Thalassemia Patients and Friends
Discussion Forums => The Spotlight => Topic started by: Fahed_FN on November 03, 2014, 05:14:50 PM
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Hi
My son is 1.5 years old and he was diagnosed with thalassemia when he was around 10 months. His hemoglobine analysis back then were as follows:
A : 14.5
A2 : 2.6
F : > 40
Hb : 8.4
Since then his HB has been ranging between 7.3 lowest and 8.9 highest. The doctor recommends to give him blood when HB is below 8 and he got transfusion when HB was 7.3. But then another doctor told us there is no need for transfusion if he is maintaining an HB above 7. This is were we are confused wether he should be getting transfusion or not, and wether he is major or intermedia. We are also not sure if we should consider BMT and start preparing for it or no need for it.
Hope I can get some guidance here on how to move forward.
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Hi Fahed_FN,
The electrophoresis looks like beta thal intermedia, as does your description of your son. According to your bio, hydroxyurea will be tried later. There is actually no reason to wait until age 2. I would highly recommend that another electrophoresis is done before starting, so you have a baseline percentage for the HbF level, as a rise in this will indicate that the drug is working. Because this is planned, I would suggest refraining from transfusion until after the drug has been tried for at least 6 months. If his Hb drops below 7, you will have no choice but to transfuse. He should also be taking folic acid daily (L-methylfolate is the preferred type) and should continue that while on hydroxyurea. It is worth trying. We do know patients freed from years of transfusion by using hydroxyurea and wheatgrass together.
BMT is not available to non-transfusing patients, so for the time being, no decision can be made on that. If his Hb drops and he must be transfused regularly, then a BMT could be considered.
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Thanks Andy for your reply,
The doctor was against giving HU before the age of 2, according to him it is better so that his body will be ready to receive this chemical medicine. my son is on Folic Acid and we are following up on him on a every monthly or 6 weeks, and the doctor will be doing another electrophoresis soon.
My son has been healthy and active so far, except for some paleness and yellowish eyes. And we are always worried about the future and how thalassemia will treat him or how it will affect his life and consequently all our lives.
I have a number of questions which i am not being able to find final answers for, i would really appreciate if you would answer or at least make it clear for us:
1 - Can he live normally if he maintains his Hb above 7? of course with the usual symptoms of anemia such as the spleen issues and other things.
2 - Does HU loses its efficiency with time, i.e if it works, will it be not boosting Hb after years of usage? and in case it does, i guess it will be too late to consider BMT as it is preferred to do it before the age of 7 right?
3 - Our doctor says some Intermedias upgrade themselves to major, if this is true, will it be permanent? i.e will he be needing transfusions constantly and he cannot maintain his Hb above 7? or it will be from time to time?
I really appreciate the time you are spending in replying to all of us, it is really making a difference.
Thank You
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1 - Can he live normally if he maintains his Hb above 7? of course with the usual symptoms of anemia such as the spleen issues and other things.
Living at 7 Hg has its issues as you mentioned. Baseline Hg in Canada is 8.5, below this level triggers blood transfusion. You may try occasional blood transfusion to keep the Hg above 8.5-9. This will help the tiredness, spleen enlargement, mongoloid appearance and other issues.
2 - Does HU loses its efficiency with time, i.e if it works, will it be not boosting Hb after years of usage? and in case it does, i guess it will be too late to consider BMT as it is preferred to do it before the age of 7 right?
Hudroxyurea is relatively new drug, long term issues and efficacy is unknown at this time. The option of BMT will always be present, however, BMT can have certain drawbacks as well. I don't want you to think that BMT is the ultimate cure, you need a matched donor (have you checked for that), GVHD issues, puberty issues, etc are common side effects of BMT.
3 - Our doctor says some Intermedias upgrade themselves to major, if this is true, will it be permanent? i.e will he be needing transfusions constantly and he cannot maintain his Hb above 7? or it will be from time to time?
If the diagnosis of intermedia is done properly including DNA testing, the intermedia will not convert to major.
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Thanks Canadian_family for your reply.
We are not thinking of BMT as an ultimate cure, all what we need is to insure a healthy and proper life for our kid through any possible cure, and apparently BMT is the only known cure so far. All I'm worried about is that HU will work for a while and then will stop being effective, by then it may be too late or too risky to consider BMT.
We are closely looking up for the new gene therapy trials but it needs years to be available for the public. And maybe it will not be available for non-transfusing patients as well.
We are currently giving him Iranian saffron which is said to be very good for the blood. We hope it will help a bit.
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We are not thinking of BMT as an ultimate cure, all what we need is to insure a healthy and proper life for our kid through any possible cure, and apparently BMT is the only known cure so far. All I'm worried about is that HU will work for a while and then will stop being effective, by then it may be too late or too risky to consider BMT.
It will be not late or risky for BMT if HU does not work. Rest assured.
We are closely looking up for the new gene therapy trials but it needs years to be available for the public. And maybe it will not be available for non-transfusing patients as well.
Given the young age of your child, you have the time on your side to wait for the results of gene therapy.
We are currently giving him Iranian saffron which is said to be very good for the blood. We hope it will help a bit.
Folic acid is another option for you to consider.
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Hi,
We have visited the doctor today and were surprised his HB was down from 7.9 in October 1st to 6.9 today. The doctor said that we shall do another test after 10 days and we'll see it it keeps on dropping or not, and we'll transfuse if it does. Moreover the doctor said that his latest electrophoresis from October 1st showed hemoglobine A at 24%
My son was sick a couple of weeks back and it was a combination on infections including teething and flu. So we were regularly visiting our pediatrician and were giving him multiple medications. I wonder if this could cause a drop in HB. He has been very active lately so I was expecting the HB level to go the other way ???
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Yes, being ill can lower Hb. His Hb is quite low now, so if it does not rise soon, a transfusion will probably be needed.
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Hi,
Today we have had the second appointment to follow up on the previous drop in HB and it was up to 7.5 after it was 6.9 11 days ago.
So the doctor considered this as a good sign and gave us another appointment after 6 weeks.
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It is a good sign and hopefully the Hb will continue to rise. I do think that it's worth trying hydroxyurea and wheatgrass together. We are seeing quite a few patients in India whose Hb was intermedia level and required transfusions who now are transfusion free after using hydroxyurea.
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Thanks Dr. Andy for your reply,
Our doctor believes there is a chance it may be delta beta thalassemia and if so his HB will rise by the age of 2. We hope so..
As for HU, he still insists we should wait until he is 2 years old or slightly before.
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With that high an HbF level, delta beta thal does make sense. It also increases the chances of hydroxyurea working once it is started.
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Dear All,
We have visited the doctor today and my son's the HB was 7 but the doctor was happy that he was growing well and active, so he decided to start the HU as of today. He is confident HB will rise to 9 soon.
We will be going again in 2 weeks to check the effect of HU on WBC and platelets but until then, we need to keep a close eye on my son as the HU is taking effect.
I do appreciate any advise from the experienced parents at this point.
Thanks
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Hi everyone,
Just an update.
Yesterday was our followup appointment for my son after starting HU 2 weeks back. Back then his Hb was 7 and the doctor advised to start with 1.25 ml per day(he is 13 kg).
Now his Hb is 6.8, so the doctor advised to increase HU to 2 ml/day.
I dont know if Hb not increasing is normal or expected at this stage. We are hoping this will change by our next visit in 2 weeks.
I will keep you posted.
Take care.
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Dear Fahed_FN,
Hope the next result will be good.
Mridula
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Thanks for updating us. We hope your child's continue to improve...
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Hello friends,
I wished I will be writing some good news but unfortunately this is not the case. Today was our two weeks follow up and HB was down from 6.8 to 6.6 even though HU is on since one month and dosage is increased to 2 ml in the last couple of weeks. So the doctors have announced HU to be inaffective as of now and to go to transfusions ASAP.
We really cannot believe HU has failed already especially when we were putting so much hope on it, we are really disappointed and we do not know how things will be in the upcoming future. Doctor may go back to HU if things stabilise when my son is 2 years old....who knows?!!!
Thank you all for all your support so far.
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A one month trial is not sufficient to determine if hydroxyurea is working. It does not sound like the doctor was sincere about trying it. The drug can be continued even when a patient is transfusing. I believe that at least a 6 month trial should be done.
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Andy said it right.
Only one month and the doctor came to conclusion. You need atleast 6 months. Continue with hydroxyurea and transfusion.
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Thanks Dr Andy and Canadian Family for your replies...
Actually I was having some doubts that HU was causing the HB to drop, is this possible? 6.6 is the lowest he has reached. I don't have the test results but the doctor said the percentage of bad cells is increasing in a way that can cause internals to enlarge as well as bone deformalities...
He has around 20% HBA which makes us wonder why he is Not producing enough HB!
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Hello friends...
This is an update. My son's HB is up from 6.6 to 6.9 and the nucleated RBCs are down from 12% to 6%. The doctor postponed the blood transfusion and the next visit is in two weeks.
Regards,
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Hi Fahed,
I was diagnosed as thal major at the age of 7. At my 21 age, I was rediagosed as intermediate. I had 150+ transfusion. My last transfusion was on June, 2009.
In my childhood my HB was around 6.8.
After 2009, b4r introducing hydroxy urea it was above 7.4
Now I'm maintaining above 8.5. HU helped me to achieve it.
Never give up. Ur child will not become major. as he grows he LL increase his HB.
But pls supplement him with adequate calcium. I have developed avian on both hip joints coz of kelfer, desirox, low calcium. Make sure to take care of ur childs born health too.
I'm leading a normal life, working too for past 4 years with 8.5+ HB except this hip joint pain.
Grow him in a positive environment. Allow him to do whatever he wants. Never discourage him or tell him u can't do this. He s also a normal child as others who needs SPL attention. Never tell his future friends abt his disorder. Never worry abt his future he will be a champ for sure.
Regards,
Tamizhan.
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Dear Tamizhan,
Thank you for your nice sharing.My baby is now 18 months old and waiting for giving Hydroxyurea to her when she will be 2.She needs transfusion after every 7 weeks.Thanks for your positive response about Hydroxyurea.
Keep us update.
Thanks
Hi Fahed,
I was diagnosed as thal major at the age of 7. At my 21 age, I was rediagosed as intermediate. I had 150+ transfusion. My last transfusion was on June, 2009.
In my childhood my HB was around 6.8.
After 2009, b4r introducing hydroxy urea it was above 7.4
Now I'm maintaining above 8.5. HU helped me to achieve it.
Never give up. Ur child will not become major. as he grows he LL increase his HB.
But pls supplement him with adequate calcium. I have developed avian on both hip joints coz of kelfer, desirox, low calcium. Make sure to take care of ur childs born health too.
I'm leading a normal life, working too for past 4 years with 8.5+ HB except this hip joint pain.
Grow him in a positive environment. Allow him to do whatever he wants. Never discourage him or tell him u can't do this. He s also a normal child as others who needs SPL attention. Never tell his future friends abt his disorder. Never worry abt his future he will be a champ for sure.
Regards,
Tamizhan.
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Dear Tamizhan,
I really appreciate your encouraging words. I hope my son and every thal will live a normal life as it is a right not a privilege. I do hove nothing but full respect to you and all brave thals out there.
Dear Lokkhi,
If your daughter is growing normal then HU may be started earlier. My son started HU when he was 21 months as the doctor said he had the weight and measures of a 2 years old so his body can tolerate HU.
Regards
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Dear Fahed_FN,
Thanks for your reply.Where are you from?My baby is now 18 months old,weight is 13 kg. and her growth is normal.Andy and my doctor both are advised not to take any decision now about HU.Wait upto 2 to 2.5 yrs.So I am waiting and now I am growing wheatgrass at my home to give her the juice.
Dear don't worry.I have followed your every post to know about your babies update.Everything will be all right. WE WILL DO EVERYTHING FOR OUR CHILD :heartpink
Thanks
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Dear Lokkhi,
I'm from Lebanon and I live in UAE. My son started HU when he was 13 Kg, I don't know if this may apply to your daughter. We are still awaiting for HU to be effective and Andy advised to try it for at least 6 months.
Regards,
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Dear Fahed_FN,
I'm also waiting for the positive and good response of HU to your son.I think it will encourage me.May the HU bring the good result.
Thanks
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Dear friends,
My son's temperature is rising for the last 3 days and sometimes exceeding 39. We are giving him medicines and suppositories to bringing it down but again it is rising.
Is this related to HU? and if yes does this indicate it is working?
Regards,
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Fever can be a side effect of hydroxyurea. The drug should be stopped temporarily and if the fever does not drop, the doctor should be called. The drug can be continued once the fever has passed but the patient should be monitored for further fever. The dose should also be re-examined. If the dose is higher than 10 mg/kg, it should be reduced to that amount when beginning the drug again.
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Thanks Andy for your quick response.
We started with 1.25 ml daily and increased to 2 ml. He is 13 kg.
Is the current dosage correct ?
Regards,
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That is a good regular dose. I would suggest stopping during the fever and resuming with half a dose for a few days to make sure the fever does not return, and then raising it back to 2 ml/day.
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Thanks Andy,
We will do and will keep you posted.
Regards,
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Dear Andy,
In the month of January my HB was 8.9. This month result shows, HB is 8.4.
For the past 1.6 years I was having hydroxyurea 500 mg OD. This time doctor advised me to increase it to 500 mg BD, morn & night. My weight is 58 & height is > 163.
Whether my body weight can withstand the HU increased dosage.
Does HU have any side effects?
And doctor asked me to check my blood HB level every 15 days as HU dosage is increased. since HU may also cause reverse action.
Regards,
Tamizhan
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Tamizhan,
What is OD and BD?
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OD - once a day. morning only.
BD - both the time. Morning & night.
TDs - morning, afternoon & night .
Previously I was having hydroxyurea 500 mg morning only.
Now doctor asked me to take hydroxyurea 500 mg morning & night.
Regards,
Tamizhan.
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Tamizhan,
The dose is a bit higher than the 15 mg/kg that I prefer, but still under 20 mg/kg daily. Hydroxyurea does have some side effects, which are mostly mild when they do occur. It may also affect fertility but that is reversible when the drug is stopped. If you are concerned about suddenly doubling the dose, try alternating days. Take 1000 mg and then 500 the following day. Try this for a week or two. If you have no ill effects, take 1000 mg daily in two doses as instructed. Also take folic acid with the drug and wheatgrass in some form. L-carnitine and magnesium are also both known to increase the effects of hydroxyurea.
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Thank you, Andy for your reply.
I'll follow the same.
I'm having calcium 500mg, folic acid 2.5 mg & vitamin -e.
Happy weekend, Andy
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Dear Andy,
I took 1000 mg of hydroxyurea for past 20 days from April 10th to may 1 st.
I.e. 500 mg in the morning and 500 mg in the evening Hoping my HB will increase.
But when I tested my Hb on may 2nd, HB has reduced to 7.1.
Jan 3rd - Hb 8.9 : I took 500 mg hydroxyurea morning alone.
March 29th - Hb 8.4 : I took 500 mg hydroxyurea morning alone.
April 10th to May 1st, took hydroxyurea 500 mg in the morning and night.
May 2nd blood test : HB 7.1.
I'm taking folic acid 5 mg once in two days.
Before having hydroxyurea in 2013, Hb was around 7.4 +.
Kindly advice, can I take 500 mg of hydroxyurea in the morning only. Or can I stop hydroxyurea completely?
Thanks in advance.
Regards,
Tamizhan.
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I think you need to give the higher dosage a little more time before making a decision. If you can get wheatgrass tablets, add them to your daily routine.
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Sure, Andy. I'll tty to get wheat grass tablet from amazon. As doctor advised, I've reduced to 500 mg.
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Hello friends,
Today we went for transfusion since my sons HB went down to 5.5 two days back from 6.9 last month, it was 5.8 today before the transfusion.
Doctor said this drop may be.related to sickness or infection, so we will continue HU and monitoring. If thing don't get better in the upcoming months then we will go to regular transfusions.
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Dear Fahed_FN,
Don't worry.Everything will be ok.All the best...
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Dear Fahed,
It is best to maintain your child's hemoglobin near 10.0 - it should not drop below 9 in order for your child to thrive. Giving your child several transfusions, closer together until a stable hemoglobin near 10 is achieved is important.
Please keep us posted,
Best,
Sharmin
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Fahed,
The Hb is very low. If it does not soon stabilize, regular transfusions should commence.
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Hello friends,
After 35 days from the last transfusion my son's HB was 8.1 yesterday. I dont know if this is because he got transfued last month or because he is on HU, in all cases it was a good news for us as it was not expected.
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Its really great... :happyyes
Don't lose your hope Fahed. :smileblue :smileblue
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Hello friends,
This is an update on my son's condition.
Today he got transfused after his HB was 5.6. Last transfusion was on May, after which his HB was 8.1 and 7.4 in June and July consecutively. Then he went on vacation for nearly 2.5 months to find out his HB is 5.6 and his spleen is 4-5 cm (he is currently sick).
BTW he is on HU since February, the current dosage is 3 ml per day and he is 13.5 KG.
I have got the feeling that he is more likely behaving as a major than intermedia, and that HU is not working with him.
I'm still hoping for things to get better soon, otherwise we are going down the road of regular transfusions and then BMT will come into the picture.
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Dear Fahed,
Please try to maintain HB 9.5 to 10.I have read in this site that low HB increase iron absorption and delay the growth development...