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.

Category: Science