Alagille Syndrome/TOF with Pulmonary Atresia

[Youyou]

Hi everyone, my son was diagnosed with alagille syndrome when he was one month old. At first they thought it was only tetralogy of fallot (TOF). At one month old he was having tet spell and had to have open heart surgery. They put a cardiac central shunt to allow better blood flow to his lung. That procedure did help him with the tet spell but left him with his left vocal cord paralyzed. Over time he couldn’t eat the way he used to and had to have a Gtube. We were told that as he’s gaining weight his small lung arteries will also grow. Fast forward 17 month they stayed the same size as he was born. In top off all the nightmares he has going on with his heart he also has cholestasis liver disease that comes with a lot of itching and jaundice not to mention gagging/vomit all the time. The medical team in Florida had decided to transfer his case to Lucile Packard Stanford California.

Over there they have a pulmonary reconstruction program. So 3 weeks ago he had an open heart surgery to repair his pulmonary arteries. The surgery itself was successful but his liver disease complicates his recovery. At first they had to deal with severe bleeding because of his inability to clot properly which affected all the other organs. They team decided to put him on ECMO which I think is a live saver and a curse. One week later his left lung collapsed after closing his chest. CT scan showed no physical airway obstruction while the bronchoscopy showed his left airway closed. Both of his kidneys stopped working. He’s still on ECMO and now on dialysis.

Despite all that I’m trying to stay positive that my baby will come out of this madness.

I need some motivations. Please share your successful stories. Thank you!

[Lois-Cldf Partner]

Thank you for sharing with us. I do hope they can get things under control for your son. x