Living With Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disease that causes thick mucus to build up in your lungs. It can also affect other parts of your body, such as the pancreas and intestines. When the lungs get affected:

• It will be hard for you to breathe. You may cough and wheeze.
• You may get sick more often as a result of lung infections. You may need to stay in the hospital.

There is no cure for CF, but there are treatments and ways you can manage your symptoms and improve your quality of life.

How to manage lifestyle changes

Managing stress

Living with CF can be stressful. Managing stress can help you manage CF. Too much stress can:

• Worsen lung problems.
• Increase your risk for infection.
• Cause depression or anxiety.
• Cause poor appetite and poor nutrition.

To cope with stress:

• Exercise every day. Ask your health care team to suggest activities that are best for you.
• Get 8–10 hours of sleep each night.
• Stay positive about your health. Try to accept that you cannot control your CF perfectly, but you can improve your symptoms.
• Breathe slowly and deeply when you need to stay calm.
• Spend time doing the things that you enjoy, such as being outdoors or spending time with friends and people who make you laugh.
• Avoid caffeine, drugs, and alcohol.
• Eat a nutritious, balanced diet, and make sure you eat enough calories.
• Relax each day. Use meditation, yoga, music, or other relaxing activities.
• Express yourself through journal writing, art projects, crafting, or playing music.

Medicines

Your health care provider may give you certain medicines and nutritional supplements to help relieve your symptoms. Avoid using alcohol and other substances that may prevent your medicines from working properly.

It is also important to:

• Talk with your health care provider or pharmacist about all the medicines that you are taking.
  • Tell your health care provider about the side effects of your medicines.
  • Ask what medicines are safe to take together.
• Take part in all treatment sessions (shared decision-making). Shared decision-making should be part of your treatment plan.

Relationships

Living with cystic fibrosis can make it hard for you to join family and friends for occasions and activities. When this happens:

• Stay positive and keep up with your treatment or health plan.
• Talk to your friends and family about your disease and how it affects you. Having strong support from family and friends is very important. You may not know how to react to their questions. Here are some suggestions:
  • Tell people when you feel comfortable with them. You do not have to tell people if you do not want to.
  • Explain that CF is a long-term (chronic) disease with no cure.
  • Explain that you sometimes will cough, wheeze, and have trouble breathing.
  • Explain that you need daily treatment (including medicines, inhalers and physical therapies) to clear your airways and stay healthy.
  • Explain that you may not be able to be around people when they are sick, or if they smoke because it is very dangerous for you if you get an infection.
• Talk with other people who are living with cystic fibrosis. Consider joining a support group for adults with CF.

How to recognize changes in your condition

Signs that your CF is being well-managed include:

• Being able to breathe more easily and deeply for longer periods of time.

Signs that your CF may be getting worse include:

• More trouble breathing.
• Depression or anxiety.
• Frequent infections, such as pneumonia.
• Losing weight without trying.
• Being very tired for a long time.

Follow these instructions at home:

• Take over-the-counter and prescription medicines only as told by your health care provider.
• Follow instructions from your health care provider about eating and drinking.
• Drink plenty of fluids throughout the day.
• Do daily therapies as told by your health care provider. You may need to do Airway Clearance Therapy (ACT) to loosen up the mucous in the lungs.
• Make sure you and all people in your household wash hands often with soap and water. This lowers your risk for infection. If soap and water are not available, use hand sanitizer.
• Do not use any products that contain nicotine or tobacco, such as cigarettes and e-cigarettes. If you need help quitting, ask your health care provider.

Where to find support

• Join a support group. There are many Cystic Fibrosis Foundation Care Centers that work with patients living with cystic fibrosis.
• Try to get a generic form of the medicines you are taking. This may be less expensive. Some makers of prescription medicines also offer help to patients who cannot afford the medicines they need.

Where to find more information

• Cystic Fibrosis Foundation: www.cff.org  
• Cystic Fibrosis Research Inc, support groups: www.cfri.org  
• American Lung Association: www.lung.org  

Contact a health care provider if you:

• Cannot eat or drink without vomiting.
• Lose weight without trying.
• Feel tired or do not have energy.
• Are constipated.
• Have diarrhea.
• Have a fever.

Get help right away if you:

• Have chest pain.
• Have problems breathing.
• Have a fever of over 101.4° F (38.5° C), especially with cough or difficulty breathing.
• Are dizzy or you faint.
• Have a lot more mucus than usual.
• Have new or severe pain in your abdomen.
• Have yellowing skin or eyes (jaundice).
• Vomit dark green or yellow fluid.

Summary

• Cystic fibrosis (CF) is a chronic, inherited disease that causes thick mucus to build up in your lungs and other parts of your body.
• To manage your condition, take your medicines as told by your health care provider. Lower stress by exercising, getting enough sleep, and eating a healthy diet. Follow your health care plan closely.
• Avoid caffeine, drugs, alcohol, and tobacco.
• Educate your family and friends about CF. Having strong support from family and friends is very important.
• Consider joining a support group for adults with CF.