Saturday, September 30, 2006

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.

Friday, September 29, 2006

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!

Thursday, September 28, 2006

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.

Wednesday, September 27, 2006

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

Monday, September 25, 2006

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),
missing ribs,
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 32 surgeries and spent more than a year 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.

As of June 2013, we are preparing for Harlie's fourth jaw surgery.  The first three were jaw reconstructions.  The first two, they took bone from her skull and implanted it into her jaw.  The third they took her fibula bone (in lower leg) and put it in her jaw.  Now they have bone to distract.

Jaw distraction is when they cut the jaw on the bottom, on both sides.  They put pins on both sides of the cuts.  Then they attach rods on the outside of the face.  Each day we will turn the screws, which will spread apart the jaw at the cuts.  New bone will grow as it heals.  Then we turn the screws again, essentially re-breaking the bone.  And again, new bone will grow.  This will continue daily for about six weeks.  In the end, we hope to make her jaw longer, which will allow her tongue to lay flat, bringing everything out of her airway, allowing her to breathe through her mouth and nose.  That's our ultimate goal anyway.  A better jaw construction will bring more normal breathing, eating and speaking.  Just a few things we want for our little girl!

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)

Sunday, September 24, 2006

Harlie's Surgeries

Here is the list of Harlie's surgeries. As of May 1, 2013, she has had 32 surgeries, with more to go!

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 - Jaw Distraction scheduled - Boston Children's Hospital

12/13/13 - 7th Heart Cath - CNMC, DC

Procedures Under Anesthesia

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

Upcoming Surgeries

Jaw distraction - July 3, 2013
Prosthetic ear placement

More jaw surgery - despite three jaw reconstructions - her jaw will not be able to grow at normal pace.  No matter what we do, the rest of her face will grow at a faster pace than her jaw.  So, she will have to have future surgeries as she ages.

Oral surgery - She has the same number of teeth, with half the jaw space.  Currently, her mouth is very arched.  So, her back teeth touch first, not allowing her mouth to close properly.  At this point, we don't know what needs to be done to fix it (other than more surgery, of course!).

And whatever else comes up!

Harlie is the busiest six year old I've ever met. For some interesting stats from her 1st year check out this post. You won't believe it. I hardly believe it myself.