Living With Alpha-1

1. Living With Alpha-1 s Jessica Morris

2. What is Alpa-1? • Alpha-1 antitrypsin deficiency is a genetic disorder that causes lung and/or liver disease. (Also known as Alpha-1 or AAT) • Alpha-1 antitrypsin is a protein made in the liver that protects the organs in your body. • The main organ it protects is the lungs.

3. Who is Effected by Alpha-1? • Alpha-1 is in inherited disorder that is often times misdiagnosed as COPD or Asthma. • It is estimated that Alpha-1 affects 1 out every 2,500 people in the US. • An estimated 20 million people in the United States are undetected carriers of an abnormal gene that causes Alpha-1, and may pass the gene on to their children.

4. Symptoms of Alpha-1 • Shortness of breath • Wheezing or non-responsive asthma • Coughing with or without sputum (phlegm) production • Recurring respiratory infections • Rapid deterioration of lung function • Unexplained liver problems and /or elevated liver enzymes

5. Treatment Options • Augmentation Therapy-IV injections of the protein. • Bronchodilators-albuterol , and steroids are some examples. • Supplemental Oxygen • Transplantation • THERE IS NO CURE FOR ALPHA-1!!

7. Questions & Answers Health Connections

8. Why it is important for individuals with Alpha 1 to get treatment even before showing signs • By the time the patient shows signs there is already significant lung damage. • With the recent increase in knowledge of Alpha 1 it now takes an estimated 5 years and 3 doctors to get diagnosed. By this time most have already lost over 50% of lung function • Treatment includes weekly infusions of Prolastine. • Prolastine is used in augmentation therapy . • Prolastin is a protein, also called alpha 1-antitrypsin. This protein occurs naturally in the body and is important for preventing the breakdown of tissues in the lungs. • In people who lack the alpha 1-antitrypsin protein, breakdown of lung tissues can lead to emphysema, chronic bronchitis, and COPD.

9. Prolastin • Although prolastin injections will help slow the progression of Alpha 1 and help extend someone's lifespan. It isn’t suggested as an automatic treatment option because it is highly expensive . • However, receiving early infusions will lower the need for transplant, because lung function less then 30% makes you eligible for transplant and disability.

10. The cost of transplantation Medical costs include: • insurance deductibles • insurance co-pays • pre-transplant evaluation and testing • surgery • fees for the recovery of the organ from the donor • follow-up care and testing • additional hospital stays for complications • fees for surgeons, physicians, radiologist, anesthesiologist and recurrent lab testing • anti-rejection and other drugs, which can easily exceed $2,500 per month • rehabilitation Non-medical costs include: • food, lodging and long distance phone calls for you and your family • transportation, to and from your transplant center, before and after your transplant • plane travel to get to your transplant hospital quickly • child care • lost wages if your employer does not pay for the time you or a family member spends away from work • If your transplant center is not close to your home, lodging close to the center before and after your surgery. Some centers offer free or low-cost hospitality houses for you and your family

11. TransplantCostChart

12. Transplantation odds • The number of people needing a transplant continues to rise faster than the number of donors. • 4,100 transplant candidates are added to the national waiting list each month but each day, about 77 people receive organ transplants. • However, as many as 18 people die each day while waiting for transplants because they are unable to take place because of a shortage of donated organs. • An extreme number rising to 105,000 on the waiting list for solid organ transplants

13. The cost and effectiveness of Augmentation therapy vs. Transplant • The Cost of Augmentation therapy is expensive, $25,000 a week, with the protein, nurse, etc..almost $30,000 a month. This is the main obstacle for most insurances and why they don’t want to use augmentation therapy as a for the first line of treatment options • For Lung transplant and individual is looking at anywhere from between $450,400 – $657,800 this includes; 30 days Pre – transplant, Procurement, Hospital admission, physician, 180 day post admission, and immunosuppressant's. Add that on top of the liver transplant that maybe needed at $523,400 for a total amount of either $973,800 or 1,181,200

14. Acknowledgements • Alpha-1 Association • http://www.alpha1.org/whatisalpha1/drugtherapy.php • www.drugs.com/mtm/prolastin.html • http://www.transplantliving.org/beforethetransplant/finance/costs.aspxh • http://www.umcaz.org/body • http://www.alpha1.org/abb/viewtopic.