Clinical Care
Staying healthy requires aggressive, life-long therapies to prevent disease progression.
Managing Treatments
Airway Clearance
Airway clearance is very important as it removes mucus from the lungs, preventing obstruction of the small airways and infections. Individuals with cystic fibrosis (CF) should perform airway clearance at least one to two times a day and more often when they are ill. The types of airway clearance techniques an individual may use will change throughout their lifespan. Individuals should choose a technique they will want to do every day.
Airway clearance
Chest Physical Therapy (CPT)
Chest physical therapy (CPT) consists of three components:
- Postural drainage
- Percussion
- Vibration
Chest physical therapy is started in infancy and can be used at any age. It is a passive form of treatment for the person with CF and an active one for the caregiver. The goal of CPT is like getting ketchup out of a ketchup bottle. You turn the bottle over (postural drainage), hit the side of the bottle to loosen the ketchup (percussion), and then shake the bottle to allow the ketchup to flow out of the bottle (vibration). Download a brochure on chest physical therapy.
Postural Drainage
This technique uses a series of gravity dependent positions to drain mucus from the lungs. Positions are limited for infants, keeping them upright or side-lying because they tend to spit up. Older children can tolerate all of the positions that an adult performs. People with gastroesophageal reflux disease should avoid any head-down positions, as it may increase reflux.
Percussion
Using cupped hands, or a manual device, caregivers create a hollow clapping sound to loosen lung secretions. For infants, the percussion is made with 2-3 fingers, a bottle nipple, or a percussor cup. Percussion is typically performed for approximately 2-5 minutes in each position. For older children and adults, CPT is performed using the whole hand, even doing both sides simultaneously, for a session lasting 25-30 minutes.
Vibration
By using their hands or manual devices on the chest, caregivers can vibrate or shake secretions from lung walls, allowing the person with CF to clear them by coughing. In infants, this force can be created by using “Tickle Me” toys, a bouncy seat with vibration, or bouncing the baby on your knee. Using a trampoline, tumbling, performing wheel barrow races, and tickling are good ways to achieve this with older children. Creating a vibration force in adults is done using one or two hands to vibrate over specific lung segments.
PEP Therapy
Positive Expiratory Pressure (PEP) therapy is done by breathing through a device that provides resistance while exhaling. This pressure keeps the small airways of the lungs open to improve the mobility of the secretions. There are many different forms of PEP therapy including: Acapella®, Flutter®, TheraPEP®, and Bubble PEP®, are some. Each treatment should be done twice a day for 15-20 minutes.
Acapella®
This oscillating device provides pressure to shake secretions from the lung walls. It comes in two different levels of PEP (blue for younger children and green for older children and adults), each adjustable. The Acapella® can be used in any position and can be combined with postural drainage positions. The Acapella® is great for kids starting PEP therapy.
Acapella®
Flutter®
Unlike the Acapella®, this oscillating PEP device does not come in different levels of PEP and is not adjustable. Therefore, it’s more difficult for younger children to use.
TheraPEP®
This PEP device comes with a visual coach to assist with the exercise. There is no oscillation, but the device has six different settings, which make it easier for younger children to use.
Bubble PEP
This is an easy, cheap and fun way for children to do their airway clearance every day. To make a bubble PEP, fill an empty milk jug half way with water; then insert a rubber tube for the child to blow through. Picture blowing bubbles in a glass of milk. Food coloring can be added to make it even more fun for the kids, friends and family.
High Frequency Chest Wall Oscillation
High Frequency Chest Wall Oscillation, or HFCWO, works by wearing an air filled vest that is connected to a compressor pump that provides a vibration force to all segments of the lungs. The frequency and pressure of the force can be adjusted to an individual’s tolerance. Different sized vests are available. A treatment should last 20-30 minutes, and should be combined with huffing and coughing to enhance the movement of secretions. Portable devices are also available that do not require a compressor. These devices work by a different mechanism to provide chest wall oscillation.
Vest
Huffing
Huffing is a forced expiratory technique that allows mucus to move up through the airways. An individual breathes out forcefully, pushing mucus from deep in the lungs into larger airways, where it can then be coughed out. Unlike coughing, huffing doesn’t collapse airways, but rather provides a gentle squeezing to move the mucus up. Picture a tube of toothpaste. If you squeeze just below the opening, you’ll get a tiny bit of toothpaste out. But if you start down low and gently squeeze while working your way up to the top, you’ll get much more toothpaste out. Similarly, huffing allows you to get more mucus up because you are gently working it up from the periphery of the lungs.
To Huff
- Inhale using your diaphragm (i.e. belly breathing).
- Hold your breath for 3 seconds.
- Tilt your head up slightly.
- Blow out forcefully and slowly through a wide open mouth (as if fogging up a mirror, NOT like blowing kisses). You should not hear any wheezing.
- Blow out for about 4 seconds.
Active Cycle of Breathing
Active cycle of breathing techniques, or ACBT, consist of three different breathing patterns to clear secretions—breathing control, thoracic expansion, and forced expiratory technique. Each one can be performed independently or in combination with other forms of airway clearance.
Breathing Control
In “belly breathing,” or diaphragmatic breathing, you use the lower chest to breathe while keeping the upper chest and shoulders relaxed. Breathing control allows for rest while performing ACBT, and prevents any increase in airway obstruction. The length of this phase varies depending on the severity of an individual’s symptoms.
Thoracic Expansion Exercise
This phase consists of deep breathing exercises, emphasizing inhalation. Individuals fill their lungs, and then hold their breath for 2-3 seconds before breathing out. This allows the air to get behind the mucus to help move it up. Individuals should take 3-4 deep breaths in this phase.
Forced Expiratory Technique
Combining huffing and breathing control, individuals breathe in deeply, hold for 2-3 seconds, then breathe out slowly and forcefully, as if fogging up a mirror. You should not hear wheezing as they blow out. Repeat 2-3 times followed by a cough, if needed, to clear secretions. Follow the huffing with breathing control.
The sequence of ACBT is very flexible, allowing you to cycle through the three phases to move secretions up so you can clear them. Alternate between breathing control and thoracic expansion exercise until you feel the need to cough, and always return to breathing control after huffing to prevent the airways from tightening. ACBT can be combined with postural drainage positions and with HFCWO.
Autogenic Drainage
This technique is done by inhaling different volumes of air to move secretions from the outer segments of the airways to the larger ones. It takes a lot of breathing control to do this well. Breathing at low-lung volumes is called the collecting phase because it loosens mucus from the airway walls. Breathing at mid-lung volumes is also called the collecting phase since it moves mucus into the larger airways. The final phase, which involves breathing at larger lung volumes to cough and clear the secretions, is called the evacuating phase.
Games
Games and toys can help strengthen the lungs and develop breathing control, while making therapy fun. Wind instruments are a great way to practice breathing control and to give your lungs a good work-out. Cotton ball races, blowing cotton balls across a table with a straw, are a good game that a child can play with siblings, friends or parents. Blowing bubbles, pinwheels, kazoos and harmonicas also make treatment fun to keep children interested.
Bladder
Urinary incontinence is a common problem for girls and women with CF due to weak pelvic floor muscles. These muscles form a sling that runs from the front pubic bone to the tail bone, supporting the structures in the lower abdomen and pelvis. Strengthening these muscles with Kegel exercises can help relieve the symptoms of incontinence. To perform the exercises, one might imagine trying to stop the flow of urine midstream by contracting the pelvic muscles, while trying not to use the abdominal or leg muscles. By tensing these muscles for 10 seconds, relaxing the muscles, and repeating this exercise, the pelvic floor muscles will grow stronger and decrease incontinence symptoms. You will notice a change after 2 weeks of daily practice. Performing a Kegel exercise before coughing will help to prevent any leakage from occurring.
Many women hesitate to discuss incontinence with their physician, but physicians may have more treatment options to offer, particularly if the pelvic floor exercises do not resolve the problem. Male with CF can also experience urinary incontinence.
Pelvic floor exercise
Bone Health
Why do people with CF develop osteoporosis?
Osteoporosis, which is common in the elderly without CF, can affect people with CF at any age. Osteoporosis makes people with CF more prone to fractures, even without trauma. People with CF often have inadequate vitamin D levels, which impairs bone formation. Vitamin D is a fat-soluable vitamin that is poorly absorbed by people with CF. Additionally, people who do not get enough exposure to sunlight, which converts vitamin D into its active form, also may be prone to osteoporosis. Accelerated bone breakdown is related to inactivity, malnutrition, and steroid use. Chronic CF lung disease also releases inflammatory factors causing more rapid bone breakdown. People with recurrent pulmonary exacerbations are at a higher risk for osteoporosis, and lung transplant recipients have an accelerated rate of bone loss related to use of immunosuppressive drugs such as corticosteroids. Men are more often affected than women, which scientists believe is related to low sex hormone levels important to bone development, and to delayed onset of puberty. Diabetes, alcohol use, and cigarette smoking may also hasten the onset of osteoporosis.
Measurement of bone density by DXA scan in individuals with CF detects early osteoporosis.
Detection of Osteoporosis
Adults with CF should be screened every 1-5 years for osteoporosis with a DXA (Dual-energy radiograph absorptiometry) scan. Using two separate X-rays, bone density is measured at the spine, femur, or other site. DXA scan results are reported as T-scores and Z-scores. These values tell doctors how far the bone density is from the expected normal value. T-scores of -1.0 to -2.5 indicates osteopenia (the early stage of bone weakening) and a score of less than -2.5 is consistent with osteoporosis. Individuals with CF should also have serum 25-hydroxy-vitamin D levels checked yearly.
Osteoporosis prevention
Preventing osteoporosis is a vital part of the overall care of those with CF.
To reduce your risk,
- Exercise to build strong bones
- Maintain good nutritional intake, especially calcium and vitamin D.
Osteoporosis Treatment
Osteoporosis is treated by maximizing nutritional intake, with special attention to vitamin D and calcium. Sunlight helps bone health by increasing active vitamin D in the body. A daily exercise program will also strengthen bones. Depending on the severity of the disease, biosphosphonates may be prescribed to help encourage new bone growth.
Diabetes
What is Cystic Fibrosis-Related Diabetes (CFRD)?
Cystic fibrosis-related diabetes (CFRD) occurs when the body does not use the hormone insulin properly. Normally, food is converted into sugar, fat and protein. As sugar enters the blood it signals the pancreas to secrete insulin, allowing cells to derive energy from food. People with diabetes either do not make enough insulin (Type 1) or do not respond appropriately to insulin (Type 2), which means that food is not converted into energy. Cystic fibrosis-related diabetes has characteristics of both Type 1 and Type 2 diabetes. Diabetes treatments aim to keep blood sugar levels as close to normal as possible.
Screening for CFRD
The Cystic Fibrosis Foundation recommends that blood glucose levels be tested yearly. During hospitalizations, glucose levels are typically measured in the morning while fasting and after eating. An oral glucose tolerance test (OGTT), performed annually in individuals over age 9 years, measures blood glucose before and two hours after drinking a very concentrated glucose drink. The OGTT, which can diagnose diabetes and milder forms of blood sugar abnormalities, divides people into four categories: normal glucose tolerance (NGT), impaired glucose tolerance (IGT), diabetes without fasting hyperglycemia (the blood sugar is normal first thing in the morning but gets high after eating), and diabetes with fasting hyperglycemia (the blood sugar is always high, even without eating).
Treatment of CFRD
Cystic fibrosis-related diabetes is treated with insulin which is administered subcutaneously. Oral medications used to treat Type 2 diabetes in people without CF are not currently recommended to treat CFRD.
Exercise
Exercise is an essential part of living with CF, as it improves mucus clearance and cardiovascular and lung function. People who exercise regularly report increased feelings of well-being and improved quality of life. There are many different options for exercise, including making it a family activity. Parents should be role models for a healthy lifestyle.
Flexibility
Aerobic Activity
This is body movement that increases heart rate. Some aerobic activities—basketball, gymnastics, jumping rope, lacrosse, running, soccer and swimming—are more beneficial than others. More sedentary activities, like golf and baseball, are still fun but will not have as much of an impact on lung function.
Weight Training
Lifting weights three times a week improves both muscle strength and cardiovascular function. Weight training also helps fight osteoporosis by strengthening the bones. You’ll get better results by increasing the amount of weights you’re using on a regular basis. A good rule of thumb is to increase the weight lifted when you can do three sets of eight repetitions at the current weight. Remember to give your muscles at least a one-day break between workouts.
Posture
Proper posture is something that most of us forget about, but poor posture can place undue stress on our bodies and adversely affect the lungs. Take a deep breath while sitting upright, then slump over and take a deep breath. When you sit upright, you can get more air into your lungs than when you slouch. People with CF tend to sit in a more slouched posture due to coughing. Exercises such as squeezing your shoulder blades together ten times will help strengthen muscles that maintain good posture. It is an easy exercise to do throughout the day.
Infection Control
Everyone inhales bacteria, but the lungs are usually protected from infection by a thin blanket of mucus that coats the lining of the airways and traps bacteria, which are then transported out of the lungs. However, the thick, sticky mucus in the lungs of individuals with CF allows the bacteria to survive. Over time, the lungs of people with CF become chronically infected with bacteria, such as Pseudomonas aeruginosa and Staphylococcus aureus. Although most infections are acquired from the environment, bacteria can be spread from one person with CF to others with CF.
Infection control is defined as “strategies that prevent the acquisition and spread of bacteria.” The most important way for people with CF to prevent infection is frequent hand-washing with soap and water or an alcohol-based cleaner. Proper cleaning of nebulizers and other equipment can also prevent the acquisition of bacteria.
The best way to minimize the spread of bacteria between people with CF is to prevent prolonged direct contact. Coughing allows droplets carrying bacteria to leave the lungs of individuals with CF. Since these droplets can travel up to 6 feet, it is important for individuals with CF to maintain a distance of 6 feet from each others with CF. To minimize the risk of spreading bacteria in clinic, individuals with CF should wear a mask when they come to the hospital or clinic. The staff typically wear protective gowns and gloves to prevent the spread of germs.
Antibiotic-resistant bacteria are becoming more widespread, including Burkholderia cepacia,Methicillin-resistant Staphylococcus aureus (MRSA) and multiple drug-resistant Pseudomonas aeruginosa (MDR-Pa). Most CF centers have specific policies for managing people that are colonized with these organisms.
For more information see Stopping the Spread of Germs (PDF) published by the CF Foundation.
Infection control
Liver Problems
Approximately five percent of people with CF will develop significant liver disease. Liver problems result when small ducts in the liver become blocked with thick, sticky bile characteristic of CF. Bile can no longer drain from the liver, damaging the organ. The resulting liver disease can be very mild, causing only slight variations in function, or very severe, leading to cirrhosis or liver failure. Individuals with CF are typically screened for liver problems with blood liver function tests annually.
Severe CF liver disease can lead to cirrhosis or scarring in the liver. The liver enlarges and there is increased blood pressure in the liver that causes the spleen to enlarge as well
Medical Therapy
When blood tests show elevated liver function testing, a medication called ursodeoxycholic acid (ursodiol) may be recommended. This medication increases the bile flow from the liver. If someone is diagnosed with liver dysfunction, fat-soluble vitamin levels need to be monitored closely. These vitamins may not be properly absorbed and additional supplements may be required. Individuals with CF and their care team must also ensure that vaccinations for Hepatitis B are up to date. Also, alcohol consumption, acetaminophen, and some herbal supplements or over-the-counter remedies can worsen liver damage and function.
Liver Transplantation
Depending upon the severity of the liver disease, transplantation may be an option. People with mild lung disease and severe liver disease are usually the best candidates for liver transplantation. In children and young adults, the estimated one-year survival after transplant is approximately 75-80 percent. Individuals who have undergone successful liver transplants usually have better nutritional status and improved quality of life. About 10 individuals with CF receive liver transplants each year in the U.S.
Medications
Antacids
There are two types of medications that block acid production that are frequently used to treat people with CF. Histamine H2 receptor blockers interfere with histamine, a signal for the stomach to produce acid. Proton pump (ATPase) inhibitors directly block the enzyme that makes stomach acid.
Drugs that block the production of stomach acid can be used to:
- Treat gastroesophageal reflux disease (GERD), in which excess acid enters the esophagus causing irritation and heartburn symptoms.
- Help pancreatic enzymes to work better. Pancreatic enzymes are packaged in tiny enteric-coated beads designed to protect them from stomach acid. Decreasing the amount of acid in the stomach usually allows more enzyme to reach the small intestine where they can digest food better in a neutral environment.
Antibiotics
Antibiotics are drugs that kill bacteria. Antibiotics are frequently administered to individuals with CF as:
- Treatment for newly acquired bacteria, such as Pseudomonas aeruginosa.
- Treatment for increased pulmonary symptoms or an “exacerbation.”
- Chronic therapy for those who have persistent or recurrent symptoms.
- Suppression of chronic P. aeruginosa infection
Antibiotics may be given orally, by inhalation or intravenously. There are several classes of antibiotics, which work by different mechanisms. Penicillins and cephalosporins, for example, affect the bacterial cell wall. Fluoroquinolones, such as ciprofloxacin, block an enzyme required for DNA synthesis and bacterial growth. The combination antibiotic trimethoprim-sulfamethoxazole blocks folic acid production needed for bacterial growth.
Bronchodilators
Bronchodilators relax the muscle surrounding the airways, enlarging them and aiding in airway clearance. These medications are also used to treat wheezing that may occur in CF or asthma. Short-acting bronchodilators, such as albuterol or levalbuterol, act rapidly to improve symptoms. Long-acting bronchodilators, such as salmeterol or formoterol, often combined with an inhlaed steroid are maintenance medications used to prevent the airways from narrowing. These latter medications should not be used to relieve acute symptoms of shortness of breath or wheezing.
CFTR Modulators
Cystic fibrosis is caused by defective chloride transport through a channel, called CFTR, which is usually located on the surface of cells throughout the body. Medications that treat the basic abnormality by improving CFTR function as called modulators. These oral medications partially restore the function of CFTR. Several different abnormalities in CFTR can lead to CF. Therefore, different modulators have been designed to fix specific problems with the CFTR protein production and function. In some cases, a combination of modulators is needed to restore chloride transport.
Mucus Modulating Therapies
These medications thin the mucus within the lungs. Pulmozyme® is the brand name for recombinant human DNase (rhDNase or dornase alfa), which is an enzyme that breaks down the DNA in the mucus in the CF airways. There is extra DNA in the airway from the bacteria and white cells that are located in the mucus. This therapy is typically given once daily before airway clearance is performed.
Hypertonic saline, a solution with a high concentration of salt, is typically nebulized twice daily. Hypertonic saline also causes people to cough increasing the amount of mucus cleared from the lungs.
Medication
Pancreatic Enzyme Replacement Therapy
Pancreatic enzyme supplements help overcome CF pancreatic dysfunction. These pills contain amylase, lipase and protease, which help to digest starches, fats and proteins, respectively. Enteric-coated enzyme preparations help prevent destruction of the enzymes in the acidic stomach environment.
Individuals with CF usually need 500 to 4000 units of lipase per gram of fat per day. Dosages can also be based on body weight, with approximately 2000 units of lipase needed per kilogram of body weight for every meal (and half of that amount for snacks). The dosage may be adjusted based on symptoms or results of a 72-hour stool collection. If a correct dosage is ineffective, this may be due to the acidity of the GI environment.
Steroids
Steroids, or corticosteroids, are potent anti-inflammatory medications produced naturally in the adrenal gland. Steroids can be inhaled, given orally or intravenously. Typically they are used to treat people who wheeze. Steroids are also the best therapy for allergic bronchopulmonary aspergillosis (ABPA).
Steroids have several side effects including: decreased height growth, glucose intolerance, high blood pressure, reduced bone density, and the formation of cataracts. These problems are more likely to occur when larger doses are used, especially for prolonged periods. Inhaled steroids have a lower risk of side effects than oral steroids.
Alternative Medicines
There are many unproven nutrition therapies that promise to cure, help, or lessen CF symptoms and ailments. Although it might be tempting to try these products, discuss all therapies with your CF team first. Herbal supplements, or other nutrition supplements, can be dangerous to your health. Information about alternative and complementary therapies can be found at the National Center for Complementary and Integrative Health website.
Reproductive Issues
There are many misconceptions about how cystic fibrosis (CF) affects sexual and reproduction capabilities. Most misunderstandings are related to genetics, fertility, pregnancy and childbirth.
Genetics
Cystic fibrosis is an autosomal recessive disorder, which means a person needs to have two copies of an abnormal, or mutated, CFTR gene to have the disease. People with only one copy are “carriers” and do not have CF. If two carriers have a child there is a 25 percent (1 in 4) chance that the child will have CF. The risk of having a child with CF is the same with every pregnancy. If someone with CF has children with someone who is not a carrier, all of the children will be carriers but none will have CF. Approximately 1 in every 25 Caucasians is a carrier of a CFTR gene mutation. Screening women considering pregnancy and their partners for CFTR mutations is recommended.
Fertility
It is very important for women with CF know they have relatively normal fertility and can become pregnant. The ability to become pregnant and carry a child to term will depend on a woman’s overall health. In general, women with good nutritional status and lung function will not be adversely affected by pregnancy. However, those who are in poor health may suffer a more rapid decline in their health after pregnancy. Whether a woman is healthy enough and has the needed support system to care for a child over the subsequent years is also a consideration.
Conversely, most males with CF are infertile and cannot father children without assisted reproductive technology. Over 95 percent of boys with CF are missing the vas deferens—the tubes that connect the testes to the urethra. Sperm is produced but it cannot leave the testes. However, since sperm is produced, assisted technologies can be used to aid men in becoming fathers.
Infertile males with CF who want children have several options. Microepididymal sperm aspiration (MESA), in which a small amount of sperm is extracted from the epididymis and used for in vitro fertilization, is another. This procedure requires the mother to take hormone injections and have eggs harvested, fertilized and re-implanted. While the success rates for this procedure have improved significantly over the years, it is time consuming and expensive. Addition options for those with CF interested in having a family include adoption and surrogacy.
Pregnancy
Having a Baby for most people with CF and partners of someone with CF starting a family raises many questions. “Is having a baby right for me?” is at the top of the list. Men and women with CF need to consider whether they have the family support to care for an infant and young children. Determining whether a parent will be healthy in 5, 10, or 15 years and ensuring adequate assistance during times of illness are paramount. People with CF need to meet the demands of taking care of themselves and their child.
Future mothers with CF should discuss pregnancy with their doctors before becoming pregnant. Patients, obstetricians and CF doctors should communicate frequently before and throughout pregnancies. Maintaining good lung function and nutritional status are key during pregnancy. Pregnant women with CF must stop taking Vitamins A, D, E and K and begin taking prenatal vitamins with supplemental Vitamin E. Antibiotics such as fluoroquinolones and tetracyclines should be stopped as well. Additional screenings for diabetes and elevated blood sugars are also needed.
Sexually Transmitted Diseases
People with CF are susceptible to all sexually transmitted diseases (STDs) including HIV/AIDS, herpes, syphilis, gonorrhea, and chlamydia. Despite reproductive problems, males with CF can still be infected by and spread STDs. It is important for men and women with CF always protect against sexually transmitted diseases.
Sinus Problems
Most people with cystic fibrosis (CF) have at least some sinus disease. The sinuses, which are usually filled with air, are typically full of thick, sticky mucus in people with CF. The goal of therapy is to decrease inflammation within the sinuses and nasal passages and to promote mucus clearance. This is typically achieved by using inhaled steroids and some form of irrigation.
Nasal Steroids
Inhaled nasal steroids are effective in reducing inflammation in the sinuses and can be used for long periods of time. The onset of action is not as immediate as decongestants, but inhaled nasal steroids provide sustained relief. Decreasing inflammation decreases the swelling within the lining of the sinuses. Typically, nasal steroids are used routinely once or twice daily, but they can be used on an as-needed basis in some cases.
Irrigation
Because the mucus within the sinuses does not drain easily, irrigation with saline can be helpful. There are several commercial irrigation solutions that can be purchased. Alternatively, irrigation saline can be made at home. The saline can be applied using a squeeze bottle that makes a mist. For older children and adults, nasal nebulizers are available.
Antibiotics
Antibiotics are required for treating an acute sinus infection. Symptoms include fever, post-nasal drip, congestion and/or sinus pain. The organisms that infect the sinuses are similar to those found in the lungs. Pseudomonas aeruginosa sinus infections are common in people with CF, as well as infections from bacteria such as Staphylococcus aureus. Sinus infections are often difficult to treat requiring many weeks of antibiotic therapy. Symptomatic improvement will also be helped by irrigation with saline.
Sinus Surgery
Sinus surgery, which removes infectious material and improves sinus drainage, is sometimes necessary when medical treatment does not resolve chronic symptoms or prevent recurrent episodes of acute sinusitis. Sinus surgery is sometimes combined with the removal of polyps, which may be interfering with breathing, causing pain, or contributing to infections. Unfortunately, nasal polyps frequently recur after removal.
Nebulizer Treatment
Many treatments for CF lung disease are delivered in an aerosol or nebulized form. Aerosol medication requires a nebulizer to hold the drug, and a compressor to force air through the medicine, allowing it to be inhaled. Many nebulizers and compressors do not deliver CF medications correctly, which is why you should always discuss your equipment selection with your CF providers.
Order of Treatment Administration
If multiple medications are being used at the same treatment session in most cases medications should be administered in the following order. Not everyone will use all these therapies.
- Bronchodilator (albuterol or levalbuterol)
- Hypertonic saline
- Dornase alfa
- Airway clearance
- Inhaled antibiotic (aztreonam, colistin or tobramycin)
- Inhaled steroid
Cleaning Your Nebulizer
To reducing the risk of infection, thoroughly wash and disinfect all parts of the nebulizer after use. Let the nebulizer air dry completely between treatments. Once dry, ensure the nebulizer nozzle is clear and unclogged.
| To Disinfect Your Nebulizer | |
|---|---|
| Boil | In water for 5 minutes. |
| Soak | In 1:50 bleach/water solution for 3 minutes. |
| Soak | In 70% isopropyl alcohol for 5 minutes. |
| Soak | In 3% hydrogen peroxide for 30 minutes. |
| Microwave | (In water) for 5 minutes. |
| Dishwasher | For at least 30 minutes. |
Do not use vinegar for disinfection since it does not kill all the bacteria and viruses that can grow on your nebulizer. If you use bleach, alcohol or hydrogen peroxide, rinse your nebulizer well with sterile water and air-dry all equipment completely.
Nebulizer treatment