advice on spleenectomy

Dear All

Wated to update you on my son Prat's condition and also wanted some advice from you guys.

Prat has been on Hydrooxyurea for almost 4.5 years now ( Nov 12, 2002 onwards). Since then he has got 2 transfusions. However this yar due to viral infections his hb has gone down from 7.5 to abt 6 and we had to transfuse him 2 times. Also he had anotherbout of viral infection and his hb went down + his WBC also went don to the  dangerous levels. Doc had stopped his hydrea for one month to asses wether it is the hydro which is bringing down the WBC or wether it is the viral infection. Anyways after a gap of 3 weeks hydrea was atarted again. Still his hb is  not coming up,he is 5.8(Oct 23, 2007). Doc wants to give hydrea   some more time and is going to do another CBC on Nov 13, 2007 . However Prat's haemtologist has said us to consider splenectomy, He has inidcated that the time has come and we shud move in that direction.

I spoke to Mr Khalifa about it, he wasnt very encouraging. i am in double mind right now, wether I shud go the transfusion way or wether we shud do away with the spleen. Wht kind of problem i can encounter in the long run.

Also I am still exploring the possibility of Recomorn and L Carnitine. Am not sure bout the side effects of these if there are any.

Will appreciate your expreinces and advice onthis matter, it will  help me to take the right decision.

thanks

Puja

Hello Puja,

Splenectomy has shown effectiveness in many transfusion dependant Thal. Intermedia by making them non-transfusion dependant without the use of HbF inducers for long time to come.

What is his Serum Ferritin level. Is his spleen too big? A spleen is a useful organ that keeps blood clean of dead cells and certain bacteria but it can become troublesome by becoming overactive and start to destroy healthy cells too. With it's removal that issue is solved but you would have to take regular antibiotics to fight bacteria otherwise your son will get sick.

I hope that your son's blood counts come back to normal soon.

Hi Puja

What is the size of his spleen and before the viral infection what was the average of his HB ?

Manal

Hello Manal & Sajid

His spleen was 2 cms enlarged when he was 2.5 years old  and now it is about 3-4 cms . I think Spleen enlargement is not a cause of concern here for the Doc. It is the Hb which is not getting to its normal level of 7.5 as it normally reaches when he is on hydrea.

Thanks

Puja

Hello,

If my daughter is in this condition, and given the spleen is not hugely enlarged and hopefully functioning properly than I may go against the Spleenectomy. I am always in the view that one procedure led to another, spleenectomy has its disadvantages as Sajid mentioned. Blood transfusion has its setbacks as well. The question is which decision you want to take. I suggest you to fully investigate how other patients are doing after Spleenectomy, are they transfused free, are there any other side effetcs etc. If the benefits outweigh the risks than go for it.

Hi Puja,

As far as i have consulted with Hemos in India they said that spleenetomy is not advisable for small age, as kids are more prone to infection and spleen help to maintains clean blood. After 10 or 12 yrs. of age it is more advisable.

But its also true that in many Thal intermediate after spleenetomy they maintains reasonable HB without transfusion.

Are you giving wheatgrass juice to Part.? It could be rally helpful to him.

Pls. keep us update. I hope Part’s HB come back to normal soon.

My good wishes are always with you and Prat.

Dimple.

Hi Puja,

Don't rush into a judgment about a splenectomy if the spleen itself, is not large or inflamed. Other means should be tried first to raise the Hb level and a splenectomy should be looked at as a last resort. Keep in mind that this low Hb was caused by a viral infection and even though Prat's Hb level has not yet risen to its previous level, it does not mean that it won't. An aggressive approach to raising the hemoglobin level and protecting the red blood cells from premature death should be undertaken. Folic acid, vitamin E, wheatgrass in extract form, resveratrol can all be safely used along with hydroxyurea to boost Hb. L-Carnitine is a natural supplement that is an amino acid synthesized within the body. It is very safe and studies have shown it to extend the life of red blood cells. It would be advisable to try it.  Recormon is a drug used to boost red blood cells. It is a brand name of erythropoietin (EPO). Recormon has many possible side effects but it is used successfully to boost the blood supply. You can read about it at http://www.medsafe.govt.nz/Consumers/CMI/r/Recormon.htm

I would also suggest trying to boost Prat's immune system. If you google "boost immune system" you will find many pages such as the following.

http://www.microsoft.com/canada/home/healthandwellness/2.2.25_boostyourbodysimmunesystem.aspx

Boost your body's immune system
Super foods help combat an assortment of ailments

By Cheryl Embrett
   
   

One of the best prescriptions for good health is also one of the easiest to follow: Watch what you eat. Every day, new studies prove that eating the right foods regularly can help reduce the risk of cancer and heart disease, banish the blues and increase energy and vitality. Here are 10 nutrient-packed "super foods" that should top everyone's grocery list.

1. Broccoli
This is one mean green! Broccoli is chock-full of the phytochemical sulforaphane, which has been making headlines because of its potent anti-cancer properties. It's also a rich source of beta-carotene (good for the eyes and immune system, among other things), fibre and vitamin C.
Toronto registered dietitian and author Liz Pearson was so convinced of the health benefits of this "King of Cruciferous Vegetables," that she called her first book When in Doubt, Eat Broccoli!: But Leave Some Room for Chocolate (Penguin Books, 1998). For fast, easy and delicious ways to add more vegetables — including broccoli — to your diet, visit 5 to 10 a day.

2. BlueberriesIf you haven't gone blueberry picking since you were a kid, here's a reason to start again. In a peer-reviewed study of 100 common fruits, vegetables and nuts, presented in the Journal of Agricultural and Food Chemistry, these little blue jewels had one of the highest antioxidant capacities (the ability to combat cell-damaging free radicals that can lead to heart disease and cancer). And, like cranberries, they appear to fight off urinary-tract infections by preventing E. coli bacteria from sticking to cells in the urinary tract.
John Stanton, president of the Running Room Canada, says he always starts his day with a big bowl of fresh fruit, including blueberries when they're in season. "It's light, it's healthy, and then I'm ready to run." For more on the power of blue, including tasty recipes for everything from wild blueberry chicken breasts to blueberry sorbet, check out Wild Blueberries.

3. Flax
"Ground flaxseed is the one thing I make sure I eat every day," says Mairlyn Smith, co-author of The Ultimate Healthy Eating Plan: That Still Leaves Room for Chocolate (Whitecap Books, 2002). Smith says flax is a great source of both soluble and insoluble fibre (which makes it a natural laxative), as well as plant lignans, which may reduce the risk of developing hormone-sensitive cancers, such as prostate and breast cancer. Flaxseed is also high in Omega-3 fatty acids, which are known to protect against heart disease. She eats a flaxseed muffin or sprinkles a tablespoon of ground flaxseed on her cereal every day. For more information on the health benefits of flax, including delicious ways to cook with it, visit the Flax Council of Canada.

4. Garlic
Since ancient times, physicians have used the "stinking rose" to treat a number of health conditions, including heart disease. Garlic contains allicin, a phytochemical that may lower cholesterol and make blood platelets less sticky, cutting the risk of clots. Mississauga, Ont., naturopath Rana Singh swears by the immune-boosting, cold-fighting power of the humble garlic bulb. He recommends chopping or crushing two or three cloves of fresh, raw garlic and adding it to whatever you're eating. For information on the health benefits of garlic and great ways to cook with it, visit The Garlic Information Centre and Garlic Sleuth. (Note: Consult your doctor before including more garlic than is customary in your diet.)

5. Green tea
Green tea is loaded with powerful antioxidants, which may protect against cell damage that leads to aging, and help prevent heart disease and cancer. Green tea contains a polyphenol known as epigallocatechin gallate (EGCg), which in animal tests has been shown to prevent the formation of tumors. Researchers at Purdue University found in laboratory tests that EGCg killed human breast cancer cells but did not kill noncancerous human breast cells.
Paulette Bourgeois, author of the Franklin the Turtle series of kids' books, drinks green tea several times a day. "It's not only healthy, it's soothing," she says. For more information on the health benefits of tea, visit the Tea Council of Canada.

6. Skim milk
"The one food I consciously remind myself to consume and monitor regularly is milk, since I know that I may not drink enough of it in the course of my regular routine to keep my bones strong," says food writer and cookbook author Dana McCauley. She's not alone. Most women don't get enough calcium, and one in four white (rate is lower in black and Hispanic women) women will develop osteoporosis. Studies have found that increasing your calcium intake can also help relieve the symptoms of PMS.
To meet your daily quota, reach for three servings of milk or milk products. After a tough workout, make it low-fat chocolate milk, advises McGill University fitness coordinator Jill Barker. "It has the requisite amount of carbs and protein needed to promote optimal recovery in the working muscles." Visit the Dairy Farmers of Canada for more tips on adding calcium to your diet.

7. Salmon
Fresh or canned salmon is one of the best sources of the celebrated Omega-3 fatty acids, which can help prevent heart attacks. Studies have also shown that Omega-3 may have the ability to offset depression, as well as protect against inflammatory diseases such as arthritis. "I try to eat salmon twice a week, as do my kids," says nutritionist Rosie Schwartz, author of The Enlightened Eater's Whole Foods Guide.
Concerned about recent reports of high PCB levels in farmed salmon? Click here to read a recent article from Health Canada's website.

8. Soy
Protein is one of the nutrients that most women don't get enough of — they should have a protein-rich food every three to four hours during the day to keep energized, says dietitian Linda Barton. "Soy is perfect since it's a plant-based protein that's low in saturated fat." Soybeans and soy protein products, including tofu, miso, tempeh and soy drinks, also contain phytoestrogens that may slow the growth of some cancers, lower cholesterol and offer some protection against osteoporosis. Visit the Soyfoods Association of North America for more information and recipes.

9. Spinach
When asked to name one of her favourite super foods, Toronto registered dietician Leslie Beck praises spinach. It's loaded with energy-promoting iron and folate, a B vitamin that prevents neural-tube defects in the fetus. It is also important in red blood cell formation, protein metabolism, growth and cell division. The leafy green is also one of the best sources of lutein, an antioxidant that benefits eye, skin and cardiovascular health. For more information on the health benefits of spinach, visit Wholehealthmd.com.

10. Tomatoes
Research has found that tomatoes, especially cooked or processed ones, can lower the risk of some cancers and heart disease. Experts attribute this to lycopene, a potent antioxidant and the pigment that makes tomatoes red. A Harvard University study found that men who regularly ate tomato-based foods had lower rates of prostate cancer.
"We love tomatoes," says Diane Clement, who founded the popular Tomato Fresh Food Cafe in Vancouver. She and her husband, Dr. Doug Clement (both former Olympic athletes), start their evening meal with their favourite tomato and bocconcini cheese salad. "The juicy tomatoes, loaded with lycopenes galore, are the perfect healthy jumpstart for any BODY," she says. For more nutrition facts and recipes, visit California Tomatoes.

* Note: Now that you're savvy to the health-promoting foods you should be eating regularly, start collecting tasty recipes that feature them, then combine them in your own digital recipe book.

Updated January 2006

Not every suggestion will apply to thal, but use whatever methods you can to boost his immune system and to raise his Hb level before deciding on a splenectomy. There is no going back once it's done and unless it is giving serious problems, the spleen is usually best left alone.

Hi Puja

I am completly against splenectomy -though i am not a doctor-  because i don't think his spleen is that enlarged. I think you should try every possible HB inducers in addition to the hydrea especially the resveratrol. By the way i have just known from a friend giving her NON transfused kid the reveratrol and had increased the F HB of her 2 years kid from 20% to 30%.

but my concern is that, you mentioned that you have been on hydrea since 2002, don't you think a Hb of 7.5 on hydrea is still low ? What is the opinion of the hema, is that the maximum the hydra can do


Manal

Hi Manal

Sorry forgot to mentione Prat's ferritin is 150 at the moment. It has incereased form 100 to 125 to 150 in last  3 to 4 years.

Also i would like to get your advice on how to take Recomoron. As I will suggest to my Doctor.

Pls advise on L carnitine, and how to take it as well.

Thanks

Puja

Dear All

Thanks for ur advice. I am going to listen to your advice and defer spleenectomy for a while.  As I am not convinced yet aboutit myself.


I am considering to try Recomoron and L Carnitine as suggested. Pls advice the procedure so that i can discuss with my docotr. i hv spoken to him and he wil like to know the  procedure as well as how is it give. Cos where Prat is being followed up they donot hv much expereince abt it. So do guuide me the procedure , side effects if any.

Pls suggest wether L Carnitine and Reservatol. Is it available in India easily.

Thanks

Puja

 

Hi Puja

Concerning Recomoron, i have no idea cause i don't use it .

Concerning L-carnitine, Ahmad is taking it from the first day he was diagnoised. First he took it twice daily for a month then twice daily 6 days/week and then finally he is taking it twice daily 5 days in a week. They are in the form of drops and the dose is one full dropper.
L-carnitine is a natural substance that is originally found in the human cells and it has no side effect at all except that the child can be a little more active . It can be taken with hydrea

Manal

Hi Puja
Sorry I read this thread late.. and I was aware of your sons condition.
If he is Thal inter.. dont you think the Hb maintained is still low? Will not such a low Hb hamper his growth?
Regarding splenectomy.. well.. I dont think 4 cms is going to cause a problem yet.. and once an Organ is taken out, it cannot be replced. So unless the docs are sure it must not be removed.
But yes, DR. weatherall had once told me that, if the spleen is removed early, then Thal inter and even Thal E could live without blood transfusions.. because they can then settle down to a low Hb.. but then they might not be that active etc.
Why dont you start transfusions for your son? If his Hb is low, then dont you think.. transfuions time to time to maintain a proper Hb would help him?
All the new medicines are not proved yet.. they are all in experimental stages.. so why not go the conventional way?

Shikha

Hello To All

Manal - Thanks for the advice on L Carnitine. i think I will also try it.  Do u hv any idea then who is using Recomoron, and whom shud I talk to it about. I will discuss it with Priya but if there were more people, atleast we can understand the drug better, Form wht i discussed with prat's Haemo, it has been used much in that  hospital on thal patients. So no feedback  on it so far. When was Ahmad diagnosed9 I mean how many years back).

Manal / Priya / - As we are all on hydrea, I want to know if Doc's hv said  how long can we use hydrea, can we just go on and on or is there a safe time to stop. Prathas been on it for 4 years.

 I am confused why doc is going the Spleen ectomy way - wether it is to stop hydrea  totally.? cos his spllen is still not so enlarged tht it could be a cause of concern. Alternatively, maybe the low hb must be worrying. i cannot understand.

Shikha - hi , Prat 's 7.5 was able to maintain his normal height n weight, therefore Doc was maintaining him on hydrea. But this year the hb has fallen to bleow 6.66 and 5.8 and he got 2 transfusions.  looks  as if it is due to viral infection. Cos his WBC also had gone way below the danger level's so we had to stop hydrea. He has been resumed on Hydrea but lets see, next test is on  Nov 15. Hopefully we can come up with some good results.

Thanks to all.

Puja

Hi Puj

Ahmad was diagnoised on July 10th, 2006 and according to my hema, hydrea will be taken forever as long as the liver and kidney functions are normal and the WBCs count are in the safe range.

Here in Egypt, there is someone i heard about him from my hema who has been on hydra for 18 years.

Actually, i don't know anyone who is taking the Recomoron

Manal

Hi Puja

Priya started taking hydrea from jan 06, but she was taking (one capsule daily) hydrea for 2 months and then one month rest. but from aug 07 she has stared regularly.

 how long time you can take it? Its true as Manal Said.

Take care

Dimple