Thalassemia Patients and Friends
Discussion Forums => Thalassemia Intermedia => Topic started by: dq on January 10, 2009, 04:24:17 PM
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Hello again...!
Hope everyone is well and in the best of spirits...?!?!
I had a small question that I’m I would love to get an answer too...
Does anyone know anything about Thalassemia Intermedia and partial seizures?
My HG is normally between 7.8 and 8.7 and I’ve developed these partial seizures when I was 19 years old.
I used to get full blown generalised seizures during sleep but those have now subsided and I only get partial ones.
The partial seizures are short lived and last about 25 seconds - I get tired after them but recovery period is usually quite short.
They happen in clusters of about 2 sometimes 3 a day every week to two weeks. So I can go without anything for about 2 weeks at a time. Sometimes even longer.
I've seen a neurologist about my issue and he is keen to get me started on Keppra which is an anti-epileptic drug as he has seen slight abnormality in one EEG.
All my previous EEG's and MRI's have been clear. My concern really is can these seizures be because of Thalassemia and low HG...?
The reason I ask is because my haematologist told me that he doesn’t really want me to take Hydroxyurea or any other form of treatment right now because I’m doing just fine.
He said these are treatments we can use should your Thalassemia get worse and begin to affect you. Presently I feel fine with the above HG and I can get on with my day just fine.
If these partial seizures are caused by the low HG, should I try the Hydroxyurea before taking Keppra?
If there are no known links between Thalassemia / low HG and partial seizures I really would like to begin Keppra to rid of these partial seizures.
I've heard that Keppra is a very effective drug and after around a year you can come off it should it control the seizures well and become seizure free.
At the same time, I don’t want to take it if the treatment is really just having to bump up my HG for example as these seizures are due to low HG or other Thalassemia related issues.......
Can anybody help....?
Many many many thanks..
Raf
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I am not the right person to answer your question so we should wait for the right people. :welcome
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Hi Raf
Actually i don't have answers to your questions but there are two important comments on your post.
First the possibility of taking hydroxyurea should be fully investigated cause it really can help in raising your HB
Second, when i was in Singapore and while talking with doctor Ali Taher from Lebonan ( American University of Bieruit), he mentioned that they are working on a study that showed that anti-epileptic drugs (didn't mention certain names) increased the levels of fetal hb in thalassemia intermedia patients leading to an increase in the total Hb
I think it will be a good idea if you can contact Doctor Ali and ask him about any relation between epilepsy and thal or there is no relation at all. He is a very helpful doctor. His email is ataher@aub.eu.lb
hope this helps
manal
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Although I have never found any article that suggests any connection between thalassemia and seizures, I have heard several reports from thals (minor and intermedia) who do have seizures. It may just be a coincidence, as a certain percentage of the population has seizures and thals would be part of this percentage, but I do believe that this needs further investigation. Regardless of the cause, you should probably try the medication. It could save you from possible injury and put your life back to normal.
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Long time ago, my elder bro who was Thal Major experienced seizure like symptoms and he was wrongly diagnosed by an experienced Neurologist to had Epilepsy :mad. Then he was given Epival for long time but that didn't solved anything. Also i have read and heard that anti-epileptic meds hut liver bad (plz discuss with your doctors).
Then suddenly i started to have seizures (conscious and unconscious) and what finally came out was the culprit was sudden drop in calcium. Since then, i am on regular calcium and Vit-D intake. This has worked for me.
But one thing i have experienced that when i got too sick (back in 2005) when i had fever and diarrhea for a month, i had 2-3 seizures and i was on calcium carbonate injections then. Also i have seen many Thal patients when going through sickness, their calcium drops.
I hope this info is of any help to you.
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Hello Andy,
My baby has thalassemia and seizure as I inform you earlier.She is now continuing phenobarbital 5 ml twice a day.Doctor advice her for continuing upto 2 yrs after her seizure.It will be complete next year May.Is it necessary to take extra calcium and Vit-D for continuing phenobarbital Please advice.Is phenobarbital reduce calcium?
I am also worried for what will be the result after stop the medicine next year.Is it not completely curable? After stop to take phenobarbital then is there any chance again seizure.Please help...
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Please reply Andy...
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Does the child still have seizures?
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Her first dose was started when she was 21 days old only and stop it after 2 months but 21 days after stopping again seizure raised then Dr. advised to continue Phenobarbital for 2 yrs 5 ml twice a day.Still the course is continuing and it will be complete on next yr May 2016.After May'2014 she have no seizure....
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It could save you from possible injury and put your life back to normal.
Hello Andy,
Taking medicine for seizure help to protect from possible injury but is it repair the previous injury? If not then how the patient will be normal in life?
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Hello Andy,
It's been a long long time mate.
I moved out of the country for a few years to work and moved back. It's been a crazy few years!
I know this thread is extremely old but I have finally had time to update it.
Basically, after numerous neurologist appointments it was simply classified epilepsy.
Keppra (Levetiracetam) wasn't the AED we tried, we decided to go with Lamictal (Lamotrogine) first.
Lamictal controlled all generalised seizures (Tonic Clonic) but did not stop the partial seizures.
As a result we tried Poly-therapy with Keppra and Lamictal in the hope that Keppra would control both so that we could come off lamotrogine however unfortunately Keppra did not stop the partial seizures so we are now in process of removing Keppra and sticking with Lamictal with the possibility of trying a new AED in a few months times.
As with all AED's changing doses takes weeks and coming of AED's takes months. So it has been a roller coaster ride.
Yikes so many things have happened over the years! Life is extremely interesting!
Anyway I will be back on the forums and posting more regularly were possible.
dq
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Welcome back Raf. I'm curious. Do you still have your spleen?
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Welcome back Raf. I'm curious. Do you still have your spleen?
Hello Andy,
yep, it's huge but I still got it.
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How big?
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22-23 cm. However, my last appointment with my haematologist said he felt (hand examination) that it was a little smaller so he's arranged for another ultrasound sometime in April to check and be sure.
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Keep your eye on the development of this drug. It's already being used in Russia according to one of our members, where it's called Jakafi. They're had great success in reducing spleen size. Dr Ali Taher is involved with the trials, leading me to believe it must have real merit. http://www.healio.com/hematology-oncology/hematology/news/online/%7B97bca27d-1532-4e8c-af6b-aa464abd37d5%7D/ruxolitinib-may-provide-alternative-treatment-for-patients-with-transfusion-dependent-thalassemia
Ruxolitinib may provide alternative treatment for patients with transfusion-dependent thalassemia
March 17, 2017
SAN DIEGO — Ruxolitinib demonstrated an improvement in transfused red blood cell volume during a 24-week treatment period compared with baseline in patients with transfusion-dependent thalassemia and splenomegaly, according to phase 2a results from the TRUTH study presented at the ASH Annual Meeting and Exposition.
Ruxolitinib also demonstrated a reduction of spleen volume in almost all study participants, according to the findings.
Ali Taher
Splenomegaly tends to worsen anemia in patients with transfusion-dependent thalassemia, which can then lead to an increase in the need for transfusions.
“The consistent reduction in spleen size from baseline over time observed in the study patients echoes similar results reported in phase 3 trials recruiting patients with myelofibrosis and polycythemia vera,” Ali Taher, MD, deputy director for academic and research affairs at the Naef K. Basile Cancer Institute at the American University of Beirut Medical Center, told HemOnc Today.
Taher and colleagues conducted a single-arm, multicenter study of 30 adults (median age, 24 years) with thalassemia and splenomegaly who received transfusions every 4 weeks.
The primary endpoint, as Taher noted, was to determine if there was a reduction in transfusion burden with the use of ruxolitinib (Jakafi, Incyte) compared with baseline – which was defined as the period between 24 weeks before the start of treatment and week 0.
The researchers also sought to determine if there was any degree of spleen volume reduction from baseline.
Patients received a starting dose of 10 mg twice-daily and were required to receive iron chelation for at least 4 weeks prior to screening and throughout the study.
Twenty-six patients completed the core phase at week 30 and 20 of those patients continued to receive ruxolitinib beyond the core study period via other mechanisms.
Mean hematocrit adjusted volume of transfused red blood cells per 4 weeks was 605 mL during the baseline period. The adjusted volume of transfused red blood cells decreased to 560 mL between weeks 6 and 30 after treatment with ruxolitinib. Mean percent change of transfusion rate was –5.9%.
The most common adverse events that occurred during the study were upper respiratory tract infection (27%), nausea (20%), upper abdominal pain (17%), anemia (17%), diarrhea (17%) and weight gain (17%).
Eleven patients reported experiencing grade 3 or 4 adverse events that were not believed to be associated with the use of ruxolitinib.
Thirteen patients experienced adverse events that were believed to be associated with the study drug. Three of those patients reported serious adverse events.
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In my case Sezuires / Epilepsy caused by low hb. I'm taking treatment since 2008 for Sezuires / Epilepsy. Right now I'm taking Phenobarbitone 60 mg in day and 120 mg in night, Clobazam 10 mg in night, Flunarizine 10 mg in night and Levetiracetam 750 mg twice a day . My Neurologist told me that if i stop my medicine it can happen again. So do as your Neurologist say.
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Hi Andy,
Yes my haematologist mentioned something about that drug!!
....but I think he said it can only be used on transfusion dependent patients?
Is this the case or can we independent patients try it?
Thanks a million Andy!
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I would say that will depend on the doctor. Some minors have spleens large enough to have splenectomy considered, including you, so I cannot see why it should be treated any differently. It does create a vicious cycle that everyone would be better off not having. The more the spleen destroys red blood cells, the more overactive the bone marrow becomes. I think some doctors will see this drug as a better option than splenectomy for minors.
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Hello Andy,
I saw my haematologist again last week and raised JakaIf with hi again and he reiterated that it's only to be used in transfusion dependant thalassemics. As I have intermedia and do not have transfusions it won't really work. My haemoglobin runs between 7-8 but recently it's dropped and were investigating why. It could be that it was the allopurinol I was taking for gout suppressing the bone marrow as its metabolised in the kidney were EPO is created to stimulate blood production. We will get a clearer picture towards mid-July if things improve as I've been off allopurinol for about 2 weeks and I believe it could take weeks for haemoglobin to recover if it does?
He did mention a new drug in phase 3 trials for non transfusion dependant thalassemics but I can't remember the specifics. I think it's available somewhere on the UK thalasemmia society website in one of their publications.
Do you have any information about these new drugs?
Thanks mate.
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The drug is Luspatercept and the phase 3 trials are now full and underway. Phase 2 trials had very good results. Patients saw good increases in Hb, lower iron loads and reduced in effective erythropoiesis. This would be of great benefit to non-transfusing patients, as well as transfusion dependent patients.
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The drug is Luspatercept and the phase 3 trials are now full and underway. Phase 2 trials had very good results. Patients saw good increases in Hb, lower iron loads and reduced in effective erythropoiesis. This would be of great benefit to non-transfusing patients, as well as transfusion dependent patients.
Thanks for that mate.
Do you know when these trials finish?
P.s. How is the drug administered?
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It's administered via subcutaneous injection, afaik. Someone wrote it might be on the market in the next 2-3 years (?), IIRC.
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Luspatercept is administered by IV. Phase 3 trials are now full and the trials are underway in several countries. I think we should have full results within two years. It will not take long for approval once the trials are complete, as long as no unforeseen problems arise. None have during phase 1 & 2.
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I'm not sure if it can be administered by IV, but in the study I think they administered it via subcutaneous injection, which, in my opinion, is the least painful or difficult administration with a syringe. I guess it could be injected into the belly fat, for example.
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Yeah, that is right. It was done through subq injections, like with desferal.
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Ah ok. Just the one injection or is it a course of injections?
p.s. Spleen measured again last week and is around 23cm.
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It's ongoing, so the injections would have to be done regularly.