Cystic Fibrosis

By: Morgan J. Brown

Definition

• Cystic fibrosis is a thick mucus that clogs the air ways and tends to cause lung diseases.

• A diseases common among Caucasians and is usually found in those of the younger verity.

Causes

• Cystic fibrosis is an inherited diseases that can only be give if both parents have the cystic fibrosis defective gene.

• It is caused by a defective gene inside the body that causes the body to produce a thick, sticky substance called Mucus.

Symptoms

• Do to there being thousands of strand of the defective CF gene many people show different symptoms.

Symptoms con.

• For infants common symptoms are delay in birth, no bowel movement between birth and forty eight hours, and salty tasting skin.

• Others are gray stool, severe constipation, and weight loss.

Tests

• Chest x-ray or CT scan

• Fecal fat test

• Lung function tests

• Measurement of pancreatic function

• Secretin stimulation test

• Trypsin and chymotrypsin in stool

• Upper GI and small bowel series

Treatment

• For lungs• Antibiotics to prevent and treat lung and sinus infections. They

may be taken by mouth, or given in the veins or by breathingtreatments. People with cystic fibrosis may take antibiotics only when needed, or all the time. Doses are usually higher than normal.

• Inhaled medicines to help open the airways • DNAse enzyme replacement therapy to thin mucus and make

it easier to cough up • Flu vaccine and pneumococcal polysaccharide vaccine (PPV)

yearly (ask your health care provider) • Lung transplant is an option in some cases • Oxygen therapy may be needed as lung disease gets worse

Treatment

• For the bowels and nutrition

• A special diet high in protien and calories for older children and adults (see: Cystic fibrosis nutrional considerations)

• Pancreatic enzymes to help absorb fats and protein

• Vitamin supplements, especially vitamins A, D, E, and k Your doctor can suggest other treatments if you have very hard stools

Death

• Most people with cystic fibrosis usually die from complications rather than the diseases itself.

• Usually they don’t live past the age of 35.

Complications

• Bowel problems, such as gallstones, intestinal obstruction, and rectal prolapse

• Coughing up blood • Chronic respiratory failure • Diabetes • Infertility • Liver disease or liver failure, pancreatitis, biliary cirrhosis • Malnutrition • Nasal polyps and sinusitis • Osteoporosis and arthritis • Pneumonia, recurrent • Pneumothorax • Right-sided heart failure