Cystic Fibrosis Living With

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If you or your child has been diagnosed with cystic fibrosis, it is important that you understand how cystic fibrosis can affect your health. You should also follow your treatment plan and see your doctor regularly.

Cystic fibrosis affects many parts and systems of the body. Complications will depend on the affected organs and the severity of disease.

Possible complications of cystic fibrosis include:

• Allergic bronchopulmonary aspergillosis (ABPA), which is an allergic reaction in the lungs to the fungus Aspergillus
• Bronchiectasis, a common complication of cystic fibrosis caused by long-term inflammation or obstruction of the airways
• cancer of the digestive tract, including the esophagus, stomach, small bowel, large bowel, liver, and pancreas
• Collapsed lung, called pneumothorax, resulting in air in the space between your lung and chest wall
• Diabetes due to damage to the pancreas, where insulin is made
• Fertility problems
• Gastrointestinal problems, such as distal intestinal obstruction syndrome (DIOS), in which your intestine becomes blocked by very thick intestinal contents
• Heart failure because of lung damage
• Hemoptysis, or coughing or spitting up of blood
• Kidney problems due to diabetes and some antibiotics, or kidney stones
• Liver disease or failure caused by blockage of the bile ducts in the liver, which may lead to cirrhosis and a need for a liver transplant
• Lung infections that may come back or be difficult to treat
• Malnutrition because the pancreas may not make enough enzymes to help digest and absorb nutrients from food
• Mental health problems, such as depression and anxiety
• Muscle and bone complications, including low bone density and osteoporosis, joint pain and arthritis, and muscle pains
• Pancreatitis and low levels of pancreatic enzymes leading to nutritional deficiencies, including low levels of vitamins A, D, E, and K
• Pulmonary exacerbations, which are episodes of worsening cough, shortness of breath, and mucus production caused by airway inflammation and blockage from an increase in Bacteria in your airways and lungs
• Salt loss syndrome, in which your body quickly loses salt, or sodium and chloride, causing electrolyte and other imbalances
• Urinary incontinence, or loss of bladder control

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

In addition to more frequent regular checkups, you may need to see your doctor for other tests.

• Abdominal ultrasound or a CT scan can look for the cause of abdominal pain, check the pancreas and liver, and look for distal intestinal obstruction.
• Blood tests 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 check for osteopenia or osteoporosis in those who are at risk. You may be at risk if you 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) scan.
• Lung imaging tests look for changes in lung function or lung infection. Early changes may be seen before you notice symptoms. In younger children, this may be done every other year to reduce radiation exposure.
• Glucose monitoring and testing check for cystic fibrosis–related diabetes.
• Pancreas function testing may include looking at an enzyme called pancreatic elastase-1 in your stool.
• Lung function tests include checking oxygen levels in your blood and spirometry, which is the most important and most 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 involves taking airway secretion or mucus samples every three months to look for microorganisms in the respiratory tract and, if necessary, treat them.

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.

• Avoid tobacco smoke, including secondhand smoke.
• Be 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.
• Choose healthy foods 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.