A mother of 3 from El Paso, Texas came to her obstetrician complaining of constant itching. Her physician suspected it to be caused by hormonal fluctuations from her then third pregnancy, and told her it would resolve after delivery. The itching persisted a year after her delivery, prompting the start of a 6-year series of diagnostic tests. The ultimate result, which changed Michelle Linss’ life, was a diagnosis of primary biliary cirrhosis (PBC), a disease wherein the bile ducts are obstructed and damaged, causing a harmful accumulation of bile in the liver. The disease progresses quite slowly, often presenting without symptoms, but for Michelle, who was discovered to have a rare, fast-progressing form of PBC, a liver transplant would be the only life-saving solution.
Michelle was first prescribed a regimen of prednisone and cyclosporine. After these failed to work for her condition, she turned to Dr. Marlyn Mayo, an Associate Professor of Internal Medicine at UT Southwestern, and a specialist in immune-related liver diseases. She complained of painful bumps under her skin, which turned out to be xanthomas — deposits of an abnormal lipoprotein called, lipoprotein X, which is a symptom of the liver’s reduced ability to process cholesterol. These bumps made movement and daily activities difficult for Michelle. Dr. Mayo then sent her for evaluation for liver transplant. Soon, Michelle was placed on the waiting list for a potentially life-saving procedure. However, her transplant assessment revealed a MELD score (based on creatinine levels, bilirubin levels, and INR) of 17, which meant she was not deemed sick enough to be prioritized just yet, and placed her further down the list.
To help her get a transplant sooner, Dr. Jorge Marrero, the Medical Director of the Liver Transplantation Program at UT Southwestern, presented Michelle as a special case to the committee that oversees transplant waiting lists. He argued the rapidly-progressing nature of the rare form of PBC Michelle has is greatly affecting her quality of life, and should be considered urgent. Fortunately, the board conceded to granting her case additional MELD points.
On August 28, 2014, Michelle received the call she and her family had been waiting for, and the liver transplantation was a success. After the procedure, she reported her chronic pruritus had disappeared, and that she no longer felt as much pain from her xanthomas.