hello there ... new and a bit shy

Hello there,
i have just joined this website today , so im a little unsure of how it works of how i can get in contact with people who have my condition ( i don't like to use the word "disease" personally)

I am 22 years old , living in beautiful Australia , and have CDA ( Congenital Dyserythropoietic Anemia ).
I was diagnosed when i was born in 1986, when i was clearly not well... "normal" again i dont like to use this language. i was ... not "well" lets just say.
I have been transfusion dependent from the first day i was born.
I had a bone marrow transplant  when i was 5 , but it didn't work I
ts hard  for me to remember all my operation times, dates etc , as my memory is not what it used to be.

In or around 2000 i  had my gall bladder removed.
In 2002 i had my appendice removed.
In 2004 i had my first myocardial infarction (heart attack) , then was in a coma for 4 weeks or so.
My hematologists and cardiologists put me on the Heart transplant list.
I have extensive iron overload , most of my internal organs  ( kidneys , liver ) failed.
I was in rehab for 6 months of so
I went from a healthy  size 10 weighing around 57kg to an emaciated 35 kg ( i am 5'5)
My ferretin level was extremely high, a level of  4000!! (normal range in around 100 )
I was on Desferal 24/7 durring my coma which brought my ferretin level down to 1000.* however , this dose not correspond to how much iron i had/have in my organs.
I learned to walk again, stand again, go to the toilet and all that jazz

I have been on Desferal  since i was a baby and just recently came on to Exjade ( oral form) , which has saved me from being on cortisone  to help with the awful heamatomers and edemas the Desferal left me with, all over my body.

My Doctors at the Alfred Hospital in Melbourne have been coresponding with a Professor in Germany , discussing weather or not i should now have my splean out .
They say it might hopefully bring my Hb up a point (10) higher.
My current Hb sits around 6-7 on a good month.
I now have One to two blood bags every 3 weeks over 4 hours each due to my cardiac history.

I was wondering if anyone could please give me some information , or better yet tell me about your experience having had a splenectomy. And offer your thoughts on wether i should go down this path.
I am currently thinking well, if it only brings my Hb up too 8 why bother!! , however it might eleviate the regularity of having to have a blood transfusion so often, and thus decrease my ferretin level and chance of iron overload again.
But what of the risks ? Like if i want to travel , can I  go to asia? places like cambodia ? what of all the defense the spleen undertakes, will i get sick more often

Thankyou for taking the time in reading a bit about me
I would love it very much for anyone to give me some constructive feedback on my question
I am happy to answer any questions if there are any parent out there , with a child with CDA as i know it is very scary and very lonely if you have no one to talk to about your son/daughters condition.

I look foward to hearing from you all
xo

I am so sorry to hear what you have gone through.
You certainly are a survivor
I came over to Melbourne in 2005 and saw professor Bowden who is based at Monash Medical Centre. It was the first time I had met Thalassaemics face to face. He was able to tell me so much about Thalassaemia and also to give me new ideas to try with the clinical mangement.
I'm just wondering if you could maybe contact him and ask to meet his patients. There might be someone gong through what you are going through.
I had my spleen taken out when I was seven so I can't remember much of the operation and such but I can tell you that I have managed to stay pretty healthy and not get too sick.
As long as I looked after my body with food and enough sleep I managed to stay out of hospital.
If you let your body get run down, you have more of a chance of getting sick. I also have the flu injection that also helps. After my operation I managed to go without the transfusions for 4 years.
Each of us are different so I would suggest you talk more with your haemotologist before making any decision.
We are all hear to listen and help you if we can
Cherieann

Hello Cherleann,
thankyou for your post , i was so happy to get one it made my day  
And thankyou , i think i have done pretty good too

I have heard of him i think , i have a doctor called steven Opat who works there too!!
i was supposed to go there last year i think to meet all the Thai patients , but apparently they are all males and i would be the only female there , and i was thinking i would be a little .. sky and out numbered i guess, is the best way to put it.
My friend Joseph , he has blackfan diamonds and he goes there every three weeks , did you meet him there?

thankyou for your information with the spleen. WOW 4 years !!! that is AMAZING i would love to go that long , i would love to travel for more than 3 weeks at a time you know!

i read a very intersting website about the risks of a splenectomy , something about Hep C , do you know anything about that?
may be interested to read it , its concerning CDA but i think the spleen info is quite beneficial ...

http://www.nature.com/bmt/journal/v27/n2/full/1702764a.html


Do you live in NZ ? i have family there, my mum comes from Levin , and i have aunties and a Nan in Wellington !!


Thankyou for your helpful insight, my heamatologist is very keen , but he is 50/50 wether it will do anything and for me thats to risky ,
he knows of no one else in aus with CDA that has had theres removed , there is two other boys with CDA and they both have theres and there doctor says its not a big enough deal to remove it , as it MIGHT only bring up there /my Hb one point from say 7 to 8 ??!!!
so im really confused !!
My doctor thinks it works great with ppl who have Thai , but knows of no cases of anyone else with CDA who has had theres out and done anything !! or any other CDA ppl at all as it is very rare.  
 
Thankyou again for your reply , i was so scared no one would want to talk to me cause i have CDA and not Thai , tho some of my best buds in OZ  have Thai

Lillie

Oh Lilly we are family here and welcome new members all the time.
I'm sorry that I did not meet your friend Joseph but I did meet a few women who were very helpful to me which is why I cant understand you saying there are only males. haha
Yes I live in New Zealand, I stay in Otahuhu although both my parents actually come from up north in the Hokianga. Beauty place where I love to go and get away from the city.
I am having my transfusion on Monday so I will try and get some info for you if I can.
You take care
Cherieann

Hi Lillie ,
i m realy sorry to hear abt your story to wat you passed through , but its life and we have to live it, soo live it to its fullest is the best option to choose , ...

secondly :::  to  The Family ,its nice to have u here .. soo please keep in-touch with us ... i hope you will find it very helping and caring and supporting family .... 

i mm not an experti soo cant say anything on health related issues soo .... 

Best REgards
Take Care
Umair

Hi Lillie,

  on the forum,you don't have to be scared at all dear,we are a big loving family here,we have another member who is not thal,she has PKD, You can see her valuable posts on our forum,so don't be shy and discuss your problems,we'll try to help as much as we can  .

Zaini.

Hello Zaini and Umair ,
thankyou for your warm welcoming    for taking the time to read my post and reply back
it made my day.

hello cherieann,

cool thats great that there are some females my age there! perhaps i went on a wrong day or spoke to an ill informed nurse there !!
i will check again soon , and perhaps go there and visit. All though i have to stay at the alfred hospital as it is better equipped to deal with my cda there, with new mri technology and new Ct scans etc.

How often do you have your treatment ?
and have u had your spleen out ?
if so when and did it change anything for you ?

thankyou

lillie

hep C:
You need some vaccins after the splectodinges...  

Rogue ,

  , , , ,
  , , , ,

Best REgards
Take Care
Umair

gosh !!!
how did you get that ? what hospital did/do you go too?
do you have your treatment in a good hospital ?
do you have all the hep c symptoms ?
do/ have  you have liver failure yet due to the hep c ?
are you on medication for it ?
do you have any babies ? with hep c ?

yes i have had all my vaccines already due to my cda i have to have every and all the vaccine available, due to my low immune system.

hello cherieann,

cool thats great that there are some females my age there! perhaps i went on a wrong day or spoke to an ill informed nurse there !!
i will check again soon , and perhaps go there and visit. All though i have to stay at the alfred hospital as it is better equipped to deal with my cda there, with new mri technology and new Ct scans etc.

How often do you have your treatment ?
and have u had your spleen out ?
if so when and did it change anything for you ?

thankyou

lillie

Hi Lillie...
Yes I have had my spleen taken out when I was 7years old. I was told that it helped my body to go without transfusions for another 3 - 4 years before I was put on a transfusion plan.
At times I have the transfusions set for once every 6 weeks but if needed sooner then I am able to go in sooner. Healthwise I'm okay. I was given a injection that lasted for 10 years and then given another one 10 years later. I have flu injections yearly, that is my choice. Over all if I look after myself properly like getting enough sleep, fluid, chelation and the correct diet then I don't have too many problems.
I only tend to get sick when I let my body get run down.
I was unable to find out more info as the main haematologist wasn't there. I'm sorry but I hope our friends on this site will be able to get the information or know where to access it.
Take care Lillie

Wow, it's amazing to read about everything you've gone through.
It's such an inspiration for someone like me, much younger than you.
I don't have any answers to your questions, but luckily, we have other more experienced and knowledgable members  to do so.

Welcome to the site, I'm new too...so this should be exciting ^^

Over all if I look after myself properly like getting enough sleep, fluid, chelation and the correct diet then I don't have too many problems.

This will be mine plan for the next year - and learning Spanish probably & visiting a friend in Romania - but overall 'your plan to life'

 

Peartree   .
Thats my plan too. I try not to over do it.