Medications Used To Treat Cystic Fibrosis
The CF Foundation recommends the following order of inhaled medications:
- Dornase alfa
- Aerosolized antibiotic
These drugs are used in combination with airway clearance techniques in a specific order designed to move mucus out of the lungs. Other medications that people with CF often take include oral and IV antibiotics, CFTR modulators, and ibuprofen, in addition to nutritional supplements such as pancreatic enzymes and vitamins.
How Cystic Fibrosis Is Treated
There have been many advances in CF treatment. Patients can take medications to help thin and clear the thick mucus from the airways, enzymes to help absorb fat and nutrients, and antibiotics to treat infections. There are also new treatments that target fixing the CFTR protein.
Airway Clearance Therapy
People with CF need to perform airway clearance therapy . This can be done using manual chest physical therapy or a device worn over the chest that helps clear airway secretions by shaking the mucus in the airways, enabling you to cough it up. Another portable mucus clearing device involves breathing through a mask or mouthpiece. This device forces you to breathe out with more pressure, dislodging mucus in trapped in the airways.
Mucus Thinning Medication
A nebulizer or inhaler is often used before performing ACT. Commonly used medications are albuterol that relaxes the airway and helps clear and thin mucus so you can cough it up more easily.
Enzymes and Nutrients
Pancreatic enzyme replacement therapies help the body absorb food and necessary nutrients. Enzymes have to be given before every meal or snack. People with CF also have to take certain vitamins that are absorbed with fat. Additional salt needs to be provided in formula or food.
Antibiotics and Anti-inflammatories
Antibiotics are frequently needed to treat bacteria that grow in the mucus. These can be given in one of three ways:
Treatment Options For Adults With Cystic Fibrosis
Daily Treatments: Treatment for cystic fibrosis is not a one-time thing it takes daily treatments, which you administer yourself, to stay as healthy as possible. Daily treatments generally include:
- Airway clearance techniques to loosen mucus and clear it from the airways
- Nutrition therapies
To maintain optimal lung function, mucus-thinning medicines and bronchodilators will most likely be added to your medical routine.
Antibiotics are used regularly for lung infections. If intravenous antibiotics are needed, hospitalization will be required.
In severe cases, lung transplantation may be required.
Clinical trials for new therapies are available for those who qualify.
Read more about transitioning to adult CF and our Adult Cystic Fibrosis Clinic.
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Tailoring Antibiotic Treatment For Patients With Cystic Fibrosis
Medical illustration of multidrug-resistant Pseudomonas aeruginosa from CDCs 2019 AR Threats Report. P. aeruginosa infections can be particularly dangerous for patients with chronic lung diseases.
Conan Y. Zhao, Quantitative BioSciences graduate student, Georgia Tech and Sam P. Brown, PhD, Associate Professor, Georgia Tech, and Alison Laufer Halpin, PhD, Associate Director, Office of Scientific Innovation and Integration, CDC
Healthcare providers can face two growing crises that affect their ability to treat bacterial infections: antibiotic resistance and chronic infections.
A difficult combination for healthcare providers and patients are chronic infections caused by antibiotic-resistant germs.
Through a project with CDC, the Brown Lab at Georgia Tech developed new approaches to study the lung microbiomes of patients with cystic fibrosis , who often get chronic infections. This work will help improve treatments for these patients.
Chronic infections are persistent illnesses that need to be treated for a long time before the infection clears.
Patients with cystic fibrosis are at particular risk for chronic infections. One of the traits of CF is build-up of a thick mucus layer coating the inside of the lungs, called sputum. Many types of commensal and harmful bacteria can survive in this environment, continually putting patients with CF at risk of lung infections. Because of these infections, CF patients are exposed to more antibiotics.
Oral Antibiotics For Respiratory Illnesses
These are medications used to treat bacteria in the lungs or sinuses that can cause an infection. Common antibiotics used in CF include:
- Amoxicillin and clavulanic acid
- Sulfamethaxazole and trimethoprim , sometimes in combination with rifampicin
Antibiotics are chosen based on what bacteria are found on respiratory cultures, the symptoms during an illness, any allergies and a childs previous response to antibiotics.
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Why Treat A Pulmonary Exacerbation
Even though the definition of a pulmonary exacerbation is not universally agreed, there is a consensus that acute pulmonary exacerbations should be prevented, where possible, or promptly and aggressively treated where prevention has failed. This is crucial to improving or maintaining lung function, improving quality of life and prolonging survival. As well as causing acute worsening of symptoms, a decline in weight or body mass index and an acute decline in lung function, pulmonary exacerbations also contribute to the long-term deterioration in lung function in patients with CF . Worryingly, up to a quarter of patients do not recover to baseline within 3 months of treatment of the pulmonary exacerbation with i.v. antibiotics . Therefore, it is very important that there is an effective approach to early identification and treatment of pulmonary exacerbations.
Dietary And Nutritional Advice
Eating well is important for people with cystic fibrosis because the mucus can make it difficult to digest food and absorb nutrients.
The pancreas often doesn’t work properly, making it even harder to digest food.
A dietitian will advise on how to take in extra calories and nutrients to avoid malnutrition.
They may recommend a high-calorie diet, vitamin and mineral supplements, and taking digestive enzyme capsules with food to help with digestion.
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Assessing The Lung Microbiome
The microbiome is a community of naturally-occurring germs. Antibiotics can disrupt microbiomes by changing the natural composition of both commensal and harmful bacteria. With a disrupted microbiome, antibiotic resistant disease-causing bacteria can take over and cause infection.
A number of studies have found connections between the lungs microbiome structure and the lungs function . For example, the presence of pathogenic germs are associated with poorer health.
These studies hint at the central role non-pathogenic germs can have to help maintain lung function. However, they have yet to answer if these germsmostly commensal oral germs in the mouth, nose, or throatare indicators of better health or actively help keep pathogenic germs at bay in the lung microbiome ecosystem.
To address this key question, the Brown Lab built an experimental modelexternal icon of the CF lung infection microbiome. A synthetic sputum recipe mimics sputum found in lungs of patients with CF. The lab adds defined combinations of up to 12 bacterial species to the model.
Together, these 12 bacterial species account for more than 90% of the bacterial community within the lungs of people with CF. Half of these germs are common pathogenic germs . The rest of the bacteria are mostly non-pathogenic oral germs often found in the lungs of people with CF.
How Are Bronchodilators Administered
Bronchodilators are inhaled through a device called a metered-dose inhaler. This device delivers a specific amount of medicine to the lungs. Less often, the medicine may be delivered through a nebulizer or a dry powder inhaler.6
Bronchodilators often come in an aerosol container that attaches to the canister. A spacer, a medical device, can be used with an inhaler to help more of the drug reach the lungs rather than getting sprayed on the back of the mouth and/or throat. After using a bronchodilator, all equipment should be cleaned to prevent the spread of germs. Most people take two puffs off their bronchodilator, about 1 minute apart.5
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Giving Antibiotics Regularly To People With Cystic Fibrosis To Prevent Lung Infection With A Germ Called
We reviewed the evidence about the benefits and adverse effects of giving regular antibiotics to people with cystic fibrosis to prevent lung infection with a germ called Staphylococcus aureus.
Cystic fibrosis blocks the airways with mucus and causes frequent airway infections. Over time, these can lead to lung damage and breathing failure. Most deaths in CF are caused by breathing failure. People with cystic fibrosis are sometimes given regular antibiotics to prevent infections from a germ called Staphylococcus aureus. However, antibiotics can also have side effects such as oral thrush or diarrhoea and long term use might lead to other more serious kinds of infection. This is an update of a previously published review.
The evidence is current to: 27 February 2020.
The review includes four studies with 401 children there were no adult studies. The children were put into groups at random and received either an oral antibiotic continuously as a prevention for at least one year or no antibiotic treatment to prevent infection with Staphylococcus aureus. All children could be given additional antibiotics if their doctor thought they needed them, based on symptoms and germs grown in their respiratory secretions. Studies lasted for a maximum of six years.
Quality of the evidence
Most recent search of the Group’s Register: 27 February 2020.
How Can Cystic Fibrosis Be Prevented
CF cannot be prevented. However, genetic testing should be performed for couples who have CF or have relatives with the disease.
Genetic testing can determine a childs risk of CF by testing samples of blood or saliva from each parent. Tests can also be performed on you if youre pregnant and concerned about your babys risk.
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Treatments For Cystic Fibrosis
There’s no cure for cystic fibrosis, but a range of treatments can help control the symptoms, prevent or reduce complications, and make the condition easier to live with.
Regular appointments to monitor the condition are needed and a care plan will be set up based on the person’s needs.
People with cystic fibrosis are treated by a team of healthcare professionals. Sometimes the condition will require treatment in hospital.
What Is Cystic Fibrosis
Cystic fibrosis affects the cells that produce sweat, mucus, and digestive enzymes. Normally, these fluids are thin and smooth like olive oil. They lubricate your organs and tissues, keeping them from getting too dry.
If you have CF, a genetic mutation increases the thickness of mucus and other body fluids. When this happens, the thicker fluids can interrupt the function of organs like the movement of air through your lungs. This can trap bacteria and lead to infections.
In more advanced cases, people with CF may have complications like respiratory failure and malnutrition.
Its critical to get treatment for CF right away. Early diagnosis and treatment can improve quality of life and lengthen ones life span.
Screening tests and treatment methods have improved in recent years, so many people with CF can now live into their 40s and 50s.
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Airway Surface Liquid Hydrators/mucolytics
Airway surface hydrators like nebulised hypertonic saline and inhaled mannitol improve mucociliary clearance when used for periods of 24 weeks. Nebulised hypertonic saline may have anti-inflammatory properties and improves some measurements of lung function . For the above reasons, nebulised hypertonic saline is being increasingly used as an adjunct to physiotherapy during pulmonary exacerbations. During acute pulmonary exacerbation, Dornase alfa appears to be safe and well tolerated but does not show a statistically significant therapeutic effect when added to antibiotics and physiotherapy . However, bronchoscopic instillation of Dornase alfa has been used successfully for lobar atelectasis in CF, which is unresponsive to medical therapy .
Is Trikafta More Effective Than Existing Cystic Fibrosis Gene Modulator Therapies
Yes, and significantly so. On average, Trikafta use leads to a 14% increase in lung function over the untreated baseline. In clinical trials of the triple combination therapy, people with two copies of the F508del mutation had a 10% increase in lung function on average compared to treatment with Symdeko , and people with a single copy of F508del had, on average, more than a 14% increase in lung function compared to treatment with the placebo.
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What Are Side Effects Of Antibiotics
Bacteria sometimes respond to antibiotics by changing so that the drug no longer weakens or kills them. This process is called antibiotic resistance. As antibiotic resistance develops, higher doses of the same antibiotic or different antibiotics are needed to get the same result.2
Other side effects of antibiotics vary depending on the drug, its dosage, and its delivery method . Gastrointestinal issues are especially common since antibiotics often kill the bacteria you don’t want along with the gut flora that helps you digest food causing diarrhea, nausea, or vomiting.
Other common side effects of antibiotics include headaches, jaundice, muscle aches, weakness, yeast infections, and rash. Again, the side effects vary greatly from person to person and between antibiotics.1
Inhaled Medications And Nebulizers
The Cystic Fibrosis Foundation and the Stanford CF Center staff recommend the following sequence for inhaled medications:
- Bronchodilators to open the airways
- Hypertonic Saline to mobilize mucus and improve airway clearance
- Pulmozyme to thin mucus
- *Airway Clearance Technique: Vest, Flutter, Chest PT, IPV, etc.
- Antibiotics . The previous therapies open and clear the airways of mucus, allowing these antibiotics to work on remaining bacteria.
* When using the Vest for airway clearance, make sure there is aerosol delivery during the entire vest session.
If you start coughing blood, temporarily stop Pulmozine, saline, airway clearance technique, and inhaled antibiotics. Call your CF doctor or nurse for further advice.
With a respiratory illness or change in symptoms:
- Begin or increase airway clearance techniques.
- Use breathing treatments as ordered you can use bronchodilators every three to four hours, and often additional Vest and/or hypertonic saline treatments are useful.
- Contact your CF doctor or nurse to see if antibiotics or additional intervention is needed.
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Antibiotic Enhancing Treatment For Lung Infections In Cystic Fibrosis
We reviewed the evidence about the use of agents to help antibiotics in treating lung infections in people with cystic fibrosis.
People with cystic fibrosis suffer from infections in their lungs as they produce thick secretions which allow bacteria to grow in them. Often the infections are caused by unusual bacteria, including Pseudomonas aeruginosa, and these bacteria become resistant to treatment with antibiotics. Long-term infection reduces a person’s quality of life and their lung function. There are no new antibiotics currently being developed which use a new type of action. New agents – antibiotic adjuvants – are needed to work alongside antibiotics to make bacteria more sensitive to either antibiotics or to the body’s own immune system, and to interfere with the formation of colonies of bacteria in the lungs.
The evidence is current to: 16 January 2020.
The review includes eight trials with 350 people with cystic fibrosis aged between five and 54 years of age. Trials compared different antibiotic adjuvants , garlic , a biological agent , nitric oxide and zinc ) with placebo and people were selected for one treatment or the other randomly. The trials lasted from two days to one year.
Quality of the evidence
To determine if antibiotic adjuvants improve clinical and microbiological outcome of pulmonary infection in people with cystic fibrosis.
Date of most recent search: 16 January 2020.
How Tobramycin Works
Aerosolized tobramycin has been extensively used in cystic fibrosis patients in order to directly deliver the antibiotic to the endobronchial site of infection, and decrease toxicity by limiting systemic absorption. Aerosolized tobramycin doses ranging from 80 mg twice or three times daily to 600 mg three times daily have been used in various clinical trials. At an 80-mg dose, preservation of pulmonary function with little or no improvement over the baseline was reported. Tobramycin, nebulized at 600 mg three times daily, significantly improved clinical and pulmonary functions and reduced the density of Pseudomonas aeruginosa in the sputum. No toxicity or nephrotoxicity was reported at either dose, investigators reported in the clinical study Efficacy and safety of aerosolized tobramycin in cystic fibrosis.
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What Is The Long
The outlook for people with CF has improved dramatically in recent years, largely due to advances in treatment. Today, many people with the disease live into their 40s and 50s, and even longer in some cases.
However, theres no cure for CF, so lung function will steadily decline over time. The resulting damage to the lungs can cause severe breathing problems and other complications.
Pulmonary Exacerbations And Inflammation
Persistent lower airway infection with inflammation is the major cause of morbidity and mortality in CF and contributes to decline in lung function. Neutrophil driven inflammation is exaggerated, prolonged and may even pre-date infection. Anti-inflammatory therapy, for example with Azithromycin, is well established in the treatment of CF lung disease. During pulmonary exacerbations, there is further aggravation of pulmonary inflammation however, this is hard to quantify and the systemic host inflammatory response is used as a marker of disease activity. During a pulmonary exacerbation, there is increased expression of some markers of inflammation and/or lung damage, for example C-reactive protein, white cell counts, interleukin-8, neutrophil elastase alpha 1-antiprotease complexes and matrix metalloproteins , in blood or serum. Circulating markers may not truly reflect the local inflammatory response in the lung however, this can be examined by measuring the sputum concentrations of bioactive lipid mediators, such as the cysteinyl leukotrienes and prostaglandin-E2 , or sputum cell counts or analysing exhaled breath or urine during exacerbations. However, none of these are routinely used in clinical practice.
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What Can I Do To Help Get Trikafta Funded Across Canada
Act Now! Lets Go #FurtherTogether
This May during Cystic Fibrosis Awareness Month, help Canadian children and adults with cystic fibrosis access the life-changing medicine they need. Join us as we go #FurtherTogether for access to Trikafta for ALL who can benefit from it, in every province and territory across Canada.
There are four actions you can take to help ensure all Canadians who need it get access to Trikafta.
Walk! Join the Walk to Make Cystic Fibrosis History and start fundraising to fuel our critical advocacy work.
Meet! Join our #FurtherTogether Meeting Challenge and get tools & tips to meet with your provincial elected official.
Write! Send a letter to your provincial elected official, using our template, and tell them how they can help.
Donate! Make a donation today to help fund the fight for access to life-saving medicines.
Take these key actions that will help to fuel and further our fight for access to Trikafta.Visit our #FurtherTogether page today to learn more.
No matter how you choose to get involved, you can help protect Canadian children from the cumulative and irreversible damage that happens growing up with cystic fibrosis. And help to lessen the decline in health of so many others living with cystic fibrosis. Please act now.