Living with Idiopathic Pulmonary Fibrosis

There are a variety of ways that IPF suffers can maintain or improve their quality of life when living with IPF.

  • Stay in shape: The most damaging consequence of lung disease and its sensation of “breathlessness” is the development of an inactive life style.  For many patients, activities of daily living like bathing and dressing can cause overwhelming fatigue. Air hunger can cause panic attacks and create distress.  People with chronic respiratory problems sometimes limit their physical activities in an attempt to avoid shortness of breath.  However, a lack of exercise works against you.  Inactivity weakens your muscles and they become less efficient. Through regular exercise muscles become stronger and more resistant to fatigue. With practice and training you can learn to perform tasks in a more efficient manner and hence require less oxygen. A formal pulmonary rehabilitation programme may be 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 fruit, vegetable and whole grains. It also includes lean meats, poultry, fish, beans, and fat free or low fat milk or milk products.  Eating smaller but more frequent meals may relieve stomach fullness, which can make it hard to breathe. As the disease progresses, breathlessness can make swallowing difficult and it may be necessary to eat a very soft diet in small but frequent  portions. Dietitians can give further guidelines for healthy eating.
  • Get plenty of rest: Getting eight hours of good quality rest every night can boost your immune system and sense of well-being.
  • Stop smoking: Avoiding environmental irritants, like cigarette smoke, is a good way to prevent further damage to your lungs. Tobacco smoking is addictive; seek help from your GP. Second-hand smoke can be as harmful as smoking yourself. Ask family and friends to refrain from smoking around you.
  • Participate in your health care: Remember you are part of the “your” health care team. As a member of the team you have a responsibility to do your part. Be prepared to ask your own questions. Bring someone with you to appointments you have and prepare a list of questions to be answered. Ask for referrals to other health team workers who may be able to help you with daily living activities, physiotherapists, occupational therapists, dietitians, and social workers.
  • Get immunised against influenza and pneumococcus: Immunisation will protect you against infections that can be particularly severe for people suffering from diseases of the lung.
  • Manage your coughing symptoms: A nagging, dry cough is exhausting. Although cough is often caused by IPF, there are other conditions that can make it worse, such as a postnasal drip, allergies, and smoking. There are a number of palliative therapies for troublesome cough and you should discuss these with your doctor.
  • Learn and practice relaxation techniques: When you are physically and emotionally relaxed, you avoid excessive oxygen consumption caused by tension of overworked muscles. Learning relaxation techniques can help to manage the panic that often accompanies shortness of breath. Anxiety and depression are common in people with chronic breathing disorders. Many fear losing the ability to function and become dependent on others. The restriction on activity due to shortness of breath may lead to isolating yourself from family and friends, adding to the depression.
  • Keep a positive attitude: Actively participating in all parts of the management of your disease is greatly enhanced by a positive attitude. You may not be able to control the course of your illness, but you can control your attitude and spirit every single day. Do you want to be sad, glad, or mad? Pick one. Do you want to be positive or negative? Choose one. A positive attitude may not solve all your problems, but it will certainly make a difference in how you cope with having IPF.

    A strong positive mental attitude will create more miracles than any wonder drug – Patricia Neal.

As IPF Progresses

There may be times when your symptoms get worse and breathing becomes more difficult, such as when you have a cold or chest infection. You may experience more mucus secretions, your sleep may be interrupted, and daily activities may become more difficult with increased breathlessness.

It is good to be prepared for these situations. Discuss a plan with a doctor or chest specialist and get clear guidelines as to when and how to seek help.  In more acute situations, it may be necessary to be admitted to hospital for more intensive treatment and skilled care. Admission to hospital need not be a frightening experience. It may be an opportunity to meet members of the respiratory team and other health care team members. Your medications will be reviewed and you may be offered support to cope with daily living.  Caring staff understand how distressing it is to be short of breath.  They will adapt a care plan that incorporates your specific needs.  A few days in hospital may sometimes be all it takes to get back to acceptable and manageable energy levels to live in a home environment.

How the community health team can help

Physiotherapists can teach you the secrets to better breathing and can provide rehabilitation so that you can get back on your feet and gain greater confidence when exercising and get you exercising more.

Learn to breathe in the most efficient manner, diaphragmatic breathing, sometimes referred to a “easy breathing”. Practice this new technique over and over and incorporate in activities until it becomes second nature. Learn to relax; restful positions encourage easy breathing. Relaxation keeps the body loose and calm. Plan to exercise regularly; as fitness increases or is maintained, breathing and activities may become a little easier.

Understand breathing and the function of the lungs. The lungs are two cone shaped sponges, with a narrow top and wide base. They lie within an airtight cage formed by the ribs, the breastbone, the spine, and the muscle of the diaphragm. The lower chest is much wider and roomier than the upper chest and hence  the lungs can soak up more air when you breathe into the lower chest than if you breathe only into the smaller area of the upper chest. Also, over reliance on upper chest muscles can result in more oxygen being consumed than is gained. Learn “easy breathing techniques”.

Physiotherapists can provide assessment, treatment, advice, and a self-care plan in home situations.

Occupational therapists will help you achieve more by doing less. Prioritizing daily activities ensures that the most important things are done within one’s abilities and limitations, reducing an often a very frustrating situation. Simplifying tasks and conserving energy will help you to achieve more.  Make a list or timetable, considering monthly or yearly chores. Not all chores have to done at once. Spread tasks, especially the heavier ones, over several days to reduce effort. Remember, many activities also involve preparation and cleaning up. It is important to pace daily activities to avoid unnecessary breathlessness. Slowing down may take a conscious effort. Make time to plan activities by identifying priorities, which will help to conserve energy. Reduce effort by having an organized home, where everything is easily reached and walking is minimized. Consider items used daily and where they are placed, e.g. the telephone and the electric jug. Work at tasks at a comfortable height level and consider sitting to do chores like ironing or peeling vegetables.

A home environment with some changes, may make life easier. Use a shower stool or bath board, hand rails, and a household trolley for moving objects around the home. Increase the height of chairs and your bed to decrease effort in standing. Use a extend-a-hand tool to eliminate bending. Some home alterations can also be considered.

There are easy ways and hard ways to tackle activities, make sure you choose the easy way.

Social workers link needs to assistance and support. Social workers are experienced in giving support and survival techniques, with advice on benefits, funding for special needs, and helping sufferers and their carers to cope from day to day. They can discuss options and possibilities for your needs and make referrals to suitable community contacts, supports and volunteers.  

Most of all they listen to needs and link ideas and possibilities to make it easier to cope with daily living.

Dietitians can make suggestions on eating well and comfortably with breathing conditions. It may be necessary to avoid large meals, and dieticians can provide invaluable advice on the preparation of smaller meals, snacks, and supplements to increase energy sources. When short of breath there is an actual need to eat high calorie foods, to provide the energy needed to breathe.

Clinical psychologists: Living with breathlessness can effect emotions in many ways. It is understandable that panic attacks, anxiety, and depression are unpleasant emotions, but action can be taken to ease them. An organized life can make for independence – place chairs around the house to rest when necessary, make sure the phone and other frequently used items are close to hand, plan strenuous activities at a time of day when breathlessness is not a problem, and carry out tasks in stages with rest in between. Anxiety is natura. Plan activities, gather information about health problems to discuss with  professionals, keep occupied, and practice relaxation techniques.  The distraction of watching television, reading, or doing crosswords can be helpful. Depression or feeling down is not surprising. Setting small realistic goals may give some satisfaction.  Unhelpful thoughts can make depression worse, so try replacing them with something pleasant. Focus on what tasks are possible and keep positive. Panic is a natural reaction to the feeling of shortness of breath, but when shortness of breath becomes long term or a chronic condition, panic is not a helpful reaction. Panic in these circumstances makes the situation worse.  Try relaxing and stay positive. Consciously practice “easy breathing techniques”. Practicing Yoga techniques or music therapy for relaxation may be helpful.

Remember, changing a habit takes practice and time. Don’t expect to do it perfect straight away, but aim at getting better each time you try.

Living with a Terminal Illness

When faced with a terminal illness, life is about living every moment and making the most of every precious day on Earth. However, dying is a big part of life. Talking about it doesn’t bring it closer – it’s about planning for life. The purpose of talking is to help people die the way they wish. The best way to approach this is holistically: taking into account the physical, emotional, and spiritual aspects of the person’s wellbeing and focusing on the patient and their family, which serve as a framework for palliative care.

Everyone deserves to die well and this is more likely to be achieved by talking about it early. Everybody, whatever their age or state of health, needs to talk about their wishes towards the end of their life with their friends, family and loved ones. The earlier we talk about it the easier it is emotionally and practically for everyone. Not talking about a person’s wishes towards the end of their lives with friends, family and loved ones can mean that they may not get what they want or die where they want. They might not have expressed their wishes about their care or funeral, or have made a will. They may simply not have said what they wanted to say.

The following are some subjects that family may like to discuss with loved ones:

  • The type of care they would like towards the end of their life,
  • Where they would like to die,
  • Whether they want to be resuscitated or not,
  • Funeral arrangements,
  • Care of dependants,
  • How they can save other lives through organ donation,
  • How they’d like to be remembered,
  • Whether they have any particular worries they would like to discuss about being ill and dying,
  • What they would like people to know before they die.

More information can be found at the English website Dying Matters.

Reference and reading material

  • Dying – A New Zealand Guide for the Journey by Sue Wood and Peter Fox
    This book has been written to help anyone who is dying, and their family and carers. What the dying person can expect, what others have found important to do and generally how to respond positively and practically to the challenges of this difficult journey.
  • A good day to Die by Lisa Birnie
    A book that tells stories of the dying, their families and friends, and the health professionals who care for all of them. And about how palliative care can give us the freedom and love when we are at most vulnerable.
  • The following helpful booklets from the Living with Cancer series are available from the NZ Cancer Society. A lot of the information is relevant for someone suffering from idiopathic pulmonary fibrosis: though the treatments may be different, the emotions and other unspoken issues are the same for cancer patients and IPF sufferers.
    • Cancer in the Family – covering talking to your children
    • Emotions and Cancer
    • Sexuality and Cancer
    More information from the NZ Cancer Society website.