Moving forward. with idiopathic pulmonary fibrosis (IPF) A guide to your journey ahead

Transcription

1 Moving forward with idiopathic pulmonary fibrosis (IPF) A guide to your journey ahead

2 3 Table of contents Now that you know more about your disease, you can begin to learn how to live the best life you can with IPF. Know what to expect. Prepare yourself for the day-to-day challenges. And, most importantly, take an active role in your care. This brochure will help you understand how IPF affects your body and what you can do to fight back against it. Use it as a guide. Learn all you can and start talking with your doctor about how to best manage IPF. Managing beyond the lung IPF and its effects beyond the lung... 4 A holistic approach to care... 6 Moving forward Preparing for what is ahead... 8 Dealing with breathlessness...10 Feeling tired or fatigued...11 Having a constant cough Having trouble sleeping Dealing with the emotional impact Having a comorbid condition Learning more about options for your care plan Antifibrotic medicines...20 Oxygen therapy...22 Pulmonary rehabilitation...26 Relaxation techniques...28 Breathing techniques...30 Living an active and healthy lifestyle...32 Lung transplant...34 Advanced care planning...36 Know your IPF care team...38 Questions for your doctor...40

3 4 5 IPF affects more than just your lungs Effects of IPF IPF causes scarring of the lung tissue. This reduces how well your lungs work and the amount of oxygen in your body, making it harder to breathe. But the effects of IPF do not stop at your lungs. Having IPF can lead to other negative effects on your body, mind, and spirit. That is why you need to take a holistic approach to managing your IPF. Breathlessness Coughing Depression Reduced physical activity It is important to tell your doctor about how you are feeling physically and emotionally. There are things he or she can do to help. Limited social activity Nutritional issues Muscle loss

4 6 7 Think about a holistic approach to IPF care This means you need to consider more than just the physical symptoms of IPF. The mental and social factors need to be addressed, too. Here is an overview of some available options for your care plan. Targeting the disease Antifibrotic medicines are available that can help slow down how fast your disease worsens. Ask your doctor about the treatment options. Improving physical and emotional symptoms of IPF Oxygen therapy can help reduce shortness of breath and improve your ability to perform everyday tasks. Learn more on pages Managing comorbid conditions Your doctor can help improve symptoms that are caused by a different condition that is not IPF. Living an active and healthy lifestyle Maintaining your overall health is important. Learn more on pages Talking about lung transplant This may or may not be something that is right for you. Talk to your doctor and learn more on page 34. Pulmonary rehabilitation can help improve your quality of life and reduce symptoms of IPF, like breathlessness and depression. Learn more on pages A psychiatrist, psychologist, or support group can help you cope if you have feelings of anxiety and depression. Relaxation and breathing techniques can help improve feelings of stress and anxiety as well as help you manage breathlessness better. Learn more on pages Comorbid condition: when you have 2 chronic conditions at the same time. For example, if you have IPF and heart disease, then heart disease would be a comorbid condition.

5 8 9 Preparing for what is ahead Knowing how IPF will affect your life gives you the power to take action. You can positively impact your life by being proactive about therapy options and changes in your lifestyle. This can help make dealing with IPF easier. Read on to learn more. Know what to expect. Know what to do. Know you have the power to take action. Information is crucial.we live much more comfortably if we understand things, understand the treatment, understand the disease, if we get the answers to questions we have. Advice from a doctor specialized in IPF, France

6 10 11 Dealing with breathlessness Feeling tired, or fatigued What to expect: What to expect: Shortness of breath affects your ability to do daily activities. You may feel like you need to stop often to catch your breath. At first, you may only feel breathless with mild physical activity, like walking or going upstairs. But, as your breathlessness gets worse, it will become harder to complete even simple tasks, like bathing, dressing, or talking on the phone. Fatigue can be overwhelming. Some patients with IPF often feel completely exhausted during parts of the day. It can affect your ability to complete daily tasks or even socialize. For example, when you have a task to do, you may have to take breaks so that you can rest up before continuing. This feeling is different from feeling breathless and having to catch your breath. Breathlessness Activity level Breathlessness and fatigue can make physical activity hard, but it is important to stay active. Learn how you can better cope on the next page. As your activity level decreases, you will begin to use your muscles less and less. This leads to the loss of muscle strength. Less muscle strength means that you will feel more tired and physical activity will be even harder. 23% of patients with IPF felt that the hardest thing about living with IPF was not being able to do the things they used to do before they had IPF.

7 12 13 Dealing with breathlessness and fatigue (cont d) What you can do: 1. Stay active 4. Breathing and relaxation techniques Sometimes, breathlessness and fatigue can make people avoid physical activity. But, it is important to stay active. Learn how to slow down and take breaks. 2. Pulmonary rehabilitation Supervised exercise can help you build up endurance and lessen breathlessness. Learn more on page Oxygen therapy Your doctor may prescribe oxygen therapy. This can make it easier to stay active and can lessen the feeling of breathlessness. Learn more on pages Breathlessness can be a very frightening and distressing feeling. It may cause you to have anxiety and you may start to panic. This will just make it worse. Breathing techniques can help you in this situation to regain control over your breathing. Relaxation techniques can be used to lessen anxiety. Learn more on pages Smaller, more frequent meals When your stomach is too full, it can make it harder to breathe. 6. Maintain a healthy weight Being overweight can put increased pressure on the lungs, which makes it harder to breathe and more difficult to stay active. As far as I m concerned, life doesn t end with this.we still live a good life. We dine out, we travel to hotels, we enjoy life, and we socialize generally. I d say don t give up, don t give up. -Patient with IPF, UK

8 14 15 Having a constant cough Having trouble sleeping What to expect: What to expect: Some people with IPF have a dry and hacking cough. You may feel the need to cough constantly without ever feeling better. You may feel embarrassed by your cough. You may even avoid certain social situations. But, it is important to stay connected to friends, family, and activities that you enjoy. What you can do: 1. Pulmonary rehabilitation These programs offer support and tips for cough management. 2. Medicine There are treatments available that may help to reduce your cough. Ask your doctor what the options are. 3. Comorbid conditions Cough can be worsened by a comorbid condition, such as gastroesophageal reflux disease (GERD). It is possible that these conditions can be treated. Make sure you inform your doctor about any other conditions or diseases you may have. Having cough makes it tough to sleep. You may even be woken up in the middle of the night because of it. Not sleeping can have a negative effect on your emotional well-being. Also, it is common for patients with IPF to have a condition called sleep apnea. This is a disorder where there are many pauses in breathing during sleep. What you can do: 1. Oxygen therapy If you have sleep apnea, your doctor may prescribe you oxygen therapy during the night. This may help keep your oxygen levels up. 2. Manage your cough If cough is keeping you awake at night, make sure you talk with your doctor to find ways to manage it. I coughed night and day, non-stop. It wouldn t go away. And that cough made people stay away from me. You start coughing, the first thing that comes to mind is, she has tuberculosis. When people stay away from you, it makes you feel bad. -Patient with IPF, Brazil

9 16 17 Dealing with the emotional impact What to expect: Dealing with IPF can take an emotional toll. Many fear losing the ability to function. They worry about having to rely on others. Others may isolate themselves from family and friends because they are embarrassed. This can lead to depression. 1 in every 4 patients with IPF has clinical depression What you can do: 1. Social activities and hobbies Keeping up with friends and family and enjoying the things you love to do can have a positive impact on your emotional state. 2. Support groups Talking to other people who are going through the same thing can help you learn how to cope better. 3. Relaxation techniques If you or your caregiver notice any of the following symptoms of depression, talk with your doctor right away: Daily sadness Loss of interest in friends and hobbies Irritability and anger Sleep and appetite problems These can help you deal with the everyday stress of IPF, while relaxing your mind and body. 4. Pulmonary rehabilitation It has been shown to help reduce anxiety and depression. 5. Counseling and/or antidepressive medicine Being depressed can rob you of your emotional strength to fight IPF to your fullest potential. Depression is a disease that needs attention. You may need to speak to a professional counselor. He or she may prescribe you a medicine that can help lessen your symptoms of depression.

10 18 19 Having a comorbid condition What to expect: Patients with IPF may often have other health problems, called comorbidities. Comorbidities are when you have 2 or more chronic conditions at the same time. Sometimes the comorbid condition can be the cause of a symptom or could worsen a symptom of IPF. GERD This is a stomach condition that causes acid from the stomach to come up to the throat, leading to heartburn. Common comorbid conditions in patients with IPF Gastroesophageal reflux disease (GERD), or acid reflux disease Lung conditions (chronic obstructive pulmonary disease [COPD], emphysema, lung cancer) Heart and vascular conditions (pulmonary hypertension, heart disease) Depression Sleep apnea What you can do: Talk to your doctor He or she will check to see if you have any comorbid conditions that need to be managed. Make sure you tell your doctor if you currently have a condition in addition to IPF, and always tell your doctor if you have any new symptoms. COPD Emphysema Pulmonary hypertension Sleep apnea A lung disease that causes the flow of air into and out of the lungs to be obstructed. This makes it hard to breathe. One of many diseases known as COPD. It damages the air sacs in the lungs and makes it hard to breathe. A type of high blood pressure that affects the arteries in the lungs and the right side of the heart. A disorder where there are many pauses in breathing during sleep.

11 20 21 Start to learn more about the available therapy options for IPF Talk to your doctor and ask questions. Be sure to be clear about your preferences and your goals. Together, you can decide what might be the right plan for you. Antifibrotic medicines In many countries, there are 2 antifibrotic medicines available to treat IPF nintedanib and pirfenidone. As with any medication, you should talk to your doctor to find out if either one is right for you. By learning more about available options, you can take an active role in your care plan.

12 22 23 Oxygen therapy Oxygen therapy is used to keep the level of oxygen in the blood above a certain level. This can make breathing easier for patients with IPF. It can also help patients function better and stay more active. Types of oxygen delivery Your doctor will assess when oxygen therapy is needed and what type of oxygen should be used Nasal cannula Two small plastic tubes, or prongs, that fit within both of your nostrils At first, you may only need oxygen to do physical activity, such as gardening or taking a walk. As the disease gets worse, oxygen therapy may be needed throughout the day, even when you are at rest. Face mask Fits over your nose and mouth and straps onto your head Oxygen concentrator A small machine that removes all the other gases from the air except for oxygen. Then you breathe in the oxygen through a nasal cannula or a mask For a long time, I had problems with [oxygen] in my nose. I was pushing it in front of me and I was avoiding going to town, but now I don t mind anymore. It s a matter of vanity. I know I feel better with the oxygen, and I don t want to go without it. -Patient with IPF, Germany Compressed gas Liquid oxygen Oxygen is stored under pressure in different sized tanks. Some of the tanks are small enough to carry around. They can be on a small wheeled cart or in a shoulder bag or backpack Liquid oxygen is delivered to your home in a large container. You then refill small tanks with oxygen from the large container Be sure to follow the instructions your home equipment provider gives you.

13 24 25 Oxygen therapy (cont d) Safety precautions Side effects of oxygen When using oxygen, there are certain safety measures you ll need to take. Remember, oxygen is flammable, so it can easily cause a fire. Install smoke detectors in every room Some people using oxygen therapy may get a dry or bloody nose or skin irritation. There are ways to help reduce these effects. Nasal lubricants and humidifiers can moisten the nose to prevent bleeding. Soft tubing or foam padding can reduce skin irritation. Talk to your doctor if you have any side effects from oxygen therapy. Traveling with oxygen Keep fire extinguishers in an accessible place Store the oxygen tank in a clean, dry place at least 6 feet away from heat, electricity, or fire If you travel, it is crucial to plan ahead. Depending on how you are traveling by air, car, or boat you will need to make certain arrangements. For example, there may be required documentation, additional charges, and special procedures for your equipment. But, it is most important to talk to your doctor to see if you are able to travel. Do not smoke near an oxygen tank For more tips and helpful traveling checklists, visit lifewithipf.com/life-with-ipf/travelling_with_ipf/flying.html

14 26 27 Pulmonary rehabilitation Pulmonary rehabilitation is a program of exercise, education, and support to help improve the physical condition of patients with IPF. A team of specialists plan programs that are designed for individual patients based on their needs, level of activity, and goals. Benefits of pulmonary rehabilitation for patients with IPF Decrease the symptoms of IPF (such as breathlessness and tiredness) Help patients function better in daily living Reduce anxiety and depression Here is a list of what pulmonary rehabilitation involves: Exercise training: With supervised training, you can learn to perform tasks in a way that is more efficient. The result is that you may find that you have more energy to accomplish daily tasks and that you are less short of breath. Breathing techniques: These are used to reduce and control breathlessness. Education about IPF: Information on IPF, how it will affect your life, dealing with symptoms, and the medications you are taking. Other components of a program may include emotional support, nutrition classes, stress reduction, and help with stopping smoking. Amongst the important strategies for maintaining patients quality of life, there is rehabilitation. Even if it has no direct effect on pulmonary fibrosis, it will have an effect on how patients feel about their breathlessness and will help them continue to do everyday exercises, and therefore help maintain their quality of life. -Advice from a doctor specialized in IPF, Italy

15 28 29 Relaxation techniques Living with a chronic condition can be stressful. Sometimes, taking a little break can help. So here s something for you, your loved ones, or anyone who needs to relax for a short while. Have 60 seconds? Sit down in a comfortable chair. Take a few slow, deep breaths and quietly say to yourself, I am as you breathe in. Then say, at peace as you breathe out. Repeat this slowly 2 or 3 times. You should start to feel your body relax into the support of the chair. Or 2 minutes? For this 2-minute mini-relaxation exercise, you will slowly count down from the number 10 to 0, inhaling and exhaling. So be sure that you re sitting in a comfortable chair or couch. To begin, breathe in deeply saying, 10 to yourself. Then breathe out slowly. On your next breath, say 9, and continue counting down to 0. When you reach 0, you should feel more relaxed. While relaxing can help relieve stress, so can keeping up with some of your favorite activities. Like gardening? Keep tending to your plants. Like reading the newspaper? Keep reading. And if you like going for a morning walk, walk as far as you can.

16 30 31 Breathing techniques When you are short of breath, practicing breathing techniques can help. Sometimes, when a person feels breathless, they get anxious, which then makes the breathlessness worse. They can begin to hyperventilate. Breathing techniques can help a person return their breathing to normal. Belly breathing best used when you re feeling rested and relaxed, and while sitting back or lying down Pursed-lips breathing helps you control breathlessness if you are beginning to hyperventilate This technique is best used if you are becoming short of breath and are beginning to feel anxious. It can help return your breathing to normal. 1. Place one hand on your chest and the other on your belly. When you begin to breathe, you should feel the hand that is on your belly move, but the hand that is on your chest should remain as still as possible. 1. Breathe in through your nose (as if you are smelling something) for about 2 seconds. 2. Breathe in slowly through your nose. Your belly should move out toward your hand. 2. Pucker your lips like you re getting ready to blow out candles on a birthday cake. 3. Breathe out very slowly through pursed lips for about 4 seconds. 3. As you breathe out slowly through pursed lips, gently press on your belly. This will push up on your diaphragm to help get your air out. 4. Repeat. 4. Repeat.

17 32 33 Tips for living an active and healthy lifestyle Stop smoking. If you are still smoking, the most important thing you can do is to stop. Secondhand smoke is also harmful ask your family and friends to refrain from smoking around you. Stay active and exercise. It can help you maintain your physical and mental health and help relieve stress. There are lots of ways to stay active walking, strengthening exercises, even keeping up with your hobbies. Eat well. This means eating a well-balanced, healthy diet. Eating smaller, more frequent meals may prevent stomach fullness that can make it harder to breathe. Get plenty of rest. Getting at least 8 hours of sleep every night can boost your immune system and sense of well-being. Enjoy friends, family, and hobbies. Keeping up with social activities and hobbies can help you stay positive. Practice relaxation techniques. When you are physically and emotionally relaxed, you avoid excessive oxygen consumption caused by tension of overworked muscles. Join a support group. Learn how others cope and what has worked for them. Share experiences, fears, and positive support. Keep a positive attitude. A positive attitude can help you and your loved ones cope with your disease. Get vaccinated. Stay on track with recommended vaccinations, such as for flu and pneumonia, to help avoid getting sick. When I go to the hospital, there are a lot of people who are just starting and they don t know how they will face it. I say they have to face it like anything else. They can look to me, I am all right and I have had it for 4 or 5 years. -Patient with IPF, Spain

18 34 35 Lung transplant Lung transplantation is a surgery to remove one or both diseased lungs and replace them with healthy lungs. The right patients for lung transplant Lung transplant has the potential to make patients live longer, but not everyone with IPF is a candidate and there are risks. Only your doctor can assess if you are a candidate for lung transplant. If you are, you will be put on a waiting list. It may take a long time for a lung that is right for you to become available. As you may know, lung transplantation comes with risks that should be discussed with your doctor.

19 36 37 Advanced care planning It may be hard. It means admitting that your health is declining. But having a clear idea of your wishes can bring peace of mind to you and your caregiver. It is important to understand and agree about which steps to take. Then have your wishes clearly stated in a formal, legal document called an advance directive. Advance directive Any preferences for medical care including: Pain medication Life-sustaining mechanical support (such as a ventilator). These are machines that breathe for you if you are not able to on your own Do not resuscitate orders, which mean that you would decline cardiopulmonary resuscitation Tube feeding and hydration. This provides nutrition and water if you cannot eat or drink on your own Organ and tissue donation after you pass away Durable power of attorney for healthcare Determine your healthcare proxy. This person would make healthcare decisions for you if you were no longer able to make them yourself Involve your doctor in your decision-making process Ask your doctor to explain treatments and procedures that you don t understand. And give your doctor a copy of your completed directives. You can change your mind Your choices may change over time or if your situation changes. And that s alright. You can change your advance directive at any time. Make sure you discuss changes with your doctor and your healthcare proxy, and then update your documents. You should discuss your preferences with your caregiver and/or healthcare proxy at regular intervals. Cardiopulmonary resuscitation: A lifesaving technique that keeps the blood and oxygen flowing when a person s heartbeat and breathing have stopped.

20 38 39 Know your IPF care team My IPF care team You may need to see many doctors for the best care possible. It is important that you keep their contact information in a convenient place. You may want to bring this along to your doctor s visits. Keep several copies of the following and have them with you on any doctor visits: The medications you are taking Any known allergies Advance directives IPF specialist Family doctor Pharmacist Radiologist Pulmonary rehab center Respiratory therapist Occupational therapist Oxygen equipment provider Nutritionist Psychologist Social worker Support groups Other doctors* * Other specialty doctors may include those that manage a comorbid condition (for example, a cardiologist or sleep specialist).

21 Important questions for your doctor when you are living with IPF Asking specific questions at your doctor visits can help you better understand your disease and help you make more informed decisions about your care. Here is a helpful list of questions for a person who is living with IPF. 1. How has my condition changed since the last time I saw you? 2. What is the best approach for managing my IPF? 3. Should I take treatments that can slow down the progression of my disease right away? 4. When is the best time to start therapy? 5. How will I know if a treatment is working? 6. When do I need oxygen therapy? 7. Is pulmonary rehabilitation right for me? 8. Am I a candidate for lung transplant? Jot down additional questions you may have here: Boehringer Ingelheim International GmbH. All rights reserved. October 2016