Thalassemia Patients and Friends
Discussion Forums => Miscellaneous Questions => Topic started by: aysam on October 22, 2008, 12:17:34 AM
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AYSAM MAZEH
BLOOD TEST RESULTS
HEIGHT 89
WEIGHT 130 800
BODY MASS INDEX 17.4
BODY SURFACE AREA 0.58
LIVER FUNCTION TEST
bilirubin total 5
bilirubin (conjugated) 2
aspartate txaminase 80
alanine txaminase 134
gamma-glutxferase 16
alkaline phosphastase 165
ca/mg/phos
calcium 2.48
MAGNESUIM 0.76
phosphours 1.81
FBC
HAEMOGLOBIN 100
WCC 19.3
PLATELETS 385
HCT 0.29
MCV 79.0
MCH 27.0
T&b
LYMPHOCYTE COUNT 9.20
CD3 T CELLS 5.15*
% CD3 T CELLS 56
CD4 T HELPER SUBSET 3.22
% CD4 T HELPER SUBSET 35
CD8T SUPPRESSOR SUBSET 1.75
%CD8 T SUPPRESSOR SUBSET 19
CD4: CD8 T CELLS 1.84
CD 16/56 NATURAL KILLER CELLS 0.46
%CD 16 56 NATURAL KILLER CELLS 5
CD 19 B CELLS 3.56
% CD 19 B CELLS 39
ATYPICAL LYMPH 0.4
BLOOD FILM
NORMOBLASTS / 100 WBC 4
TOXIC GRANULATION +
ANISOCYTOSIS +
POIKILOCYTES +
POLYCHROMASIA +
OVALOCYTES
ROULEAUX +
FRAGMENTED CELLS +
TARGET CELLS
COAGULATION-PLASMA
PLASMA PROTHROMBIN TIME 14.1
INR 1.0
APTT 30.7 *
FIBRINOGEN 2.7
IRON STUDIES
FERRITN 3723 DONE 13 AUG 08
BLOOD TRANSFUSED 14 AUGUST 08 SO MUCH HIGHER NOW
SEND AWAY FERRITN LEVELS 1995
GLUCOSE 4.9*
ALBUMIN 50
GLOBULIN 25
A/G RATIO 2.00
ANTI TTG ANTIBODY 2*
ANTI TTG ANTIBODY INTERPREATION TTG ANTIBODY
VITAMIN C 57
CAN ANYONE TELL ME ANYTHING MOST OF MY SONS RESULTS HAVE A (H ) BESIDE THEM WICH MEENS HIGH
AND THEY TELL ME NOT TO WORRY HE WILL BE FINE :mad ??? CAN ANYONE TELL ME WHAT ALLL THESES MEEN IS MY SON IN DANGER PLEASE :banghead
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Mariam,
I have sent a copy of his results to a thalassemia doctor in the US. Please be patient as I await his response. I wish I could give you more, but I am not sure what to tell you yet.
I will respond as sooon as I get a response,
Sharmin
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Mariam,
The things that stand out to me are the high liver values (ALT & AST) and the low mineral levels (calcium etc.) The liver values may be a result of his high ferritin and it needs to be addressed. The higher Exjade dose may get the iron to level off but probably isn't high enough to cause much of a drop. Has Asyam ever had desferal administered by IV? I would like to know if he has a bad reaction when taken IV, rather than subcutaneously in his abdomen. If he can tolerate desferal through IV, it can be used to get his ferritin down. Sometimes patients are given IV desferal 24/7 for as long as 7-14 days and it causes a drastic reduction in desferal. However, patients using this method usually have much higher ferritin levels, so the doctors may not want to choose this method but rather try the Exjade route first. Dr. Wood said to go to 250 mg (20 mg/kg) and then go up in 5 mg/kg increments at a time until 30mg/kg is reached if necessary. One thing is, you have to have some patience. The optimum dose has to be gradually reached and it will take time to find what the correct dose will be. Serum creatinine levels need to be regularly monitored.
Does Asyam eat well? Is he a picky eater? The mineral levels are low and this can be largely corrected by diet. I know it isn't easy with kids but he needs to eat fruit and veggies and minimize the empty calories kids love so much. If it's a real problem getting him to eat well, a nutritionist may be able to give you some ideas. Thalassemia doesn't leave much room for error so diet becomes even more important than with non-thal kids. Celiac disease was mentioned and this can also deplete minerals and other nutrients. Has he shown any reaction to products containing wheat? Bloating, diarrhea or stomach discomfort? If this is any possibility it should be checked out.
None of his test results are so high that there should be concern of any immediate danger. They do show that there are some issues that need to be addressed, primarily his high ferritin and low minerals. The higher dose of Exjade should keep things level or possibly decreased and a higher dose should be eventually used as long as he tolerates it and his iron stays high. The nutritional problem also should be addressed. Is it his diet or a problem that is depleting nutrients? This must also be answered. Things can turn around with some work and at his age, it will prevent damage from happening as long as these problems are addressed. Ferritin over 3000 is high but at his age it is not the same as one who has had a ferritin over 3000 for 20 years or more. It needs to come down but it poses no immediate danger.
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OK ANDY THANK YOU YES HE EATS EVERUTHING AND HE LOOKS VERY WELL
HIS FERRITIN IS ACTULLY 4000 I FOUND OUT TODAY SO NOT GOOD
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Andy,
is the random glucose value of concern? do these tests any kidney related problems?
Also Andy, a ferritin of 4000 for a two year old child only transfused 20 times? A child who is chelating while many kids don't even chelate at that age? What is causing the high ferritins? has a ferriscan or squid been done? These questions have been puzzling me all day which is why I did not know exactly how to respond.
Sharmin
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ANDY WHEN YOU SAY HIGH LIVER TEST HOW HIGH YOU MEEN SHARMIN I REALLY AM SO SORRY I HAVE MADE YOU SO CONFUSSED ALL DAY BUT YOU COULD BE RIGH MY SON MAY HAVE SOME OTHER KIND OF PROBLEMS BECUSE HSI WHITE CELLS ARE ALWAYS HIGH ALWYS ABOVE 15 THEY GO TO 30 WICH IS TO HIGH
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Mariam,
Don't worry Mariam, your child's well being is worth the research and confusion. I don't mean to indicate that your child's liver iron tests indicate that he is in any danger. It is just that it would be unlikely that his ferritin would be that high from transfusions alone especially because he began chelation so early. Perhaps there is another factor leading to the increasing ferritin, and perhaps you need to look into whether he has celiac or some digestive issue. None of this indicates that your child is in immediate danger - your focus should be chelation and supplementing. Could you please tell me his transfusion frequency and volume - if this is high then it would explain the high ferritin otherwise we are back to square one.
Inflammation - can also give elevated ferritin levels as well as some of the other results that you are concerned about - and this may or may not be related to celiac (I am guessing).
Don't worry, just try to get answers and tests from your doctor. Can you get ferriscan or squid there? that would help us greatly in determining if his iron is actually high or if the ferritin is elevated due to some other cause - the blood tx volume, frequency will also help. BTW Mariam - this doesn't have to be urgent - just things to keep in mind when you visit the doctor.
thanks Mariam,
Sharmin
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HE GETS 220 ML BLOOD EVERY FOR WEEKS WHAT IS celiac HOW DO I DO THE TEST
I TOLD DOCTOR TODAY THAT I WANT A FRERRI SCAN DONE SHE SAID SHE WNATS TO GET LIVER BIOPSY
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You can view the following sites for more info about celiac disease - but note this is just for your knowledge of what the disease is - I am not suggesting that Aysam necessarily has it.
http://digestive.niddk.nih.gov/ddiseases/pubs/celiac/
http://www.celiac.org/cd-main.php
The transfusions you describe are NOT LIKELY to cause the ferritin levels that Aysam presents with. That is why it thought that 2 scenarios are possible - 1. the high ferritin reflects some inflammatory condition rather than tranfusion related overload 2. something else, besides the transfusions, is causing iron overload in the body.
the liver biopsy or ferriscan will give us this information - whether ferritin correlates with actual liver iron concentration. My son has had to liver biopsies and he did well. However, you need to be sure that they are being performed at an experienced center.
testing for things like celiac can also give us information - modifying his diet can alleviate this - as with celiac children often don't tolerate foods containing gluten or wheat.
I hope this helps a little Mariam,
Sharmin
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Ferriscan and MRI are very unlikely to be used at his age because it is not easy to get a child to lie still long enough to do the scan. The ferritin does seem to be too high to explain as caused by transfusion alone. If there has been some infection it could explain a lot. If he eats a lot of iron foods it may also contribute, since his Hb has been allowed to drop fairly low before transfusions. I would also like to know if there is any sign that hemolysis is taking place which would lead to low Hb. Do you have results for reticulyte count? I think if celiac was present, he wouldn't enjoy eating and would have complaints afterward. If his stomach doesn't bloat and he doesn't complain of pain after having breads or cereals, celiac isn't likely. Does he ever complain after eating?
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sharmin yes they told me about this when he had the test of his bowls blood in them but they also said they were normal he hasnt got celic
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that's good to know. i do wonder why he bloats so much - was that related to his chelation perhaps?
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ANDY NO
he never complains after eating and eats almost everything and anything good in his eating and rinks plenty of jusices apple orange and water so no problem there ANDY can you eplaine one thing fron the day he was born he has had high white cells always above 18 reachase sometimes 30 why ? what could this be once they said they toght he had cancer but one doc said he thinks aysam is just like his his white cells arealwys high
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you know i think it is related to exjade or desfral i think thats the bloating is ot a concern as much as teh feritin is 4000 for his age his weght is more a concern and he liver im more worried about dont u agree
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Mariam,
Absolutely, his ferritin levels and liver function are most important. I was just trying to determine if there is an alternate explanation for his elevated ferritin (such as inflammation) - other than transfusions. But as Andy said, since his hg was allowed to go quite low between transfusions perhaps he has absorbed more iron through the gut then he would if his pre transfusion hemoglobin was kept between 90 - 100.
I would stick with water or milk for drinks - juice can cause elevations in glucose levels, which in turn lead to an increase in insulin levels to deal with the high glucose - which can lead to a tolerance to insulin - resulting in diabetes. Some juice is okay - just not a lot :wink
Sharmin
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sharmin
white cells ?
why with my son they have been so high all his short llife
over 18 always i know when u have a viral and infections it tend to go up but with aysam its never been at a normal range
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Mariam,
Sorry I can't explain. I was trying to draw everything together, but back to the drawing board.....I'll let you know what else I can come up with. The doc hasn't gotten back to me yet.
Sharmin
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this is why i thought that an autoimmune issue may be present, causing inflammation, high ferritin and elevated white cells.
http://www.wrongdiagnosis.com/symptoms/high_white_cell_count/causes.htm
List of 7 causes of High white cell count
This section shows a full list of all the diseases and conditions listed as a possible cause of High white cell count in our database from various sources.
Acute inflammation
Bacterial infection
Chronic myelomonocytic leukemia - high white cell count
Fungal infection
Physiological
Pregnancy
Viral infection
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The high white cells may also point to hemolysis. http://www.bloodindex.com/leukocytosis.php
Among the causes of neutrophilia are...hemolysis (destruction of blood cells)...and hematologic abnormalities.
The low Hb before transfusion also suggests hemolysis as does the possibility of antigen involvement in the ANTI TTG ANTIBODY result. Is there a possibility that he has had an alloantibody reaction to the transfused blood? It would explain a lot.
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Could the antiTTG play a role in any of this?
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Thanks Sharmin. I had actually copied and pasted the wrong line into my post. I meant ANTI TTG ANTIBODY. It all does seem to point towards infection or hemolysis or both.
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i dont know really he never had areaction if it is a viral or inflamatry he would not have it all his life would he ?
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ut can i add he has had many herpes before
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i want to thank everyone for your replys really
i love you guys its like aysam is part of all your famaliy
i have noticed he is actully pale not yellow and dark circules around his eyes you know when you dont get enough sleep like that
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Aysam,
Antibody related inflammation is something he would have all of his life - or as long as he has had the antibody. Antibodies are something his body can chronically produce - in this case it is autoimmune antibody - which may or may not be responsible for some inflammation or chronic high levels of white blood cells.
hemolysis would explain the elevated bilirubin too, right? My son did not have high WBC with hemolysis - but that does not have to be the case for everyone.
I think that elevation of WBC in herpes would be associated with an outbreak, the counts would not be elevated all of the time. Every bit of info helps though!!
Sharmin
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Mariam,
:hugfriend :hugfriend
Is he due for a transfusion soon?
See how he looks in a day or so.
Sharmin
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no he isnt due he only was transfussed one week ago not even so no but he isreally white werid i am so cinfussed and excusted and tierd of this and he is only two i stillhave somany years to battle you knowmaye aysam when he gets older he willl get better
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you know nearly everyone here has or knows or famaliy memmber with thal it seemsim the only one who hasnt got good medical help and goodi nformation and good treatment even thou i am suppose to be in one of the best countrys
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Subject: RE: about my son please read urgent
Date: Fri, 2 Nov 2007 15:29:27 +0000
Hello, Mariam, I will try to answer your questions.
First of all, I think that you should follow the advices of Lebanon doctor. They are absolutely right, and I fully agree with her.
I try to answer your questions, as below:
“when can we start using the iron overload machine pump what levels of feritin:”
after 18 months from first tx and when about no more then 18-20 tx are done. Give at least 30-40 mg/kg/day of desferal for at least 5 days per week.
“if the blood goes to 9 is this to low”:
it is better to maintain pre-transfusional HB between 9 and 10, that is to transfuse as soon you find she has HB below 10.
“why is his heart beating to fats is this related to thalassemia”:
Yes, I think it is related to anemia and nose occlusion. So, if you transfuse you may improve the heart beat.
“what other problems can my son face with this chronic illness”:
virus hepatitis from blood born viruses if blood is not adequately screened for virus and also you may get immunization if blood is not well screened for ABO subgroups (CcEeDd, Kelly Cellano antigens on red cells, and you should use filtered blood for white cells (or done before, or at the moment of transfusion using appropriate filters)
“is there anything we can do here to help him more:”
follow this rules for transfusion and for iron chelation and he can be stabilized in better conditions, to afford alternative donor transplant (as MUD donor or Mother HLA mismatched donor)
“is there anything you can do to help my son get cured and what are the percentage in succeeding as the doctors here told me that there is a 5 % chance if he was to have a match with his sitters and if not 2% chance:”
It is not true; you will have a 90% chance to be cured in case of both a matched sibling and a good clinical conditions (class 1 of risk). In case of no donor available if he is in good condition and he can find a matched unrelated donor (MUD) he can have the same percentage of success as with a matched sibling. With the mother HLA haploidentical (only partially identical) we have we have about 60-65% chance of cure but still we face a rejection rate of about 35-40%.
As you probably know I am taking care of bone marrow transplantation in Italy Rome, I am clinical responsible of IME transplant center. I am not directly involved in the conventional treatment of thalassemia, that is done in the country centers. However, we take care of patients that arrive to Rome with a program of transplantation. Who gave you my name? how did you get in contact with me? I wait for your answer..
Paola Polchi, M.D.
IME Centre Rome, Italy
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Dear Mariam,
Sorry because you could not understand… I never use to read back, when I write..
‘Red pilot’ !!!, he phoned me today.. his name is Khalifa, he is father of a child we transplanted, is a good friend of me and is always trying to help people in the thalassemia families. So, I understand, now.
1st question:
About Ferritin 1000 or 18 tx? I will follow both, which is first. Ferritin is not always reflecting iron but also a phlogistic process, as tonsils and noose.. Because iron enters with the transfusions given I think that the amount of blood given is the more precise marker to know how much iron he received already. Starting earlier you may give desferal only 2-3 times per week, but after 18 months approximately from first Tx if you give about 1 tx/month you should give regular desferal. This is because there is a calculation, for that I can extrapolate the expected liver iron concentration to be about 6 mg/ g of liver. In case of lower levels of iron concentration, desferal may be more toxic.
2nd question:
About hemopoietic stem cell transplant:
Stem cell transplant is made to correct the genetic defect that produces anemia in the thalassemia patients. We documented that the possibility of success of the transplant is different depending on the HLA compatibility and on the clinical conditions pre-transplant. This is what we call the class of risk. It is statistically identified, and derives from history of iron chelation (regular or irregular); hepatomegaly and presence or not of fibrosis in the hepatic portal spaces (this is seen in the pre-transplant liver biopsy).
it is always STEM CELL, we can take them from marrow, from peripheral blood or from other sources, as cord blood or as fetal liver and so on…. We chose the source based on the clinical need: when it is a standard transplant, from an HLA matched sibling the marrow is the best source, because it gives enough cells, and low GVHD, that is the graft versus host disease, an immunological reaction of the new marrow to the recipient. When we use a donor that is not matched, as the mother, we need more amount of stem cell to bypass the resistance and we need to manipulate them to eliminate lymphocytes, so we need much more cells. Then we take both, peripheral blood stem cells and marrow stem cells, so we can obtain more cells and of good quality to reach success. If there is an HLA identical donor this is the preferred donor. So, I suggest to look for an unrelated donor, in the bank. If no donor is available, then you will have the mother as alternative,(unless you deliver new child, potentially identical donor!!)
When I say HLA mismatched, I mean partially matched: always a child get half genetic heredity from mother and half from father. Only rarely it happens that by chance mother or father come out HLA identical (when mother and father share some thing between them two).
So normally the mother is identical to her chid fro those antigens that she transmitted directly and is different from the half transmitted from the father to the child. The mother has an advantage as donor, because during pregnancy there is a tolerance of the mother for those antigens, paternal, that she did not transmit to her child, and in the other side, the child became tolerant to the non inherited maternal antigens. Those are the background of using mother. She is better, but also a sibling that is mismatched for the non inherited maternal antigens may be used. Generally it is a child so not enough cells can be obtained from him. This is way we prefer the mother.
We transplanted 17 patients (of them 12 aged between 2 and 8 yo) from HLA haploidentical mother (haplo means 1 haplotype is identical and one haplotype is not). Of those transplanted, 15 are alive and 2 died (they were cured from thalassemia when they died). ) 11 patients had engrafted marrow, but only 9 are alive and without thalassemia, and 7 patients are alive and back to thalassemia. This is what I mean when I say about probability of 65% to be cured, 35-40% rejection, 12% of death.
I think that your child is in class 1 (regular chelation, no hepatomegaly, no fibrosis expected) at the onset of the transfusional history. He has hepatomegaly now, but this is going to improve with adequate transfusion regimen, suppressing his expanded erythropoiesis. Therefore, he does not have organ damage due to iron accumulation and to inadequate transfusions, and also he has low risk to develop comlications due to the good standard of therapy in your country. This is what I mean when I say in good clinical conditions. He is in class 1 but has no donor. Then, I think that you have time. So, you can wait for further improves in the transplant success. In any case, even a child in class 1 with an HLA identical sibling may die for transplant related complications (about 5%, that is 5 out of 100 children in class 1 may die as consequence of transplant). In case of not HLA identical donor, as I told you, about 10-12%, 12 out of 100, may die as consequence of transplant. This is low compared to other diseases, but still is a risk, so I can not tell you that risk is 0.
I fully understand your concern, probably you should look in the bank of MUD donors or wait (until he is 4-5 years old at least, may be), proceeding with adequate treatment. In any case if you are willing, you may apply for stem cell transplant officially in our institution (IME) and you will receive a program and presumed cost. The timing of every thing is complex, requiring before the transplant itself, a period of about 3 months for clinical investigation on donor and recipient, for harvesting and preparing the stem cells from the donor and giving some oral chemotherapy to the child. Only after this you may proceed to the transplant that may require at least 4 month after and sometime longer. So totally, is about 7-9 months at least.
To arrive in Rome, you may be supported by your ministry of health (so, ask for support, I do not know how) or by yourself that is costing about 120 thousands Euro, also the staying in Rome may require either or to rent an apartment, or to ask for help by an association of volunteers that help families during the stay in Rome, in a community manner.
Do not be hurry, take your time. New improvements are expected continuously..
Ciao,
Dr. Paola Polchi
IME Rome, Italy
p.polchi@fondazioneime.org
mobile phone: 0039 3384467829
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Mariam,
Please see Smurfette's post:
http://www.thalassemiapatientsandfriends.com/index.php?topic=2032.135
This young lady has done very well in Australia - she is offering you some great advice and direction which I believe will help our little guy very much.
Sharmin
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Dear Mariam
I read Dr. Paola reply to you and thanks for sharing because it is very informative but i can see that you are now distracting you attentions in many directions. Thinking of a bone marrrow is okay but from my point of view is that you should now concentrate on two main things
1- Removing the iron overload before it damges any organ. this will not be achieved unless you give the right dose for your child according to his weight or else you are giving him medicaton with no use. So as Andy said increase the dose slowly and gradually until it is suitable for him especially that he is on regular transfusion.( it doesn't make any sense giving chelators without a suitable dose)
2- Try to find out what is the cause of the increased white blood cells. This will answer many questions including the abnormal increase in ferritn
I know that your special circumstances and being introduced to many thins at one time could confuse, depress and annoy you and at the end the target will not be achieved. So lets togather set these goals and work on them.
I think i will be able to get you Dr. ali Taher's phone number and please read Smurfette post as Sharmin told you cause she will be of great help to you
Good luck :hugfriend
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Mariam,
Is Asyam getting filtered blood and does he have herpes infections often?
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I'm sorry for your child.
My suggestions may be out of the way, but though I want to share my thoughts.
1) Is he diagnosed with thalassemia?
2) Is his ferritine tested more times?
3) What about neonatal hemochromatose? The results looks like this, I believe.
4) i dont have thal but, pyruvate kinase deficiency, and we also collect iron from our food. A lot of persons don't agree with this idea, but there are results of that and I really believe in this.
5) How old is your son?
6) Infections can raise his ferritine level.
Take care,
Dore
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To all the good people here,
I have spoken to Miriam today and suggested she get a second oppinion. From what she told me was that she has an appointment with a specialised thal haematologist at the hospital that I go to and its also for children as well...
I am sure that the doctor will do all the necessary tests that need to be done and she will get answers I am certain of that..
Its just that hospital that she takes her son to, isnt equiped with doctors who know about thal and dont understand...If I ever happen to meet one of those doctors I am sure that I would probably give him a piece of my mind..
Mariam you will be taken care of at the Sydneys Childrens Hospital.. I am sure of that... Dr Sue Russell is one of the best..
Hope your all well..
Peartree Girl I can answer all those questions for you..
Yes
Yes
Yes and more yes
yes we know that infections can raise our ferritins.. please read other posts..
Thank you
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Thanks Smurfette for your great help. We all appreciate it so much and i am sure now that Mariam is in good hands.
manal
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yes, I did read the whole topic now.
a) Too much Vit C has a bad effect on chelation therapy
b) regular transfusions, regular chelation (never stop immediately, only by too worse reactions).
My personal experiences: I'm allergic for all chelations meds. Kelfer/Deferiprone/L1 gave me swollen knees and because of that I was unable to walk good enough. And I couldn't stop eat. I was always hunger. All the time; day and night.... I got a reserve for my try of Exjade ;)
desferal: deadly allergic: swollen. red. itchinng. If you say he bloats, will that say swollen?
Exjade: I'd all side effects. sick, sleepy all the time, dizzy and black for my eyes. I'm give it a 4th try at the moment. Every 2weeks I take an other 125mg tablet.
c) I know it's vey difficult for a child, but are this tests done fasting? That will give (more) regular results.
I hope the second opinion will go well and that they find soon what your son has.
Take care,
Dore
p.s. my english is a bit bad at the moment :$
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Hey Mariam
How are you doing, hope Aysam is doing well
Please update us about his situation
Wish you all the best
manal
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I hope little Aysam is doing fine Mariam,please update us.
Zaini.
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Hi Mariam,
I hope that Aysam is doing well - please update us on his treatment.
Sharmin
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Hello,
Hoping that Aysam is doing well.
Take care.
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Hi Mariam ,
How is Aysam doing ??.. ,i hope & pray that he will b fine , pleas keep updating us to his health progress , it will give us the feel satisfication to us, that Cute boy is being treated well ... it will realy nice to hear abt his healt & himself . :goodluck lots wishes and :flowers for Aysam ..
Best Regards
Take Care
Umair