growth etc

i have been norn and raised in UK, i have been transfused from few month and chelated from around 1 years old. I am 25 now. However still my growth and development has beeen affected. I am short 5ft only weigh 7 stone, and look like 12 years old, have long limbs and short upper body, i read that this only happens due to poor chelation or transfusion, but my consultants  say i have always had good treatment. so what has gone wrong, and what can i do. only thing they give me is oestroen but i still look so young and sound young and its so hard to be takn seriously everyone thinks i am a kid 

 

Also wnat to know why do i have gbig stomach, is it due to spleen or liver. I do not have desferal anymore, anm on exjade.

Also i read a lot of your posts that to have good iron levels you need to be under 500, however my consultant says that is too dangerous as it can be toxic.
I have always been very good at cheklation, on desferal i always remianed around 1000, since being on exjade it has gone up 1to 1600 but it is stable and my consultant is happy with this, as my t2* showed little iron in heart and liver, so why does everyone say 500 is alarmingly high?

Another thing i want to complain about  , do any of u feel thal is not ur only problem.
I have vision probs and hearing (due to desferal-why i dont know because i have been good chelator), oteoperosis, thyroide disorder, endocrine disorder, coeliac disease. falling hair-can anyone suggest anything to me?
I get so angry why me why i have ebverything wrong, i ignore it all and try and live a normal life but usually that means not complying with treatment for these other conditions.

Eesha,

The growth problem is thalassemia is one that frustrates both patients and doctors, because it is not related only to iron load. Yes, iron does affect the pituitary, but it appears that thalassemia itself, creates conditions that lead to the shrinking and eventual disappearance of the pituitary gland, regardless of iron status. My own opinion on what may help prevent this is that antioxidants should be used in thalassemics from birth and throughout their lives, because most of the non-iron related damage that is done in the body of thals is related to the oxidation process that is constantly ongoing, due to the unmatched alpha globin chains and constant breakdown of red blood cells. I would not be surprised that part of the treatment for thalassemia in the future will be to utilize powerful antioxidants as early as possible to try to counter the damage caused by the oxidation of free radicals throughout the body. Substances like Fermented Papaya Preparation (FPP) show great potential in limiting the oxidation process. IP6 (phtyic acid) and green tea extract are also well known sources of antioxidants, as is natural vitamin E, that all thals should be taking. With early intervention and constant control of iron levels, we may see thals develop more completely.

Treatment for thalassemia is always evolving and new understanding of how to manage care is ongoing. At the thal conference in New York City in October, 2009, doctors posed the question "How low should we aim for iron load in thal patients?" This was the first time I have heard this answer. "We now think that iron levels of a normal person may be the best goal for thalassemics." This is very new and our member, Lena is in a center where very close to normal ferritin levels are being maintained. However, to have iron levels this low (as low as 100), very close and regular monitoring is required. If this is not possible, then the 300-500 range should be striven for. Thal specialists no longer accept 1000 as the goal, even though most doctors treating thal are very happy to see patients around this level, and speaking realistically, this level may be the best that can be hoped for in most patients at this time...but I do expect this will change rapidly in the coming years as the benefits of a normal iron level in thals are fully realized. Doctors now believe that chelation should start as early as possible because any high iron load does damage and interferes with normal development. This means that we will finally see what happens when no iron load ever develops and what this means to normal physical growth.

It isn't iron that damaged your sight and hearing. This is caused by long term desferal use in some patients. This is a big reason why a variety of chelators is necessary. I have heard doctors say that no patient should use just one chelator for a lifetime. Changing chelators will help to minimize long term side effects of a particular chelator and all current chelators do have side effects.

Some of your problems may or may not be related to thal (celiac disease, for example). Some may be related to nutritional deficiencies which are caused by thal. I would suggest getting your vitamin D level checked. It should be a minimum of 30. Deficiency is common in thals and non-thals because we do not get the necessary daily exposure to the sun. Vitamin D is necessary for many other nutrients to be utilized in the body and deficiency has many effects. Thinning hair is one, although thyroid disease is also a cause. You should also realize that low iron in the heart and liver do not necessarily mean your glands were unaffected, The pituitary gland can have an iron load not seen elsewhere. Growth hormones are most successful when begun by puberty, but may still have some benefit for you.

Andy,

A question for our information. Do you think long term use of desferal can cause developmental problems in an individual.

Thanks

Yes. Sharmin's son was switched to Exjade partly because there were concerns from his doctors that desferal was slowing his physical growth.

there r many thals in my thal center they never been compliant  with chelation and they lived many years of their lives with a high iron overload but they look's quit normal ...   wat we can say abt it ,,  ii like to call them  LUCKY THALS  lolzz....  check the gallery of this site and se sajid's pic he was slim no faty look ,  height more than 5 but his iron was in 7000 range by last year july and they r  around 22 yrs old ,... 


Umair

Umair,

You have just brought up one of the biggest problems with thalassemics. Because they do not see the damage being done to their organs, they often do not believe it is really happening until they are in a severe condition of iron overload. We have to continue to try to educate everyone so they know what is really happening in their bodies.

Andy ,
there's a lack of counseling .. i dont know if docs r failing consistantly or the thals didn't get wat docs have to tell them .... when i was child docs kept saying me sumthing abt chelation ,, here is a demo of our conversation

docs ... stick with chelation ,,,
i :  why  ,,,,  
doc : othervice iron will  harm ur body n organs especialy ur heart ..
I : so then ?? wat will happen to me then ??
Docs : u could die in young age ....
I : isn't it better  lolzz....

so , they never told me that it will effect my life in many ways like growth prob , height and other probs like  it diabetes n all that ... they kept telling me that i will die .. and that wasn't a big deal for me to start chelation to avoid  dying in young  age  at that time bcoze i was a kid and it sseems me better to die than a needle ...  . but when then i realized that we even cant die bfore our time then i started thinking seriously abt all this .......  then a question came in my mind that if we cant die bfore our time then why all that IS for  then i realised that , its for a good quality life, a life like normal people ... a normal life. ....but it was a lil bit late ... .but not too late.... :D

if u  remember once i mentioned abt my friends ,, who never been compliant with these things.... i just told them  " u ppl cant die bfore ur time ,, but there r 2 options for u ,, to start chelation n live a healthy quality life ,,, or to keep living like this and  day u'll on bed like i was ... ...  ( sorry for been too straight forward with those thals.. but as that worked fine ,, so , everyting is fine ))

so , we've to change the way of counseling.. to make thals agreed for chelation .... we have to tel them that these things r  to avoid  complication that cud take u to death,  but these treatments r  more  for a good healthy life ....


Umair

I always thought the same question with the difference of being on the other side of the table.

My stats for those who dont know.

I’m 24 male thal major diagnosed at year one of my life.
Since then treated with high transfusion scheme i.e transfused with multiple RBCs at a time once a month. I remember when i had 7-8 hb in my childhood doc transf me 3 or even at later stages 4units at a time.

My chelation though wasn’t worst but IT WAS NOT as it should have been. And if I try to recall I would say I hardly used desferal consistently for 2weeks in my childhood. It was only the COMPELLED chelation after the transfusion in hospital i.e doc always wrote to inject i.v desferal after transfusion and I had to remain admit in hospital for 2 days and one night.

My past records show 8000 ferritin when I was of around 13-14yrs.

I remember it was only when i reached 15 that I became more knowledgeable about my disease and importance of chelation and started serious chelation by 16yrs of age. And I managed to make it under 2000 in 2004.

After that to this day … I have NEVER BEEN too consistent with low or high iron levels. As like my lifestyle and habits my ferritin level keeps changing its graph. Just for an example, because of my last years’ complex educational studies my iron went up high to 8000 and in 2months up to right now it’s less than 2000.

But, even with that poor chelation my medical condition is given as:

•   Doctors say those with better maintenance of HB has good height than others… I see more patients with scarce transfusion history taller than me. Im just 5’2..but still its NORMAL for thal of those days.

•   On complaining of my short height, during my childhood I got tested growth hormones ad as they were NORMAL I was said to be short because of my genes.

•   My t3,t4,TSH and testosterone were ALWAYS normal.

•   My calcium, magnesium and other similar counts NORMAL.

•   I was diagnosed as HCV + and elevated AST levels…but on PCR HCV came negative and I never had any treatment for it. ATM my ast/alt are NORMAL just being Alt slightly higher/

•   My Spleen still intact, slightly enlarged, still considered as a NORMAL for a thal.

•   As we see facial and bone structure deformation in thal. Despite of being looking yonger than my age, my all these features are normal. (I had rare facial hair just a year ago, maybe high testosterone due to weightlifting brought complete beard by now) 

•   My MRI,echo etc revealed normal results of all body organs.

•   NEVER HAD any-sort-of  health problems osteoporosis, pain, diabetes etc. can be idealized by knowing I do weightlifting.

•   Never had any side-effects with any medicine—be it desferal (no matter what quantity and duration), kelfer, folic acid, vit C, chelox, ferrinil, ferriprox.

I question myself as to how such a careless thal major be that fortunate?

The question should be an eyebrow raiser for every one, for every doc too. How patients with good medical coverage and better iron levels can incur more damage to their body than the ones with careless and ignorant past treatment?

The only answer I can give to myself is that I was just blessed by Allah Almighty. What else? No nothing. Sometimes I think that I never deserved being that lucky, because I know how much ignorant I have been to my body. May be perhaps after years we will come to know the answer.

Having read that bore novel, I would say that Eesha you aren’t alone with those difficulties in this world. Everyone in this world is tested by Allah. We do have problems in our lives with thalassemia, but there are people in this world that are deprived of eyes to see the beautiful sceneries of the world that you see, some cant walk a foot to even reach washroom, many don’t have hands to even get themselves a tasty bite of chocolate cake which we all fight for, others cant even hear melodious voices of their loved ones whereas we punish our ears with unable-to-understand-too-fast rap. Every one in this world is tested for his/her result in the Hereafter. We shall always thank Allah and struggle to get ourselves accomplished in our objective of being a champ no matter what barriers we might face.

Always be confident and happy of what you are. But if not—then watch this to get yourself a courage boost www.youtube.com/watch?v=T23ZXFi5Kt4

Well said Waleed 

Eesha,


Andy is right to mention the new ferritin goal to be in normal levels. Believe it or not, my last ferritin count was 68. What would your doctor say? 
Well, I continue chelating although it has reached the normal levels of a non-thal. At a little more relaxing rate, I would say, but non-stop. I personally believe in keeping the fe at the level of around 100.
Your consultant should not be happy with a fe level of 1600, that was a medical status of some 20 years back. Nowadays, things have changed. What Andy has mentioned about the low fe level that was said in the New York Conference,has been applied in my Unit some ten years ago. And let me tell you, things run under control as to the drug toxicity that you have mentioned.
So, rearrange your(and your consultant's)goals and do your best. Good results will soon arrive.

Lena.

PS: what about combination of ferriprox and desferal? Have you tried this? I mean, why just exjade? Exjade is good in low ferritin levels -- to maintain them low. It's not effective when fe level is higher -- in fact it's less effective that the other two- this is my personal experience. If anyone knows differently, please say so.

Lena is right. It is easier to get the ferritin down with a combination of desferal and deferiprone (Ferriprox, Kelfer). To get a dramatic ferritin reduction with Exjade, it is necessary to use a dose of 40 mg/kg daily. Although some patients can handle this dose, it is difficult for many, due to the side effects of Exjade. When Exjade is the only suitable drug, this method is recommended for those in a state of severe iron loading (T2* score of under 10). Still, desferal and deferiprone together will give a much more thorough chelation than Exjade can give, because deferiprone can more easily get to places the other chelators don't effectively reach, like the brain.

I also want to emphasize again that anyone attempting extremely low ferritin levels through chelation, does need to be closely monitored, as Lena's group is, so that doses can be adjusted and most importantly, that you don't put yourself in a position of a negative iron balance. We need to look at the desired ferritin range in relation to what type of treatment and monitoring is available. If it is not possible to get regular close monitoring, then a range of 300-500 should be the lowest target.

Just to provide an evidence aid. Recently my Fe went higher than 8000 and within 2 months using desferal + deferiprone (ferriprox) i got it down under 3k with no side effects at all.

Waleed thanku for ur responsee i agree with it.

Just one thing is that i cant use deferiprone to get my iron down as it cased me neutropoenia.

my consultant is a really good one and he said care is different around the world but getting ur iron too low will mean that the chelator will start working at other things like ur organs so will harm u.

eesha how low ur iron may get ... it will always b increasiing as approx 250iron in approx 250ml .. so the we will almost always need chelators .and i guess wihout overdosage the chelators are unlikely to cause any damage to organs . i read here in forum a patient has iron of 40 ..and thats too gr8 for a thal. Howver u may not need to be that much conscious that wid low iron ur chleators will hurt u but what we all thals really need is to worry about getting iron lower and lower as much as we can. Normally its just with oral that we wud get neutro counts lower ...but wid desferal they r usually good.