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Is there any treatment to maintain normal height in Thal. Major
Sharmin:
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?
Andy Battaglia:
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.
askari:
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.
Zaini:
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.
Andy Battaglia:
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
--- Quote ---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.
--- End quote ---
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
--- Quote ---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.
--- End quote ---
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.
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