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Alpha-1 Lung Disease
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Alpha-1 lung disease is basically genetic emphysema. The first indications are shortness of breath with exertion. A family history of relatives who died young with lung disease or liver disease with little or no history of drinking are strong indicators of the genetic link. It is caused by a defect in your genetic make-up and spurred by smoking, exposure to irritants and/or other chemicals that you may have been exposed to. Several Alpha doctors have speculated that there is another genetic factor that triggers this effect on the lungs, but to date it has not been identified. Many patients have never smoked but most can identify some source of irritant that they have been around at some point in their life (second hand smoke, cleaning fluids, excessive use of chemicals). Treatment for Alpha-1 is similar to regular emphysema in many ways. Patients must stop smoking, eat healthy diets and exercise often to stimulate muscle and bone strength. Patients are encouraged to stay away from people with colds and to wash their hands often. They must find a good pulmonologist to treat any infections quickly and to monitor the progression of the disease. If needed, augmentation therapy (Aralast or Prolastin) will be initiated by your pulmonologist and given routinely by IV therapy. These plasma products replace the lacking protein in your blood. You can read more about these products at their web sites. Though clinical trials were not carried out on these products, the FDA approved them and many patients have had success and remained healthy for many years. It is up to you and your doctor whether you will begin this therapy. For an in-depth description of Alpha-1 lung disease, advance to the following web site: http://www.thedoctorsdoctor.com/diseases/a1at_deficiency.htm Your doctor will probably order lung function tests (PFT's) . The following web site offers a good explanation of each of the tests as well as a view of the lungs. http://www.vh.org/adult/provider/internalmedicine/Spirometry/SpirometryHome.html We always recommend that you ask the technician to make an extra copy of the test results for you. You should take this copy home with you and begin a personal medical file if you do not already have one. Any time you have a test taken, ask for a copy of the test results right then or ask the doctor to mail a copy to you. You are entitled to these and make it much easier to refer to when you have questions later. Keeping an active role in your own health care is imperative.
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