Thalassemia Patients and Friends
Discussion Forums => Miscellaneous Questions => Topic started by: Dave on July 18, 2006, 05:44:16 AM
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Hi, my girlfriend's daughter has recently been diagnosed with thal. She is thirteen and right now they are discussing the possibility of blood trans and possibly removing her spleen. I kinda set out on my own to see what was out there for support for my girlfriend and her daughter. Obviously we are all scared and worried about what to expect. Do you feel thirteen is to young for her to use a website like this? Does anyone have any sugestions as to where we should go from here? Are there any teens on this site who might be able and or willing to share their experiences with another teen? Thanks for any help.
Dave
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Hi Dave!
Welcome to the site. I hope that you will find all the information that you require about Thalassemia.
If she has pulled through her childhood to teens without any Transfusions; then you can be sure that she does not have Thal. Major. It appears that she might have Thal. Intermedia!
In many Thal. Intermedia cases it is observed that once the spleen is removed, there is no further requirement of blood transfusions as the troubled organ is removed and thus the red blood cells are no longer destroyed by it.
As far as the age limit to access this site is concerned; well, there is no age limit to obtain knowledge and all patients and their family/friends can also dig in to help each other out.
I'm sure she will find this place very friendly and everyone will try their best to help her out.
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Hi Dave,
As Sajid said, it is most likely thal intermedia. If it was major she would have required transfusions long ago.
The choice will be between splenectomy and transfusions. Splenectomy may eliminate the need for transfusions, at least for now and maybe permanently. Intermedias can have hemoglobin levels that stay just high enough to avoid transfusions but on the low end, Hb of 6-7, life can be difficult and transfusions will help to greatly improve the quality of life. If this can be avoided by splenectomy, it is usually the better of the two choices, as transfusions will lead to iron overload. However, even without transfusions, intermedias often acquire iron overload from their diet as the body absorbs excess iron while trying to build new red blood cells. Intermedias are advised to keep their Hb up and their dietary iron low and to also drink tea with meals, as it reduces the amount of iron absorbed by the gut,
She will also need to be on nutritional supplements, especially high doses of folic acid which help in building new red cells. I think her reading posts on this group will greatly help her understand thalassemia and its implications. It will help her to cope both physically and emotionally to find the support she will find here.
Where are you located and where is she being treated? t is very important to have doctors familiar with thal, as many doctors have only a passing knowledge as they see so few cases of thal in some areas.
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Hi Dave!
Welcome to the site. I hope that you will find all the information that you require about Thalassemia.
If she has pulled through her childhood to teens without any Transfusions; then you can be sure that she does not have Thal. Major. It appears that she might have Thal. Intermedia!
In many Thal. Intermedia cases it is observed that once the spleen is removed, there is no further requirement of blood transfusions as the troubled organ is removed and thus the red blood cells are no longer destroyed by it.
As far as the age limit to access this site is concerned; well, there is no age limit to obtain knowledge and all patients and their family/friends can also dig in to help each other out.
I'm sure she will find this place very friendly and everyone will try their best to help her out.
Thank you for the info, I do believe it was beta thal intermedia that was diagnosed. This site seems just what I was looking for.
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Hi Dave,
As Sajid said, it is most likely thal intermedia. If it was major she would have required transfusions long ago.
The choice will be between splenectomy and transfusions. Splenectomy may eliminate the need for transfusions, at least for now and maybe permanently. Intermedias can have hemoglobin levels that stay just high enough to avoid transfusions but on the low end, Hb of 6-7, life can be difficult and transfusions will help to greatly improve the quality of life. If this can be avoided by splenectomy, it is usually the better of the two choices, as transfusions will lead to iron overload. However, even without transfusions, intermedias often acquire iron overload from their diet as the body absorbs excess iron while trying to build new red blood cells. Intermedias are advised to keep their Hb up and their dietary iron low and to also drink tea with meals, as it reduces the amount of iron absorbed by the gut,
She will also need to be on nutritional supplements, especially high doses of folic acid which help in building new red cells. I think her reading posts on this group will greatly help her understand thalassemia and its implications. It will help her to cope both physically and emotionally to find the support she will find here.
Where are you located and where is she being treated? t is very important to have doctors familiar with thal, as many doctors have only a passing knowledge as they see so few cases of thal in some areas.
Thank you for your insight. Right now she is living in Rhode Island. In my online research I found the site for Thal treatment at Children's Hospital Boston. It seemed very good and I was thinking about directing them there. Do you have any knowledge of that facility? In reading what both you and Sajid wrote I feel a little better. I think my concern with having her acess the site was her confronting the possible terminal nature of thal. Protective instinct I guess. I do feel now though that any negatives would be far out weighted by the positives of this site. Thank you again.
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I think my concern with having her acess the site was her confronting the possible terminal nature of thal. Protective instinct I guess. I do feel now though that any negatives would be far out weighted by the positives of this site. Thank you again.
Welcome to the site, Dave. You don't really need to worry too much about the above for a thal intermedia. We have a member here, who is above 55. And, a couple of others who are above 40. With proper treatment, you really shouldn't have to worry about it. In any case, IMO, it is better to know and deal with it, rather than not know.
As Andy mentioned, the choice is really between taking out the spleen and blood transfusions. I would strong advise taking out the spleen -- she may probably eventually need some transfusions, but you can postpone it for many years (like a couple of decades) if you take out the spleen now.
Take care now,
Poirot
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Thanks for the advice. I set out on my own for info and now I'm actually pretty excited to share what I found. Thanks for the piece of mind. This site is great.
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Hi Dave
Welcome to the site. I understand the protective instinct you have for your loved one. But she is also at a age where she makes her own decisions about her life. The decisions she makes will impact her Thal and overrall health. For example her diet and activities. My wife is Beta Thal Intermedia. She had a splenectomy 5 years ago. We too had to make a choice to "to splenectomise or not to splenectomise". Not always will the Hb go up after splenectomy, as was the case with my wife. But her overall health improved, she was able to eat larger meals. She was able to do more phsical activity which helped in improving her calcium absorption. She gets transfusions but not as regularly as a Thal major would. I HIGHLY recommend this site and also become a member of the Cooleys Anemia Foundation. They are truly awesome. Thal Intermedia is complex and there is a significant overlap in clinical severity.
Regards
Bharat
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Children's Hospital at Boston has an excellent thal center. It it one of the comprehensive care centers in the US.
Ellis Neufeld, MD
Phone: 617-919-2139
Fax: 617-730-0641
http://children.photobooks.com/directory/profile.asp?dbase=main&setsize=5&last=Neufeld&searchButton.x=31&searchButton.y=12&pict_id=9902250
Children’s Hospital of Boston
Boston, MA
617-355-6000
617-355-0443 (TTY)
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Hi Dave: This site is definately fantastic. I've learned so much through this site and I'm really greatful to the people who made + maintain + contribute to this site. I'm sure you'll find the same in few days.
Bharat,
I'm struggling through the decision to go for splenectomy or not :dunno and would appreciate inputs from you as you have already dealt with it when your wife's splenectomy was performed.
You said in the previous post that after spelenectomy, her Hb level didn't change. Can I ask you, what use to be her Hb level before splenectomy and after? Also, how big was her spleen at the time of splenectomy? And what factor/s contributed in taking a decision? Was her requirement for blood increasing? OR was any test perfomed to conclude hypersplenism?
Thanks,
Hallu
ps: I'm not sure if this is the right place to ask Bharat this question as this thread deals with Dave's query. But since Bharat brought this up here I found it ok to post.
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Hello Hallu,
It is OK to post your question to a particular person in a thread if it is related as other users might find the information useful.
You can also send someone a PM(Personal Message)/IM(Instant Message) by clicking the small balloon text icon with IM in it besides the blue profile icon.
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Bharat,
I'm struggling through the decision to go for splenectomy or not :dunno and would appreciate inputs from you as you have already dealt with it when your wife's splenectomy was performed.
You said in the previous post that after spelenectomy, her Hb level didn't change. Can I ask you, what use to be her Hb level before splenectomy and after? Also, how big was her spleen at the time of splenectomy? And what factor/s contributed in taking a decision? Was her requirement for blood increasing? OR was any test perfomed to conclude hypersplenism?
Thanks,
Hallu
ps: I'm not sure if this is the right place to ask Bharat this question as this thread deals with Dave's query. But since Bharat brought this up here I found it ok to post.
Hi Hallu
The decision to remove the spleen varies with each patient. Normally, Physicians often use splenectomy to decrease transfusion requirements. In some cases they become transfusoin free. In my wifes case her spleen was 17.5 cm. (normal 11cm, above 20 cm would be severe) She had pains, would not be able to do any physical activity and she would not be able to eat well. She did not have any transfusions at that time. After the splenectomy she felt much better and her diet improved. Her surgery was done through open traditional method. If you do take the decision to remove the spleen, please consider laproscopy. Your cost,hospital and recovery time may be significantly reduced. Again, the decision varies with each patient. Some studies have shown that during laproscopy patients bleed more. An ultrasound test was done to conclude hypersplenism. But she was also jaundiced and you may want to evaluate any damage to the liver. Where do you currently get treatment.? My wife had her surgery in India. Wonderful doctors and great hospital and a fraction of the cost compared to the US.(if you have insurance issues). Let me know if there is anything else.
p.s Her hgb was between 5-6 prior to splenectomy.
Thanks
Bharat
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Thanks Bharat for sharing this with us. I'm based in Washington DC for the last 5-6 years. If I consider splenectomy, I'll definately ask for laproscopy.
As of now, I have a big spleen (around 20cm). As per my doctor, spleen at times starts producing red cells too (for reference to this theory please see : http://www.thalassaemia.org.cy/books/ABOUTTHA.PDF). So the main question is, if it's certain that destruction is more than the construction(if at all) then splenectomy is beneficial else not(assuming you are fine physically carrying enlarged spleen). Unfortunately, there's no direct test available to confirm this so I've been on wait and watch situation for sometime.
In your wife's case spleen removal did not improve her Hb, which is very interesting and confusing too. As per theory if hypersplenism is detected then removal of spleen should improve Hb. Also, you mentioned that her Hb levels were around 5-6 before splenectomy. Now that's also interesting because she was fine without transfusions at those levels.
I've a follow up appointment in few weeks and will get to know more. Will let you know what I decide.
Anyways, thanks for extending help.
--Hallu
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Hi Dave,
I feel this site will be wonderful for your girlfriend's daughter. I wish I would have had access to such great information and support when I was growing up. I am 55, and have thal intermedia. I did not get the medical help I needed until late in life because the doctors I saw did not know much about intermedia. I did OK growing up, but was sick often and struggled with pain and energy levels. I didn't find out I had thalassemia until I was in my early twenties.
I had my spleen out about 12 years ago because it wasn't working. It was 19 cm and I was short of breath if I walked 5 feet across a room. I couldn't work and had terrible flu symptoms and would have to go to the emergency room often. My hemoglobin levels were in the 6 -7 range and I had no energy and looked yellow. It wasn't until I got advice, and went to a thal center in Oakland CA, that I finally got my spleen out. They couldn't do laproscopic surgery because of the size of my spleen. I felt much better after it was out, but still had to start regular transfusions 8 years later, and should have started them years earlier. I started getting chronically sick with infections and was not able to work. Transfusions helped that tremendously. I had to have my gall bladder out also, which is typical for thal.
In my case, I was more able to tolerate having intermedia when I was younger. As I got older, symptoms got worse - especially when I was in my twenties. I was doing a lot then which didn't help - finishing college, starting a teaching job, playing in a band. Doctors where I lived didn't have any experience with thal. Even going to the Mayo Clinic back then wasn't much help. I think going to the thal clinic in Boston is the best thing you can do. I started getting the care I needed when I went to the thal clinic in Oakland CA. It really helps to see specialists when you have this disease.
I have had a really great life, have adopted 2 children who are now teenagers, have taught school for 29 years, and have played in bands on and off for years. I also have had lots of medical issues to deal with, and have had to use medical leave from my job at different times. Luckily this has worked out OK. If you stay on top of your medical issues by seeing specialists at a thalassemia center, eat healthy, get rest when you need it (I spend lots of time resting), keep your spirit strong, and have good family and friends for support, then you can do fairly well with intermedia - at least I have, though I can't speak for everyone. I'm not saying it has been easy, I have had some really low times, but who doesn't in life?
I was so happy to find this site; I had never met or talked to anyone else with thal before, and I felt so relieved to hear what others had to say. I no longer felt alienated and alone. I would be glad to talk to this young girl through e-mail or on the phone at any time if she wanted to do that. Otherwise, good luck to you all! Jean :wave
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Dave,
I have been to the Children's Hospital in Boston last month. They have a Thalassemia Center and have doctor's who know Thalassemia. There are quite a few patients who are Thalassemic's and come to the Children's Hosptial for blood transfusions
-Narendra
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I just came across this article and it can only add to the debate about splenectomy. This is a difficult choice, as we have heard from many patients who were helped by splenectomy. However, recent research indicates that splenectomy can lead to other problems.
http://www.emedicine.com/ped/topic2232.htm
Splenectomy is frequently recommended for patients who are no longer able to maintain an adequate Hb level. It is usually performed to restore the Hb steady state in patients who are not receiving blood transfusions and frequently succeeds in averting the need for regular transfusions.
Recent observations and case reports have shown that splenectomy in such patients may cause serious venous thrombotic events ranging from deep vein thrombosis to pulmonary thrombotic lesions complicated by pulmonary hypertension. For this reason, one should delay or reconsider such a procedure whenever possible. In support of such a recommendation is the fact that many children who underwent splenectomy to avoid becoming transfusion dependent have only a transient effect and the need for regular transfusions later was the rule in most of them.
Surgery is always a final option and all facts should be weighed before proceeding.
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Andy you are absolutely right!
God has put that organ there as it does some good things in the first place.
Removing it should be the last option!
I was advised to remove it during my platelet episode but gave it one more chance. They gave me hyper transfusions of platelets and Vitamin K shots. Thus after that it started working and I am still hanging on to it.
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I just came across this article and it can only add to the debate about splenectomy. This is a difficult choice, as we have heard from many patients who were helped by splenectomy. However, recent research indicates that splenectomy can lead to other problems.
http://www.emedicine.com/ped/topic2232.htm
Surgery is always a final option and all facts should be weighed before proceeding.
Hey Andy,
Thanks for that quote ...... doctors are sounding more like lawyers now. On one hand, OTOH, OTTH ..... ha!
The truth of the matter is that there is risk in everything we do .... we could be killed causing the street, for heaven's sake, by a drunk driver. The anecdotal evidence is that an overly large majority of intermedia thals (and E-thals) have benefitted from taking out the spleen at an early age ....... and you can find plenty of them in this group itself. And, calling the effects transient is really laughable in the case of intermedias ..... i think 20-30 years is a hell of a transient period, to avoid transfusions and iron chelation. Hey doc, I will take that anytime!!! We have to remember that thals are too small a group to be statistically significant in most cases, so medical opinion is just that. Hence, I would take thal anecdotal evidence over medical opinion anyday!
And, this is the first time that I have heard of splenectomies causing DVT (note all the lawlery language around this assertion!) .... Also, I notice that the study does not quote any standard medical trial, just some 'case reports'?
BTW, in many cases, you can take out the spleen these days by laparoscopy, instead of open surgery.
Anyways, my two cents there.
Poirot
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Hi
As far as my knowledge and experience goes, splenectomy has helped a whole lot of E thals and I agree with Piorot.
When my daughter was young ( she is now transfusion dependent E-Thal), she did not need blood till she was 4 years of age. Then Dr. Bernedette Model ( UK) had advised me to take out her spleen.. maybe if I had done that, we could have avoided transfusions, or maybe we could have deferred the transfusion regime.
Spleen really does very little in an adult. And now there are such good life time vaccines available. And the surgery is very very simple.
My daighter had her spleen removed at age 21 and then it was a major surgery. Now it is no longer a major surgery. My daughter does not even take penicillin . She has had the Puemovax vaccine and so far she is just fine. She is 29 years.
Spelectomy will definetly postpone her transfusion if at all.
Shikha
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As I said, this article will add to the debate about splenectomy. Many people swear by it and others question if it was indeed a help. In Lisa's case it meant every cold and infection had to be carefully watched, as they often became serious life-threatening infections. A simple cold often landed her in the emergency room with raging fevers. The spleen is a huge part of the body's immune system and without it, much care needs to be taken.
Whether it provides some relief from transfusion dependency seems to vary from case to case. Some seem to not even get any temporary relief as far as frequency of transfusion and others find substantial relief. I would not just write off this article because these findings haven't been observed before. If these findings are further corroborated, it will make a serious argument against splenectomy, just as the arguments in favor of instant tonsillectomies vanished over the years (much too late to save my tonsils).
As I said, surgery should always be a last resort. You can't go back once you do it. As was Sajid's experience, the problem was corrected over time, without splenectomy. In some cases there may be no choice as a ruptured spleen can create all sorts of new problems, including death.
As I requested long ago in our MSN group, I would like to have everyone share their spleen stories with us. It is a very difficult decision to make to have a splenectomy and those considering it can greatly benefit from your experience. We can look at study after study, but personal experience often can outweigh those when one has to make that decision. So, everyone, please share your experiences.
Thank you.
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I completely agree with Andy as splenectomy is indeed a very sensitive and difficult subject. My observation is earlier doctors used to recommend splenectomy more liberally as compared to today. These days they recommend to keep the spleen as long as possible as it's a well established fact now that spleen plays a very important role in immune system.
There are vaccines available for only few known bacterial infections but then there are many other bacterial infections which are not even known yet and a splenectomised person can be in trouble if infected with them.
However, in case of hypersplenism, benefits of splenectomy outweighs the risks associated with it. Unfortunately there's no direct test which can prove hypersplenism state... it's always a best guess.
--Hallu
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Hi Jemma,
Living in LA LA land is not so bad because it kept us out of the looney bins(LOL) :rotfl :rotfl :rotfl :rotfl
DO not get mad with us that is because we all have different point of view on the same subject,
I'm positive that we all have your best interest at heart and we wish you the best .You are welcome to express your concern and worries ,on the other hand you need to appreciate others , honesty.
life is a roller coaster and we are all trying our best to survive with minimum difficulty as posible,learn to digest every word with a pinch of salt or sugar :wink,
Take care there is an answer somewhere, good luck .
regards Kathy.