's milk everyday. We all are so pleased and happy for not only Harlie, but mommy, daddy and Murphy too! If Harlie could have picked her parents herself...she would have picked Tom and Christy! These two people have been thru some pretty tough times, but their love for their children has been amazing. Their love for each other stronger than ever. As Tom's mom, I may sound a little prejudice when I say I couldn't be more proud of my son. I have always felt that his choice for a wife couldn't have been better. I hope and I pray that thru the difficult times they may face in the future that they remain strong in their convictions and continue to grow in their marriage. They are truly committed to each other and their familiy.I have had the pleasure of spending two weeks as the babysitter for Murphy and now am ready to leave tommorrow to return to Pennsylvania. I will miss my "little man" but will be back when I am needed. Thank you for all the prayers, kind words and love you have poured out to Tom, Christy, Murphy and Harlie. I cannot express the gratitude I feel. Harlie still has many surgeries to face, the next one getting her trach and feeding tube in, hopefully at the end of next week. Then she can come home! With the hands of many trusted surgeons, all your prayers and most importantly, the Grace of God, we will get Harlie thru them.Again, thank you. Grandma

Day 10

Well, today was the first day of my new commuting routine. I think it will work. It was so great to go home last night and see Murphy and my house. It reminded me of who I am - it is amazing what being away for a month will do! I was afraid that I would forget how to be a mom or that he would forget - but we went right back as if I've never been away. (except we let him stay up late and eat M&Ms - which I would NEVER do normally!)

Well, Harlie is doing well. They transferred her to NICU today. I don't think that means much, just juggling around patients for space reasons. I think I'm going to like her there better because all the nurses are used to working around babies all the time. They said that I can bring in a mobile to put over her bed. That will be fun - it will be my first official purchase for her!

When I get down about all that she's going to have to go through in the next 5-7 years or so, I just remember that I get to go home and decorate her room. Already she's beaten the odds, so who knows what she'll be able to accomplish when she's bigger and stronger!

Well, thanks for checking in and have a great day!

We're Home

Yep, got here at 8:20 pm after another quiet day in the PICU. Harlie slept most of the day. All snug under a blanket and gettting lots of "Good Eats".

Looks like end of next week for the next round of surgeries. Then if all goes well, my little girl will be home a week or 2 (or 3) after that. Christy and the Mom's will be going up in the morning for the day. I'm going to work.

Well it's now 10:03 and I'm going to bed...my own bed!

"and that's the way it is."Tom

Nice and Calm Day

Well the title says it all. Today was simply that, nice and calm. Harlie is doing well with her post-op recovery. She had a few more lines removed today so she is looking less and less connected each day. Its a welcome change for us as we continue to monitor her progress.

Mom is also doing well. She is working on providing food for the little one and I think thats just great. Speaking of which, Harlie is on a slow continous feed now and hopefully her insides will do their job and we'll start seeing some weight gain during the next few weeks.

We decided today to leave the Ronald McDonald house tomorrow after our visit. Mom and I are pretty home sick...especially mom. The plan is for Christy to commute as often as she can (and I'll join her as often as I can). I suspect she will have a few volunteer travel companions as we try this approach. I think being home at nights with Murphy and sleeping in her own bed will be very good for all of us. Again, hopefully we can find a balance that works for our family.

Thanks to all for the kind words we get to read each night in the Guestbook. It is really good to hear all the support Harlie and the family is receiving.

"I'm Tom Holton, you stay classy Richmond"

Monday

Well, the family meeting has been cancelled. It was determined that it was too difficult to get all her doctors in one room (there are more than 10 I think). So, we are just going to start to meet with them one on one. Which, personally, I like better. The thought of being in one room with all those doctors was a little intimidating. They will still communicate with each other to determine the priorities of what she needs done and when. But now we can focus on one area at a time.

There was talk of removing the breathing tube (ET tube) today. They have reduced the air coming from the ventilator to practically nothing - so she is breathing on her own - room air. But, just a few mintues ago, it was determined that it was just too risky to remove the tubing at this time. Evidently, it requires a team to get her intibated, and it just isn't worth the risk. (clearly, we agree!)

I'm sure things could change, but it looks like in the next few weeks they are going to put the trach in and a feeding tube in her belly. I know we will learn how to take care of her - but it is a little unsettling to know that we are going to have to feed her through a tube in her stomach instead of, well, the normal way. I think our sense of normal, will not be normal anymore.

I am told that she won't be able to cry - well, make the noise I mean. And I have to admit that makes me sad. A mother should know her baby's cry, and I have no idea what it sounds like. And it makes me terrified, because what if she needs me in the middle of the night???? How will we know? They tell me that they have a way of communicating and letting us know that they need us. I hope they are right.

As far as how she is doing today, she is doing well. They were able to get all the swelling down. Now she looks REALLY skinny. They haven't been able to weigh her because of the ET tube, but clearly she has lost some weight. Just a few hours ago they finally started to feed real food for the first time (my milk). So, hopefully all will go well so she can start to pack on some weight. Well, that's all for now. Talk to you later.

Post-Op Day 2

She looked good today. The swelling is starting to go down. She looked pretty comfortable (all things considered) all day. The hospital is so quiet on Sundays. Her nurse said that they are going to try to let her breathe on her own tomorrow. They are reducing the amount of air coming from the ventilator. They are going to leave the tube in place, but try to cut it off so her lungs can work on their own.

I really hope we get some solid information tomorrow. The days are getting harder to get through with so many unanswered questions. Everything that is good or necessary, rather, seems to have a negative side effect, too. It is a constant balancing act. And one that if you hear too much - is overwhelming.

Post-Op Day 1

Harlie is doing well after her surgery. I think she looks pretty good. They are keeping her sedated for her comfort. She does have some swelling, but they tell me that's normal and they are working on that. There's not much to report on right now. Which I guess, is a good thing.

Tom went home today, but he'll be back tomorrow. He had to do some work and get in some Murphy time - and get me some clothes. The family meeting was postponed to Monday because of her surgery on Friday. I may have mentioned that already. Anyway, today was pretty exhausting, so I'm cutting this one short.

Day 4 - First Heart Surgery

It is 1:42pm and Harlie is now in surgery. I can't believe that my 4-day old baby is going through this. Today, they are putting a band around her pulmonary artery to reduce the blood flow to her lungs. This will help her overall blood circulation and will reduce the amount of work her heart has been having to do. They are also going to implant pacemaker wires (not sure if they are going to be installing the recording/battery device at this time). I am told that this is really no big deal and that she will never even know that its there.

She is having to receive blood transfusions. Unfortunatley, we just found out today. If we had known, we could have given her our blood because Harlie, and Tom and I are O Positive. The nurse said that if any of our loved ones are O Positive, and want to donate to her, they can. You just have to come here to do it - they don't take any blood given elsewhere.

Her recovery time after this surgery is 4-6 weeks. It takes 6 weeks for her sternum to completely heal, since they have to cut it to get to the heart. I wish they could cut mine instead, but clearly that wouldn't do her any good. Because the risk of infection is great after heart surgery - nothing can be done to her until after she heals.

So, the critical airway tube will stay until she can have a tracheotomy installed. They really want to get this done because she will be much more mobile and then we can finally hold her. This also means that she will stay here for at least 6-8 more weeks. The goal is to get everything done that we have to have done in DC, and then the other things, hopefully Richmond (or even Norfolk) will be able to provide. We all need to get home and be a family again. It has been 3 weeks since I left and I really want to be a mom to Murphy again. I miss him terribly.

6pm - Well, she's out of surgery and in CICU recovering. That CICU is a crazy place - way too stressful for a mom and dad. She looks good. Dr. Jonas said that she did great and all went well. They might have to go in later to adjust the band, but that's no big deal he said. (HA! - I love how these doctors keep saying that all this stuff is "no big deal") WHATEVER!

Now Dr. Jonas is saying that he wouldn't have a problem if they wanted to put the tracheotomy in after the next 10 days. See how the information keeps changing?? And let me just explain that I have NEVER been in the least bit interested in the medical field. The word "challenging" just isn't strong enough!

Day 3

She looks really good today. Her skin color is great and her eyes are not as swollen. Tom and I are getting used to seeing her like this. We are going to take some pictures tomorrow and post them. I hope that you are prepared.

We met with the heart surgeon today, Dr. Jonas. He explained what he will be doing, which I'll summarize for you later. After tomorrow's surgery, her next one will probably be in 6 months.

Spoke to Dr. Guzzetta yesterday. We finally got some good news! Her chest mass is significantly smaller than it was prenatally. And there appears to be working lung tissue in her right lung. Not only that, but back at 24 weeks, the chest mass was squishing her other organs (mainly her heart), and now everything seems to be almost exactly where it should be! They were shocked - don't know what happened, but we're thrilled. But, back at 24 weeks, they thought the mass was a CCAM III - now they aren't really sure what it is. So, they are going to do some more tests to figure out what to do. Most likely, that surgery will be postponed. The only thing that they will probably have to take care of in the near future is the blood vessel going from the heart straight to the mass. We'll know more about that later.

Now for some more bad news... they found something wrong with her spine in her lower back. Three vertebrae are out of line. So, they are running some tests to see if her spinal cord is okay. She moves her legs and feet, so I don't think it's that serious. They said - and imagine this - it's nothing that another surgery couldn't fix. She might have to wear a temporary brace in the future, but it is way too early to know for sure.

Baby News - Day 2

Hi everyone,

Here is the news:

Harlie Caroline Holton (fyi - Caroline means "little and strong")
Born at 1:11pm on
September 25th
5 pounds 3 ounces
18.5 inches long

I think you all know that we were anticipating the defects with her heart and lungs. Unfortunately, there were other defects that were not detectable during the pregnancy. The past few days have been very difficult, obviously. We are doing our best at taking all the information in and processing it best we can. I believe that Tom and I are getting stronger each day, learning what we can, and accepting that the road ahead is going to be very challenging for our family.

We have been advised by the counselors here at Children's to not look too far into the future, and to just take it day by day. Everyone that we have dealt with here at Children's has been great. They are all very understanding and compassionate and they are always keeping Harlie's comfort level in mind. They are keeping her asleep since all the tubes would be quite scary and uncomfortable for her. They also hope to start feeding her tomorrow. So far, she has been given all her nutrients directly into her blood.

One of the hardest things that we are dealing with is the fact that we can't hold her for right now. As Tom and I are ready, we will give you more details. At this point, we are still learning new information everyday. The doctors are in the information gathering stage, running a bunch of tests, reviewing x-rays, mri's, ct scans, etc. The game plan is to have a "family meeting" on Friday, where all of her doctors (and there's a lot), Tom and I, the social worker, genetic people, etc. meet and go over her issues. They have run a lot of genetic tests and we are hoping that they will have some answers for us then.

They also think that they are going to do a small heart surgical procedure on Friday in anticipation for the more major one(s) in the future.Thank you again, for thinking of us. I hope that you will continue to keep Harlie in your prayers.

Her nurses say that despite all she has going on, she still shows lots of spunk and is generally doing well all things considered.We'll keep you updated as we can. We left the hospital today and are now staying at the DC Ronald McDonald House, not very far from Children's. Email is the best way to contact us as our cells are off while at the hospital and at night we can take the time to sit and read emails.

Thanks again for your kind words and prayers.

Love,
Tom & Christy

Harlie's Story

In May of 2006, at 16 weeks pregnant, we found out that our baby had a mass growing inside her chest. The only thing we could do was wait and see how fast it was growing. Originally we did not want to know the gender. After this appointment, we talked about it and I said that I had to pull for this baby. And I needed to know who I was pulling for. The next day I called the office and they told me that it was a girl.

At 20 weeks we had another ultrasound and it was discovered that the mass was growing fairly rapidly, most likely preventing normal lung development on her right side. The danger was that the mass would grow and put too much pressure on her heart, causing heart failure (hydrops) during the pregnancy. The plan was to monitor her and me very closely. If hydrops were to develop, it was possible to have open fetal surgery to try to help her.

At 23 weeks, we were sent to DC for further testing. After an MRI and fetal echocardiogram, we were shocked and devastated to learn that she had a rare, complicated heart defect called Congenitally Corrected Transposition of the Great Arteries or L-TGA. In very basic terms, her heart was formed in a mirror image. So everything that is normally on the right, is on the left, etc. She also had a large VSD (a hole in between the ventricles of her heart), and a small right ventricle. Because of her heart defects, fetal surgery for the chest mass would not be possible. The combination of these two, rare, unrelated defects left us with a 5% chance of ever bringing our little girl home. The only thing we could do was wait and hope.

The pregnancy was extremely painful for us. I had weekly appointments and many more tests. All tests came back normal. No reason for these defects. Nothing I did during or before the pregnancy had anything to do with why this happened. We hoped for the best and prepared for the worst.

At 37 weeks, I was induced at Washington Hospital Center in DC. (She would need to be transferred to Children’s National Medical Center next door immediately after birth). She was an easy delivery for the most part. Although, not surprisingly to me, the cord was wrapped around her neck a few times (she was a VERY active baby). As soon as she was born, we knew she had more problems than we anticipated. She had trouble breathing, and we only heard a faint squeak from her. I got to hold her for only a few seconds and they took her away. I had to wait 3 weeks to hold her again.

Harlie has Goldenhar Syndrome (a craniofacial syndrome), VACTERAL Association, and Congenital Lobar Emphysema. There is no known cause for these defects – it is just “luck”. There is no genetic code associated with these abnormalities, nor are there tests to confirm diagnosis. In fact, Goldenhar and VACTERAL are both a matter of opinion based on a collection of abnormalities. The defects vary from patient to patient, so no two cases are exactly the same.

Harlie’s heart defects are: L-TGA, VSD, intermittent 2nd degree heart block, small right ventricle, 2 small Superior Vena Cava’s (instead of 1 large SVC), and subaortic stenosis.

Her other defects are:

Underdeveloped lower jaw (micrognathia),

microtia and atresia of the left ear (missing left ear and no canal),

no hearing in left ear, (will get a BAHA in the future)

moderate hearing loss in right ear (has a hearing aid now)

dermoid, cleft and coloboma in left eye (her eye didn’t close properly),

skin tags on both sides of face,

vertebral anomalies (hemivertebra in several places, butterfly vertebra, and kyphosis scoliosis),

misshapen skull,

anoperineal fistula

congenital lobar emphysema.

Everyday since her birth has been an adventure for our family. Thankfully, the chest mass had shrunk considerably, so that was put on the backburner. At just 4 days old, she had her first heart surgery. They put a band on her pulmonary artery and placed a pacemaker. Her second heart surgery was the Glenn, in March 2007. She spent almost 3 months in the hospital after that surgery.

In August of 2007, her chest mass reared its ugly head and surgery was required. Her upper right and lower right lobes were successfully removed, allowing the middle lobe room to grow.

Because of her underdeveloped lower jaw, her mandible occludes her airway, causing an upper airway obstruction, which didn't allow her to swallow or breathe from her mouth and nose. So at 16 days old she had surgery for a tracheostomy and gastrostomy (feeding tube).

Since the trach is placed below the vocal cords, no air passes through (which is what causes sound).  So when she cried, she made no sound.  This meant she didn't learn to cry to get what she needed or wanted.  And she didn't learn to coo or babble.  Her mouth wasn't used to suck on a bottle, swallow formula, or cry, or coo, so her muscles just never learned to move around the way they are supposed to.  Combine that with an abnormally developed jaw and you have a lot of challenges to overcome to learn to swallow and talk.

To date, she has had over 60 surgeries and spent more than her fair share in the hospital. Despite everything, she is doing great. Most physicians and therapists cannot believe her medical history when they see her. She is super smart and more stubborn than anyone I have ever met. We think she is beautiful. And she is worth every ounce of effort we have to give to keep her safe and sound.

Harlie has taught me so many things. The most important so far, I believe, is to be thankful. I try not to dwell on what problems we have, instead I try to be thankful for the problems we don’t have. I am eternally thankful for the body parts that work properly. I am thankful for her spunk and spirit. I am thankful for my husband and sons. I am thankful for our family. I am thankful for the friends that have stayed by our side, and for the friends that have come into our life since Harlie’s birth. I am thankful for our wonderful nurses. I am thankful for all the great doctors and nurses that have taken care of her, and me. I am thankful for our social worker. I am thankful for the trach board. I am thankful that God gave us Harlie. And I am thankful for every single person that has reached out to us in some way, supported us, made us dinner, cut our grass, listened to us talk, helped us take care of Murphy and Cooper, wrote to us, sent us cards, gifts, care packages, contributions to The Harlie Fund, etc. Every “little” thing made a difference to us, and continues to make a difference to us.

To all of you, I am thankful.

Love,
Christy and Tom (and Murphy, Harlie and Cooper)

Harlie's Surgeries

Here is an incomplete list of Harlie's surgeries.

09/25/06 - Born at 1:11pm, 5 pounds, 3 ounces, 18.5 inches

09/29/06 - 1st Open Heart Surgery - PA band and PDA ligation and placement of pacemaker leads

10/11/06 - Tracheostomy

10/11/06 - G-tube (peg) Placement (feeding tube)

10/18/06 - Removal of skin tags on both sides of her face

02/19/07 - 1st Heart Cath

03/06/07 - 2nd Open heart surgery - the Bilateral/Bidirectional Glenn (she spent over 9 weeks in the hospital with a chylothorax)

03/08/07 - Tracheostomy re-do (stoma closed after heart surgery - they remove trach for heart surgery due to risk of infection)

06/13/07 - 2nd Heart Cath

08/14/07 - Right Lower Lobectomy and Upper Plication (removed most of her upper right lung lobe and all of her lower lobe)

10/04/07 - Anoplasty

05/14/08 - Nissen Fundoplication and Mic-key Button Placement (a nissen is to stop vomiting and a Mic-key is a low-profile feeding tube)

06/03/08 - 1st Bone Graft Jaw Reconstruction (harvested bone from her skull and placed into her lower jaw to try to extend it forward to bring her jaw out of her airway)

08/05/08 - Jaw wires removed

08/19/08 - 3rd Heart Cath

01/13/09 - Ear Tube Placement

03/31/09 - 4th Heart Cath

04/16/09 - 5th Hearth Cath and Transesophageal Echo

06/08/09 - 3rd Open Heart Surgery - the Damus Kaye Stansel procedure (DKS)

11/20/09 - Ear Tube Placement

12/10/09 - 2nd Bone Graft Jaw Reconstruction

02/11/10 - Jaw wires removed and debridement of jaw wound (a part of the transplanted bone was infected and had to be removed)

06/10/10 - 6th Heart Cath

07/13/10 - 4th Open Heart Surgery - the Fontan

3/28/11 - Spinal Fusion Surgery in two places (and ear tube placement)

05/02/11 - Infection in incision, irrigated and debrided 4 times (done in the OR under anesthesia) with wound vac, requiring a 9-day hospitalization

03/13/12 - 5th Heart Surgery - Pacemaker generator placement

08/03/12 - Bone Anchored Hearing Aid surgery on left side.

08/24/12 - Fibula Free Flap Mandibular Reconstruction.  Her fibula from her right leg was taken and placed into the right side of her jaw.

08/24/12 - Left Eye Closure Repair

02/12/13 - Bone Anchored Hearing Aid - Abutement Placement

07/03/13 - Mandibular Distraction - Boston Children's Hospital

8/23/13 - Abscess in jaw requiring four separate debridements under anesthesia, MCV

10/30/13 - Removal of hardware in jaw - Boston Children's Hospital

12/13/13 - Heart Catheterization - CNMC, DC

02/07/14 - Hearth Catheterization - CNMC, DC

08/11/15 - Laryngoscopy and decannualtion! - CNMC, DC

10/09/15 - Tracheocutaneous fistula repair (stoma closure), CNMC, DC

08/08/17 - TMJ Ankylosis Release - Boston Children's Hospital

08/10/17 - Emergency tracheostomy - Boston Children's Hospital

11/02/17 - DLB - CNMC, DC

12/14/17 - Heart Catheterization - CNMC, DC

02/01/18 - DLB - CNMC, DC

2/21/18 - Laryngotracheal Reconstruction - CNMC, DC
                Spent 23 days on paralytic and sedation

03/03/18 - Cardiac Arrest/CPR

09/13/19 - DLB - CNMC, DC

03/06/20 - Mandibular Jaw Distraction - Boston Children's Hospital

05/19/20 - Hardware Removal - Boston Children's Hospital

Procedures Under Anesthesia

Bronchoscopy - 12+
CT Scans - when she was younger she needed anesthesia, now she doesn't!
Ear Tube Placements - 4
Auditory Brainstem Response Tests (hearing tests) - 2

For some interesting stats from her 1st year check out this post.