Thalassemia Patients and Friends
Discussion Forums => Thalassemia Intermedia => Topic started by: Keep Smiling on November 15, 2009, 05:17:13 PM
-
what is the difference between homozygous beta thalassemia and hetrozygous beta thalassemia?
because najaf has got homozygous one..........
-
homozygous beta thalassemia is the beta thalassemia major, while heterozygous - I think- is the thal minor, when one is just a carrier. I am sure for the homozygous because that's mine, for heterozygous I am not sure 100%. We'll need some confirmation from others.
Lena.
-
Lena has it correct. Heterozygous beta thalassemia is thal minor, while homozygous is major. Hetero means different and homo means the same. When two genes are both beta thal, it leads to major.
-
you said "When two genes are both beta thal, it leads to major."
so what is the case with thal intermedia????? then intermedia is caused by one one beta thal gene?????
also najaf has not got the symptoms of beta major. in four years she has been trasnfused only three times... her genotype is B+/B+
-
Thalassemia intermedia can occur in several cases. One case is have homozygous beta thal and heterozygous alpha thal - where the alpha thal trait decreases the severity of the thalassemia. Another case is where the patient has homozygous beta thal, however they produce high quantities of fetal hemoglobin to compensate for the lack of adult hg - the fetal hg may be low at certain times and a transfusion may be required. Also, some mutations are not as severe as others so some amount of hg may be made, making the thalassemia less severe - therefore the patient would be a thal intermedia rather than a major.
Sharmin
-
Hello Keep smiling
Thalassemia intermedia is a clinical classification, not a genetic classification.
Both thal major and thal intermedia have two muted genes. In thal major the genes are severly muted that they are not able to produce red blood cells. While in thal intermedia the two genes are mildly muted allowing to produce little''good'' blood (that is why HB in thal intermedia can be maintained around 6 to 7) that keeps the patient going without transfusion for a while.
So genetically speaking both inermedia and major are the same in terms of the number of muted genes, but they differ in phenotype as thal major is dependant on transfusion while thal intermedia are either transfuion independant or transfuse 2 to 4 times a year or when they get sick (meaning irregular transfuion)
manal
-
Exactly like Sharmin said it will be determinate by different things.
As you said Keep smiling, Nafja has the genotype ß+/ß+. This means that her chains has an remaining activity, that can lead to thal intermedia. Most of thal major has ß°/ß° or ß°/ß+, some other intermedias shows this genotype ß++/ß+. By knowing about 230 different mutation only for ß thalassemia we can see many differences in phenotype of this disorder.
God Bless
Gabri
-
so to put all together..............
no specific line can be drawn between thal intermedia and major,,,,,,,,,,,,,
there are a number of factors which determine the condition of a patient such as the type of mutation, the number of mutated genes i.e one or two, the production of haemoglobin etc....
and all these things are linked with each other affecting each other,,,,,,,,,,,,,,
is it so??????????
-
what is the possibe minimum lower limit of pre-transfusion level of Hb???? :rolleyes :rolleyes
Najaf Hb dropped to 6.5 yesterday.........should we trasnfuse her immediately?
this level has been dropped within 23 days of trasnfusion? isnt it too rapid drop?????
Abbas
-
Abbas,
If we don't want effect child's growth,it is advised that pre transfusion hb should be kept till 9.5 ,not below then that,and this is a difficult decision since Najaf is an intermedia,have you guys decided to put her on regular transfusions? This is a bit fast drop but it also depends on how much blood was transfused last time?Do you know that? And as you said Najaf has gone pale and is not well and gets tired,i would transfuse her if i were you,have you contacted her doctor?
-
she was transfused almost 230ml last time............i think its too much
and i think it was pure blood not red blood cell packet.......
Abbas
-
What is Njaf's weight? and why was she transfused whole blood ??? she only needs red blood cells and with a filter.Transfusion volume should be 15 ml/kg of body weight .
Zaini.
-
Actually last time when she was transfused she was not diagonised as thalassemia yet so whole blood was transfused,her weight is 15 kg,
another thing, u said if we had decided to put her on regular trasnfusions? is it matter? i mean if we say "no" then what alternative we have?
by the way, we will transfuse her regularly to avoid retardation in growth........
Abbas
-
Some intermedias do survive with out transfusions,or may be occasional transfusions,thats why i that question,i am personally in favor of regular transfusion so that growth won't be effected,but once you start transfusion,keep a look out for ferritin.
The point of transfusing a child regularly is keeping his/her growth normal,and it would be possible if you keep her pre transfusion hb to 9.5 and not let it go below that,so 6.5 is pretty below that,once you'll start transfusing regularly,a pattern would establish and most of the time you would know how many weeks she'll be able to hold her hb between transfusions.When my daughter starting transfusing,we didn't know that its not good to let her hb go below 9.5,doctor told us that hb of 8 is the limit,so we used to go at 6 weeks,but when we came to know that its not good,we changed the pattern,now we go every fourth week,but in between a period of time came when we had to go every three weeks,because her hb used to go low,i don't know what was the reason,but we were able to change that pattern to 4 weeks again after we started carao fruit syrup.You can read about carao in this thread
http://www.thalassemiapatientsandfriends.com/index.php?topic=1259.0;highlight=carao
http://www.thalassemiapatientsandfriends.com/index.php?topic=1791.0
Hope this helps :)
Zaini.
-
Thanks Aapi.....
so what is the medical name of Carao syrup?? i mean i can get it by any medical store by saying "Carao Syrup"
-
Abbas,You are welcome :)
Unfortunately Carao is not available in Pakistan ,You can order it from www.tropicalhealthfoods.com ,It is kinda expensive for us as it is in dollars but it worked for my daughter and it has worked for lot of intermedias by helping them maintain their hb and prolong their transfusion intervals.
Zaini.
-
hi,
i had been at home so long, sorry for my absence..........
here is the CBC and hb report of Najaf:
Hb 7.1
hematocrit 26.6
RBC 3.22
MCV 82.2
MCH 22.2
MCHC 26.8
HB A2 1.5
hB F 98.5
WBC 14.7
NRBC 9/100WBC
Platelets 193
so we got Najaf checked, and doctor says it does not look to be the case of thalassemia instead it looks to spherocytosis, the doctor is very professional, experienced and is the president of Thalassemia Society of Pakistan. she asked for reticulocyte count and osmotic fragility for confirmation.
spherocytosis means that rbc are not of normal shape and are sphero type, because of this spleen takes them as abnormal cells and they are destroyed soon. her spleen is also a bit enlarged which is common for spherocytosis..it is caused by defects in proteins of cell membrane of RBC
now i have questions in my mind.......
can sphero cytosis cause regular hb drop as we have transfused Najaf 3 times in 3 months and hb was pretty low at around 7 every time?
can spherocytosis justify 98.5 hb F??????
is it a combination of thalassemia and spherocytosis?
it is also to be noted that Najaf's body temperature especially of palms and forehead is high every time even in winter it looks as though she has just come from the heater. in september she had very svere fever for almost 10 days and then she got 2nd ever (first in 5,6 after birth) transfusion, even after this tx she got fever again ( i wrote this may be its of some help)
Abbas
-
Abbas,
I am sorry i don't know much about spherocytosis,but why would her HB F be so high if she doesn't have thalassemia? ??? May be Andy would be able to shed some more light on that.
Zaini.
-
Abbas
We had a post here about spherocytosis, i will try to find it and send you the link.
All i know is that it is recommended for those patients to do splenctomy after the age of 5 because this stops the destruction of red blood cells and eliminates transfusion
manal
-
I found this one post about spherocytosis,this is not much,
http://www.thalassemiapatientsandfriends.com/index.php?topic=780.msg5911;highlight=spherocytosis#msg5911
The thing is you'll get the clear picture after you do those tests Dr Javeria suggested,so keep us updated with the results.
Zaini.
-
This latest electrophoresis report has a major difference from the previous report.
http://www.thalassemiapatientsandfriends.com/index.php?topic=3181.msg32057#msg32057
previously a doctor in multan took some tests and i think electrophreseis too
i m stating some of her results if u could understand
Hb A2 2%
Hb F 55.6%
Hb A 42.34
global chain variant = N/C
possible genotype B+/B+
VDLC/ TLC=18400
Poly 37%
lympho 56%
retics 18.8%
platelets 140000
normoblasts 16
below is the quotation by the testing lab
" severe hypocloromia, microsytosis. moderate macrocytosis, anisocytosis, poikilacytosis, many spherocytes, pessay cells, target cells and normoblasts.
http://www.thalassemiapatientsandfriends.com/index.php?topic=3206.msg32872#msg32872
here is the CBC and hb report of Najaf:
Hb 7.1
hematocrit 26.6
RBC 3.22
MCV 82.2
MCH 22.2
MCHC 26.8
HB A2 1.5
hB F 98.5
WBC 14.7
NRBC 9/100WBC
Platelets 193
Spherocytosis would not cause a fetal hemoglobin level this high. It is normally a few per cent and at the most 15-20%. The difference in the two reports concerning HbA and HbF are inexplicable, so someone has made a mistake somewhere. 42% HbA wouldn't suddenly disappear. There have been rare cases reported where thal major and spherocytosis co-existed ( http://www.turktel.net/cgi-bin/medshow.pl?makale_no=56327 ). Beta thal major is the only explanation for zero HbA and over 90% HbF. The previous report looked more like thal intermedia (B+, B+), with possible spherocytosis. This new report makes little sense, so I would suggest that they have the electrophoresis test done at another lab for comparison sake.
I would say there is a strong chance the child will need regular transfusions. If it is spherocytosis alone or combined with thal, a splenectomy may be considered. Splenectomy might eliminate the need for transfusions if it is spherocytosis. Both thal and spherocytosis call for folic acid supplements, so make sure the parents are giving a daily dose of folic acid.
-
Andy,
i was also thinking on the same lines, only spherocytosis cant cause such a high HbF, either it is a combination of thalassemia and spherocytosis, or some mistake has been made in reports.
but the 2nd report is from the most authentic lab of Pakistan ( the Aga Khan University Hospital, anyhow we will do electropheresis again
yes, she is taking folic acid right from the beginning
Pray that Najaf has only spherocytosis.....
Abbas
-
Abbas,
If the second test was done by Aga Khan,i'll go with that,and high Hb F indicates thal major,but do repeat electrophoresis along with the tests dr Javeria suggested if you can,this will give a clear picture.
Good luck and praying for Najaf,
Zaini.
-
Andy,
But how can Najaf be a thal major and she only tansfused 3 times during her past 4 years???
manal
-
Manal,
This is why I think the earlier test was accurate and this current test cannot possibly be correct. Zero HbA results in regular transfusions. This looks more like the thal intermedia that was originally diagnosed, with the possibility of co-existing spherocytosis. Whoever did this second test, made a mistake. It can't be 90%+ HbF and not be regularly transfusing unless there is also co-existing hereditary persistence of fetal hemoglobin (highly unlikely). I don't think we'll know anything for certain until further results are obtained. If it is intermedia with spherocytosis, a splenectomy may remove all need for transfusions.
-
Hi,
Andy and Manal thanks for having a close look on reports.....
May second report is incorrect...
We are going to have tests very soon.
Abbas
-
Thanks Andy for your reply.
Abbas,
I wish her all the best
manal
-
Hi,
Najaf's Osmotic Fragility is going to be done today, and appointment with doctor is also due today.....
till today's evening everything will be decided....
keep ur fingers crossed
Abbas
-
Good luck Abbas,we are praying for Najaf :pray .
Zaini.
-
an update,
Najaf's retic count is 12. will some one shed some light on that??????
Abbas
-
Abbas,
Good Luck ,buddy !!... praying for Najaf :) ....
Best Regards
Take Care
Umair
-
hi,
i have just returned from Lahore, we got reports for Osmotic Fragility and Echocardiography,
everything was normal i mean both Osmotic Fragility and Echocardiography.
this concludes that Najaf does not have HS.
so doctor says it is Thalassemia....
She decided to take Najaf as Intermedia......
yet she advises to take HU and folic acid and avoid transfusions as much as possible....
she says the threshold for transfusion will be Hb 7....
Abbas
-
doctor has advised HU at the dose of 16.6mg/kg/day for Najaf.
will it work for Najaf????
Any information regarding the use of HU will be most welcomed :biggrin
Abbas
-
Abbas,
You are talking about Hydrea or Hydroxyurea? Manal's son is taking it right now,she'll be able to guide you more.
Hydrea or hydroxyurea is basically a fetal hb inducer,it induces fetal hb which in turn results in increasing hb level,but sometimes it works for some one and for some it didn't work,and there are a couple of tests which you should do regularly while on Hydrea,as it can lower blood counts.
Zaini.
-
yes i m talking about Hydroxy urea......... May it work for Najaf
which tests should be done regularly?????
Abbas
-
Abbas,
It might work for Najaf,as she is being treated as thal intermedia,wait for Manal she'd be able to tell you more about tests and precautions.
Zaini.
-
Abbas,
Hydroxyurea works to raise hemoglobin levels in many patients and is a long offered therapy for non-transfusing intermedias. It does seem to work better in patients who have a natural ability to produce higher than normal levels of fetal hemoglobin, so it may have a good chance of working here. Folic acid is an absolute must as it helps to build red blood cells. Since the child has been determined to be intermedia, I would assume the report of B+/B+ is accurate. This does often manifest as intermedia and with the high fetal hemoglobin level, it does give hope that hydroxyurea will help.
-
Hi Abbas
I have answered your PM in details, please check it and feel free to ask anything that is not clear
Take care and good luck
manal
-
thanks Andy, Zaini and Manal for ur detailed reply.......
God bless u all...
Abbas
-
Hi,
last night i talked to my mother and she told me that Najaf is doing very well........
she looks more cute, even healthier.....everything is going right yet.
Abbas
-
I am glad to hear the good news Abbas, good luck and please update
manal
-
Hi Abbas,
Glad to hear that,has she started Hydrea?
Zaini.
-
Hi,
yes she is on HU from last sunday.....
Abbas
-
hi,
Najaf is having problem in taking HU, she weeps a lot :rotfl
i think it is something like powder, i have not seen it yet. is it too much bitter or anything else, or should we mix in something like milkshake,juice etc?
another thing, should we start supplements for Najaf? today i talked to her doctor and she said supplements are not recommended for children of age 4...
what all of u say?
Abbas
-
Abbas,
Actually my son doesn't have any problem with HU in terms od taste. It is true that it does not disolve completly, some of the powder floats, that is why you should mix it very good before you pull the required dose to make sure that you are taking the medicne and not just water.
There is no problem mixing it with a something, but you need to make sure that the juice is completly swallowed
What i do is that i put 5ml of water, empty the powder in the capsule and mix it with water. After stirring, i pull with a syringe 4ml (this is my son's dose according to his weight) and i give them to him and throw the rest of the ixture in the glass.
As for supplements, it is very important to be on supplements especially thals as there bodies are in contionous need for vitamins because anemia put stress on the body and therefore these elments are quickly being depleted.
Please read the posts in the nutrition and supplements corner and feel free to ask anything that is not clear, but in short she must be on
1- folic acid
2- multivitamin and minerals (with no iron or vitamin C)
3- Vitamin E from a natural source (this is a required antioxidant)
4- Calcium, vitamin D, zinc and magnesium (usually found all togather and many of us use Osteocare, and it is found in syrup, chewable, tablets,..)
5- L-carnitine (given 5 days per week)
manal
-
Manal,
i have been reading through nutrition corner, thats why i asked about supplements..
but why her doctor did not advise supplements?
should we start supplements without doc's advice?
Abbas
-
That is strange too for me Abbas, but you can discuss it again with the doctor
manal
-
Manal,
i m totally confused what to do...
Abbas
-
Abbas,
This is the usual behavior of doctors here,but which doctor are you talking about? Dr Javeria or any local doctor?
Zaini.
-
Abbas
I would advise to talk again with the doctor and show him/her some research. There are 5 important papers concerning L-carnitine for example and its effect on thalassemia
http://www.thalassemiapatientsandfriends.com/index.php?topic=892.0
Improvement of Cardiac Function in Thalassemia major Treated with L-Carnitine
http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowAbstract&ArtikelNr=76522&Ausgabe=229926&ProduktNr=223829
Assessment of Puberty in Relation to L-carnitine and Hormonal Replacement Therapy in {beta}-thalassemic Patients.
http://lib.bioinfo.pl/auth:El%20Beshlawy,A
Apoptosis in Thalassemia major Reduced by a Butyrate Derivative
http://content.karger.com/ProdukteDB/produkte.asp?Doi=87890
Diastolic Dysfunction and Pulmonary Hypertension in Sickle Cell Anemia: Is There a Role for L-Carnitine Treatment?http://content.karger.com/ProdukteDB/produkte.asp?Doi=89472
Pulmonary hypertension in beta-thalassemia major and the role of L-carnitine therapy.
http://www.ncbi.nlm.nih.gov/pubmed/19065439?dopt=Abstract
So lets try first to show these studies to your doctor before deciding to take them on your own though i don't think there would be no harm
manal
-
hi,
sorry, i had gone to home for Moharram holidays!!!!
zaini, i m talking about dr. javeria....
what to do now???
Abbas
-
AOA Abbas,
Dont worry dear , its not her (Dr.Jovaria Mannan) fault ... the supplement we talked abt here mostly are not available in our Country .. but , the supplement those r available here in our country and those r imported onez , doctors dont know abt them , as for those medicines there r NO Pharmaceutical persons or medical wrap to tell doctors abt those medicine .... when i was prescripted for calcium n Vit-D Pharmacy man gave me simple calcium + vit-D supplement which was much cheaper but only has calcium n Vit-D in that in much lower dose ... but , when i got to know Abt Osteocare and asked him abt its availability , he said "yes , its available " i bought one and in my next visit to my Doctor i showed her that and asked her abt that , she saw and said "where did u found it ? " she said its better than anyother supplement so u may start it by now .... so , wat i want to explain is, its not our doctor's fault , there r NO medical wrapers for imported medicine so how can our doctors get knowing these supplements ... i never hesitate to ask abt any supplement to my doctor ... doctor cant visit Pharmacies to have a hawk eye on these suplements .. so , if u think that a supplement is good for Najaf , then take a packaging of that supplement and discus abt that to doctor .. if that wud b good doctor will sugest her that ...
C ya Dost .. i wrote alot :D ....
Best Regards
Take Care
Umair
-
Abbas,
Yes doctors might be unaware of supplements not available in our country,but i won't say that its not their fault,they should be investigating more and more about how to give proper and advanced care to their patients,but sorry to say,they don't,i started my daughter on quite a few supplements which were not prescribed by her doctor,i learned about them on the forum,i asked question if they had any side effects,and then i tried them,coz supplements are necessary contrary to what doctor say or think.
I am not asking you to do what i did,but use your own common sense and judgement,you can also do some tests to check if Najaf is deficient in any particular mineral or vitamin.
Zaini.
-
hi,
i try to search multivitamins and minerals available here, then Najaf will be started with these supplements at low doses........
on my next visit to doctor i will take the approval......
Abbas
-
they (docs) cant go and check the each n every availale medicine in Medical store's shelf .... but, onetihing that is possible is , importer of those medicine ccan creat a medical wrap for advertisement of their imported medicine to aware doctors about the available more effective medicine , and to increase heir sales .... it will bring more competition in medicine market of our country n hopefully then our local producer will produce more effective medicine ... i thin , its not doctor's fault or less doctor's fault n more our n importer's fault ... i think we people have to tell doctor if we can find more quaility product in medical store ... as i said bfore, i never hesitated to show any supplement ot my doc bfore starting that ....
Umair
-
Umair,
i will search for such supplements and then show these to doctor,
Abbas
-
they (docs) cant go and check the each n every availale medicine in Medical store's shelf .... but, onetihing that is possible is , importer of those medicine ccan creat a medical wrap for advertisement of their imported medicine to aware doctors about the available more effective medicine , and to increase heir sales .... it will bring more competition in medicine market of our country n hopefully then our local producer will produce more effective medicine ... i thin , its not doctor's fault or less doctor's fault n more our n importer's fault ... i think we people have to tell doctor if we can find more quaility product in medical store ... as i said bfore, i never hesitated to show any supplement ot my doc bfore starting that ....
Umair
Yeah they can't go to medical store and check every shelf but whats internet for ??
Zaini.
-
hi,
here is problelm.....
on last thursday Najaf got transfused from Ali Zaib (where umair gets transfusions) because her hb was around 5, and amazingly yesterday again it was 5, Najaf will be transfused tomorrow again,
it means 2 transfusion within 10 days....meanwhile najaf has not been eating anything, anything she is given she refuses to eat it, if she takes milk she vomits it immediately......
did any of u did 2 transfusions in 10 days......
i m too much worried.....
Abbas
-
Abbas,
transfusions don't go by days - they go by hb, which means a transfusion is scheduled according to the Hb. Two transfusions in 10 days is not something unsual. However, are you sure the second HB count was correct? There are always possibilities of mistakes. The only thing you should check is if Najaf is sick with something, as you have said that she does not eat anything and vomits -- she may have a cold or something. Check that out.
Lena.
-
Abbas,
Was Najaf sick before getting the transfusion? How was she eating before she took blood?
There are at least a couple explanations. She may have been ill when she got the transfusion or became ill since. This might cause a drop in Hb. Another possibility is an antibody reaction which made her sick and also caused the Hb drop.
Blood can be transported for testing but you will need the cooperation of those taking the sample. Blood is stored with a preservative (EDTA) and kept at low temperature (2-8° C).
Also, make sure you know her blood type and make sure the blood she is given is always the blood intended for her.
-
Lena,
this winter Najaf has been very resistent to cold and flu etc...there is nothing wrong without vomiting and refusal to eat.......
yes i too had that point in mind so we got Hb report from two labs one stating 4.5 and other with 5. so hb drop is for sure....
Andy,
Najaf had been ill i mean vomiting etc before the transfusion....and the condition persisted even after transfusion.....
can we say that the blood quality was not good may be blood was old enough or something like that ??? :rolleyes
Abbas
-
hi,
Oh my Goodness, how difficult these days were....
najaf was transfuse again on 25 january and after 5 days her hb was 3.5.... :rotfl
at the same time she was having fever...we contacted her doctor and she said Najaf must visit her........
there she was hospitalized for 2 weeks... the main problem was fever which was uncontrolable...after every 12 hrs she had svere fever..
there were a number of possibilities i.e malaria, typhoid, Tuberclosis, but everything ruled out...
at the same time she was transfused 3 units in hospital, doctor was not able to diagonise the cause of fever....
but at the end Dr. Javeria concluded that she had "autoimune hemolytic anemia"
direct and indirect Coombs tests were positive,
at last she got rid of fever and then the problem arose of transfusion because her blood was not getting cross matched even with O negative, she was given steroids and is still taking....
even todays evening there was no cross match with O -ve. :rotfl :rotfl
one more thing her Fe is 5300... :rotfl
i will let u know with rest of her testing later, i m in a bit hurry.....
-
I am so sorry to know all you have been through :( Please keep us updated and what is the doctor's plane to fight this autoimune H.A.
Is she going to continue on steriods for a while??? Please make sure that she always gets the right blood??
Is she still on hydroxyurea?????
manal
-
My son also has autoimmune hemolytic anemia. His blood type is also O-. The first line of treatment is steroids. 2g per kg of body weight for 8 days, then half that dose for for 8 days. See if that works. Prednisone should be given a couple of days prior to, during and after transfusion.
IVIG can be used as a second line treatment if prednisone is ineffective. If her spleen is enlarged, splenectomy may be required. The other therapies all work better if splenectomy is done (if spleen is large).
Lastly, ritixumab may be used in conjunction with ivig.
If none of these methods are successful, BMT is considered as it can cure both AIHA and thalassemia.
It is best to start with pulses of prednisone before considering any other treatment.
Please keep me posted,
Sharmin
-
Manal,
doctor is fighting AIHA with steroids and she is on steroids yet... Najaf has also resumed HU.
Sharmin,
she is taking Prednisolone 0.4g/kg/day, lets c if it works...her spleen is also enlarged i.e almost double of the required size { i will let u know the exact figure later}
doctor says AIHA is not permanent, antibodies are automatically killed after completing their tenure of 15 to 20 weeks......with the help of steroids they will be suppressed soon..
Abbas
-
Abbas,
I am really sorry to hear what Najaf went through,it must be hard for your family too,i hope what the doctor is saying is correct and Najaf won't have to experience this again,
Best wishes,
Zaini.
-
Zaini Insha Allah,
lets hope so........
Abbas
-
hi,
sorry for being late..
during the last fortnight Najaf has been a lot finer....
she is much healthier and active...
her hb is even more than our expectation
last week; 9.5
this week: 10.2
i havent been able to understand this increase in Hb ??? ??? is this due to HU?
also her direct and indirect Coombs test have been negative this week. can we say that she is free of AIHA now???
najaf is also taking DFO 5 times a week....
hope all of u to be fine
Abbas
-
It may be too early to determine if this is all due to hydroxy, but the preliminary signs are good. It does appear that the steroid has done its job and knocked out the antibody, which would keep the Hb level higher.
-
Andy,
I am quiet confused, is it okay to use hydroxyurea in the presense of antibodies??
manal
-
Manal,
Is there any known problem using hydroxyurea when alloimmunization (antibody reaction) has taken place? I had a very hard time finding any reference to it. I did finally find one case where transfusions were stopped in a woman with sickle cell and hydroxyurea was started to boost her fetal hemoglobin level. One year later, she had not transfused and was doing much better.
http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B7W6S-4GJMPM8-2&_user=10&_coverDate=12%2F31%2F1993&_rdoc=1&_fmt=high&_orig=search&_sort=d&_docanchor=&view=c&_searchStrId=1252900609&_rerunOrigin=google&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=ad51342dec303d6e9a70e5ce2fd3e290
Summary
Alloimmunization following multiple transfusions, a frequently observed complication in sickle cell anemia patients, may make subsequent transfusion attempts extremely hazardous.
The case of a young woman with sickle-cell anemia is reported ; she was hospitalized for the treatment of extensive invalidating leg ulcers. The transfusion program that was initiated as a prerequisite to skin allografts had to be stopped, due to rapid occurrence of multiple alloimmunization.
Broad spectrum alloantibodies precluded further selection of compatible blood units.
In an attempt to get round this impossibility, a treatment with Hydroxyurea was initiated in order to boost synthesis of F-hemoglobin. After one year under this treatment, the patient's clinical status has clearly improved without requiring any new blood transfusion.
-
Abbas,
I am really really glad to hear this :) i hope Najaf will not have to face antibody issue ever again ,Good luck :) .
Zaini.
-
Thanks Andy for your efforts. :hugfriend
I was just wondering that since hydroxy is an immunosuppressent, so could it help in reducing the antibodies produced by the body or there is no relation
manal
-
Thanks Andy, Manal and Zaini sis,
thanks to all for your kind words....
Abbas
-
hi,
hope u all to be very fine...
Najaf's Hb is 10.5 for the last two consecutive weeks......... :hugfriend
today she had a little fever,, tomorrow is her first day at school :rotfl :rotfl
wish her luck,,,,,,,,,,,,
Abbas
-
Best of luck dear Najaf :hugfriend .
Zaini.
-
hi,
an update on Najaf,,,,,
she has been going to school last week with great enthuism..... :biggrin
her Hb is 12.5....hmmmm
and her SF is 507 which was a month ago 5300..... :rotfl
and now she has been advised asunra from her doctor........
her electropheresis has also been done, i will update u soon
Abbas
-
Abbas,
I am happy she is enjoying her school.
Concerning the ferritin reading, no doubt that there was a wrong test because no way she can have this drop in one month and most probably she is in the 500 range since she is recently transfused as i suppose.
But since she will be starting Asunra (on 500 level of ferritin), please moniter her ferritin so it won't drop than the normal range. Also moniter her liver and kidney functions every month
Good luck and keep updating
manal
-
Thats a really great news :) when was last she got transfused? Her hb is pretty good :thmbsup .
Zaini.
-
Abbas,
gr8 news :) .. best of luck , and many many best regards to lil Najaf ..........
-
Thanks Umair....
Zaini sis, she was last transfused on almost 2nd week of february........i think its good enough!!!!
here is the CBC of Najaf...
CBC
Hb 12.5 11---14
haemotocrit 39 34--40
RBC 5 3.9--5.3
MCV 82.6 75--87
MCH 24 24--30
MCHC 30 31--37
WBC 11.8 5--15
Neutrophils 57.1% 25--45%
lymphocytes 36.1% 40--60%
eosinophlis 1.5% 1--6%
monocytes 4.4% 4--8%
basophils 0.9% 0-1%
platelets 234 50--400
here is the Electropheresis:
Hb 11.8 11---14
haemotocrit 41.1 34--40
RBC 4.91 3.9--5.3
MCV 83.8 75--87
MCH 24.1 24--30
MCHC 28.8 31--37
HbF 100%
SF 507.3 ng/ml
Najaf is taking Exjade 29mg/kg/day.
Comments are awaited
Abbas
-
Abbas,
Things are looking much better these days for Najaf. The Hb is staying in a good range and her ferritin is low. I would remind you to closely monitor her ferritin level, as the dose of Exjade may need to be reduced if her ferritin drops much lower, since she is not transfusing regularly and new iron is not being regularly introduced into her body.
I am pleased with her progress these past two months. This is quite encouraging.
-
Andy,
thanks for your appreciation and encouragement, we will have a look at SF...
Abbas