Living with it!

Allergies, blood and immune system
1

Hi all,

I have a bit of a long winded story, but would love any thoughts or inputs that anyone has…..

I am a 32 year old female. I was diagnosed last december with Hemochromotosis (C282Y) with a ferritin level of 1400. My mother is the same, as is my maternal uncle, and my father and sister are the lower level one (can't remember what thats termed as, but not just carrier). They have all had pretty standard treatment for it. I live in a country in Asia (my family are from England) which does not have any practising understanding of the disease as its so uncommon here, and I have found getting support hard - even though where I am is known for its great medical care.

I haven't faced much in the way of symptoms - I do get very flushed, I have had very painful and irregular menstruation, joint pain, lethagy and about 5 years ago developed a lot of trouble with the discs in my lower spine (which they have said may have been connected with the Hemo).

Aside from not having much awareness of the disease here, the biggest challenge has been how difficult it is getting any blood out of me! They tried about 10 times on 3 occasions and nothing worked……I did everything I was supposed to (drank a ton of water, did some light exercise etc just before) and nothing worked. And after seeing a number of different doctors who were top of their tree hematologists, they still could not work it out. I then saw a respected specialist who suggested a cathatar in my arm which I went for as it just seemed nothing else was working. And it did seem to help, I had 3 weeks of plebotomoies from the cathatar and my ferritin level was dropping (I do a weekly blood test as its so easy to do here) and would flush the cathatar with heparin twice a day. But in the 4th week, i developed a bad pain in my upper arm, and it transpired that I had an Upper Arm DVT :-( So they took the cathatar out very quickly and put me on blood thining injections (clexane). And we were back to square one, and with no phlebotomoies for 3 weeks while I worked out what to do next, my ferritin (which had dropped to 1209) had risen again to 1370.

I was then introduced, by chance in fact, to the Vice President of Vascular Surgery of the biggest and most respected hospital here. A nice guy, who is known to be conservative in his approach and not one to push surgery when its not required etc. He suggested going via the femeral veins in my thighs (next to my groin) and see how that worked. And after almost 4 months of doing it almost every saturday (wipes out my weekend for sure!), I am down from 1370 to 200. We alternate the side each week and although the blood is still thick its now thinner than it was, and the 600ml he takes each week comes out in about 2 minutes. Its done with all care taken, and they put in an anaesthetic injection first, then a larger needle goes in that pushes through a wire right down the vein - and then it pulls the blood out into a bag (once the 500ml bag is full they pull out about 100ml seperately). Sorry for the lack of medical terminology!

We did try a few weeks ago to get the blood out of my arms again, now that the blood is thinner, but it still did not work. Even getting the blood out for my weekly blood test takes a few tries.

What I am not sure about is the following:

- Will there be lasting damage to my femeral veins. And if so, will there be any reasons why that is a problem later in life?

- At what level do I stop doing this?

- And then once stopped how do I maintain it?

And, if anyone has any ideas about what I have gone through and their honest opinion on it, I would be open to hearing anything. As I do get little input here about the actual condition I am still a bit in the dark. My mother is wonderful and knows a lot about it all now, especially as she was one of the first in our family to be diagnosed and its gone on to be diagnosed in about 10 further family members (mostly on the maternal side). But being on the other side of the world from them and doctors who treat the condition reguarly, I am a bit lost for actual advice on living with this.

Thanks in advance to those who can help, and wish you all well!

:-)

2

Hi,

I am not sure about damage to your femoral veins but I have been venisected for 13 years now and the only problems I have come across is the scar tissue. It becomes harder to insert the needle and a new position has to be used. As far as your current Venisection goes once you are down to acceptable iron levels you are started on maintenance Venisections. Your iron levels will be brought down to about 15 to 20 and then venesected every 2 to 3 months, but this is up to your consultant.The maintenance venesections as you may know already is for the rest of your life. I will say that if you are filling 600mls in approx 2 mins be very aware that you may suffer from hypovelemic shock. Believe me when I say if that happens you become very ill as I found out todayas my blood pressure plummeted down to 77 over 44 which is very bad as I like you fill the bag in just a couple of mins.

Hope this helps a little.

3

Not that I want anyone to have issues when it comes to having their phlebotomies, but I'm glad to know that I'm not the only person.

My veins are deep, you can't see them at all when you look at my arm. I've had horrible experiences with getting my phlebotomies done in part because of this and because my veins are small and the needle is so hard. There is only one section of my right arm that you can draw blood from and that vein always rolls so unless the phlebotomist is good, it's always painful. To top it off, my blood comes out like mad so I need to have an IV at the same time or else I will pass out because my blood pressure drops far too quickly. I think the average to fill up a bag is 15 minutes but I'm generally done in about five or under.

I've also had it where they've tried to get my veins about 4 of 5 times which is very painful and causes me anxiety, and they were so fed up I told them just to take it out of my hand. So they attached an IV needle to a tube and literally just let me drip into a jug. It was very classy. I felt like I was in some strange vampire movie or something.

I did have something strange happen to me the second last time I got it done. I hadn't had it for about six months, and some blood came out but then for whatever reason it clotted and nothing more came. I don't know why, they couldn't figure it out as this has never been an issue for me, but that's just what happened. But I just went this past November and it was perfect. I had half of a bag of IV fluid, then they started the phlebotomy, only took a little wiggle on the inside to connect with the vein, and it was all good. No passing out, no nothing.

I feel for you two though, very much so. I thought that my issues were bad but at least with enough pain and struggle 99% of the time I'm getting that blood out of me.

Have neither of you heard of the pill form though? I'm not sure where you both live or what's offered but where I am, if you struggle with having the blood taken out via phlebotomy they can give you a pill that breaks down the iron. You should really ask about it. I know here it's only given when you have severe issues with the normal treatment.

4

*because the needle is so large* not hard. Sorry.

5

Gee lu, I hope everything easier for you now as you should be on a maintenance schedule of once every 3 months by now.

Hi Megan, glad to bump into you again. I thought I was bad enough trying to get blood out of me - they often have to try 4 times. It is more problematic when I have been left sitting in the chair for too long - my blood seems to stop flowing. It also seems to be very thick and black, and when it finally gets going, gets light red towards the end.

Apart from my ferritin iron which is kept lower than normal now, my TS% is always high, and my iron is always high. So I am having a search about thick, black blood.

I must pass on the idea about having an IV at the same time to an elderly lady who has a lot of problems too. As well as the groin position. Thank you both for that.

6

Ask for a port a cath. Much easier. I always have a full bag of fluids afterwards. Rehydrates and prevents low bp. Mine dropped to 66/50. Fluids popped it back up to normal. I was dx'd after my hystarectomy in 2003. My ferritin was 1750. I had phlebotomis 2x's a week for 6 weeks to start. Maintenance is where I am. My L5/S1 discs fused arthritis in my neck mid back. Liver & spleen damage which led to me being a sudden inset diabetic. I had normal sugars prior. I also have some memory issues. Not severe. Joint damage in my hands from "iron fist" . The Asian countries don't know. It's predominant of northern European countries also known as the "Celtic Curse". Hope some of my experience with this was helpful. Take care.