Augmentation / Replacement Therapy
There are three medications – augmentation or replacement therapies as they are called – that have been developed specifically for the treatment of Alpha-1 lung disease. Prolastin is currently the only augmentation or replacement therapy approved for use in Canada. 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: CTS Alpha-1 Guidelines 2012
Prolastin alpha-1 replacement therapy from Grifols
Zemaira alpha-1 replacement therapy from CSL Behring
Aralast alpha-1 replacement therapy from Shire
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). 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.
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.
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.