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A common hereditary disease in which exocrine (secretory) glands produce abnormally thick mucus. This mucus can cause problems in digestion, breathing, and body cooling.

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Human Growth Hormone for Children With Cystic Fibrosis: A Review of the Research for Parents and Caregivers

This guide is for anyone who takes care of a child or teen with cystic fibrosis (CF) and helps make decisions about treatment. You may have heard about using human growth hormone (HGH) for children with CF and want to know more about the research.

Comparative Effectiveness Review Summary Guides for Consumers [Internet] - Agency for Healthcare Research and Quality (US).

Version: March 4, 2011

The use of regular vitamin A preparations for children and adults with cystic fibrosis

Cystic fibrosis can lead to certain vitamins, such as vitamin A, not being properly absorbed by the body. This can result in problems caused by vitamin deficiency. A lack of vitamin A (vitamin A deficiency) can cause specific problems such as eye and skin problems. It can also be associated with poorer general and respiratory health. Therefore people with cystic fibrosis are usually given regular vitamin A preparations from a very young age. However, too much vitamin A can also cause respiratory and bone problems. The review aimed to show whether giving vitamin A regularly to people with cystic fibrosis is beneficial or not. However, the authors did not find any relevant trials to include in the review. They are therefore unable to draw any conclusions regarding the routine administration of vitamin A supplements and recommend that until further evidence is available, local guidelines are followed regarding this practice.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2014

A tube fitted inside a vein to allow drug injections for people with cystic fibrosis

Cystic fibrosis blocks the lungs with mucus and causes frequent infections and lung damage. Infections are often treated by giving drugs intravenously (through a vein), but regular injections can damage veins. Another option is to use a totally implantable vascular access device (TIVAD). There are different types of device and people with cystic fibrosis or their families can be shown how to administer drugs through them. TIVADs have no external portion attached when not in use and long‐term maintenance is quite easy. Some people feel that the devices improve their self‐image while others do not like the way they look. Sometimes TIVADs cause blood clots and infections. We looked for randomised and quasi‐randomised controlled trials of these devices in people with cystic fibrosis. The review found no trials to include. Reports of the use of TIVADs in people with cystic fibrosis suggest that they are safe and effective. They also suggest that certain interventions might reduce the risk of complications; however, these issues have not been examined by randomised controlled trials. We think a multicentre randomised controlled trial is needed to assess the efficacy and possible adverse effects of TIVADs in cystic fibrosis.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2012

The training of muscles that cause the chest to expand in order to take air into the lungs for people with cystic fibrosis

Cystic fibrosis is the most common life‐limiting genetic condition in Caucasians. The life‐expectancy of newly diagnosed patients is increasing. Inspiratory muscle training may improve quality of life, lung function and exercise tolerance in people with cystic fibrosis, so that these are closer to the levels found in people who do not have cystic fibrosis. It may also boost the clearance of mucus. Inspiratory muscle training can be performed without the help of a carer and wherever the individual feels appropriate. We searched for randomised or quasi‐randomised controlled clinical trials to determine the effects of inspiratory muscle training in the treatment of people with cystic fibrosis. We included eight studies with 180 participants in the review, but we could not combine results from these studies to answer our questions, because the studies either did not publish enough details or did not use the same standard measurements. Given this, we cannot recommend the use, or not, of this intervention. We do recommend that future studies make more use of health‐related quality of life and exercise tolerance measures. We also suggest there should be agreement upon standard measurements used.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2013

The timing of inhalation of hypertonic saline in people with cystic fibrosis

Inhalation of a strong, sterile, salt water solution (known as hypertonic saline) through a nebuliser improves the physical properties of sputum and stimulates cough in people with cystic fibrosis. Regular inhalation improves the clinical outcomes of people with cystic fibrosis. It is not certain whether it is better to inhale hypertonic saline before or after clearing the airways with physical techniques, nor whether it is better to inhale it in the morning or in the evening. We looked for trials that compared these different timing regimens, but were unable to find any such trials. In the absence of this evidence, people with cystic fibrosis might be advised to inhale hypertonic saline before airway clearance, because this is the only timing regimen with established efficacy. Because the long‐term efficacy of hypertonic saline has only been established for twice‐daily inhalations, clinicians should advise patients to inhale hypertonic saline both in the morning and in the evening. However, if only one dose per day is tolerated, the time of day at which it is inhaled could be based on convenience or tolerability until further evidence is available.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Antibiotic treatment for nontuberculous mycobacteria in people with cystic fibrosis 

Nontuberculous mycobacteria are bacteria that are in the same family as tuberculosis and are commonly found in the soil and water. These bacteria can be found in the lungs of people with cystic fibrosis and can cause their lung function to worsen. Although there are guidelines on which antibiotics to use to treat lung infection due to these bacteria, these recommendations are not specific for people with cystic fibrosis. It is also not clear which are the most effective antibiotics. The main purpose of this review was to determine whether treatment with different antibiotic combinations for nontuberculous mycobacterial infection would improve lung function or decrease the frequency of chest infections in people with cystic fibrosis. We did not find any randomized controlled trials which we could include in the review. Randomized controlled trials are needed to inform clinicians which are the best drugs for treating these infections in people with cystic fibrosis. In the meantime, clinicians should follow the current guidelines for the diagnosis and treatment of lung infections due to nontuberculous mycobacteria in the general population.   

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Inhaling antibiotics to treat lung infection in people with cystic fibrosis

Cystic fibrosis is an inherited disease which results in abnormal mucus in several parts of the body. The main organ affected is the lungs which are susceptible to infection by certain bacteria, the most frequently isolated being Staphylococcus aureus and Pseudomonas aeruginosa. Infection causes inflammation that results in progressive damage to the lungs. We aimed to find out if inhaling antibiotics would reduce the effects of infection. We wanted to learn whether this treatment would improve lung function, quality of life and survival. We also looked for any adverse events. This review includes 19 trials with 1724 people. We found that inhaling an antibiotic to fight these bacteria improved lung function during the study and reduced frequency of exacerbations. However, there was more resistance to antibiotics, tinnitus and change in voice in the treatment groups. Further research is needed to show whether the benefits can be maintained in the long‐term and to work out the best antibiotic to use and at what dose.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2011

Physical training to improve exercise capacity in people with cystic fibrosis

The progress of lung disease in cystic fibrosis leads to abnormal breathing during exercise. This limits people exercising, which in turn affects health and body image. Physical training is designed to improve physical, heart and muscle strength through aerobic and anaerobic activity. Aerobic exercise involves training for a length of time such as distance cycling or running. Anaerobic exercise involves training intensely for a short time such as weight training or sprinting. A lack of regular physical training may lead to more severe lung disease and a reduced ability to perform day‐to‐day tasks. The side effects of exercise include dehydration, damage to muscles and bone fractures in those with low bone mineral density. This review includes seven studies with 231 participants. Due to different study designs, we were not able to combine any data. Some studies were short‐term and did not show differences between treatments. However, it is not likely that training for less than one month would be beneficial. There is some evidence that physical training produces benefits and that these are influenced by the type of training programme. There are not enough studies in this review to compare aerobic training to anaerobic training or to a mixture of these two. The review provides some evidence that the benefits of training are maintained after training has ceased, but it is not clear for how long. Physical training is already part of the care package offered to most people with CF and there is no evidence to discourage this.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2012

Antiviral treatment for influenza infection in people with cystic fibrosis

Cystic fibrosis is a genetic, life‐threatening disorder affecting multiple organs. People with cystic fibrosis have a higher risk of chronic lung disease. Influenza can worsen the course of the disease in cystic fibrosis by increasing the risk of pneumonia and secondary respiratory complications. During a pandemic, flu symptoms may be more severe and complications more frequent. Severe cases of pandemic flu have occurred in people with underlying chronic conditions including people with CF. Although there is no evidence that people with CF are more likely to contract this infection than healthy people, the impact for them could be greater and the outcome worse as the lower respiratory tract is affected more often. Antiviral agents form an important part of influenza management and include the neuraminidase inhibitors zanamivir and oseltamivir. These drugs can limit the infection and prevent the spread of the virus. We did not find any trials to support or refute the use of neuraminidase inhibitors for influenza in people with cystic fibrosis. However, limited data from previous studies have shown that these drugs can be effective in healthy people and may be useful in high‐risk populations if used rationally. However, the question of the safety and effectiveness of neuraminidase inhibitors for treating influenza in people with cystic fibrosis remains unanswered.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2014

Vaccines for preventing infection with Pseudomonas aeruginosa in cystic fibrosis

Cystic fibrosis is a hereditary disease where thick mucus is produced in the lungs. Pseudomonas aeruginosa and other bacteria cause long‐lasting lung infections which result in permanent lung damage. Vaccines aimed at reducing infection with Pseudomonas aeruginosa have been developed, and it is important to know whether vaccination can prevent lung infection. We searched for randomised controlled trials and included three trials with 483, 476 and 37 patients respectively. No data are available from one of the large trials, which is unpublished. In the other large trial and in the small trial, the risk of getting a chronic infection was not decreased. In the large trial, one patient was reported to have died in the observation period. In that trial, 227 adverse events (four severe) were registered in the vaccine group and 91 (one severe) in the control group. We cannot recommend the use of vaccines against Pseudomonas aeruginosa.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2013

Vaccines for preventing influenza in people with cystic fibrosis

People with cystic fibrosis have blocked airways which results in frequent airway infections. Infections with viral diseases like influenza ("the flu") can worsen lung damage. Doctors therefore often advise people with cystic fibrosis to be vaccinated against influenza every year. We searched for studies which compared different vaccines or compared vaccination to placebo. We were able to include four studies with 179 people in the review. Most (143) were under 16 years old. No study compared one vaccine to placebo. There were a high number of drop outs in two of the studies. Vaccination does result in an immune system response to the types of influenza used in the vaccine. However, this response may not result in protection against influenza infection or lung damage. There were a high number of adverse events, but none were serious or persistent. There is no evidence to show if regular influenza vaccine benefits people with cystic fibrosis.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2014

Interventions to clear meticillin‐resistant Staphylococcus aureus (MRSA) from the lungs of people with cystic fibrosis

We looked for evidence to determine the effect of different ways of clearing meticillin‐resistant Staphylococcus aureus (MRSA) from the lungs of people with cystic fibrosis.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Pneumococcal vaccines for children and adults with cystic fibrosis

Cystic fibrosis is an inherited disease which causes mucus in the lungs to be more sticky than normal, leading to frequent lung infections and lung damage. Pneumococcus is a type of bacteria that can cause infection throughout the body, including in the urine, blood stream, fluid around the brain and in the lungs. These infections can be very serious and may lead to severe illness and death. There are two types of vaccine that can be used to help protect people against some forms of pneumococcus bacteria. One vaccine is used in children under two years of age and the other is used in older children and adults. This review aimed to find trials that compared giving the vaccine against no vaccine or an inactive (placebo) form to assess how useful vaccines against pneumococcus bacteria could be in reducing serious illness and death in people with cystic fibrosis. No trials were identified that assessed these vaccines in people with cystic fibrosis. The evidence to support the routine use of vaccines against pneumococcal infection is not strong; however, many countries are now recommending all children have the vaccine as part of their childhood immunisation programmes.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2014

Antibiotic treatment for Stenotrophomonas maltophilia in people with cystic fibrosis

Stenotrophomonas maltophilia is a bacteria which is resistant to several antibiotics. Over the last 10 years this bacteria has increasingly been found in the lungs of people with cystic fibrosis. Chronic infection with Stenotrophomonas maltophilia has been found to be linked to pulmonary infections. However, at present, it is unclear if people with cystic fibrosis should be treated for this bacteria when it is grown in their lungs. The purpose of this review is to determine if treatment with different antibiotic combinations for Stenotrophomonas maltophilia will improve lung function or decrease the frequency of hospital admission in people with cystic fibrosis. We also want to review the effect of treatment of chronic Stenotrophomonas maltophilia infection in order to clear this bacteria from the lungs of a person with cystic fibrosis. We did not find any randomized controlled trials which we could include in the review. We did find one trial of antibiotic treatment for pulmonary exacerbations which included patients with Stenotrophomonas maltophilia, but the patients in the trial also had infections caused by other bacteria and we were not able to obtain data separately for the different causes of infection. Randomized controlled trials are needed to inform clinicians as to whether they should treat Stenotrophomonas maltophilia infection in people with cystic fibrosis. In the meantime, clinicians should use their clinical judgement when considering this question.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Tube feeding (mainly via the nose into the stomach or directly into the stomach) in people with cystic fibrosis

We planned to review the evidence about the effects of tube feeding (via either the nose or the stomach) in people with cystic fibrosis.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Treatments to cure long‐term infections with Burkholderia cepacia in people with cystic fibrosis

We reviewed the evidence for antibiotic treatment to cure early infection with Burkholderia cepacia complex in people with cystic fibrosis and prevent it becoming permanent.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2014

Elective (regular) versus symptomatic intravenous antibiotic therapy for cystic fibrosis

Chronic infection of the airways by Pseudomonas aeruginosa in people with cystic fibrosis is associated with deterioration in respiratory function. Intravenous antibiotics are the standard therapy for pulmonary exacerbations caused by this micro‐organism. Many centres advocate the use of elective (regular) three‐monthly antibiotics to reduce the frequency of exacerbations and therefore slow the deterioration of lung function. Alternatively, intravenous antibiotics are only prescribed when symptoms indicate. Elective therapy may encourage multi‐resistance to antibiotics. This review aimed to identify randomised and quasi‐randomised controlled trials that evaluated the results of the two different approaches. No clear conclusions were identified. This review will no longer be regularly updated. Searches will still be undertaken on a two‐yearly basis by the Cochrane Cystic Fibrosis & Genetic Disorders Group. If, in future, relevant trials are identified, the review will be updated again.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2015

Drugs to control the movement of salt in the lungs of people with cystic fibrosis

Usually salt is absorbed from liquid on the surface of the lungs to stop fluid building up there. In cystic fibrosis too much salt is absorbed and there is little liquid left on the airway surfaces. As a result people with cystic fibrosis have problems clearing their lungs of sticky secretions. Thus the lungs are left open to infection. Drugs applied directly to the lungs may prevent too much salt being absorbed. This may lead to better clearance of mucus and less lung infection and damage. The review includes five studies with a total of 226 participants. All studies looked at short‐acting drugs applied directly to the lungs compared to placebo. When the results from three studies over six months were combined, the placebo group showed a slower rate of decline in forced vital capacity than the treatment group. However, each of these differed in their results. A further study showed that people receiving a sodium channel blocker before receiving hypertonic saline did not achieve better lung function or mucus clearance than those receiving placebo before hypertonic saline. We found no evidence that short‐acting sodium channel blockers administered directly to the lungs improve respiratory status in people with cystic fibrosis. There was even some limited evidence of a decline in lung function. Trials of long‐acting sodium channel blockers are ongoing.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2014

Not enough strong evidence about the effects of positive expiratory pressure (PEP) devices for chest physiotherapy for people with cystic fibrosis

Cystic fibrosis (CF) causes frequent respiratory infection and blocks the airways with mucus secretions. Chest physiotherapy is frequently used to try to clear these secretions out of the lungs. Positive expiratory pressure (PEP) devices provide pressure behind the mucus to try to push it out of the lungs. The review of studies found only weak evidence about the effects of PEP. The evidence does not show that PEP is more effective than other methods of chest physiotherapy. There was some evidence that people with CF may prefer PEP to other chest physiotherapy methods, but more research is needed.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2009

The timing of inhalation of dornase alfa in people with cystic fibrosis

Inhaling the enzyme dornase alfa reduces the stickiness of sputum and improves clinical outcomes in people with cystic fibrosis. It is not certain whether it is better to inhale dornase alfa before or after clearing the airways with physical techniques. It is also not clear whether it is better to inhale it in the morning or in the evening. We included five trials with a total of 122 participants. In these trials the length of treatment ranged from two to eight weeks. Four of the trials compared inhaling before to inhaling after the airways had been cleared and found no overall difference in clinical outcomes. However, in children with well‐preserved lung function, inhaling of dornase alfa after airway clearance techniques was better for small airways function. However, this did not affect quality of life or other outcomes. In the remaining trial, morning versus evening inhalation had no impact on lung function or symptoms. Therefore, for many people with CF, the timing of dornase alfa inhalation (before or after airway clearance or the time of day) can be based on practical reasons or individual preference. 

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2013

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