Primary Immunodeficiency Association

About This Booklet
People with antibody deficiency are especially prone to respiratory infections such as sinusitis, bronchitis and pneumonia. In extreme cases, these infections can lead to unpleasant feelings of breathlessness, even after short bursts of activity. As a result, it can be tempting to take the easy option and give up trying to lead an active life. Yet this in itself can pose problems, not just to the way you feel (your physical well-being), but also to the way you feel about yourself (your morale). Being confined to the home isn't much fun for anyone!

Strange as it may seem, the solution lies not in stopping all exercise, but in ensuring that you get enough exercise. Just a few simple exercises performed for a few minutes a day can make all the difference to your physical and mental well-being.

This booklet introduces some self-help techniques for improving your condition, whether you experience mild breathlessness only or have severe problems as a result of damage to your lungs. It also suggests exercises for young children who have chest problems as a result of antibody deficiency.

Note: The information in this booklet is intended as a guide only. Always consult your own doctor or physiotherapist before you start an exercise programme.

The PiA gratefully acknowledges the help of the following people in the preparation of this booklet: Sister Sheila Cochrane and Claire Hilton, both of the Hope Hospital, Salford; Barbara A Webber of the Royal Brompton Hospital, London; and Dave Threlfall of Sheffield Children's Hospital.

Breathlessness and Inactivity: Breaking the Circle
"Exercise? You must be joking! I can't even run ten yards without gasping for breath!"

Breathlessness is common in people with antibody deficiency and is particularly disabling in cases of severe bronchiectasis (see page *). Indeed, many people avoid trying to become breathless because it is an unpleasant feeling and they believe it is harmful. Unfortunately, this can lead to a vicious circle of inactivity as they tend to avoid effort and exercises-which results in a loss of physical fitness, which in turn leads to worsening breathlessness on exertion, which results in...and so on.

In this way, people may progressively restrict their activities until they feel unable to work or even leave the house.

Almost anyone with antibody deficiency can benefit from an exercise programme, whether or not they have lung damage. Getting puffed easily doesn't necessarily mean that you shouldn't take regular exercise. You may simply have become unfit.

Remember, the right sort exercise does you no harm at all-and can do you a great deal of good. Even when it causes breathlessness, exercising can:

Increase the fitness of your muscles
Reduce the fear of breathlessness
Reduce the feeling of breathlessness
Increase your ability to exercise
Improve your well-being and self-confidence
Break the vicious circle of inactivity

However, if you experience any chest pain or severe breathlessness while exercising, stop immediately and consult your doctor before starting again.

"I have Common Variable Immunodeficiency and cycle to and from work, winter and summer. My lung function tests are near normal."

Not everyone can aspire to such levels of fitness, but most of us can go part of the way.

The key to improving your fitness is to take the right sort of exercise for you and to follow an exercise programme, however simple. You can exercise almost anywhere: around the house, in the street, on flat ground or using the staircase at home. If you're able to get out and about, you may be able to swim, go for bike rides, exercise in the gym or do aerobics. However, check with your doctor or physiotherapist first if you haven't done any of these for a long time.

You can design your own programe to meet your needs and facilities. Just follow these simple guidelines:

Exercise regularly: ideally every day, although three times a week is better than nothing.
Don't be too ambitious at first! Build up your exercise programme gradually. This will prevent painful muscles, unnecessary breathlessness and loss of confidence.
Begin by exercising for a few minutes, enough to make you slightly breathless, but not enough to make you stop.
Gradually increase the distance you cover or the time during which you exercise as the days and weeks go by. Often, you can double the amount of exercise you take in just a few weeks.
Set yourself weekly targets and keep a record of your progress, so that you can see how you are getting on.
Take a positive attitude to life!

Exercises for Children
Useful exercises for young children include trampolining (on a small trampette), skipping, star jumps and squat jumps. A child's exercise programme should always be enjoyable and safe, and should avoid excessive breathlessness.

"At the age of 14 I was selected for both the school hockey team and the local hockey team."

If you're the parent of a child with antibody deficiency, encourage your child to participate in PE and games at school where possible. Joining a sports club is another possibility. However, make sure that the teacher or instructor is aware of your child's limitations.

Sharing an activity, such as going swimming together, is a good way to maintain the physical fitness of the whole family.

If You Have Severe Chest Problems
"I'm 50 and my consultant says that because I wasn't diagnosed for a long time, my lungs have been damaged. I can barely walk from one room to another, so all this talk of exercise is pointless!"

If there has been a delay in diagnosing antibody deficiency, your lungs may have been damaged through persistent and severe respiratory infections. In some cases the airways (bronchi) become distorted and produce large quantities of infected sputum. This condition is called bronchiectasis.

These symptoms can reduce your ability to exercise actively. In this case, you'll need more specific exercises to relieve them. The remaining sections of this booklet show you simple exercises to improve your breathing and clear sputum from your chest.

Chest Exercises (1): Improving Your Breathing
Even if you can't manage to take active exercise, breathing exercises are essential to keep your lungs clear and free of infection. Practising breathing exercises can help you to:

Your physiotherapist can show you positions to help reduce your breathlessness when it is bad. However, here are two simple exercises that will help your general breathing: breathing control and deep breathing.

Relaxed Breathing Control
This exercise will help you to control breathlessness, particularly after coughing, huffing (see page *) or taking active exercise.

Relax your head, neck and shoulders. Your physiotherapist can show you some exercises to achieve this.
With your shoulders dropped, place your hands across your stomach just below your ribs
Breathe out gently through your mouth.
After a long breath out, breathe in gently, keeping your shoulders relaxed.

This exercise should not tire you. If it does, ask your physiotherapist for advice, as you might not be doing it correctly.

Deep Breathing
This is an exercise to help you make better use of the lower parts of your lungs.

Relax your head, neck and shoulders and place your hands across your stomach, as in the previous exercise.
Breathe out gently.
Keeping your shoulders relaxed, take a deep breath in, feeling your ribs rising under your hands.
Hold the deep breath for a second, then breathe out gently through your mouth.

A child can be encouraged to take deep breaths through the use of blowing games (for example, blowing tissues) or even by playing a musical instrument.

Chest Exercises (2): Clearing Sputum
Sputum and coughing are common problems in people with lung damage. The lungs produce excessive quantities of sputum, which have to be cleared from the chest.

Thick, sticky sputum can cause long, tiring and painful bouts of coughing, particularly in the early morning.

In many people with bronchiectasis, the sputum is normally transparent with a white or greyish tinge and is free from infection. However, acute infections of the airways-bronchitis-cause the sputum to thicken and turn yellow, creamy or green. Bronchitis is best treated as early as possible with an antibiotic prescribed by a doctor. A sputum sample should be sent to your GP or hospital. In the case of a child, a cough swab may be easier to obtain.

Exercising to Clear Sputum
Although it isn't possible to stop the production of excessive sputum, you can improve the way it is cleared from your chest.

Relaxed breathing control to avoid tightness of the chest and exhaustion
Deep breathing exercises to loosen the sputum
Huffing to move the sputum along the airways

If you produce only about two or three tablespoonfuls of sputum a day, you can do the exercises either sitting or lying down, first on one side and then on the other. However, if you have bronchiectasis and produce more than one cupful of sputum a day, you will need to lie in positions in which gravity helps to drain the affected areas of the lungs. This is often known as postural drainage.

A physiotherapist must advise you on the postural drainage positions that are most appropriate for you.

Clearing Sputum in Children
Babies and small children will swallow their sputum, but as soon as a child is old enough it should be encouraged to expectorate.

Postural drainage for small children is best performed across the parent's knee, before a feed. As the child grows older, postural drainage can be performed over a wedge of foam or pillows. It's often difficult to persuade a toddler to remain in one position for any length if time. However, running or jumping up and down between short sessions of postural drainage can actually help, as they often stimulate a cough.

Remember to seek advice from your physiotherapist before practising postural drainage on your child.

Huffing
Huffing, also known as the forced expiration technique, helps to remove sputum more effectively than coughing. It's less exhausting than coughing, too.

To produce an effective huff, follow these steps:
Take in a small breath.
With your mouth open, squeeze the air forcefully out of your lungs, as though you're breathing onto a mirror before wiping it clean, or as though you're doing a peak flow. Your stomach muscles should contract firmly, but your throat muscles should not tighten.

The huff must be long enough to move the sputum up from the smaller, deeper airways into the larger, higher airways. If it is too short, it won't be as effective.

An essential part of the huffing technique is to allow a short period for relaxation and gentle breathing control after every one or two huffs. Between 15 and 30 seconds should be enough. This will prevent the feeling of tightness in the airways and chest, and allow you to relax and recover your breath comfortably. Remember, relaxed breathing control is also helpful after a bout of coughing.

A Suggested Programme for Clearing Sputum
Here is a suggested series of exercises for clearing your chest:

Relax and breathe gently using the relaxed breathing control method.
Take three or four deep breaths, breathing out quietly.
Pause for some relaxed breathing control.
Huff once or twice.
Pause for some further relaxed breathing control.

If you use a bronchodilator inhaler, use it just before you do these exercises.
If you have a small amount of sputum, you may be able to clear it by doing this sequence of exercises for five or ten minutes while you're sitting in a chair. However, if you develop an acute infection which causes an increase in sputum, you may need to do the exercises lying on a firm surface for about ten minutes, first on one side and then on the other.
Always clear your chest every morning. A hot drink may help you if you wake feeling dry.
If you have a lot of sputum, clear your chest in the evenings as well, before going to bed. This may help you sleep.
If you have bronchiectasis, seek advice from your doctor and physiotherapist about what positions to use and how often during the day you should do physiotherapy.

Other useful ways to clear sputum include the Flutter valve and the PEP mask. For more information, contact your physiotherapist.

If you have any questions about your chest condition, contact your doctor, immunology nurse or physiotherapist.