Hello there! "New here!"

  • 9 Replies
  • 8527 Views
Hello there! "New here!"
« on: February 14, 2012, 08:15:45 AM »
Hi my name is Eric. I am a 23 year old Asian Male.i live in the US. i have had Thalaseemia for my whole life. I am very scared because i don't know what to do. i had my gallbladder removed when i was 10. and my Spleen is huge . and i don't know i  should remove it, because if i do. Isn't it gonna be really easy for me to get sick easily? because doesn't your spleen help fight virus and bacteria in your body? I'm scared on on what to do. i want to take care of it as soon as possible. the thing is, i don't want to talk out my spleen unless i have too. because i hate having surgery. how many if you guys out there removed there spleen? and how did everything turned out after that? any regrets? was it beneficial? for you? Also Is there a cure for Thalaseemia yet? the type i have is ALPHA and i know blood transfusions is treatment but i don't like that idea but if i have to i will. im taking folic acid right now for medication. I always so tired and jondas it sucks. i heard they have like stem blood transplant but that operation is risky. i may feel sad and depressed sometimes but on a bright side at least i don't have cancer right. Any advice with from you guys would be very much appreciated im glad im not alone.


Thank you

*

Offline Laura

  • ***
  • 155
  • Gender: Female
Re: Hello there! "New here!"
« Reply #1 on: February 14, 2012, 11:41:05 AM »
Hello and welcome to the forum!

I have the same question since I was your age more or less. Im 29 rigth now and I still have my sleen since my doctor hasn't been able to give me good reasons to do it. The size has been the same for ages and I don't require more transfussions so for me there are not enough reasons to take it out and for my doctor it is not a must right now although she's always talking about the possibility and asking ne to consider it.

Hope you will find the rigth path. There's a lot of them and each of us has to fond his own. Just talk to your doctor and ask for the consequences of both options.

Kisses,

Laura
The most important thing in life is not what you achieve but the fact of fighting for it.

*

Offline Narendra

  • ****
  • 462
  • Gender: Male
Re: Hello there! "New here!"
« Reply #2 on: February 14, 2012, 02:10:38 PM »
Welcome to the forum. What type of Alpha Thalassemia do you have?

There are 4 types of Alpha Thalassemia
  • One gene alpha thal (also called silent carrier)
  • two gene abnormal - alpha thalassemia trait
  • three gene abnormal - Hemoglobin H Thalassemia
  • Thalassemia Major - This is when the fetus needs blood transfusion while in the womb

Refer to more details for Alpha Thalassemia on http://www.cooleysanemia.org/updates/pdf/Alpha_Thalassemia.pdf

Are you on regular blood transfusions? There are a lot of folks who have removed the spleen in their 20's and 30's. There is pros and cons to removing the spleen. Your answer to the above questions can help you provide better replies to your queries.

Re: Hello there! "New here!"
« Reply #3 on: February 14, 2012, 04:09:49 PM »
The type I have is the 2nd choice out of the four you listed ^. So I'm missing 2 Genes. And I'm 85% that is correct from what my DR. Told me. I don't have Regualr blood transfusions like I normal would I think the last one I had was last September 2011 when I had surgery on my nose. But yeah other than that I don't do them at all. I know I should though. I was have very high iron levels as well. What worries is me the most is my spleen because it's massive. And I'm Afriad if in a car accident or something it will erupt can die from interal bleeding. That's like my biggest fear.

*

Offline Narendra

  • ****
  • 462
  • Gender: Male
Re: Hello there! "New here!"
« Reply #4 on: February 14, 2012, 05:02:45 PM »
What is your Iron levels? You might need to start chelation to remove the excess iron.

Here is some information from the Standard of Care Guidelines for Splenectomy

Quote
The use of splenectomy in thalassemia has declined in recent years. This is partly due to a decreased prevalence of hypersplenism in adequately transfused patients. There is also an increased appreciation of the adverse effects of splenectomy on blood coagulation. In general, splenectomy should be avoided unless absolutely indicated.

Splenectomy is indicated in the transfusion-dependent patient when hypersplenism increases blood transfusion requirement and prevents adequate control of body iron with chelation therapy. An enlarged spleen—without an associated increase in transfusion requirement—is not necessarily an indication for surgery. Patients with hypersplenism may have moderate to enormous splenomegaly, and some degree of neutropenia or thrombocytopenia may be present.

Annual transfusion volume exceeding 225 to 250 mL/kg per year with packed red blood cells (hematocrit 75 percent) may indicate the presence of hypersplenism. The volume calculation should be corrected if the average hematocrit is less than 75 percent. The possible development of alloantibody should also be ruled out. Splenectomy should be avoided unless there is an inability to maintain iron balance with optimal chelation, or if there are clinically significant complications such as pancytopenia and marked enlargement. Often, hypersplenism develops because of a low pre-transfusion hemoglobin. Increasing the pre-transfusion hemoglobin to between 9.5 and 10 may
reverse hypersplenism.

If a decision to perform surgery is made, partial or full splenectomy is the option. Partial splenectomy is a complicated surgery utilized to preserve some splenic function. It should be reserved for infants requiring splenectomy. Full splenectomy can usually be performed by laparoscopic technique. However, open procedure is necessary in cases of marked splenomegaly. The indications for splenectomy in hemoglobin H–Constant Spring patients are different than in beta-thalassemia disorders. Transfusiondependent infants with hemoglobin H–Constant Spring respond rapidly to
splenectomy but require prophylactic anticoagulation because of a high incidence of serious thrombosis.

Patients must receive adequate immunization against Streptococcus pneumoniae, Haemophilus influenzae type B, and Neisseria meningitides prior to surgery. Splenectomy should be avoided in children younger than five years because of a greater risk of fulminant post-splenectomy sepsis.

After splenectomy, patients should receive oral penicillin prophylaxis (250 mg twice daily) and be instructed to seek urgent medical attention for a fever over 101º Fahrenheit.

Post-splenectomy thrombocytosis is common, and low-dose aspirin should be given during this time. Another complication following splenectomy is the development of a thrombophilic state. Venous thromboembolism, more common in thalassemia intermedia and hemoglobin H–Constant Spring, can develop following splenectomy.

Patients should have annual echocardiographic measurement of the pulmonary artery pressure to monitor for development of pulmonary hypertension.

Talk with your doctor in this regards.

Re: Hello there! "New here!"
« Reply #5 on: February 14, 2012, 10:20:41 PM »
My Iron levels are pretty high. i use to take this medication called EXJADE for a while so my iron levels will go donw. but Ive stoped. last time i check last September, my iron levels also know an as Ferritin level right? was 97% ? im not to sure. but Hemoglobin was 7.1.

*

Offline Andy Battaglia

  • *****
  • 8793
  • Gender: Male
  • Will thal rule you or will you rule thal?
Re: Hello there! "New here!"
« Reply #6 on: February 15, 2012, 12:53:04 AM »
Yellowboy,

I suspect that you have HbH disease, a 3 gene deletion. I say this because your Hb is very low and this is not normally found in 2 gene deletion alpha thal. Also, your Hb is low even for HbH, so it might even be HbH constant spring, in which transfusions often are necessary. There is one other exception that could cause similar problems to what you describe and that is when one of the alpha genes is triplicated (the triplicated alpha gene is known to exist in Asian populations). When one triplicated gene is combined with one more alpha deletion, a thal intermedia-like condition can occur, even though only 2 of the 4 genes are affected. It would be helpful to know exactly what your true classification is, and this can only be done through a DNA analysis that would look for both deletions and mutations on the alpha genes.  Have you ever had a DNA test done?

How much folic acid do you take daily? Do you also take any other supplements?  Have you ever had a liver MRI? In thal intermedia, iron load often cannot be accurately be determined by ferritin test. A scan is necessary to determine if chelation should be continued. An Hb of 7 is considered to be the line where transfusions should begin. Your spleen is overactive and obviously affecting your Hb level.  I believe to accurately determine why, you will need the DNA test, as the possibility of a triplicated gene must be investigated. When that gene is present and one other alpha deletion is present, an unstable hemoglobin can be produced which breaks down very quickly. This would explain why your spleen is so large, as it filters the "bad" blood out. It would be extremely unusually for a 2 gene deleted alpha thal to have your symptoms. I feel you would benefit greatly by being seen at one of the US Centers of Excellence. You need to be seen by doctors familiar with alpha thalassemia so you can get a proper diagnosis and proper treatment, if any is needed. You need to find out if you can improve the quality of your life. This can only begin with getting a diagnosis that explains your symptoms. Even if you are treated locally, being seen at one of the Centers is highly recommended. For transfusing patients, annual visits are the norm.
You can contact Eileen at CAF to find which center is closest to you.
Cooley's Anemia Foundation
330 Seventh Avenue, #900
New York, NY 10001
(800)522-7222
Fax: 212-279-5999

Patient Services Manager
Eileen Scott

Let us know about the folic acid and supplements. There may be some things you can do to help yourself, but I will be honest. There are not many prescribed treatments for alpha thal. But I do feel that you can do a few things to improve things. One thing you should be aware of is that certain flat beans, like fava beans should be avoided because they can cause a sudden hemolytic crisis, which will cause a drop in HB. Certain chemicals and fumes, like moth balls can also cause this. There is a partial list at http://www.thalassemiapatientsandfriends.com/index.php/topic,3410.msg34830.html#msg34830 
I have also asked Bobby to post about his management of his enlarged spleen.
Andy

All we are saying is give thals a chance.

*

Offline Bobby

  • ***
  • 156
  • Gender: Male
Re: Hello there! "New here!"
« Reply #7 on: February 15, 2012, 03:10:20 AM »
Hi Eric. I'm Bobby and I have Beta Thal Intermedia. We seem to have a very similiar background. I had my Gallbladder out at 13 and as a child I was alway tired and sleepy. I've had serveral blood transfusions here and there, the last one being 6 years ago and almost 20 before that. I'm 36 by the way. My ferritan levels were very high a couple years ago, but 5 months of Exjade brought it down nicely. From 2200 to 750.

My spleen is also very large, it always has been and my hematolgist will often suggest removal if it gets bigger, but I'm opposed to it. Even though it's large(.62cm) I plan on keeping it as long as possible. It's doesn't hurt and my Dr. says it works fine. It will bother me if I run for longer than 15 mins at a time, but I'm not a big runner so that's fine. I workout on a regular basis, mostly with weights and I eat plenty to keep from losing weight. I believe working out helps me more than any other treatment. I feel better when I do. As far as supplements I take a daily multi-vitamin without iron. GNC MegaMen Sport and I drink protein shakes before and after workouts. I will sometimes take a N.O supplement, but I've been cycling off the last few months. Starting back in a week or so.

I think as long as your spleen is healthy and doesn't cause any pain that you should keep it. I have been active in a number of sport and fortunately haven't had any collisions or reason to worry about it. Feel free to email me if you have any questions I can answer. Take care, hope to hear from you soon.


Re: Hello there! "New here!"
« Reply #8 on: February 15, 2012, 04:29:42 AM »
Thank you guys so much for your support. i really do appreciated it. Andy i will get back to you on what time of Thal i have. i know its serious.
For medication i use to take folic acid daily and now i just stopped. but im going to start taking it from now on, Doesn't folic Acid help produce
red blood cells? And as far as my high iron level i should talk to my DR about getting back on EXJADE. I did take it for a while but it got expensive
And my insurance is not the best. but yeah thank you for your help Andy. i hope i don't have a serious type of it. I know that my life is fine because
I had a liver biopsy like 2 years ago. And the DR. says my liver is in good shape.

Bobby- I'm glad there is someone out there that is almost similar to me. I don't want to take out my spleen unless i have too.. i don't exercise daily
which i should but its very hard for me. im always tired just walking up the stairs at work. i do need to take care of myself better if i want to live long.
For work. i have a sit down job. i work in a call center. and i weight 180lbs. i really want to lose weight and get a 6 pack but im always afraid gthe spleen
will get the way. My spleen does ache sometime, my doctor says its so big that there mild compression on my left kidney but its no big deal. Me having THAL
i am always depressed. i don't have the confidence to do much in life. i really want to go collage but im afraid its gonna be overwhelming for me. Physical and Mentally 
And is "slow growth" a symptom?  for thal? because im 23 and i look like 18.  And much of my body did not grow. i have looked the same and feel the same since highschool
 is that Normal because of what i have?

Bobby and and Andy- My biggest fear is to live pass 50... that's what im really scared of. is it like hunting's disease where you only have a certain time to live?
i one day want to have kids and a family. I hope i get to see that day.

*

Offline Bobby

  • ***
  • 156
  • Gender: Male
Re: Hello there! "New here!"
« Reply #9 on: February 15, 2012, 06:01:44 AM »
Eric,

I understand. Try and remain positive though. Sometimes I get lethargic and depressed, but a little self evaluation gets me back on track. I was always told I couldn't play sports because of my spleen. However I did because it made me happy. I wasn't the best athelete, but I tried hard. Dr.s told me I shouldn't have children because of my Thal and I have 2 amazings kids. Healthy, smart and happy. While its important to take care of yourself, I don't think you should limit yourself. Go to school, find a wife and plan for the future, get a 6 pack. I use to fear not living past 40, but at 36 I feel like I'm stronger than I was in my 20's. I don't think that you should worry about that at all.

Do you have any hobbies? Being good at something you like will give you more confidence. I'm into sports and last year I coached 15yr old boys basketball team, my company softball team and a summer camp at the YMCA. In most cases I was the fastest person on the field. If I can do it you definitely can. You're young and you have plenty of time. :thumbsup Go be great my friend.

As far as growth. I started out slow. Even now people say I have a young face. Until 9th grade I was smaller than the smallest girl in my class. I did grow to 6'2.5" by the time I graduated high school. Possibly due to an increase in appetite from sports and my parents are tall. Mom - 5'9" and dad 6'0". I am 180lbs, but for the last 10 years or longer I've been trying to make it to 195lb. The closest I got was 190lb for about a month and I lost it.  :dunno lol. I keep trying though. I have read many other Thal friends in this forum mention growth being slow.

I hope I was able to help. This Forum is full of awesome people and Andy definitely knows more about Thal that anyone I've ever seen. Welcome!

 

SMF spam blocked by CleanTalk