Julia Elizabeth was born on December 19, 1997 by C-section because of minor complications with her Mom. My 17 year old daughter had a normal pregnancy (as normal as could be expected) despite her age. She was in her senior year of high school and did an excellent job preparing for her child and maintaining her grades. She had all the right plans in place to finish school, even go on to college and although I was not overjoyed about becoming a grandma at 38, I was sure my daughter and her boyfriend could handle the baby. Her OB let both Julia’s father and I go in to the OR room that morning. There was a little problem getting Julia out of the birth canal but she was never distressed. Julia scored fine on the Apgars, she was perfect! We both got to hold her and then she was taken to the nursery for the routine check up. We went back to recovery, and watched patiently for 2 hours as other families were bonding with their newborns. My daughter was doing well, and was moved to a regular room. Julia’s father went to find out where their baby was. My daughter got settled in her room and he came back to report that Julia had low blood sugar and a nurse thought she heard a heart murmur so they were having a doctor check her before she came to the room to be with us. Another couple hours went by and we went to find out where Julia was.

That’s when we met Dr. Dillon, Julia’s cardiologist. He took us to a consult room and explained Julia’s heart defects to us. He said she had aortic atresia, and no significant separation of her ventricles. Without immediate intervention she would not survive. We were offered the dreaded three treatment choices. It was so hard to believe that her perfect little body could have such a critical defect hiding inside! We chose the palliative surgeries because they would offer her a chance to live, and could be done right away. We also had to decide where she would be taken because there is no hospital in our area that would do open heart surgery on an infant. We chose Children’s Hospital Medical Center in Cincinnati Ohio because it was closest to our home, and we knew Julia’s parents would need lots of support during this time. Julia rested comfortably that night in the NICU, an IV in her umbilical cord to keep her PDA open and too many heel sticks for blood tests to count. The transport team from CHMC came for her in the morning. Julia’s parents were allowed to bathe and hold Julia that next morning, and then she was on her way.

Dr. Peter Manning did her surgery 3 days after she was born. The first night after her Norwood, a cardiologist sat at the end of her bed and carefully monitored her. She explained everything she could about Julia’s heart defect, treatments, outcomes, complications as she adjusted levels of medications and the respirator. The staff cared for her with love and compassion and helped our family accept the reality of Julia’s condition. They made sure Julia had the same nurses each day and made it easier for us to cope with all the equipment she was hooked up to. Because they got to know us they could explain things in terms we could understand. We are indebted to Dr. Luby Abdurrahim, (and to everyone that came running to her aid), for saving Julia’s life the evening after her chest was closed. Either she had a blood clot in her shunt, or a spasm, but for some reason she went into cardiac arrest as our favorite nurse was bathing her. Even her surgeon was back in the hospital within minutes of the code. 45 minutes went by before we found out God had spared Julia. We found out that nearly all faiths were represented at her bedside as they resuscitated her, and together prayed for His help.

It was another week before she was finally able to come off the vent for good. Several attempts were made but there was too much swelling her in throat. A few doses of steroids did the trick. She also had to be weaned off the narcotics slowly. She cried for hours after extubation until they figured out her body had become addicted to morphine. The next big hurdle was to get Julia to suck the bottle. She was in a step down until for another week until she learned how to suck, swallow and breathe. After choking down 20ccs, while sopping up another 10ccs dripped down her neck, we learned how to gavage feed. She came home from the hospital with an NG tube for feedings, (using a high calorie mixture of breastmilk and formula) and 3 medicines, captopril, Lasix, and baby aspirin. It took a couple months before Julia was able to take all her food by mouth without falling asleep.

A home health nurse came to our house weekly for a few months to track Julia’s progress. Six months later Julia had to go back to Cincinnati and have stage 2 done. She had a bi-directional Glenn procedure done at 7 months and recovered faster than the first surgery. It was easier in some ways the second time, we knew what to expect. It was also very hard to let them take Julia through those double doors because in 6 months time Julia had become the center of our home. It was agony to see her in pain or upset and not be able to swoop her in our arms and comfort her. Within a couple weeks she was back to her happy little self, though.

Progressively during the next year Julia was becoming short of breath with light physical activity. During a catheterization she had a stent placed in her left pulmonary artery. We left home that morning expecting to have a cath and maybe stay overnight. Eight days later she was released to go home. Lesson learned...always take your toothbrush and a change of clothes when going to the hospital with Julia. She began taking coumadin to prevent clotting. That began her close relationship with the local lab techs, as she is monitored closely. It is so routine for her now that she tells them what they are going to do and that she is doing a good job!

Julia started Early Intervention at 4 months old. She is behind developmentally but is making steady progress. She could sit up at 8 months, crawled at 13 months, stand at 18 months, walked at 24 months. She has Sensory Integration Dysfunction and mild autism. We had her evaluated by a team of experts in Cincinnati for delays and peculiar behavior and she was given the diagnosis of an autism disorder called PDD NOS. I can’t help but wonder if the trauma she suffered the night of her chest closure isn’t the reason for Julia’s delays, but at this point the only important thing is that she continue to develop and learn to her full potential.

She also has strabismus, (crossed eyes), that we were told could only be corrected with surgery. She had two surgeries and saw 3 different ophthalmologists. Her eyes are aligned straight now, but she gets vision therapy that involves prism lens glasses and patching to get her eyes to work together. An optometrist does this kind of therapy. The glasses have made a huge improvement in her coordination and attention span. She also had ear tubes put in when she was 2 years old because of recurring ear infections.

Julia began attending preschool in February 2001 and she is doing very well. She has 7 other disabled kids and 4 on target kids in her class and receives O.T., P.T., and speech therapy. She likes to go to school and we can see progress in her social skills. She will have an extra-cardiac conduit Fontan surgery some time in the future.

Update: December, 2001

Julia had her Fontan operation done on October 4, 2001. She was in surgery 6 hours. Dr. Manning performed an extra-cardiac conduit Fontan, and was able to put in a conduit large enough to support her growing body. In addition to the Fontan, the team opened her LPA along her existing stent and stitched a patch to enlarge the artery. Then a larger stent was put in to keep the vessel open. Our hopes are that she will not have to undergo any more open heart procedures.

Julia endured the surgery day very well. She was extubated the day after surgery, but by evening her left lung collapsed. CPTs were done very gently, but caused bleeding around the suture lines. Two days and 4 units of blood later, she was back on the path to recovery. Then a series of nasty rashes began. Some were reactions to the tape (pressure bandages), which left her skin looking like 3rd degree burns, another kind of rash broke out all over her back, chest, and armpits (that was from the bed linens). A bad goose-egg bump on the back of her head was discovered, from laying with her head tilted to one side during surgery. All together dermatology counted six different types of rashes. She was so uncomfortable until she got an air mattress that circulated air underneath her. Antihistamines were given to help with the itchies, and her Mommy blowing on them also helped a lot. As the rashes, bumps, and bleeding subsided Julia quit drinking, eating, and playing. !

The dreaded pleural effusions set in. She had a pigtail catheter placed, and soon was back to her happy self. The total stay was 15 days. Her saturations are now in the the mid to upper 80's and Julia has the energy level of a normal 4 year old. She takes captopril, Lasix, Aldactone and coumadin. It is indeed a hard journey to make - but worth every minute to look at her so pink and playful now. We just celebrated her 4th birthday and look forward to many, many more.

Update: December 2003

Julia is 6 years old now. She had her fenestration closed via catheterization in January 2003 and is usually full of energy and fun. She has progressed to chewable tablets for almost all her medication now and knows all the names and doses. She has invented games of most procedures or treatments she has, and for the most part she expresses positive feelings about them. If it is going to hurt she always puts on a stern face and says "it only lasts a second so be still and you'll be done" She objects to having her teeth worked on. She will have dental surgery to take out some loose teeth and clean the others. A minor surgery stills requires that she stop her coumadin, take antibiotics and because she is older now, her showtime is 3:00 p.m. It doesn't matter how many times we go through this, surgery is the scariest thing I know.

Each year that we celebrate her birthday—we consider it a blessing from God and we are fortunate. Her parents still worry over common colds, scraped knees, eating enough vegetables, while her Nana thinks that she is so lucky to have these normal troubles. So many others have been lost while we enjoy our Julia that it will always seem bittersweet to love her so much. She continues to teach us that life is precious and today is all that matters.