Navigation Links
Older patients offer insight into the future of cystic fibrosis
Date:5/19/2010

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."


'/>"/>

Contact: William Allstetter
allstetterw@njhealth.org
303-398-1002
National Jewish Medical and Research Center
Source:Eurekalert

Related medicine news :

1. Confiding Eases Older Mens Stress Over Sexual Problems
2. Older Patients More Apt to Be Happy With Plastic Surgery
3. Older Chinese consumers perceive themselves younger than actual age
4. Level of frailty predicts surgical outcomes in older patients, Johns Hopkins researchers find
5. Older, Abused Women Suffer Poor Mental Health
6. Older people in assisted-living facilities sleep poorly
7. Older Adults Have Lower Rates of Mood, Anxiety Disorders
8. Friendship and confiding in spouse eases stress over sexual issues in older men
9. Declining social security benefits keep older men in workforce
10. H1N1 influenza hits older children
11. Study Published in Alzheimer's & Dementia: The Journal of the Alzheimer's Association Shows Algal DHA Improved Memory and Learning in Healthy Adults Age 55 and Older
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:6/25/2016)... ... ... closing of Bruton Memorial Library on June 21 due to a possible lice infestation, as ... of head lice: the parasite’s ability to live away from a human host, and to ... one in the event that lice have simply gotten out of control. , As lice ...
(Date:6/25/2016)... ... ... On Friday, June 10, Van Mitchell, Secretary of the Maryland Department of ... recognition of their exemplary accomplishments in worksite health promotion. , The Wellness at Work ... Symposium at the BWI Marriott in Linthicum Heights. iHire was one of 42 businesses ...
(Date:6/24/2016)... CA (PRWEB) , ... June 24, 2016 , ... Marcy was in a crisis. Her ... would lash out at his family verbally and physically. , “When something upset him, he ... he would use it. He would throw rocks at my other children and say he ...
(Date:6/24/2016)... ... , ... Topical BioMedics, Inc, makers of Topricin and MyPainAway Pain Relief Products, join The ‘Business ... to $12 an hour by 2020 and then adjusting it yearly to increase at the ... minimum wage, assure the wage floor does not erode again, and make future increases more ...
(Date:6/24/2016)... ... 24, 2016 , ... Strategic Capital Partners, LLC (SCP) in ... investment capital for emerging technology companies. SCP has delivered investment events and ... more than a million dollars of capital investment for five companies. ...
Breaking Medicine News(10 mins):
(Date:6/26/2016)... Jazz Pharmaceuticals plc (Nasdaq: JAZZ ) announced ... Act of 1976, as amended ("HSR"), with respect to ... CPXX ) expired effective June 24, 2016, ... previously announced on May 31, 2016, Jazz Pharmaceuticals and ... Jazz Pharmaceuticals has commenced a tender offer for all ...
(Date:6/24/2016)... SAN FRANCISCO, Calif. , June 24, 2016 ... GBT ), a biopharmaceutical company developing novel therapeutics ... significant unmet needs, today announced the closing of ... shares of common stock, at the public offering ... shares in the offering were offered by GBT. ...
(Date:6/24/2016)... Dehaier Medical Systems Ltd. (NASDAQ: DHRM ) ... medical devices and wearable sleep respiratory products in ... with Hongyuan Supply Chain Management Co., Ltd. (hereinafter referred ... to develop Dehaier,s new Internet medical technology business. ... Hongyuan Supply Chain,s sales platform to reach Dehaier,s dealers ...
Breaking Medicine Technology: