Clifton Street

Atley's story - Part four. Atley today and giving back.

For those of you who know Atley today, he is a happy and active 9 year old boy.  His feeding tube was removed two years ago and he has been holding his own with food.  Around the same time, his strength and stamina surged allowing him to give up his wheelchair.  He takes ballet lessons twice a week and dances in as many ballets as he can.  He will never play contact sports or be a fast runner, but he makes up for his low muscle tone with incredible muscle strength.

Atley, 9 years old.

 

Unlike some congenital heart defects, Atley's defects can not be cured or repaired.  His surgeries are merely palliative, meaning they extend the function of his heart, but in no way "fix" his condition.  However, if Atley had been born even 10 years earlier, he would have died in the hospital without chance of any surgeries to extend his life.  There are very few, if any, adult survivors of his condition.    So, what does this mean?  More research is constantly needed to keep Atley, and others with congenital heart defects, alive to reach adulthood.

Because Atley will need heart surgeries, experimental treatment, and treatments that haven't even been invented yet, we have spent a great deal of our time raising money for congenital heart defects reserach and bringing heart defects awareness to the general public.  The other, larger, goal of our charity work was to bring comfort and understand to new parents of children born with heart defects.  Heart defects are not part of newborn screening and are often missed on ultrasounds, yet they are the most common birth defect.  When Atley was born, there was no support group for heart parents, no network of others to connect with, and very little information about Atley's defects. 

My good friend, Valerie, and I decided to create a foundation to help new parents of heart children by offering support groups, fun family activities, care packages for hospitalized children and their families, and fundrasing for heart defects research.  Valerie's son, Noah, lost his battle with congenital heart defects just before his second birthday.  We underestimated the impact we would have on the Kansas City area through our efforts, and throughout the world, with our congenital heart defects awareness products.  Please visit our foundation site to learn more about our Kansas and Missouri efforts to help heart families.

Valerie Janke Rexin and Julie Thomas Bowles co-founders of the Congenital Heart Defects Families Assn., a 501(c)(3) not-for-profit, at the AHA Heart Walk 2004

 

Since 2002, our Kansas City Heart Walk team has raised well over $100,000 for the American Heart Association's continued research of congenital heart defects and their cure.  Last year, we raised close to $50,000!

Jonah Rexin Iost his brother Noah to congenital heart defects) and Atley, Heart Walk 2008

The AHA Heart Walk is a family affair!  Julie's entire family was in from England and Colorado.

 

In 2005, Atley had the honor of being the AHA Heart Child.  Atley, Parker and I spoke at many events to raise congenital heart defects awareness and research money for the American Heart Association.  The job was demanding, but so exciting and rewarding.  Atley designed the AHA holiday cards for 2004, was featured in a newspaper article, was on Fox News and spoke on the radio.  Of course, mom did most of the talking, but Atley stole the show.

Atley and Mom speak on Fox News.

Atley speaks on stage at the Heart Walk with Congressman Dennis Moore (D) - Kansas

 

After his 2005 success at AHA Heart Walk child, Atley continued to raise awareness and funding for congenital heart defects through many personal appearances at AHA events.  He was one of the few boys I know who always owns a tux!

Atley with his heart sweetie (hypoplastic left heart syndrome) at the Heart Ball, 2007.

 

Thank you for reading Atley's story. 

 

Atley's heart story - Part three. Catching up.

Atley's first birthday was quite a milestone.  We threw a huge birthday party for Parker and Atley and Parker ate both the birthday cakes.  Atley still wasn't eating at all, had severe stomach discomfort, and wasn't even sitting up unsupported.

Life had become an endless therapy session for Atley.  We had in home physical therapy, speech therapy and occupational therapy several times a week.  Atley also attended the feeding clinic at Children's Mercy in Kansas City.  As always, progress was very slow, but he was making progress, especially in PT.  By 18 months old Atley was finally crawling, and he walked not long after his second birthday.

And he's off!  Atley crawling around 18 months old.  He is hooked up to his portable feeding pump.

As Atley started walking, his low muscle tone made it difficult to control his feet.  Leg braces were in order, and Atley stayed in braces for about two years.  Also, Atley could not walk any distance at all requiring a wheelchair most everywhere he went.

Atley and his physical therapist try some skating to help him walk.

His left foot is almost dragging; this was helped by his leg braces.

Atley always loved a new set of wheels!  First grade, his last wheelchair. 

At age 4, Atley needed a third heart surgery to close a hold between the top two chambers of his heart.  We traveled to Chicago to have the surgery performed at Children's Memorial Hospital by one of the most qualified doctors for this surgery.  The surgery was a huge success, allowing Atley to have more oxygen to his bloodstream, and less respiratory infections.  In fact, he has not had pneumonia since this surgery.

Atley's heart during is third heart surgery showing the large metal mesh device used to close the large hole between his atria.  The wire loop above is part of where his sternum is wired together.

When Atley was five, we decided his health was stable enough to allow him to finally enjoy a very special event, his "wish" from the Make-A-Wish Foundation.  Atley chose to visit Walt Disney World and Give Kids the World resort in Orlando.  It was truly a magical trip for Atley and his sisters.  Make-A-Wish and Give Kids the World strives to give an ill child's family a wonderful time, as well as, the wish child.  Atley was showered with VIP treatment at Disney and Universal Studios, but nothing quite compared to private Disney character visits and meeting Santa at Give Kids the World.  After all his surgeries and struggles, it was exiting to have a week of pure magic.  The only non-magical moment was the Florida humidity which killed Atley's feeding pump the first night.  Luckily, the resort had a backup for us to borrow!

Ariel started a "Princess lip print chain" going on Atley's cheek.  By the end of the day, he had 6 princess lip-prints and refused to wash his face that night!

 

 

Atley's Heart Story - part two. The long road to recovery.

After Atley came home from the hospital at two months old, he relaxed and enjoyed being held and cuddled.  He was almost always smiling and happy, but developmentally, he was far behind a two month old baby.  Atley was very weak and could not bottle feed.  Even breathing was difficult, and he had a horrible time gaining weight.  Atley's strokes during his first fragile week of life had left him with hypotonic cerebral palsy.  This is a neurological disorder caused when the brain does not send enough signals to the muscles, causing them to be floppy and look very thin.  There is no cure for this problem, but building muscle strength helps compensate for the low muscle tone.

 Keller holds Atley on his first day home, June 2000.

 

 
  Atley's big, gummy smile, 3 months old.

 

 Atley and "super-chunk" Parker, 4 months old.

At seven months old, Atley had stomach surgery for reflux (fundoplication) and had his uncomfortable nasal feeding tube replaced with a more permanent feeding tube leading directly into his stomach.  This style of feeding tube a " button" is comfortable and hidden under clothing.  Atley's stomach became much worse after his fundoplication and his feedings had to be stretched out to almost 24 hours a day.  He had a small, portable feeding pump to keep the food going all day long.

Atley showing off his "button" and fundoplication scars - 7 months old.

A Nissen Fundoplication stomach wrap to stop severe reflux.

 

Please stay tuned for Part Three...

Atley's heart story - part one. The first two months; the first two surgeries.

February 14th is not only Valentine's Day, but also Congenital Heart Defects Awareness Day.  Did you know congenital heart defects are the #1 birth defect and leading cause of infant deaths due to birth defects?  I sure didn't until Atley was born.

As many of you know, my 9 year old son, Atley, was born with a very severe congenital heart defect called Hypoplastic Right Heart Syndrome due to the damage this defect causes to the right side of the heart.  This heart defect left Atley’s right ventricle, pulmonary arteries and pulmonary branch arteries very small and underdeveloped (hypoplastic) because the right side of his heart did not develop properly in utero. 

Even after four Level II ultrasounds, Atley’s severe heart defects went undetected until hours after his birth.  Atley was immediately transported to Children’s Mercy Hospital in Kansas City, where he underwent open heart surgery shortly after birth to create an artificial connection (B-T shunt made of Gore-Tex) between the aorta and the pulmonary artery to deliver blood to the lungs. The left ventricle must take over as the sole pumping chamber for the entire heart, and the shunt allows for gravity to aid blood flow to the lungs.  

 

Atley - 26 weeks gestation.  You can see his heart in the ultrasound picture.

Because Atley was very unstable, had several strokes and heart attacks while on maximum life support, his doctors did not expect him to survive his first week of life.  I was dismissed from the hospital quickly, as the doctors wanted me to visit Atley before his likely death.

 Atley - 3 days old.  We could only touch the top of his head because of all the equipment.

Atley was on 16 IV drugs, a ventilator, and more beeping alarms than you can imagine.

Not a beautiful newborn picture.  Atley was swollen to twice his birth weight due to heart failure.

Atley's hospital recovery was very slow and compounded by Atley’s need for another heart surgery at one month old, and his severe ventilator dependence.

Atley - one month old.

 
I was so excited to hold Atley for the first time when he was 7 weeks old.
 

Atley finally came home from intensive care at Children’s Mercy at two months old, but his weakened condition, including brain damage caused by his strokes, led to complete reliance on a feeding tube and need for continuous oxygen. 

Atley - 2 months old and leaving the hospital.  We did not travel light!

 
Parker and Atley meet at two months old.  Parker was over 11 lbs and Atley was 7lbs, 7oz.

 

Please stay tuned for the next chapter of Atley's Heart Story.