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By: Cassandra Petrilla
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

 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
transmembrane (CFTR).

 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
    inherited?


 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.
Intestines
 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.


                                                            Lungs
                                          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
                                          grow.
                                           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
failure.
NO CURE
                                                   FOR CF!!!




 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
necessary.
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
 http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html
 http://www.yourgenesyourhealth.org/cf/have.htm
http://www.ask.com/bar?q=how+do+you+treat+cystic+fibrosis&page=1&qs
rc=0&ab=2&u=http%3A%2F%2Fhcd2.bupa.co.uk%2Ffact_sheets%2FMosb
y_factsheets%2FCystic_fibrosis.html
 http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html
http://www.lpch.org/clinicalSpecialtiesServices/COE/PulmonaryCareCF/ov
erview.html

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Cystic Fibrosis....Cassie

  • 2. It is an inherited disease of your secretory glands, including the glands that make mucus and sweat.
  • 3.  Cystic fibrosis of the Pancreas  Fibrocystic Disease of the Pancreas  Mucoviscidosis  Mucoviscidosis of the Pancreas  Pancreas Fibrocystic Disease  Pancreatic Cystic Fibrosis
  • 4. 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 transmembrane (CFTR).  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.
  • 5. How is CF inherited?  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.
  • 6. 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.
  • 7. Intestines  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. Lungs 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 grow.  This leads to serious lung infections and over time can severely damage your lungs.
  • 8. • 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 failure.
  • 9. NO CURE FOR CF!!!  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 necessary.
  • 10. Prevalance Rate of CF-1 in 31,000 in U.S. get it
  • 11.  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