Saturday, February 9, 2008

Life Expectancy in Shwachman-Diamond Syndrome

If you are looking for the words “Life expectancy” you will likely not find it in medical literature…BUT you can find many articles that list median survival and various other stats. Are you reporting for a statistics class or are you doing a project for another class? In Stats, median range is usually given---

In fact, the median survival is listed in literature as 35 yrs. See the article titled: Diagnosis, Genetics, and Management of Inherited Bone marrow failure syndromes which can be accessed from this link: Shwachman-Diamond Syndrome Medical Articles This article includes a section on SDS that says (page 36)

“…and many patients evolve to Aplastic anemia, MDS or Leukemia. The median age for the latter in the literature reports is 18 years, and the age dependant cumulative probability of leukemia is greater than 70%.”


There is a chart in this article on page 30 that lists the following for SDS:


Age at cancer, median (range)-----------------18 (2-43)

Cumulative probability (%) of cancer by age 40-50-----71

Projected median survival age*------------------------35

% alive or dead greater than or equal to 16 y of age----22