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Staphylococcus aureus

Question
Hello,
We got to know one month after the birth of our now 3-months-old daughter that she was suffering from CF. She carries the DF508 and G3272-26A mutations.
She is followed every month at the CF Center. She has every day respiratory physiotherapy and is until now pancreatic sufficient.
My question is, we just got to know that she already has Staphylococcus aureus, she was therefore treated wiht amoxicillin and clavulanic acid for 15 days + respiratory therapy sessions two times a day to help evacuate because she has a sort of bronchitis with green sputum ... Will she be colonized by Staphylococcus aureus all the life or is it possible that there is no trace any more at the next visit, is it possible to eradicate it? The pulmonologist was very evasive about it. I do not understand how this bacterium works.
Does this Staphylococcus make the disease develop faster (bronchi and lungs damaged)? Isn’t it too early to be colonized by this bacteria at 3 months ??
We're a little clueless ...
Thanking you,
Sincerely,
Answer
Hello,
F508del mutation and 3272-26A->G are each one "CF causing" mutation if associated with another CF causing mutation. This is the case of your daughter who carries two mutations which confirmed the diagnosis, suspected, I guess, on a positive screening test.
Of the nearly 1,900 known mutations of the CF gene (CFTR), the F508del mutation is the most common, found in 70% of patients. The 3272-26A->G mutation is much rarer. It is found associated with the F508del in 139 of the more than 25,000 registered patients in the largest database (CFTR2 database).
These 139 patients:
• have in average the same sweat test value than the other patients;
• are pancreatic insufficient in less than one in two (46%) against 9 out of 10 for all patients (suggesting that 3272-26A->G mutation is a bit functional). This is the case of your daughter who is currently pancreatic sufficient (it should be checked regularly, about every two years because pancreatic insufficiency can sometimes occur secondarily);
• and have a chronic respiratory infection with Pseudomonas aeruginosa in one of 3 (32%) compared to more than one in two (54%) for all patients.
The occurrence of lung infections is the main consequence of the disease. CF gene mutations result in a thicker and more viscous mucus which does not adequately protect the airways from microbes and other inhaled particles, accumulates in the lungs causing infections. This is the justification of long-term treatment, including chest physiotherapy, maintaining a good nutritional status, therapeutic education of the parents or the patient to identify the warning signs and regular monitoring for detect signs of an infection that have to be treated early to aim eradication of the germ and reduce the risk of relapse and irreversible respiratory injuries.
The Staphylococcus aureus, like other bacteria, secretes various substances which attack tissues and cause damage which can become irrreversible if infection is not treated early and effectively.
Staphylococcus aureus is the organism most frequently implicated in CF children and adolescents. It is found in 50 to 75% of bacteriological samples of those patients. His mere presence in the air and upper respiratory tract is not always a sign of infection (a large percentage of the general population are healthy carriers of the germ), but the fragility of respiratory patients with cystic fibrosis leads to be particularly vigilant and treat whenever there are even mild signs of infection (fatigue, decreased appetite, failure to gain weight ...).
In summary, Staphylococcus aureus infection is especially common in CF children and infants. It is important to detect the warning signs for early treatment and avoid the gradual onset of irreversible damages. This is the importance of monitoring by a team of a specialized center. Do not hesitate to ask questions, to ask about opportunities for patient education and talking to them, in confidence, your concerns.
Sincerely.
Gilles RAULT, MD, Roscoff CF Center
04.11.2014