Study Finds Adults with Rare XML Immunity Disorder Leading Productive Lives


By Staff
(AXcess News) Memphis, TN - A recent study by St.

JudeChildren's
Research Hospital found that adults with a rare genetic
immune-system disorder that prevents them from making antibodies
nevertheless appear to be moderately healthy and lead productive
lives. A report on this study appears in the current online edition
of Clinical Immunology.
The study of 41 adults with X-linked agammaglobulinemia (XLA)
showed that they can function as relatively healthy, productive
individuals, even though they remain
vulnerable to chronic, low-grade
infections. These individuals had a mean age of 4 years (range 1
month to 53 years) when their diseases were diagnosed; and 27 of
the patients had family histories of XLA.

The study was based on
results of a questionnaire filled out by each participant
concerning current and past medical problems and quality of
life.
"Until we did this study, there was almost nothing in the
medical literature about adults with XLA," said Mary Ellen Conley,
M.D., a member of the
Department of Immunology at St.


Jude and senior author of the report. "In fact, old reports we
read stated that the vast majority of these patients have
chronic lung disease by age 15."
XLA is a rare disease that is inherited through a mutation in
the Btk gene on the X-chromosome-one of the two types of
sex chromosomes. Treatment
includes aggressive use of antibiotics and replacement of the
missing antibodies with gamma globulin.


"Almost all of the adults with XLA had chronic medical problems;
however, these problems did not interfere with normal daily
activities, and the quality of life in this group was equivalent to
that of the general male population of the United States," said
Vanessa Howard, R.N., M.S.

N., a nurse practitioner for the
Immunology service at St. Jude and first author of the paper. "Our
study is reassuring and helps to put into perspective the ability
of such patients to thrive with proper care, despite this
potentially devastating disease.

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