Patsy Custis, Larry McClarey and Sudep

The Father of our country, George Washington, was never blessed with biological children.  When he married his wife Martha, she was a widow, and she brought two children into their marriage:  John Parke Custis, who was four, and Martha Parke Custis, who was two, and who was called Patsy.  Washington raised the two children as his own.

At the age of eleven or twelve Patsy began to have epileptic seizures.  The Washingtons consulted numerous doctors and attempted endless cures, all to no avail.  Modern medicine was not yet even in its infancy, and anti-seizure medications were over a century in the future.  However, even then it was known that epilepsy was not usually a mortal disorder.  Patsy had frequent seizures but she came out of them each time with no discernible harm.

On June 19, 1773 Patsy was  at Mount Vernon talking to her brother’s fiancée, Eleanor Calvert.  Patsy went to her room to retrieve a letter from her brother who was away at college.    Eleanor suddenly heard a strange noise and found Patsy on the floor having a seizure.  Her parents were summoned and George Washington placed her on her bed.  Family letters describe Washington kneeling at Patsy’s bedside, tears streaming down his face, praying for her recovery.    After only two minutes, Patsy died.  She was buried the next day, George writing to his brother-in-law, that his “sweet, innocent girl had died”:    [Patsy] rose from dinner about four o’clock in better health and spirits than she had appeared to have been in for some time; soon after which she was seized with one of her usual fits and expired in it in less than two minutes without uttering a word, a groan, or scarce a sigh. This sudden and unexpected blow … has almost reduced my poor wife to the lowest ebb of misery.

This is one of the earliest accounts of Sudep (Sudden Unexpected Death in Epilepsy).  The mechanism by which Sudep causes death is still mysterious.  It strikes young people, those between 20 and 40 are at highest risk, who are otherwise in good health except for their seizures.  It often occurs at night, and is usually unwitnessed.  The victims are often found in a prone position on their beds, or near their beds.  It is rare, striking one out of 1,000-3,000 of epilepsy sufferers each year.  In order for a death to be considered to be Sudep there can be no other explanation for the death.  The mortality figures on Sudep are uncertain because death certificates often do not indicate Sudep as the cause of death.  It is estimated that some 45,000 Americans die from Sudep each year, which puts it ahead of vehicular accidents by 13,000 for the year 2011 as a cause of death.  Go here to learn more about Sudep.

I was ignorant of Sudep until May 19, 2013.  My son Larry was going to graduate from high school that week.  Due to his autism he was able to stay in special education until he turned 22 on September 5, 2013.  Thus, this was his last year and he was due to participate in commencement along with his younger sister on May 24.  His last day of school had been during the morning on May 17.  To mark the day I took my bride and Larry and his brother, who was home for the summer after finishing his junior year at the University of Illinois, off for a movie and supper in Bloomington.  We saw Iron Man 3.  Larry always enjoyed super hero movies, something he shared in common with his Dad.  The next day Larry and his brother got their hair cut and we spent the rest of the day at home.  Larry was in good health, as he almost always was.  Except for his occasional seizures, Larry throughout his life enjoyed very good health.  He was tall and lean and in good shape.  His seizures we thought were under control with his seizure medication, Larry having on average perhaps one or two seizures a year, always at night during hot weather.  After a seizure he would invariably fall into a deep sleep until morning.

The evening of May 18 was completely typical until after I went to bed.  I gave Larry his anti-seizure medication just before I and his mother turned in.  I was told later by his brother that he came up to the room that he shared with his brother at around 11:30 PM, about an hour after I and my wife had gone to sleep, and Larry was having a seizure. It appeared to be a normal seizure and his brother stayed with him until it passed and Larry fell into a  deep sleep.  Then his brother turned in.

At some point Larry awoke and went down stairs to fix a snack of skittles and milk.  I found both out when I went down stairs in the morning.  (Try as I might, Larry would usually forget to put the milk back in the refrigerator.)  Larry then went back to bed.

Around 2:15 AM I awoke to the sound of pounding.  We had a tower floor fan on in our room, which we purchased just the day before, which muffled the sound.  I was not fully awake and at first I thought the sound was someone pounding on the door to the house and the thought occurred to me that perhaps Larry had gone for a midnight stroll and that a neighbor had brought him back.  Then I thought perhaps it was thunder.  It never occurred to me that the sound was Larry having a seizure.  After the sound ceased I fell back asleep almost immediately, and till the day I shut my eyes in death I will bitterly regret that I did not get up to investigate.

My wife and I got up around 4:00 AM, our usual time for rising.  After breakfast I went up stairs at around 6:15 AM to do Larry’s “Daddy reading” with him.  This was a morning ritual that Larry and I had been following since he was a small boy, where Larry would read a page or two from a book to me.  His brother had already risen and was in the bathroom.  Larry was lying on his bed with a blanket pulled over him, his normal manner of sleeping.  I sat down on the floor and said, “Good morning Lad!”  He didn’t move.  That didn’t alarm me as sometimes I had to awaken him.  I pulled the blanket away and saw Larry was face down on his bed.  I realized instantly that he was dead.  I pulled him over and felt that rigor mortis had set in.  We called 911 and sent his brother to fetch our parish priest who came and gave my son the Last Rites.  I was thankful that I was emotionally numb during this time period and was desperately hoping this was a nightmare from which I would awaken.  It was Pentecost Sunday.  My mother had died on Easter Sunday, 1984 and my Father-in-law died on Palm Sunday 1997.  Larry had difficulty speaking due to his autism and the tongues of fire of Pentecost suggested to me that this was ordained by God, along with the fact that Larry had completed his schooling and that God had work for him to do.  I had often told my kids the story of the Questing Beast from the tales of King Arthur and how that quest began on Pentecost.  When they were younger as we were driving to Mass on Pentecost I would offer them a dollar if they spotted the Questing Beast.  The thought occurred to me that Larry had now gone after the Questing Beast alone.  Such musings kept at bay the intense grief that I needed to hold back as I and my family made arrangements for Larry’s funeral mass and burial.

An autopsy was performed on Larry and revealed no physical problems that led to his death.  His heart was in excellent shape as were his brain, lungs and all other vital organs.  His death was a classic case of Sudep.

Because death from Sudep is rare among sufferers from epilepsy, a debate exists among doctors as to whether it should be discussed with patients and their families.  I say yes.  Even if I had known about Sudep it is entirely possible we would have done nothing differently with Larry, the risk of death being so slight.  On the other hand it is possible that I would have purchased a seizure alarm.  In cases of Sudep attempts to save the lives of people going through it have been unsuccessful in the few cases observed, but I would give everything I possess if I could somehow go back in time and have gone to Larry’s aid when I heard the pounding that dark night.

At any rate I hope that anyone who has epilepsy, or who has a loved one who has it, will become informed about Sudep.  I wish with all of my heart that I didn’t acquire my knowledge until it was too late.