BREATHE EASILY WITH ALPHA-1 Facts and tips for living with alpha-1 antitrypsin deficiency

Because the lungs are life

Living with alpha-1 antitrypsin deficiency Patient Information Program

DEAR READER, Like most people, you probably first heard about alpha-1 antitrypsin deficiency when it was discovered and diagnosed in you or someone close to you. For many patients it is truly a “discovery”, because after years of uncertainty they finally receive reassurance and the best possible treatment. However, it is just as important for patients to take the initiative themselves and adapt their own lifestyle and daily activities to the realization that they have alpha-1 antitrypsin deficiency. The following pages contain information to help you understand alpha-1 antitrypsin deficiency and show you what treatment options are available. Above all, you will find a wealth of tips and ideas from experts on the following topics: Prevention – avoiding harmful influences Lung sport – keeping physically fit Food and drink – maintaining a balanced diet Stress – relaxing and breathing easily again

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Of course you will not be able to incorporate all these ideas in your everyday life – nor do you have to. But if you devote just half an hour every day to your body, you’ll be on the right track. Needless to say, you can turn to your doctor for advice and help if you have any questions. We wish you many relaxed moments and a “second wind” even on bad days.

ALPHA-1 ANTITRYPSIN DEFICIENCY – WHAT IS IT? Alpha-1 antitrypsin deficiency is caused by faulty hereditary information leading to a more or less pronounced reduction in the production of certain proteins known as proteinase inhibitors (PIs for short). Alpha1-antitrypsin (AAT), the most important of these substances, is responsible for protecting the lungs. These proteins are produced in the liver. However, their main site of action is the lungs. With every breath you take, not only oxygen but also small suspended particles (dust, harmful gases, viruses and bacteria) are drawn into your respiratory organs. The majority of them are trapped in the nose and upper airways and cause no further harm. The remaining invaders are attacked by your immune system, which includes white blood cells (leukocytes). These “blood police” render pathogens harmless. Once they

have done their workor after a natural aging process, the white blood cells die, releasing extremely aggressive enzymes. One of them, neutrophil elastase, can be dangerous for you if your lungs do not have enough of the protective substance which in healthy individuals immediately binds to the released elastase and inactivates it: alpha-1 antitrypsin (AAT). If the hereditary information is defective, less alpha-1 antitrypsin is present in the bloodstream to keep the dangerous neutrophil elastase in check. Over a period of months and years neutrophil elastase gradually attacks the walls of the air sacs in the lungs, destroying their elasticity and turning them into “limp sacs”, which then collapse with every expiration. The medical term for this disorder caused by destruction of the air sacs is pulmonary emphysema.

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PULMONARY EMPHYSEMA AND ITS CONSEQUENCES Pulmonary emphysema is due to destruction of the air sacs in the lungs (alveoli) by inflammatory processes. This reduces the surface available for oxygen uptake and gas exchange, as the remaining alveoli become “limp sacs” that collapse with every expiration. The body then no longer receives an adequate supply of oxygen, and the patient feels this mainly as a sensation of being short of breath. Routine daily activities (e.g. climbing stairs) become increasingly difficult. As the disease progresses, the number of functioning air sacs diminishes continuouslyand air cavities in the lungs grow, because increasingly large sacs develop. At the same time the elasticity of the lungs decreases as a result of the enlarged air cavities, so that the lungs hyperinflate, thus damaging more lung tissue.

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In addition, the hyperinflated lung tissue impedes the surrounding lung tissue. As a result, gas exchange is further limited, i.e. oxygen uptake becomes poorer, and carbon dioxide is not sufficiently released. In time, respiratory function is impaired, followed later by damage to other organs (e.g. the heart). Initially, emphysema may not cause any symptoms for years, as the lungs have relatively large functional reserves. The first signs of emphysema associated with alpha-1 antitrypsin deficiency typically occur between the age of 30 and 40. Later these are joined by excessive sputum production and often respiratory infections. Any further risk factors, e.g. infections and especially smoking, accelerate the destruction of lung tissue and thus the loss of physical resilience.

To sum up: Alpha-1 antitrypsin deficiency is caused by a genetic defect which, among other things, leads to a reduction in the production of the most important protective protein for the lungs, alpha-1 antitrypsin (AAT). Consequently, the walls of the air sacs (alveoli) are destroyed, and pulmonary emphysema develops.

Genetic test Today it is possible to arrive at a fast, reliable diagnosis of AAT deficiency using just a tiny amount of blood. With the help of a test kit a few drops of blood are analyzed to identify the gene responsible for the synthesis of alpha-1 antitrypsin. With this test it is also possible to determine the disease type quickly and reliably.

THE DIAGNOSIS – WHO IS AFFECTED?

Because all hereditary information in humans is doubled, some individuals with alpha-1 antitrypsin deficiency have two defective genes (homozygous deficiency type), while others have one healthy and one defective gene (heterozygous deficiency type). Moreover, different versions of a gene, called alleles, can arise as a result of mutations. Accordingly, the clinical picture may be mild or severe, depending on which alleles are present.

Whether or not a person is genetically predisposed to develop AAT deficiency can be determined today with the help of various tests. Serum levels Your family doctor can order a blood test if there is reasonable suspicion of AAT deficiency. The test measures serum levels of alpha-1 antitrypsin and therefore determines whether the blood serum contains normal amounts of the protein. A greatly reduced serum level is an initial indication of the disease. Your family doctor may refer you to a pulmonologist to carry out further specific tests.

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Hereditary pattern for two sets of parents affected differently (Mendelian diagram) AAT deficiency

Normal Genotype of the parents

M

Z

M

Carrier

M

Possible genotype of the children

or

= Homozygote

Z

Carrier

Z

M

Normal

M

M

Carrier

Z

M

Carrier

M

Z

AAT deficiency

Z

Z

Z

= Heterozygote

Alpha-1 antitrypsin deficiency most commonly affects the lungs. In addition, around 10% of individuals with the homozygous deficiency type develop liver disease in early childhood. This can lead to severe cirrhosis (degeneration of the liver). In some cases both the liver and the lungs are affected.

THERAPEUTIC OPTIONS – HOW THE DISEASE IS TREATED Because the cause of alpha-1 antitrypsin deficiency is genetic in nature, there is as yet no cure for the disease. Nor is it possible to repair damage to lung tissue once it has occurred. However, there are a number of ways to maintain or improve health. Treatment of the disease therefore concentrates on two aims: To relieve symptoms and improve the patient’s quality of life with the help of drug therapy To slow down the further destruction of lung tissue or halt progression of the disease

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Drug treatment for shortness of breath To relieve distressing attacks of shortness of breath, the pulmonologist may prescribe short-acting, fastacting beta2 agonists or anticholinergics. These drugs, which reduce bronchial spasms, are administered in the form of rapidly acting sprays (metered aerosol sprays), powder for inhalation or forms suitable for inhalers. Often cortisone is also required during long-term treatment. In many cases you only need to inhale the cortisone. It acts specifically in the airways and does not have any of the side effects you may fear. However, there are also situations where you may have to take cortisone tablets. Place your trust in your doctor, who will set up a treatment plan tailored to your needs. Do not be put off by “well-meaning” friends. Always remember that cortisone does not relieve acute shortness of breath. It has an antiinflammatory action that takes hours or even days to work.

Adapting your lifestyle No matter what efforts are undertaken to relieve the symptoms of alpha-1 antitrypsin deficiency with drugs, you as the patient are in the driver’s seat. You can determine how the disease develops by modifying your lifestyle and behavior. First and foremost, you must immediately quit smoking tobacco in any shape or form. This must be observed with no ifs or buts! Tobacco smoke significantly accelerates and worsens damage to lung tissue. If you are a nonsmoker, you’ve already taken an important step in the right direction. It is also crucial for people with alpha-1 antitrypsin deficiency to avoid infections of the airways. In addition, you can influence your general well-being by following a suitable diet and exercise regimen. For this reason the following pages of this brochure focus specifically on what you can do for yourself. They contain valuable tips on how you can adapt many aspects of your lifestyle. And the best thing about it is that a lot of what you can do for your health is actually fun. Have a go!

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WELL PROTECTED

How you can protect your lungs in daily life

WHY IS IT SO IMPORTANT TO PROTECT THE LUNGS? Certain irritants can accelerate the decline in lung function.Some of these irritants, for example tobacco smoke (the most important one by far), air pollutants and dust, are environmental. But factors produced by the body itself can also adversely affect lung function, for example substances produced by the body in response to an infection. The best protection for your lungs is to avoid such harmful irritants wherever possible.

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ENVIRONMENTAL POLLUTANTS

Smoking is especially harmful for people with AAT deficiency. It releases increased amounts of neutrophil elastase, thus accelerating damage to the lungs. In addition, cigarette smoke destroys what little AAT is present in the lungs.

People with AAT deficiency should avoid the following environmental irritants:

If you smoke, you should quit as soon as possible! Certainly it is no easy matter to stop smoking, but you can do it. There are programs to help you kick the habit. Have a word with your doctor or contact the German Airway League (www.atemwegsliga.de or phone 0 52 52 / 93 36 15). Some alpha-1 centers also run quit-smoking courses. You can find information on such courses on the internet at www.alpha-1-center.de. Even passive smoking is harmful! Ask everyone who smokes in your vicinity to refrain from doing so.

PROTECTION

TOBACCO SMOKE

Irritants at the workplace Be sure to wear a mask if you are exposed to dust particles or vapors at work. If possible, ask to be transferred to a workplace with more favorable working conditions. You may want to speak to an environmental officer or occupational doctor. Air pollution Heed any ozone warnings on the radio, especially in the hot summer months. If ozone levels are high, try not to venture outside and avoid physical exertion. Offene Kamine Open woodburning fireplaces release harmful particles that can lead to deterioration of lung function in individuals with AAT deficiency. You should therefore avoid open fireplaces and woodburning stoves.

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PULMONARY INFECTIONS Your body reacts to an infection by producing more white blood cells to fight the invading microbes. However, white blood cells release neutrophil elastase, which, as mentioned, leads to deterioration of lung function in individuals with AAT deficiency. It is therefore important to prevent infections. Should you nevertheless contract an infection, you should have it treated promptly and effectively. Avoiding and treating infections Avoid contact with anyone who has a cold or flu. Before meeting friends, check to make sure that they do not have an acute infection. You should get used to consulting your doctor at the first hint of a cold or flu-like symptoms.

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You should also talk to your doctor about getting immunizations against flu and pneumococcal infections. Practical hygiene tips Wash your hands regularly. Wear a mouth mask to protect yourself against droplet infections (e.g. when on public transport). The risk of infection is increased in crowded medical practices. You should therefore go to your doctor early in the morning, for example, when there are fewer people about. Try to keep away from (coughing, sneezing) people with a cold who could infect you.

PROTECTION

To sum up: Don’t smoke! Even passive smoking is harmful. Avoid exposure to environmental irritants. Minimize the risk of infection for your lungs by consulting your doctor at the slightest sign of an infection.

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KEEP MOVING

How to stay fit with breathing techniques and sport Authors: Michaela Frisch, Bad Dürrheim PD Dr. Thomas Köhnlein, Hannover

Sport and exercise therapy are steadily gaining importance in the treatment of lung and airway diseases. And rightly so, because nutrition, physical therapy and exercise, alongside medication, inhalation therapy, long-term oxygen therapy and noninvasive ventilation, are the main pillars of holistic treatment.

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THE AIMS ARE ALWAYS TO: improve their ability to cope with everyday activities improve individual disease management achieve the best possible course of the disease.

In cases of severe lung involvement due to alpha-1 antitrypsin deficiency, physical therapy can be prescribed by your GP or specialist. The same applies to rehabilitation exercise. Both are provided on an individual basis or in (pulmonary) exercise groups.

Stamina and strength training are used to help patients cope with the challenges of daily life. The aim is not only to build muscle mass and strength but also develop coordination. The end result is an improvement in the ability to get around and function confidently in everyday situations.

Of course, exercise and physical therapy do require a certain amount of time and organization. Commitment and dedication are needed to reap the potential benefits, and it is understandable that many patients report that it takes a great deal of effort at first to take advantage of professional physical therapy or an exercise program. But what you can gain by taking these steps is well worth the effort.

Many patients report that after a few weeks of regular exercise they feel more confident and in control, even though they’re still restricted in what they can do. Patients with alpha-1 antitrypsin deficiency in particular can benefit from these positive effects.

Most patients in the early but also in the advanced stages of pulmonary emphysema become better able to manage their disease and improve their quality of life after just a few weeks of a regular, balanced exercise regimen.

LUNG SPORT

enhance the patient’s quality of life

The following breathing and strength exercises were put together by Michaela Frisch, an experienced sports teacher. They can easily be done at home.

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BASICS OF BREATHING Breathing is vital for life and is an automatic reflex. As a result, we all gradually acquire bad habits over the years. We have to become aware of those habits in order to change them. For starters, try to become aware of your breathing in everyday situations. For example, what happens to your breathing when you’re walking, climbing stairs, lifting a drinks crate or talking?

Considering that we take approximately 25,000 breaths every day and that around 10,000 to 12,000 liters of air passes through our lungs in the process (the equivalent of filling a hot-air balloon), it’s quite shocking to think how we take breathing for granted – at least until we’ve been diagnosed with a lung disease. On the following pages we are going to show you various self-help breathing techniques to prevent further damage to your lungs and to help you cope better with the stresses of everyday life. They can be used alongside your drug therapy and other treatment and will help you to regain your quality of life. These self-help techniques should become an integral part of your life. You’ll learn how to cope with your disease, manage it independently and take control of it. We wish you enjoyment and success as you learn and practice.

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LUNG SPORT

The pursed lips technique One of the most important self-help techniques – particularly if you’re exerting yourself – is the pursed lips technique: breathing out with slightly pursed lips. The pursed lips technique prevents the airways from collapsing by equalizing the pressure. The exercise shifts pressure from the bronchi to the lips (the Bernoulli effect) to ensure slower, more measured expiration from the airways. By pursing your lips, you can slow down the outward flow of air so that you exhale more and for longer. This results in deeper breaths. Initially it helps to practice this breathing when you’re relaxed. You can then combine it with mild exertion until it becomes second nature. To begin with it is often helpful to vocalize the sounds “f”, “s” or “sh” so that you can hear your breath escaping slowly but without pressure. In time you’ll be able to practice the pursed lip technique soundlessly. .

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Abdominal breathing Another very important self-help technique is to reduce respiratory work by using the abdominal muscles to breathe (also known as diaphragm breathing).The aim is to breathe more deeply and avoid using the auxiliary respiratory muscles, which can make you breathless and sap your strength. This will help you improve the mobility and function of your diaphragm. To teach yourself abdominal breathing it is a good idea to lie down in a comfortable position (with something underneath if you prefer, e.g. a body pillow). Place both hands on your abdomen and consciously breathe in through your nose into the place where your hands are positioned on your abdomen. Breathe out using the pursed lips technique. By practicing while lying in various positions you will become more aware of your body and learn how to control your breathing. In time you’ll be able to combine this breathing technique with various levels of exertion until it becomes second nature. .

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Positions that facilitate breathing relieve your chest of the weight of your arms and shoulder girdle, thus relaxing your auxiliary respiratory muscles (reduced respiratory work). As a result, the air volume in the lungs increases and narrowed bronchi dilate (increasing breathing volume). Your back muscles relax and, because the muscular work is reduced, less energy and oxygen are consumed. Stagecoach position Sit with your legs hip-width apart. Support your upper body with your hands (straight back, relaxed abdominal muscles). Straddling position Straddle a chair backwards and place your lower arms on the back of the chair. Lay your head on your lower arms.

Standing position Face a wall, the back of a park bench or a railing and support yourself with either one or both arms. If using one arm, place your other hand on your hip. LUNG SPORT

POSITIONS TO MAKE BREATHING EASIER

Crouching position Crouch down with your knees together, heels apart and your buttocks between your feet. Place the palms of your hands on your thighs. Bend your arms slightly. Important: Straight back, relaxed abdominal muscles. Goalkeeper position Stand with a straight back and your legs hip-width apart. Bend your knees slightly. Support your upper body by placing your hands on your knees. Find the most comfortable position for you!

Variation: Put a large cushion on a table and place your lower arms on top of it. Lay your head on your lower arms.

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BREATHING UNDER EXERTION When you breathe while under exertion you should make sure that short-term controllable exertion (e.g. lifting a drinks crate, pulling on your trousers, etc.) is combined with breathing out. Remember: Exhalation – Exertion Inhalation – Relaxation

ATEMÜBUNGEN IM SITZEN Place both hands on your abdomen. When you inhale try to breathe into your hands. When you breathe out, exert slight pressure with your hands to aid expiration.

In the case of sustained exertion that you can’t control, you should use the pursed lips technique and abdominal breathing. It’s important to adapt your breathing according to the type of exertion or movement you’re engaged in and not the other way round (e.g. if you start walking more quickly, it’s wrong to start breathing more quickly). The following exercises are intended to deepen your breathing and increase the mobility of your ribcage.

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Let your arms hang down by your sides. As you inhale turn the palms of your hands outwards and pull your shoulders back and down. Relax slowly as you exhale.

Place your hands on your waist (under your ribcage). As you inhale, twist your upper body slowly so you’re looking over your shoulder. Turn back as you exhale. Change sides.

LUNG SPORT

Let your arms hang down by your sides. As you inhale, raise one arm over your head as far as possible. Your body will bend slightly to the opposite side. Lower your arm as you exhale. Change arms. Variation: As you inhale push your elbows back. Bring them forward again as you exhale.

Stretch both arms out at shoulder height with your thumbs uppermost. As you inhale, bend your arms and bring your hands in front of your chest (as though you’re scooping up an armful of fresh air). As you exhale, stretch your arms out again (pushing away the old, used air).

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BREATHING EXERCISES IN THE LYING-DOWN POSITION

Lie down on your back with your legs outstretched. Place your right arm alongside your head. As you inhale, stretch your right arm as far as you can (making yourself taller). Relax as you exhale. Change sides. Variation 1: As you inhale, stretch out your right arm and right leg (making your right side longer). Variation 2: Hold this position for several breaths. Variation 3: Do the exercise lying on your side with the lower leg slightly bent.

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Lie on your side and bend both knees slightly. Bend your upper arm and place your hand behind your head. As you breathe in, slowly twist your upper body behind you keeping your knees together on the floor. Return to your original position as you breathe out. Change sides. Variation: Hold the twisted position for several breaths.

PHYSICAL EXERCISE

The only way to break this vicious cycle is to follow a suitable exercise program.

Regular physical exercise as a way of life ... strengthens your muscles improves your stamina boosts the oxygen content of your blood

LUNG SPORT

A vicious cycle People who are prone to become short of breath, especially during physical or mental exertion, often develop a protective mechanism known as avoidance behavior. This means that they tend to steer clear of anything they instinctively feel could cause shortness of breath, particularly physical activities. This leads to de-conditioning of the cardiovascular system, a reduction in coordination and progressive weakening of the entire musculature. But the poorer the condition of your muscles, the more oxygen you need. This can increase your risk of falling. In addition, you could suffer from osteoporosis. Your ability to cope with exertion worsens. The result could be progressive deterioration of your quality of life, often accompanied by social isolation, and you could become depressed.

reduces your body’s oxygen needs improves your shortness of breath strengthens your immune system. Thus, a suitable exercise program can improve your quality of life despite your illness and help you cope better with everyday activities. Take the plunge and start to actively manage your illness. However, before you get going, you should talk to your doctor and find out your exercise tolerance by undergoing appropriate tests (lung function, blood gas analysis, exercise ECG, six-minute walking test, etc.). Get active!

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EXERCISE GUIDELINES Start slowly. Go at your own pace, for instance when walking or climbing stairs. It isn’t important what others think or how quickly others move. It’s better to exercise several times a day rather than overdoing it once a week. (Taking a 15minute brisk walk three times a day for example in the house or garden has proved effective.) Use every idle moment in your day to do something for yourself and your health. Find a specific time in your day to exercise within your own four walls (while watching your favorite TV series or the news). Take every opportunity and use objects in your home to keep active and exercise.

Set yourself realistic, achievable goals. Being too ambitious is just as detrimental to success as not being ambitious enough. Allow yourself breaks in between. Always start with warm-up and stretching exercises. Vary your training in terms of intensity, extent of movement and pace. Be aware of your body’s alarm signals. If you have a tight feeling in your chest or feel light-headed, you should stop exercising immediately. To conquer your lazy streak always keep in mind the reason you’re training: to improve your own quality of life. Monitor and make a note of your training and progress and modify your exercise and your physical limitations in consultation with your doctor.

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Stretch out both arms at shoulder height while sitting or standing and move your shoulder blades back and down..

Sit upright on a chair and stretch one leg just above the floor in front of you. Flex your toes towards you, maintaining your seated position.

Exercise selection 1. Hold your arms in the air. 2. Describe small up and down movements with your arms. 3. Again, using small movements cross your arms in front of your body and open them again slightly. 4. Make small circles with your arms.

Exercise selection 1. Keep your leg in the air. 2. Describe small up and down movements with your leg. 3. Make a figure of eight. 4. Bend your leg slowly and then stretch it out again. Keep your toes flexed.

Variation: Vary the pace of the movements: small, quick movements or big, slow movements.

LUNG SPORT

EXERCISES FOR ARMS AND LEGS

Variation: Additional exercises: 1. Box with your arms to the side, in front of you and towards the ceiling. 2. Hold out your arms at shoulder height. Make fists with your hands then open them again.

Or additionally: 1. Walk on the spot. 2. Alternate placing your heels in front of you.

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MY HOME IS MY OWN FITNESS STUDIO You can enjoy varied and inexpensive exercises by using your imagination and various objects in your own home. Here are a few examples to stimulate your creativity:

Towel/tea towel Hold the towel at shoulder height with both arms stretched out in front of you and pull on the towel as hard as you can. Bend your body forwards as you’re doing so. It doesn’t matter whether you’re sitting or standing. Maintain this position without holding your breath. Variation: Move your arms gently up and down.

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Cooking pot Hold the pot by the handles and pull on it as you breathe out. Release as you breathe in.

Broom handle Rest the broom on the floor so the handle is upright to the side of you while you are sitting or standing. As you breathe out push the broom as hard as possible into the floor. Release as you breathe in. Variation: As you do this exercise, step forward, alternating legs.

LUNG SPORT

Doorframe Stand in a doorway. Put your hands on each side of the doorframe and push as hard as you can against it. Lift one leg and pretend to ride a bike. Change legs.

Purse / oxygen cylinder in its case Grip the object by the handles or straps with both hands and hold it in front of you with slightly bent arms.

Variation: Pedal forwards and backwards, changing the pace.

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Plastic bottle (empty or filled with sand) Move the bottle around your body or head while sitting or standing. Change directions. Variation: Alternate lifting your legs and pass the bottle under the raised leg or circle the bottle around your leg (change legs and direction).

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Sheer pantyhose (instead of a Theraband®) Stand on the feet of the pantyhose and hold them at the waistband. As you breathe out, bend your arms (with elbows pointing outwards). Lower your arms as you breathe in. Variation: Use small, quick movements and breathe normally.

Exercises with a Theraband® Place your left leg on the Theraband® and hold the other end with your right hand. As you breathe out pull to the side and up. Relax as you breathe in. Repeat several times, then change sides.

LUNG SPORT

Hold the Theraband® from below at shoulder height with slightly bent arms. As you breathe out pull on both ends (don’t raise your shoulders). Release as you breathe in. .

Sit upright with a straight back. Wrap the band around your thigh once and grip it with both hands resting on your thighs. As you breathe out push your legs apart. Relax as you breathe in. Always keep the band slightly taut.

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STRETCHING EXERCISES Place both arms at your sides and turn the palms out to the front. Lower your head slowly to the left and push your right arm slowly towards the floor. Hold the position. Change sides. Cross your hands behind your head and push your elbows back as far as you can. Hold the position.

Setzen Sie sich aufrecht und gerade hin und strecken Sie das rechte Bein nach vorne. Die Ferse aufsetzen und die Zehenspitzen hochziehen. Bend forward as far as you can. Hold the position. Change sides. Always hold the stretches for approx. 30–40 seconds.

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PULMONARY EXERCISE ISN’T A COMPETITIVE SPORT!

These exercise groups, which are set up specifically for people with respiratory problems, don’t overburden the doctor’s budget. Their quality is assured because the instructors are specially trained and they are required to take regular refresher courses. Despite the various prescription options (as a rule 50 sessions over 18 months, or 120 sessions over 36 months in cases of severe chronic pulmonary infection) and the duration of an exercise session (90 minutes), this is not enough for the entire week. What you learn should become an integral feature of your daily exercise regimen.

Breakdown of a pulmonary exercise session Warm-up phase Training + peak-flow measurement + warm-up exercises or games (with or without equipment, as group games or exercises with a partner, e.g. numbers game, playful variations).

LUNG SPORT

Talk to your doctor about a prescription for group pulmonary exercise. It’s easier and more fun to train with like-minded people. You can also benefit from exchanging information with other people.

Exercise phase In various starting positions with or without equipment as functional exercises to build muscle and stamina, combined with breathing and stretching exercises. Stamina phase Stamina games or interval training for example, again in combination with breathing exercises and stretching exercises. Games phase In this phase stamina training takes second place to having fun with movement and group dynamics (including training with cognitive and fine-motor skills). Relaxation phase Variable, interchangeable with the games phase (baking pizzas, coping with weather changes, hedgehog ball massage, Progessive Muscle Relaxation, relaxation stories).

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HEALTHY EATING

How to adapt your diet to your disease Author: Dr. Gudrun Zürcher, Freiburg

The right dietary habits play an important role in maintaining healthy lung function. A healthy diet keeps the body strong, providing it with all the nutrients needed for our organs to function well, including the lungs. A diet tailored to individual needs also affects our body weight.

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YOUR “IDEAL” WEIGHT

BODY-MASS-INDEX (BMI)

In adults weight is evaluated on the basis of the BMI (body mass index). Your BMI indicates how heavy you are in relation to your height. Your weight is “normal” if your BMI is between 18.5 and 24.9 kg/m2. Accordingly, a man or woman 1.60 m tall should weigh between 47.4 and 63.7 kg.

You can estimate your BMI using this table: Height in meters Weight 1.50 1.55 1.60 1.65 1.70 1.75 1.80 1.85 1.90 1.95 2.00 30 kg 13.3

8.8

8.3

7.9

7.5

35 kg 15.6 14.6 13.7 12.9 12.1 11.4 10.8 10.2

12.5

11.7

11.0 10.4

9.8

9.3

9.7

9.2

8.8

40 kg 17.8 16.6 15.6 14.7 13.8 13.1 12.3 11.7 11.1 10.5 10.0

We know however that in patients with pulmonary diseases a BMI under 21 kg/m2 is not desirable and that a BMI over 25 kg/m2 is acceptable as long as there is no further weight gain. This particularly applies to people over 65.

45 kg 20.0 18.7 17.6 16.5 15.6 14.7 13.9 13.1 12.5 11.8 11.3 50 kg 22.2 20.8 19.5 18.4 17.3 16.3 15.4 14.6 13.9 13.1 12.5 55 kg 24.4 22.9 21.5 20.2 19.0 18.0 17.0 16.1 15.2 14.5 13.8 60 kg 26.7 25.0 23.4 22.0 20.8 19.6 18.5 17.5 16.6 15.8 15.0 65 kg 28.9 27.1 25.4 23.9 22.5 21.2 20.1 19.0 18.0 17.1 16.3

If you’d like to calculate your BMI exactly, you can do so by using the following formula:

NUTRITION

70 kg 31.1 29.1 27.3 25.7 24.2 22.9 21.6 20.5 19.4 18.4 17.5 75 kg 33.3 31.2 29.3 27.5 26.0 24.5 23.1 21.9 20.8 19.7 18.8 80 kg 35.6 33.3 31.3 29.4 27.7 26.1 24.7 23.4 22.2 21.0 20.0 85 kg 37.8 35.4 33.2 31.2 29.4 27.8 26.2 24.8 23.5 22.4 21.3

How to calculate your BMI Body weight in kilos divided by the square of your height in meters.

90 kg 40.0 37.5 35.2 33.1 31.1 29.4 27.8 26.3 24.9 23.7 22.5 95 kg 42.2 39.5 37.1 34.9 32.9 31.0 29.3 27.8 26.3 25.0 23.8 100 kg 44.4 41.6 39.1 36.7 34.6 32.7 30.9 29.2 27.7 26.3 25.0 105 kg 46.7 43.7 41.0 38.6 36.3 34.3 32.4 30.7 29.1 27.6 26.3

Example:

86 kg = BMI 24.9 1.86 x 1.86 m

110 kg 48.9 45.8 43.0 40.4 38.1 35.9 34.0 32.1 30.5 28.9 27.5 115 kg 51.1 47.9 44.9 42.2 39.8 37.6 35.5 33.6 31.9 30.2 28.8 120 kg 53.3 49.9 46.9 44.1 41.5 39.2 37.0 35.1 33.2 31.6 30.0 Underweight

Normal weight

Overweight

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However, for people with a medical condition the body’s composition is particularly important and also the percentage of muscle mass. BMI is only a measure of body fat and gives no indication of the percentage of muscle. Muscle mass is not just low in underweight people, it can also go unnoticed in people of normal weight and those with a high percentage of body fat. In addition, muscle mass is reduced every time we lose weight, either unintentionally due to poor diet or intentionally by following a slimming diet. Problems associated with being overweight and underweight If you do need to lose weight, your diet should be adjusted in consultation with a nutritionist and a doctor. On no account should you go on a starvation diet. Protein should make up approximately 20% of your total energy consumption so that you lose as little muscle mass as possible. An accompanying exercise regime is also important.

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Studies have shown a clear correlation between a high BMI and the risk of complications. The World Health Organization (WHO) has published the following classification table:

WEIGHT CLASSIFICATION FOR ADULTS BASED ON THEIR BMI Category

BMI

Risk of complications associated with obesity

Underweight

18.5 or less

Low

Normal weight

18.5–24.9

Average

Pre-obese

25–29.9

Slightly raised

Obese class I

30–34.9

Raised

Obese class II

35–39.9

High

Obese class III

From 40

Very high

YOUR WAIST GIRTH We also know that not just a raised BMI but also body fat deposited around the waist is an independent risk factor for the development of metabolic diseases (e.g. diabetes) and cardiovascular disease (e.g. heart attack and stroke). To put it simply, the bigger your waist, the greater the risk. This also applies to people who are otherwise slim. The following limit values apply: Waist girth and risk of metabolic and cardiovascular diseases Waist girth in men

Waist girth in women

Increased risk

94 cm or more

80 cm or more

Markedly increased risk

102 cm or more

88 cm or more

People with alpha-1 antitrypsin deficiency should therefore also be aware of their waist girth.

Problems associated with being overweight and obesity The body fat deposited around your midriff can restrict the mobility of your diaphragm, making breathing more difficult. Obesity puts a strain on the entire cardiovascular system and the lungs, because your body is then carrying an increased “load” and consumes more oxygen. You are more at risk of metabolic and cardiovascular diseases. Problems associated with being underweight In the case of malnutrition and unwanted weight loss, reduced muscle mass and weakening of the respiratory muscles could impair pulmonary function and the uptake of oxygen. An insufficient supply of energy and nutrients can weaken your immune system and increase your risk of infection. Patients with pulmonary diseases in particular are more prone to frequent and severe bouts of respiratory infection. Moreover, if you develop another illness or have to undergo an operation, you’ll have less energy in reserve to draw upon.

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THE HEALTH-MAINTAINING DIET

THE MAIN NUTRIENTS

Energy needs To maintain or achieve an appropriate body weight it’s a good idea to assess your own energy needs, which depend on your routine daily activities:

Your diet should consist of roughly the following proportions:

Bed rest: 25 kcal per kg of body weight

PROTEIN 15 – 20 % FAT 30 – 35 %

Gentle activities: 30 kcal per kg of body weight Moderate activities: 35 kcal per kg of body weight Vigorous activities: 40 kcal per kg of body weight Women require around 10 percent fewer calories than men. When calculating energy needs actual weight is used in the case of people who are underweight, of normal weight or overweight, and the ideal weight in the case of people who are obese. This can be calculated using the following formula: Height in meters minus 100 = ideal weight (e.g. a woman 1.65 m tall should weigh around 65 kg). Note: If you are obese your actual energy requirement is at or somewhat below your calculated energy requirement. If you are underweight you should eat more than the estimated requirement.

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CARBOHYDRATES 35 – 55 %

Proteins (1 g supplies approx. 4 kcal): Proteins are the building blocks for all living organisms and form an essential component of cells, tissue, antibodies, enzymes, hormones, and clotting factors. Lack of protein can lead to a weakened immune system and impaired mental and physical performance. Merke: Proteins cannot be replaced by any other food! Animal protein is more valuable than vegetable protein. Overweight people in particular need a lot of protein. Fat (1 g supplies approx. 9 kcal): Fat is the most important energy supplier. It supplies essential fatty acids and is also needed to absorb the fat-soluble vitamins A, D, E and K from food.

Suggested amounts – how much of each food group is healthy: Of course the goal of nutritional advice is not to add up calories every day with a calculator and lose your enjoyment of food completely. Instead, try to enjoy and appreciate your change of diet. Maybe you can find someone to join you and try new culinary dishes together In the following, foods are divided into seven groups. The information on the amounts of from each group to be consumed correspond to daily intakes of 1800 and 2200 calories (the latter in parentheses).

Carbohydrates (1 g supplies approx. 4 kcal): Carbohydrates also supply energy, some of which rapidly becomesavailable. They are needed especially for the muscles, brain and nerve cells. Dietary fiber is made up of carbohydrates.

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GROUP I Grains, grain products, potatoes These contain: carbohydrates, fiber, vegetable protein, minerals (potassium, magnesium, iron, selenium, zinc), vitamins (B1, B6, C, folic acid), secondary plant substances. Per day Bread 200 (300) g (4 [6] slices) Getreideflocken 20 g Potatoes 200 (250) g (boiled) or rice or noodles 210 (270) g (boiled). Eat wholegrain products if tolerated.

GRUPPE II Vegetables, salad These contain: carbohydrates, fiber, minerals (potassium, magnesium, iron), vitamins (A, B2, B6, C, folic acid), secondary vegetable substances. Pulses These contain: carbohydrates, fiber, vegetable protein, minerals (calcium, potassium, magnesium, iron, zinc), vitamins (B1, folic acid, K), secondary vegetable substances.

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Per day Vegetables, pulses 200 g or more Salad 50 g or more Note: Pulses often cause flatulence, which can lead to shortness of breath due to the raised position of your diaphragm.

GRUPPE III Fruit Fruit contains: carbohydrates, fiber, minerals (potassium), vitamins (C), secondary vegetable substances. Nuts These contain: vegetable protein, vegetable fats, minerals (calcium, potassium, magnesium, iron, selenium, zinc), vitamins (B1, K), secondary vegetable substances. Per day Fruit 200 (250) g or more Note: Nuts, particularly walnuts, are valuable foodstuffs that are also beneficial in small quantities.

GROUP IV

GROUP V

Milk, dairy products These contain: animal protein, animal fat, carbohydrates, minerals (calcium, iodine), vitamins (A, B2, B12, folic acid).

Meat, poultry, game, sausage These contain animal protein, animal fat, cholesterol, purines, minerals (potassium, iron, selenium, zinc), vitamins (B1, B2, B6, B12).

Per day Milk/yoghurt ¼ liter / 250 g Cheese 50 g (up to 45% fat) Low-fat products are preferable if overweight. Note: Milk and milk products are our principal calcium suppliers and should therefore be eaten on a daily basis.

Fish Fish contains: animal protein, animal fat, cholesterol, purines, minerals (potassium, iron, iodine, selenium, zinc), vitamins (D, B1, B2, B6). Note: Preferably saltwater fish such as herrings, mackerel and salmon because they contain omega-3 fatty acids. These are good for patients with pulmonary disease because they have an anti-inflammatory effect. Saltwater fish also supplies us with sufficient vitamin D. Eggs These contain: animal protein, animal fat, cholesterol, minerals (potassium, iron, selenium, zinc), vitamins (A, D, B12, folic acid). Per day Meat, poultry, game: 120 (150) g and sausage: 70 (100) g: choose low-fat products 2 to 3 times per week Fish: 150 g 1 to 2 times per week Eggs: 2 (3) per week

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GROUP VI

GROUP VII

Fats These contain: essential fatty acids (linoleic acid), fatsoluble vitamins (A, D, E).

Drinks Suitable drinks: uncarbonated drinks, drinking water, fruit and herbal teas, diluted fruit juices, coffee and black tea in moderation.

Per day Butter, margarine, oils (preferably rapeseed oil), Spreadable fat: 15 (20) g Cooking fat: 15 (20) g Oil: 1 tablespoonful Note: The total amount of fat needed (spreadable fat and cooking fat combined) is about 1 g per kg of body weight. Vegetable fats are preferable.

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Per day About 30 to 40 ml per kg of body weight Alcohol is classified as a drink rather than a beverage. Alcohol should be consumed with food but not every day. Pregnant women should avoid alcohol. The tolerable amount for men is not more than two glasses per day (20 g alcohol), and for women, not more than one glass per day (10 g alcohol). There are 20 g of alcohol in ½ liter of beer or ¼ liter of wine.

Tips Several small meals are better than three large ones: they put less stress on your system and you will be able to function better. Watch out for any food intolerances (e.g. flatulencecausing foods). You can eat an unlimited amount of vegetables because they contain fewer calories. They are therefore an ideal and versatile food for people who are overweight. For those who are malnourished or underweight, high-calorie drinks or meals are beneficial. Ask your doctor or a nutritionist .

Patients with pulmonary disease may also have an increased need for certain nutrients, e.g. antioxidants (vitamins, A, E, C). Vitamin D deficiency is very common. In this case you should consult your doctor about taking multivitamins (health insurance companies will not pay for these). Three times the daily requirement can be taken without causing harm. Caution should be exercised if you suffer from other diseases. Be especially careful about the dose of fatsoluble vitamins you take. It is important that you talk to your doctor about this.

Daily exercise helps you to build up muscle tissue. If you don’t eat enough or if you have an unbalanced diet and are unable to change your eating habits, you may have a nutrient deficiency. This doesn’t just mean the main nutrients (protein is particularly important in this respect) but also minerals, trace elements and vitamins.

To sum up: For people with alpha-1 antitrypsin deficiency it is sensible to strive towards the “ideal” weight. Talk to your doctor if you are obviously over- or underweight. A balanced diet and exercise program are important to maintain lung function.

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COMPLETE RELAXATION How to breathe when under stress Author: Michaela Frisch, Bad Dürrheim

WHAT IS STRESS? Stress is a state of mental or emotional strain or tension. The physical or mental reaction to stress is triggered by stress factors, known also as stressors. The body responds to such internal and external stimuli. These events and their consequences are subconsciously categorized into good and bad and are stored in the subconscious. A vicious cycle can then develop where cause and effect become indistinguishable from each other. Stress accompanies us throughout our lives, and we often cause it ourselves. Everyone reacts differently to stress and copes with it in different ways.

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For this reason learning relaxation techniques, which can be easily integrated in your daily routine, is an important part of disease management for respiratory patients, the aim being to avoid increasing respiratory work further and as far as possible to avoid shortness of breath. There are also exercises to “breathe away” stress and reduce anxiety, thus improving one’s quality of life. Regular breathing exercises can help strengthen your immune system and enable you to cope better with everyday situations. .

Physical reactions to stress Everyone reacts differently to chronic mental and physical stress. Typical symptoms of stress are a racing, pounding heart, often accompanied by a feeling of tightness in the chest or neck and shortness of breath. Hot flushes or chills, tremor and weakness at the knees are also common. Some people also report a numb or tingling feeling as well as dizziness and faintness. In others stress is manifested as pain, nausea, abdominal distress or sweating. Dry mouth and increased salivation are other common physical reactions to stress. Of course many of these physical symptoms can also be triggered by other factors or may be an indication of other conditions. It is therefore advisable to observe them (e.g. by keeping a stress log) and if you are in doubt to consult your family doctor or pulmonologist.

STRESS

Breathing and stress Breathing plays a key role in all relaxation techniques. This reflects the close relationship between breathing and stress. Patients with respiratory diseases are particularly aware of how tension, pressure and turmoil (internal and external) affect their breathing. A person’s current state of health and subjective wellbeing can affect them as much as the people around them, a recent event or a new, unusual situation.

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Subjective perception of stress Many feelings associated with stress – for example halting breathing, a sensation of having a lump in your throat or a queasy feeling in the stomach – can also be a short-term consequence of nervous tension. The same applies to the subjective feeling of having a stuffed nose or of having too much on your plate. If such feelings persist over an extended period and are accompanied by a feeling of tightness in the chest or fear of losing control, they are often a sign of excessive stress. What is relaxation? To put it simply, relaxation is the conversion of tension, pressure, or anxiety into a personal balance of harmony, inner peace and control.

How do you achieve relaxation? Physical relaxation can be achieved by controlling your muscles and breathing. Many relaxation exercises are therefore based on similar principles. You can free yourself of distressing thoughts with the help of concentration exercises. From the range of options available for relaxation, each person can choose his or her own way. What is important is not the method of relaxation but the relaxation itself. The following section contains information on popular relaxation methods. Take every opportunity you can to try them out.

Autogenic training (AT) Autogenic training was developed in 1910 by Professor Johannes Heinrich Schultz. The German psychiatrist and psychotherapist used his knowledge of hypnosis to devise a relaxation technique in which the practitioner repeats a set of visualizations. The term autogenic means arising from oneself. Essentially, sensations

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such as heaviness (reduction of muscle tension), warmth (increased circulation) and rest (reduction of the heart rate and breathing rate) are evoked to influence the body, spirit and mind through the power of thought (auto-suggestion). Autogenic training – exercise Your left leg is pleasantly heavy and warm. Your right leg is pleasantly heavy and warm. Both your legs are pleasantly heavy and warm.

Progressive muscle relaxation Realizing that it is easier to perceive one’s inner muscletension than one’s own heart rate, the US physiologist Edmund Jacobsen developed the technique of progressive muscle relaxation in the early 1930s. In this method, tensing the muscles does not serve as a strength exercise. Rather, the technique focuses on conscious phases of relaxation. To this end muscle groups are tensed one after the other so that the person doing the exercise is able to feel them consciously and then relax them.

Your left arm is pleasantly heavy and warm. Your right arm is pleasantly heavy and warm. Both your arms are pleasantly heavy and warm. Your entire body is pleasantly heavy and warm.

STRESS

With each expiration your relaxation becomes deeper.

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Progressive muscle relaxation – exercise Important: Always let your breathing flow freely. Do not hold your breath.. Assume a relaxed position.Close your eyes when you feel the need. Place your hands loosely on your thighs. Make a fist with your right hand tensing the muscles, but not too tightly. Hold this tension for a few seconds. With your left hand feel the tension in your right forearm. Then open your fist and allow your arm to rest loosely for up to 30 seconds. Feel the relaxation slowly radiating out. Change sides.

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Concentrated breathing Concentrated breathing is the conscious combination of breathing and movement. It complements other selfhelp techniques for respiratory patients such as abdominal breathing, the pursed lips technique and positions to ease breathing. Concentrated breathing – exercise As you breathe in, make a loose fist with one hand (do not clench it). As you breathe out, open your fingers slowly. Think of holding a butterfly as you breathe in and releasing it along with the old stale air, distressful thoughts and stress as you breathe out.

Visualization and suggestion Like imaginary travel, the visualization of an inner image calls up positive memories, experiences, feelings and thoughts that can help you relax.

Imaginary travel – exercise

Meditation In the ancient art of meditation the practitioner turns his or her gaze inward. Screening out external stimuli stops the senses from being overwhelmed by mental, emotional and physical impressions and perceptions. Meditation can be object-free, where your thoughts are given free rein while you concentrate on breathing, or object-oriented where you focus fully on a certain object or a chosen word.

You are lying on a beach in warm, soft sand. The sun is warming your body from above. You hear the twittering of birds and the rushing of the sea. You feel how your breath rises and falls, flowing in and out like the waves.

STRESS

Imaginary travel Imaginary travel stimulates positive thought by means of inner visualizations. The pleasant feelings and memories thus evoked are used to achieve relaxation.

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Tai chi The “dynamic art” of t’ai chi ch’uan (or tai chi for short), which developed in China and is still widely practiced there, is a combination of Asian martial art, healthy exercise and meditation with movement. The name is a compound of the Chinese words tai (body) and chi (life energy). In the Western world tai chi is sometimes known as shadow boxing, because its movements are executed in slow motion. Each movement is accompanied by its dynamic opposite (“everything has its order”). Practitioners are able to re-evoke the feeling of tai-chi-induced mental calm and control in stressful situations.

Tai chi – exercise Stretch out your left arm at shoulder height with your palm facing inward. As you breathe out, pass your right hand from your left shoulder, along your outstretched left arm to your hand. As you breathe in, slowly bring your right hand to your left shoulder along your outstretched left arm. Repeat several times. Rest. Change sides.

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Qigong – exercise Assume a relaxed standing position with your feet hip-width apart. Place your hands in front of your chest with your elbows bent. Your fingertips are touching and your palms are facing upward. Take a short step backward with your right foot. Slowly extend your left arm in front of you with your index finger pointing upward and your palm facing away from your body. Follow your index finger with your eyes. At the same time draw your right elbow further back. Hold this position briefly. Then slowly return to your starting position. Change sides.

STRESS

Qigong The Chinese art of qigong is mentioned in 2000-yearold sources, but its tradition probably goes back much further. It uses slow, flowing movements coordinated with conscious breathing and meditation and seeks to harmonize the forces of yin (relaxation) and yang (activity). The aim is to balance the inner life energy (chi). The disturbed energy flow is converted into a free exchange between the two opposite poles.

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Yoga Yoga has been an integral part of Indian philosophy for some 2000 years. Its aim is to achieve physical, mental and spiritual harmony by carrying out specific physical exercises. Modern forms of yoga have since developed alongside many traditional yoga schools. What they all have in common is physical awareness and spiritual discipline. Yoga exercises are a combination of physical postures (asanas) and breathing exercises (pranayama) that strengthen the muscles and relax the body. Opposing forces working on the body are balanced by means of physical exercise.

Tipps und Empfehlungen There is no such thing as good or bad relaxation. Each person must find his/her own way to relax. What is important is not the method of relaxation but the relaxation itself. Take every opportunity you can to try out and test the individual relaxation techniques, for example during a rehabilitation exercise session or at a course organized by the adult education center or your health insurance. To start with, do the relaxation exercises at rest and without stress. Later you will be able to use them automatically in stressful situations. Always pay attention to your breathing. Let it flow and never hold your breath. This can always be complemented by the pursed lips technique, where you consciously breathe out slowly through slightly opened lips.

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AUTOREN Lungenemphysem - COPD Deutschland Lindstockstr. 30 45527 Hattingen Tel. 02324 / 999000 [email protected] www.lungenemphysem-copd.de Deutsche Atemwegsliga e. V. Im Prinzenpalais/ Burgstr. 12 33175 Bad Lippspringe Tel. 0525 / 933615 [email protected] www.atemwegsliga.de AG Lungensport in Deutschland e. V. Wilhelm-Theodor-Römheld-Str. 20 55130 Mainz Tel. 06131 / 9718832 [email protected] www.lungensport.org

Michaela Frisch Espan Klinik Gartenstr. 9 79073 Bad Dürrheim PD Dr. med. Thomas Köhnlein Medizinische Hochschule Hannover Abteilung Pneumologie Carl-Neuberg-Str. 1 30625 Hannover Dr. med. Gudrun Zürcher Medizinische Universitätsklinik Abteilung Innere Medizin I Schwerpunkt Hämatologie/Onkologie Sektion Ernährungsmedizin und Diätetik Hugstetter Str. 55 79106 Freiburg i. Br.

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