The life expectancy of people with CF is steadily increasing.
According to the database of the American CF Association, it is clear that the life expectancy of patients improves according to the decade in which they were born; moreover, in 2005 lung function was 10-15% higher for all age groups, compared to measurements made in 1990 (Fig. At the end of the article).
Data from the German registry of CF patients show that the median age of patients increased from 11 in 1995 to 17 in 2005. The number of adult patients in 2005 was close to 50% of all patients. Similar observations have been reported from the UK, Israel, and other European countries.
What caused the change? What are we doing today that we have not done before, and in other words, what are the developments in the treatment of patients with CF that have enabled these distinctions?
Treatment at CF centers
The first report documenting the benefits of CF center treatment came from Denmark. In 1982, Nilsson and Schutz reported that patients at the only CF center in Denmark, located in Copenhagen, had a significantly better survival rate than patients treated by local doctors.
What is the difference between treatment by a CF center compared to general clinics? Walters and others from Birmingham, UK, reached the medical service received by adults in specialized clinics for CF and the service they received in public clinics. They found that CF clinics performed more tests and more treatments: ranging from simple lab tests (blood tests, mucus culture, oxygen saturation, chest x-ray, weight, measurement of lung function), more intravenous antibiotics, and larger doses of pancreatic enzymes. As a result, they suffered from less severe pulmonary symptoms and were more satisfied with the professional aspects of the treatment they received. For patients in a painful pulmonary condition – deficiencies were found in the overall medical treatment, in terms of neglect of performing fewer tests, and signs were found that there was a lack of adequate treatment of respiratory and digestive symptoms, regardless of the type of clinic.
The new CF Adult Center in Papworth, Cambridge, UK, has created a unique opportunity to compare referrals from different clinics to the new center. They found that patients referred from another CF center had significantly better clinical conditions than those referred from general pulmonary clinics. Patients who continued CF treatment had better lung function, better nutrition with a BMI of 21.6 compared to 18.3, and better results on chest X-rays. Interestingly, patients referred from the CF center had a higher percentage of infection with pseudomonas and sputum than those referred by general clinics. Still, despite the higher infection rates, they were in a significantly better lung condition.
What are the reasons for the differences between CF centers?
Data from the American CF Association database and the German Cystic Fibrosis Quality Assessment Project indicate significant differences between the centers in terms of the clinical condition of patients. In search of the factors influencing outcomes in CF, Johnson and others conducted an epidemiological study of CF that focused on the centers; they analyzed the clinical condition of patients in 194 centers. They compared the top 25% of centers with the best patient status and the bottom 25% with the least good patient status. They found that patients in the top 25% were better nutritional. In addition, all age groups in the top 25% had more clinic visits, more lung function tests, and more frequent phlegm cultures were tested.
Similarly, Padman and others, who studied the treatment patterns of CF centers for children, found that in centers that achieved the highest lung function, more infants were diagnosed at an early age and had a higher weight for their age at the time of diagnosis. Moreover, in these centers, the frequency of routine visits was higher, and also there were more visits due to pulmonary exacerbation. In addition, more phlegm cultures were taken, and high-frequency intravenous antibiotics and oral steroids were used. Moreover, top-quarter sites seem to treat patients more often, no matter what led to the decision to treat, especially those patients who are considered blue with mild lung disease.
Commitment to a treatment routine – to prevent premature changes and disease progression
Initiating early and aggressive treatment at a stage when there are still no apparent signs of significant lung disease may delay the development and progression of the disease. The currently accepted treatment strategy that has led to an extension of life expectancy in CF includes early diagnosis, early and aggressive nutritional support, an early increase of mucus clearance by mucus thinners (polemics and hypertonic saline), daily drainage of mucus in physiotherapy exercises, antibacterial and antibacterial treatments for Early, early treatment of exacerbations of the disease by antibiotics, application of effective hygienic measures to prevent infection both inside and outside the CF centers, as well as early detection and treatment of complications such as diabetes, ABPA, nasal polyps, and others. However, many patients and their parents show resistance to treatments that they are tired of, even though their goal is to prevent the deterioration because it is slow and invisible to the eye. Such a routine may require daily physical therapy practice, inhalations, and nutritional support, exhausting and time-consuming, which many patients and their parents find difficult to perform. When the signs of the disease are not noticeable, the good feeling and appearance of health give a false sense that the condition is not active, reducing the motivation to indulge in daily CF treatment. The slight invisible deterioration will lead to bronchitis and bacterial growth, leading to further lung damage and the development of bronchiectasis, an irreversible condition.
Various testing methods may indicate the early presence of infection, inflammation formation, and structural changes in the health of young patients with CF. Early intervention, when these changes may not yet be visible, may halt or reverse the direction of disease progression. Current methods for assessing early changes in CF lung disease have limitations. Their sensitivity to detect early changes is low, and they have a limited ability to determine the nature and type of infection or illness. Many of these tests cannot be performed on young children who do not cooperate. Since testing for early lung damage, today is complex and invasive. In many centers, some of these tests are not available. Early intervention with physiotherapy, phlegm, and antibiotics should also be considered in asymptomatic children without sophisticated tests. It is essential that there are accurate and straightforward signs for early assessment of the disease in children, preferably before the condition becomes symptomatic. Early intervention is likely to halt disease progression, but it remains to be determined with certainty whether early intervention leads to reversal of disease direction in its early stages.
The American CF Association has set up a committee for the treatment of lungs, which has issued guidelines for the use of drugs intended to treat lung diseases. The procedures are appropriate for most patients but may need to be tailored to individual needs, as decided by the physician. As mentioned, some patients and families find it challenging to maintain an ongoing routine of all CF treatments. These treatments can be very tiring and require a long time for physical therapy, inhalations, and nutrition. The feeling or appearance of a good health condition gives patients a false sense that the disease is inactive and thus reduces the motivation to continue the tedious daily treatment. This condition, of untreated exacerbation, will lead to further deterioration, including bronchitis and infections, leading to lung damage and irreversible condition.
It is not clear whether chronic care means lifelong care. The physician must diagnose and decide whether the specific treatment is still beneficial to the patient or discontinued.
Aggressive early treatment in exacerbation of lung disease
Along with routine clinical care, it is essential to identify and treat early respiratory exacerbations. It is now clear that early aggressive treatment for lung conditions exacerbates the long-term medical outcome. Exacerbations in the lung condition are a dominant feature in the lives of cystic fibrosis (CF) patients. These exacerbations are associated with clinical deterioration and are often associated with reduced lung function and evidence of a recurrent local inflammatory response. Repeated pulmonary exacerbations cause worsening of lung function, and cumulative damage to the lungs occurs over time. Prevention of pulmonary exacerbations is essential. There is currently no consensus on defining respiratory exacerbations and how to treat them. There are no uniform definitions for respiratory exacerbations, and there is also no proof of the validity (validation) of the proposed reports. Several large clinical trials have recently been published on new therapies in CF. Respiratory exacerbations were one of the targets. Each study used different definitions for respiratory exacerbations. The signs and symptoms that best predict pulmonary exacerbations are summarized in Table 1. In most CF centers, the current reports of acute exacerbations are based on subjective clinical evaluation by CF physicians and are defined based on their “feelings” that the exacerbations are severe or moderate. . Early antibiotic therapy, given intravenously, may delay or reverse the early findings to airway damage before establishing irreversible bronchiectasis damage.
It is not clear whether chronic therapy means lifelong therapy. It is essential for the clinician to closely monitor each patient and decide whether a specific treatment continues to benefit the patient or whether it should be discontinued.
An aggressive approach to pseudomonas infection prevention
Pseudomonas aeruginosa continues to be the leading cause of cystic fibrosis lung disease. Chronic pseudomonas (PA) infection has been linked to accelerated lung function decline in CF patients. The use of infection control measures, frequent testing of pseudomonas infection, and aggressive extinction of early pseudomonas infection have reduced the incidence of chronic pseudomonas infection. The presence of early-stage airway inflammation and its progression due to recurrent respiratory tract infections create an ongoing inflammatory process, which is airway and lung tissue destruction.
Early extinction of PseudomonasPseudomonas
There is accumulating evidence that antibiotic treatments started early and close to the onset of the infection effectively delay chronic pseudomonas infection. Some studies show that early treatment may, in most cases, eradicate the bacterium. Because in the initial stage, PseudomonasPseudomonas that attacks the airways of CF patients, are very sensitive to many specific antibiotics, pseudomonas extinction is often achieved in the early stages of infection, and the development of antibiotic resistance is not a significant problem. The high success rates of bacterial death increase the likelihood that early antibiotic treatment will have a beneficial effect on lung function, compared to CF patients with chronic infection, and thus may result in higher life expectancy of CF patients treated early.
In the early stages of lung disease in CF patients, only a few clinical findings may be found; however, bronchoscopic studies (within the bronchi) have documented the presence of infection and inflammation in the lungs of CF infants who are in a perfectly healthy condition. Therefore, it is imperative to perform routine tests of phlegm culture frequently, with each visit to the clinic, even in patients who do not have PseudomonasPseudomonas. In some centers, phlegm or BAL cultures are used for patients who do not secrete mucus. This may allow early detection of pseudomonas infection in patients who are still asymptomatic.
It is extremely important to offer all patients this “extinction regimen” because pseudomonas infection causes a worse clinical condition; Lower respiratory function, lower weight, height and BMI, and a greater number of hospitalizations.
Pseudomonas is a bacterium found in the natural environment, including at school and the workplace. Therefore, it is recommended to take precautions to prevent infection from the setting with pseudomonas, and many families with CF children take care of this. It is important to note that most cases of pseudomonas infection do not occur in the hospital. However, there are reports of conditions among patients. Infectious strains of pseudomonas threaten CF patients who do not have pseudomonas through exposure to an increased risk of infection. There are no adequate means of controlling the disease. Clinical experience also suggests that early infection with contagious strains, which are resistant to many antibiotics (multiresistant), maybe more difficult to eradicate than random strains of pseudomonas.
Pseudomonas may be contagious from other patients because siblings often carry the same strain. Specific themes have been reported to those not previously infected during social events such as summer camps and social activities. The spread of antibiotic-resistant strains and the link between an increase in the number of chronically infected patients and the time of their presence in the CF center also indicate the possibility of infection between patients.
For a patient who has not yet been infected, even a few minutes in the company of a patient already carrying the bacterium may be sufficient for an infection to occur. The concern is even more significant in the case of strains resistant to many types of antibiotics.
Poor nutritional status is associated with weight and height delay in children with CF. Early studies have shown a link between the degree of nutritional status and the severity of lung disease. In patients without fat loss in the stool, a slower rate of deterioration in visual function was found, but not necessarily in direct contact. Improved respiratory prognosis in patients with CF who have normal fat absorption and a slower rate of deterioration of lung function was found in children with CF without fatty stools. In the past, growth retardation and weight loss seemed inevitable in the case of advanced lung disease; however, in the 1970s, Toronto Clinic for CF showed that a high-fat, high-calorie diet promoted standard growth patterns and improved life expectancy. Studies in the longitudinal section have shown that the deterioration in lung function and malnutrition are interrelated and that malnutrition is likely to increase with age. It has recently been shown that low vitamins A and E levels have been linked to an increased rate of exacerbation of lung disease in CF.
The energy needs of patients with CF vary widely and have been determined to be about 120-150 percent of the needs of healthy people of the same age, sex, and size. As short-term solutions, simple techniques should be explored, such as increased feeding, nourishing overnight. In the long run, nutrition in connection with the stomach or intestines has accelerated the growth rate and an improved ratio of weight to height.
The best achievements of early diagnosis of CF patients are in nutrition. Infants diagnosed early by nationwide infant scan are more likely to have a more normal height and weight than those diagnosed following the onset of symptoms.
We reviewed and evaluated the evidence supporting the use of prolonged drug administration to maintain lung function in patients with CF. In general, it is essential to emphasize those specific medications will depend on each patient’s medical condition and the preferences of the patients or their parents.
The signs and distinctions are the main predictors of a worsening of the condition I have seen
Changes in phlegm (in quantity or frequency)
Shortness of breath
Anorexia or weight loss
Decrease in energy level
Changes in tests and breath indices
Decrease in spirometric indices (volume of visibility)