Is there any treatment to maintain normal height in Thal. Major

Hello friends
I observed that thal Major patients are short in height as compaired to normal person.
so i would like to ask that is there any treatment(medical Or deit) to maintain the normail height for the patient of Thal. Major.

thx and bye

Hello Mr. Mumtaz,

That is due to poor treatment. If you under transfuse your child then the lack of oxygen will retard normal functions of the body, furthermore Iron overload will damage the endocrine glands which result in stunted growth and development due to hormonal dis balance.

To avoid all that and to make sure that your child lives like normal person, keep his pretransfusion Hb above 10 and his SF low by transfusing on time and being faithful to the chelation therapy.

If you follow this then you can avoid all the complications that can happen to Thals.

Sajid,

I would beg to differ with you on this topic... Only because its not always the case.... We here in australia and in some other parts of the world have thal majors who are taller than your average person...I have met some larger than life and taller thals in my life time..

For instance Miakis late husband was a tall man!!!  And chelation therapy at the time wasnt available when he was growing up...I am short because my parents are of short stature too...


So Mumtaz there is no diet... but if your child does have growth problems you should speak to your heamatologist and they would be able to advise you on growth hormones if they are available to you...But with todays modern technology and modern treatment I dont think that there would be any problems..

Hope this helps..

Hi,

We think its a combination of factors discussed by Sajid and Smuefette. Genes play an important role in the height but can be effected by iron overload and low Hemoglobin levels. There is no set criteria some people may be effected more than others.

Regards

Genetics does play a big role in height, but getting sufficient blood as a young child has much to do with growth. Under-transfused children normally will not grow to their normal height and will also develop skeletal issues. And as Sajid mentioned, iron damage to the glands can also be a factor in growth. Overall, thals do tend to be shorter than non thals but there are exceptions as noted by Smurfette. Stan was tall and I have met other thals who have normal heights.

Proper treatment with excellent compliance will greatly increase a thal's chance of attaining normal height.

I would like to add that in cases where growth has already been slowed by the complications of thal, that growth hormones are often used to stimulate growth. The best success rates occur when the growth hormone intervention takes place at the onset of puberty, rather than later. Early intervention is essential in hormone replacement treatment.

Hi,

I have a question here,though my daughter is too young for that right now,but still i would like to know this,specially from adult thel females,like smurfette or miaki or sydneygirl,that do thals start having periods just like normal females,or do we have to give them medicines?bcoz once i met a mother of 16 years old thal girl, and she told me that they had to do some harmone therapy for her periods to get started, is this what happens to everyone,or there are any exceptions?once a heamotologist told me here that once your child nears the age of 10,start monitoring their harmones,to keep a check on reproductive system and everything,and to make your kid hit puberty at the right time,i need a little help and guidance here,my daughter is only 6 and a half years right now,but it is good to be acknowledged.

Waiting for your replies,

ZAINI.

Hello Zaini,

Sorry for the delay in replying..Your question you asked is one to be asked by your endocrinologist...It all depends on when your daughter started her desferal treatment and how long she has been on it for...

You still have to do the test to see how her hormones are doing..but some girls that I know do take some sort of hormone therapy...but your daughter is still young and if she is chelating as she is supposed to...I am sure that she wont have any problems..

All females vary too...So its better to ask the edocrine doctor..

Hope this helps..

Thanx Smurfette,

My daughter started desferal when she was 4 years old,and her transfusions were started when she was three years old and now she is 6 and half years old,but from last one and a half year she is also taking ferriprox(defriprone),her S.F last checked was 1327,i know it's high but i am working hard on it,so i hope and pray that there will  be no problems in future.

TAKE CARE,

ZAINI.

hi zaini,
how often does your daughter do desferal?
vic

My son is almost 9yr.  In his first 2 yrs he was on the 75th percentile and until the last couple of years his height remained constant on the 50th percentile - which I was happy with.  This year however, he seems to have fallen to 10th percentile!  I am very concerned.  I know that he has grown in the past few months - and I have not charted him since that time - but I am afraid.

The growth which has occurred - happened since we began giving him IP6 - I wonder if IP6 may have helped - perhaps by removing iron from his pituitary etc.??

Should I be worried? Andy?

Thals often do remain short even with proper chelation. Ambareen comes to mind as one who has maintained a low ferritin level throughout her life, yet is shorter than normal, even if for some reason her height has never stood out to me as unusual.

Sharmin, it is extremely important that your son be seen regularly by an endocrinologist as he approaches and goes through puberty. If it is determined that he should undergo hormone replacement therapy to promote growth, it is essential to start at the beginning iof puberty if maximum results are to be obtained. An endocrinologist can tell you if this will be necessary.

I agree that IP6 may have a positive effect as it works within every cell in the body and does inhibit the effects of iron in the cells. Your son is basically a "guinea pig" for the use of IP6 in thal, but a safer substance to use on a "guinea pig" could not be found, as IP6 occurs naturally in every cell in the body.

Sharmin, I will do some more reading on this subject and let you know what I can find. Do make sure he is receiving adequate calcium and vitamin D, as it does promote bone growth.

hy hi
i am 24 n my chelation theraphy started whn i was 6.my cycles hv nt started yet i am taking hormones for the past 6 years but still no use is it sumthng to worried about.

Hi Vic,

Zainab is having desfral twice a week,as she is on ferriprox also, about one year ago it was 5 days  a week.

ZAINI.

The issue of growth among thalassemics is a complex one, with various factors being responsible for reduced growth. We have discussed low hemoglobin levels and high iron levels as both contributing to inhibited growth in thals. However, in an ironic twist, the treatment for high iron levels also inhibits growth. Chelators are known to remove minerals besides iron and this can also contribute to stunted growth. It has long been recommended that patients using desferal should take zinc supplements in addition to folic acid. For many years, these two supplements were the only recommendations for thals. Now you can add calcium and vitamin D to that list, as calcium is also depleted by chelation and vitamin D is necessary for absorption of calcium. Calcium is essential for bone growth and development and deficiency can lead to both slow growth and weak bones. Trace minerals may also be required as supplements.

There may also be other reasons why thals are shorter than normal on average. A study in the UAE found that thals were deficient in several amino acids.

From http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6T57-4DS69N5-7&_user=10&_coverDate=02%2F01%2F2005&_rdoc=1&_fmt=&_orig=search&_sort=d&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=34ba485e23457f9d37a25638eceaeefe

There may be a marked reduction in essential amino acids in the serum of children with thalassemia major and this is related to decreased growth in affected children...most plasma and urinary essential amino acids were found to be lower in thalassemics. Thalassemic patients were also found to be significantly growth impaired for age, both in height and weight compared to controls.

Conclusion

Lower plasma values of essential amino acids and a decrease in urinary amino acids occur in thalassemic patients. Growth impairment both in height and weight also occurs in thalassemic patients compared to a control population.

It is recommended that young thal children whose growth is below the norm should undergo growth hormone therapy at the onset of puberty to promote more normal growth.

There is an excellent article about growth in thal patients at
 http://www.ijppediatricsindia.org/article.asp?issn=0019-5456;year=2005;volume=72;issue=2;spage=159;epage=164;aulast=Low

Despite advances in medical therapy, growth retardation and hypogonadism (failure by the sex glands to produce sufficient hormones) continue to be problems observed in transfusion-dependent patients with thalassemia major. Abnormal GH (growth hormone) secretion may be seen in some of the patients but the majority of the short thalassemic patients do not have GH deficiency. The low serum IGF-1 and IGFBP-3 concentrations in short thalassemic patients with normal GH reserve and serum GHBP levels suggest a secondary GH insensitivity state. Supraphysiological (beyond normal) doses of GH can overcome this partial GH insensitivity state in these patients resulting in improvement in the short-term growth as well as limited evidence on an improvement in the final adult height. GH therapy appears to be safe but careful regular monitoring for the development of side effects should be performed while the patients are on GH treatment.

This is very interesting. Even though thals may test for normal amounts of growth hormones, by treating with added growth hormone, the patients do grow more normally, leading to the conclusion that there is something causing the bodies of thals to be insensitive to the normal amount of growth hormone. When increasing the GH above normal levels, it seems to correct this insensitivity to some extent.

The addition of zinc, calcium, and vitamin D along with a general mineral supplement may help the bones of thal children grow more normally. In addition, growth hormone therapy at the onset of puberty may play an important role in helping thals achieve more normal growth.