Cystic Fibrosis - Living With - Living With

Regular checkups with your doctor may be part of your follow-up and treatment. How often your regular checkups take place will depend on your age. Younger patients, and those who have just been diagnosed, will have more frequent visits. As adults, you may see your doctor less often, perhaps every three months and then once a year for an evaluation.

Regular checkups may include:

• Education about airway clearance, infection control, energy, and nutrition goals
• Height and weight measurement, and calculation of BMI
• Physical activity guidance
• Physical exam, including examination of the heart, lungs, and abdomen to check the liver and for abdominal pain
• Physical therapy evaluation and needs
• Psychological assessment and support

Return to Treatment to review possible treatment options for your cystic fibrosis.

In addition to more frequent regular checkups, you may need to see your doctor for additional tests and evaluations, which may include the following:

• Abdominal ultrasound or CT scan to look for the cause of abdominal pain, check the pancreas and liver, and look for distal intestinal obstruction.
• Blood tests to check for diabetes, infection, liver disease, side effects of medicines such as damage to kidneys, nutritional status including complete blood count, and vitamin levels.
• Bone mineral density tests to check for osteopenia or osteoporosis in those who are at risk. You may be at risk if you are take corticosteroids long term, have severe lung disease, or do not get adequate nutrition. Testing of bone mineral density may be done with an X-ray test called a dual-energy X-ray absorptiometry (DEXA or DXA) scans.
• Chest CT scan to look for changes in lung function or lung infection
• Chest X-ray to look for lung abnormalities and infections. Early changes may be seen on X-ray before you notice symptoms. In younger children this may be done every other year to decrease radiation exposure.
• Colonoscopy to monitor for colorectal cancer because of increased risk.
• Glucose monitoring and testing to check for cystic fibrosis-related diabetes.
• Lung biopsy to test for specific bacteria.
• Pancreas function testing, which may include looking at an enzyme called pancreatic elastase-1 in your stool.
• Pulmonary function tests, which includes checking oxygen levels in your blood and spirometry, which is the most important and widely used tool to assess lung function in cystic fibrosis. Regular spirometry is used to monitor lung function in people age 6 and older and may be done in children as young as age 3.
• Respiratory sample smear and culture, which involves taking airway secretion or mucus samples every three months to look for microorganisms in the respiratory tract, and if necessary, treat them.
• Review of caloric intake, and pancreatic enzyme replacement.
• Review of treatments and medicines, including a pharmacist’s assessment.

Staying healthy is an extremely important part of cystic fibrosis care. Your medical team will work with you to develop a plan for lifestyle changes that can become part of your everyday life. These include the following:

• Avoiding tobacco smoke, including secondhand smoke.
• Being physically active to improve lung function. Physical activity helps with airway clearance and improves bone mineral density, muscle strength, flexibility, and posture. Before starting any exercise program, ask your doctor about what level of physical activity is right for you.
• Healthy eating to improve overall health. Healthy eating is also important for normal growth in children who have cystic fibrosis. You may need to increase your food or calorie intake by eating more foods or by eating high-energy foods. A high-sodium eating plan or supplementation with sodium may be recommended at times.

To help you prevent complications and reduce the risk of infections, your doctor may recommend the following:

• Continue your treatments, including medicines, supplements, and daily airway clearance techniques. Treating infections and pulmonary exacerbations is important to preserve lung function and slow the progression of disease. Lung disease is the major source of CF-related complications.
• Practice good hand hygiene whenever it is appropriate, such as before and after taking medicines and breathing treatments, before you eat, after you use the bathroom, and after blowing your nose. Wash your hands often with soap or use an alcohol-based hand sanitizer.
• Receive recommended vaccines, which includes routine immunizations, vaccine for pneumococcus, and an influenza shot every year at the start of flu season. Anyone who lives with you or whom you see often should also get regular vaccines.

Your doctor may recommend medicines to treat complications of cystic fibrosis, including:

• Antibiotics to prevent or treat lung infections and pulmonary exacerbations.
• Insulin to treat diabetes caused by destruction of the pancreas, if needed.
• Medicine to help unclog ducts in the liver and improve bile flow. This includes ursodeoxycholic acid. This may improve abnormal liver function blood tests.
• Nutritional supplements when healthy eating is not enough. Your doctor may recommend supplements such as calcium, multivitamins, oral pancreatic enzymes, sodium, or vitamin A, D, E, and K.
• Oxygen therapy to treat low levels of oxygen in the blood. This may improve the ability to be physically active and attend school or work.
• Surgeries such as lung transplant may help people with advanced lung disease and respiratory failure. Liver transplant may be an option for advanced liver disease such as cirrhosis. A person who undergoes a lung transplant will also need pulmonary rehabilitation after the surgery.

Cystic fibrosis may have serious complications. Call your doctor if you believe you have any of the following:

• Pulmonary exacerbation, which involves a worsening of lung symptoms, such as more coughing or wheezing, chest congestion, and a change in mucus color. You may also have weight loss, a poor appetite, or fever.
• Hemoptysis, which may be a sign that an artery has broken and is bleeding into the airway.
• Sudden shortness of breath or chest pain, which may be a sign of a pneumothorax, or collapsed lung.

Return to Signs, Symptoms, and Complications to review complications.