Here is some stuff from Dr. Warwick from a couple of years ago:
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<br /><b>COUGHING</b>
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<br />Keep in mind that almost all CF patients have a super strong ability to suppress coughing. They have learned this at home, at school, at concerts, at movies, in crowds and at churches and other places. The balance between structured coughing, irritation induced coughing, and spontaneous coughing and the subconscious learned suppression of coughing is an area in need of more research. It is one area where patients and families with CF can do their own experimentation.
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<br />I have prescribed the following aerosol during each vest therapy for over 30 years with such good success that it continues to be my choice as the most effective aerosol for CF:
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<br />Mix and nebulize for aerosol treatment:
<br />n-acetylcysteine 20% 4 ml
<br />Albuterol unit dose (2 ml ampoule) 1 vial
<br />Intal unit dose aerosol (2 ml ampoule) 1 vial
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<br />This yields 8 ml of a 10% solution, which can be nebulized in less than 30 minutes with a good nebulizer. I recommend stopping the aerosol when the nebulizer begins to sputter. The mixture provides anti-inflammatory, bronchial dilation, mast cell stabilization and reduces sputum. The 20% Mucomyst is concentrated enough to irritate airways in some patients so that I prescribe diluting it to 10% with albuterol and Intal. The albuterol's main action is to assure dilated airways to improve the deep deposition of the Mucomyst. The Intal's main action is to counteract the high probability (about 40%) that allergies and mast cells and may interfere with treatment.
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<br />The CF mucus is dehydrated and becomes functionally normal by the addition of water. There is no molecular abnormality of CF mucus. Since CF mucus is normal dehydrated mucus any treatment that will add water to it will increase the ability of mucus to protect the airways.
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<br /><b>AN ALTERNATIVE WAY TO USE n-ACETYLCYSTEINE </b>
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<br />The benefits of n-acetylcysteine aerosols are sometimes offset by the odor of the aerosol, which resembles rotten eggs, the stickiness of the not-inhaled n-acetylcysteine aerosol that can interfere with function of some computers, television sets and other electronic equipment, and there is the risk of bacterial infection if the nebulizer is not carefully sterilized daily. Some of my patients have substituted three times a day of oral n-acetylcysteine as NAC capsules size 600 mg or more, two or more teaspoons of 10% solution or one teaspoon of 20% solution.
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<br />Some of these patients still benefit from the use of Intal and occasional bronchodilator inhalations by means of metered dose inhalers. Some of these patients do not use any aerosols except antibiotics.
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<br /><b>GLUTATHIONE AND HYPERTONIC SALINE AEROSOLS</b>
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<br />Glutathione is a combination of three amino acids: glutamate, cysteine, and glycine. Cysteine is the active amino acid in this molecule. NAC is a more stable form of cysteine because it has only an acetic acid group attached. NAC is more water-soluble and is the most cost effective way to increase glutathione levels in the body. N-acetylcysteine in solution is sourer than glutathione but NAC comes in capsules. The cysteine amino acid part of both molecules provides the antioxidant, antiinflammatory and mucolytic actions of both molecules.
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<br />Hypertonic saline increases the water in the periciliary fluid. It does this by attracting water to reduce the hypertonic saline to normal saline concentration. This extra water floats the mucus high enough that the cilia can beat and move the mucus. This activity lasts until the hypertonic saline is diluted to normal saline concentration. I do not know how long this takes to happen.
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<br />A balancing consideration is that our studies have showed that the regular twice a day use of HFCC with either trapezoid waveform machines will increase the water content of water in the sputum for at least 12 hours after the last regular twice a day treatment. While the published studies show the immediate effectiveness of hypertonic saline aerosols I prefer two trapezoid HFCC treatments since that appears to increase of water in the mucus with round the clock effectiveness. Based on one patient's improvement with hypertonic aerosol and the good reports in the literature I regard hypertonic saline aerosol as a therapy worthy of physician and patient experiments as an addition to, but not as a replacement for, n-acetylcysteine. My current approach to recommend only 3% saline may change.
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<br /><b>PULMOZYME AEROSOL </b>
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<br />The Minnesota CF Center aggressive aerosol treatment component of preventive airway clearance protocol largely excludes Pulmozyme. My observations have been that Pulmozyme benefits only 1 in 10 of my patients all of which use Mucomyst aerosols.
<br />· Milla's observations showed, when Pulmozyme was added to the treatment of a hundred such Minnesota patients, that these patients had a more rapid drop of FEV1 than their matched patients who did not use Pulmozyme (<a target=_blank class=ftalternatingbarlinklarge href="http://thorax.bmjjournals.com/cgi/reprint/53/12/101tm4).">http://thorax.bmjjournals.com/...reprint/53/12/101tm4).</a>
<br />· Suri, in The use of human deoxyribonuclease (rhDNase) in the management of cystic fibrosis. BioDrugs. 2005;19(3):135-44, reports that "the response to treatment is heterogeneous and only a proportion of PATIENTS with CF actually benefit from the treatment."
<br />· Rochat et al. suggests a mechanism for a rapid worsening when using Pulmozyme aerosols (<a target=_blank class=ftalternatingbarlinklarge href="http://erj.ersjournals.com/cgi/reprint/9/11/2200).">http://erj.ersjournals.com/cgi/reprint/9/11/2200).</a>
<br />· Cobos, et al, European Journal of Pediatrics, 159: 176-181, February 2000, found "benefits of DNase in daily practice are limited but apparently can be maintained in the medium term in some patients. ...the benefits are doubtful in around 50% of patients".
<br />· Barker, et al Pediatric Pulmonology 38: 70-74, May 2004 found "improvement of exercise performance with DNase is restricted to a subgroup of CF patients and may not be predicted or identified by spirometry and subject report alone".
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<br />If your Doctor prescribes Pulmozyme aerosol discuss these papers with her. To be fair, on the positive side, Pulmozyme has a record of improving the lung function for many patients when the Pulmozyme was compared to "normal saline" aerosols. I have not seen a comparison of Pulmozyme with an effective aerosol such acetylcysteine.
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<br />I believe that Pulmozyme may have an adverse effect for some patients seen as simultaneous rapid loss of lung function. I recall observing, during my early prescription of Pulmozyme, three patients who coincidently developed a rapid worsening of lung function when using Pulmozyme. The possibility that this rapid loss of pulmonary function might have been a possible adverse effect of Pulmozyme was not investigated. If such coincidence occurs, I would investigate the possible association so to be able to confirm or rule out a provoking association of Pulmozyme with such a coincidental worsening of a present infection. My observation of only infrequent benefit (@ 10%) and my concerns about the equally rare possibility of an unexplained rapid worsening of lung function in a patient using Pulmozyme have biased me to prescribe Pulmozyme only to the 10% of patients who have experimented and are convinced that Pulmozyme is helping..
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<br />On the positive side Pulmozyme is a wonderful enzyme designed to break the long DNA molecules into very small pieces. A solution of DNA is very, very viscous. A solution of the Pulmozyme broken fragments of DNA is very thin and runny. The first solution is very hard to move with a cough; the second solution is so watery that almost any force, even gravity, will move it.
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<br />Consider my picture of what might happen when a patient with CF, without Pulmozyme in his airways, when a coughing spasm occurs. The coughing spasm can empty enough of the air from the lungs so that some smaller airways are closed. Without Pulmozyme, the thick mucus will tend to adhere to the airway wall and the coughing may deform the cartilage and predispose to the development of bronchiectasis as the adjacent cartilage is injured. Such a result of a coughing spasm may be a common injury when a patient, not using Pulmozyme, has a coughing spasm.
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<br />Then consider might happen when a patient with CF coughs with Pulmozyme in his airways. Then when a coughing spasm occurs with the thin and runny sputum produced by Pulmozyme. Instead of damaging the bronchi, what happens may be what happens in a toothpaste tube that is squeezed in the middle so that toothpaste goes on the brush and another part goes deeper into the tube. A similar event might occur in the bronchi with some of the thin and runny liquid of DNA fragments and bacteria being forced deeper into the airway branches, spreading the bacteria and infection. Such an event may occur rarely and only in a patient taking Pulmozyme who has a coughing spasm. Such a rapid loss of lung function could be looked on as a new, unlucky and unexplainable event that has to be treated with more antibiotics.
