Last month, the University of Kentucky was officially designated as a Clinical Resource Center (CRC) by the Alpha-1 Foundation. Alpha-1 antitrypsin deficiency (AATD) is an inherited genetic condition that may cause damage to the lungs, liver or both. As a CRC, the University of Kentucky will provide comprehensive care to patients with AATD, including diagnosis, treatment, education, counseling, long term management and research.
A simple test can help diagnose AATD, but a majority of AATD patients remain undiagnosed. There is currently no cure for AATD. The Alpha-1 Foundation was established to help address these gaps. With the goal of curing AATD, the nonprofit organization began by supporting research and building a network of alpha-1 antitrypsin expertise around the country. They developed the Clinical Resource Center concept and began recruiting locations to aid in having geographically dispersed centers of care, meeting patients where they are and encouraging them to enroll in the foundation’s research registry.
All CRCs go through a rigorous designation process and are required to adhere to the highest standard of care and best practices. Currently, the foundation holds more than 120 centers across the nation, and the University of Kentucky now has the honor of being included.
Malik Khurram Khan, M.D., the UK HealthCare pulmonologist who spearheaded this designation, said, “With UK earning this designation, we are now a recognized center by the Alpha-1 Foundation, the leading organization committed to AATD, to provide excellent comprehensive care to our AATD patients. Our designation as a CRC underscores our expertise in advanced lung diseases, including COPD, lung and liver transplants, hepatology and access to a state-of-the-art pulmonary rehabilitation facility, smoking cessation programs, intravenous augmentation therapies and genetic counseling.”
This designation puts UK on the map for both patients and providers who are looking for expert care. It will provide people across the state with access to an alpha-1 antitrypsin center with a multidisciplinary care team and resources to help diagnose and manage this rare genetic disease. This will also serve as a center of education for other providers and patients across Kentucky and serve as center of research to help advance AATD care.
People with AATD can have severely decreased levels of protein called alpha-1 antitrypsin in the blood, which is primarily made in the liver. This protein protects the lungs from damage from inflammation, which is accelerated by inhaled irritants like cigarette smoking. Deficiency of alpha-1 antitrypsin leads to damage to the lungs resulting in a condition called emphysema/chronic obstructive lung disease (COPD). The buildup of abnormal alpha-1 antitrypsin protein in the liver cells leads to liver damage and scarring.
AATD is the most common genetic cause of COPD and emphysema. In the U.S., AATD affects roughly 1 in every 3,500 people, and there are an estimated 100,000 people with severe alpha-1 antitrypsin deficiency. AATD patients usually develop their initial signs and symptoms of lung disease between age 20 to 50, which can include progressive shortness of breath with exertion, chronic cough, wheezing, frequent lung infections and early onset emphysema, which in severely impacted patients is a potential indication for lung transplant surgery.
Appropriate patients can also be treated with intravenous protein replacement and other therapies, such as bronchoscopic valve placement, to reduce symptoms and the progression of lung disease. Liver disease in AATD can develop even in infancy, and around 10% of adults with alpha-1 antitrypsin deficiency develop chronic liver damage, which can require liver transplantation in severe cases.
UK HealthCare is the hospitals and clinics of the University of Kentucky. But it is so much more. It is more than 10,000 dedicated health care professionals committed to providing advanced subspecialty care for the most critically injured and ill patients from the Commonwealth and beyond. It also is the home of the state’s only National Cancer Institute (NCI)-designated Comprehensive Cancer Center, a Level IV Neonatal Intensive Care Unit that cares for the tiniest and sickest newborns and the region’s only Level 1 trauma center.
As an academic research institution, we are continuously pursuing the next generation of cures, treatments, protocols and policies. Our discoveries have the potential to change what’s medically possible within our lifetimes. Our educators and thought leaders are transforming the health care landscape as our six health professions colleges teach the next generation of doctors, nurses, pharmacists and other health care professionals, spreading the highest standards of care. UK HealthCare is the power of advanced medicine committed to creating a healthier Kentucky, now and for generations to come.
View the article here: UK HealthCare receives designation for treatment of alpha-1 | UKNow
Find your local Alpha-1 CRC: https://alpha1.org/find-an-alpha-1-specialist/
