It is an inherited disease of your secretory
glands, including the glands that make
mucus and sweat.
Cystic fibrosis of the Pancreas
Fibrocystic Disease of the Pancreas
Mucoviscidosis of the Pancreas
Pancreas Fibrocystic Disease
Pancreatic Cystic Fibrosis
What causes CF?
When a mutation called delta-F508 occurs in a single gene on chromosome 7.
The gene that is infected contains instructions for building a protein called cystic fibrosis
The gene will there forth produce CFTR that does not work and chloride ions can not
pass through the membrane.
In 70-80% of the cases in the US the gene is completely missing from the membrane.
If CFTR is missing, chloride ions are not reabsorbed.
How is CF
A person gets CF when they inherit 2 mutated CFTR genes
, one from each parent.
The parents might not have the disease because they have a
normal copy of the gene on their other chromosome.
CF is a recessive disorder.
1. Your sweat is saltier than most people.
2. The doctors then use the “sweat test”.
3. During the test, the doctors use a sweat-inducing
chemical called pilocarpine.
4. If the reading comes back that your chloride ion
is above 60 mmol/liters, then you have CF.
The digestive enzyme that your pancreas makes (breaks down your food so you can
eat) cannot reach your small intestines.
Without this enzyme you cannot fully absorb fats and proteins.
This can then lead to vitamin deficiency and malnutrition because nutrients leave
your body unused.
Your mucus becomes thick and sticky.
The mucus will eventually build up in
your lungs and block the airways.
This buildup makes it easy for bacteria to
This leads to serious lung infections and
over time can severely damage your lungs.
• CF causes your sweat to leave your body 5 times saltier.
• This leads to abnormal heart rhythms.
• Your balance of minerals in your blood becomes upset
and can cause health problems.
• Have increased risk for diabetes or osteoporosis.
• Causes infertility in men and makes it harder for
women to get pregnant.
• Most common death of people with CF is respiratory
You can take supplements containing enzymes to help digestion.
You can take replacement enzymes such as pancreatin.
You can reduce chest infections with frequent physiotherapy
and either occasional or continuous antibiotics.
You can take physiotherapy which involves vigorous massage to
help loosen the sticky mucus.
In severe cases a lung or heart and lung transplant operation is
Prevalance Rate of CF-1 in
31,000 in U.S. get it
Cystic Fibrosis Worldwide
Canadian Cystic Fibrosis
Cystic Fibrosis Research, Inc.
Karen’s Climb Foundation
Pennsylvania Cystic Fibrosis, Inc.
Reaching Out Foundation
St. Louis Second Wind Transplant Association