Cystic Fibrosis: A Geriatric Problem

Cystic Fibrosis:
A GERIATRIC PROBLEM

Warren. J. Warwick
Minnesota Cystic Fibrosis Center, Minneapolis, Minnesota, USA

Summary
This essay discusses the scope of the problems related to growing old with CF and suggests a way to prepare for that unknown future. The few available clues suggest that the future for older CF patients cannot be predicted by observations of children and young adults. Knowledge concerning the future for aging CF patients will require the collaborative efforts of adults with CF, CF Center Directors, Cystic Fibrosis Worldwide (CFW) and funding organizations.

Background
In a few years many of today’s middle aged patients with cystic fibrosis will be living into late middle age and will be eligible for retirement because of age. In a few years many more of these middle-aged patients with CF will reach retirement and become aged.

Right now these patients are seeking health care from CF physicians who don’t know whether there is a ‘CF Disease of Aging’ or whether CF patients have the same age related diseases as other aging people.

These CF physicians do not know the pattern of diseases of aging in CF patients, how to prevent and treat these diseases and how patients with different complications and CFTR genotypes will respond to conventional prevention technology and treatments. CF physicians are inexperienced in diseases of aging, because few older adults with CF are found in any CF Center.

“…the effectiveness of treatments for other older patients
have yet to be examined and tested in them.”

Those aging with CF have the most stake in the lack of this knowledge and the greatest risk for their health because differences in aging as compared to the rest of the population are not fully identified and the effectiveness of treatments for other older patients have yet to be examined and tested in them.

Internationally, adults with CF need to become proactive and work with the world’s CF Center Directors to develop patient and physician controlled epidemiological studies in aging CF patients and to lobby industry, foundations and governments to fund studies in diagnosis and treatment of the problems revealed by the epidemiological studies.

Observations from the Minnesota CF Center and Some Questions Raised
Analysis of the CF patients in the Minnesota CF Center who have reached forty years of age suggests that surprises will be found when this epidemiological study is done.

The Minnesota CF Center cares for 2.25% of US CF patients. In the past five years there have been no deaths under 15 years of age and only two between 16 and 20 years. For the whole Minnesota CF population the actuarial survival age (the life table age to which 50% of the population is calculated to survive) is 47 years. Can our hopes for an actuarial survival age of 65 years be reached for present CF adults? How long will it take for the actuarial survival age to reach 70?

The Minnesota database clinical records, which go back 26 years, show that 6% of our patients (76) have reached age 40. In this group there are 60% males and 40% females. But a surprise, in the sixth decade (the 50’s) females outnumber males 2:1. Do women have a better survival than men after the menopause?

The F508/F508 Genotype occurs in 52% of the 700+ genotyped patients in our database. Among those patients age 40 and over the prevalence is 42%. However in those patients diagnosed before 1962, when the National Cystic Fibrosis Research Foundation started the Minnesota CF Center, the prevalence of the F508/F508 genotype is 79%. The F508 mutation was found 27 of 30 times in the fifteen patients who met these criteria. Is the decreased frequency of the F508/F508 genotype in the whole group of age 40+ patients due to dilution by diagnosis of other genotypes in patients diagnosed late in life? There was increased prevalence of the most severe CF genotype (the F508/F508 genotype) having been diagnosed in patients before modern treatment was available at CF Centers. Is this a sign that the F508/F508 forecasts a better long-term prognosis?

“Does being overweight improve the health and survival of aging patients with CF?”

Seventy five percent of the over 40 patients are above average weight and 15% of these are obese. Does being overweight improve the health and survival of aging patients with CF?

Survival curves also show no difference in survival for women as compared to men. Is this a sign of post 40 changes in expected outcomes? Or is this the problem of small sample size obscuring an underlying pattern? If sample size: in which direction?
Three of the 13 deaths after age 40 were due to non-CF related diseases: one post surgical septicemia, one malignant melanoma and one due to AIDS. Will the proportion of deaths due to non-CF related diseases increase?

Other problems that affect function and survival of older people are incompletely incorporated into the Minnesota database. These include hypertension, diabetes, cancer, fractures, strokes, prostate problems, depression and cardiovascular diseases. Prior health statue, health care behavior, social and demographic factors affect health outcomes. What detection and monitoring should be utilized to identify risk factors and what interventions should be used for older patients with CF to avoid and treat such diseases?

Recommendations
This type of question is the basis for advocating a partnership of adults with CF and CF Center Directors to establish the collaborative study of aging in CF patients.

CF Adults are critical because they are well organized, play an important role within international and national organizations, have access to government (parliaments and congresses), they can be advocates with government members, private foundations and industries interested in helping the fight against CF. They have the ability to contact new patients, to recruit new patients and to empower hope for the future as CF patients grow older.

“…data collection and research efforts will be required.”

The CF Center Directors need partnership with the adult CF Groups for all of these reasons as well as to help in the design and administration of the research project. The CF Center Directors need to be encouraged to work for this common goal and both the enthusiasm of their adult patients and the development of funds to support the data collection and research efforts will be required.