summary of survey re the use of Singulair for

[Guest guest]

Dear support group participants,

As you may recall, I recently conducted a survey of the membership

to find out how many children with had used Singulair and what

results they had gotten with it. The survey is about to be finished;

if you would like to participate and have not yet done so, please

click on " files " at the left of the web page, and click on the third

file on the list which is the Singulair survey.

Here is a summary of the results so far: 5 parents filled out the

survey, or gave me enough information to fill it out; 3 of these

children seem to have typical syndrome, and two

are " atypical " . 4 of the 5 children are boys. All 5 children had

been treated with prednisone, and 4 of the 5 had a good response to

this and are still using steroids at the onset of fever episodes. 4

of the 5 children eventually developed shorter cycles after using

the prednisone for 1 or more cycles.

All 5 children were prescribed a daily dose of Singulair; 4

of them were prescribed the usual recommended dosage for their age,

and one is taking twice the usual dose. None of the parents noticed

any side effects of the Singulair. 4 of the 5 are still taking

Singulair, and all 5 parents think the Singulair improved the

child's fevers to some extent. Two of the children had had one

fever shortly after starting Singulair, and then had not had another

fever as of the time they responded to the questionnaire: 56 days

in one case, 70 days in the other (they had been having fevers on

average every 21 days and every 12 days, respectively, before

starting the Singulair). One took the Singulair for only about 6

weeks; he had a fever 23 days after starting it, then went 78 days

without a fever, even though the Singulair had been stopped near the

beginning of this 78 day period. One child had a significantly

longer period of time between fevers (from an average of 12 days to

average of 120 days) and the maximum temperature went down from 104

to 101 degrees. The duration of fevers also decreased, from an

average of 16 days to an average of 8 days. The fifth child had a

less pronounced improvement on Singulair, with the time between

fevers increasing from 14 days to 18 days, and maximum temperature

decreasing from 105.8 to 102.2 degrees. Of the 3 children who

continued to have fevers on Singulair, one noticed less problems

with rashes after starting Singulair but the other two did not have

changes in their other symptoms associated with the fevers.

In addition to the 5 children described above, 3 other

parents e-mailed me with partial information about their children's

trial of Singulair. These were one girl and 2 boys; all 3 of these

children are still having fevers but have had a decrease in

frequency, maximum temperature and in 2 cases, shorter duration of

fever on Singulair.

Thus, from the results we have so far, we can conclude that

treatment with Singulair does not cause side effects in children

with and that some children seem to benefit greatly from this

treatment with significant reduction or even cessation of fever

episodes. Others, however, have only a small response with slightly

longer times between fevers, shorter episodes and somewhat lower

fevers. It appears, at least in the few children we have heard

from, that the effect of Singulair is seen within the first month or

two, so it seems reasonable to consider giving this medication a

trial for 2 or 3 months; if no benefit is seen within that time it

is probably not going to help. Particularly for children whose

fever cycles have become very frequent, requiring doses of

prednisone every 2 weeks or so, a trial of singulair would be a

reasonable next step, in hopes of reducing the risk of steroid side

effects.

As mentioned above, I am basing these statements on only a

few patients; if anyone reading this would like to contribute more

information about the use of Singulair for treatment of ,

please respond to me at michelle.lierl@....

Thank you for your help with this project.

Lierl, M.D., Cincinnati Children's Hospital Medical Center