My thalassemia minor findings in India

(We have been discussing this case via email).
I am still inclined to believe that you are having episodes of favism, not totally unheard of among beta minors in this group. The high uric acid and sudden drop in Hb that was experienced point to an outside influence that is causing sudden hemolysis. Is your particular thal mutation one known for a more unstable hemoglobin? That is a question that should be answered. The fact that your father also has high uric acid does make me wonder if he may be a silent thal carrier. I would still like a definite answer on whether the triplicated alpha gene has been tested for. This is not normally done in DNA testing for alpha thal and must be looked for specifically. Its presence would explain everything you have told me.

All,

A much expected stability has been seen in my health last 3 weeks. What changed in last 3 weeks?

i) My Thyroxine Sodium dosage increased very minutely.
ii) My body weaned out all traces of Febuxostat and Allapurinol by now
iii) Ayurveda prescribed medicines to cleanse the system internally (liver, intestines)
iv) Homeopathic medicine to tackle Hb, Uric Acid and Liver growth
v) Folic Acid 5mg a day, twice daily
vi) No consumption of Febuxostat and Allapurinol tablets to control uric acid

As usual, the B12 and Vitamin D dosage is going on as advised by Andy and levels seem to be stabilised.
Liv 52 syrup has started 10 ml twice a day from last 5 days.

Health is still not upto the mark but better than April 1st week. Bilirubin remains high.
But hopefully after 90 or 180 days, things could shine.

Hoping for the best.

Mid-June 2017 a CBC blood work was done with liver and uric acid.

Hb reached 10.4 g/dl now, hematalogist suggested to cut down folic acid dose to 5mg per day instead of 10mg per day
Uric acid is still hovering at 9.3, pain has now started reaching wrist joints, ankle joints, finger joints. Hematalogist suggested taking Allapurinol 100 mg per day, but I have not consumed any uric acid medication since 16th April.

SGOT and SGPT have reached near normal levels of 38 and 35.  What is disturbing is the Indirect Bilirubin continues to remain high at 6.25 (its come down from 6.98). Doctor said hemolysis is part of your cycle and indirect bilirubin will remain high".

Last few days Mumbai weather was excessive humid, my kidneys were not able to flush naturally inspite of water intake.
I feel that my kidneys have been overloaded in last 2 months due to excess hemolysis as I was taking folic acid 10mg per day.

Help on uric acid is hardly forthcoming from ayurveda and homeopathy from last 4 months.
At home I started consuming Flax seeds + sesame seeds mixture to control uric acid.
No major improvement.

As Andy stressed the importance of Vitamin E 400 mg per day, I have started taking the same since few days.

We have several thal minors reporting high uric acid and even gout. This can be directly related to the high turnover of RBC's. This says to me that these patients have a more unstable hemoglobin than thal minors who do not have this issue. This may be a function of the particular genes these people possess. I know in one case, the combination of beta minor and a triplicated alpha gene, which results in unstable hemoglobin being produced, is the cause. The triplicated alpha gene is seldom looked for in DNA testing and may be overlooked as the cause of high uric acid in some of these patients.

I would really like affected patients to share their stories with each other and see if there is anything common among them and also, if they have discovered any remedies for this problem.

I had some good recovery months during May, June and early July. Just mid-July got some bladder/prostate infection and urine stopped flowing. Prostate appeared normal. An infection was diagnosed. No debris found in locked urine sample.

Could there be any role of uric acid crystals for such infections and blockages due to uric acid debris in prostate/urethra?
Doctor ruled out such a thing.

Could uric acid crystals form stones in the kidney? USG showed nothing and a very normal kidneys.

@jsbhavsar - by no means am I a doctor however I have experienced similar situation to your uric acid and haemoglobin issue.

I have thalassemia intermedia and my haemoglobin generally runs much lower then yours. Normal around 7.0 - 7.8.
I only take folic acid 5mg once a day and Lamotrogine for my infrequent partial seizures.

anyhow;

My uric acid has always been high since birth and I got my first gout attack in August 2015. It was on my right toe.
After explaining the gout attack to haematologist which then referred me to a Rheumatologist who said the high uric acid was due to massive red cell turn over (thalassemia).
Rheumatologist mentioned there is no need to start allopurinol unless another attack occurs within a year.
Next attack occurred exactly a year later and rheumatologist recommended to try allopurinol to lower uric acid and prevent further attacks (levels were around 550ummol - 600ummol).
I delayed starting allopurinol until January 2017 and then started at 100mg once a day, uric acid dropped but not low enough so we moved to 200mg and then 300mg and finally 400mg.
We finally dropped the uric acid levels to 286ummol and everything was OK. Crystals started dissolving and I got a gout flare (which is usual when you drop your uric acid levels).
However with every increase of allopurinol we saw a slight drop in haemoglobin and by the time I reached 400mgs haemoglobin had dropped to 5.1.
Doctors weren't sure if it was the allopurinol or if there was an internal infection or if my thalassemia just naturally got worse.
I was too worried to continue with allopurinol so I stopped it. 2 weeks after stopping my haemoglobin climbed to 5.7.
Another blood test 3 weeks later and my haemoglobin went to 7.2 and a further week after that it went up further to 7.5.
My uric acid shot up to 760ummol at first and slowly went back down alone to approximately its original amount of 600ummol.
Doctors are not entirely convinced it was due to allopurinol but I haven't changed anything else, they said in rare cases it can cause anaemia but they are not sure that was the reason.
They said it could have also been a temporary internal infection, or the inflammation from the gout attack I got when I got to 300mg allopurinol.
Anyway, I am due another blood test next week to see if everything is still OK and back to normal.

My rheumatologist has now recommended I start febuxostat to try and control my uric acid but I am extremely worried in case the same thing happens again.
The problem is gout is a horrible insidious disease and it will get worse and worse over time if left untreated (gout attacks and arthritis) but I am also too worried to start febuxostat.

I don't know what to do - @Andy do you have any pointers on this one (tests to check if febuxostat is compatible, G6PD, etc)?



In answer to your questions:

1. Uric Acid crystals can cause kidney problems yes.

2. This is the problem with high uric acid levels, crystals will form and they will deposit in most joints and will cause pain everywhere. high uric acid also increases cardiovascular risks.

3. If your uric acid levels are very high it will be impossible to bring them down using homeopathy.
Good water intake helps flush uric acid and Cherries are also known to reduce uric acid levels. You can eat the cherries fresh or you can purchase concentrated cherry juice.
Low fat dairy products also help reduce uric acid levels (milk, cheese, etc.). Avoid very cold temperatures as this can cause quicker crystal formation (uric acid crystallises in lower temp).  

Let me know if you need further help with regards to uric acid and gout. (you can also visit this site for information on gout: http://www.goutpal.net).

dq,

My concern about febuxostat, is that the molecule contains sulfur, which is known as a potential cause of hemolysis in thals. Seeing that allopurinol  already apparently caused hemolysis, and it's the last thing you need, I would be hesitant to recommend trying a drug containing sulfur.

Because it is known that some foods, along with many chemical fumes, can cause hemolysis in thals, my only suggestion is to keep food diaries and try eliminations to see if anything improves. There may be foods you both are reacting to, and only eliminating one food at a time can help determine which food(s) may be an issue. This has to include spices, etc, including common foods like onions and garlic. Those two should probably be part of an elimination trial at the same time, where normally, one food at a time is eliminated. An article I ran across recently made me wonder about the effects of high garlic use in humans. It is well known that they can both cause hemolysis in cats and dogs. Could high consumption be an issue for someone with red blood cells that are more fragile than normal? Mice and humans share many characteristics. If this happens in mice, should we be looking at it as a possible cause of unexplained hemolysis in humans? This lengthy article can be seen at http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0015358

Hi Andy,

Totally agreed. The only thing that gives a little confidence factor was the fact I had inflammation at the time (and still have some now) which the haematologist said might be the reason although I am not entirely convinced. The main drop in haemoglobin seemed to have gone in line with dose increases. Getting the exact dates will be tough however I think when I was on 100mg of Allopurinol my body seemed to cope ok with no hb drop although I was only taking 100mg for a month before increasing the dose so I wouldn't know if the drop would have occurred without even increasing the dose further.
Another possibility could be with every increase of Allopurinol a haemoglobin followed but its impossible to tell without starting allopurinol again.

With regards to garlic and onions, I eat tons of the stuff, I love it unfortunately.. but I have always had tons of the stuff and never noticed a hb drop, I admit I may have slightly increased my intake during the allopurinol months?

The food elimination trial does seem logical however I didn't really change my eating habits for haemoglobin to drop in the first place?
The only things I did do was increase my intake of apple cider vinegar and cherries both of which supposedly helps with uric acid reduction / removal.
I also increased my water intake significantly which I believe can dilute blood, but I doubt it can reduce it from hb 7.0 to hb 5.1..?
Can the inflammation of my gout attack really have that effect and drop hb?

Allopurinol also has anaemia as one of the rare side effects. Where does that leave us thal gout prone people, what alternatives do we have?


Assuming one takes Luspatercept in future does the mechanism of working increase blood cell life reducing high right turn over?
Ultimately if that was the case, you have a cure for gout in thalassemia patients right there right?

hello Andy, just a little bump on this thread.. cheers..

Luspatercept greatly reduces the ineffective erythropoiesis, which is one of the two goals of transfusion. This would greatly reduce the RBC turnover, which would mean far less bilirubin, fewer gallstones and less spleen enlargement (in those with spleens). It also reduced the iron load in patients.

There are a few people here reporting this same very high uric acid problem. I am hoping that comparing notes can help. What is causing the high blood turnover? The RBC's are fragile to begin with, but what is the reason for their demise?

Hello Andy, thanks for the info on Luspatercept.


It seems as if this is a serious problem amongst thals. I assume the demise has something to do with the general length of time they live in thal patients being much shorter.
The body seems to constantly create to replenish as the general lifetime of the red cells in thals live less so during the death of these cells they release their uric acid?

I mean correct me if I am wrong on this but isn't the issue with thals that they don't produce healthy cells thus unable to carry much haemoglobin?

Yes, many of the RBC's produced by by thals are defective and carry no useful hemoglobin. In addition, the RBC's are more fragile in general, and can break down prematurely, especially when exposed to certain substances, like sulfur or chemical fumes. With the cases of high uric acid, the only explanation I can see is that the fragile RBC's are breaking down quickly, resulting in a high turnover of RBC's and the waste products that come with that.

Things were fairly OK in July-August. Late August I had stress and fatigue. I did come out of it.

Since last 8 days, I have more stress and fatigue. It was very hot and humid in Mumbai around 14-15-16 September. I sweated a lot.
From 18th September I started keeping my blood pressure at 110/70, 110/65 too. I took rest one day (yday) and saw the doctor. He advised that it was 110/74 and would first start Enerzal (energy drink with electrolytes). Things did not improve much. Saw doctor again today. I had angina type pain, low feeling and low energy when speaking on phone calls etc. Basically no energy left in body kind of.

Doctor advised I continue on electrolytes for few more days three times a day. Also Pantoprazole + Domeperidone capsule started for few days.
It was 120/80 today and during work pressure was 110/80.
Eyes are looking quite pale yellow. Tired, fatigue and stressed.
For a few weeks I had stopped the Vitamin E gel cap. I started it gain today.

I have gone through the Febuxostat and Allapurinol stages in March-April. I will suggest thal patients not to engage on those drugs.
One of my own childhood doctor in interior India suffers from high uric acid level. He takes Allapurinol 300 mg tablet. His kidneys have stopped functioning since last 8-10 months.

I really dont know what should be done for below things:
- Entire day watery mouth feeling due to high bilirubin
- Excessive stomach bloating symptoms
- Joint pain in morning to afternoon hours
- hardening of stomach line, the softiness is long gone

Is Lesinurad (Zurampic) the new medicine the future? Is it better than Febuxostat and Allapurinol?

A Mumbai Homeopath doctor claims to have cured a heavily suffering liver patient completely well in few months of time. His Fees are extremely high, very high. When hospitals had claimed for a transplant, the patient is walking with normal lifestyle.

Is Homeopathy the way forward? I have been taking it since 6 months and have not seen much improvement.

Zurampic can cause kidney failure, so it may not be the improvement you're hoping for.

I have to feel that the heat, air pollution and noise of Mumbai all add to the stress. I have a dear friend in Mumbai who is thal minor and she reports so many problems to me that I have to believe the environment if a major factor in her health.

I just cannot imagine and find hard to describe how hard have these 3 weeks been on my health.

First it was low BP and pain in abdomen.
Then it was the Vitamin D3 shot 600,000 IU taken on my waist on 26 September morning. 48 hrs later, I was in a Cardiology hospital ICU. I had sharp acute pain on my back side exactly behind my heart. Immovable, immobile, inability to sit or walk for 80 minutes.

Expert Doctors on that day diagnosed it has "D3 fat soluble oil based injection put strenuous load on already weak liver, resulting gastritis".
Next 48 hours went normal but weak.

28-Sep went for a very detailed Hematologist and Cardiologist evaluation at a large bone marrow transplant medical centre.
as blood was being drawn there, I caught a viral fever that afternoon.
Within 3 hours of that I had 102.5F fever, pulse 125 and BP 109/58.
Could not stand, walk move. Was taken to a hospital by Dad.

The doctor diagnosed it as "regular viral fever" and suggested 3 days antibiotics "Cefadexine 325" along with Paracetamol 650 mg 4 times a day. I barely recovered from that illness on Oct 3 or so but not fully.

The prolonged weakness from last 3 weakness is still very bad on me. I am feeling far far weak than what it was in April-May this year. The viral fever did much harm to me, it did far more bad on my red cell health as well. Such high fever came on my body almost after 18-20 months.

I am desperately looking at Homeopathy treatment now to boost my Hemoglobin and reduce uric acid and increase bodily strength.
I want to zero down on two or three homeopathy dilutions for 2 to 3 months to boost Hb and red cells and reduce uric acid.