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Paresis of the diaphragm

Question
Dear expert team,
for several months, I am suffering of an idiopathic paresis of the diaphragm. The conductance of the phrenical nerve is diminished and as a result the diaphragm shows a slight high stand on the right side.
I have already experience in CF for 38 years and I did loose 800 ml of lung volume due to the paresis (from 3.4 to 2.45 liters). Infections are seldom...since 1992 about 6 times i.v. antibiotic courses because of the finding of Alcaligines. I am not Pseudomonas positive, no diabetes, but a severe malnutrition with a BMI of 16, (177cm 51 kg), for 2 years I know about the diagnosed osteoporosis, t-score 2.7.
I have the question, if there are any case reports in the CF-register of a paresis of the diaphragm and if my mutation, my genotype dF508:405+1G->A probably is the cause of the problem...and above all: how can this paresis be treated?

Greetings from Switzerland
Answer
Hello,
I myself do not know any patients with CF and idiopathic paresis of the diaphragm, furthermore, I was not able to find any case in the scientific literature.
Functional deficits are called idiopathic in such cases, in whom no cause can be found. Often, the consequences of an accident or of an operation are suspected to be the cause of a paresis of the diaphragm; if that can be excluded as well as a tumor or changes at the cervical spine (osteoporosis?) or changes in the central nervous system, we talk about unexplained or "idiopathic". Just this year a spotaneous healing of an idiopathic paresis of the diaphragm has been reported, however not in a CF patient.
It appears from the information given above, that it is not possible to find any hints for a correlation between a certain genotype and the appearance of such a rare problem.

Concerning the therapeutic options in case of a paresis of the diagphragm:
the therapy is not trivial: often one can only try to treat the symtomps by
intensifying the physiotherapy to overcome the loss of lung volume.

Yours sincerely,
Prof. Dr. TOF Wagner
20.11.2008