In the UTMB Cystic Fibrosis Clinic, we provide hope for our CF patients through specialized care and management of their conditions, especially those patients challenged by possible lung failure. We can help maintain a high quality of life through intensive, patient-centered medical management, education and nutritional support, rehabilitation, and the latest therapies to help stall the progression of respiratory failure. When medically warranted and appropriate, we can guide a patient through the lung transplantation process.
Cystic fibrosis was a lethal pediatric disease; in the past 50 years, it's been transformed. Work and research has extended the average life span of a CF patient so that today, someone diagnosed with CF in early childhood can expect to live through his or her late 30s, adding a precious decade or longer to many lives. And, the search continues for a possible cure.
What is Cystic Fibrosis?
Cystic fibrosis (CF) is an inherited disease characterized by an abnormality in the body's salt, water- and mucus-making cells. It is chronic, progressive, and is usually fatal. In general, children with CF live into their 30s.
Children with CF have an abnormality in the function of a cell protein called the cystic fibrosis transmembrane regulator (CFTR). CFTR controls the flow of water and certain salts in and out of the body's cells. As the movement of salt and water in and out of cells is altered, mucus becomes thickened. The thickened mucus can affect many organs and body systems including:
There are about 30,000 people in the US who are affected with the disease, and about 1,000 babies are diagnosed with it each year. It occurs mainly in Caucasians who have a northern European heredity, although it also occurs in African-Americans, Asian Americans, and Native Americans. Approximately one in 31 people in the US are carriers of the cystic fibrosis gene. These people are not affected by the disease, and usually do not know that they are carriers.
Usually, cases are identified in infancy (70 percent are diagnosed by age two) but some cases escape recognition until adolescence or early adulthood. Patients who aren’t diagnosed in childhood may exhibit frequent bronchitis or coughing spells, or may even have been admitted to the hospital several times for pneumonia. Fortunately, when we’re able to identify the disease in young adults--as we've done at UTMB--we can help alter the course of the disease.
Cystic fibrosis is a complicated illness that requires clinical care by specialized physicians, nurses and other healthcare professionals including dietitians, respiratory therapists and social workers. Multidisciplinary teams like those available at our medical center are recommended for care of CF and have been shown to result in less illness and longer lives for CF patients.
Even with great care, CF is relentless and progressive, leading to respiratory dysfunction…and ultimately, lung failure. Transplantation may become necessary, and transplant patients can have a tremendous improvement in the quality of their lives, resuming near-normal lifestyles. Although transplantation offers some challenges, many patients who’ve undergone lung transplants are doing well a decade after surgery.
Dr. Vincent Valentine, FCCP, FACP was born in New Orleans, attended LSU Medical School and completed his fellowship at Stanford University Medical Center in Pulmonary and Critical Care Medicine and an advanced fellowship in Heart-Lung and Lung Transplantation. He and his wife, Cathy, have two daughters and one grandson. Dr. Valentine’s clinical interests are in lung transplantation and end stage lung diseases, especially the diffuse parenchymal lung diseases, cystic fibrosis and pulmonary hypertension. Dr. Valentine’s research interests are in lung transplantation, defining and managing acute and chronic rejection as well as CMV disease in the lung allograft, infectious complications and their relationships to chronic rejection, pulmonary complications in the immunocompromised host, and managing patients with cystic fibrosis and patients with idiopathic pulmonary fibrosis. He has participated in many different research studies mostly translational in nature, completing 10 and has published 30 articles with more than 100 abstract presentations.
Dr. Valentine serves patients at:
Friendswood Medical and Surgical Clinic
121 West Parkwood
Friendswood, TX 77546
UTMB University Hospital Clinics
1005 Harborside Dr.
For more information or to schedule an initial evaluation, contact Gisele Lombard at (409) 747-1825 or Elizabeth Heltz at (409) 772-3115
Dr. Vincent Valentine, medical director of the lung transplant program at UTMB, appeared on the health program “Your Health First ” with Dr. Joe Galati, heard on KTRH-AM (740, Houston), on Jan. 10, 2010. He discussed cystic fibrosis and the advances being made at UTMB. Valentine is a leading authority in all facets of lung disease, including cystic fibrosis. Hear the segment, beginning at the 12:05 mark.
Debbie Cheramie, of Morgan City, La., has been living in Galveston for almost a year, after undergoing a double-lung transplant at UTMB’s Texas Transplant Center. “During this holiday season, people reflect on the blessings the year has given them. I have much to be thankful for; I have a new lease on life and am liberated from the suffering of my old lungs. I have gotten exceptional care at the University of Texas Medical Branch.”
Debbie Cheramie (front center, in brown and stripped top) and members of the transplant and support team that gave her a new lease on life at UTMB Health.
Matthew has CF, and was 28 when he received his lung transplant at UTMB in the summer of 2008. Matthew is now living in Louisiana, and is healthy, active and recently married, a "wonderful young man" according to the staff who helped care for him in Galveston, where this fish was caught.
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