Thalassemia & Strokes

Has anyone experience stroke like episodes?

I have had 2. The first time was when I was 16, I had paralysis on the left side that only affected my limbs, same thing happen again when I was 18. Once again it only affected my limbs (arm & leg). Only difference was it wasn't as bad and it affected the opposite side. I am now a paraplegic and Nobody knows why, Any ideas?

read more about it at http://ariadnesjourney.weebly.com/2-years-ago-on-this-day---march-8-2011.html

Hi Ariadne,

As though thal major isn't enough. I just read your bio. Something I see no mention of is whether you have ever been seen at any of the thalassemia centers of excellence in the US. The closest one to you would be in Chicago. Dr Thompson is a great doctor. If you aren't already going to a center for an annual evaluation, you should start. There is absolutely no comparison between the thal centers and anywhere else. Considering your various problems, it would definitely help to be in the comprehensive program used at the centers. I believe it would be beneficial for your health issues to be approached in the context of thalassemia.
Children’s Memorial Hospital
Division of Hematology/Oncology
2300 Children’s Plaza, Box #30
Chicago, Illinois 60614
Phone: (773) 880-4618
Fax: (773) 880-3223

In recent years research has shown that splenectomy in thals should not be done routinely as had been the case in the past. This is due largely because it has been shown that removing the spleen adds to the hypercoagulable state in thalassemia, leading to a higher incidence of clotting in splenectomized patients and some have observed a higher incidence of stroke in these patients.
I also want to point out this link as the symptoms sound similar to what you have reported. http://jcn.sagepub.com/content/21/1/75.abstract

Moyamoya Syndrome in a Splenectomized Patient With β-Thalassemia Intermedia. We describe a 14-year-old Japanese girl with β-thalassemia intermedia who developed moyamoya syndrome after splenectomy. This patient had compound heterozygous mutations of the β-globin gene and received occasional transfusions. After splenectomy at 12 years of age, she transiently required partial exchange transfusions for leukoerythroblastosis but attained transfusion independence. Two years after the splenectomy, transient ischemic attacks occurred repeatedly with right hemiparesis or left paresthesia. Magnetic resonance imaging revealed bilateral stenosis of the internal carotid arteries and dilatation of the perforating branches with the formation of moyamoya vessels but not infarctions. The strict adherence to aspirin and dipyridamole has led to no stroke or progression of the vasculopathy for 8 years. Moyamoya disease has been reported in a patient with β-thalassemia major. Cerebral vasculopathy can be a rare but grave consequence of the thromboembolic complications in β-thalassemia major/intermedia.

I can't say that this is what has happened to you, but it does deserve investigation, as Moyamoya Syndrome can lead to paralysis on one side of the body.

How well is your thalassemia managed? What is your normal pre-transfusion Hb and what is your ferritin level? Which chelator are you using? Have you had any liver or heart scans?

Hi Andy,
Yes, I have been to a Thalassemia Centers of Excellence. When I was really little; I used to go to CHOP once a year and see Dr. Alan R. Cohen, MD , (GREAT DOCOTOR!) but then he stopped seeing a lot of patients or something.  I don’t really remember the full details why though. I saw him since I was 3 -8 years old.  Then for about 3 years I didn’t go any were until the Thalassemia Centers of Excellence came out. Throughout middle school and high school I saw Dr. Thompson at Children’s Memorial Hospital in Chicago. I can honestly say I wasn’t very impressed with their thalassemia center. I felt like they were too pushy to start Exjade; Exjade at that time was just coming out of the experimental phase. So I wasn’t very willing to try it, because I know my body acts differently than others and the side effects were basically the same as Desferal, which I am currently on 7nights a week for 12hrs. I am also allergic to Desferal, and have been hospitalized a couple of times to desentazation or something like that, but I have been doing great since they mixed hydrocotzoin to my desferal. Also another reason was when I had the stroke like episode for 2 years they kept ignoring the fact that I had one, because they probably don’t know why I add one, just like everyone else. They were also really pushy about wanting me to be on birth control. Which I refuse to go on because, obviously birth control can cause strokes. I rather figure out why and were these stroke episodes are coming from; my endocoronoligst here my hometown agrees with me on this. Also Chicago was really pushy and way into the fact of fertility for me. Which I can say I don’t care if I have kids or not. My kind of kids would be four legged and hairy instead, because I AM A MAJOR DOG LOVER!...lol
I have currently switched to a center that is not yet a Thalassemia Centers of Excellence, but they will most likely be one in the future. I see Dr. Lakshmanan  Krishnamurti, MD (I AM REALLY IMPRESSED WITH HIM!) at Children’s Hospital of Pittsburgh, PA and I see the head cardiologist out there due to fact was having sinus tachycardia. My MRI (T2* & R4*) showed I had a lot of Iron my heart, but my liver looked good. My ferritin levels are in 1,100. My pre-transfusion hemoglobin ranges from 8.5-9.7. I get transfused every 3 weeks no matter what my hemoglobin is. My mom tries to keep my hemoglobin close to 10, other wise I get sever back pain, headaches, and nose bleeds. If my hemoglobin is under 9 I get 2 units of washed packed red blood cells.
As for splenectomy I had to have it because I was on the experimental Buytrate trail in Boston, MA; Year 2000, when I was 8years old with Dr. Susan P. Perrine. I don’t believe I have Moyamoya Syndrome because my MRI’s show no trace of narrowing of the blood vessels. However my MRI’s dose show a brain lichen that I hade for years then all of a sudden it disappeared an new one formed on opposite side of the frontal lobe. So someone thought I might have night time night time seizures. I do have neuro-sleep arena and I have read somewhere that neuro-sleep apnea can be miss diagnosed for sleep apnea. I am on a bipap and a had a somewhat resent sleep study that showed my setting are right for me yet my mom. Has witness me thrashing around violently in my sleep many times and I have shared bed with little sister at a Ronald McDonald House and when we both got up in the morning she was way at the end of the bed and had her arms crossed like she was in a coffin and she had a bloody nose. My mom is pretty sure I wacked her in my sleep because in the morning she kept saying “I don’t want to sleep with Ariadne.” I also a apparently have some “staring spells”she has seen. Another thing my MRA showed was thing of the Myline, but everbody just say its due to my thalassemia. Do you know other thalassemics with thinning of the myline? Because someone mentioned to us transverse myelitis before, so my mom looked it up and I have a lot of those symptoms.

I am actually a big fan of Dr Krishnamurti. I really like his patient-centered approach. How low was your T2* score? Have you ever tried Ferriprox? It would probably clean out the heart iron better than desferal is doing and was recently approved by the FDA. Combination desferal and Ferriprox is the most effective chelation method currently available. Do you know what your specific beta thal mutations are? I'm curious if there has been anything unusual observed with the mutations that might indicate thal is affecting other conditions.

I can't find much on a connection between thal and Transverse Myelitis, but this was brought up previously by a thal minor who also has TM. You can the post at http://www.thalassemiapatientsandfriends.com/index.php/topic,3262.msg33038.html#msg33038

I don't know what my score was on the T2* but Dr, Krishnamurti recommended increasing my desferal from 600 to 800 and then have the MRI repeated in 6months, but my mom explained to him that we can't just jump up to that high of a dose Because of allergic reactions, so instead every month I go up 50 and once I reach 800 I will stay on that until I go back in a year for a repeat MRI. Then we will just have to wait and see what the next MRI shows and what he would recommend.

I have heard of Ferriprox, but nobody has ever recommended it for me. All I know is that I have mutation on chromosome 11 and I also have another chromosome defect on number 12 which gave me SCADD. I m also will be tested for more chromosome defects that involve mitochondrial & metabolic disorders.

Very sorry to hear about your strokes - as Andy says, as if Thal is not enough.

May I also add that you should talk to your doctor about taking aspirin regularly - as a blood thinner - or equivalent.

This is in conjunction with your other post on High WBC counts - I am fairly sure now that your platelet counts would also be high.

Any with their spleen out and high WBC/platelet counts should monitor the coagulation factor/time of their blood - also, thrombosis is a possibility if you take long flights or long car rides without moving about during the entire journey.

Wish you all the very best,

Poirot

That should be Deep Vein Thrombosis (DVT).

I've had a few strokes as well, my first one when I was about 16. I too had my spleen removed at the age of 13, coincidence?

My left side was completely numb, doctors still don't know what caused it...

I was put on Warfarin but the constant blood tests were too much for me and now I'm merely on Aspirin.