Dominantly Inherited Alzheimer's

It was Soren Kierkegaard who observed, “Life can only be understood backwards, but it must be lived forwards.”  I was only slightly aware of Kristie’s grandmother passing away with dementia when we were dating.  And I remember her father would joke about “the Wheeler Curse” when someone in his family acted strange or did something odd.

Little by little, I learned after we were married how not only Kristie’s grandmother Esther, but also her great grandmother Louisa (whose surname was Wheeler) and her 2nd-great grandfather George Wheeler, and his father Levi Wheeler, all died with severe dementia.  In a terrible irony, I recall Kristie telling me how she would go with her college roommates to explore the old, abandoned Utah territorial insane asylum.  Little did she know, as I was able to learn much later, that Esther, George, and Levi all spent time there; and it was where George and Levi, Kristie's 2nd and 3rd grandfathers, died. 

While attending the 4^th annual Dominantly Inherited Alzheimer’s Network Conference in 2018, I learned there were approximately 640 families in the world at that time who carried hereditary genes causing early-onset Alzheimer’s.  Dominantly Inherited Alzheimer's is very rare, making up less than 1% of all cases of Alzheimer's.  In affected families, the odds of a child getting a mutant gene leading to early dementia before age 60, were 50/50, depending on whether they got that particular gene from their parent who had the mutant gene, or their other parent who didn’t.  Kristie’s father had the gene and her mother did not.  They had seven children – three of whom inherited their father’s PSEN1 gene mutation and were all diagnosed with early-onset Alzheimer’s while in their 50’s.

While caring for my wife during her final years, I researched her family history and learned the following:

·   Levi Wheeler was born in the state of Maine in 1812, where he learned the lumber business from his father Simon Wheeler.  He moved to Illinois where his wife died, leaving him with 5 children; who he left with relatives while he went to the gold fields in California where he became rich supplying the miners with lumber.  In 1854 he sailed around South America and up the Mississippi River, writing “I had enough money to pay for the care of my children and buy a most up-to-date saw mill that was run by steam.”  He brought his family and sawmill (supposedly the first steam powered mill west of the Mississippi) to Utah, where he was very successful.  According to his obituary in 1886, Levi died “at the Territorial Insane Asylum, where it was found necessary last summer to place him in consequence of him becoming demented.”

·   George Wheeler was born in the state of Maine to Levi Wheeler above in 1844, and came to Utah in a wagon train with his father in 1854.  He worked with his father, eventually taking over and running the steam powered saw mill.  His death was listed as “general deterioration and exhaustion from senile dementia” in 1912 at the Utah State Mental Hospital where his father had died.

·   Louisa Wheeler Stephenson was born in 1874 to George Wheeler.  She would marry shortly before turning 18 years of age and had 10 children.  Her great-granddaughter, Irene Jeppsen wrote, “As Louisa grew older, she was afflicted with the same malady that affected her father and grandfather Wheeler.  Not knowing what to call it, the family referred to it as the Wheeler Curse.  Several of her children were also afflicted with the dreaded disease.” She died at age 70 from pneumonia.

·   Esther Luella Stephenson Neilson was born in 1895, the daughter of Louisa Wheeler Stephenson.  She married at age 19 and had five children.  Her granddaughter, Irene Jeppsen wrote, “Esther was in her mid-50s when her health showed the deterioration of the dreaded Curse. The next few years she became more confused, forgot who people were, and how to do the simplest tasks. She couldn't remember where she put things and lost her false teeth and other items. For a time, someone was hired to come to their home so she was never left alone. She also spent time hospitalized in Provo as her grandfather had done.”  Esther was placed in a nursing home at age 62 and resided there the last six and a half years, most of that time as a chronic invalid.

·   George Edgar Neilson was born in 1924, the fourth of Esther Luella Stephenson Neilson’s five children - of which three would be diagnosed and pass away with dementia.  He married at age 25 and had seven children – three of who inherited his PSEN1 gene mutation, resulting in early-onset Alzheimer’s.  George was diagnosed with Alzheimer’s in his late 50’s, requiring him to sell his dental practice.  He lived with his daughter Kristie for 3 years, the last year while he was bedfast, passing away from pneumonia.

·   Kristie Neilson Goates (my wife) was born in 1950, the first of George Edgar Neilson’s seven children.  She was diagnosed with Alzheimer’s at 56 years of age through cognitive testing after showing signs of dementia 2 or 3 years earlier.  She participated in Alzheimer’s research at the Oregon Health Science University where she was genetically tested and found to have the inherited (Wheeler Curse) PSEN1 gene mutation.  She passed away at age 69 after spending seven and a half years in memory care.

I don’t know if you would call it an advantage, but I became very familiar with the issues and problems related to Alzheimer’s after Kristie’s parents moved in with us, and we helped care for her father during his final 3-year decline, ending with his demise.  It was sorted of like a forced apprenticeship to experience and learn first-hand the complications and travail related to this family malady.

Your comments or willingness to share experiences below related to early-onset Alzheimer’s would be helpful and appreciated.

Resource Links

• What is Dominantly Inherited Alzheimer's Disease (DIAD)? - Dominantly Inherited Alzheimer's Disease (DIAD) is a rare form of Alzheimer's that causes memory loss and dementia in individuals - typically while they are in their 30s to 50s.  The disease affects less than 1% of the total population of people with Alzheimer's. 

• The Dominantly Inherited Alzheimer Network - An international research effort focused on dominantly inherited Alzheimer's disease, which is led by Washington University School of Medicine in St. Louis. 

• DIAD Family Conferences and Webinars - Sponsored by the Dominantly Inherited Alzheimer Network Trials Unit and the Alzheimer’s Association, with grant support from the National Institutes on Health/National Institute on Aging. 

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