Treatment Options for Alpha-1 Lung Disease
Updated: April 28, 2009
Augmentation / Replacement Therapy
There are now three medications - augmentation or replacement therapies as they are called - that have been developed specifically for the treatment of Alpha-1 lung disease. The aim of these drugs is to replace or augment the missing alpha-1 antitrypsin (AAT) protein and in this way, hopefully, slow or stop the progress of disease. All are given by intravenous infusion once a week.
A statement by the Canadian Thoracic Society on augmentation therapy describes their use - Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: A Canadian Thoracic Society clinical practice guideline
Prolastin alpha-1 replacement therapy from Grifols (formerly Talecris)
Zemaira alpha-1 replacement therapy from CSL Behring
Aralast alpha-1 replacement therapy from Baxter Healthcare
Please Note: The only one currently approved for use in Canada is Prolastin.
Other research projects are ongoing to try to develop augmentation / replacement medication that can be taken by inhalation but none is yet on the market.
Bronchodilators are used to open up the narrowed breathing passages. The same airway opening inhalers used by people with chronic obstructive lung disease (COPD) are used by Alpha-1 emphysema patients. These medications fall generally into two categories:
These drugs work on the narrow passageways and neutralize the nerve pathways of the cholinergic system in order to stop spasm of the small muscles that encircle airways. Anticholinergic drugs include: short-acting Atrovent (ipratropium bromide) and long-acting Spiriva (tiotropium).
These drugs have a different target than the anticholinergics. Beta2-agonists target the beta2 receptors in the muscles encircling the airways. This is a safe and selective version of the well known drug, adrenaline. Drugs in this category include: short-acting bronchodilators such as Ventolin (salbutamol), Airomir (salbutamol), Apo-salvent (salbutamol), Bricanyl (terbutaline), Beretoc (fenoterol), and long-acting bronchodilators such as Oxeze (formoterol), Foradil (formoterol) and Serevent (salmeterol).
These drugs are used to reduce inflammation and are not ‘rescuers’ as are the bronchodilators. They are used mainly to prevent inflammation.
These can be taken by inhaler and go directly to the lungs and do not get into the blood system. Drugs in this category include: Flovent (fluticasone), Qvar (beclomethasone), Alvesco (ciclesonide) and Pulmicort (budesonide).
Prednisone is taken orally and is a powerful anti-inflammatory. It is sometimes used when there is an exacerbation or flare-up of Alpha-1 COPD.
When people routinely inhale two drugs regularly, they may be given a combination inhaler. At present, the beta2agonist bronchodilators and the inhaled corticosteroids are commonly combined this way. In Canada, available combinations of this type are Advair (fluticasone, salmeterol) and Symbicort (budesonide, formoterol).
People with Alpha-1 may suffer from lung damage during chest infections and need to treat them early and aggressively so that damage is minimized.
Supplemental or home oxygen may be required if a person’s oxygen levels are persistently low. There are currently three common types of delivery systems.
Oxygen is stored in liquid form in a reservoir that looks a little like a three-foot tall robot. Oxygen tubing can be hooked up to it directly or it can be used to fill portable canisters. Portable canisters are becoming more user-friendly and compact. The canisters are usually of two types: continuous flow where oxygen is flowing both as the person inhales and exhales, and on-demand or pulse systems that release oxygen only on inhalation. This newer technology allows the oxygen to last almost double the time of the continuous systems.
The concentrator is a machine about the size of an end table. It is plugged into a wall outlet and extracts oxygen from the room air. It is the least expensive oxygen delivery system but is restricted to in-home use. Its other disadvantage is that it is rather noisy and can run up the electricity bill.
Cylinders contain oxygen in the form of compressed gas and can be fitted with a conserving device to prevent the escape of oxygen so that they can last a long while if not in use. Cylinders can be portable but are quite heavy and must be pulled around on a cart. They are the type most often seen in hospitals for ambulatory patients.
Flying in airplanes with supplemental oxygen is possible, however, there are several issues to consider. For more information please read our page on flying with oxygen.
Lung Volume Reduction Surgery (LVRS)
Lung Volume Reduction Surgery (LVRS) involves cutting out those parts of the over inflated lung that are no longer performing their function. The theory behind this is that by removing the useless tissue, the remaining healthy tissue will have more room to expand and work more efficiently. This surgery is thought not to be very appropriate for people with Alpha-1 as the damage to their lungs is mostly in the lower lobes whereas LVRS is usually performed on the upper lobes. There is a study underway currently to assess the value of LVRS. It involves a cross-section of people with emphysema, not just those with Alpha-1. More information on this study can be found here.
Lung transplantation is a treatment for people with severe Alpha-1 emphysema and is available in selected centres across the country. Depending on the circumstances, an individual may undergo a single or double lung transplant. While life expectancy after transplant is increasing and surgical techniques and medications are constantly being refined, it is still a serious step and is not a cure for Alpha-1. (Remember that most alpha-1 antitrypsin is produced in the liver. You may get a new lung but you have not changed your liver; you will still have Alpha-1.)
In one sense, individuals are exchanging one disease for another, as they will require lifelong medication and medical attention after they have undergone a lung transplant. For more information on lung transplants and life after transplant, visit the Second Wind website.
One of our community members had a double lung transplant in 2008. She wrote about the process in detail and generously allowed us to publish it. Please read “Sharon’s Extreme Medical Adventure.”
This website is designed to support, not replace, the relationship that exists between you and your physician.
It is not the intention of this website to provide specific medical advice but rather to provide the Canadian Alpha-1 Community with information to better understand their health and their diagnosed disorder.
Specific medical advice will not be provided and Alpha-1 Canada urges you to consult with a qualified physician for diagnosis and for answers to your personal questions.