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Helen - Interview 2

Age at interview: 77
Brief Outline: Helen's late husband, Ivor was diagnosed with Factor XI Deficiency in 1995, which explained why he had always bruised and bled easily. In 2002, Ivor died from a stroke two days after a minor operation in which he agreed to try a new clotting agent.
Background: Helen is the widow of Ivor. Helen and Ivor had two children, Ruth (an IT consultant, aged 55) and Colin (an accountant, aged 51). Ethnic background/nationality: British/Jewish

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Helen’s husband, Ivor, was diagnosed with Factor XI Deficiency in 1995 having always bled and bruised easily since a very early age. Before he was diagnosed, he would take vitamin K pills if he needed a tooth extracted and this would clot the blood. The Factor XI was detected after a stay in hospital for two hernia operations and Helen describes how Ivor was pleased to be able to put a name to his excessive bleeding. People generally have a Factor XI reading of between 75 and 150 and Ivor’s was three.
 
In 2002, aged 77, Ivor went into hospital for a minor operation and agreed to try a new clotting agent. The operation was successful and he appeared to recover well. Two days later, however, he became very drowsy and that evening he had a stroke and died. The death certificate recorded his death as ‘stroke caused by Factor XI Deficiency’.
 
Ivor died on the Friday of the Golden Jubilee weekend which meant that the hospital would not be able to perform a post-mortem until the following Wednesday at the earliest. This was not acceptable to the family who wanted Ivor to be buried as soon as possible. The hospital staff were understanding of their wishes and once the death certificate was issued, the funeral was arranged for the Monday, in accordance with Jewish practices. The family felt that Ivor would have wanted this.

Both Ruth and Colin had their blood tested after their father’s diagnosis. Ruth’s reading is at the low side of normal while Colin has the condition but not at a problematic level. Both their medical records have the condition recorded in case there is a problem with future medical treatment, although the family have found that many medical professionals are not aware of the condition. They also discovered that other family members from their father’s side had the same condition. As both children have married non Jewish people, there was little risk of them passing on the condition.  

 

Colin and Ruth knew that their father bled easily but did not realise this was caused by a...

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Colin' Let me put a fraction of context into this. The normal level for Factor XI is between 75 and 100. Dad’s was 3. So it was really very low.
 
Helen' Colin’s the only other one …
 
Colin' I’m the next lowest, but I’m nowhere near as low as dad. I mean my reading is 45. And anecdotally when, obviously we discovered that dad had this problem, Ruth and I were both checked out. We discovered that …
 
Ruth' And our uncle was as well.
 
Helen' But what is interesting which we discovered, I think while he was still alive, but, other members of his family, that come from his father’s side, also suffered from this. One of his first cousin, and this first cousin’s daughter also had this condition. Again, it wasn’t diagnosed until after his was.
 
And so once he was diagnosed how it was that you were brought in to have your testing done?
 
Ruth' We were just advised to…
 
Colin' We went to see our GPs.
 
Ruth' Because, yes, because his count was so low. And, I think there was a strong feeling that it was hereditary. I think people knew that anyway. So we were advised to be tested.
 
Helen' And [brother’s name] my brother-in-law as well.
 
Ruth' Yes, so it was really the three closest.
 
Helen' It was the blood relatives, yes.
 
Ruth' Relatives. And because none of us had a particularly worrying count, although it was low, it wasn’t silly low. And it just stopped there. So none of [brother’s name] children were tested. And [relative’s name] wasn’t.
 
Helen' Haemophilia is passed down through the mother but it’s only males who get it.
Colin' Yes, that would be right. So it’s not even sort of consistent. Its one of these things that seems to jump, possibly mutate a little, I don’t know.
 
Did you know if your husband’s father or mother had the same problem?
 
Helen' Well it came from his father’s family.
 
Ruth' Yes, but did his father have the same problem?
 
Helen' I don’t know. Well I wouldn’t have thought so, because …
 
Ruth' Nobody ever mentioned it. Did they?
 
Helen' No, I mean as far as I know, I mean he did have operations over the years, and I never heard that he bled, you know, kind of unusually.
 
Ruth' But it wasn’t just the operations was it, because we knew that if dad cut himself.
 
Helen' Yes.
 
Ruth' That he would bleed. It wasn’t just surgery. It was …
 
Helen' No that’s true, yes.
 
Ruth' Anything.
 
Helen' I never heard that grandfather did so I’m assuming he didn’t. I don’t know.
 
Ruth' No I was never aware that he did.
 
Helen' No, no.
 
Colin' I don’t think we would have been though because these things were sort of brushed under the carpet.
 
Ruth' Well no, because I think we just kind of knew that dad did. It was just something that was there.
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Helen's husband, Ivor, was relieved to find out that the problems with his blood that he had had...

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Helen's husband, Ivor, was relieved to find out that the problems with his blood that he had had...

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So can you remember, what did he think when he was told, after all these years of having this blood that didn’t clot that there was a particular…?
 
Helen' I think he was happy that there was actually a name for it. I mean he knew, you know, he had a bleeding problem.
 
Colin' Yes.
 
Helen' But at least we knew what it was.
 
Colin' Yes, I think it was largely a case of, you know, I told you so. We knew there was something wrong.
 
Helen' Well…
 
Colin' And it was a case of identifying it, possibly coming up with, you know, a cause and I think to be fair, knowing Dad he quite enjoyed the celebrity for a little as well.
 

Ruth' Yes, he probably did [laughs]. 

 

Ruth and Colin learned from the doctors that their father's condition - Factor XI deficiency ' is...

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Helen' We did have a communication from them eventually, while dad was still alive giving the results of all this.
 
Ruth' Oh really.
 
Helen' Yes, and saying that not only did it affect Ashkenazi Jews, but also Japanese.
 
Colin' That’s peculiar, yes, well…
 
Helen' Yes, it’s a strange mixture.
 
Colin' Well it is, and it isn’t, there is a common link and that’s the assumption that a quirk of this type generally starts because of a lot of intermarriage amongst families. Ashkenazi Jews I am sure you would know, because you have obviously done a little research into this, like our family, the majority come from Eastern Europe and they were in sort of small villages and cousins married cousins a lot. The same I gather happened in rural Japanese communities, so it may not actually be as impossible a link as you think.
 
Helen' What was interesting when we went to the [hospital] and again I can’t remember the name of the, of the consultant we saw. A lady doctor. She was very, very nice, and she asked Dad why, this is only prevalent among Ashkenazi Jews, who are from Eastern Europe and not Sephardic Jews who tend to come from Israel and then this was the same thing that Dad was explaining to her about this …
 
Ruth' No, it was about intermarriage.
 
Helen' … sort of intermarriage.
 
Colin' Yes.
 
Ruth' The other thing that I don’t know whether it was at all relevant, is that having a blood group of B …
 
Helen' He was AB.
 
Colin' Dad was AB.
 
Ruth' He was AB.
 
Helen' Yes.
 
Ruth' But we’re both B. But the B side of it is prevalent among Ashkenazi Jews and Africans I believe.
 
Helen' Oh right.
 
Ruth' And very few other people are B.
 
Helen' [Laughs] Yes.
 
Ruth' So we can, kind of if you like prove that Dad had real Ashkenazi blood.
 
Helen' Yes, yes, yes.
 
What about the link with the Jewish community then? Well how did you experience that, sort of finding out that he had this condition and that it was linked to being an Ashkenazi Jew?
 
Ruth' We didn’t. The doctors must have told him, when he was diagnosed.
 
Colin' Yes. They also told me that at [hospital] when I got checked out that it was something that was… The only incidences they had ever come across were in Ashkenazi Jews, because they asked me, sort of obviously politely and discretely you know, is your background Ashkenazi Jewish? Which I’ve never, ever denied, so yes, it is known amongst the fraternity that know about Factor XI deficiency.
 
Ruth' I suppose that’s a big difference that you went to [hospital]…
 
Colin' Yes.
 
Ruth' …and there’s a fairly big Jewish community nearby.
 
Colin' Yes. In fact Factor XI deficiency is known in some circles as Jewish haemophilia. It does have many characteristics in common with haemophilia.
 
What did the post-mortem say?
 
Ruth' The authorities agreed not to ho
 

Since he was a child, Ivor had had several episodes of excessive bleeding during which he needed...

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Since he was a child, Ivor had had several episodes of excessive bleeding during which he needed...

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Well the first thing obviously that he remembered was when he was about five years old he had his tonsils out and I don’t know how long they used to keep them in hospital then, maybe a day or two. Anyway, after he came home he had a haemorrhage and had to go back in. So that was obviously the beginning of it but nobody knew what it was. You know.
 
He said, he told that over the years if he ever needed a tooth extracted, once they had discovered the first time that it bled, he used to take Vitamin K pills two or three days beforehand which certainly, you know, set up the clotting thing. 
 
Now he had a heart bypass in 1989 and had no blood problems. But we realised that because he had blood transfusions, there was sufficient clotting factor in these to stop excess bleeding, so he didn’t have that, but he was allergic to, it was plasma wasn’t it that they once or twice tried to give him and it used to give him the shakes. Do you remember? So they could only use a certain thing if they ever wanted to give him something to stop bleeding for any reason. Over the years they found out what it was that they had to give him.
 
He was under [Hospital], the haematology department there and after, he had a fall, and he fell down some steps outside and hit his face, his nose and his nose didn’t stop bleeding. We took him to the hospital and he was in for a few days. And what they did then, they gave him infusions, it went on for about four days, wasn’t it. Something like that of whatever it was that they give him that brought factor XI up to a reasonable amount. And the bleeding stopped.
 
Now what happened in the end was that he had two hernia operations, and each time he bled a lot afterwards. After the second operation, a student doctor who had been reading up on his notes said, “Every time it says on it, ‘excess bleeding’ but nobody’s ever investigated it further.” She said, “Would you stay in another day?” - because he was due to go home, you know - “and I’d just like to take, do some blood tests and things like that.” Which is what they did.
 
 

Helen was advised that it was all right for her to continue to donate blood after being diagnosed...

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Helen was advised that it was all right for her to continue to donate blood after being diagnosed...

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And our uncle as well yes. By that time I had been a blood donor for 30 years. So I knew that I didn’t have a particular problem with the level that I had. I did of course get in touch with the National Blood Service straight away because I was slightly more concerned that I might be giving people blood that was actually doing them, as much harm as good. I was reassured by the National Blood Service that this wasn’t the case. The doctor I spoke to had not actually come across the condition before, which gives an indication as to how uncommon it is. However, he told me that I would not be doing anybody any harm, because people would not be receiving my blood in sufficient quantities to dilute their natural Factor XI level. So they were happy for me to go on donating blood as long as it was not causing me any problems. It never has done and I continue to donate regularly. 

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