Adeline, Velocardiofacial Syndrome, Tetralogy of Fallot Pulmonary Atresia VSD MAPCAS
Adeline was born September 24, 2013 at Crouse Hospital in Syracuse, NY with a rare congenital heart defect that was paired with a chromosomal deletion called Velocardio Facial Syndrome.
September 9, 2015 was Adeline’s first open heart surgery. Operating was Dr. George Alfieris at Strong Memorial Hospital in Rochester, NY. This surgery was approximately 11 hours. She had a few expected complications and also a couple that were not expected, but overall her full stay was 14 days.
The chronic steroid use caused her adrenal glands to shut down. Over time her lungs started to become a larger issue after spending the better parts of September-April each year in and out of the hospital, usually testing positive with multiple respiratory illnesses at once. With her specific heart diagnosis and chromosomal deletion, the severe pulmonary issues Adeline ended up being diagnosed with isn’t the “norm” for children like her.
September 12, 2018 was her second open heart surgery also performed by Dr. George Alfieris at Strong Memorial. She did have more severe complications this time. We were there for a month. She came home with a central line for me to administer infusions around the clock for an extra four weeks after discharging the hospital.
Before, between, and after both open heart surgeries she dealt with countless hospital admissions, other procedures, imaging, bloodwork, and testing.
In April 2019, due to the constant wear and tear on her lungs over the years that had finally taken its toll, her pulmonary specialist had exhausted all of his resources from different medications, medical devices, biopsies etc. He referred us to get a second opinion at the Morgan Stanley Children’s Hospital Columbia University in NYC. They had only one diagnosis in mind which is a progressive illness and would need a double lung transplant.
In July 2020 we went to Pittsburgh, PA to visit the Children’s Hospital there which is home to the top lung/heart transplant specialists in the country.
Adeline’s heart is so complex she could not get a heart transplant without a lung transplant or vice versa. She endured a week for a very intense and busy transplant evaluation. This included 8 to 10-hour days of testing, imaging and bloodwork, and meeting all of the specialists she had the possibility of working with if she were a transplant candidate. Her last day there she had a cardiac catheterization. The cardiologist placed a balloon in an artery in hopes it would cause stronger blood flow to the lungs and also place a coil to close an artery off with the intention of aiding the artery he ballooned.
The transplant team consulted and they deemed she was not a candidate for a double lung/heart transplant since her mortality rate was at 94%.
In December, 2020 we went back to Pittsburgh for another cardiac catheterization only to find that the interventions he implemented ended up doing nothing for her heart, but (not due to the balloon and coil) her pressures had actually worsened. At the post-op consult I felt the sincerity in the Doctor’s eyes, voice, body language. He assured me I should never wonder if I left a single stone unturned. He expressed “This child is here today because of you. Because of your persistence, advocacy, fight, and instincts.”
Adeline was placed in the care of Hospice of Jefferson County on April 27, 2022 and took her last breath early September 11, 2022.
Despite all she had been through, this witty, tenacious, brave, smart, pretty little 8 year old was always smiling.
