Living with Alpha-1

Living with Alpha-1

It is very important that 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 given here 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 Alpha-1 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 Alpha-1 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 Alpha-1 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 Alpha-1 Antitrypsin (AAT). A single cigarette destroys all the Alpha-1 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.

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
  • Aerosols
  • Powders
  • Dust and dirt
  • Bacteria
  • Mould and mildew
  • Household cleaners
  • Insecticides
  • Fireplace smoke
  • Asbestos
  • Radon gas

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 Alpha-1 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.
  • 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 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, spirit-based 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.

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;

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.

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 least 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:

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 Alpha-1. 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.
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 bacteria.