Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Older Patients Offer Insight into the Future of Cystic Fibrosis

20.05.2010
Patients Over 40 Once Unheard-of Now a Growing Segment of the Population

An emerging population of middle-aged cystic fibrosis patients contains significantly more females and includes a large proportion of patients who lived for decades without a diagnosis or specialized care, according to research published by researchers at National Jewish Health.

The comprehensive analysis of this over-40 cohort, published online May 20 in the American Journal of Respiratory and Critical Care, may help guide future treatment of cystic fibrosis as survival past 40 becomes increasingly common.

"As both care and diagnosis have improved, cystic fibrosis has been transformed from a uniformly fatal childhood disease to a condition where survival to middle age and older is possible," said National Jewish Health pulmonologist and lead author Jerry Nick, MD. "We were surprised to discover that the majority of patients diagnosed as adults were females, representing a striking reversal of the "gender gap" in cystic fibrosis, and that they have delayed, but equally severe disease."

Cystic fibrosis (CF), an inherited disease of the lungs and digestive system, affects about 30,000 people in the United States and is the most common genetic disease among Caucasians. A single defective gene causes a missing or non-functional channel for chloride to travel into and out of cells. Lack of this channel disrupts the water balance in the lungs of CF patients, causing the development of thick, dehydrated mucus, which serves as a fertile environment for bacterial growth. Most patients die of respiratory failure brought on by repeated severe bacterial infections in the lungs.

In 1962 the median predicted survival for children with cystic fibrosis was 10 years. Today, it is 37, and children diagnosed today can expect to live into their 50s. Improved care is helping patients live longer. In addition, many more patients with non-traditional symptoms are being diagnosed for the first time as adults.

These two groups - those diagnosed as children and those diagnosed for the first time as adults - comprise two distinct populations of CF patients over 40. Those diagnosed as adults usually have a genetic mutation that produces a partially functional gene and a slightly different set of symptoms from those diagnosed as children. Most commonly, they have functional pancreases and fewer digestive problems.

Dr. Nick and his colleagues analyzed epidemiological and health data on 156 CF patients over 40 year of age who receive care at National Jewish Health, the largest adult cystic fibrosis clinic in the nation. In addition, data were analyzed on nearly 3,000 patients from around the nation who were included in the Cystic Fibrosis Foundation Patient Registry from 1992-2007.

The researchers found that the fate of females changes considerably in the older CF population. It has long been recognized that a "gender gap" is present in CF, favoring males. Historically, females have been diagnosed later, had a poorer prognosis, and survived fewer years than males.

Accordingly, Dr. Nick's analysis showed that fewer females diagnosed as children survived to age 40. However, among those diagnosed as adults, females represented a significant majority, accounting for 72 percent of patients in Colorado and 54 percent nationally. Among the adult diagnosed patients, females survived on average 9 to 14 years longer than males.

The complex factors that account for the differential fate of female CF patients is not understood, although Dr. Nick believes it could be a mixture of behavioral and biological factors.

Dr. Nick's findings also indicate that patients diagnosed as adults do not really have milder diseases -- as is commonly believed -- just a delayed onset of an equally severe form of the disease. Although patients diagnosed as adults live longer than those diagnosed as children, the adult-diagnosed patients lose lung function as rapidly those diagnosed in childhood, and approximately 85% die of respiratory failure or post-transplant complications.

Dr. Nick believes there is a significant number of adults whose CF remains undiagnosed. His analysis indicates that once those patients are accurately diagnosed, proper care can significantly improve their health. Patients diagnosed as adults and subsequently followed at a CF center reversed progressive lung function decline and improved their lung function for at least four years.

Older patients commonly do not get specialized CF care. It is generally recognized that the team approach to treatment provided by the 112 CF Foundation-accredited Care Centers results in better clinical outcomes. However, less than half of long-term CF survivors continued to be seen at CF Centers as they pass 40 years, with the fewest among the adult-diagnosed patients.

"In the coming years, more and more cystic fibrosis patients will be living into their 40s, 50s and beyond," said Dr. Nick. "Our findings concerning the role of gender, in survival, progression of disease, and type of care in current long-term survivors provides important insights that will help us prepare for better treatment of the steadily aging CF population."

National Jewish Health is known worldwide for treatment of patients with respiratory, cardiac, immune and related disorders, and for groundbreaking medical research. Founded in 1899 as a nonprofit hospital, National Jewish remains the only facility in the world dedicated exclusively to these disorders. Since 1998, U.S. News & World Report has ranked National Jewish the #1 respiratory hospital in the nation.

William Allstetter | EurekAlert!
Further information:
http://www.njhealth.org

More articles from Health and Medicine:

nachricht Correct connections are crucial
26.06.2017 | Charité - Universitätsmedizin Berlin

nachricht One gene closer to regenerative therapy for muscular disorders
01.06.2017 | Cincinnati Children's Hospital Medical Center

All articles from Health and Medicine >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Can we see monkeys from space? Emerging technologies to map biodiversity

An international team of scientists has proposed a new multi-disciplinary approach in which an array of new technologies will allow us to map biodiversity and the risks that wildlife is facing at the scale of whole landscapes. The findings are published in Nature Ecology and Evolution. This international research is led by the Kunming Institute of Zoology from China, University of East Anglia, University of Leicester and the Leibniz Institute for Zoo and Wildlife Research.

Using a combination of satellite and ground data, the team proposes that it is now possible to map biodiversity with an accuracy that has not been previously...

Im Focus: Climate satellite: Tracking methane with robust laser technology

Heatwaves in the Arctic, longer periods of vegetation in Europe, severe floods in West Africa – starting in 2021, scientists want to explore the emissions of the greenhouse gas methane with the German-French satellite MERLIN. This is made possible by a new robust laser system of the Fraunhofer Institute for Laser Technology ILT in Aachen, which achieves unprecedented measurement accuracy.

Methane is primarily the result of the decomposition of organic matter. The gas has a 25 times greater warming potential than carbon dioxide, but is not as...

Im Focus: How protons move through a fuel cell

Hydrogen is regarded as the energy source of the future: It is produced with solar power and can be used to generate heat and electricity in fuel cells. Empa researchers have now succeeded in decoding the movement of hydrogen ions in crystals – a key step towards more efficient energy conversion in the hydrogen industry of tomorrow.

As charge carriers, electrons and ions play the leading role in electrochemical energy storage devices and converters such as batteries and fuel cells. Proton...

Im Focus: A unique data centre for cosmological simulations

Scientists from the Excellence Cluster Universe at the Ludwig-Maximilians-Universität Munich have establised "Cosmowebportal", a unique data centre for cosmological simulations located at the Leibniz Supercomputing Centre (LRZ) of the Bavarian Academy of Sciences. The complete results of a series of large hydrodynamical cosmological simulations are available, with data volumes typically exceeding several hundred terabytes. Scientists worldwide can interactively explore these complex simulations via a web interface and directly access the results.

With current telescopes, scientists can observe our Universe’s galaxies and galaxy clusters and their distribution along an invisible cosmic web. From the...

Im Focus: Scientists develop molecular thermometer for contactless measurement using infrared light

Temperature measurements possible even on the smallest scale / Molecular ruby for use in material sciences, biology, and medicine

Chemists at Johannes Gutenberg University Mainz (JGU) in cooperation with researchers of the German Federal Institute for Materials Research and Testing (BAM)...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

Plants are networkers

19.06.2017 | Event News

Digital Survival Training for Executives

13.06.2017 | Event News

Global Learning Council Summit 2017

13.06.2017 | Event News

 
Latest News

Touch Displays WAY-AX and WAY-DX by WayCon

27.06.2017 | Power and Electrical Engineering

Drones that drive

27.06.2017 | Information Technology

Ultra-compact phase modulators based on graphene plasmons

27.06.2017 | Physics and Astronomy

VideoLinks
B2B-VideoLinks
More VideoLinks >>>