A little of a lot.

Since it's been so long since I've updated you on Harlie, I'll start there.

Her heart

It has been over a year since her last heart cath (it was in December 2013).  I feel like a lot has changed since then, which is good. She had her pacemaker adjusted and she's been able to spend some good time on room air. I'm hoping that means good things for how her lungs are doing.

So, right now I'm working on getting a date for a heart cath soon. I didn't want to have to worry about it with our Disney trip in December. I thought it wasn't smart to get it done before - what if something happened during the cath that would've prevented her from going or what if we got bad news?  So, we decided to wait until after the holidays.

It's interesting to look back at my decisions sometimes. In 2013, I wanted her to have the heart cath, despite the fact that it was done on December 13th. I could have thought it was too risky to go into the hospital so soon before Christmas - what if something happened during the cath that would've prevented her from being home for Christmas or what if we got bad news? Instead, I had hope and thought, what if they can do something that could get her off the oxygen before Christmas?

I think I'll always have a glimmer of hope.  But, I also think after eight years of reality slapping me in the face, I can't deny that things don't always work out the way I want them to.  Let's just say that I've gotten a lot of practice in learning to live with disappointment.

Her back

Anyway, another area I need to work on is her back.  The last time we saw her orthopedic surgeon (a year or two ago, I can't remember) she said that when Harlie complains about back pain regularly, we need to do something. She really did not want to do another surgery.  She would rather wait until she grows more (like when she turns 12 or so). But, if Harlie is in constant pain, waiting wouldn't really be an option.  The pain is most likely due to the area in her spinal fusion that became infected. The infection forced her surgeon to remove the bone graft and didn't allow for optimal healing.  Doesn't this just look like it could be painful?

It's so gnarly because after it got infected, they had to put a wound vac in it for several days.  And she had to go into the OR several times for debridement.  It was a horrible time.

Anyway, at this point, she complains about back pain almost every single day.  Some days are really bad and she might tell me that her back hurts ten times. For a while I asked her if we needed to go to the doctor and she would say no. Until one day, she said yes. That means she's pretty serious.

Then, when we were in Disney, I noticed that she would sit in her wheelchair while leaning to her left.  And, about six months or so ago, she stopped using alternating steps while going up or down the stairs.  We worked really hard in physical therapy and at home to remind her to take alternating steps.  And for a while, she would point out to me when she was doing it without being asked (so she was proud of herself).  But then it just stopped.  And she absolutely refuses to do it.  Period.

So, something is up. Darn it.  So, I'm working on getting that appointment scheduled, too.

Sleep Study

Another thing I need to think about is getting a sleep study date.  We've been toying with doing this for years and have yet to accomplish it. I'm pretty sure that my reasons for/against a sleep study are more complicated than I would like them to be.

We've been able to cap her some lately, which is nice. And the other night she asked to sleep with her trach mask around her nose and mouth instead of it being around her neck. I took advantage, and switched the trach mask for a face mask and capped her.  That way she would get what she wanted, and oxygen, too.  She fell asleep like that just fine and maintained good sats for a while.  But, I had a hard time sleeping knowing that she was capped without proper monitoring. So, around 1am I got up and took the cap off and returned her to a trach mask. She was making some noise (almost like snoring - definitely some obstruction noise), but her sats were at 84 instead of 91. Not sure if that means anything really. I just don't know.  I suppose we should do it, just to get some sort of data, if nothing else. But, I'm just not excited about anything having to do with it.  And decannulation (getting the trach out) isn't that simple. She has so many other issues.  I'm just afraid (among other things) that it might complicate another thing or that it will be temporary.  Anyway, far too deep of a discussion for today. So, as usual, I put off the decision for another day.

Hearing Aid

We finally got in to see her audiologist a few weeks ago.  We haven't seen her in far too long. But, as it turns out, it's been FIVE years since she got her very first hearing aid.  I can't believe it.  It almost feels like it happened yesterday. Anyway, she's due for a replacement.  Apparently they don't last much longer than that.  So, she got a mold of her ear and got to pick out another aid.

She puts this foamy stuff in her ear and lets it harden.  Then pulls it out to send it to the company to have the ear piece made.

She picked out a pink hearing aid (the one she has now is purple).  It should come in soon.  Oh, the things we find exciting!

Nursing

Another new thing for us is our nurse, Terri is back.  Harlie was happy to see her.  Terri hasn't worked here in a little over a year.  We missed her. We also made another change and let the nurse that was sharing the week with Brandy, go. I can't go into details, but I just felt like I had no other choice.  We've been so incredibly lucky with our home health nurses that I've never really felt like complaining about having nurses that much.  I mean, the requirement of needing them totally sucks and I don't know a soul that would argue with me there.  But, we've had such wonderful people that I've always felt good about it. They allow me to live as normal of a life as possible and I am eternally grateful for that.

But, home health nursing is a weird arrangement. The nurse is employed by a company. But they work in a patient's home. So, as a patient's mother, I am not their boss.  But, I have a say in what they do with my daughter. It is such a gray area. And when you want to make a change in nursing staff, you tell the agency, who then tells them.  There is no notice given to the nurse.  Which stinks for them. I shouldn't have to explain that it's just not smart to give a nurse notice. It puts the patient and patient's family in a vulnerable position.  But, typically in this business, there usually is another family who needs help.  It always seems like there are more patients than there are nurses.

So, making this change back to Terri wasn't as well received by one nurse.  And she sent me a horribly ugly message a couple of weeks later.  It was upsetting to read. It's clear she didn't like me and judged me poorly in a number of ways.

Think about that for a minute.

Someone was in my HOME for over a year, and was a caregiver to my child with limited communication abilities (whom I love more than words could convey) - and didn't like me.  Judged me.  Doesn't that sound horrible?  One should never have to have someone like that in their home.  I feel so violated.  Can you imagine having someone in your home watching you parent?  And judging how you live your life?  Ugh!  It's an awful thought! As if our life isn't painful enough anyway.  I keep thinking about some of the things she said and how we never asked for any of this. I wish we never needed a nurse. I love our nurses, but I would have been perfectly happy having three healthy children and never meeting them in the first place.

So, for now, I am done with strangers.  I would rather go without than having to start over with someone new right now.

Independence

On Monday, the kids didn't have school.  And we didn't have a nurse scheduled.  So, after Harlie's teacher came in the morning, I took all three kids - all by myself - to the mall.  I've never done that before. Harlie got a Build a Bear gift card for Christmas and all three had some Christmas money to spend.  So, we went and Harlie picked out Mikey, the TMNT, of course.

And Cooper picked out Toothless from How to Train Your Dragon.

Then, we went to Toys R Us and let them spend the rest of their money.  Murphy picked out two Disney Infinity characters, Cooper picked out some Trash Pack things with what he had left over after getting Toothless, and Harlie picked out a Baby Alive baby with a toothbrush and toothpaste.  They were all very happy.  Then we went home.  Success!  It's times like these - just being a regular mom, and not a special needs mom - that I've lost.  I'm ready to do more of that, and less medical stuff.

Feeding

Oh, and I almost forgot... another milestone for Harlie! I think I can officially say, that after eight years, Harlie finally knows what hunger is.  Wow!  She will tell me that she's hungry and will let me feed her.  YAY!  It doesn't happen every feeding, of course.  But, I will take it!  I'm very happy about this development.

My nephew

I've been able to spend some good quality time with this little guy lately...

His name is Chase, and he is my younger brother and his fiancee's baby.  He's about to be six months old. He's super cute.  And I've been keeping him a couple of days a week for the last few weeks.

Harlie took this picture.
She cracks me up.

Harlie loves him and helps me throughout the day.  She gets me his clothes and diapers and wipes.  And always makes sure he has a toy within reach. Last night, I wanted to get a pic of the two of them together.

And she decided she needed her eye patch.

And he clearly thought she was crazy.

Rooney might be a little jealous.

Or he saw that my arm had no support, so he came to my rescue.  Either way, he's such a good dog and is so tolerant.  Chase likes to grab his hair, and he just takes it.  Just another reason why Pugs are so great.  Man, I love that dog!

Okay, I think that's all I can do today.  Chase will wake up from his nap anytime now.  I have another post already started.  I am trying really hard to work this blogging thing back into my life.  I missed it!

Thank you for reading!
~Christy xo

Appointment with the surgeon...

...eh, not what I hoped for.

She wanted Harlie to wear the brace for another month.  But we compromised with two weeks.  That way she will have it off for school.  I told her that she doesn't complain - it's me that hates it.  And I recognize that the long-term goals of healing are more important than the pain of dealing with the brace, so if she really wanted us to keep it on, I would.  But, she said that two weeks will be fine.  That way when she goes potty at school, she'll be able to do it on her own.  It is hard to get her shorts up and down with the brace because it goes past her hips.

So, I'm thinking her surgeon would not have approved of this activity...

Notice Cooper posing.  What a ham!

Weeee!

And of course, some video...

How could I deny her this fun?  There is only so much activity I can make her miss.

Anyway, when she gets home after school, we need to put the brace back on her for the rest of the day.  And over time we can wean her off the brace by waiting longer and longer to put it back on her.  She said that it can actually be more of a discomfort to stop wearing the brace cold turkey because all those muscles haven't had to work much over the last five plus months.

I asked if she could resume normal activity once the brace comes off.  I had my hopes up for a gymnastics birthday party for her since she loved it so much and hasn't been able to go since the surgery.    But she said no.  No gymnastics until she sees her next and she will decide then.

So, the next time we see her?  MARCH 2012!!!!  I am SO bummed!!!  She loved gymnastics!

On a positive note, the surgeon said that her x-rays looked "beautiful."  On the side view one, she is much straighter than she was.  She said that she could start to curve forward over time - but she hopes that doesn't happen.  Of course.

Harlie is so cute getting x-rays.  She stands there and does everything the x-ray tech asks her to do.  And he was hilarious because he said, "okay, move your little foot back some.  Exactly." and then "bring your little feet together.  Exactly".  He was cracking me up.

Oh, and she can only carry a light backpack.  Which, I already knew, really.  But it just goes to show you how long this recovery is taking and how many things are affected.  Spinal fusion surgery is no joke.  I think I would have to say it has been the worst surgery so far.  And I sure hope we don't have to do it again.

On the way home there was an earthquake that measured 5.9.  The epicenter was in Mineral, VA, 25 miles from our home.  According to FB, it was felt from North Carolina to Canada.  We were in the car not that far from home, and we felt nothing.  I guess when you're moving 70 miles an hour in a car, you don't notice the ground shaking.  So, I apparently missed the biggest news story of the day.  Oh well.

Oh, and would you believe our luck?  We decided just a few days ago to try and take the kids to the beach - together - all of us - as a family.  Harlie has never been.  Tom took Monday off.  And after I run the Patrick Henry Half Marathon this Saturday, we were going to go to Virginia Beach to stay with some friends who we haven't seen in a long time.  We were going to go to the beach on Sunday and take a baby pool and umbrella for Harlie (since she cannot go into the water - other than just her feet, of course) and we were going to have so much fun!

And now Hurricane Irene is totally screwing up our plans.  The only FUN plans we had for the entire summer.  I am so, so disappointed!!!!  And I don't think we can squeeze it in after the storm passes.  A whole summer and the kids have done nothing exciting.  The boys and Tom went camping and boating a few weekends ago - but Harlie had to stay home with me.  I am so mad at myself for not making more of an effort earlier in the summer.  But it was SO busy!!!  Well, I am not giving up hope yet - but according to reports, it is not likely to happen.

Oh!  And my nephew, Charlie, is playing in the Babe Ruth Little League 13-year old World Series Tournament in NY.  Well, tonight his team won and Charlie got MVP!!!  He hit the winning run.  So now they play again tomorrow night.  What a great experience for him!  Congratulations, Charlie!!!

Okay, that's it for today.  More later!
Thanks,
~Christy

Ortho Appointment

Well, crap.  There I went again, and got my hopes all up for something that wasn't to be.  Seems I do that a lot.  

So, it's FOUR MORE WEEKS in the cast!  And that was the worst case scenario!!!  We go back on May 24th to have it removed.  But, it gets better.  Here's how it will go down:

We will go up and they will cut the cast down both sides ("turtle shell" it).  They will remove it, and measure her for her custom made brace.  She will feel the cool air and freedom.  Then they will put the cast BACK ON and secure it in place.  We will come back home for two or three days (time for them to make the brace).  Then we will go back up to have the cast officially removed.  Again, she will feel the cool air and freedom.  Then they will put on her brace.  She will wear it during her waking hours for the next several months.  Yay us!  

I didn't ask, but I'm assuming the brace will allow her to bend at the waist.  Which will be a very welcome change!!!  I think she'll still be happy and relieved to have the cast gone as long as she can bend at the waist again.  

As far as how she is doing... her surgeon said that she is happy with how her x-rays looked today.  Here they are:

Before surgery: note how her hips are so uneven.

After surgery

And here they are side by side, which I think really shows the difference.



Wowza!  All of her crookedness was between her ribs and her hips.  In the Before photo, it looks like her spine is coming straight from her right hip!  It is pretty exciting to think about how she's going to look after this is all said and done.  It will be a welcome change to be able to actually see a positive change after a surgery.  And I bet it will feel really good to her!

Side view of the screws

Here is a copy of an image from her CT scan (which takes three dimensional photos, basically) before surgery, of course:

 

A close up of the two bottom areas that were abnormal:

Also, the reason why there is only one set of screws when she had two different areas fused is because the fusion in the upper area (butterfly vertebra) was done by modifying (shaving and cutting) the existing vertebrae and using bone grafts that will eventually heal and fuse together over time - without the need for screws.  She said that this will hopefully save some of her growth plates in that area, whereas the screws eliminate the growth plates in that area forever.

No new cast today, either.  A new cast would require OR time and anesthesia, and it's just not worth it.  So, they used the cast saw to cut away some areas of the cast that were particularly bothersome and/or dirty.  In the back, all the padding and soft border covering came off.  So, he did his best to make it better and more comfortable for her.

She was not a happy camper.  And Brandy got a work out trying to hold her still.  Gabe did the best he could, but she was far from cooperative!!! Harlie, not Brandy, I mean.  Brandy was very helpful and cooperative, as usual.

He also removed the gauze covering over her incision.  It was truly disgusting and a breeding ground for bacteria!!!  But, surprisingly the incision looked really neat and clean (meaning the cut) with no stitches that you could see.  She said the stitches were put underneath.  Crazy.  It was a relief to see that there is no infection.

So, that's that.  Four more weeks.  And three days.

Thanks!
~Christy

Surgery Update - 3pm

A nurse just came out to tell us that the surgeon did BOTH fusions.  She said that she (the surgeon) would be out soon to talk to us about how things went.  Tom asked her if Harlie did okay for them and she said, "She's stable."  Hmmmm... not sure how to take that.  English is not her first language, so I'm hoping it's just a cultural thing and not a the doctor has to tell you that something very serious happened in the OR kind of thing.

The nurse said that they still had to cast her - so it would still be "a while" before we see her surgeon.  While I'm anxious to see her - I don't want to see her!  I'm dreading what she's going to look like in this cast.  And that sounds so stupid after having to see her after two jaw reconstructions.  Those were the WORST.

And that reminds me - I haven't really talked about Murphy and how he's doing with this surgery.  We talked to him a week or so ago about it.  He asked if she would come home looking like Frankenstein.  Oh, my poor kids.  All of them.  I really hope they develop good coping mechanisms and don't try/like drugs.  I've seen my fair share of A&E's Intervention.  They all had something traumatic happen to them when they were kids.  Well, most of them anyway.

Murphy did his best to ignore me as much as humanly possible for the entire day.  I had to resort to sending him next door.  Thanks, Cami!  Then Cooper was a whiny mess.  He could clearly see that something was amiss as our mass of baggage grew in the hallway.  Murphy was eating dinner and Cooper ran up to him and threw his arms around him and said, "hug."  Oh, a moment to treasure!!!!

Okay, back to right now.  They have the TVs on in the waiting room and we are watching some horrid soap opera.  There is a very inappropriate scene of romantic nature on right now.  EW!  Isn't this a children's hospital????  Tom can't stand it and keeps making funny comments.  He just said, "and now he's going to fumble with her bra."  Yes, it's that kind of scene.  Hey fellow stranger parent sitting next to me in the waiting room, good TV viewing, huh?  Oh, I'm sorry, that's you snoring.  Sorry, didn't mean to wake you.

Oh, the joys of having a complex child and spending a mass amount of time in uncomfortable situations. Good times!

Anyway - got off track a bit there - the waiting isn't really the worst part.  It's all the days in the hospital afterwards that are hard.

Okay, I'll update again when I know more.
~Christy

Ortho Pre-Op Info

The pre-op appointment with the ortho surgeon went fine.  She answered all my questions.  Here's the deal:

Surgery:  Spinal fusion and removal of vertebra in lumbar spine plus ear tube placement and bronchoscopy

Date:  March 28th

Length of stay: 4 to 6 days - give or take

Cast:  Pantaloon, from just below armpits to mid-thigh, on left leg only.  The right leg will be cast-free.  A hole will be drilled on the left side for her g-tube (which is good, because that is the side with the most cast).  The left leg will be bent slightly at the hip.  So, she won't be completely horizontal, which is great.    But, I have no idea if that slight bend will be enough to get her into a reclining car seat.  So, we'll just have to see what they say then.

She might come out of the OR with NO cast.  It will depend on how her body does during the surgery - meaning how her heart and body do under anesthesia and how long she's under.  If they have to give her a lot of fluid, then they will have to wait a few days to let her settle down before casting her.  If she comes out without the cast, they will have to keep her pretty heavily sedated to keep her from moving around.  I really hope this isn't the case.   In July, after her 4th heart surgery, even though she was completely "loaded" with every narcotic, pain medication and sedative around - she STILL sat up in bed just hours after surgery.

Brace: might be an option after the first month.  She said she will have a more specific plan after the surgery when she knows how much work she did and how she thinks it went.  The problems with a brace are 1) she is very active and 2) a brace doesn't fit as well as a cast, which can lead to problems during recovery.  She said she would rather not do a brace for those reasons, but if Harlie isn't tolerating the cast, then perhaps after the first month, a brace might work.  We'll see.

Activity:  The surgeon said that she can weight bear - which totally shocked me.  Personally, I don't see how in the world she could manage the extra weight of the cast - especially since it will be considerably more heavy on her left side.  She would certainly have to be very supported - but perhaps we could do that with her old walker.  I would imagine she would have to hop a little on her right leg - which she can't do now, much less with the cast.  I guess it will come down to Harlie's desire and determination.  I'm pretty anxious to see what she'll do.  But the thought of her falling while in the cast makes me cringe.  So, I'm not sure what I'm going to be comfortable with her doing.  Again, we'll just have to see.

Follow-up Appointments:  None, unless we are having issues/problems.

Incision:  I'm certainly a little nervous about there being an incision under a cast that we can't see for 4-6 weeks.  And she also said that she will have to have a bone graft of some sort when she puts everything together after removing the abnormal vertebra.  In my head I screamed, "BONE GRAFT?!?!?"  ACKKKK!!!! (You might remember the last bone graft experience we had.)   But, in reality I, very calmly, but with a look of concern said, "Bone graft?" Then she said, "If it gets infected, she'll get a fever and we'll know."  Ha!  If only Harlie was that normal.

Future Growth:  Unfortunately, she said she can't do anything to save the growth plates in the area she's fusing.  Once they are fused together, they won't grow.  That is the very unfortunate part about doing this surgery at such a young age.  She still has a lot of growing to do!  So, she will be short waisted - her lumbar spine will be down a vertebra and then two will be screwed together.  So, that's a bummer.  But, something I expected, nonetheless.  Waiting longer will just make things harder/riskier to fix down the line.

So, overall it was a good appointment.  And it was relatively quick - just under two hours.

I was so bummed I forgot my camera.  I would have loved to show you how darn cute and funny Harlie was when she was getting x-rays.  They took a bunch and Harlie was SO good!  She can now stand all by herself - STILL - while they take them.  She follows instructions as to how to stand and where to put her arms.  And she smiles when doing it - like she's having fun!  I think she thinks the light shining on her is funny.  I don't know.  But, it's really funny to watch.  The only ones that we had to struggle with was when she was laying on the table and her hips had to be straight, but her upper body had to lean to the side.  She didn't like that one at all.  Maybe it was uncomfortable for her.

Oh!  During vital check the nurse put a pulse ox probe on her finger.  It read her oxygen saturation percentage at 86% (which is great for her - that number is between 95-100 for a normal person) and her heart rate in the 80s I think.  Well, he looked up at the monitor and said, "It'll level out here in a minute." And so we waited.  It finally dawned on me that he was thinking/hoping that her sats would get higher.  So, I said to Brandy, "Wow, 86, that's great".  And he said, "oh, that would be very bad".  hehe  So, I had to tell him that number was not going to increase any time soon.  We'd all die of starvation waiting for that to happen.  Ahhh, I don't know why that makes me laugh.  I must have a sick sense of humor.

It reminded me of when Harlie was just a few months old.  Back then we were establishing Harlie's doctor list, so the nurses didn't know her yet.  And they would always try to look so calm when asking me, "So, what are her sats normally?"  And then I would tell them, "In the 70s and 80s".  And they would say, "Oh, Thank God!  She had me scared!"  It cracked me up every time.  Weird.  I know.

So, that's it.  Okay, more later!
Thanks!
~Christy

Another Pre-Op Today

Today we head back up to Northern Virginia for our pre-op appointment with Harlie's surgeon.  I am so excited about this one because I am hoping that I'll be able to get a much better idea of what to expect.  I've got a whole list of questions for her, so I hope she's ready!  It's hard to believe her surgery is just four weeks from yesterday.

At the end of last week I got a call from her ENT's office with a date for her ear tubes and bronch - March 8th.  Yay!  But, unfortunately the team working on her case for her spinal fusion surgery said they were not comfortable with her having two procedures under anesthesia so close together.  So, they have coordinated her ENT doc to come in and do the ear tube and bronch right before her surgeon does the spinal fusion.

On the surface, that certainly makes more sense.  I just didn't intend for her to go this long with fluid/issues with her ear.  And her speech therapist said that if her ear is full of fluid than it sounds to her like she's underwater.  Which I suppose I already knew.  So, in the next several weeks we will start to try to explain what's going to happen - and I just wanted to give her the best opportunity to hear and be able to understand what we're saying.

But, the thought of having to do the IVs, anesthesia, recovery, etc. twice in 20 days isn't appealing, either.  And I'm thinking that it would only make things more traumatic for her.  So, we'll just have to make do with her hearing and hope that it doesn't become infected in the meantime.  Although now that I'm thinking about it, I guess I could take her to see her pediatrician for antibiotics if that happens.

Changing the subject...

Last night Tom and I went to a MOPs (Mothers of Preschoolers) event.  Normally we meet once per month - just the moms.  But every year around February, they have a MOPs and POPs event and the dads come, too.  There is a speaker and usually the topic has something to do with marriage.  Last night it was a couple who teaches communication tips for couples.

They had a hand-out and it showed a communication technique that we had to think of something that bothered us and use the format to see if it helped communicate what was the real problem.  They had some good tips - but I'm not sure about this one.  It's called the XYZ technique:

When you do X, in situation Y, I felt Z.

So, this was mine:

I changed "you're selfish" to:  When you only consider how you feel about a dog, when we discussed it last, I feel like you don't want me to be happy.

And this was his:

When you ask me for a dog, everyday (which, for the record isn't true!), I feel like you are trying to kill me.

So, it appears we haven't made any progress towards getting a dog.  And I have had two dreams that we got a puppy in the last week.  One day, we'll have a puppy.  I hope.

Okay, enough yapping, I have to get ready for our road trip.  More later!

Thanks,
Christy

Back to reality...

I haven't had a chance to really research this yet.  However, I thought I'd just show you an e-mail I just received regarding Harlie's body cast (in two months):

So, I spoke with Nancy in the ortho clinic regarding the cast/car seat issue. She looked up the type of cast that Harlie will be in and it looks like it will be a pantaloon cast which goes around the waist and down the leg. The legs may be held apart with a bar. She will be unable to bend at the waist or sit upright. Nancy said that there are no car seats for children in this type of cast and that they do have a loaner program for “ez on vests” which some children use while laying down in the backseat. 
However…she said that because of the trach issue, Harlie may require ambulance transport. Apparently, they end up using ambulance transport for many of their kids who require full body type casting. Does your insurance have ambulance coverage just in case?


WHAT?!?!?  Ambulance transport???

I'm going to need a few minutes.

Okay, I'm going to need more than a few minutes.

Just off the top of my head, the ambulance transport hugely impacts everything.  EVERYTHING!

School, doctor's appointments, therapy (I'm thinking speech is all she'll be able to do and even now that's a maybe), walks around the neighborhood or mall or just to get out of the house.  I suppose the cast does that on its own.  If she's strapped in while laying down on the bench seat in the 3rd row - just getting her in and out of the car is going to be an issue.  I certainly won't be able to do that by myself!

For six to eight weeks!!!

Now I know this isn't the worst thing in the world - trust me.  But, oh boy is it really gonna suck.

Okay, I'm going to calm down now.  I will deal with each thing in its own time.  Thinking about it and worrying about it two months prior doesn't really do much for me.  It sounds like I won't be able to do that much planning anyway.  I'm going to have to wait and see how she looks and how she'll be transported and what she can do, etc. before we can figure things out.  Although I might want to look into homeschooling (not me - by someone else) for that time period.  It doesn't sound like she'll be able to take the bus to school.  Or be able to participate while laying down the whole time.

Oh, my sweet little girl.  There are just not enough hugs and kisses in the world....

~Christy

PS - And some of you think I can't handle a puppy.  A puppy is a cake walk compared to this.  (I say with a smile).  And just to open myself up to more criticism/support - after much research, I've decided the perfect pet for our home is a

Pug. 

I think our family could use some smiles when this whole thing is behind us.

Thanks!

We have a date.

Ahhh, another date.  Haven't had one of those in what - five months?  Well, it was a nice stretch.

March 28, 2011.  Spinal Fusion Surgery.  In DC.  Be there or be square.  Just kidding.

But the best part is that this surgery requires THREE pre-op appointments - all in Northern Virginia.  Cardiac pre-op, anesthesia pre-op and surgery pre-op.  And none are on the same day.  Nice.

I really need to look into a mileage reimbursement program.

So, back to feeding for a moment... I talked with Brandy and we came up with a plan to give me and Harlie a break from feeding.  She will feed Harlie breakfast, lunch and an early dinner and I will give her a snack before bed.  Basically we switched her snack and dinner.  If I choose to give it to her orally, great.  If not, I'll tube it.  Tonight, I tubed it.  We went all night with no anger or tears.

Last night I forgot to mention that I was so angry with her/the situation/Goldenhar Syndrome/God that I had to go to CVS and the grocery store to try to clear my head.  It was late.  It was COLD.  And I HATE to run errands like that.  And honestly, the trip wasn't even necessary.  Well, I suppose it was given the situation, but you know what I mean.  Tom put her to bed while I was gone and I didn't even say goodnight to her.  I'm not proud.  But I was mad and needed some time away.  Tonight was a better night - for all of us.

And Murphy had a great night - we got notice that school has already been cancelled for tomorrow due to the prediction of 3 to 5 inches of snow tomorrow.  Still not a flake to be seen.  I don't even think it's due to start until late morning - like 9-10am.  Crazy.

As always, thanks for reading!
~Christy

Ortho Appointment Details

I won't keep you waiting.  It's surgery time.  Well, we're shooting for April.  I need to call her scheduler tomorrow to schedule it.  But I think we'll try to work it around Spring Break, if possible.  Which kills me.  Because while everyone else is doing something fun, we'll be doing ... that.

Here's the skinny:

There has been a significant change in her spine since last year (and not in a good way).  She has a hemi-vertebrae (half a vertebrae) at the bottom of her spine (sacrum), right above her left hip.  Not only is it half of a vertebrae, it is very abnormally shaped and it looks like it's a part of the one above it.  It's weird.  This is the one that needs to be addressed first.

She also has another hemi-vertebrae in her c-spine (her neck).  The surgeon said the higher the problem, the riskier the surgery.  We will wait on this one as long as possible.

She also has a butterfly vertebrae in her lower/middle of her back (lumbar).  That's the one that you can see - as it sticks out (kyphosis scoliosis).  When you hear the term scoliosis, most think the spine curves to the left or right.  But, with hers (in this particular area) it makes the spine curve front to back.  So, from a side view, it looks like a "c".  So, the abnormal section in her sacrum causes a left/right shift and this one causes a front/back curve.  I hope that makes sense.

She wants to do the sacrum surgery first.  She wants to see how removing the hemi-vertebrae (and then fusing the vertebrae above/below it with pin/screws) will affect the rest of her spine.  It might do enough good that we can wait a while longer on the other areas.

She said she doesn't want to wait anymore.  Too risky.  The worse it gets, the harder the surgery, the harder the recovery.  Since her problems are structural (meaning it's not just a curvature - the structure is abnormal itself) a brace is not an option.

The surgery itself will take about 3-4 hours.  And she will come out of the OR with a BODY CAST.   A BODY CAST!  UGH!  She said she is too wild for anything less.  Here's what her and Cooper were doing BEFORE the surgeon came into the room (you can see the kyphosis in her back):

Brandy took Cooper out of the room so I could talk to the surgeon.  But, Harlie remained pretty wild.  I think she was feeling a little sassy with her new hair cut.

So, a body cast for 6-8 weeks.  She will put only one leg in the cast and will bend it slightly so we can get her in the car seat.  The surgeon gave me a time frame of 4-6 months.  Since she'll be in the cast for 6-8 weeks after that, we are going to try for April, so she can be out of the cast before the summer heat hits.  She's already so hot-natured as it is.  Oh, and if everything goes great - it will be a 3-4 day hospital stay - in DC.  Oh, that drive is so hideous!!!  It took 4 hours to get home tonight!!!

So, that's it.  Not looking forward to it, that's for sure.  But, it is months away still, and within 7-9 weeks it will all be over.  And hopefully, she'll be much "better" after.

And if you didn't see my earlier post, check it out.

Thanks!
~Christy

Ortho Appointment Tomorrow

Just a real quick one tonight.  We have a very busy day tomorrow.  I am going to try to squeeze in some preschool for both Harlie and Cooper, a haircut for Harlie, and an appointment for me before heading up to Northern Virginia for Harlie's orthopedic appointment at 2:30pm (Tom says it's impossible and that I have completely overbooked the morning, which is probably true).

I think the last time she saw this doc was a year ago.  Probably longer.  And she wanted to see Harlie in six months.  But, that just couldn't happen with Harlie's heart surgery this summer.  There's only so much we can do.  It kills me how busy Harlie's schedule is - and that she can't accommodate every doctor's requests.  There's just too many!!!

And she's walking, jumping a little, trying to run (getting faster) and she's in gymnastics.  So, it's kinda easy to put her spinal issues on the back burner.  I'm hoping that since she's doing all those things, we can continue to put off her spinal fusion surgery (if that's still the way the surgeon wants to go).

The last time we spoke, the surgeon said that the longer we put it off, the more difficult the surgery will be (on both the surgeon and Harlie).  But the reason why we want to put it off is so she can grow.  Once you fuse the vertebra together, there will be no more growth in those areas.  And she's only four and still has a lot of growing to do.  So, we'll just have to see what the x-rays show tomorrow.

I have really enjoyed these long breaks from serious appointments.  And I'm not looking forward to the decisions regarding her spine.  No fun!!!

As always, I'll let you know how it goes!  Wish us luck!

Thanks!
~Christy

Spinal Surgery Appointment

Harlie's appointment with her orthopedic surgeon went well. I was secretly hoping that she would say that Harlie's spine has not gotten worse since earlier this year, and that we could wait.

Well, what do you know I got what I wanted! WoHoo!!!

She went over the CT scans with us and showed us where the abnormalities are, and what our options are to correct them - or make them better anyway.

Luckily - none of the abnormalities are in her thoracic spine, which is fabulous. That's the part of your spine that needs to grow to be able to handle the growth of your heart and lungs.

Her abnormalities are in her cervical, lumbar and sacral areas. I'll get Tom to scan in the pictures so I can show you.

But, basically it came down to this ... the risks of doing the surgery are greater than the risks of waiting to do the surgery. So, she will see Harlie every six months to watch the progression of her kyphosis scoliosis. The goal is to buy her some time - hopefully one to two years.

She said that three years old is very young to get spinal fusion surgery. Once you fuse that part of the spine - there will be no growth in that area. So, clearly, the more time you give them to grow, the better it is for them in the long run. Plus, she will have to wear a brace or a body cast for three months - and the more wiggly the child, the harder it is for them. If we could wait a couple of years - say till she's five, then she might be better able to understand what's going on, which will help her recovery.

I'll explain more when I have the pictures to show you.

So, I'm very happy that we can put this on the back burner for now. I was really dreading this surgery. Well, I still am. But now it's far away again, so that's good.

Ahhhh, it feels good to get good news every once in a while.

Take care,
Christy

CT scans Friday

So, I got some bad news today. I was on the phone for a while today with the Radiology department at the children's hospital in DC. Harlie is scheduled for her CT scans at 8:30 am on Friday. Well, after several phone calls and difficult conversations - it turns out that we have to be at the hospital at freaking 6am Friday morning. Which means we have to leave at freaking 4am! This is going to cause one heck of a crummy chain reaction, that's for sure.

There is so much to this story, it is hard to figure out where to start! Honestly, I feel sorry for anyone reading this post. It might make your head spin.

Plastic Surgery

A few weeks ago, we got a second opinion from a plastic surgeon at the hospital in DC. The plastic surgeon that did her first jaw surgery is in Norfolk, VA. So, Tom and I went and met with the DC guy about Harlie's jaw. But he can't really tell us anything until he sees what's going on inside. Which means that he needs to see CT scans - current ones.

One thing to keep in mind here is that getting a second opinion is not an easy thing to accomplish. It took THREE months to get that appointment. In a perfect world, she would have gotten the CT scans first - but the surgeon needs to order them, and in order to order them, he has to see you first.

Spinal Surgery

She needs CT scans for her spinal surgery. I started working on getting the CT scans scheduled back in JULY! And she will finally get them October 9th.

Now I knew that the plastic surgeon would want them, too. So, I made the CT scan appointment for after the plastic surgeon appointment so he could put his order in and they could do all of them at once, to consolidate her anesthesia time.

In order to truly get a second opinion on her jaw, we'll also need to meet with her surgeon in Norfolk. And he'll need current CT scans, too. So, to avoid having to get CT scans done in Norfolk, I will get a copy of the study done on Friday on disc and will take it to him.

Backing up for a sec, her original CT scan date was Wednesday October 14th. When I called to schedule the appointment with her Norfolk plastic surgeon, his assistant told me his next clinic day (he only has clinic days once a month) was Tuesday, October 13th. The day BEFORE her CT scans! So, that would mean I would have to wait till November to see him. But, he isn't going to have clinic in November because of some other thing - so that put us in December! Ugh! More time wasted!!! I was terribly bummed, but what are you going to do? I scheduled our appointment for December, kicking myself for this whole second opinion idea. Keep in mind that our original plan was for her to have jaw surgery this fall - which clearly, didn't happen.

So, I thought about it and decided it could not wait until December. So, I called and rescheduled the CT scans for Friday the 9th, and then called back her Norfolk guy and she got us in to see him on the 13th. Perfect. Whew.

Today, in my conversations with Radiology, it turns out they had her scheduled for conscious sedation instead of general anesthesia. And as I've been told, general anesthesia is "safer" for her in that she is more closely monitored by an anesthesiologist and better equipment (?). I haven't done much research on this because, quite frankly, there's only so much time in a day. And really, it doesn't matter because if an anesthesiologist tells me it's safer, well, I'm not going to argue.

Anyway, they told me that they didn't think they could get an anesthesiologist on such short notice. Short notice? I started working on this in JULY!!! The scheduler knew about her complexities because she is the one that told me she couldn't have an MRI - weeks ago. Anyway, I tried to stress how important the timing was of these CT scans. If she can't get them on Friday, that will set us back an additional TWO MONTHS!

So, after a while they called back and got everything straight. And that's when they told me about having to be there at 6am. Brandy is going to kill me when she finds out. And I swear, home health nurses just don't get paid what they deserve. But, given the crucial timing, I'm thankful it will still work out, so I guess I shouldn't complain.

Oh, they also told me that she was scheduled for a lumbar puncture (ew!). When I asked why the woman said, "because the patient is complaining of back pain." WHAT? I told her she needed to double check that paperwork, as my daughter just turned three, and she hasn't complained to me - much less to someone up there. She called me back to tell me that she's getting that so they can put contrast in there for the CT scans. Oh, okay. Now that makes sense. All's good. Although the words "lumbar puncture" kinda freak me out. Which is kinda funny considering all the words I've heard and learned since Harlie's birth.

So, Friday is going to be a loooooong day. And for the chain reaction... Saturday morning I will get up early in the am to go run 9 miles. The farthest I have ever run in my life. Exciting stuff.

Then as soon as I get back I will have just enough time to eat, and get ready to go to work. I'm working this Saturday where I used to work - just helping out when I can. I will work 12-5. Then I will come home and get ready to go to my 20 year high school reunion. 20 years. Holy moly. Talk about a long couple of days with lots of stuff packed in them.

And, if you read - and understood - all this, I'm proud of you.

Tomorrow is Harlie's hearing test. I'm dying to know the results. Her appointment is at 3:30.

Well, that's it for tonight. And I said that I didn't feel I was up to blogging tonight. Ha! I'm sure I'll regret it in the morning.

Thanks,
Christy

Spinal Update

As you may remember, Harlie still needs to have surgery on her spine. We've been seeing a local orthopedic surgeon for check ups. While I like him just fine, he only operates at St. Mary's Hospital. And that hospital simply cannot handle Harlie. So, Harlie's cardiologist in DC recommended a surgeon there, which works out great. I am definitely most comfortable with her having surgery there. We met this surgeon back in the spring. And she agreed with our local doc that surgery would need to happen sooner rather than later, meaning sometime this year or early next. She ordered x-rays, CT scans and an MRI.

But then she had heart surgery. At one point I was thinking that they could do all that stuff while Harlie was recovery from heart surgery. But during her recovery I realized that was not an option. She had been through enough and her healing was way more important than those studies. They would have to wait.

So, after numerous phone calls and a whole bunch of time I finally got the scans scheduled for October 9th. During the scheduling, she asked me a bazillion questions. One of them was, "does she have a pacemaker?" Well, not exactly. She has the leads, but they are not hooked up to the battery device yet. The leads were installed during her first heart surgery at four days old and were placed for future hookup, when needed. She put me on hold for about 5 minutes. And then came back to tell me that she cannot have an MRI. As long as she has those leads, she cannot have an MRI. Here's why. And I'm thinking that she'll have those leads for as long as she has her own heart. This is quite unfortunate. I had no idea this was the case. Not that it matters, really. We couldn't have done anything differently. Still, it's pretty scary to know that she cannot have an MRI. An MRI shows soft tissue, while a CT scan shows bone. And a lot of stuff can grow in soft tissue. And it doesn't make me feel very good about going into spinal surgery without them being able to see her cord clearly.

So, I've been speaking with the surgeon's coordinator about it. She's spoken with her surgeon and they are ordering some additional studies in hopes of being able to piece them all together to give them the best information possible.

Now, just today I realized that we don't have an appointment scheduled for after these studies are done! Can you believe that? Once she sees all the information we have to talk about what procedures she thinks Harlie needs. And then we'll need to set a surgery date. So, tomorrow I'll be on the phone to schedule that appointment. I just hope that I don't have to wait three months to get in to see her!

Craniofacial Plastic Surgery Appointment

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

Sounds like a winner of a year!

Well, just a short one today. Talk to you later!

Take care,
Christy

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.