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| My Babysite | | | Hi. This website is about our little girl, Harlie, and how she changed our lives forever.
At 16 weeks pregnant, we found out that Harlie had a mass growing inside her chest. The only thing we could do was wait and see how fast it was growing. At 20 weeks we had another appointment 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 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 is backwards. 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 rest of my 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.
After a quick assessment of her, the doctors came in to tell us what they found. They didn’t see everything at the time, but this is what we ended up learning over the course of her first year:
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).
Her other defects are: Underdeveloped lower jaw (micrognathia), posterior-rotated low-set ears, dysmorphic left ear with no canal, dermoid, cleft and coloboma in left eye (her eye didn’t close properly), skin tags on both sides of face, vertebral anomalies (missing C6 and C7, C1 fused to the occiput and hemivertebrae L1, L4), missing ribs, misshapen skull, anoperineal fistula and congenital lobar emphysema. This is what we know so far.
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. (Her third heart surgery will be the Fontan, at around age 3.) In August, 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 tongue cannot lay flat, so it stays retracted in her airway, causing an upper airway obstruction. So at 16 days old she had surgery for a tracheostomy and gastrostomy (feeding tube). She is completely tube fed for now. Because of the trach, she cannot make any sound.
To date, she has had 10 surgeries and spent more than 5 months in the hospital. Despite everything, she is such a sweet, happy, agreeable baby. She hardly ever cries – only when she’s in physical pain – and she lets all her doctors examine her without complaint. We think she is beautiful. And she is worth every ounce of effort we have to give to keep her safe and sound.
To see a video montage of her first year in review, go to My Favorite Links and click on Harlie’s First Year. Don’t forget to turn the sound on.
You can keep up with us by reading My Baby Journal. I try to update it often. Please feel free to sign in the Guestbook. We love reading all the messages of support from friends, family, and people we don’t even know. I can’t begin to explain how much those messages have helped us over the past year. So, don’t feel silly. No pressure to say anything profound. Sometimes, just a simple “thinking of you” can do wonders.
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 agreeable, happy spirit. I am thankful for my husband and son. 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, 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 and Harlie)
THE HARLIE FUND:
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. Please see the Journal entry for December 10, 2006, titled THE HARLIE FUND for more information. |
|  | | Recent Growth Chart | |
| 08/26/2008 | 23 months | 22 lbs. 0 oz. | 32.25 inches |
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| | | Updated Albums | 
Vacation 2008
07/24/2008 | 
Jaw Reconstruct
06/03/2008 | 
Nissen Surgery
05/21/2008 | |
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Visitors since 09/28/2006 |
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