London’s Heart

My name is Shelly and I am the proud mother of a smart and sassy 6 year old girl named London.  London was born with a single ventricle congenital heart defect called Double Inlet Left Ventricle. She had her first open heart surgery, BT Shunt, at 5 days old.

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Then at 4 months old, London had her second open heart surgery, the Glenn, followed by the Fontan at 3 years old.

Her recovery time following the last surgery was delayed due to excess chest fluid, and would require London to remain at Phoenix Children’s Hospital for a month.  Dr. John Nigro performed all three ofLondon’s open heart surgeries.

After the Fontan surgery, we could breathe again. The surgery was no longer looming in our future, and I truly thought we were out of the woods for good.  About 2 years after London’s Fontan surgery (when London was 5),  she developed a wet cough that would last for a week, and then disappear for two weeks, and then come back again.

It was a never-ending nightmare. We were told by several pediatricians that she suffered from seasonal allergies or respiratory viruses that just needed to run their course.

However, about 3 months into her coughing spells, London started coughing up what looked to me like chunks of “raw chicken.” These things were filling in her lungs. When placed in water, these things took on a life of their own. We soon figured out that London was coughing up bronchial casts and she was diagnosed with plastic bronchitis.

London’s quality of life was pretty poor. She was in and out of the Cardiac ICU undergoing several treatments and procedures. London struggled with every breathe that she took, and she would turn purple trying to cough these things up.

Photo8 (2)It got to a point where London was coughing up casts daily. There was always a risk that these casts would block her airway and she’d suffocate. Night time was a very scary time, as she would be up all night struggling to breath. And during the day, she was hooked up to several breathing treatments and an airway vest system which, in total, took up to about 3 hours a day.

There was little to no research or treatment plans that were out there for this condition, the true cause of it was unknown, and the only cure seemed to be a heart transplant.

I did find a small support group of parents online whose children had plastic bronchitis. It was here where I connected with 2 families whose children had benefited from a breakthrough lymphatic procedure developed at Children’s Hospital of Philadelphia by interventional cardiologist Dr.  Yoav Dori. I was hopeful that Dr. Dori could cure London of her plastic bronchitis, something no other doctor had said was possible.

Photo9We had never traveled out of state, but we were desperate to help save our daughter’s life. In June of 2014, London was the third patient to have ever undergone this procedure which was done by Dr. Max Itkin and Dr. Dori. It was a very long 8-hour procedure in which afterwards we found out that London has a lymph abnormality- a second thoracic duct where there would normally only be one, that was dumping fluid straight into her lungs. Doctors Itkin and Dori successfully closed the extra duct.

Before we left for our trip to Philly, Dr. Pophal, London’s PCH cardiologist, presented us with a 3D model of London’s heart to take with us to share with the CHOP team, who hadn’t cared for London yet. London even carried her model through security in a cooler. Luckily, we had no questions asked!

I had read an article online previous to this, about this printing at PCH, and I HAD to have one of these hearts.  I was one of those kids in school that hated history because if I couldn’t see it and touch it, I couldn’t understand it.

Photo10I have had London’s heart defect explained to me so many times, but it,honestly, goes in one ear out the other. It was so exciting for us to have London’s printed heart to fully understand what was going on inside her little heart. Her 3D printed heart is in a special place in our home.

One day I saw London holding up the 3D heart and holding it up to her chest looking confused. She asked me, “Mom, where’s my heart?” I soon realized that she was looking for the shape of a Valentine’s heart.

Since London’s lymphatic procedure, she has been cast-free and this procedure has been a cure for her plastic bronchitis. Shortly after her successful lymphatic procedure at CHOP,  we got to enjoy a couple days at Disney World.  London rode Thunder Mountain seven times and loved every minute of it.

IMG_6898We have been very fortunate to have such a great team of doctors and nurses whohave cared for London and our family through this journey. I am amazed and so very thankful for dedicated doctors and cutting-edge technology that saved my daughter’s life.