Alpha 1 Awareness UK
Alpha1
Antitrypsin
Deficiency
Living With Alpha1
A lifestyle guide for
people diagnosed as
having the genetic
condition Alpha1
Antitrypsin Deficiency
www.alpha1awareness.org.uk
It is very important for Alphas to do everything possible to protect their
lungs and liver and to maintain overall good health. Your medical
specialist can give you advice on how to do this. Always take note of this
advice.
The advice is generally addressed to Alphas (PiZZ) but there is a growing
appreciation that carriers (PiMZ) are also at increased risk for disease
development. Therefore, we advise carriers to manage their environmental risk
factors to the same extent as Alphas.
Each Alpha is different. Your condition, your symptoms and how your body
reacts to stress and irritants are almost certainly quite specific to you. Learn
from your body. Become the expert on yourself.
Risk Factors - Smoking
First among the controllable risk factors associated with development of lung
disease in Alpha1 is exposure to cigarette smoke. Whether from personal use
or secondhand exposure, cigarette smoke has been undeniably shown to exert
the greatest risk.
For the smoker, inhaling cigarette smoke is known to accelerate the destructive
processes in the lungs and for someone with Alpha1 Antitrypsin Deficiency
(AATD or A1AD) the damage done to the air-sacs in the lungs leads to
emphysema. But it is not only the risk to the smoker that is of concern.
Secondhand smoke can also cause considerable damage to the lungs of Alphas
who do not smoke themselves.
In addition, children of smokers appear to have a much higher incidence of
asthma than children of non-smokers. If your child is either an Alpha or an
Alpha1 carrier, the risk of developing lung disease in later life will be greatly
increased by exposure to secondhand smoke.
The chemicals created from burning tobacco destroy Alpha1 Antitrypsin
(AAT). A single cigarette destroys all the Alpha1 Antitrypsin in the lungs.
Because each cigarette smoked results in a repeated episode of irritation and
inflammation, this process may therefore account for a significant portion of
the lung disease seen even in the general population. Among individuals with
normal AAT levels, this damaging process generally occurs gradually. The
symptoms of lung disease in these individuals, if they occur, tend to develop
during their 50s or 60s. Alphas, with their reduced AAT levels, may develop
these symptoms as early as in their 30s.
Although cigarette smoking is recognised as a controllable risk factor, it is
clear that, in many cases, it is not an easily avoidable one. Some Alphas live
with smokers who are unwilling to quit. Although some Alphas never take
another puff of a cigarette after they are diagnosed, this is not always the case.
It is important to understand that dealing with a cigarette addiction, as with any
addiction, may be very difficult. Successful change takes knowledge, help from
others, and a long-term commitment to health.
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Risk Factors – for Your Liver
Chief among the substances known to cause liver damage is alcohol. Excess
alcohol consumption is by far the most common cause of toxic chemical
damage to the liver in our society as a whole. Other agents that are inhaled,
swallowed, or absorbed through the skin can also cause damage to the liver.
Among these are solvents, toxic alkaloids, pollutants, dietary supplements, and
prescription and over-the-counter drugs.
Many medications have been implicated as a potential cause of liver damage.
The widely used pain medication paracetamol is one such drug. Paracetamol is
generally safe when taken as prescribed. However, when taken in excessive
doses, either all at once or over a period of time, it can cause severe damage to
the liver. If you drink alcohol then you should at least halve the recommended
dosage of paracetamol.
Risk Factors – In the Home
Aside from tobacco smoke, other potentially harmful substances encountered
in the home environment include
 Fumes

Household cleaners
 Aerosols

Insecticides
 Powders

Fireplace smoke
 Dust and dirt

Asbestos
 Bacteria

Radon gas
 Mould and mildew
There is no conclusive evidence showing a direct relationship between the
development of specific lung diseases and exposure to any of the common
substances found in the home. However, people who are sensitive to
substances such as cleaning products, soaps, deodorants, and cosmetics may
experience respiratory difficulties with exposure. People with Alpha1 or those
with known or suspected allergies may experience difficulty breathing when
exposed to pet hairs, mould, mildew, pollen, or other airborne particles.
Persistent exposure by sensitive individuals to any of these elements may lead
to long-term respiratory problems.
Fumes from badly vented paraffin stoves, gas heaters. Some building
materials can give off small amounts of formaldehyde (chip board,
fibreboard and some plywood).
• Consider using a mask whenever the potential for breathing harmful fumes
exists. See the note below on the use of masks.
• Open windows and increase ventilation when you are in areas where fumes
may accumulate or install extractor fans. Remember to inspect fans and
clean them routinely.
• When working on your car in a garage, keep the garage door open and
prevent exhaust fumes from entering your home.
• Keep lids secure on solvent containers. Always use a mask and gloves when
handling these substances to avoid or reduce inhaling the fumes and
protect your skin from contact.
• Have heaters inspected and serviced regularly. Check pilot lights on gas
heaters and stoves to make sure the gas flame is burning blue, not yellow or
orange. Consider using a different form of heating.
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• If you are having construction or renovation work done in your house then
request that spirit based solvents are not used, or at least are only used
when you are absent, and with as much ventilation as possible at the time.
Aerosols.
• Aerosols include deodorants, hair sprays, perfumes, air fresheners and
spray disinfectants.
• Use roll-on deodorants, pump sprays, solid air fresheners, and non-toxic
alternatives.
Talcum powder is readily airborne and can be inhaled, causing irritation
and potential respiratory problems.
• Avoid use or powder sparingly to avoid breathing airborne particles. If you
must use powder, consider using a mask.
Household cleaning/insecticidal products, ammonia-based products, spiritbased products, oven cleaners, bleach, spray furniture polish, paint sprays,
and mildew remover sprays. Use cautiously as these products pose a threat to
your liver, as well as your lungs.
• Minimise your use of toxic chemicals. Seek less toxic or more natural
alternatives as much as possible. For example, baking soda or a solution of
vinegar and water serve quite well as household cleaning products.
• Use commercially available pre-moistened dusting/polishing cloths or a
cloth slightly dampened with water.
• Use extra caution when using chemical sprays — make sure the room is
well ventilated and wear a mask.
• Be aware of the nature of chemicals that come in contact with your skin,
because these can be absorbed through the skin and affect your liver. The
insecticides used for trees and shrubs not only kill bugs, but can be
absorbed through the skin and damage the liver. Use gloves, a mask, hat,
and protective clothing every time you handle these substances. Protect
your skin from exposure as much as possible.
• If irritating chemicals must be used, arrange for someone else to do the
cleaning and leave the home until the fumes have dissipated.
Dust and dirt can accumulate quickly and cause difficulty in breathing.
• Avoid all dusty situations, including shaking rugs, vacuuming, sweeping,
and dusting. Have someone else do the cleaning if possible. If you must be
the one who does the dusting, wear a mask.
• When cleaning areas where dust and dirt routinely accumulate, use a damp
rag or mop to reduce airborne particles. Remember, many household
appliances collect dust and other irritants and should be cleaned regularly.
• You may wish to consider the use of a HEPA (High Efficiency Particulate
Air) filter in your home. Vacuum cleaners are available with HEPA filters.
Open fireplaces and wood burning stoves pose risks for individuals with
respiratory difficulties. Consider alternatives
• Provide for cleaning of the chimney on an annual basis, or more frequently
if needed.
• Before lighting the fire, make sure the damper is open. For a wood stove,
be sure it is in proper working condition, the seams are tight and sealed,
and the joint to the stack is in good condition.
• Burn only firewood, not paper, charcoal, nor other items that can cause
toxic fumes.
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Bacteria, mould and mildew can accumulate in areas in the home that are
damp, especially bathrooms, kitchens, basements and garages. (The soil of
house plants is also known to support the growth of mould and mildew.)
• Increase ventilation in bathrooms and kitchens with fans vented to the
outdoors. Wash tile and grouted surfaces frequently. Re-grouting may be
needed occasionally.
• Wash and replace sponges used in the kitchen and bath frequently,
especially during the cold and flu season. If you want to soak them in a
bleach solution then get someone else to do it for you. Replace soiled hand
towels with fresh ones routinely.
• Seal leaks and waterproof basements. Wipe up any leaks or standing water
as soon as possible.
• Clean humidifying/dehumidifying units regularly. Empty water trays in air
conditioners, dehumidifiers, and refrigerators frequently.
• Clean and dry water-damaged carpets, or remove and replace them
altogether.
• Consider growing indoor plants in an enclosed vivarium.
Asbestos and radon gas (rare in the UK but this gas is sometimes detected in basements).
• This is a specialised area. If you have any concerns then seek expert help.
• The Health Protection Agency will be able to advise you; www.hpa.org.uk.
Risk Factors – Outdoors
Here you have less control over fumes and other irritants that other people
accidentally or deliberately put into the air. The general advice is to think
ahead and remove yourself from the polluted area as soon as possible.
Watch bonfires and fireworks from a safe distance, for you this may be more
than others may consider safe. Be careful with barbecues especially when they
are being ignited using solid or liquid firelighters.
When travelling by car you may be caught by fumes from other vehicles. If
you are in a traffic jam or in a long tunnel close all windows and switch the air
control to RECIRCULATE. When your car is serviced make sure that the air-filter
for the interior is in good condition. If you need to fill up with fuel do not do so
when a tanker delivery is being made at the service station.
Heed any air quality warnings on the radio or television, especially in the hot
summer months. If ozone levels are high, try not to venture outside and avoid
excessive physical exertion.
Avoid contact with anyone who has a cold or flu. In addition, try to stay away
from crowds and large gatherings, especially in winter.
Health – Exercise
Exercise can improve your mental outlook as well as your physical health. An
exercise routine is critical for all Alphas, even those with minimal or no
symptoms. A pulmonary rehabilitation exercise programme is recommended
for Alphas with lung problems. These programmes include exercise, breathing
retraining, education, dietary advice, and when necessary, help to stop smoking.
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Health – Hygiene
Frequent and thorough hand washing, specifically after touching surfaces
frequently used by others, coughing or sneezing, and handling soiled tissues,
will greatly reduce the potential for infection. Hand washing after using the
toilet, changing nappies, and before handling or preparing food is extremely
important to reduce your potential for spreading illness and infecting others.
Studies have repeatedly confirmed hand washing is the best first-line defence
against the spread of infection. Good hand-washing technique is not related to
using an expensive antibacterial soap. The chief benefit of hand washing comes
from the friction of rubbing your hands together and thorough rinsing.
Alphas who use nebulisers, inhalers and masks should pay particular attention
to keeping the apparatus clean. Advice on how to do this will be written in the
manufacturer’s user guide (or instruction sheet included with each inhaler). If
you use an inhaler regularly then you may think that you are familiar with the
manufacturer’s instructions. Occasionally, re-read the paper that comes with
the inhaler to confirm that you are getting the best out of your medication.
Oral hygiene is especially important for users of inhalers and nebulisers. The
active drugs and sometimes the propellants can irritate the gums, tongue or
throat. Rinsing the mouth with water can stop this but if a problem persists then
seek medical advice.
Masks can be very useful to protect you from air-borne infections, dust, pollen,
etc. The simple tie-on mask you may have seen used in hospitals is designed to
prevent the wearer from spreading infection to others. It does not prevent the
wearer from acquiring airborne infection from others. When buying masks to
filter at lease 94% of airborne particles look for the following markings: EN
143 class P2, EN 149 class FFP2 or NIOSH N95. For protection from aerosols
the higher standard FFP3 is recommended. If your work involves working with
hazardous materials your employer must provide suitable masks or respirators.
Contact the Health and Safety Executive for more details: www.hse.gov.uk.
Health - Diet
A healthy diet is an important part of optimising short term and long term
health for Alphas. Eating a balanced diet provides us with the range of
nutrients our bodies need and also helps maintain a healthy weight. Being a
healthy weight (categorised as a BMI of between 20 and 25) can help reduce
the risk of developing other conditions such as heart disease and diabetes but
can also help your Alpha1. Being underweight can increase risk of developing
infections (including those of the respiratory system). Being overweight can
put increased pressure on vital organs (including the liver and lungs) and
therefore make them work harder. If you are concerned about your weight,
speak to your GP who may refer you to a Dietician.
An important aspect of a balanced diet is dietary fats. There are different types
of fats found in the diet and the balance of these is essential for achieving good
health. Cakes, biscuits, takeaways, butter, cream, meat fat and poultry skin are
examples of foods that are high in saturated fats. These fats increase the levels
of cholesterol in the blood which collect on the inside of blood vessels and
restrict blood flow to and from major organs. These foods should be kept as
occasional treats. There are two main fats that are classed as good fats.
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Monounsaturated fats can be found in olive oil, rapeseed oil and margarines
made from these oils. They actually help to lower cholesterol levels in the
blood and should replace butter, lard and dripping. Omega 3 fats help to protect
the body by making the blood less likely to clot and can be found mainly in
oily fish (salmon, mackerel, trout, sardines, pilchards, fresh tuna) but are also
found in smaller quantities in other foods. As part of a healthy diet it is
recommended that we should aim to eat one portion of oily fish per week in
order to help protect our heart. It is worth remembering that all fats are high in
calories, even the good ones. Olive/rapeseed oil and margarines based on these
oils need to be used sparingly for those who are wishing to reduce their weight.
Vitamins and minerals are essential nutrients that have many roles within the
body. They help tissue function and structure, vision, bone strength, circulation,
nerve transmission, metabolism and help prevent oxidative damage. A large
proportion of vitamins and minerals can be found in fruit and vegetables.
Eating five portions of fruit and vegetables each day ensures that we get
sufficient quantities and variety of vitamins and minerals. Examples of a
portion are:
 One medium piece of fruit (e.g. apple, orange banana)
 Two smaller fruits (e.g. kiwi fruit, satsuma)
 A handful of berries or dried fruit
 A serving spoon of vegetables
 A bowl of salad
 One glass of fruit juice (150mls)
There is no need for vitamin and mineral supplements if you have a well
balanced diet and reach your five a day for fruit and vegetables. High doses of
certain vitamins and minerals can sometimes be dangerous.
Health – Help your Doctor
If your doctor is not already aware that you are an Alpha then tell him/her
immediately about your condition.
Since lung infections can be very serious for an Alpha they must be treated at
the first signs. Even if the infection is caused by a virus there may be
complications from bacteria and this can be treated by antibiotics. Many
Alphas keep a small supply of antibiotics in their homes
For severe exacerbations your doctor may give you a tablet or an injection of a
corticosteroid such as prednisolone. After this treatment you may be given
tablets to take over a number of days in reducing amounts. It is most important
that you stick to the recommended programme
A yearly flu vaccination is recommended for individuals who are at increased
risk for serious complications from the flu; these include lung-affected alphas
of any age. Additionally, it may be prudent for individuals living in the same
household to have a yearly vaccination.
Ask your GP if he/she recommends a 5-yearly vaccination against
pneumococcal viruses.
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Alpha1 Awareness UK
Alpha1 Awareness UK is a registered charity (1125467)
Postal address:
Alpha 1 Awareness UK
PO Box 2094
Bristol BS35 9DE
Email: [email protected]
Website: www.alpha1awareness.org.uk