Daily Self Care Guidelines

The Cystic Fibrosis Foundation (CFF) has identified several daily activities that promote long-term health in patients with CF. We strongly recommend that you perform these activities daily, and become knowledgeable and expert in how these benefit your health. We will help you learn the best techniques for you, and why they work. We recognize that doing everything everyday will be challenging, but the more you do, the better you will be.

Below you will find links to the CF Center’s Daily Self Care Guidelines. Or, click here for a printable PDF.

Airway Clearance Techniques

Airway clearance techniques (ACT) are important because they help both normal and abnormal secretions move through (up and out) the airways. Techniques include CPT, positive expiratory pressure (PEP), oscillating PEP (flutter and Acapella), autogenic drainage (AD), active cycle breathing technique (ACBT), the vest, intrapulmonary percussive ventilation (IPV), and vigorous exercise.

Recommendation: ACT should be done twice a day when you are well, and 4 times a day when you have increasing respiratory symptoms (e.g. cough, wheezing, congestion, etc.)

Goal:  Develop a working knowledge of several types of ACT’s and know when to increase their use from 2 to 4 times a day.  With time, become expert in use of ACT.

Nutrition

The CFF has found that maintenance of normal nutrition and a normal weight is associated with better health, better lung function, and longer life.  Many people with CF have lower than normal weight.  Because you have higher energy needs (need more calories) than those people without CF, you need to eat more calories—110% - 200% more than of what other people eat.  It is important that you eat a high-calorie diet, as well as a balanced diet (meats, fruits, vegetables, and dairy). The CFF recommends that we calculate weight for height in young children, and Body Mass Index (BMI) in those people 3 years and older. If your BMI is below the optimal level, or if you are not gaining weight appropriately, the dietician will work with you to help plan for improving your nutrition and growth. Often, she will suggest supplements to enhance your calories. Tube feedings at night may be helpful to further enhance your calories.

CFF defines Nutrition for children 2 – 20 years old as follows:

Optimal nutrition:      Greater than 50% BMI for age
Sub-Optimal:             25%-50% BMI for age
At Risk:                      10%-25% for age
Urgent Need:              Less than 10% BMI for age.

CFF defines Nutrition for adults Greater than 20 years old as follows:

Women should have a BMI of 22 or above.
Men should have a BMI of 23 or above.

Recommendation:  Eat three balances meals and two snacks each day.  Take your pancreatic enzymes, if prescribed, with meals and snacks.  Nutritional supplements may be useful if advised by your dietician.  Tube feedings at night may also be helpful in some people with CF.

Goals:  Develop knowledge of nutrition, including types and amounts of food, and how good nutrition helps your lung function. Understand the need for and dosing of pancreatic enzyme replacement therapy (PERT).  Learn how to calculate your BMI.  Have a working knowledge of your BMI.

Exercise

Regular exercise has many benefits for all people.  It is especially important for people with CF. There is evidence that physical fitness enhances the immune system and helps a person fight infection.  People who are physically fit have less severe and less frequent episode of infection.  Exercise may also be a valuable part of airway clearance techniques (ACT).

Recommendations: Thirty minutes of exercise daily.  Cardio/aerobic exercise, (stamina and endurance exercise) should be done on most days.  Examples include brisk walking, biking, jogging, swimming, and use of aerobic exercise equipment.  Strength building exercises, for older children and adults, should be done 2-3 times weekly. People may use stack weights systems at a fitness center or inexpensive dumbbell weights at home.  Your physical therapist can help you develop a personal exercise program for you.

Goals: Develop a working knowledge of types of exercise.  Achieve and maintain a state of aerobic fitness.  Achieve and maintain a better than average level of strength.

Medications and Treatments

Medications may be prescribed to maintain your health for a variety of reasons, including prevention of vitamin deficiency, enzyme replacement therapy, inhalers for asthmatic breathing, nebulizers for mucous clearance, and nebulized antibiotics.  Specific examples include vitamins, pancreatic enzymes, inhaled albuterol, inhaled corticosteroids (ICS), pulmozyme, hypertonic saline nebulized, nebulized tobramycin, nebulized colistin, and azithromycin (Zithromax).

Recommendations: Use medications as prescribed.

Goals: Use medications on a regular basis and understand the reason for the use of each medication and know the dosing.

CF-Related Diabetes

CF-related diabetes (CFRD) is common in people with CF.  It is detected by doing an oral glucose tolerance test (OGGT), which is recommended yearly beginning at age 10.  If CFRD is present and not treated, the patient may be more susceptible to infection and may have a poor immune response to fight infection.  You may also have difficulty maintaining an adequate weight if CFRD is not well controlled.

Recommendations: Get yearly Oral Glucose Tolerance Test (OGGT) beginning at 10. If you have CFRD, monitor your blood sugar before mealtime and as instructed, count your carbohydrates that you eat to adjust your insulin, and administer your insulin as prescribed.

Goals: Know how diabetes can affect your growth and your health, and proper treatment can improve your health and weight. Maintain adequate nutritional status.

Respiratory Infections/Exacerbations

Acute respiratory infections occur periodically in people with CF. If these infections are not treated quickly, they may trigger a lot of secretions (mucus) and pus, and eventually cause damage to the bronchial system.  Repeated infections over the years results in progressive loss of lung function.  We refer to acute infections as Pulmonary Exacerbations (PEx).  We developed a scoring system to help you and us detect and treat P Ex quickly and aggressively.  The CF centers whose patients have the best lung function treat P Ex often and quickly. We want you to call at the first sign of lung infection, so that we can treat you promptly. Symptoms to watch for include increase in sputum amount and/or color, increased cough or wheeze, shortness of breath, blood in the sputum, sinus congestion, feeling ill, low energy, fever, chills, sweats, poor appetite, weight loss, chest pain, poorly controlled sugar levels in those with CFRD.

Recommendations: Know the symptoms of Pulmonary Exacerbation (Pex), and call the center early if any new symptoms develop. At the first sign of a respiratory infection, remember to increase your airway clearance techniques (ACTs) to 4 times a day. We would like you to call if you have any new respiratory symptoms.

Goals: Identify and treat your Pulmonary Exacerbations early to maintain optimal lung function.