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Understanding Cystic Fibrosis

Incidence and Prevalence

CF Statistics

Incidence and prevalence

CF is one of the more common, life-shortening, inherited diseases among Caucasians and effects about 70,000 people worldwide.¹¹

According to the case report "The prevalence of cystic fibrosis in the European Union" registry analyses revealed a mean prevalence of 0.737/10,000 in the 27 EU countries (see table 1).¹²

Adapted with permission from the author - Farrell PM , The prevalence of cystic fibrosis in the European Union, J Cyst Fibrosis (2008) (doi:10.1016/j.jcf.2008.03.007)

In the United States approximately 30,000 people have cystic fibrosis and 1 in every 3500 Caucasian babies are born with the disease.⁸; Most individuals are diagnosed by the age of 3 years; however, nearly 8% of all newly diagnosed patients are 18 years or older. One in 31 Americans (about 12 million people) is a carrier of the defective gene. Many do not know they carry the gene, as they have no overt clinical signs of the disease.

CF affects both genders, but females become infected with Pseudomonas aeruginosa earlier, and have worse pulmonary function, nutritional status, and earlier mortality from lung infection. It also affects people from all racial and ethnic groups but is most common among Caucasian people whose ancestors came from northern Europe. About 65% of patients with CF have moderate-to-severe lung disease based on FEV₁ measures.^4,8

Costs

The United States spends $314 million each year on CF, with a mean cost per patient of $13,650 (1996 dollars).¹³ The severity of the illness greatly impacts annual spending per patient with a mean cost of:

• $6300 among patients with mild CF
• $11,400 among patients with moderate CF
• $43,300 among patients with severe CF¹³

Based on data from patients with CF enrolled in a health maintenance organization, 47% of total costs are attributable to hospitalization, primarily due to exacerbation of lung disease.¹³ Thirty-five percent of patients with CF are hospitalized at least once annually for the treatment of lung infections, including pneumonia.¹⁴

Source: Braunwald Atlas of Medicine⁴⁷

Life Expectancy

Research and better treatment of lung infections and other conditions mean that patients with CF live to a median age of nearly 37 years based on 2005 data. In addition, at least 40% of the CF patient population is age 18 years or older. Early antibiotic treatment of airway colonisation with Pseudomonas aeruginosa can delay onset of chronic lung infection in patients with cystic fibrosis. Whether the pathogen is eradicated by this treatment is unclear.¹⁵

According to the Cystic Fibrosis Foundation (CFF), 354 people died in 2004 due to CF. This number is down from the 419 deaths of CF in 2000.

Source: Braunwald Atlas of Medicine⁴⁷

Source: Braunwald Atlas of Medicine⁴⁷

Adults living with CF

Life Experiences

Improved management of lung disease, including infections, allows patients to live longer, more productive lives.⁸

• More than half of adults with CF have completed some college courses or obtained a college degree
• More than half of the adults with CF are working full time or part time
• About one quarter of adult CF patients attend school
• Approximately one third of adults with CF are married.

Source: Adapted from; Cystic Fibrosis Foundation. Patient Registry Annual Data Report. 2007⁴⁸

Quality of Life

Quality of life in patients with CF appears more closely associated with the disruptive effects of lung exacerbations rather than with the severity of underlying disease.¹⁷

• Although patients with CF experience declines in physical functioning and lung function, patients and their parents rate these changes as only modestly impacting their quality of life¹⁷
• Psychosocial functioning among patients with CF seems to mirror that of the general population.¹⁷

Therefore, medications that prevent or treat exacerbations could positively affect how patients experience CF.

There are a number of questionnaires available to measure and monitor quality of life (QOL) in Cystic Fibrosis. One example is the CFQ-R questionnaire which is both validated in many languages and is disease specific. Please find below a link to an article by Christopher H. Goss and Alexandra L. Quittner, regarding Patient-reported Outcomes in Cystic Fibrosis.

Patient-reported outcomes in cystic fibrosis, the Proceedings of the American Thoracic Society Aug 1;4(4):378-86 (2007)

Healthcare Utilization

As lung disease progresses, the healthcare needs of people with CF are considerable.

• Adults have more severe pulmonary disease than children, putting them at increased risk for serious complications
• Each year, adult patients make an average of 4.7 CF clinic visits, experience 1.5 acute exacerbations, and are admitted to the hospital 1.0 time.¹⁸

In response to the increasing number of adults with CF, care has shifted from a pediatrician's office to adult care providers.

• Recognizing the growing number of adults with CF, the Cystic Fibrosis Foundation (CFF) recommended in 2002 that adult patients with CF be cared for by an adult practitioner
• The number of adult CF programs in the United States has grown from less than 10 in 1992 to more than 79 in 2002.⁸

References:
4. National Heart Lung and Blood Institute. What Is Cystic Fibrosis. US Department of Health and Human Services National institute of Health 2005;1
11.Cystic Fibrosis Foundation – www.cff.org . Accessed August 2008
12. Farrell, P. The prevalence of cystic fibrosis in the European Union, Case Report, March 2008
13. Lieu TA, Ray GT, Farmer G, Shay GF. The cost of medical care for patients with cystic fibrosis in a health maintenance organization. Pediatrics 1999 June;103(6):e72.
14. Moss RB. Administration of aerosolized antibiotics in cystic fibrosis patients. Chest 2001 September;120(3 Suppl):107S-13S.
15. Ratjen F., Doring G., Nikolaizik W.H. Effect of inhaled tobramycin on early Pseudomonas aeruginosa colonisation in patients with cystic fibrosis, (2001) Lancet, 358 (9286), pp. 983-984
17. Britto MT, Kotagal UR, Hornung RW, Atherton HD, Tsevat J, Wilmott RW. Impact of recent pulmonary exacerbations on quality of life in patients with cystic fibrosis. Chest 2002 January;121(1):64-72.
18. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest 2004 January;125(1 Suppl):1S-39S.
47. Braunwald Atlas of Internal Medicine
48. Cystic Fibrosis Foundation. Patient Registry Annual data Report. 2007.