Mucoviscidosis

WHAT’S THAT?
Cystic fibrosis (CF), also known as Mucoviscidosis, is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine.

WHO’S AFFECTED?
Most common in: people of Northern European ancestry (~1 of 3000 newborns)
Least common in: Africans and Asians
First recognized by Dorothy Andersen in 1938

SYMPTOMS OF THIS DISEASE
Difficulty breathing;
Coughing up mucus;
Poor growth;
Fatty stool.

HOW CAN WE DEAL WITH IT?
There is no known cure.
What can help:
Antibiotics;
Antibiotic azithromycin long term;
Inhaled hypertonic saline and salbutamol;
Lung transplantation;
Pancreatic enzyme replacement;
Fat-soluble vitamin supplementation;
Airway clearance techniques.
Average life expectancy is between 42 and 50 years.

WHAT IS THE CURRENT SITUATION?
The prognosis remains unfavorable.
Mortality rate is 50-60%(less with late and inadequate therapy)
The genetic counseling of families with cystic fibrosis patients is very important.
The average life expectancy of patients:
In European countries: 40 years
In Canada and USA: 48 years
In Russia: 22-30 years