HI......everyone

Hi Andy....and other friends...

I am new to this forum and would like to tell you all about my 6.6 year old son. He was diagnosed with Thal major at age 1. He was admitted to hospital at 5 months for otitis media and his hb levels went down to 8.5. The pediatrician kept him under observation and advised electrophoresis at age 1. And the truth came out.

The first two years were horrible. He kept falling sick every now and then.....Then it was time for school. One year was hell. Hospitalisation, sickness, infections, missed schools and delayed development. But hb level never went down from 7.5 to 8 g/dl. So no transfusion was needed. He would normally bounce back to hb levels 10 g/dl. He was then diagnosed with intermedia.

At age 4, his hematologist advised hydroxyurea (hydrea). Two years since, he is been on it. Immediately the effects started showing. The paleness gave way to a little red.s We have regular ferriscan and hematograms. Level of hb (at times of infection) would remain at 9 g/dl. No organ megaly. Bones and dentures are fine......
The best part is the quality of life of my son was much improved. The lethargy and infections were all gone. Two years since he has been attending school in a regular manner. And wonder what since the last 8 months he did not have any serious infection. touchwood. He takes hydrea 500 mg every alternate days, folic acid and zincovit everyday.

I have shifted to another city and would be meeting up a new hematologist and pediatrician this saturday for an evaluation.
The only problem I am facing in the new city is his new school.......His timings are 8 am to 4 pm. His teachers complain that he falls asleep in class everyday.....I have got to check his hb levels soon.

Can it be because of hudrea?? His hematologist had said the advantages of hydrea outwieghs the side effects. Is it true?

Thanks for accepting me in this community.........
Indy

Hi Indy,

Welcome on the forum,I am really glad that hydrea is working for your son,i am no expert on hydrea but i don't think hydrea would have anything to do with his falling asleep,were the timings same in his last school? If he has to stay longer in school then he is used to,he might take his time getting used to it.

Our moderator Manal's son was on hydrea,she'd be able to help you more with that.

Zaini.

Hi Zaini......
Thanks for answering...U are the first one to reply. The school timing were much lesser as he was in kindergarten (9--12). The timings are way bit greater now. Probably yes.....he is not able to cope up with the timings.

Thanks
Indy......

Indy,

I think his sleepiness is probably related to low Hb. This is common even in thal minors. I would strongly suggest adding L-carntine and magnesium as they both can add to the effect of hydrea. We have many discussions on the use of hydroxyurea and yes, it has benefits that far outweigh any negatives. I would suggest typing hydroxyurea into our search engine and reviewing the various threads about use of the drug.

This thread follows Manal' experience with her son's use of hydroxyurea.
http://www.thalassemiapatientsandfriends.com/index.php?topic=3352.msg34138#msg34138

This is from that thread and explains some of the other benefits.

http://www.thalassemiapatientsandfriends.com/index.php?topic=3352.msg34156#msg34156

The increase in HbF does show that the hydroxyurea is working. The goal is to increase production from the gamma gene, while reducing the ineffective erythropoiesis in the bone marrow. Every study I have seen has shown a continuing increase in HbF into and after the second year. I would definitely recommend patience. The benefit to the blood by reducing the excess alpha chains should not be overlooked. The reduction and prevention of extramedullary hematopoiesis, which is extremely common in non-transfused intermedias is another major benefit.
There is a nice powerpoint about the use of hydroxyurea in thalassemia at
http://www.thalassaemia.org.cy/pdf/syria/day%202/Day%202%204.pdf

Note the other positive effects of hydroxyurea, in addition to HbF induction, including the neutralization of the excess alpha chains by the gamma chains. The excess alpha chains are one of the biggest problems in thalassemia and their reduction is a big positive.

I would suggest explaining to his teacher that he does have a low hemoglobin level and requiring an afternoon nap is quite common, even among thalassemia minors. Some flexibility by teachers is often necessary and if they understand and are included in the situation, they sometimes will be more cooperative.

Thanks Andy

I am going to see a hematologist tomorrow.....I will keep you posted.    Since I am based in India, will u be able to tell me by what name L-carnitine+magnesium is available since this is a generic name.

Though, in India we have many good medical facilities where one can get management advice regarding thal, its not available to all. I am fortunate to be in Delhi where my son was taken care of by a good hematologists in All India Institute of Medical Sciences (AIIMS). With the ongoing research, hydroxyurea has proven beneficial, but I am sorry to say that in small town India, many doctors are not aware of all this.

Hoping one day in developing country like mine, this disease doesnt remain fatal anymore.....

Good luck with Saturday's appt & welcome on board!
I have btw pyruvate kinase deficiency but required the same treatment except hydrea.

Hello Indy and wecome to the site

I am happy that hydrea has given you son a better quality of life and happy too that we can share our expierence toghather

I must agree that it is a long day in school for a young boy. Try to give the hyrea before sleeping rather than the morning because it may make him feel dizzy (though i assume that being on it for a couple of years, he may be okay with it now)

It is important that you give him a good brakfeast (when it contains carbohydrates, it gives a lot of energy) before he leaves home with a good multivitamin. This helped my son too

Also make sure that he sleeps at least 10 hours during the night

manal

Hi Indy and really anyone wanting to learn more about the importance of nitric oxide to health and survival in thalassemics,

L-carnitine and magnesium are separate supplements. Magnesium can be found alone or combined with calcium. There is an extensive article about L-carnitine at http://lpi.oregonstate.edu/infocenter/othernuts/carnitine/

These are some of the forms it can be found in as a supplement.

Supplements

Intravenous L-carnitine is available by prescription only for the treatment of primary and secondary L-carnitine deficiencies.

Oral L-carnitine is available by prescription for the treatment of primary and secondary L-carnitine deficiencies. It is also available without a prescription as a nutritional supplement; supplemental doses usually range from 500 mg to 2,000 mg/day.

Acetyl-L-carnitine is available without a prescription as a nutritional supplement. In addition to providing L-carnitine, it provides acetyl groups, which may be used in the formation of the neurotransmitter, acetylcholine. Supplemental doses usually range from 500 mg to 2,000 mg/day (95).

Propionyl-L-carnitine is available in Europe but not the U.S. It provides L-carnitine as well as propionate, which may be utilized as an intermediate during energy metabolism (94).

We have had quite a few discussions about L-carnitine here. I recommend that all thals take L-carnitine, but it does have an added benefit when added to the hydroxyurea program. Chelation can remove one serious threat to the heart in thalassemics, but L-carnitine and related supplements can help to counter the other cause of heart failure in thalassemia and the pulmonary hypertension (PHT) that is so common in thal intermedia and major. Nitric oxide (NO) is depleted in thalassemia and this leads to severe damage to the ventricles of the heart and also leads to PHT. L-carnitine helps to counter this NO depletion. Chelation alone is not the key to long term heart health in thalassemia. Diet and nutrition play very important roles. Ensuring that the nutrients that are rapidly depleted in thalassemics are replaced is very important to long term health and quality of life, and NO is one of the most important things to be working on.

There is a short list fo foods that are high in L-carnitine in the article mentioned above. Another list can be seen at
http://www.l-carnitine.org/natural-sources.php

L-Carnitine natural sources

Many foods provide natural sources of L-Carnitine where people can consume it through their everyday diet. Rather than taking supplements, you may get this amino acid from different foods, such as those mentioned below (amount per serving may vary, check nutritional information related to L-Carnitine for more details on this matter). Dairy products and red meats generally contain the greatest concentrations of it.

Apricots
Artichokes
Asparagus
Avocado
Bacon
Bananas
Beans
Beef (steak, ground)
Bee pollen
Brewer's yeast
Broccoli
Brussells sprouts
Buckwheat
Carob
Chicken breast
Cod fish
Cooked rice
Corn
Cottage cheese
Eggs
Garlic
Ice cream
Kale
Legumes
Lentils
Millet
Mustard greens
Oatmeal
Okra
Orange juice
Parsley
Peanut butter and peanuts
Peas
Pork
Rye
Seeds (sunflower, sesame, pumpkin, etc.)
Tempeh
Wheat bran
Wheat germ
White bread
Whole milk
Whole wheat bread



Diets

A vegetarian (or vegan) diet tends to lead to less daily ingestion of L-Carnitine, sometimes as low as 1 mg/day. Those who have a balanced, omnivorous diet often consume anywhere from 20 to 200 mg in a day.

Other sources

Aside from natural food sources and plain supplements, L-Carnitine is also found in items like some energy drinks and over-the-counter vitamins.

Any nutrient that helps to increase NO levels can be important for thals. Interestingly, tea which we know is an iron chelator and also a strong antioxidant, also serves to increase NO levels.

http://lpi.oregonstate.edu/infocenter/phytochemicals/tea/index.html

Endothelial function (blood vessel dilation)

Vascular endothelial cells play an important role in maintaining cardiovascular health by producing nitric oxide, a compound that promotes arterial relaxation (vasodilation) (19). Arterial vasodilation resulting from endothelial production of nitric oxide is termed endothelium-dependent vasodilation. Two controlled clinical trials found that the daily consumption of 4-5 cups (900-1,250 mL) of black tea for four weeks significantly improved endothelium-dependent vasodilation in patients with coronary artery disease (20) and in patients with mildly elevated serum cholesterol levels (21) compared with the equivalent amount of caffeine alone or hot water. Improvements were noted in comparison to an equivalent amount of hot water. One of these studies noted that caffeine, provided at an equivalent dose to that of tea, had no short-term effects on endothelium-dependent vasodilation, suggesting that non-caffeine components of black tea may be responsible for the reported short-term vasodilatory effects. Indeed, flavonoids contained in tea may exert such effects (22); for more information, see the article on Flavonoids. Several small studies have suggested that green tea, or its major catechin, EGCG, may have similar vasodilatory effects (23-25). The beneficial effect of tea consumption on vascular endothelial function could help explain the modest reduction in cardiovascular disease risk observed in some epidemiological studies.

Arterial relaxation is the key. In PHT, the artery between the lungs and heart loses flexibility due to low nitric oxide levels, resulting in the heart having to work harder to pump the blood. Maintaining the flexibility of the arteries and the heart proves to be just as important to survival in older thals as is chelation.

@Andy

Thanks for sharing info about L-carnitine. I had been to the hematologist and he gave a thums up to Ady (my son). He said the organs are OK and only an hemogram has to be done.

He also recommended wheatgrass for Ady along with Hydrea. I have got wheatgrass powder which I will start giving from today.

He has also changed the dosage of hydrea. Its 500 mg capsule one everyday from Monday to Thursday. My son weighs 20 kgs. Is the dosage OK?

@Manal

Hi Manal...thanks. i have read all your discussion and am happy to find you because both of us are on the same boat. Yes, we can share more about hydroxyurea.

@Dori.....Hi and thanks............

Hi Indy,

I think what we have learned from Manal's experience is that there is no "right" dose of hydroxyurea. To find the right dose takes trial and error. Manal's son recently had is dose lowered and his Hb rose! I believe this has much to do with the combination of L-carnitine and maybe more importantly, finding that balance where hydroxyruea stimulates fetal hemoglobin production while suppressing the production of the abnormal red blood cells. I think the best thing to do is to continue to revise the program until optimal results are seen.

I also want to make sure you are aware that the typical Indian diet is loaded with herbs and spices that have great positive value, so make sure your son continues the Indian tradition of diet and avoids as much as possible the refined food diets that are being introduced from the west.

hi indy,
it seems that you are from new delhi,
can you refer me some good hematology doctors  in delhi region.

thank you.