Courage is not the absence of fear, but rather the judgment that something else is more important than fear.

- Ambrose Redmoon

The way is not in the sky. The way is in the heart.


Monday, February 25, 2013

Day 25: Meet Lily (happy 7th birthday Lily) and John

Day 25 brings a HUGE HAPPY 7th BIRTHDAY to LILY!!!!  Lily and her mommy are very close to my heart.  Lily is so sweet and gentle when she plays with Xavier!!!  He just loves her.  She is an inspiration to many heart families....speaking of inspirations...wait to you read the amazing story of John.  He is such a miracle and has the BEST SMILE!!!  I hope you enjoy these two stories of HOPE!!!


Lily’s Story
By:  Julia Rowbotham (Lily’s Mom)

My husband and I were overjoyed to be pregnant with our first child.  At week 19 of the pregnancy we went for an anatomy scan to find out if our precious baby would be a girl or a boy?  I remember being so excited to find out what we were having so that I could go buy our first “pink” or “blue” outfit for the baby to wear home from the hospital.  The ultrasound was taking a very long time, and the technician kept coming and going from the room.  After what seemed like an eternity, we were told that the doctor would come in to speak with us.  The doctor came in, reviewed the images and told us he was fairly certain our baby had a heart defect.  The left ventricle looked very small, and he was not sure it would be viable.  Lots of medical terminology we had never heard before was thrown our way, but the only statement I really remember is, “worst case scenario would be that the baby has Hypoplastic Left Heart Syndrome a very rare and often fatal defect.” 
          After what seemed to be the longest 4 weeks of our lives, we made our first of many visits to the Fetal Heart Program at Children’s Hospital of Philadelphia (CHOP) where we met Dr. Rychik.  The fetal ultrasound was repeated, and we sat down with the doctor to discuss the findings.  We were told that our baby had what is called Hypoplastic Left Heart Syndrome (HLHS), a congenital heart defect.  Our options were to either terminate the pregnancy or for the baby to undergo a 3-series staged reconfiguration of the heart.  It was then that our lives changed forever!
          Lily Jane Rowbotham was born on February 25, 2006 weighing 6 lbs, 6 ounces at our local hospital.  At 4 hours old she was emergency transported to CHOP to prepare for her first battle as a heart warrior.  I was discharged the following day and able to join her at what would be our home for the next month.  On February 28, 2006 at 3 days of age, Lily had her first open-heart surgery the Norwood with the amazing Dr. Tom Spray.  To this day I will never forget seeing her for the first time after surgery.  Nothing can prepare you for seeing a baby so small with so many tubes, wires, and monitors.  It still haunts me……
          On March 14, 2006 following several days of low oxygen saturations and blood pressure, Lily was taken to the cath lab where a stent was placed due to shunt stenosis.  As we waited to hear that the procedure had been a success, a doctor from the vascular team came in to tell us that during removal of the catheters the right iliac artery had been torn.  They were going in to do emergency surgery to clamp the artery, stop the bleeding, and save our daughter’s life.  Needless to say, we were crushed!  How much more could we possibly ask this tiny baby to endure?  Why was this happening to us?  Why our baby?  It was a great moment of soul searching for me in realizing that this war would not consist of just one battle, but many battles that would test our faith, courage, and strength.
Lily pulled through, but the emergency vascular surgery was a huge setback for her.  She was placed on twice daily Lovenox injections to prevent clotting in the stent, and she had issues with feeding and getting enough calories to grow.  After what seemed like a lifetime, she was discharged from the hospital at the end of March.  I remember being terrified to take her home.  What would we do with this fragile heart baby?  Would we be able to care for her on our own?
On August 7, 2006 Lily had the second open-heart surgery of her staged reconstruction which is called the bi-directional Glen.  I would say that for Lily, this surgery was the hardest to recover from.  Her little heart never seemed to like the Glen circulation all that much, and those hemi-headaches were horrible!  We were able to come home after two weeks, and Lily became what we would refer to as an inconsolable baby.  She cried all the time, especially during the night.  She had horrible reflux which left her unable to sleep flat on her back, so she spent many hours rocking in a swing.  We struggled so much with knowing whether the issues she was experiencing were heart-related or just normal baby milestones. 
After one particularly miserable evening and speaking back and forth with our cardiologist Dr. Donner, we brought Lily into the ER at CHOP.  She was hooked up to all the heart rate monitors and we were shocked to see that her heart rate was 260.  She was thrashing around and screaming and just looked miserable.  Lily was admitted to the CICU, and after more monitoring was diagnosed with EAT (ectopic atrial tachycardia).  She was started on propanolol and monitored for a week as an inpatient.  The next several months were spent going back and forth to CHOP trying to find a medication that successfully controlled the EAT.  Propanolol, and Sotalol were tried with no long-term success.  The idea of doing an ablation of the areas causing the arrhythmia was also discussed, but the procedure was extremely risky in such a small child.  Finally, thanks to the wisdom and perseverance of a fabulous doctor, Lily was started on amiodarone.  The amiodarone proved to be the only medication that worked for more than 2 weeks, but one of its side effects is that it drops the heart rate and can cause bradycardia events.  On December 8, 2006 Lily had a pacemaker implanted by Dr. Spray to counteract the effects of the medication and keep her heart rate in an acceptable range.
Lily finally started to grow and thrive.  We were blessed to experience many months of excellent cardiology follow-ups and great feedback from her doctors.  On July 10, 2009 at three-and-a-half years of age Lily had her third and final open-heart surgery of the staged reconfiguration, an extra-cardiac fenestrated Fontan.  The surgery was on a Friday, and she was discharged the following Monday after a three day admission with no complications!  Our Lily was a warrior!!  In August she was taken off of the amiodarone, and was no longer experiencing any episodes of EAT.  Her little heart seemed to love this newly found Fontan circulation. 
It is now over three years later, and our Lily is living and thriving!  Who would have known that after such a difficult start to life, she would grow into the most beautiful, intelligent, feisty, and loving young lady we could ever imagine.  She is in the first grade in a public school district.  She loves math, reading, art, ballet, and gymnastics.  She is a miracle J
My hope for Lily is that she always sets goals for herself and works to achieve those goals.  I want her to live life as a “heart kid” to the fullest, and never let anyone or anything tell her that she is not good enough or strong enough to succeed.  I wish that one day her heart journey will inspire her to help others and to share her experiences with the world.  I am excited to see what is to come…..the sky is the limit for Lily!


Textbook pregnancies. 
I thought I would never know anything different. Our first baby came the first year of marriage. Our second, just 18 months later. With two csections under my belt, and one boy and one girl, I figured that would be it. The perfect little family. God was calling us to a different plan. He knocked on our doors, on our hearts. We began to realize that our son had no brother, our daughter no sister, and that we considered our children the greatest treasures and gifts we could have been given. And from then on we decided we would let God plan our family. If we could trust Him with our eternity, we could trust Him with our fertility. And the babies came. Breastfeeding spaced them about 2 years apart. After eight healthy children, I found myself pregnant again in July 2009. Sadly, that pregnancy ended in a terrible hemorrhage and I lost the baby at 12 weeks. Devastated, I felt really strongly that I wanted to have another baby. Just one more, please Lord, to hold, and never again would I take a new life for granted. I took all my vitamins, took walks every day, drank water and ate good food and 3 months later found that we had been blessed again, our rainbow baby. 
I spent the whole pregnancy terrified. I couldn't put my finger on it, but from the beginning of his pregnancy I felt something was wrong. I checked hourly for blood after the scary gushing of the previous pregnancy. At an early sonogram, the dr said that the baby was so small she couldn't really measure and not to get my hopes up. Another one a few weeks later showed a living baby but she said that the heart looked so tiny she could barely see it. She said that she would be surprised if everything turned out fine because something just didn't seem right to her either. We fretted and worried and tried to just keep praying as the weeks ticked by. I purchased a doppler to use at home to try to comfort myself listening to his heart, but to my dismay I heard lots of skipped heartbeats. It did not sound right at all. At 19 weeks, I was referred to a neonatologist since I was now the ripe age of 37. The week before that sono, an online friend discovered her child had a heart defect. I felt a jolt. What if that was what was wrong with my baby? The night before, I told my husband that I was pretty sure there was something massively wrong with the health our little one, and it might be a heart defect. He was puzzled and unconvinced. The kids were so excited to find out if they had a brother or sister on the way, but all I could think about was would we even be bringing home a baby in September of 2010…. I blogged about it to my relatives the night before as well… saying "if only I could promise them that everything would be ok."
A couple of minutes into the ultrasound and I saw the tech's eyes get as big as saucers. I knew the baby was alive because he was kicking. She focused on the heart. Zoom in. Zoom out. Hmmm. "Well I'm going to go get the doctor," she says… "Is it the heart?" I asked, not shocked, but deeply scared to hear the answer I practically already knew. "Uh, yeah, I think we need a better look. Something is not right." The dr. came in and after just a quick look said that he was certain our child has hypoplastic right heart syndrome, and we'd need to see the cardiologist soon. We took our two preschoolers who went along and sat outside in the sunshine with our sunglasses on, sobbing. Surely I would wake up from this terrible dream. 
The cardiologist and neonatologist spent an hour looking at his heart every month. It was a tricky one, not a typical defect but several mixed together. The rest of the baby looked great to them. Towards the end of the pregnancy my amniotic fluid levels kept getting higher and higher. They kept an eye on it weekly and almost sent me to have some drained, but just in time it would go down a little, then back up. Those same weekly appointments they would run a non stress test, and I would have to listen to his erratic heart and try not to cry the whole time. Everyone who heard it was concerned. We had a plan of care meeting with all of his doctors, and another neonatologist listening in to his details said that he thought it sounded like the baby had a syndrome to him, one with restricted swallowing abilities. I could not wait to get him out and into the hands of the drs at Texas Children's in Houston. 
We made a plan to have our son transported to Houston after birth. That way I could stay with my family and not have to go live there 3 weeks in advance, and I could keep my OB who was going to let me try another VBAC. After those first 2 csecs, the rest were VBAC!! But I still made new doctors nervous. No one would let me try except her, and I trusted her so very much. I just had to stay here. 
Two days before my due date, I went into labor. What a shock, none of my others came before the due date! I wasn't ready yet, had saved many things for those last few days..but off we went in the middle of the night to have our son. I made it to an "8" but his heart was not tolerating the labor well. The OB on call was going to let me VBAC for her partner until she watched his heart rate on the printout, and then convinced me that we were playing a risky game. To be safe, we needed a csection immediately. We consented, and John Alexander was born Sept 20, 2010, 8 pounds 14 oz. John for the patron saint of the heart, and Alexander meaning "warrior."
"Mama, we have an ear defect. I just want you to know before you see him. Do you want to see him?" the nurse said to me. The room was buzzing but everyone looked at me to see what my reaction would be. Of course I wanted to see him… please right away…. and he was beautiful, pink, and perfect to me. It was love and such relief that he would get the help he needed for his heart now that he was born. He had his echo run soon after, and they were worried. He needed a balloon septostomy to make his hole between the ventricles bigger soon. They called for transport and baby John was driven by ambulance in the rain. I held him for five minutes on a pillow so as not to mess up his arterial line before he left, and we had him baptized. 
I had to heal up from my csection before I could go to Houston to be with him, so dh went first to be with him. I joined him a couple of days later, staying updated by phone & pics. John had his first surgery at 7 days old, an arterial switch for his transposition of the great arteries. He did not end up needing the BT shunt for his tricuspid atresia because he had a balance of blood flow to the lungs. As for his ear defect, we learned that he has Goldenhar Syndrome, a group of craniofacial defects typically affecting one side of the face and can have organs involved, such as the heart. It occurs very early in embryonic development, about the sixth week as the heart is coming through the head into the chest cavity. It is VERY rare to have Goldenhar cause hypoplastic right heart, geneticist estimated 1 in 1,000,000. His right ear did not form, just like the right side of his heart. He will eventually have complete ear reconstruction with rib bone graft (series of 3 surgeries), fat deposited in cheek, jaw bone distraction, eye socket surgery, and possibly more. 
He struggled eating after his first surgery. We didn't know if was his mouth, which has some nerve paralysis from the Goldenhar, or his heart not strong enough, and his stomach volume was also low. We consented to a Gtube, hoping to take him home. But he became unstable with his sats when he cried those next few weeks. His pulmonary arteries would clamp down and he would de-sat to the 40s, 30s, 20s, and require blood transfusions & oxygen blasts to overcome. He was too unstable to take home. He needed a shunt, but if we could just make it to the Glenn, we could avoid another surgery. John and I lived on the cardiology floor for 12 weeks until he was big enough to have the second surgery, the Glenn. It was extremely hard to be away from my family during this time, they were only able to visit twice with school & work & large family life with one parent keeping them booked. 
John rocked his Glenn surgery. It also included aortic work since his aorta was not growing well. He did have left vocal cord paralysis but no aspiration. Just 8 days later we were able to leave for the first time and go home. It's been 2 years since then. We haven't been back. John has been very stable and wonderful, super smart and funny! He still has eating challenges that we are in therapy for. But he is enjoying life with a party of siblings always showering him with attention. He is our joy, and we have learned so very much from him. 
We are expecting his next and hopefully last for many years to come heart surgery, the Fontan, either this summer or next. 

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