Xtra Special Heart: Valentine's Day: MEET WESTON and JACOB

Day 14, Valentine's Day, brings us the stories of Prince Weston and Jacob the Great!! Two CHOP kids with amazing stories of triumph and down right FIGHT!!! I LOVE, LOVE, LOVE these boys and their families (and adore their mommy's very much) ...May your hearts grow ten times after reading their stories!!!

MEET WESTON!!

God, blessed us with our second son, Weston. He is now 6 years old. He
was born in the gorgeous mountains of East Tennessee. He loves those
mountains (the dirt, the rocks, the bugs), but he doesn't live there
anymore. He is currently inpatient at Children's Hospital of
Philadelphia (CHOP) Cardiac Care Unit. He's been there since June of
2011 waiting a heart and double lung transplant. Right now, He, his
youngest sister, and I have traveled to Philadelphia to stay for his
medical care, leaving his Daddy and five brothers and sisters behind
in Tennessee.

Shortly after his seemingly ordinary birth, Weston was diagnosed with
aortic and mitral stenosis and an abnormal left ventricle. At 9 days
of age, he was transferred from Tennessee to Duke University Medical
Center, where he underwent a valvuloplasty. Two months after that, he
had a repeat valvuloplasty. Afterward, we went home to Tennessee for
a few glorious months, but had to return to Duke when Weston was 7
months old. He needed further surgical intervention. A few weeks
after that, he returned to Duke again for a conduit replacement and a
St. Jude Valve placement.

After all of that, we had three great, easy years back in our
beautiful East Tennessee mountain home. Weston was able to remain at
home with us in relatively good health until he was three years old.
Then, he began to have episodes where he would turn blue and show
seizure like activity. He would stiffen and many times, we thought he
wasn't going to come back to us. This was a terrifying time for us. We
brought him back to Duke, again. This time, however, we learned his
condition was more involved that we previously knew. Weston was
diagnosed with pulmonary hypertension. The physicians and experts
determined that Weston would benefit from an in depth cardiac repair.
We arrived at Duke to have his 3rd open heart surgery but after
meeting Weston, Dr. LOdge said he had prayed about it and could not
perform the surgery. God had intervened. He explained that Weston
needed a much more extensive repair than he felt comfortable doing so
the search was on for a new surgeon. While Weston was turned away at
many top hospitals,
Dr. Spray at CHOP indicated that he was willing to attempt the complex
repair, so Weston was transferred to CHOP. The odds were not in
Weston's favor but he underwent a
complex open heart surgery in August of 2010 and had a fast recovery
and celebrated his 4th birthday at CHOP.

We all hoped so hard that the open heart surgery would resolve the
pulmonary hypertension. That this would be the end to all of his
heart's struggles and he could return to the life of a rambunctious
little boy who loves to run barefoot in the grass, play in the mud,
and help his dad around the house. Unfortunately, it did not. Weston
continued to have episodes of pulmonary hypertensive crisis. After
ten more months of continued terrifying episodes and what felt like
tons of unanswered questions, we YouTubed Weston's episodes and sent
them to every doctor we knew along with Dr. Hanna at CHOP. He told us
to come immediately because he could tell that Weston was in crisis.
Upon arrival in June 2010, Weston developed endocarditis. After
having a few episodes on the floor, he was transferred to the cardiac
intensive care unitl and immediately listed for a heart and double
lung transplant.

We entered the world of indefinite waiting. We had no idea when we
left our home in Tennessee that day that it would be the last night
that Weston would be spending there for a very long time. We had just
found out the week before we came that we were expecting number 7.
She is our Philly baby because she was born and has spent the first
year of her life here. While the outcomes for heart and lung
transplants in children are rare
and outcomes are fair at best, the transplant is the only way for
Weston to survive. He was able to leave the hospital in November of
2011 (yes almost 18 months later and 2 more birthdays later!).
Thankfully, he is thriving outside of the hospital walls and we are
praying for a Miracle or his Gift of Life. God has big plans for our
little boy-bigger than Adam and I could have ever realized 6 years ago
when he was born.

God has blessed Adam and me with 7 amazing children, 7 years old and
younger. However, our family has been broken apart by Weston's
illness. I am the primary caregiver for Weston. That means that I
live in Philadelphia with our now 1 year old daughter and Weston. We
try to rotate the other kids up so that we can spend time together and
bring the kids all up so they can have time together. Adam had to
return to work in Tennessee in order to keep our medical insurance and
try to keep up our everyday bills. Our family members care for the
other children while Adam is at work and he spends time with the kids
on the weekends. Our wonderful, large family has gone from all living
in one house, to being spread apart in 3 different households in two
separate states.

Despite everything that Weston has endured in his six short years, he
has the best attitude. You can't spend time around him and not come
away utterly blessed. His doctors and nurses love him. New staff are
always surprised at his tenacity and spunk! His days in the hospital
are quite busy. He goes to physical therapy, occupational therapy,
speech therapy, school, music, and art! He enjoys all of the things
that young boys like -- Superman, Spiderman, Stars Wars, and Batman.
He loves playing tricks on his nurses and doctors.

We are overwhelmed with the love and support for Weston and our
family. We appreciate it and know that God is blessing us along the
way with such wonderful friends and family. Please follow his page on
facebook "Praying for Weston" or at www.cotaforwestonk.com or see his
video at http://vimeo.com/54067010

* update: Weston is out of the CCU and living at the Gift of Life house, still waiting for his transplants. I hear from a little birdie that the family may soon be moving to an apartment in the city...xoxo

MEET JACOB!!!

November 18, 2009 was a day that I will never forget. My husband and I went for our 20 week ultrasound expecting to hear if we were having another little boy or a baby girl. The joy of learning that we were having another baby boy was short lived. Right after the ultrasound tech got extremely quiet and we knew something was not right. She told us that there was something wrong with the heart. We were devastated and so afraid for our son. The next day we went for a heart echo and his diagnosis was confirmed, our son was going to be born with Hypoplastic Left Heart Syndrome (HLHS). He also had a restricted atrial septum. This is the only outlet blood had to move through his heart and children with that added defect have an even lower chance at survival. We wanted him to have the best chance possible so we decided to travel 9 hours to have him at The Children’s Hospital of Philadelphia.

Jacob was born on March 23, 2010. Within hours of his birth that Septum became intact and they were losing him. He was rushed in for an immediate heart catheterization to open it up. His xray showed his lungs were flooded and the doctors were afraid he would not make it. Jacob did make it, 9 days and 2 bacterial infections later on April 1st 2010 he was stable enough for his first open heart surgery (Stage1: Norwood). This was also the day we were able to hold our baby for the first time. Kissing him good-bye and watching them wheel him to the OR was the hardest thing I have ever done. I told him to be strong and that I loved him. Jacob did very well during his surgery and was off the ventilator 5 days later.

Jacob continued to improve however he had very labored breathing. He couldn’t drink much by bottle because it was too risky and to tiring for him. He had a g-tube and Nissen surgery done at a month old. After 8 weeks in the hospital Jacob was able to come home!

At 4 months old we went back to CHOP for Jacob’s second open heart surgery (Stage 2: bi-directional Glenn). Jacob recovered amazingly well and we were home just 6 days later.

You would never have guessed he had just had open heart surgery. Jacob came home on Oxygen because his oxygen would drop whenever we tried to wean him at the hospital. He was on it for a month and then once fully recovered was able to keep his numbers where they should be.

Jacob started drinking by mouth and within a few months he no longer needed to eat by his g-tube. This kid has a HUGE appetite; he made up for lost time lol. When he turned a year old we had some more testing done because of his “unique” breathing. We learned that both of his vocal cords were paralyzed. Scar tissue had built up around the vocal cords tethering them together. His airway was the size of a pinhole. It is amazing he could even breathe without assistance. Only 5% of kids with an airway like this can breathe without a Tracheotomy tube and he not only has half of an airway but also has half of a heart!

Jacob had a complete airway reconstruction on April 25, 2012 to get rid of the scar tissue and help him breathe normally. It was a rough recovery with many bumps but he was able to come home 3 weeks later. He no longer has a loud strider and has recovered beautifully from the surgery.

Jake will have his final staged open heart surgery (Fontan) this summer. We are nervous but also ready to get this surgery behind us. We never know what tomorrow will bring and cherish every moment we get with our precious beautiful boy. He has brought more life and joy to our family then I could have ever imagined.