Alpha is a protein that belongs to the serpin superfamily. Serpins are proteins that are identified as protease inhibition activity. It is a genetic condition passed on from parents to their children through genes.
Sometimes it’s a serious lung disease or liver disease in adults. There’s at least 100,000 people with Alpha. The most common signs and symptoms are; Shortness of breath, Wheezing, Chronic cough and sputum, Chest colds, Decreased exercise tolerance, Asthma or allergies, Bronchiectasis, Eyes and skin turn yellow and Swelling of the abdomen. Alpha is identified in all populations. 1 in every 2,500 Americans have it. 19 million people in the U.S. have one normal and one defective Alpha gene.
The people who have that, are called carriers. If there a parent, they would be a carrier which means they pass it through their children. Emphysema is the most risk factor. If you are diagnosed with COPD, emphysema or asthma, get tested for Alpha. The most serious liver diseases are cirrhosis and liver cancer. You need to take a blood test to find out if you have it. Another name for Alpha is AAT. AAT can’t get to the lungs so what happens is that enzyme fights infections when left unchecked, the enzyme damages the lung tissue which then causes emphysema.
116,000 carry Alpha worldwide. Most people with Alpha are European. If you smoke with or not with Alpha, you should stop or it will put inflammation in your lungs. The Alpha organization was found in 1991. The symptoms begin at 50 to 60 years old and sometimes early in life. If you have red, warm lumps under your skin, you may have Alpha. If your family history had Alpha, you may want to get checked.
Lung disease doesn’t happen until you’re 30 to 40 years old but if you smoke, it will happen earlier. If you barely have AAT for example just normal liver and lungs are good, you don’t need a treatment but you shouldn’t drink and exercise. You’ll need to get a shot every five years and you should also get a flu vaccine every year. If you have lung disease, you would have to do therapy unless you smoke.
If you do therapy, AAT will increase blood levels but sometimes you would need to get surgery. If you have liver disease, you may need a liver transplantation. Its uncommon in asians. Sometimes inhaled steroids can help lung issues.
Emphysema is when you can’t breathe or when its hard to breathe. Cirrhosis is when the liver is scarred. Scarred means it prevents the organ from working.
The disease that make lumps under the skin is called necrotizing panniculitis. The Alphas protein goes in the bloodstream. The protein helps the organs from the bad proteins. The lungs are the main organs that AAT protein protects. People with AAT deficiency who have liver or lung disease die each year.
About 3 percent die from liver or lung disease. Once again if you’re smoking, you’ll have about 20 years. AAT proteins that are in the liver aren’t the right shape so then they get stuck in the liver cells, so that’s when the proteins can’t get to the organs that the body protects like the lungs. If the AAT proteins were not protecting the organs, there will be diseases. A faulty gene which is most common can cause AAT deficiency is called PiZ. If one from each parent has two PiZ genes, there’s a chance you have AAT deficiency. If one parent has a PiZ gene and a normal AAT gene is from another parent then you won’t have AAT deficiency unless you pass it to your children.
Even if you weren’t smoking, the risks for lung disease will probably go up if you’re exposed to dust, fumes or other toxic substances. If you have lung disease, the doctor will do a CT scanning which is a high resolution computed tomography scanning. The doctor will also do lung function tests. Lung function tests measure how much air you can breathe in and out, how fast and how well your lungs give oxygen to your blood. All of it will show how bad your lungs are or how your treatment is working. CT scanning is a x ray of your whole body, it shows if you have emphysema or a lung disease. If you are having symptoms, you doctor will give you medicine called inhaled bronchodilators and inhaled steroids, it will open your airways so you can breathe easier. It can also treat Asthma and COPD.
The flu and pneumococcal are vaccines that will keep the illness from getting worse. Pulmonary rehabilitation is a rehab which will help you be your best. If your oxygen is really bad, then you can get more oxygen. If you have breathing problems, you can get a lung transplant.