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Maintaining Your Health

Maintaining Your Health

While your doctors, nurses, and other care providers will help you to manage your disease, you are your most important advocate. You are the center of your treatment. 

People who take an active role in their own care frequently have better results in the long run. Here are a few steps you can take to make sure you maximize your care:

  • Speak up for yourself. If you have any concerns with your treatment or do not understand something about your disease, it is essential that you talk to your care providers. They want to make sure that you are able to maintain your health and will assist you with these issues.
  • Be prepared for your visits. Bring a list of your questions or concerns to each visit. Being prepared goes a long way in communicating with your providers – you cannot assist in the decision making process if you do not understand the factors involved.
  • Take notes during your visits. You will likely receive a significant amount of information during your health care visits and this can be overwhelming. Do not be afraid to take notes to help you remember important treatment issues later on.
  • Let your family and friends help. Emotional support is very important to maintaining your health. This is especially true if you are trying to modify your lifestyle. Quitting smoking, exercising more, or changing your diet is difficult; let those who care about you help you accomplish your goals. Do not be afraid to bring people who support you to your health appointments.
  • Contact advocacy and support groups. Reach out to community or national groups that assist people with pulmonary fibrosis. It can be very useful to find out how other people manage their disease - they may give you invaluable tips or tools that make it easier to manage your health. You can then pass your tips on to others to help support the community.
  • Stay informed. Continue to learn about PF. The more you know, the better you will be able to manage your disease and recognize when you need to access health services. Be advised that there is misinformation on the internet; to ensure your safety, make sure you access reliable sources of information, such as advocacy groups and respected scientific and medical sources.

YOUR CARE TEAM

Care Team Flow Chart

monitoring

How will my doctor monitor my PF?

Every doctor will have a different strategy to monitor your PF. Usually, doctors use a combination of the following to determine if your disease is stable or changing:

  1. Symptoms
  2. One of the most important signs that something has changed in your lungs is a change in the amount of breathlessness you experience while exerting yourself. A new or worsening cough can also be a sign that something in the lungs has changed. If you experience one of these problems, your doctor will first try to determine whether your symptoms are due to progression (new scar tissue in the lungs) or something else (an infection, a blood clot, a heart problem, etc.).

  3. Pulmonary Function Tests (PFTs)
  4. Doctors routinely order one or more of the following pulmonary function tests to monitor your PF:

    • Spirometry (spy-ROM-it-tree)
    • Lung volume measurement
    • Diffusing capacity (DLCO)

    Spirometry is performed by taking a deep breath in and then blowing out as hard and fast as you can through a mouthpiece connected to a machine. Spirometry measures how much air you can blow out (called the forced vital capacity or FVC) and how fast the air comes out. For most people with PF, monitoring the FVC can tell your doctor whether your disease is stable or progressing and whether your disease is mild, moderate, or severe. It’s easiest to track your FVC expressed as a percentage of normal. Normal is between 80 and 100% of the predicted value (which is based on your age, gender, height, and ethnicity). As more scar tissue develops in your lungs, your FVC will go down.

    Lung volume measurements can tell how much air your lungs can hold. Just like FVC, the results are expressed as a percentage of normal. The result that is usually tracked is called the total lung capacity (TLC). Normal is between 80 and 100% of the predicted value for age, gender, height, and ethnicity and decreases as more scar tissue develops in the lungs. This test is not performed as frequently as spirometry as it is more costly and time consuming and provides similar information as spirometry.

    Diffusing capacity (DLCO) measures how easy it is for gases (like oxygen) to move from your lungs into your bloodstream. Scar tissue makes the transfer of oxygen less efficient. Just like FVC and TLC, DLCO is expressed as a percentage of the normal value and goes down as more scar tissue develops in the lungs. Do not be surprised by a DLCO that is much lower than the FVC or TLC— that is expected in people living with PF.

  5. Walking Tests
  6. Doctors use a walking test to see how far you can walk (usually in six minutes) and how low your oxygen levels go when you walk. Sometimes the test is performed when you are using oxygen, and sometimes it is performed without oxygen. Doctors will compare your walking distance and your oxygen levels to your results from previous visits to determine whether your lung disease has progressed. Doctors often also use this test to determine whether or not you need to use supplemental oxygen while exerting yourself in your home and outdoors.

  7. HRCT Scans
  8. A high resolution computed tomography (HRCT) scan is a test involving X-rays that lets your doctor see a picture of your lungs. An HRCT scan gives a much clearer picture of your lungs than a regular chest X–ray or even a regular CT scan. An HRCT scan is a necessary test to diagnose PF. When repeated later on, comparing the change between two HRCT scans can tell your doctor if there is more scarring in the lungs. Some doctors perform multiple HRCT scans each year, some once per year, and some only when there is a change in your symptoms, spirometry, or walking test results.

    There are other tests doctors sometimes use to better understand your disease and its impact on your health, including arterial blood gas testing, cardiopulmonary exercise testing, and an echocardiogram. An arterial blood gas directly measures the amount of oxygen and carbon dioxide in your bloodstream and helps your doctor understand how well or poorly your lungs are functioning. Some forms of PF may also affect your heart. To investigate this possibility, your doctor might order an echocardiogram (or ultrasound) of the heart or a cardiopulmonary exercise test.

LIVING WITH PF

What is it like to live with pulmonary fibrosis?

First of all, you should know that each person’s experience living with PF is different. There is no “usual” experience. Some people have severe symptoms, and some have none at all. Some people live an almost normal life for many years, but others experience severe symptoms on a daily basis. The following discussion focuses on the common experiences that many, but not all, people living with PF report.

Early on, people with PF may have no symptoms at all. The most common symptoms are breathlessness during exertion, bothersome cough, and fatigue (tiredness). As the lungs develop more scar tissue, symptoms worsen. Shortness of breath initially occurs with exercise, but as the disease progresses patients may become breathless while taking part in everyday activities, such as showering, getting dressed, speaking on the phone, or even eating.

People living with PF share many of the same challenges faced by people living with other chronic diseases, such as

  • dealing with side effects from medications and other therapies;
  • distress resulting from the symptoms of your disease;
  • frustration resulting from the limits your disease places on your body;
  • inconvenience and cost of medical tests and frequent doctor visits, sometimes far from home; and
  • worrying about the “three big Fs”— your family, your finances, and your future.
In addition, people living with PF often need to use oxygen therapy, a burdensome but important treatment that may add inconvenience and frustration to your life.

Over time, most people with PF experience progression of their disease. When doctors use the term progression, they mean that there is more scar tissue in your lungs, which usually shows up on breathing tests (lower numbers or percentages on spirometry and diffusing tests). More scar tissue can make you feel more breathless than before. Often progression also means that you might need to start using oxygen, use more oxygen than before, or use oxygen for longer and longer periods during the day or night.

It is important for you to understand that everyone seems to experience his or her own pattern of progression. Remember, there is no “usual” experience. Some people remain stable for years before progression begins. Others seem to have rapid progression starting from the time they receive their diagnosis. Most people fall in between these two extremes, with periods of stability alternating with periods of progression.

Some people will develop more rapid progression over days or weeks— this is called an “acute exacerbation.” This rapid progression can include increasing breathlessness and cough, the need for more oxygen, and consideration of hospitalization. Sometimes there is an obvious explanation, such as pneumonia (a lung infection), heart problems, or blood clots in the lung (a pulmonary embolism). But in many cases, things are worse without an obvious explanation— this is called an “acute exacerbation,” which is a medical term for “things got worse all of a sudden.”

Doctors can recognize an acute exacerbation based on your symptoms, oxygen levels, CT scan results, and other tests. Usually doctors will find that your lungs have “ground-glass” on your CT scan, which means that some parts of your lungs appear grey or hazy. This groundglass effect can result from infection, fluid build up, inflammation, or microscopic injury to the lungs.

An acute exacerbation can be a serious event, and for many it can be fatal within days or weeks. When an acute exacerbation is severe and the lungs are failing to put enough oxygen into the bloodstream, doctors often bring up the possibility of being treated with a mechanical ventilator (a “breathing machine”) attached to a plastic tube that is placed through your mouth into your windpipe. Most people with PF treated with a mechanical ventilator do not survive very long. It is very important that you discuss this possibility with your doctors before this kind of treatment is needed, so that you can make your wishes known regarding this kind of invasive therapy. While this is a sensitive topic, it is important to discuss with your decision maker to ensure your wishes are honored as your health declines.

What is the life expectancy for someone living with PF?
Pulmonary fibrosis, particularly idiopathic pulmonary fibrosis (IPF), is a serious, life-limiting illness. It is very important to understand what type of PF you have as different forms of PF affect patients in different ways. It is difficult to predict how long you will live. An important goal is to learn to live with this diagnosis despite its serious nature.

No one can tell you how long you will live with PF. You may have read or been told that, on average, people with IPF live three, four, or five years, but this statistic only applies to people diagnosed with idiopathic pulmonary fibrosis, and the statistics for the many other kinds of PF can be different and are often better. Also, there is a lot of variation in how long people with PF live. If four years were the average, that would mean that half of people living with PF live longer than four years. Some live much longer.

Your doctors may be able to give you more information about their expectations (another way of saying “on average”) of what’s to come for you. For example, people with very low breathing tests, rapidly progressing disease, and those who require a lot of oxygen do more poorly than others.

If your doctor believes you are at risk to die in the next six months, they may recommend that you receive hospice care, which is intended to help people who are dying have peace, comfort, and dignity. People receiving hospice care receive treatments to control pain and other symptoms. Hospice care also provides support to families. Care may be provided at a hospice center, but can also be done in nursing facilities, hospitals, or often at home. You can learn more at:

lifestyle

There are a variety of things that you can do to maintain or improve your quality of life while living with pulmonary fibrosis. The National Institutes of Health and the Mayo Clinic offer a variety of recommendations for patients, several of which are referenced in this section.

  • Stay in Shape. One of the most damaging consequence of lung disease and its sensation of breathlessness is the development of an inactive lifestyle. For many with PF, activities of daily living, like bathing and dressing, can create overwhelming fatigue.  "Air hunger" can create panic attacks, and produce negative psychological effects.  People with PF sometimes limit their physical activities in a n attempt to avoid breathlessness. The lack of exercise works against you; inactivity weakens your muscles making even the simplest daily activities even more difficult. Regular exercise can strengthen your muscles and makes them more resistant to fatigue. With practice and training you can learn to perform task in a more efficient manner. By being more efficient you need less oxygen for the same amount of work. The result is that you may find that you have more energy to accomplish daily tasks and that you are less short of breath. A formal rehabilitation program (pulmonary rehabilitation) is preferred because it allows for observation during exercise and it can be tailored to your specific needs. 
  • Eat Well.  A healthy diet includes a variety of fruits, vegetables, and whole grains. It also includes lean meats, poultry, fish , beans, and fat-free or low-fat dairy products. A healthy diet is low in saturated fat, trans fat, cholesterol, sodium (salt), and added sugar. Eating smaller, more frequent meals may prevent stomach fullness that make it harder to breathe. If you need help with your diet, ask your doctor to arrange for a dietician to work with you. A nutritionally rich diet that contains adequate calories is essential. A dietician can give you further guidelines for healthy eating.
  • Get Plenty of Rest. Getting at least eight hours of sleep every night can boost your immune system and sense of well-being.
  • Stop Tobacco Use. If you are still smoking, the most important thing you can do is to STOP SMOKING. Due to the addictive nature of tobacco, this can be difficult. Seek the help of your physician to find a smoking cessation class or other beneficial methods to help you stop smoking. Second-hand smoke can be as harmful to you as if you were smoking yourself. Ask your family and friends to refrain from smoking around you.
  • Learn and Practice Relaxation Techniques. Learning relaxation techniques can help you manage the anxiety that often accompanies breathlessness. Joining a support ground and/or seeing a counselor can help you cope with your feelings and the anxiety and depression that are common in people with PF. The restriction on activity due to shortness of breath may lead to isolating oneself from family and friends, adding to the depression.
  • Join a Support Group. Just knowing that there is someone out there that knows how you feel is comforting. Share ideas, share fears, and share joys.  
  • Participate in Your Health Care. Remember you are a part of a health care team that includes your doctors and nurses. They will be asking you a lot of questions. As a member of that team you have a responsibility to do your part. Be prepared to ask your own questions. Be a participant. Bring someone with you to each appointment and prepare a list of questions to be answered by your physician during your visit.
  • Help Others with PF. Consider participating in the Pulmonary Fibrosis Foundation's advocacy program. You may gain strength in knowing that you are helping future patients and researchers by advocating for the pulmonary fibrosis community.
  • Keep a Positive Attitude. Actively participating in the management of your disease is greatly enhanced by a positive attitude. A positive attitude can help you and your loved ones cope with the disease.

FOR THE CAREGIVER

I am a caregiver for someone with pulmonary fibrosis. What do I need to know?

Whether you are spouse, partner, child, or friend, caring for someone through a serious illness is a challenge. There are only two rules.

  1. Do what needs to be done each day to take care of your loved one.
  2. Take care of yourself. The day-to-day life of a caregiver can be physically and emotionally draining.
Here are some suggestions that may help you be an effective caregiver.

Be actively involved in your loved one’s medical treatment.
  • The diagnostic and treatment journey for patients with PF can be confusing and difficult. By participating and helping them in their journey, you may alleviate anxiety and frustration for both you and your loved one.
  • Bring a list of questions that you want to ask your medical team.
  • Go to doctor appointments and write everything down.
  • Be prepared to advocate for the patient if you feel it is necessary.

Know which medications your loved one takes.
  • Keep a current list of all medications and supplements.
  • Print out copies of the current medication list to hand to medical staff to copy for their records.
Know how to operate any medical equipment used by your loved one.
  • A handheld pulse oximeter is an inexpensive device that measures oxygen levels on the tip of the finger.
  • Many PF patients need supplemental oxygen. Know how to operate the oxygen concentrator and tanks in case of an emergency.
  • Plan for adequate oxygen when away from home.
Let your loved one do everything he or she can for as long as possible.
Don’t take away a PF patient’s independence any sooner than necessary,
but also be prepared to take over responsibility for things your loved
one may no longer be able to do now or in the future, such as
  • driving;
  • ordering and dispensing medications;
  • paying bills, managing money, and filing tax returns; or
  • buying groceries and cooking meals.
Help your loved one maintain a healthy diet and exercise program.

Learn about Advanced Directives (Living Wills & Medical Power of Attorney), Physician Orders for Life-Sustaining Therapy (POLST), and Palliative Care options, even long before they seem to be needed.
  • These documents should be kept in a place where they are easy for you to find and also on file with your local hospital.
  • It's important to select a person to be your decision maker when you are no longer able to make decisions, and make that person aware of your wishes and those of your loved one with PF so that he or she can honor those wishes.
Learn more at Help prevent respiratory infections.
  • Everyone older than six months of age in the home needs a flu vaccine every year, including you.
  • Adults over age 65 need to be vaccinated against pneumonia. Younger adults with certain health conditions may also need to be vaccinated against pneumonia.
  • Use hand sanitizer frequently.
  • Notify your loved one’s primary care provider immediately at the onset of a cold, cough, or flu.
  • Keep your loved one away from large crowds.
  • Know when to say NO to visits from family or friends; to sitting in a hospital emergency waiting room with sick people; or anything else you feel is unsafe for your patient. You are the best judge of what is OK or not.
Take care of yourself.
  • Be willing to ask for and accept help.
  • Keep up with your own medical appointments.
  • Find time for yourself. Get your hair cut, schedule a massage, go to a movie, or have lunch with friends.
  • Find someone you can talk to when things are hard.

questions for health care providers

Your health care providers are partners in the treatment of PF. It is important that you have a firm understanding of your disease and how you should care for it. Ask your providers about anything relating to your treatment that you do not fully understand. Here are a few questions that may help you manage your care: 

  • How will PF impact what I can and cannot do? You are probably already aware of the limits of what you can and cannot do in your everyday routine. There may be activities that you do not regularly engage in which may be impacted by your pulmonary fibrosis. This can include traveling by air or visiting high-altitude places. Your care providers can help you identify what activities may present some challenges. 
  • What should I do and whom should I contact if I have any problems with my PF? Having an action plan in place in case of problems is a must for anyone with a chronic disease. Ask about where you should go, whom you should call, and what you should do during nights and weekends if you have a problem with your pulmonary fibrosis. You should also know whom to contact if you have any questions and concerns about your current care, including your treatment. 
  • When is the right time to start or switch treatment? The decision to start therapy depends on your health and desires. It should be made in conjunction with your health care providers and those who will help support you, such as family and friends. Once you have started therapy, you may need to change it as your disease and needs evolve. When your treatment is no longer working well, it is time to discuss other treatment options. However, even when treatment is working there may be better options for your needs. Asking about what options are available is a good way to find out what changes are happening in the treatment of PF and ensures that you get the best treatment for both your disease and lifestyle. 
  • Will my treatment interfere with other medications I am taking? It is very important that your care providers know all of the medications that you take regularly — both prescribed and over-the-counter — as well as any vitamins or other nutritional supplements. Asking your providers about drug interactions helps ensure that all of your medications are reviewed for potentially dangerous interactions.
  • Are there resources that can help me lead a healthier lifestyle? Improving your lifestyle is a critical component of improving quality of life. However, changing things like how much you exercise, your diet, and smoking habits can be very difficult. Care providers may be able to direct you toward resources that can help you modify your lifestyle and adopt healthier behaviors. These can include support groups, dieticians, and personal trainers. Showing an interest in adopting healthier behaviors is the first step in accomplishing these changes. 
  • Is a clinical trial right for me?  Clinical trials are a potential resource for patients who may not have many good treatment options. Whether you should enroll in a clinical trial depends on many factors, including what trials are available in your area, whether you are motivated to participate, and your current condition. If you are interested in participating in a clinical trial, talk with your care providers about options; they will help you determine if a clinical trial is right for you.

palliative and end of life care

The purpose of both palliative and hospice care is maintaining a patient’s comfort and quality of life. While this care does not specifically treat pulmonary fibrosis, it is designed to improve the quality of life for anyone dealing with a chronic illness. 

PALLIATIVE CARE

Using a multidisciplinary approach, palliative care can involve physical, psychosocial, and spiritual factors in the treatment approach. Teams may include physicians, pharmacists, nurses, religious leaders, social workers, psychologists, and other health care professionals. These teams focus on concrete goals including relief from breathlessness and other distressing symptoms, spiritual care, development of support systems, and encouraging an active lifestyle.

HOSPICE CARE

Hospice care is a type of end-of-life care, intended to help people who are dying find peace, comfort, and dignity. It is generally reserved for those who have less than six months to live and often includes treatments to control breathlessness and other symptoms to maintain comfort. Hospice care also provides support to families. Care can be provided at a hospice center but can also be done in nursing facilities, hospitals, or at home.

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