Patient stories
Congenital Portosystemic Shunts (CPSS), a rare malformation which can be treated
Congenital porto-systemic shunts (CPSS) are rare malformations causing the blood from the intestines to bypass the liver. This rare malformation can have a significant impact on health, but there are solutions to treat it and to prevent the severe symptoms. In this video, discover more about CPSS, their symptoms, and how to treat them. You’ll also find a moving testimonial of a family affected by this rare disease.
Our daughter was hospitalized for a week as she had a very low saturation. We reached 55% saturation. The doctors were telling us "We consult each other, we have meetings between cardiologists and pulmonologists to find out what we can do", until a given moment when the head of the pneumo department told us "Did we go to see the liver?" and the answer was no. We did a first investigation and that's how it all came together. We discovered the portosystemic fistula.
My daughter has had heart surgery and some complications but after two and a half years we couldn't get there, she was not capable of weaning from oxygen and we didn't understand the cause In fact, and we were doing neurological, cardiological consultations and at one point, we even considered the two heart-lung transplants. It was very complicated.
Before I remembered nothing. I was always tired, sleepy, small problems of understanding also. My brain was always foggy, I was never on time, could remember nothing at all. When I had surgery, things got better. I now remember things like my card code, knowing a little more about the value of money. I am no longer under secure guardianship. Today I work in a central kitchen center. I'm going to do more things and it's better this way.
We realized, almost ten days after the operation, that we saw her less tired, less breathless and even though the oxygenation rate was fine, the saturation rate was not necessarily adequate but we could see that it was better, it was obvious. And afterwards, gradually of course the saturation improved. We managed to lower the oxygen and after, weaning took six months. In fact everything got better gradually and we could resume a normal life after a year.
