Consultation Recommendations

So I got the final report on my visit to see Dr. Moss at Stanford. Here are the highlights:



dx2001 due to history and sweat test 56

1993 tests were 35-50

P67L E60X AND.....I didnt know this M470V polymorphism

M470V "which has been described in the literature as potentially contributing to CF phenotype in certain circumstances". Whatever that means, will have to look it up.



Current concerns: noted inflammation past two years and recurrent use steroids which seem to be diminishing in efficacy. Her major concern bas been the use of systemic corticosteroids to treat flares of apparent ABPA, as well as several allergic injection reactions.



Notes:

multiple allergens, immunotherapy and xolair in use.

Xolair effectiveness demonstrated by increase in symptoms 4 days after missing an injection.

Sleep test normal.

Bronchoscopy and lavage showed only achromabacter though PA cultured periodically too.

Positive pancreatitis dx in childhood. I DIDNT KNOW THIS EITHER---I always said I was sure that was what it was but they must have sent him something where it was positive!

gastroesophogeal reflux treated with prevacid



Then they went over a 'review of systems and physical exam'

this part strikes me as funny I had to read it a couple times...

Neurologic: Grossly intact

ha ha is that the most horrid way to describe intelligence ever? It talks about in other sections about me being a good historian, pleasant, alert, a chemist with an active life, blabbedy blah but too funny to describe the overall marker that way.



ok now the good part



Impressions and Recommendations:



History and tests available to me are consistent with ABPA, although she has not had aspergillus ilsoated from sputum or bronchial lavage. This is not a fact atht woudl by any means exclude the diagnosis of ABPA, nor woudl a positive cluture indicate ABPA w/o other elements that are present in her case, icnluding wheezing and rop in pulmonary funcation, as well as bronchiectasis, elevated IgE and speciif cIgE and IgG antibodies to aspergillus.



I recommend that she continue on her xolair therapy. With regard to monitoring her IgE, if the immunoCAP methodology is used, this has been showen to be non interfering in measurement of total IgE (RG Hamilton, JACI 2006;117:759-66). However, otehr methodologies could cause a problem. We will obtain total IgE today as well as aspergillus specific IgE and aspergillus precipitins to check her serological status at present.

Therapeutically, I recommend alternatives to current pattern of frequent steroid usage. I would recommend starting on a triazole antifungal, probably voriconazole is to be preferred because she is on prevacid for GERD and itraconazole is poorly absorbed unless the stomach is acidic. Therapeutic dose for voriconazole is 1-6mcg/mL steady state trough level. I also recommend that she increase her N-Acetyl cysteine from 600mg BID to 900mg TID (woohoo I'd been hinting about this to no avail since my doc always uses 600mg BID).

I did mention another option is amphotericin nebulization, but given number therapies and time constraint it would be burdensome.

We also obtained a sputum for fungal cultureas it is possible that aspergillus may be isolated. If a routine bacterial culture is sent, then overgrowth may prevent detection of aspergillus.