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FA Death: When and How Return to Index
Most famously quoted by Benjamin Franklin in 1789, " this world nothing can be said to be certain, except death and taxes.", this certainty of death while true for all does have a certain extra ominousness for those with the life-shortening disorder Friedreich’s Ataxia (FA). The question of FA death, the manner and timing, has come to the forefront a number of times both in FAPG and other forums so this parent-based paper will speak to the question.

Before proceeding it needs to be stated that
  • This paper is not the result of a formalized study, has not been exhaustively researched with hospital records or autopsies, and does not have statistics like standard deviations, ranges, confidence levels, etc.
  • The data is a compilation of inputs from family members of passed FA'ers with their best information about their relative's death.
  • Any forward looking use of the data to forecast the timing or manner of the death of any FA'er would be very ill-advised, especially
    • Because the earliest data point is pre-2000 and in the last 20 years clinical medicine has improved a lot.
    • And in light of the soon-to-come approved treatments for FA. The Past in this case will not define the Future.
  • At most the parent-based data confirms several of the many historical ways a FA'er may pass from this earth. If this is helpful the objective of the paper will have been fulfilled.
  • These records of death have been purposefully anonymized. FAPG and Facebook forums are world-wide support groups and this data reflects that. Please respect the privacy of FA families and do not fruitlessly try to identify specific passed FA'ers. Thank you.
So let’s first give the short answer: no one knows when nor how anyone will die, unless it is scheduled beforehand (not covered in detail in this paper). One's life can end at any moment, and in innumerable ways independent of FA, and indeed FA'ers have been killed in some of those ways, automobile accidents being just one.

Some formal research HAS been done on FA timing and cause of death. In 2011 this article, based on 61 deaths, was published as a result of a FARA sponsorship and the leadership of Dr. David Lynch. Notice it has standard deviations, ranges, etc.

So now for the longer and parent-based incomplete answer. Here are two graphs based on 85 deaths, one age-to-cause and the other cause-to-age, giving the reader two different perspectives/vantage-points on this question with no real answer. FA'er sex (M/F) is roughly 50/50 so no listing is offered. Click on either graph to open a larger view in a new window. Note MOSF is "Multiple Organ System Failure."

Note the effort to group similar causes of death together. You may not agree fully with the groupings and that's OK. The ranked groups do show what many in the FA community know and FA publications share:
  • The FA heart is most susceptible to failure. 48% in this data group and 59% in Dr. Lynch’s study. This author would lean toward the 59% because of the increased rigor of detailed medical review.
  • Weak lungs, aspiration, and low stamina lead to pulmonary problems.
  • UTI’s, bladder, and other infections can take hold quickly.
  • FA’ers are not immune to other life hazards and maladies.
  • Some advanced FA’ers have "opted out", choosing to not suffer further.
  • Some of the rest of the groupings taken together signal the importance of the FA’er and family staying alert to changes in health, being FA-knowledgeable and using that knowledge to better advocate with medical personnel on proper FA care.
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