Ayla’s Story 

On June 17, 2018 our beautiful darling daughter, Ayla Gunner Prushansky, was born term at 11:37pm after only 97 minutes of natural labor. She was born on Father's Day and what a gift she was. 

When it came time for us to be discharged home Ayla failed the Cardiac Discharge Screen but no one could figure out why. Ayla was pink, she had no structural abnormalities, my pregnancy was low risk, and she was not having any difficulties breathing. It did not however change the fact that her oxygen levels pre and post (oxygen sent to the brain and to the body) were not matching, therefore, she was moved to the neonatal intensive care unit at Cedars-Sinai Hospital. 

We were told that it appeared she had a case of PPHN, persistent pulmonary hypertension of the newborn, in which the babies circulation does not switch over from fetal circulation to circulation outside the womb, meaning that her lungs had not opened appropriately and it was causing a lot of strain on her heart.

Days went by in which Ayla seemed to be improving clinically but her echo-cardiograms were getting worse. We chose to move her to Children's Hospital of Los Angeles.

Shortly after the move, Ayla’s condition deteriorated quickly leaving her on a ventilator to breath. When that was no longer sustaining her, she was put on ECMO (extracorpeal membrane oxygenation or in other words a heart-lung bypass machine). 

Still, Doctors thought it was just one of the worst cases of PPHN they had seen in years but wanted to rule out a genetic condition called Alveolar Capillary Dysplasia. Since Ayla was on ECMO it was easy for them to draw her blood. We waited an entire week before we got the results that her blood tested positive for a mutation of the FoxF1 gene (Chromosome 16) that is directly related to ACD. The only option for treatment was a bilateral lung transplant. Both of us were genetically tested which showed we were not carriers of the mutation, meaning Ayla's mutation was a random mutation with an unknown cause. 80-90% of ACD mutations or deletions are of an unknown cause. 

This lead us on our journey to CHOP, Children’s Hospital of Philadelphia, as they are one of the only Children's hospitals in the country that perform such an operation. We had been in touch with Dr. Frank since the early start of Ayla's condition at Cedars and felt confident that this was the place that she needed to go. Unfortunately, Ayla had other plans. 

Her wonderful doctors sat us down to explain that she would not make it to transplant as none of the traditional PPHN treatments worked to control her condition. It was at this time that we decided it was best to withdraw care. We had her brother Jory come to the hospital to meet her.

Soon after we spent the last few precious hours we had with our beautiful little girl who would soon become an angel. Ayla gave us an amazing ten hours of pure love and sweetness before she passed away. Ayla fought hard for four weeks. She died on July 16th, 2018 in my arms holding her father's hand. 

ACD is a rare condition in which there is a deletion or mutation within the FoxF1 gene resulting in the alveoli not being constructed properly and in which the pulmonary veins are misaligned - sadly this disease is fatal. Through Dr. Frank and CHOP, some major advances in pulmonary vascular research are underway and we feel compelled to lead the charge in raising money to fund research for not only ACD but for all pediatric vascular lung diseases that claim babies lives. Join us in the fight to help these precious angels stay with us here on earth. 

Please check out Ayla’s Video at this link below for more pictures: