It seems that a Danish sperm donation facility failed to determine that one of its donors had an uncommon genetic disorder and might have passed it along to several of his “offspring.” The usual screening apparently failed to uncover the genetic markers for the disorder and as many as five children might have “inherited” the abnormal genes.
The disease in question is called Neurofibromatosis Type 1, or Von Recklinghausen’s Disease. It is characterized by multiple types of neurological tumors and cafe-au-lait spots–patches of light brown pigmentation whose color is reminiscent of coffee with milk.
Severe Neurofibromatosis
Severe Neurofibromatosis
Cafe au lait spots (black arrows) and a Neurofibroma (white arrow)
Though controversial, and over the years other diagnoses have been entertained, this might be the disorder that afflicted Joseph Merrick, The Elephant Man.
Joseph Merrick, The Elephant Man
A medical nightmare for these children and their families.
The Crime Fiction Writer's Blog 
Diane Schultz
September 25, 2012 at 2:02 pm
Ah, but then you get into the spiritual discussion of souls and whether or not they would have transcended this union and found another body to inhabit. Terrible questions to have to ask.
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Patti Brooks
September 25, 2012 at 4:07 pm
What a possible nightmare for five parents!
My mysteries are set in the horse world and my WIP is set on a horse breeding farm where transported semen is used, That is what brought me to read and comment on this thread.
One of my characters in “GPS Illusions,” wishes to hurtle revenge on the owner of the farm. He substitutes semen from a horse on the opposite side of the spectrum from what is being bred on the farm and substituted it in shipments to dozens of clients across the country that have paid top collar for semen from the high profile stallion for their best mares. The mare owners are looking for high powered show horses that wave their shapely legs like the Rocketts. The semen they are unknowlingly receiving is from very docile, huge, big boned, slow moving draft horses. The resulting colts will find a place in the horse world, but not the high glamour horses needed to win in the show ring. My character works a deal with the doctor in charge of the DNA lab that registers foals to approve the colts’ DNA in spite of being sired by a stallion that the registry will not approve of.
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Elizabeth L. Semien
September 26, 2012 at 4:52 am
I wonder — Has the facility been held responsible? My godfather had NF1 and looked similar to the man pictured in the article. He would wear a long, dark cloak and only go out at night if he went out at all. Though we look at these pictures with interest, to see it in reality can be scary for someone caught off guard. The nodules are fleshy, not bone with NF1, hasn’t it been proven that John Merrick had Proteus Syndrome and not NF1?
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D.P. Lyle, MD
September 26, 2012 at 10:58 am
Many believe he did indeed have Proteus Syndrome—and that’s likely the case—but it remains controversial. Whatever he had it was a very severe form of it.
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Jill James
September 26, 2012 at 10:51 am
My husband has NF, but much less severe than the pictures in the post. We had a heavy-duty discussion before we married on whether we would have children or not. We decided to try once and we lucked out that our son has no signs of the genetic disorder, although we feel he might be a carrier and should think really hard about children too.
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Sandy Parks
September 26, 2012 at 3:07 pm
You certainly know how to give anyone who has used sperm donation nightmares. For this reason, I’m glad I never had to go this route. Another interesting post.
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