Medical interventions
- Sweat test. A sweat-producing chemical is applied to a small area of skin. The collected sweat is then tested to see if it's
saltier than normal. - Genetic testing. DNA samples from blood or saliva can be checked for specific defects on the gene responsible for cystic
fibrosis. - Imaging tests. The severity of the damage to the lungs and intestines can be monitored using an X-ray, CT scan and an MRI.
- Lung function tests. This measures the size of your lungs, how much
air you are able to inhale and exhale and how well your lungs delivery oxygen to your blood.
- Sputum culture. Sputum is tested for bacteria
- Organ function tests. Blood work is done to test liver and pancreas function. Children over 10yrs should be tested for diabetes.
nursing interventions
- Chest physical therapy. Loosening the thick mucus in the lungs makes it easier to cough up. Chest physical therapy helps loosen mucus and is usually done from one to four times a day. A common technique is clapping with cupped hands on the front and back of the chest.
- Mechanical devices also can help loosen lung mucus. The options include:
Chest clapper, a hand-held device that mimics the effect of cupped hands clapping over the ribs
Inflatable vest, a device worn around the chest that vibrates at high frequency
Breathing devices, usually a tube or mask through which you exhale while performing breathing exercises
- Pulmonary rehabilitation. Your doctor may recommend a long-term program to improve your lung function and overall well-being. Pulmonary rehabilitation is usually done on an outpatient basis and may include:
Exercise training, Nutritional counseling, Energy-conserving techniques, Breathing strategies, and Psychological
counseling or group support or both - Surgical and other procedures.
Oxygen therapy. If your blood-oxygen level declines, your doctor may recommend you sometimes breathe pure oxygen to
prevent high blood pressure in the lungs (pulmonary hypertension).
Endoscopy and lavage. Mucus may be suctioned from obstructed airways through an endoscope.
Lung transplant. If you have severe breathing problems, life-threatening lung complications or increasing resistance to
antibiotics used to treat lung infections, lung transplantation may be an option. Because both lungs are affected by cystic
fibrosis, both need to be replaced. Lung transplants for people with cystic fibrosis are controversial because studies indicate
the procedure is associated with many complications, and may not prolong life or enhance quality of life.
Feeding tube. Cystic fibrosis interferes with digestion, so you can't absorb nutrients from food very well. Your doctor
may suggest using a feeding tube to deliver extra nutrition while you sleep. This tube may be threaded through your nose to
your stomach or surgically implanted into the abdomen.
Bowel surgery. If a blockage develops in your bowel, you may need surgery to remove it. Intussusception, where a section of
bowel has folded in on itself, also may require surgical repair.
