Hereditary Diseases..

[Dale]

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
 

[Billyblue]

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

[Dale]

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

[Billyblue]

Thanks Dale
I was just trying to clarify the matter for you!         

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

[Dale]

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

[romak]

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

[adief/CGH]

 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.

[aghadowey]

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.

[GrahamSimons]

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.