Myth #4: People with CF should not drink milk because it makes too much mucus

Fact: While it may feel like it, milk does not increase mucus production.

A 2005 review of studies concluded that there was no link between milk consumption and mucus production.

In one study, participants infected with the common cold virus reported symptoms of increased in mucus production after drinking milk, but when their mucus production was actually measured there was no statistical difference.

People with CF need to be able to perform airway clearance in order to get as much mucus as possible out of their airways. There are many devices available to assist with this such as vibrating vests, percussors, and even a technique called “clapping” which is performed by another individual.

Additionally, there are medications that have been developed to help thin the mucus and make it easier to cough out. These include hypertonic saline, DNase (Pulmozyme) and guafeneisin, which is an expectorant.

Milk, particularly whole milk, is actually a good choice of beverage for people with CF. It contains plenty of calories, fat, protein and vitamin D to help keep the body healthy. Milkshakes made with ice cream and whole milk are one way for people who are pancreatic insufficient to put on some much needed weight.

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Source: http://www.abc.net.au/health/talkinghealth/factbuster/stories/2007/11/28/2102703.htm

What is BiPAP?

BiPAP stands for Bi-level Positive Airway Pressure. It is a breathing apparatus that helps people get more air into their lungs. The airways are kept open using a BiPAP machine. BiPAP machines are adjusted to dual settings so that the pressure of the air coming in on inspiration can be different from the pressure of the air being exhaled. This is beneficial because it allows the patient to get more air in and out of the lungs without having to exert a lot of muscle energy.

A person’s forced vital capacity (FVC) as determined by a Pulmonary Function Test is largely what determines if a BiPAP machine is necessary. BiPAP is often used for people who have experienced pneumothorax (or collapsed lung) and as a result have loss of ability of the air sacs at the outermost portions of the lungs.

BiPAPs are usually used while a person is sleeping. It is used for conditions other than just cystic fibrosis. Sleep apnea sufferers for example, are often fitted with a BiPAP machine and face mask. Unlike supplemental oxygen, BiPAP masks are worn so that they completely enclose the mouth and nose. Most manufacturers of BiPAP face masks have different models available and all are designed to be as comfortable as possible.

Therapies that Use Positive Expiratory Pressure (PEP)

Cystic fibrosis patients have difficulty clearing the mucus from their lungs not only because the mucus itself is so sticky, but because the airways are inflamed or otherwise tightened. Inflammation may be from bronchiectasis, bacterial infection, allergies or pneumonia. Clearing the airways is much better accomplished when the bronchi are less inflamed and constricted.

Therapies involving Positive Expiratory Pressure (PEP) are designed to keep the airways opened when the patient breathes out. There are a few different PEP devices, each having their own limitations and benefits. The way that PEP therapy works is that it keeps the lungs inflated with air. Basic science tells us that two things cannot occupy the same space. In people with cystic fibrosis, the mucus occupies precious space in the airways, preventing them from taking a full, deep breath.

PEP devices are either stand-alone, or can be used with a nebulizer. All PEP devices work by having a one way valve that creates resistance when the patient breathes out against it. With PEP therapy, the pressure difference that occurs when a patient breathes out allows the lungs to fill with additional air, which in turn will push the mucus out as the lungs try to return to an equilibrant volume.

In simpler terms, PEP therapy functions very similarly to trying to get mustard out of a squeeze bottle. If you were to just squeeze the bottle without shaking it, you would only get a puff of air, and that little bit of watery mustard (which I call a “musquirt”.) When you shake the bottle, you move the amount of air in the bottle to the bottom, which will force the mustard out when you squeeze.

PEP therapy is very effective when combined with postural drainage. However, the postural drainage positions, especially the ones in which a patient must lie face down, may make breathing against a PEP device too difficult. Some PEP therapies combine vibratory pressure, which helps loosen the mucus and dislodge it from the linings of the airways. The Flutter ®, Acapella ® and the Quake ® use a combination of vibrations and PEP to promote airway clearance.

Below are some pictures of different types of PEP therapy:

1) PEP mask

2) Acapella

3) Flutter

4) Quake

5) PEP attachment with nebulizer

What You Need to Know About Pseudomonas Aeruginosa (PA)

Pseudomonas Aeruginosa, commonly referred to in the cystic fibrosis community as simply "PA," is one of the most prevalent and difficult pathogens to eradicate. As an opportunistic pathogen, this bacteria takes hold mostly in people who are in weak health, or are already fighting another type of infection. In people who have normal immune systems and are otherwise healthy, P. aeruginosa will not affect them. P. aeruginosa is often referred to as a "water bug" because of it's ability to survive and grow in moist environments. Whirlpools, jacuzzis, and public swimming pools are likely places for P. aeruginosa. to grow.

P. aeruginosa infections can occur anywhere in the body. Cystic fibrosis patients are particularly susceptible to acquiring P. aeruginosa infection in the lungs. Patients who are undergoing intravenous antibiotic therapy, are on a respirator, or have chronic bouts of pneumonia or bronchitis are at greater risk of contracting PA. Patients with cystic fibrosis with pseudomonas lung infections experience coughing, decreased appetite, weight loss, tiredness, wheezing, rapid breathing, fever, blue-tinted skin, and abdominal enlargement.

P. aeruginosa can produce a number of toxic proteins which not only cause extensive tissue damage, but also interfere with the human immune system's defense mechanisms. Part of what makes PA so strong is that it has very little requirements in order to live. It readily adheres itself to the body's cells. This increases the risk of the infection reaching multiple systems within the body as the infected cells are transported. Additionally, P. aeruginosa doesn't need much food to survive. Even in a body that is already severely depleted and immunocompromised, PA can live. It should also be noted that P. aeruginosa is a common problem in hospitals. Because of its ability to survive, proper contact precautions and cross-infection protocol should always be used by patients and caregivers.

Perhaps the biggest challenge in treating PA is that it is resistant to many antibiotics. Medications that doctors often use as their first defense in treating lung infections in CF, such as ciprofloxacin, have little or no effect on PA. There are two forms, mucoid and non-mucoid P. aeruginosa. In the mucoid form, the bacteria has an even stronger outer-coating, making it difficult for antibiotics to penetrate the cell to kill the infection. Furthermore, the mucoid form is able to survive and reproduce more readily than the non-mucoid form. The mucoid form is what leads to more chronic respiratory infections in cystic fibrosis,

Treating an infection may involve using inhalation therapy, oral antibiotics, intravenous antibiotics, or some combination thereof. Most often doctors will treat a PA infection by prescribing "cocktail" or combination of intravenous antibiotics in the hopes that the benefits of each antibiotic will combine to produce what's called a synergistic effect. Medications utilized for this purpose include ceftazidime (Ceftaz, Fortaz, Tazicef), ciprofloxacin (Cipro), imipenem (Primaxin), tobramycin (Nebcin), ticarcillin-clavulanate (Timentin), or piperacillin-tazobactam (Zosyn). A full course of antibiotics usually lasts from 4 to 6 weeks. Studies have demonstrated that a combination of respiratory therapy (such as inhaled Tobramycin or Ceftazadime) combined with intravenous therapy, works best to deliver the medication to where infected mucus is produced, and where it later resides in the airways.

Many cystic fibrosis patients who have not been infected by P. aeruginosa, are prescribed prophylactic (preventive) antibiotic therapy. Azithromycin for example, is one such medication that has been researched in accordance with the Cystic Fibrosis Foundation, and has demonstrated positive results in improving the lung function of patients infected by P. aeruginosa. Further studies are being done to determine how well Azithromycin will reduce the instances of infection caused by P. aeruginosa. Currently there are studies being carried out to develop a vaccine for PA.



Supporting links:

Cystic Fibrosis Foundation: Azithromycin

Inhibition of PA using Azithromycin and other macrolide Antibiotics

Todar's Online Textbook of Bacteriology, "Pseudomonas" (2004)

Heatlh A to Z: Pseudomonas Infections

Eradication of Pseudomonas Aeruginosa in Cystic Fibrosis

Microbe Magazine "PA Resists Antibiotics"

Acquisition of PA in Children with CF

Bronchodilators and Nebulized Medications

"Recurrent wheeze and breathlessness are common in people with cystic fibrosis, and bronchodilators are commonly prescribed," according to lead author Clare Halfhide, M.D., of the Royal Liverpool Children's Hospital in England. "Despite their wide-scale and often long-term use, there is limited objective evidence about their efficacy in cystic fibrosis."

Source: Center for Advancement in Health. Article "Little Evidence Behind Bronchodilator Therapy For Cystic Fibrosis"


The findings of that study were published in October, 2005. It is the most current information I can find on the topic. Dozens of health-related news media ran the article, so I'm confident that it's not just a hack study published by some entity with a hidden agenda.

I used to do CPT without a bronchodilator unless my asthma was acting up or I'm fighting infection. Now that I have a ThAIRapy Vest from Respirtech, I always take a puff of my Xopenex HFA inhaler before starting my airway clearance regimen. Xopenex is very similar to albuterol. It is a bronchodilator. Many patients find that Xopenex does not make them as jittery or anxious as albuterol does.

I also use bronchodilators both as a "rescue med" and as part of my preventive maintenance even if I don't do them at the same time as CPT. Bronchodilators are most effective when they are done first in the lineup of "usuals." Below is the proper order of med-neb treatments, as reported by another CF patient's clinic's recommendation:

1. Bronchodilator (albuterol or xopenex)

2. Mucolytics (pulmozyme THEN hypertonic saline)

3. Inhaled antibiotic (TOBI or colistin)

4. Long acting bronchodilator (serevent or foradil)

5. Inhaled steroid (flovent, advair, or pulmicort)

Here's why the order of operations is so important when managing CF.

The bronchodilator helps open up the airways. The more open the airways, the better the chances of inhaling the other medications deeply enough into the lungs where they can be most effective. Mucolytics are "mucus cutting" medications. They help break up the secretions so that they can be coughed up more easily. In CF patients, the mucus and the lungs are typically dry, which is why hypertonic saline is so useful. Hypertonic saline works by adding moisture to the lining of the lungs, resulting in a slippery surface conducive coughing out sputum. Following sputum clearance of the airways, the lungs are further able to take in the nebulized mist of the antibiotics. At this point there is a greater likelihood of the antibiotics taking hold in both small and large airways. The treatment concludes with inhaled steroids which help keep the airways open for an extended period of time (i.e. 8-12 hours when it is time for another treatment.)

The length of time to complete all these steps varies somewhat depending on the type of nebulizer/compressor system being used. Before I had a PARI eFlow to nebulize my tobramycin antibiotic, the process took approximately an hour. Also, when I was not feeling well, or was more congested or taking TOBI, it used to take me as long as 1-1/2 hours to complete the process. Now that I have an eFlow, the process is dramatically faster and takes 45 minutes or less.

Although the article I cited at the beginning of this post says that bronchodilators are shown to be ineffective treatment, bronchodilators used in tandem with the other treatments CF patients need are effective indeed. My opinion is that they are an important piece of the overall treatment. While they may be ineffective on their own, they should not be discounted altogether.

Types of Airway Clearance Devices

Cystic fibrosis patients have abnormally thick, stick mucus that is difficult to remove from their airways. In order to prevent infection a combination of medications and airway clearance techniques are used. In the past, the methods of airway clearance we cumbersome, time consuming, and, at times, downright uncomfortable for both patient and care giver. One such example is the "clapping" technique. Sometimes referred to as "beatments" or "thumps" by CF sufferers, the process involved having a caretaker cup his or her hands and physically beat the patient's back in a rhythmic manner. By clapping each section of the lung's lobes, the mucus was dislodged to a point that it could be coughed up and spit out.

Over the last decade many developments have been made in the world of airway clearance devices that make expelling the mucus much easier. Although some patients do still prefer the clapping methods to which they have grown accustomed, newcomers to the disease have many options for airway clearance.

Intrapulmonary Percussive Ventilator (IPV)

This device, available in some hospitals and often called the "Percussion Air" stimulates the airways by directing several bursts of air into the lungs at a predetermined rate. A pneumatic flow interuptor sends a pulsing flow of air at a rate of 100-300 blasts or "cycles" per minute. The device is attached to a special mouthpiece and nebulizer cup so that the patient can benefit from medication simultaneously with the percussion treatment. Due to the high frequencies at which the air pulses, some patients have difficultly tolerating such a method. However, great benefits exist for those who have good posture and have been properly instructed in the technique of using and IPV.

The Flutter(r) Mucous Clearance Device

The Flutter is a small, handheld device that creates positive expiratory pressure (PEP) in order to stimulate the lungs to vibrate or "flutter" enough to dislodge the sticky mucous. The device itself consists of a weighted metal ball resting in a cone that sits atop a diaphragm through which air can flow. When the patient exhales, the cone and ball move around creating the "fluttering" motion that results in PEP. This causes pressure in the airways, keeping them open longer than would happen just through simple tidal breathing. The cap on the end of the device can be twisted to different settings to make the pressure lesser or greater depending on the patient's ability.The range of benefit of the Flutter depends largely on the patient's ability to exhale against the device. Longer, sustained breaths and the use of a technique called "huff coughing" are the best ways to benefit from using the Flutter.

Acapella (tm)

Similar to the Flutter, the Acapella employs the use of positive expiratory pressure. This device is considerably larger than the Flutter, but has a greater range of resistance than can be "dialed in" on one end. One advantage of the Acapella is that the t-piece of a nebulizer can be attached to it, allowing the patient to inhale medication while also engaging in airway clearance. The only drawback is that many home nebulizer-compressor systems do not deliver a high flow rate through the nebulizer cup. This results in wasted medication and decreased benefit for the user. In the hospital setting, however, the air flow can be adjusted to a rate that is high enough to push the medication through the device and reach the mouthpiece, ultimately benefiting the patient. Most often, the Acapella is used independently of nebulizer treatment.

Vests

The most favored and beneficial airway clearance device used by CF patients is the Vest. Different models are available from different companies, and determining which one to use is left to personal preference. The vest works by using a method called high frequency chest compression. The Vest is inflatable, and is connected to a compressor by two air hoses. Once switched on, the vest fills with air, and the air begins to oscillate, thereby loosening the mucus. The frequency (measures in Hertz) and Pressure (psi) can be adjusted according to the needs of the individual. Some patients wear their Vests while exercising, or while inhaling aerosolized medication. The technique of "huff coughing" is also applied while a patient is undergoing a vest treatment. Huff coughing is a type of deep exhalation that enables the airways to stay open longer. It's similar to how a person would breathe if they wanted to see their breath in the cold air, or breathe fog onto a window. When breathing in this way during a vest treatment, it's easier for the patient to cough up the mucous that is being dislodged.

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Additional Resources:

American Thoracic Society List of Airway Clearance Devices (includes Insurance and Cost Information for Acquiring Them)

How To Huff Cough Effectively

Respirtech inCourage vest

The Flutter Valve (From Axcan Pharma)

Hill-Rom Vest

Acapella PEP Therapy

Percussionaire Company