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Pseudomonas

Question
Hello,
My two nephews the eldest 4y and the youngest 11m are suffering from CF. They carry the F508del / 3272 26 AG. The youngest has been screened at birth, while the eldest only now (false negative at birth). The eldest had no care for 4 years. Lungs appear more damaged thereby. He just had a Staph and was treated for it. Now he has a Pseudomonas germ that, from what I read, is bad. He began a course of aerosolized antibiotics for 28 days. Is it normal to get this germ at his Age? Are antibiotics effective? Can he contaminate his little brother? Thank you for all the information you could give us. I must say that the CF Center doctor doesn’t provide a lot of information about their condition. We do not know if it is for sparing the parents, but it’s not easy to know about their heath condition.
Answer
Hello,
Your two nephews have therefore inherited the same mutations of the cystic fibrosis gene, the CFTR gene: F508del mutation, the most frequent (it is present in more than half of the patients) and 3272-26GA mutation that is much rarer (is only found in 186 of the nearly 40,000 patients registered in the US CFTR2 database).
The mucus that covers the various mucous membranes of the body, including the respiratory mucosa, normally acts as a protective lubricant. Its thick, viscous character in cystic fibrosis is responsible for various symptoms, especially sinus and bronchial congestion that promotes the development of germs and infections. These infections (also called "respiratory exacerbations") can cause irreversible damage if they are not detected and treated early with adapted antibiotics.
The involved germs are varied. The most frequent in the first years of life are Haemophilus influenza and Staphylococcus aureus. The "Pyo" (scientific name "Pseudomonas") is becoming more common with time, but may appear in the first years of life as with your oldest nephew.
Cystic fibrosis patients may be contaminated by the natural environment or by another infected patient either directly or via a caregiver.
It is therefore important:
• to respect the hygiene rules to prevent transmission of germs:
o in hospital, given a greater risk of transmission due to care or contact between patients, particularly strict rules are established;
o in everyday life and in the case of your two nephews, should be particularly vigilant on the application of general rules of hygiene: frequent hand washing (especially before and after eating, after toilet use ), use of towels and a personal teeth brush.
• to provide parents (and your two nephews when they are older) to participate in therapeutic education sessions for recognizing signs of respiratory exacerbation (increased cough, sputum changes in older adults, decreased appetite, fever, fatigue ...)
• to perform respiratory secretions samples at each CF Center visits and come before and in case of respiratory exacerbation suggestive symptoms;
• to set up a treatment early including appropriate antibiotics:
o any respiratory exacerbation, whatever the germ;
o the mere presence of Pseudomonas in bronchial secretions, even in the absence of other symptoms, then check its disappearance after treatment: the aim is to prevent this organism to settle in the lungs because of chronic infection that is usually associated with a more rapid decline in lung function.
In summary:
• Yes, Pseudomonas colonization and infection of the lungs can occur in young CF children;
• Yes, there are effective antibiotics on Pseudomonas oral, inhaled or intravenous depending on the clinical situation and the resistance of the germ;
• Yes, Pseudomonas lung infection usually results in a more rapid decline of respiratory function when chronic; hence the importance of early and effectively treat the mere presence of this organism in the bronchi to prevent chronic infection;
• Yes, there is a risk of transmission of Pseudomonas from one nephew to another if the germ did not disappear after antibiotic treatment; however, respect in the daily life of simple hygiene rules can significantly reduce this risk.
Sincerely,
Gilles RAULT, Roscoff CF Center
03.03.2015