CYSTIC FIBROSIS
ONE OUT OF EVERY 2,500 BIRTHS IN THE UNITED STATES WILL BE DIAGNOSED WITH CYSTIC
FIBROSIS. THIS FACT MAKES CYSTIC FIBROSIS ONE OF THE MOST COMMON GENETIC DISEASES IN THE
NATION. ABOUT 30,000 AMERICANS HAVE THE DISEASE, BUT
EVEN THOUGH CYSTIC FIBROSIS IS THE NATIONS MOST COMMON GENETIC DISEASE THE MAJORITY OF
AMERICANS KNOW LITTLE ABOUT IT. CYSTIC FIBROSIS IS RELATIVELY COMMON IN CALCASTION
PEOPLE BUT RARE IN AFRICAN-AMERICAN. THE DISEASE IS VERY UNCOMMON IN MONGOLIANS. FIVE
PERCENT OF THE POPULATION IN THE UNITED STATES ARE CARRIERS OF THE GENETIC DISEASE.
CYSTIC FIBROSIS, SOMETIMES CLASSIFIED AS MUCOVISCIDOSIS, IS A DISORDER IN WHICH THE
EXCRORINE GLANDS SECRETE ABNORMALLY THICK MUCUS. THIS LEADS TO THE OBSTRUCTION OF THE
PANCREAS AND CHRONIC INFECTIONS OF THE LUNGS, WHICH GENERALLY CAUSES DEATH IN CHILDHOOD
OR EARLY ADULTHOOD. SOME MILDLY AFFECTED PATIENTS MAY SURVIVE LONGER. PATIENTS WITH
PANCREATIC INSUFFICIENCY TAKE PANCREATIC ENZYMES WITH MEALS. THOSE WITH RESPIRATORY
INFECTIONS ARE TREATED WITH ANTIBIOTICS, MOSTLY WITH AEROSOLS THAT RELIEVE CONSTRICTION
OF THE AIRWAYS. PHYSICAL THERAPY IS USED TO HELP PATIENTS COUGH UP THE OBSTRUCTING MUCUS.
INTESTINAL OBSTRUCTION, WHICH OCCURS MOSTLY IN INFANCY, MAY REQUIRE SURGERY.
IN 1989, RESEARCHERS FOND THE ABNORMAL GENE THAT CAUSES CYSTIC FIBROSIS. THIS GENE IS
LOCATED ON CHROMOSOME 7 . A PERSON WHO HAS TWO CYSTIC FIBROSIS GENES HAS THE DISEASE . A
PERSON THAT CARRIES ONE OF THE GENES DOES NOT HAVE THE GENETIC DISEASE, BUT IS A
CARRIER.
THE SYMPTOMS OF CYSTIC FIBROSIS SOMETIMES OCCUR IMMEDIATELY AFTER BIRTH. MUCUS
SECRETIONS MAY APPEAR IN THE BABY'S INTESTINES, WHICH CAN CAUSE OBSTRUCTION IN THE
INTESTINES. IN ALL CASES, THE CHILD WILL GAIN LITTLE WEIGHT RIGHT FROM BIRTH, BECAUSE THE
PANCREAS IS NOT PRODUCING ENZYMES. LITTLE TO NO NUTRIENTS ARE ABSORBED IN THE CHILD'S
SYSTEM. A CHILD WITH CYSTIC FIBROSIS MAY HAVE REOCCURRING RESPIRATORY INFECTIONS, ALONG
WITH COUGH AND FEVER. THIS MAY BE MORE SEVERE AND PERSISTENT THAT NORMAL THIS IS A RESULT
OF THE THICK, STICKY MUCUS THAT WILL HOLD AND TRAP GERMS IN THE BRONCHIAL TUBES. IT
SHOULD BE TAKEN IN TO CONSIDERATION THAT CHILDREN WITH CYSTIC FIBROSIS HAVE LARGE
APPETITES AND EAT A GREAT DEAL. IN SPITE OF THEIR MALNUTRITION, THEY ART NOT IN PAIN AND
DO NOT GENERALLY FEEL IT.
EXTRACTS OF ANIMAL PANCREAS, IN POWDER OR GRANULE FORM, ARE PRESCRIBED TO REPLACE THE
MISSING ENZYMES FROM THE PANCREAS, AND THE AMOUNT OF FAT IS DECREASED IN THE CHILD'S
DIET. WITH THIS TREATMENT THE CHILD BEGINS TO GAIN WEIGHT. TO KEEP THE LUNGS FREE OF AS
MUCH MUCUS AS POSSIBLE , THE PATIENTS MAY NEED TO HAVE DAILY RESPIRATORY PHYSICAL
THERAPY. ANY RESPIRATORY INFECTION THAT ARISE ARE TREATED WITH LARGE AMOUNTS OF
ANTIBIOTICS.
CYSTIC FIBROSIS CAN NOT YET BE CURED. ALTHOUGH THE IDENTIFICATION OF CHROMOSOME 7 HAS
PAVED THE WAY FOR GENE THERAPY. ANTIBIOTICS AND ENZYMES ARE NOT THE ONLY TREATMENTS FOR
CYSTIC FIBROSIS. ONE RELATIVELY NEW TREATMENT IS A BIOTECH DRUG THAT THINS THE MUCUS,
WHICH HELPS THE LUNGS FUNCTION BETTER AND REDUCES THE RISK OF INFECTIONS. GENE THERAPY IS
STILL IN EXPERIMENTAL STAGES.
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