Day 28: Meet Adelaide, Martin and Xavier

I am so sad that today is the last day of pediatric heart month.  It has been such an honor to share the stories of fellow heart families.  I hope that their strength and faith has been a source of inspiration.  All our heart warriors and angels have a story that needs to be shared!  

Day 28 we get to meet Adelaide, Martin and Xavier...I hope they make you smile!!!!  KNOWLEDGE IS POWER!!!  HEART HUGS!!!!   

MEET ADELAIDE!!!

Today, I saw a YouTube video that someone posted on Facebook… it was a video of a nurse giving a newborn baby a bath.  It was a very sweet video, and though it initially made me smile, I soon found myself tearing up as I watched the cuddly, sleepy, tiny newborn baby be cradled and bathed… because I missed out on so much of the joyful newborn baby experience.  I don’t often feel sorry for myself, but today I did – as I watched that video of that newborn baby, I felt sorry for the fact that I didn’t get that experience.  I didn’t give Adelaide a bath until she was almost three months old, because she spent the first eleven weeks of her life at Childrens Memorial Hospital.

Adelaide was born with a condition called hypoplastic left heart syndrome – HLHS.  We were unaware of her diagnosis before she was born, and we spent the first few days of her life in what I can now call (in hindsight) blissful naivety.  Looking back, I am so glad we had those days of cuddles and snuggles and happy family time, because they helped keep me going in the months that were about to come.  Adelaide passed two APGAR tests with flying colors, was pink and happy, and breastfed like a champ – we had no idea anything was wrong.  It wasn’t until she was three days old that her condition was discovered.

The last night before we were supposed to take Adelaide home, I had a sharp pain in my right leg.  Because I had a C-section, the doctor ordered an ultrasound of my leg to make sure everything was okay.  We sent Adelaide to the nursery so my husband could come to the ultrasound with me; this was the first time she had been away from us aside from the daily weight checks, as she had roomed in with us for our whole hospital stay.

Thankfully, my leg was fine.  However, while Adelaide was in the nursery, she turned grayish and started breathing rapidly.  One of the nurses noticed this and called the on-call doctor, who happened to be a cardiologist with the local childrens’ hospital.  He quickly ordered an echocardiogram, which showed that Adelaide had HLHS.  Basically, the left side of her heart was very underdeveloped and non-functional.  (It’s at this point that I have to say, it was nothing short of divine intervention that things unfolded as they did – I was given that pain in my leg for a reason… to make sure that Adelaide was under the constant watchful eyes of the nurses in the nursery, instead of in our dark hospital room as we slept, so that the nurse would notice her change in color – call the doctor – order the test – discover her life-threatening condition.  I don’t want to even think about what would have happened had she not been exactly where she was at the exact moment she needed to be.)

Another cardiologist came into my room around 3:30 that morning to explain Adelaide’s condition to us.  She let us know that within her first years of life, Adelaide would require three corrective surgeries to reroute the “plumbing” of her heart.  One would need to take place within the next several days, one around 4-5 months of age, and one around 2-3 years old.  Adelaide was going to be transported directly to Childrens Memorial Hospital in Chicago.  Before she was taken there, my husband and I were able to visit her in the NICU.  We were relieved to see that aside from some IV lines and bandages, she still looked like our daughter, and she didn’t seem to be in any pain.
Later that day, I was discharged from the hospital and my husband and I went to see Adelaide at Childrens – the place that would become our home away from home over the next three months.  

The first few weeks of Adelaide’s hospital stay were a busy blur of nurses, doctors, medicines, infections, and medical terminology I never thought I’d have to learn.  She was originally scheduled to have the first corrective surgery (The Norwood Procedure) at 8 days old, but it was postponed due to an infection in her bloodstream.  Thankfully, the infection cleared quickly and she had her Norwood at 13 days old.  The day of Adelaide’s surgery, our friends and family waited with us in the waiting room – anxiously awaiting updates from the APN as the surgery progressed.  After almost eight hours, Adelaide’s surgery was complete.  Things had gone as well as possible, and her heart had started beating on its own after she came off of the bypass machine – which was a very good sign.

After a week or so of incredibly fast recovery, unfortunately, Adelaide took a few steps backward.  Though she had been successfully extubated a few days after The Norwood, she started having trouble breathing on her own and ultimately had to have the breathing tube put back in to help her breathe and take some of her body’s workload away from her heart.  (If her body was struggling too much to breathe, it put more strain on her heart, which is bad in general – but especially bad for a baby who only has half of a heart and is recovering from open-heart surgery.)  Her heart function had also decreased and was not improving despite the breathing tube’s assistance to help her body recover.

It was at this point that we first met with the heart failure/heart transplant team.  I can’t remember exactly how or when the conversation we had with them changed from, “We just want to introduce ourselves to you in case we need to start talking about transplant as an option,” to, “We need to seriously talk about the process of listing your daughter for a heart transplant,” but… that happened, and one month exactly after Adelaide had her first open-heart surgery, we listed her for a heart transplant.

Adelaide was listed as status 1A – the most critical status, because she was on a ventilator and required a constant drip of IV drugs to help her heart function.  After we listed her, my husband and I struggled to deal with the fact that the thing we were most anxiously waiting for – a new heart for our daughter – meant that another family was about to experience the greatest tragedy and our worst nightmare.  It didn’t seem fair, but it was our reality.

Five short days after listing Adelaide for transplant, we got “the call.”  They had found a suitable donor heart for Adelaide and though they still had to go physically see the heart to confirm, all preliminary tests were very promising.  “The call” came the day after we had had a prayer service at our church, where friends and family came together to pray for a miracle.  “The call” also came on the eve of my husband’s and my fourth wedding anniversary.  We were overwhelmed, scared, excited, nervous, and overall – hopeful.  The signs were all favorable – our miracle was happening.

Adelaide went into her transplant surgery just after midnight on our wedding anniversary.  She was seven weeks old.  Around 7:00 that morning, the surgeon came out to tell us her surgery had gone very well, and that her new heart had started beating on its own – again, a wonderful sign.  

Adelaide had a very smooth recovery from her transplant.  When she was extubated three days after her surgery, she gave us her first smile – and it was in that moment that we knew she was going to be okay.

That’s not to say that life post-transplant isn’t without risks, or possible complications.  Adelaide will be on anti-rejection medicines for the rest of her life, and those medicines carry a looooooong laundry list of possible negative side effects and complications.  If you really want to know what those are, you can Google them – I’m not going to go into it here.  Because of her anti-rejection medicines, Adelaide also has basically no immune system, so she’s much more susceptible to infection and disease than your average person.  So yes, there are a lot of things that could pop up down the road and that may challenge us.

But, as her transplant team so often says (and we love them for having this mantra) – they didn’t save her life to not let her live.  They encourage us to help her have as normal a life as possible.  While we use caution and try not to take her out in public more than is necessary during the height of flu season, we still go out to dinner as a family now and then, and she loves to go to Target with us.  When she’s a bit older, we will take her to kid-friendly activities at the library, or at the park-district – and from the way she moves, I think she’ll love taking dance lessons when she’s ready!  And, we fully intend to send her to preschool, kindergarten, middle school, high school… college.  We will always be cautious, but we will never hold her back.  Her life was saved so she can LIVE. 

Adelaide is now almost 17 months old and is thriving.  Though she did have some slight physical and feeding delays due to spending the first three months of her life in the hospital, she quickly caught up and hit most of her baby milestones right on time.  She is currently a spunky, energetic, sweet, loving, talkative, smiley, adorable little girl who loves to dance, adores penguins, and gives the cutest little kisses.

I am so thankful to have this opportunity to share Adelaide’s story with those of you reading this, and I hope you have enjoyed learning about our little heart warrior.  I also hope that her story and journey can help spread even a little awareness about congenital heart defects and the impact they have on children’s and families’ lives every day.  Thank you for taking the time to read Adelaide’s story!

 http://nearadelaide.blogspot.com.

Meet Martin!!!

We found out about Martin's heart condition at our 20 week ultrasound. I remember that the technician seemed to be struggling to get the pictures she needed of Martin's heart. She called the doctor in. It was the first time we had ever had a doctor perform an ultrasound on one of our babies. He looked for a long time, and told us he had to go see another patient and that he would come back in a few minutes. While we were waiting, my husband told me to prepare for the doctor to say that something was wrong with our baby. I didn't want to believe it or even think about it. When the doctor came back and resumed the ultrasound on our baby's heart, Steven asked him if he thought there was a problem.  "Yes," the doctor said, "The left side of the baby's heart is missing. I believe your baby has a condition called hypoplastic left heart syndrome, and he will need open heart surgeries to survive." I was dumbfounded. The doctor eventually had to leave the room again, and after he walked out, I lost it. We had our other three children with us in the room as well, so I tried hard to pull it together for their sakes. We prayed together right there as a family for our little baby. Our routine 20 week ultrasound ended up lasting for over three hours.

The next week we met with our dear cardiologist, Dr. Malpass.  He performed a fetal echo, and then sat us down to talk about what we were facing. He was very blunt, but kind. He told us about the survival rates, the complications that often arise, the procedures that would have to be done. He answered our questions, sometimes even with tears of his own. He told us he would pray for our Martin.

The days leading up to Martin's birth were full of anxiety and tears, many tears. Every night we would play music for Martin. We prayed that the Lord would make him big and strong. One day we were at a birthday party and there was a woman there with her brand new baby. As I looked at that tiny newborn, I couldn't imagine a baby that small having his chest cut open, sternum cracked, and heart stopped for surgery. I cried all the way home. Steven constantly reminded me to lean on the Lord. The hymns that we always sang in church were suddenly so meaningful, and helped bring peace and comfort. Our family read through Psalm 34 many times during those days. The Psalm begins with "I will bless the Lord at all times; His praise shall continually be in my mouth." That is what we sought to do. How wonderful it is to have the Lord to lean on, especially in times like these!

I gave birth to Martin on February 3, 2012 in the OR of MUSC hospital in Charleston. He weighed in at exactly 7 pounds, my biggest baby! He was immediately taken away for an evaluation. I got to see him briefly, a couple hours later, as they were taking him to the ICU. He looked so perfect, so beautiful and peaceful. Three days passed by and on the fourth day, Martin had his first, big open-heart surgery. Handing my baby over to the anesthesiologist and watching him get taken away to surgery was the hardest thing I have ever done. Dr. Kavarana performed the surgery (Norwood) and it lasted for over 9 hours. Later that evening, we finally got to see him, and nothing could have prepared me for the moment when I first saw our tiny baby with his chest open and tubes and wires everywhere. It was hard, but I had to keep reminding myself that all this was saving his life.  We are very grateful for Martin's surgeon, Dr. Kavarana. He was amazing. Even though he had been in surgery all day working on Martin's heart, he stayed with him the whole night as well, and made sure that Martin remained stable.

Recovery was long and difficult. It seemed like that breathing tube would never come out, and Martin HATED it. He would cry, but no sound would come out. It was very sad. I wanted to hold him so badly, but they wouldn't let us because of all that he was hooked up to. After a number of days, our little fighter decided that he had had enough. Martin extubated himself during the night while the nurse wasn't looking, but his numbers dropped and the tube had to be put back in. We tried our best to comfort him, even though we couldn't hold him. Those days were hard. Babies around us passed away, there was much heartache, and much prayer. Finally, the glorious day came when the breathing tube came out! Martin was so much happier. After waiting for weeks, we finally got to hold him again. Eventually he started drinking from a bottle, but they wouldn't discharge us until Martin had a full feed every 3 hours. We won't ever forget those stressful feedings, trying to get him to eat so that we could get him home. We were finally discharged at 6 weeks post op! Being home again and united with our children was so wonderful, and Martin loved it.

Martin had a heart catheterization done at 5 months to see if he was ready for the next surgery. He had a very rough time with it. Everything ended up looking good heart-wise, but he really screamed and struggled in the recovery room, and could not be consoled. He came back to us with needle marks all over him from the IV attempts. He had definitely been traumatized. After that procedure he had night terrors for months, and he would wake up screaming every 1-2 hours at night.

He had his second stage surgery (the Glenn) at 6 months. Things went much more quickly and smoothly this time around, and we were home in a week! He had a difficult time getting off the pain meds, but he was back to his normal self a few days after getting home. His final heart surgery will probably happen around 2-3 years old.

You'd never know that Martin has half a heart. He has so much life and energy in him. He loves his siblings so much, and they are always loving on him and finding new ways to make him laugh. We celebrated his first birthday a couple weeks ago, what a joyous time that was! I would never have chosen this path for myself, but God knew what we needed. We are so thankful for all that He has taught us on this journey, and especially for the gift of Martin and his little half heart.

You can read more about Martin's story at www.babymartinlee.com

  

MEET XAVIER!!!

 I am a single mother my son Xavier Smith was born with a broken heart. I was not aware anything was wrong with him according to my obgyn everything was going as it was suppose to. He was born August 11th 2012 got released August 13th 2012 from the hospital and I ended up taking him back to the ER on August 14th 2012 because he was not eating anything anymore by mouth at first I was told something was wrong with his lungs they kept checking his oxygen and couldn't believe the numbers they were getting they kept getting different machines hoping one would show better number but it didn't happened they finally realized something was really wrong. Being we live in a really small town they got a hold of another hospital when they mentioned everything going on they knew right away what was wrong with my son they immediately started bagging him by hand for over 2 hours until they got the transport team and helicopter to us. He was diagnosed with HLHS. Had his Norwood procedure on August 21st 2012, and his Glen on November 27th 2012. He is doing great now and I am so blessed and thankful to have him here.

i also just started him a page

http://m.facebook.com/pages/Teamxaviersmith/423124511089038?id=423124511089038&refsrc=http%3A%2F%2Fwww.facebook.com%2Fpages%2FTeamxaviersmith%2F423124511089038&_rdr

Day 27: Meet Aly and Violet!!

Day 27 you will meet Ms. Aly and Ms. Violet.  Both little girls beat the odds and are living beautiful lives with mended hearts.  SUPER CUTIE ALERT!!! 

MEET ALY!!!

My husband and I had been married for 3 years when we decided it was time to add one more to the family. On November 14, 2008 I found out we were having a baby! We were ecstatic! For some reason, I just knew I was pregnant. I bought pregnancy tests and took one every day for 5 days before that. I just had this feeling~ I knew we were pregnant. Finally, I got a positive one! (2 actually - one for back up :) ) For the next 6 months we blissfully planned for the arrival of our baby. We painted a nursery, we registered for gifts, picked out a crib... the fun stuff. :) At our 20 week ultrasound, the tech was really struggling to get a good view of the heart. She kept saying, "I can see it, I just can't get a good picture of it." She didn't seem concerned, she just kept laughing that we had a stubborn little baby who wouldn't move the way she needed her to. We laughed right along with her and didn't think twice about it. Never once did it cross my mind something might not be right.

Little did we know, that when we got this picture taken our little girl was hiding more than just her “little girlness”.  We thank god on a daily basis that our Ultrasound Tech paid such close attention and caught that something just wasn’t right with the heart.

On May 9, 2009 I went for my level 2 ultrasound to get a closer look. I wasn't nervous at all. Again, it never crossed my mind something might not be right. Our world came crashing down that day.

Our daughter was diagnosed with Hypoplastic Left Heart Syndrome at 30 weeks gestation. We were devastated but we didn't give up. We were thrilled to find out that the University of Michigan was ranked 3rd in the nation for treating this CHD and was located a mere 2 hours from home. Our daughter, Aly was born at U of M on June 30, 2009.

Isn't she beautiful?!

She scored 8 out of 9 on her APGAR tests and did very well on the days leading up to her first surgery. Aly was born via C-Section so I was unable to visit her until she was over 24 hours old. It was 7 days before I got to hold her. And even when I did I wasn’t really “holding” her. I was holding blankets and tubes and wires with a little tiny baby in the middle of it all.

This picture was taken just a few short hours before her first open heart surgery.

She had her Norwood procedure on July 6, 2009. She survived surgery and did really well until they got back up to the Pediatric Cardiac ICU. She had a rough patch that evening of trying to get stabilized but pulled through.

Underneath that heart shaped gauze is a yellow membrane that was covering her chest. We could look through the membrane and actually see her heart. Her chest was left open for 4 days following surgery.

8 days later, she went back into the OR for a shunt revision. The shunt that had been placed in her tiny heart was narrowing at one end and had to be shortened. 14 days later, we brought our baby girl for the very first time.

We struggled with feeding and gaining weight, as many heart babies do, but we made it to her Hemi-Fontan procedure on December 4, 2009. Aly did unbelievably well and we were home 5 days later.

March of 2010 we returned to the University of Michigan for the Fontan, the final stage of the anticipated surgeries.  Aly’s surgery went very smoothly and after staying a few extra days to deal with some post operative drainage, we were on our way home 8 days after surgery.  

This picture was Aly, 10 days post Fontan.

The last two years have been amazing since Aly’s Fontan.  She has lived her life like any heart healthy toddler would, other than taking medication twice a day.  She has really thrived- she has been to Disney World, to the beach, camping, loves to swim, started preschool, started ballet and tap class…. I could go on and on.  Aly is my hero- she has endured more in three and a half years than most will in a lifetime. She is the strongest little girl I know and motivates and inspires me on a daily basis.  She lives her life despite her heart defect, not consumed by, and that is more than we could have every hoped and dreamed for her.  Feel free to follow along with Aly and her adventures at www.alyjeansspecialheart.com

“Once you choose hope, anything is possible.” Christopher Reeves

MEET VIOLET!!!

I found out I was pregnant in November 2011 at just 6 weeks. We were nervous because I had just had a miscarriage at the end of September and had two before that in 9 months. I went to the doctor to be put on progesterone and a baby aspirin. I had found out I have a clotting disorder (Factor II) so I was being seen by a perinaologist. 
Everything was going well and at the nuchal scan, her fold measured 2.7mm, which was on the higher end of normal. Coupled with my blood work, I had a 1 in 2mil chance for the main three trisomies. Fast forward 7 weeks to my anatomy scan.
We had found out we were expecting a little girl at 16 weeks and named her Violet Reese. We were excited to see her again and we thought it might be the last time seeing her before her birth. Boy were we wrong.
The doctor came in and immediately started looking at her heart slides. I felt a bit weird about it, but brushed it off. About five minutes later, my world came crashing down.
They took us to a conference room and mentioned things like "heterotaxy" "DiGeorge Syndrome" "Trisomy 18" and "termination". Needless to say we were heart broken. Our little girl was going to be born with a broken heart. It was never on our radar so we were blind sided.
The rest of my pregnancy was filled with lots of appointments, amnio, fear, but most importantly, joy. We were not going to give up on her.
She continued to grow perfectly and prove the doctors wrong. I was scheduled for a c section due to her breech presentation on July 10, 2012. From the get go, she was telling the doctors to kiss her butt. She was born at a healthy 6lbs 15oz and scored two 9s on her APGAR tests. She was wheeled away and in that flash, I saw a fighter who was going to beat the odds.
Her formal diagnosis is: ASD, VSD, hypoplastic right pulmonary artery, hypoplastic right lung, PAPVR on the right side and those veins are hypoplastic, Dextrocardia, and an interrupted IVC. She has Heterotaxy and Scimitar Syndrome. 

She spent 6 days in the NICU and never needed any intervention. At two weeks old, she was admitted for congestive heart failure and was placed on two medications. She stayed another six days and we went home for about four weeks.
She was admitted again on August 26th and got discharged on October 17th. During that time, she had two surgeries. She had a PA band placed on September 4th. During that surgery, her left vocal cord was damaged. She had a swallow study done and they found a trace amount of aspiration. We moved with a G tube and Nissen Fundo on September 26th. 
Since her discharge, she has been healthy and avoided any re-admissions  She has gained nearly 7 pounds in four months. Because of this growth, her repair is tentatively scheduled for the spring (it was supposed to be in July). Violet is currently in PT due to a delay of almost two months physically. She will be starting ST soon because she has stopped taking a bottle.
We have been extremely lucky with her health during this winter and count our blessings every day. She just had her 7 month 'birthday' on the 10th, one that we did not know if she would make it to. Violet is truly the greatest thing that has ever happened to us. Of course, I do have my down moments, but being around a child who has been through so much already and still laughs every day, how could I not smile? =)

Day 26: Meet Kaitlyn and Klara Jane

Day 26 brings us the stories of Kaitlyn and Klara Jane...two beautiful little girls, born with broken hearts but unbreakable spirits!!!

MEET KAITLYN!!!

Kaitlyn was born May 24th,2008 weighing 5lbs 5oz. She appeared to be a healthy baby, apart from a little jaundice that she developed due to her being 4 weeks premature. Doctors assured us that everything will be fine and that the jaundice will go away. Sure enough a couple weeks later, it did and we had our minds set on starting our life as a family. 

When Kaitlyn was 6 weeks old we had her go over to Grandma's house so me and her dad can have a night out by going to a dinner and a movie. After we returned about 10pm, she told us that Kaitlyn sounded a bit raspy in the chest and that she thinks that she might have a chest infection (his mom's a nurse). So we brought her to the ER immediately just in case, but wasn't too concerned since a cold is something that can be cured with antibiotics. Upon arriving, we noticed that Kaitlyn was starting to turn a little blue. They hooked her up to the O2 machine and her stats were in the high 80's. That set off alarms. At first they thought that she just had pneumonia so they admitted her to the PICU to start her on antibiotics and to give her oxygen. The next morning, the doctors were still a little concerned about the way she was breathing and her O2 since she was on oxygen. They ordered her a EKG and that's when our lives turned completely upside down and we knew at that time that no antibiotic was going to cure whatever she had. 

The pediatric cardiologist at the hospital sat us down and told us that our daughter was born with a severe heart defect called Double inlet left ventricle also known as Single ventricle and that she needed to be airlifted to Minneapolis, Minnesota to the University of Minnesota Fairview to undergo open heart surgery. I felt at that time that my heart got ripped out of my chest and my brain was going a million miles an hour of thinking what is going to happen to our little girl. I called my family and my best friend and they all met us at the hospital. I think I finally lost it when they had a Chaplin come in and say a prayer for our daughter. I think at that time, that's when reality started to hit that we might lose our baby. 

The hospitals airplane flew us to Minneapolis within a couple of hours. Everything was happening so quickly, but we knew that we needed to stay strong for Kaitlyn. We got in touch with the hospital's social worker and she set us up in a hotel room for a couple days until a room at the Ronald McDonald House was available. 

Three days after arriving in Minneapolis, I was holding Kaitlyn and all of a sudden she started having seizures. I put her back on her bed and they got a resident doctor who then contacted a neurologist. They ran some tests and confirmed that Kaitlyn suffered 3 massive strokes due to all of the blood going around her body and some of it traveled to her brain, caused embolisms, and they burst resulting in her having strokes. We were devastated at not knowing what the outcome of our baby was going to be. They delayed surgery for a couple more days to be sure that she was strong enough to go through it.

On July 23rd, 2008 and almost 2 months old, Kaitlyn underwent her first open heart surgery. It was the longest day in my life. While we were in the waiting room, the song from Alabama "Angels among us" came on the radio. I immediately got goosebumps. It was almost like it was a sign from God that angels were there looking over her. After 8 hours of surgery, Kaitlyn was done. We were told to go up to the PICU to wait for her arrival. We went up there, and about 45 minutes went by and I sensed that something was wrong. Soon after thinking that, I get a call to go back down to the waiting room because the nurse wanted to talk to us. I feared the worst. After getting down there, her primary nurse was almost in tears. She told us that when they went to move Kaitlyn from the table to the bed, her heart developed an air pocket and her heart stopped. Her surgeon pumped her heart with his two fingers for six minutes and brought her back. She was alive but just barely. 

We went back up to the PICU and when she arrived, I could barely recognize her because she was hooked up to so many machines, including ECMO, and she was so puffy that she looked like a year old baby instead of a 2 month old baby. She was so critical at that time that we weren't even aloud to talk to her nor touch her because stats would drop. I can't even tell you how badly I just wanted to scoop her up and make her feel better, but knowing that I couldn't, broke my heart.

The next 48 hours after Kaitlyn having surgery was the most critical. She about died twice after that and we were trying to prepare for the worse. All of a sudden, Kaitlyn started to get better. She got off the ECMO and then a couple weeks later she got off the ventilator. Things started to go in the right direction for once and before we didn't think we were ever going to go forward, nor did we think we were going to walk out of that hospital with our baby.

After 77 days in the hospital, Kaitlyn was discharged on September 26th, 2008. That was the best day of my life. 

Kaitlyn had her second open heart surgery June 26th, 2009 and she did great. We were in the hospital for a total of 10 days. We, along with doctors and nurses, was amazed that she did so great, despite the first time she was in the hospital. We got discharged the day before the 4th of July, and that next day we went down to the bridge in Minneapolis as a family and watched the fireworks. That was the best Independence Day of my life.

Today, Kaitlyn is 4 years old. She is in early childhood and next year she will be starting Kindergarten. She's growing up really fast and is staying as healthy as she can be. Since she did have the strokes when she was a infant, she did develop Cerebral Palsy and Epilepsy. She can't walk, talk, and she has some vision loss. Despite having all these setbacks, they do not slow her down. She is always happy and her laughter fills our house everyday. We couldn't be more proud of our heart princess/warrior. 

MEET KLARA JANE!!!

We discovered that Klara had a congenital heart defect at our 18 week ultrasound. During the scan of her heart it was very obvious that something wasn't right. Our doctor referred us to a pediatric cardiologist in Orlando. The following week we had our first ultrasound echocardiogram with the cardiologist; it was then that he confirmed Klara had Tetralogy of Fallot.

Tetralogy of Fallot has four characteristics. Ventricular septal defect (VSD) - There is a hole between the two bottom chambers (the ventricles) of the heart that eject blood to the body and lungs. Overriding aorta - The aorta, the large artery that takes blood to the body, is on top of both ventricles, instead of just the left ventricle as in a normal heart. Pulmonary stenosis - There is a narrowing of the pulmonary valve, the area below the valve, or the pulmonary arteries, which carry blood from the heart to the lungs. Hypertrophy - The right ventricle becomes thicker and more muscular than normal as a result of working harder to pump blood through the narrow pulmonary valve.

Her condition is on the more severe spectrum of this defect because in addition to the normal four characteristics, she had no pulmonary valve and her very small pulmonary arteries were not connected in the normal location of her heart; that is called pulmonary atresia without a pulmonary valve. As a result of no pulmonary valve and her pulmonary arteries not being attached, her body created multiple aorta pulmonary collateral arteries (MAPCAs); these collaterals created a pathway for her heart to pump blood to her lungs. Lastly, her VSD was extremely large.

Being that we knew Klara had a heart defect, we delivered her in a hospital on the other side of the state that was capable of handling a cardiac baby. On the day that we had her, there must have been twenty people in the surgical room. I had the surgical team that performed the cesarean and Klara had her own special cardiac team of specialist waiting for her. Immediately after she was born, her team did a quick assessment, allowed me to take a peek as they rolled her by in the isolate and then took her straight up to the Cardiovascular Intensive Care Unit (CVICU) where she would stay until discharged.

The CVICU team allowed Klara’s father to stay by her side the whole time. It wasn’t until early evening that I was allowed to go up to the CVICU to see my precious baby. I was not allowed to directly breastfeed because they had to measure everything that Klara took in so I had to use a breast pump all the time. As with almost all cardiac babies, it is recommended to give them a high calorie diet; therefore, I also had to add powder formula to my breast milk which is called fortifying. We were taught several ways to keep her awake during feeds; cardiac babies tend to tire out really quickly during feeds.

Though it was expected that she would need surgery immediately, she didn’t; Klara had great blood flow to her lungs thanks to her MAPCAs. However, Klara did have a battle with jaundice while she was in the hospital. She became really weak, refused to eat, and started dropping weight quickly. As a result, she had to be put under bilirubin lights, which is a phototherapy tool,  had to have a NG tube put in so that she could still get her nutrients. After a few bumpy days, Klara started doing well and we were allowed to take her home after two weeks.

Klara had to be closely monitored by her cardiologist and pediatrician once we were home. Feedings were such a battle because she would easily tire out and the high calorie diet would result in her throwing up her feeds. Regardless of the battles we faced with the feeds, she continued to gain weight enough and maintained decent oxygen stats to post pone her repair surgery until she was bigger.

 It wasn’t until she was 7 months old that she went in for her first open heart surgery. We took Klara back over to All Children’s Hospital for her first open heart surgery. We stayed the night before in the Ronald McDonald House. I remember we hardly slept that night. The awful thoughts of the worst kept flooding my mind. All I wanted to do was just hold her. I was so scared, yet anxious, about the surgery!

November 12, 2012: Surgery day! On the morning of her surgery calmness came over me. It was comforting that the hospital staff members were familiar faces that we had come to know on a first name basis from our visits from the last few months. We had asked for little contact with anyone on the morning of her surgery. Our pastor was there to lead us in prayer before she went back and our parents were with our older daughter in the surgical waiting area. The time had come for us to walk her to the surgical room door way. We tried to remain in positive spirits as much as we could up until this point. After handing her over and watching her be taken back to the surgical area, we lost it! My husband and I just held each other and cried.

Klara’s surgery was initially scheduled to last between 6-8 hours and consist of performing the following 1) Install the T-conduit in the location where the junction of her pulmonary arteries meet and enter into her heart. The T-conduit also contained a pulmonary valve. The T-conduit containing the valve is made here in the hospital by the surgeons. 2) Patch the Ventricular Septal Defect with a GOR-TEX (Teflon) material.

The actual surgery went a little different and much better than planned! The surgeons noticed that her pulmonary arteries from the left and right lung were indeed connected to a main pulmonary artery. The main pulmonary artery that is supposed to lead into the heart was just a few millimeters away. This meant that instead of installing the synthetic T-conduit, the surgeons were able to physically attach the artery to its appropriate position in the right ventricle of the heart. From drawings the surgeon showed us, it is under our impression that because of the way her arterial anatomy formed, there was some extra slack in her pulmonary artery for them to pull from.

The surgeons made a slice in the pulmonary artery and added a piece of monocusp to increase the diameter of the artery. Then the surgeons installed a pulmonary valve made from her pericardium, which is tough double layered membrane that covers the heart. The valve behaves like a one-way check valve in that it will only allow blood to flow in the direction of the lungs and not allow any blood back into the heart. This is different from the multiple flap-like valves we all have. The large hole in the ventricle septum was patched with GOR-TEX which also corrected the overriding aorta. There was a little bit shaved off the muscular wall of the right ventricular. Then her aortic valve was tightened up to the atrial septum so it would no longer be as large and not leak. The surgery was finalized by closing her up and wrapping her sternum with stainless steel wires.

Not including prep and post-surgery work, the surgery ended up taking 4 hours. We are very grateful that the bypass time was significantly reduced and that they ended up using her own tissue in instances they were originally going to use synthetic material.  Though the surgery was expected to be rather complex, it was truly by the precise planning of her surgeon and positively the blessing of our Lord that it was all completed in a matter of hours! We were always told that it could go either way once they actually see her heart and for Klara it went for the best!

When we first were able to see Klara she was already extubated but still rather out of it from her anesthesia. She had so many tubes coming out from all over but we were over joyed to see our little baby again! The first night of surgery was really rough because the pain medication that she was given was not working well and she actually was having an allergic reaction which was making her more irritated than anything. It took till the following afternoon to get all her pain medication worked out and Benadryl for her allergic reaction then she was resting peacefully. By the following day, day 2 of post operation, she was already starting to be her smiley self! It was amazing! The more they removed tubes and lines the more and more she started doing better. By that Friday, 4 days post-op, she was released from the hospital with flying colors!!

Again, Klara’s case was by far not the norm for many heart warriors. However, it just goes to show that every case is different. What we anticipated to be a complex lengthy risky surgery turned out to go smoother and more successful than we could have every imagined! Klara is now 10 ½ months old, almost walking, eating everything in sight, dancing machine, chunky monkey, flirty eyes, and bundle of joy!

She continues to be monitored by her cardiologist and pediatrician closely being that we’re just a few months post-op but they all couldn’t be happier with her progress. She still has 40% blockage in her pulmonary arteries and leakage in her VSD patch; however, we’re optimistic that her arteries will start growing in time and scar tissue will patch over the leak in her VSD. This won’t be her only open heart surgery; it’s unknown when or how many more open heart surgeries she will need but for today, we are enjoying every moment we have with her. We’ve seen so many fellow heart parents have to say goodbye to their warriors, so we take each and every day for the amazing blessing that it is!

This is the day which the Lord has made; Let us rejoice and be glad in it. ~Psalms 118:24

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!!!

MEET LILY!!!  

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!

MEET JOHN!!!

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.