Even in the same child, symptoms may worsen or improve as time passes. One of the first signs of cystic fibrosis is an excessively salty taste to the skin. People with cystic fibrosis tend to have higher than normal amounts of salt in their sweat. Most of the other signs and symptoms of cystic fibrosis affect the respiratory system or the digestive system. The thick and sticky mucus associated wit
h cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause:
Persistent cough
Wheezing
Repeated lung infections
Repeated sinus infections
The thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these digestive enzymes, your intestines can't fully absorb the nutrients in the food you eat. In cystic fibrosis, a defective gene alters a protein that regulates the normal movement of salt (sodium chloride) in and out of cells. This results in thick, sticky secretions in the respiratory and digestive tracts, as well as in the reproductive system. The affected gene, which is inherited from a child's parents, is a recessive gene. With recessive genes, children need to inherit two copies of the gene, one from each parent, in order to have the disease. If children inherit only one copy, they won't develop cystic fibrosis, but will be carriers and possibly pass the gene to their own children. Complications:
Bronchiectasis. Cystic fibrosis is one of the leading causes of bronchiectasis, a condition in which damaged airways widen and become flabby and scarred. Chronic infections. Thick mucus in the lungs and sinuses provide an excellent breeding ground for bacteria. Most people with cystic fibrosis have almost constant infections in their lungs and sinuses. Collapsed lung. Repeated lung infections damage the lungs, making it more likely for the lung to collapse. Nasal polyps. Because the lining inside the nose is inflamed and swollen, it's more likely to develop large or multiple polyps — soft, fleshy growths inside your nose. Respiratory failure. Over time, cystic fibrosis can damage lung tissue so badly that it won't work anymore. Lung function typically worsens gradually, and it eventually can become life-threatening. Nutritional deficiencies. Thick mucus blocks the tubes that carry digestive enzymes from your pancreas to your intestines. Without these enzymes, your body can't absorb protein, fats or the fat-soluble vitamins — A, D, E and K. Diabetes. The pancreas also produces insulin, which your body needs to use sugar. Cystic fibrosis increases your risk of developing diabetes. Blocked bile duct. The tube that carries bile from your liver and gallbladder to your small intestine may become blocked and inflamed, leading to liver problems, such as cirrhosis, and sometimes gallstones. Intussusception. Children with cystic fibrosis are at higher risk of intussusception, a condition in which a section of the intestines folds in on itself like an accordion
Many men with cystic fibrosis are infertile because the tube that connects the te**es and prostate gland (vas deferens) is either blocked with mucus or missing entirely. Although women with cystic fibrosis may be less fertile than other women, it's possible for them to conceive and to have successful pregnancies
Osteoporosis. People with cystic fibrosis are at higher risk of developing osteoporosis, a dangerous thinning of bones. This may be linked to the body's inability to absorb vitamin D, which helps build strong bones. Electrolyte imbalances. Because people with cystic fibrosis have saltier sweat, this can upset the balance of minerals in their blood. Symptoms include increased heart rate, fatigue, weakness and low blood pressure. How do you Know? Sweat tests
DNA tests
Blood tests
Treatments:
Antibiotics. These drugs are used to treat and prevent lung infections. They may be swallowed in pill form, inhaled in a mist or delivered intravenously. Mucus-thinning drugs. Drugs that reduce the stickiness of your mucus make it easier to cough up the mucus, which improves lung function. Bronchodilators. Medications such as albuterol — delivered by an inhaler or a nebulizer — help keep your airways open by relaxing the muscles around your bronchial tubes. Pancreatic Enzymes. To break down fats that your pancreas cannot handle on its own. Chest clapper. This hand-held device can mimic the effect of cupped hands clapping over the ribs. Inflatable vest. This device vibrates at high frequency to loosen chest mucus. Breathing devices. Performing specific breathing exercises while exhaling through the device's tube or a mask may also be helpful. Feeding tube. Cystic fibrosis interferes with digestion, so you can't absorb nutrients from food very well. This tube may be threaded through your nose to your stomach, or surgically implanted. Lung transplant. Your doctor may suggest lung transplantation if you have severe breathing problems, life-threatening pulmonary complications or increasing resistance to antibiotics used to treat lung infections. Because both lungs are affected by cystic fibrosis, both need to be replaced. Bowel surgery. If you have developed a blockage in your bowel, you may need emergency surgery to remove it — especially if that part of your bowel has died. Staff, Mayo Clinic. "Definition." Mayo Clinic. Mayo Foundation for Medical Education and Research, 02 Mar. 2010. Web. 07 Mar. 2012. .
