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Ireland (Historical Counties) => Ireland => Down => Topic started by: Dale on Wednesday 18 January 12 08:22 GMT (UK)
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Kia Ora
Greetings from New Zealand!
I have an Irish family from Co Down c1800-1865 of whom about 50% inherited a severe disease - in this case Huntingdons Disease (HD) which can afflict one either as a juvenile or perhaps middle age.
The gene which causes this disease was not isolated until 1993 meaning generations were affected without understanding the reasons.
I would love to hear from anyone who has discovered this among their forebears as it is a definite sign of relationships!
Death cert info may indicate St Vitus Dance or chorea, as a cause. Some may have been classified as imbeciles or idiots and placed in an Institution.
I have a possible female death between 1843 and 1865 and would love to know how this person may have been treated in Ireland during this time- institutionalised? If so where? Cared for at home? Would help have been available at the time? How would a death have been registered?
The family had connections (probably employment) at Crossnamuckley, Ballyhaft, Ballybuttle and Balllyblack and Balllycopeland as well as Newtownards between 1830 and 1843.
If anyone wishes to reply by PM I am happy to elaborate!
Regards
Dale
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Hello Dale
I note you say a d.c. may say COD as Chorea or St. Vitus' Dance but you feel it was really Huntingdon's.
These are not the same thing. I had chorea as a child but I definitely don't have Huntingdon's.
Just thought I should point this out in case people think they are the same.
Dawn M
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Chorea is an abnormal involuntary movement and can be associated with various neurological disorders. The Huntingdons chorea wasn't defined as such until the gene was discovered in 1993 . This means that early death certs (1837- 1993 ) often use a more general term.
It is only if you know the family history and see the correlation moving through specific branches that you can see the pattern of inheritance.
If a parent has the gene there is a 50/50 chance of any child they may have inheriting it.
In the particular Co Down family I am researching there were 11 children. Five developed HD - I have death certs and medical records . 2 died young from "ordinary' ailments. The other 4 lived long and healthy lives apart from such problems as inflicted by WW1.
Of the 5 who developed HD the pattern continues as the gene has been passed randomly to their children. Although there is not yet a cure, there is a test available, so folk can choose to perhaps adopt children rather than run the risk of inheritance.
Unfortunately medical definitions on Death Certs varies considerably depending on the knowledge available at the time!
Hope this clarifies the matter for Dawn and Others.
My long shot hope was that that anyone knowing that the HD gene runs in their family may make contact as it would provide a possible definite link back to Ireland to explore!
Dale
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Thanks Dale
I was just trying to clarify the matter for you! ;D ;D ;D ;D
Some genetic diseases are definitely linked to areas of the world.
for instance, sickle cell anaemia is usually only found in people of negro ancestry; coeliac disease is very prevalent in Ireland. etc. etc.
Cheers
Dawn M
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Kia Ora Dawn
Amazing isn't it? I figure my Irish farmers thought their problems stemmed from the soil and went to the other side of the world to escape! No such luck! :(
I do not know who might have been left behind and continue to wonder if there is anyone who might have a familial relationship through HD occurring in their family. :'(
Cheers from the South Pacific
Dale
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Hi Dale
My cousin's mother had Huntingdon's and 3 of my cousins have it. She was from Derbyshire and was one of 14 children and I think about 8 of them had it. I thought it developed in the UK with Viking and English genes.
Regards
Robyn
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I am researching a Derbyshire family affected by Huntington's disease and trying to establish which branch of the family was originally afflicted.
I have a death certificate from 1866, the deceased a 37 year old female with cause of death given as consumption. Is it possible, given gaps in medical knowledge in former years, that consumption covered a multitude of sins, as it were, in the case of the deceased dying of various illnesses and diseases whether hereditary or acquired.
Adie.
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Whilst it is possible that someone with an inherited disease could have died from tuberculosis (consumption) it is probably quite unlikely that a cause of death of 'consumption' could have been mistaken for a disease such as Huntington's.
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Just to complicate matters - at least some cases of HD are new and are not in that sense inherited. The cause is a repetition of a DNA sequence: if the repetition is too long, HD is triggered. I don't have my textbooks to hand (used to teach about this) but from memory the repeat sequences can get longer by mutation generation to generation until the disease is triggered.(The Lancet, Volume 369, Issue 9557, Pages 218 - 228, 20 January 2007) What's more, the repeat sequence can increase in length during life, altering the expected age of onset of symptoms. (Hum Mol Genet. 2009 August 15; 18(16): 3039–3047.)
While the gene itself wasn't identified and sequenced until relatively recently, the inheritance pattern has been well understood for many decades.
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Hi
In my Irish emigrant family I have identified 2 generations prior to emigration and another 5/6 since the 1860s.
Death certs can be misleading as although the deceased had been admitted to an Institution suffering from Chorea or St Vitus dance or similar terms the actual cause of death was given as pnemonia or phthisis or even suicide. Coroners Inquests have clarified matters somewhat!
I have medical records for admissions and patient records written over years of "treatment" which clearly describe the symptoms for HD although the actual gene wasn't identified until 1993. This meant that for some families there were beliefs that it was something "caught" from grass or animals or something similar. Unfortunately many myths pervaded and some families were instructed not to talk about it- to the extent that I currently have dealings with a 60 year plus woman who found out in her fifties that she had a brother who had been institutionallised.
If you want to understand more local HD societies have websites and there is quite a lot online (choose a reputable site!)
Briefly if one parent has HD any child has a 1 in 2 chance of inheritance. For example out of a family of 11, 5 girls and one boy inherited HD. The other 5 children did not inherit and neither did their children or grandchildren.
Hope this helps Adie. DID the 37 year old woman have children ? Were any of them affected?
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Dale, you will find that many death certificates will give pneumonia as the cause of death, particularly in the elderly.
This is because it is the ultimate cause of death.
If you were to see the doctor's actual certificate, you will find that it would say something like
cause of death: (a) pneumonia; (b) cerebral vascular accident
or (a) pneumonia, 3 days; (b) Huntingdon's disease 6 years
But often on the government issued death certificates they only put the ultimate cause, which is why you see so many for pneumonia.
Dawn M
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Thanks Dawn - after researching for decades and collecting hundreds of certs I am fairly familiar with both the certs, medical admissions papers and patient records. Any time I have a query I consult a senior geriatrician in the family- very handy these rellies sometimes! ::)
Deaths in an Institution in this country almost always include a Coroners Inquest which also helps as any contributory factors are covered.
Dale
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Well, it's handy to have someone 'in the business' isn't it?
I just gave you that info as you sounded as if you didn't know why so many have pneumonia as cause of death. Didn't mean to tread on your toes. Sorry.
Dawn M
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No probs Dawn I just wanted to make it clear that I understood the nuances, various resources available and hopefully provide others with some alternatives to understanding not only of HD but other diseases!
I certainly wish I had found that particular rellie some time ago as I foundered through a maze of half truths, family rumours, and downright ignorance!
Cheers ;D
Dale