PVNH stands for Periventricular Nodular Heterotopia. It is a rare disease which is also known as BPH, BPNH or PNH. It also includes a variant, PVNH4 (Ehlers Danlos variant).
PVNH Support & Awareness was created as a legacy for my daughter, Ella Dupont Bedassie. Ella was 7 months and 20 days when she passed away from respiratory failure brought on by what we did not know at the time was a rare disease: Periventricular Nodular Heterotopia, Ehlers Danlos variant (PVNH4).
Ella had been afflicted with end-stage emphysema most likely before she was even born but it was only discovered days before her 3rd month birthday. And even though Ella suffered from an un-curable disease, you would never have known it. She was and still remains (in my head and in my heart) a vivacious little girl with a smile that spread from ear to ear, feet that danced up in the air to the beat of her own drums and fingers that quickly wrapped themselves on daddy’s fingers to hold or mommy’s hair to twirl.
Ella was hospitalized at BC Children’s Hospital (BBCH) three times, totaling 147 days. The first stay was 4 days, the second stay was 27 consecutive days where I was with her 24/7 and the last one in ICU spanning over 4 months at 116 days (104 of which were in ICU). We were fortunate that we lived minutes away from BCCH but it quickly became obvious to me that most families do not have that luxury.
When Ella was battling for her life, it was clear that Ella’s mission and my mission when Ella would be healthy would be the same – to learn as much as possible about her condition to help other families affected by it.
This site is for families affected by Periventricular Nodular Heterotopia.
This is for Ella, so that her death is not in vain and her memory keeps on living, forever.
