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Pseudomonas
- Question
- My 9 year old daughter has a lot of lung problems. She has been extensively worked up for CF (Nasal PD, rectum biopsy, genetic examination, sweat test, etc.). The conclusion was that she doesn’t suffer from CF. None of the tests done were abnormal, but it was concluded that she is a CF carrier. Despite many examinations it is unclear what is really wrong with her. At present she seems to be infected with Pseudomonas. Everywhere I read that this is very specific for cystic fibrosis. Should the diagnosis of CF be considered again?
- Answer
- Dear parent,
We understand your concern about your 9 year old daughter. She carries one CF mutation and she is infected with Pseudomonas. Other than that there is no evidence that she suffers from CF. As you can understand without examining your child it is impossible to give specific advice about your daughter. We can only say that patients need to fulfill certain criteria to be suffering from cystic fibrosis. If a person has clinical symptoms suggestive of cystic fibrosis, they need to have either a positive sweat test, or found to carry 2 CF mutations, one on each CFTR gene or have an abnormal nasal potential difference measurement or abnormal intestine current measurement.
A person infected with Pseudomonas has a clinical sign that corresponds with CF. This is however not completely specific for CF. It has a high specificity but certainly not 100%. There are other diseases such as immotile cilia syndrome (primary ciliary dyskinesia) whereby patients have chronic lung infection and can be infected with Pseudomonas. Recently the term CFTR related disorder has been introduced to determine patients who have a CF like disease course but who do not fulfill all diagnostic criteria for CF.
Equally important is knowing what the correct diagnosis of a person infected with Pseudomonas is the fact that this person should receive the correct treatment so that no further lung damage can occur. In any case we advise you to discuss the specific case of your daughter with your doctor.
Kind regards
K. De Boeck - 02.12.2010
