I am a girl who fell in love, got married and wanted to have a regular ol' ordinary family. What I ended up getting was everything but.
This blog chronicles our journey as a family who was blessed with a very special child.
From the day we found out that she had only a 5% chance of living to the present, life has been a true roller coaster of emotions. And most surprisingly, more rewarding than I ever thought possible.
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Due to the requests of many caring people, our family set up a legal trust fund, "THE HARLIE FUND" to help with Harlie's care. You may click here for more information or click on the donate button above.
The Harlie Crew
We invite each fan of Harlie to join the following initial crew members and commit to make a monthly contribution to The Harlie Fund.
*Roger Smith DR Racing
*The Lawyers and staff of Kane, Jeffries, Cooper & Carollo, LLP
With Christy's willingness to share, we have worried about, prayed for and rejoiced in the accomplishments of a remarkable girl. The members of The Harlie Crew together show their support by contributing monthly. If you would like to join us, please call Bill Jeffries at (804) 288-1672.
So, I took Harlie to the doctor today. She has another ear infection - and this is WITH her tube! Ugh! He said he was more worried about her left lung than her ear. So, he's treating her ear infection with some antibiotics to try and stop her from getting worse - on both fronts.
She has her pre-op heart catheterization on March 10th and she must be "healthy" or they can't do it - which would push everything back. And we don't want that. So hopefully the meds will help her get better soon. When she's this junky we really can't get anywhere with her feeding or speech therapies. Her secretions are way too much for her to handle. We are going through bibs like crazy. Within minutes they are soaked. (Donna - I KNOW you know what I'm talking about!!!). Yes, it's disgusting, but we're pretty used to gross stuff around here.
After I got back from taking her to the doctor I ran to the gym, got 4.5 miles in and then came back home and rushed out for her first speech therapy appointment with our new therapist, Michelle. Harlie was definitely NOT excited about it. It is definitely very different to have to go to the clinics vs. them coming to our house. It takes a few minutes for her to get comfortable enough to get something accomplished. Whereas when they came here, they could just get down to business.
Anyway, after being there a few minutes, Harlie signed "home" and "hat" and grabbed her coat to leave. I love that she can "tell" me what she wants! While I knew we wouldn't get much therapy done with her being sick, I also knew that Michelle and I would have to do a lot of talking to bring her up to speed on Harlie and where we are and where we want to go. She asked me what Harlie knew, what our routine is like and what is on the horizon for her, medically speaking. We talked about communication and what I like/don't like about some communication methods.
Michelle was a sign language instructor, so she likes sign language and is very fluent signing while speaking. I find it amazing, really. So far, I have enjoyed signing with Harlie very much. And, while it certainly has a major negative (not everyone knows sign) it provides the "easiest" form of communication for my family (I believe) so far. So, we have decided for now that signing will be our main form of communication. And when right, we'll start to look at some communication devices (computers) for the future.
Please keep in mind that for the past 6 months, we believed that Harlie would be decanned this spring or summer and verbal communication would be right around the corner. Ha! While that was naive, and probably pretty stupid on our part, it is what we desperately wanted. So, now that we know her next jaw reconstruction will be in September, it will be spring or summer 2010 before that will be a possibility again. So, we have to look a little more long-term and right now, signing is the best means of communication for her and our family.
This means that we have to learn to sign while we are speaking all the time so she can see us using it and so she can better learn - not only signs - but language development. This is called "modeling". And this is also very important because as she gets older, her frustrations will grow if she can't communicate her needs/wants to us, causing unwanted behavior issues.
While Harlie wanted to leave to go home (which she clearly communicated) as soon as she saw Michelle signing to her, she perked up. She watches the signs and immediately tries to do them herself. She knows what signing is and what she gets out of it. It makes me VERY happy to see her communicating and it is so exciting to think of all she will be able to say!! Michelle showed me some tips on how to teach her to string the signs together (important for language development) and by the end of the appointment she signed "I want to go home" vs. "home" and "hat". Crazy, isn't it?!?! And while it might not seem important for her to sign sentences since I clearly understood her when she signed just two words, it makes a huge difference when she can verbalize. Which, we will still continue to work on. Ideally, she will learn to verbalize and sign at the same time, just like us, until signing isn't necessary anymore (down the road a bit, of course).
So, what does this mean to those that see Harlie on a regular basis? Yep, I'm going to ask you to please learn some sign so not only you can communicate with her - but so she can communicate with you. It's a toddler vocabulary, so it's really not difficult. In fact, it is quite fun - especially when you can understand her. So, to all my family and friends, let's talk about this more in person. You won't find a more appreciative person around - I promise! I'm talking about me, of course!
So, I got home after another busy day and checked my e-mail. Now, before I share this message, let me tell you that I have some wonderful neighbors, Cami and Mike. They have two kids, Catie and Cole. Cole is 4 and he is Murphy's best friend. This is the message:
First, just let me tell you I'm sorry your gang has been sick and if you need anything, just let me know.
Now about your future son-in-law, Cole has expressed to us several times that he is going to marry Harlie. He's even figured out the wedding attire. He wants Harlie to wear Catie's flower girl headpiece and he will put on his black coat from Aunt Jenny's wedding and then they can get married. Should we set a date?
That is the cutest thing ever. Brandy noted that Cole attends preschool and out of all the girls he knows, he chose Harlie. Awww! My heart is so warm!!!
On Thursday we had our last session with Beth, our speech therapist. It was sad. We REALLY liked her. And as a finale, Beth got Harlie to mimic her making some animal noises. It was really cute. I got some video, but haven't had time to get it off camera yet. Hopefully I can do that this week.
Harlie has been fighting a cold I'm guessing, for a few days now. She's been coughing so much that she hasn't been able to wear the PMV at all. And she's been very quiet. Even without the PMV she usually makes some noises, but lately, she hasn't made a sound. I'm guessing she's just tired and not her energetic self. She's been taking long naps every day and going to bed early every night. Definitely NOT her usual routine! And she's back on oxygen while she's sleeping. She's been having these coughing fits that make her lips, fingers and toes turn all blue. It's been awful. I was letting her fight it on her own, but after today, I am taking her in tomorrow to see her pediatrician.
Cooper's been coughing a lot lately, too. He had a really bad night last week, so on Thursday I took him in to see the pediatrician. He has his 5th double ear infection. He will be 5 months old this coming week and he's already had 5 double ear infections - that we've found! So, I called an ENT and scheduled an appointment for Friday. Hopefully, they can accommodate our schedule and get him tubes before we leave for Harlie's heart surgery. I really don't want to have to deal with both at the same time. What a logistical nightmare that would be. I think they typically want to wait till the baby is 6 months old - but he'll be close enough and he clearly needs them.
So, both Harlie and Cooper are coughing like crazy. We've been suctioning Harlie every 2 minutes - ugh. And then last night (Saturday night) Murphy comes in our room at 3am (okay, so Sunday morning I guess I should say) and throws up. Right on our floor. Then again in the bathroom. Lovely. Well, I wish it were lovely - it was actually one of the grossest things I've ever seen. I'll spare you the detailed description. I'll tell you this much - it isn't coming out of the carpet.
So, today was a very lazy Sunday. Murphy only ate a half of a banana all day. He finally ate some toast at dinner. And Harlie slept a lot. She had a fever and I gave her some meds and she perked up a bit. Finally made a few grunt-like sounds, which is a good sign.
I have more to write, but will have to do it tomorrow. There's still so much to do before bed!
So, they called and gave me a date. A date for her third heart surgery - the Fontan. It is April 2nd. Wowzers. It felt so weird to write it in my calendar. I wanted to laugh and cry at the same time. It will be nice to get this behind us, to have Harlie's heart function better and to see some better numbers on her monitor for a change! But, I know what lies ahead and it's not pretty.
We have to go up to DC the day before for her pre-op stuff. Then we will have to stay the night up there and then take her back to the hospital first thing in the morning. I can't help but feel bad that she has NO idea of what's coming. I suppose it is better that way. But, still...
Well, sadly, tomorrow we have our last session with Beth, Harlie's speech therapist. We will certainly miss her and I really hope that we will see her again. Murphy took right to her, too and I know he will miss her, too.
Luckily, for Harlie's feeding therapy we will get the same OT that we had from the beginning, Allison. She used to come to our house for feeding therapy until she moved to the clinic at the Children's Hospital here in Richmond. So, since we are losing Beth, it just worked out great that we can get Allison again. Although now instead of them coming to our house for therapy, we will have to go to the clinic. Budget cuts with the state, blah, blah... So, we go on Friday to see Allison. And we will also get Harlie's blenderized diet recipe. I am really excited about that. I am crossing my fingers that she won't have a reaction to anything in it. Normally, you introduce foods one at a time, but putting a child on a blenderized diet doesn't really allow you that kind of time. So, we're taking a risk, but I think it's a low one.
Beth also did one hour a week working on communication with Harlie. So, we will meet a new person on Monday for our first session of speech therapy. I hope we like her and that Harlie takes to her.
Well, that's it for tonight. I am going to get off the computer so I can relax for a little bit.
You know, I KEEP telling myself that I will NOT over-fill my day with too many appointments! Yet, I continue to do it!!! What is wrong with me???
Well, I know how it happens. At first there's just one appointment. Great. And then there's one appointment on each day. Then, I find out that Harlie needs to have another appointment done by a certain time. And when I go to schedule it, there's two appointments. Fine, I say. I can handle that. Then something else comes up and there's no other day that works for the other party, or me for that matter, and BAM! We have three appointments in one day. And while that might not sound that bad to you, it is. Trust me. It is exhausting. For all of us.
Today Harlie got another RSV shot. I really can't wait till spring when she doesn't have to get anymore. I am hoping this will be her last year. She's had them for three years now. But, no RSV, so that's good. This shot really hurt her. She was anticipating it. She is definitely becoming more aware about what is going on. So, since she was fighting a bit, I guess it hurt more than usual. She cried for about 20 minutes. And she's NEVER done that! But, she's okay now.
Then I had to go and order her next month's food supply. Then I had to rush home to collect Brandy and all the kids to go to see her local cardiologist for an echo (an ultrasound of her heart). She had to have this in preparation for her upcoming heart surgery. I took Murphy with us because he had a VSD (ventricular septal defect - a hole in between the ventricles of the heart) at birth, that closed up on its own. However, before his surgery this summer, the doctor noticed a murmur, so her cardiologist said he would be happy to take a look. He's good, no issues.
I think Harlie having the RSV shot earlier made this appointment much harder. They couldn't get a blood pressure reading because she was too upset. And the echo wasn't any better. He said it is hard to tell because she was crying during most of the echo. And getting the echo doesn't hurt her. It's just an ultrasound on her chest - but she didn't want any parts of it. But that's okay, her heart cath will tell us all we need to know. She needs to have a cath in preparation for her surgery, as well. That should happen in the next few weeks I'm guessing. Then all the info will go to DC for them to see before her surgery.
That's it. Oh! And her head is getting better. It is less swollen now (the worst was yesterday - it was huge!) But now it is black. Nice.
Yesterday I went to go get Harlie to give her a feeding. She was playing in the playroom with Murphy and doing just fine. So I tell her she has to come with me for her feeding. And on her way out the room she tripped over a toy and landed head first into the corner of the door. I was right there and couldn't do a thing to stop her. Ugh! She hit it pretty hard and the door didn't budge, so I expected a long, pitiful cry. But she only cried for a minute and then went on about her business. It swelled up a little bit, but nothing like what I was expecting. (Because she's on baby aspirin every day, she bruises really easily.)
Then I went to work for a few hours. While I was gone she took a 3-hour nap! That never happens! And when I got home Tom said that he thought she was getting sick. He had to suction her a lot (which is pretty odd for her now - thank goodness!) and at about 6:30pm she looked VERY tired. So I took her upstairs and she vomited - a lot. She felt hot, so I took her temp and she had a fever of 103. I don't know where this came from! Out of the blue! And she needed suctioning a lot throughout the night (never fun - especially when you don't have a night nurse). Anyway, she slept till 8:30 this morning. And within minutes of being awake she vomited black stuff (something she's done before but never with this amount of volume). Doctors assume the black is blood, but no one really knows why.
Anyway, her head didn't look that bad. Definitely swollen, but not blue or anything and you couldn't even really tell. You had to know what you were looking for to see it. So, at about 11:00 this morning I went upstairs to get ready for work and when I came back down this is what her head looked like...
Luckily, it is hurting me more than her! It just looks so painful to me! And since I wasn't expecting it, when I looked at her I gasped! She was probably thinking, "what's your problem"? It was just so weird to not see anything one minute and then see that!
But, how funny is it that she is smiling for the camera now? I just point it at her and she smiles - that's her smile by the way. I forgot to mention that Dr. Magee said that since she has a facial cleft (a very mild one) some of the nerves in her face never developed which makes her smile weak. She can't bring the corners of her mouth up. It's possible she can have some sort of micro surgery later on in life to help (a funny story, but I'll have to save that for later). But, hopefully in time the other muscles in her face will compensate to make her smile stronger.
So the appointment went pretty well, overall. Dr. Magee agreed that her jaw has receded. Although he said that it could also be that the rest of her face has grown, but her lower jaw didn't grow. He said that we just don't know the growth potential of her jaw. Who's to say that it will have normal growth? Again, it didn't form normally, so who knows?
He explained that her skin was so tight (since it covered a very small jaw) and when he gave her more of a jaw - her skin had to stretch to cover it. With the constant pressure of the skin being tight, the bone eventually gives in to the pressure and recedes. It works the same way as braces on teeth. The constant pressure of the braces move the teeth as the bone gives in.
So, we try again. In August or September. Of course I am trying to keep the beginning of Kindergarten open and free so we can focus on Murphy starting school. So we are going to think about either the beginning of August to do the surgery, or the END of September. We just need to figure out which will be less hard on Murphy. For those of you that have seen the pictures of her after surgery - it was a bit traumatic for everyone. So, will it be better for him to see her like that before or after he starts school? We have a few months to think about it since his surgery schedule doesn't go out that far.
So, I've e-mailed her DC cardiologist with a tentative surgery plan for the year.
Fontan (3rd heart surgery) - Spring (possibly April) Craniofacial surgery - August or September Spinal Fusion - Winter
Just a quick post to say that we are going to Norfolk tomorrow to see Dr. Magee - Harlie's plastic surgeon. I'm assuming that he'll be able to look at her and give us some idea of what our options are. I'm guessing he'll want another CT scan at some point. But hopefully he'll be able to tell me enough to get back with her cardiologist in DC to make some sort of "plan" for when her heart surgery will be. Not knowing how all these surgeries are going to play out is hard to live with.
I've been in kind of a grumpy mood I guess. Tom said he could hear the anxiety in my voice. I suppose I snapped at him. Maybe a couple of times. I just don't want to hear bad news. Waiting for an appointment, hoping for a good one, and then hearing bad news... UGH! I just don't want anymore of that! Is that too much to ask???? Probably. Especially at this stage in the game. We still have a lot of appointments ahead of us.
This week is Congenital Heart Defect Awareness Week (February 7 - 14). I was checking on The CHD Blog my friend runs and saw that she posted the following video. I'll be honest. I cried. Like a baby. To think of Harlie older and dancing around really got to me. Well, watch and you'll see what I mean...
I guess at that particular hospital they don't do many Fontan's (Harlie's next heart surgery). Although I did notice one Glenn (that was Harlie's last surgery when she was 6 months old). I'll have to ask my friend who runs that blog...
Anyway, I think the video is great. It was very moving for me. I hope you enjoyed it, too.
As I was putting Harlie in bed tonight, she was crying about something. So I gave her a hug and put her back in her crib. Then I told her I had to give her her water (she gets 3 ounces at 8:30 with her aspirin). So, I get the tubing and hook her up and give her the water. She's so good, she just lays there patiently waiting for it to all go in. Then she was still whimpering a little, so I picked up her stuffed bunny and gave it a hug and then gave it to her to hug. So she did, and then laid it down on her bed, where she is supposed to lay. Then she signed "water" and pointed to the tubing and then pointed to the bunny. So I said, "you want to give your bunny some water"? And she nodded her head yes. So I gave her the tubing and she pretended to hook it up to its belly and then pretended to give it water. She acted like she was waiting for it to go in and then signed "all done".
So, we went to DC on Tuesday to meet another orthopaedic surgeon about Harlie's spinal issues. Well, actually we went to the outpatient clinic in Fairfax. That location got us an appointment faster and, actually, it was a lot easier and quicker to get to. This surgeon specializes in spinal issues which makes me feel better. And she is at Children's National, which is where her heart surgeon is, so I certainly feel more comfortable with her having this kind of surgery there - where she's already well known by the cardiac team. Anyway, I liked her a lot and I feel like she took her time educating me on what Harlie has and what it means.
Basically, Harlie has a few issues:
1. cervical spine deformities - missing some vertebra and she has some fusion (doesn't need attention at this time)
2. Lumbar spine deformities - butterfly vertebrae (1 location), and 1 hemivertebrae (half a vertebral body)
3. Sacrum deformity - 1 hemivertebrae at the very bottom
The outward curvature that you see in this picture is called Congenital Kyphosis Scoliosis.
I've done some research trying to find a good site that explains it well but I had no luck. From what I can gather it is not very common. The congenital version, I mean. Older people can develop kyphosis over time, but that's not the same thing.
Anyway, she said the area that is most severe is in her sacrum. The hemivertebrae there is causing the tilting in her hips. She said that there are two options for correction.
One option is to remove the hemivertebra, in both locations, then fuse the surrounding vertebra in both locations. This is appealing because it will most likely straighten her spine immediately. The negative with this option is that because of the fusion necessary, it will leave her little room to grow. And she means growth in her torso. Which is important to give your heart and lungs the room they need to function properly.
The second option is to put in growth rods. While this might seem appealing to optimize her growth potential, there are some negatives. One is that it puts her in the OR every 6 months for adjustment. And it might not work on the hemivertebra as effectively as removing them, which means we might end up having to remove them anyway. At least that's what I think she said. If there is the option to do the growth rods first, and then removing the hemivertebra, I don't know. And that sounds quite painful and risky to me.
And to make things even better, if I understood her correctly, since she has abnormalities in her vertebra to begin with, her growth potential is questionable. I think she said that since it isn't normal, then normal growth can't be assumed. Or something along those lines.
She said that we will have to do something within the next 6 to 12 months. I told her about her upcoming heart surgery and potential craniofacial surgery (will know more about that next week). She said that both of those surgeries could come first and if necessary we could push her surgery back a little more to give her a little break. I just think that those 3 surgeries are way too much for her in a 12 month period. I mean she's just 2 for crying out loud! But the risk is that the longer we wait to do surgery, the worse it becomes and more difficult the correction will be.
Anyway, Harlie will have to have an MRI and CT scan before surgery. So she said that she would talk to Harlie's cardiologist up there to see if we can work those tests in during her stay/recovery from heart surgery. The MRI is to make sure there are no issues with her spinal cord and the CT scan is to see a full view of the bones.
I think that about sums it up.
Oh, and after that appointment, we left and rushed back to Richmond to take Cooper to the doctor in the afternoon. He has his 4th ear infection (and he's just 4 months old!). I don't know how old they want kids to be before they get tubes. My doc said that the youngest he's had get them is 6 months. Which is really quite funny to me. I mean, I understand it, of course. But, Harlie had heart surgery at 4 days old, and she was born 3 weeks early! So basically she had a major surgery at 37 weeks gestation. So funny all the fuss for ear tubes. Clearly, my perspective is different, well, unique perhaps. Not normal at all. But what is anyway?
Oh, and Cooper weighs 16 pounds. Boy is he chubby! It is so much fun having a chubby baby.
We took some quick family photos before Tom left to go watch the game (at 12:30!!! - the game started at 6:30).
Actually, Tom left to go "tail gate" with all the other fans in the parking lot of the bar we all gather to watch the games. I couldn't leave that early - had kid duty, of course - so I missed the party. I got there later and still had a lot of fun.
Just a few of the Steelers fans...
Tom said that while they were partying, 7 police cars showed up. The officers told them they were fine - but they had to check it out anyway. Tom suspects a Baltimore Ravens fan that walked by called the police. Ahh, sore losers. But all was well and no one got in trouble.
Well, it was a great game. As classic Steelers games are, they had us all having heart attacks there near the end. But they pulled it out and the bar went crazy. Good times....
Today we went to DC for Harlie's orthopedics appointment (to get a second opinion on her spinal issues). I'll write more about that later.
Just a quick post to say how happy we are that we get to keep Traci, our physical therapist! WooHoo!!! She got a job with a company that contracts with the county, so she gets to keep Harlie as a patient. We are very happy for her - and us! Now if we could just keep Beth...
Murphy is 11 and is in sixth grade. He is constantly making us laugh with his crazy sense of humor and wonderful imagination. He is a great big brother and adjusted with no problems to this role. He has been an easy kid to raise from the get-go (despite the fact he entered this world 5 weeks ahead of schedule).
Harlie is 9 and is in second grade. She has Goldenhar Syndrome, VACTERL Association and a lung defect that required the removal of 2 lobes on the right side. After over 45 surgeries (4 of them open heart), she is remarkably age appropriate. She is the sweetest, funniest little girl I've ever met. You can't help but fall in love with her.
Cooper is 7 and is in first grade. He is a very talkative, high energy and affectionate little guy. He is all boy and he is a very funny kid. We are so lucky to have him!
Rooney was born on February 5, 2012. He has been a wonderful addition to our family. He is such a great little dog and I just can't imagine life without him now! I love him so much!!!