Important Endocrinology Information for Thalassemia Patients

While we were in Oakland, we were fortunate to have a visit with an endocrinologist.  He was absolutely amazing with us.   He gave lil A a very thorough examination and he spent a lot of time discussing his care with us.  

Although in the normal range, lil A is a little smaller than his friends - but the endocrinologist assured us that lil A like many other kids his age is 2 to 3 years behind in development.  This is normal - and it is actually good news because when other kids have stopped growing - lil A will continue to grow.  You should have seen the smile on lil A's face when he was told that he would probably end up being quite tall!  

Here are some pointers that we received in order to optimize growth in thal children - 8 to 10 hours of sleep, adequate nutrition, exercise, supplements with cal/mag/vitamin D and trace minerals such as Osteocare, and  keep iron levels low so that iron does not accumulate in hormone producing glands, exjade is better than desferal during the growing years as desferal interferes with growth of the spine, shortening the upper body.  

The following tests should be done annually, (more frequently if abnormal symptoms are present):

-- IFG-I
-- IGFBP-3
-- Free Thyroxine
-- Prolactin
-- Lytes
-- Morning cortisol
-- Fructosimine level
-- Oral Glucose Tolerance Test
-- Bone age

The endocrinologist told us that it is EXTREMELY important to prevent iron accumulation in the pancreas to avoid diabetes (as Umair has warned us all about).  He also said that all thals need to be extremely focused on eating a low GI diet.  Stay away from white breads, sugary foods - please check the sugar content of all foods and drinks.  High sugar content in food, and especially juices puts a load on the pancreas.  Eat whole grain and natural foods only. Jamba Juice etc may contain fruit but the sugar content is dangerous for thals (and non thals). Protein shakes and bars can also cause problems because of the sugar content.  Stay away from juice!  Juice is very bad for everyone.  

Sharmin

Thanks for this very useful info.

You are welcome:)  I am slowly reviewing my notes - I will posting more info over the next few days. 

Sharmin

I hope everyone pays attention to these dietary recommendations. This is one area where thals can have a very positive effect on their own health. Eat whole unprocessed foods as much as possible. Food should be nutritious and never just empty calories. Good eating habits can make a big difference to the long term health.

Coincidentally, this is exactly what I told hopefulmommy at dinner last night. She has a beautiful daughter who loves her fruit and veggies.

Sharmin,

That was extremely helpful,i'll be looking forward to your other posts  .

Zaini.

Why is juice bad??? Which juice is bad?
What about those sugars? Untill how many sugar is ok? I know that we can't take products what contain more than 4%. (from the Hemachromatose books, google IDI Iron Disorder Institute. Still haven't bought my books)

What about those sugars? Untill how many sugar is ok? I know that we can't take products what contain more than 4%

I don't know where this number exactly came from, but it's the fructose that has the most deletrious effect.
You can find fructose in fruits, juices and table suger (sucrose = glucose + fructose).
You only have to eat fruits - all the other foods containing fructose should be reduced - and sweetened with glucose not table sugar...
Fructose itself is responsible for metabolic syndrome, excessive uric acid production, excessive lipid production, high cholesterol, diabetes - there is no end to it. Add to it problems caused by iron overload, which also causes metabolic problems in different organs, then you have a serious problem.
Particularly useful publication describing molecular mechanisms of action:
http://www.ajcn.org/cgi/reprint/58/5/754S.pdf

But you can find it anywhere - just google: fructose metabolic syndrome

Looks like our bodies are not used to fructose which is in our menu for only about 100 years...
But there is simple solution - sweeteners (unfortunetely they are not completely safe, here the cure may be even worse than disease) or glucose (personally, I prefer this one, but it is not as sweet as fructose).
Funny thing that fructose is recommended for diabetics, because it does not cause insulin release...
Maybe there something to it, but rather it's stupid recommendation.

Ok, I hope you will not be too shocked.

Have a great one!

B.

During the symposium, Dr. Wood presented a very interesting study about the pituitary gland and the pancreas in thalassemia patients.  Where iron will be stored in thalassemia patients is difficult to predict, some will store iron in the pancrease even if their liver is not severely overloaded and some will not store it in their pancreas.  The important thing to remember is of course to keep iron levels low, and to have a diet that prevents diabetes.  A low GI diet, and as Bigg has said watching the fructose intake is important.  This takes us back to consuming natural foods, whole grains and produce.  I would like to add that thals should be having more water than others, because they are taking supplements in order to help the liver.  Also,  chelators should be assisted by water because iron is often released by the excretory system.  I am assuming that water intake would help and that it would keep kidneys healthier.

The study was quite interesting as it related to the pituitary, as they found that thals tended to have a smaller pituitary gland.  I missed some of this lecture - but Andy should know a lot more about it.  Therefore, hormone replacement may be indicated for patients to replace some of the function of the pituitary (am I right Andy?).

Iron overload in the pituitary may also affect it's function - therefore once again chelation chelation.

Considering how which organs will be affected by iron loading is difficult to predict, I do think that combination chelation is a good idea because different chelators have different mechanisms - they work at different levels and in combination they can remove iron from organs having a synergestic effect in protecting organs from iron damage.

Antioxidants further protect the body by binding with free iron that remains and preventing iron damage.

Is anyone taking fermented papaya?  I am hoping to start lil A on fermented papaya soon.  I think that we would all benefit from taking it.

Sharmin

Puja's son is on fermented papaya

ehh what means fermented? And why is Papaya good? I am reading all the posts that are waiting for me. This topic is very informative.

I've had a lot of endocrine issues that were diagnosed over the last many years-

Hypothyroid, with antithyroid antibodies,
Pituitary Microadenoma, High prolactin,
Elevated Lipase/Amylase (One of these was elevated, I don't remember which, - sorry),
LH, FSH, Estrogen, Dhea, - All of these out of normal ranges,

Not yet tested completely for Cortisol related issues but might have some imbalances on and off.

I think its always a good idea for all thals to get endocrine checks just to be sure everything is ok.  I took a long time to get diagnosed because my symptoms were always misleading. Getting tested on a routine basis might have saved me a lot of time and suffering!

I check regularly now, and get prompt treatment when it is required.

My hemoglobin is always between 9 and 10, but it was the endocrine health problems that kept me really very miserable.

Dore,

Fermentation. http://www.biology-online.org/dictionary/Fermentation

An anaerobic (without oxygen) cellular process in which organic foods are converted into simpler compounds, and chemical energy (ATP) is produced...Fermentation occurs naturally but humans have used and controlled the process. It is used in the production of alcohol, bread, vinegar, and other food or industrial products: 

Other fermented foods include yogurt. Papaya is a very nutritious fruit that is loaded with vitamins, minerals and enzymes (that aid in digestion). The fermented papaya preparation was developed in Japan decades ago. It has been the subject of many studies that have shown it to have powerful antioxidant properties and an ability to fight excessive free radicals. For more on FPP, see

http://www.osatousa.com/prese_01.html
http://heartspring.net/papaya_antioxidant.html#fermented

i remember .. i was always short than my age fellows  ... i always raised this issue with my father and doctor checked me twice for all those endo tests and wut he concluded then is that my hormones are really normal and that he cannot advice any treatment but what he said is many ppl are naturally short and this may happen with me .... and yes it really happened ..... im just 5'1 or 2  .... and really of same  height as of my maternal uncles  ...still endo tests r fine ... conclusion -- doctors say that ur height was naturally short as ur genes were more oriented towards ur mother's side  and ur height MAY HAVE BEEN afected by ironoverload though my tests were normal ....

I cant grow vertically (im 23.9) but horizontally i will ...... 

I am disagree  that taking sugar is really harmful for thal or anyone.. If you have  normal and active life. There is no harm to sugar, because sugar is still important for many purposes, ofcourse not too much
  In endocrinology matter, I really want to say, please check your hormones levels. Because of thallsemia major, patients always have deficiency and if you concern your endocrinolgist in early age, you can also save from delay puppetry problems.
About the pancreas effect by iron, yes it do effect. But there is research going in Brampton hospital London on genes. I am also part of this research as voluntary . Basically this research  is about genes which allow to deposit iron into the heart and other organs.  Doctor , sorry I forget his name. He told me that according to his research he observed that some thal patients have a lot iron in heart but not in liver. Some patients have iron in liver and pancreas but not in heart. So this research he actually  wanting to target those genes, so in future we will have some kind of treatment that stop deposing iron into organs. Hope fully 

Hi,
Can a nyone tell me from what age are these annual endocrine tests required?
Zahra