Web directory of Maldivian blood donors

Thanks to Falim group

Maldives blood line is a web directory of names of blood donors with their names, blood group, addresses, contact telephone numbers and last reported donation date.

Maldives blood line is not new. It has been there for some time now. I strongly recommed that all current donors and future list their names and keep their information updated on this directory. This is a tool that could be very handy, especially for Maldivians with Beta Thalassaemia major.

Q&A: Safe Ferritin levels?

Your questions answered.

Your Questions:
What is in your opinion a safe Ferritin level for a patient with beta Thalassaemia major?

My Answer:
Ferritin levels are the most commonly measured body iron storage measurements. While it may not be the most ideal measure of iron level in the body, it has continued to be a very useful tool because of the relative ease of measuring it.
It has been a regular practice to use changing patterns of Ferritin levels as a measure of the body iron status. It is important to note that one single measurement of Ferritin level, in itelf, is less useful than trends or changes in Ferritin level over a period of time. I think that target Ferritin level for patients with beta thalassaemia major should be based on the benefit that level would bring. That is the positive effect on the prognosis in terms of reduction in risk of complications and improvement in survival.

• Ferritin levels above 2000 micrograms/Litre for long periods of time are known to increase the risk of multi-organ damage as a complication of iron overloading. Although used in some places, target Ferritin levels of around 2500 micrograms/Litre are not ideal because of this reason.
• Ferritin levels between a 1000 and 2000 micrograms/Litre are a bit better. However, there still is an increased risk of organ damage if Ferritin levels remain above a 1000 micrograms/Litre for a long period of time.
• Ferritin levels below a 1000 are, based on current data, better targets because the risk of organ damage are so significantly reduced that it is very close to no additional risk.
• The high end of "normal" Ferritin level for the general population is around 150-300 micrograms/Litre dependent on age and gender. It is quite possible to achieve this level. There are several people who have been able to safely reduce their Ferritin levels to this level and maintain it over long periods of time. However, currently there is a bit of concern whether achieving such low Ferritin levels is of significant extra benefit than having a level of less than a 1000 micrograms/Litre. There have been reports of increasing incidence of Desferal side effects in individual who have very low Ferritin levels. This could perhaps be reduced by using lower doses.

My current practice is to encourage patients with beta thalassaemia major to first try to get their Ferritin levels to below 2000 micrograms/Litre. Those who can achieve this will be encouraged to lower it furthur to less than a 1000 micrograms/Litre. And once that is possible have it reduced and maintained around 500 micrograms/Litre.

I think it is very important to communicate this entire plan to the patient at the very beginning, so that they can themselves monitor their progress and know that each of the set targets are steps in achieving the 500 mark.

Having said that, from a prognosis point of view maintaining Ferritin levels anywhere below a 1000 micrograms/Litre is considered a good target. And the 500 micrograms/Litre is no universally accepted as the best Ferritin level.

A note to readers:
Reductions in Ferritin levels can take time. It requires a lot of effort from the patient as well as their families. It can be achieved with Desferal safely. I would like to remind readers that it is absolutely vital that you discuss with your treating doctor regarding setting targets and planning for lowering your Ferritin levels, especially with respect to your Desferal dosage. No change to treatment should be brought about without consulting your doctor.

Role of wheat grass juice in beta Thalassaemia major

Your Questions answered.

Your Question:
What are your thoughts on wheat grass juice and its role in reducing the transfusion need?

My answer:
Wheat grass juice has been used as an energy boosting drink for many years. There have been many “beneficial effects” attributed to it based on anecdotal evidence. The juice is made by blending (or extracting juice from) the green leaves and shoot of the wheat grass. This is the wheatgrass product that has been studied.

I am aware of other preparations (including: wheat grass extracts which have apparently had chlorophyll removed from it, nasal and oral sprays and powder forms) being “trialed” in different parts of the world.

The essence of the discussion is based on a 2004 Indian study[1], which reported that a subgroup of patients with transfusion dependent beta Thalassaemia major who took wheat grass juice had had a significant reduction in their transfusion requirement. I must emphasize here that this was only seen in a subgroup of patients and not in all of them.

There hasn’t been a lot written about wheat grass juice in medical literature. However, with the findings of the Indian study there has been an increased interest in obtaining more information on this “new find”.

The current thought is that there is an as yet unidentified ingredient in the wheat grass juice that is able to increase the production of fetal haemoglobin. Although significant, the increase in fetal haemoglobin, and the subsequent reduction in the need for transfusion was not enough to altogether stop transfusion for any length of time.

This is all very interesting and exciting from a research point of view and it could be the impetus for new research into newer forms of treatment in the future.

However, I must warn readers that at this stage all this is experimental. Nothing is conclusively proven. I believe that there is a benefit, but this has to be established through more research.

My current view is that wheat grass juice be treated as an experimental agent with potential benefits in the future. I do not recommend it as part of general routine treatment of transfusion dependent beta Thalassaemia major.

Those people interested in using wheat grass juice, or its other forms, should only do so under guidance and supervision of their treating doctor so that any benefit may be documented properly. The evidence to date is that the “beneficial effects” are seen slowly over a period of several months. Therefore it is not a quick fix.

The side effect profile of wheatgrass juice (or its extracts) is not well documented at this stage. It is claimed that the side effects are minimal and that the most significant of them is a skin rash (possibly allergic). Please be aware that natural products can and do have side effects contrary to what some people would like us to believe.

It should also be noted that chlorophyll, the green pigment in plants, is a source of iron. Therefore individuals with an already high Ferritin level should be extra vigilant with their Desferal injections should they chose to try wheat grass juice. They may in fact not be good candidates to try wheat grass juice. This needs to be discussed with the treating doctor in detail before any trial is started.

1. Marawaha RK. Bansal D. Kaur S. Trehan A. Wheat grass juice reduces transfusion requirement in patients with thalassemia major: a pilot study. [Clinical Trial. Journal Article. Randomized Controlled Trial] Indian Pediatrics. 41(7):716-20, 2004 Jul.

Permission obtained from reader to post question

Vitamin C in Beta Thalassaemia

Your Questions answered.

Your Question:
"Why are we given vitamin C?" a patient with Beta Thalassaemia major.

My answer:
The iron accumulated in the body as a result of long term transfusions and increased absorption from the gut is “stored” in the body in many different forms. Not all these forms are available for Desferrioxamine (the active ingredient in Desferal injection) to combine with. The iron that is in the forms that can combine with Desferrioxamine is called “chelatable iron”.

What Vitamin C does is to mobilize the iron in the different storage forms to become “chelatable iron” thus enabling Desferrioxamine to combine with it and remove it from the body.

This action of vitamin C is very short lived and therefore it is important to time its use with the Desferal injection. It is recommended that the vitamin C tablet be chewed and swallowed at the time of inserting the needle for Desferal injection (pump).

Please note that use of too much vitamin C can be very harmful in patients with iron overloading.

Permission obtained from reader to post question

Exjade gets FDA approval

The new once-daily oral tablet Exjade (Deferasirox, also known as ICL670) has been approved by FDA in the US.

Exjade is currently the only once daily iron chelator on the market. Its availability and approval for use in patients with chronic iron overload (from as young as 2 years of age) would revolutionise the management of illnesses like Beta Thalasaemia major.

Read the Novartis press release

Your question: Desferal injection technique

Your Question:
What is, in your opinion, the best way to inject Desferal?

My Answer:
I have had the experience of helping patients with Beta Thalassaemia major use Desferal injections by different injection techniques.

• Intravenous injections:
  I am personally very uncomfortable with patients using Desferal intravenously (direct injection into the blood via a vein) as an infusion. The risk of sudden high doses entering the blood stream and causing major problems is my worst fear with this technique. Theoretically, using IV Desferal in a hospital setting is relatively safer that at home. This is because in a hospital it should be easier to deal with potential problems than at home. I personally think that IV Desferal use is too intrusive, invasive, relatively unsafe and potentially troublesome. It is also a lot more challenging for patients to acquire the skills necessary to self administer Desferal IV. I personally don't recommend this method.
• Intravenous via a porta cath:
  I have seen some patients who have had subcutaneously (just under the skin) implanted catheter devices on their body into which they self inject (as a slow infusion) their Desferal doses. I consider this to be virtually the same as intravenous injection. It is definitely a lot easier in this case to gain IV access for injection, but it is associated with potential complications with the catheter itself. I personally don't recommend this technique either. Immediate side effects are common with both these techniques and are not very well tolerated by most patients who have tried it.
• Subcutaneous via pump:
  This is till date, the most widely used, most widely accepted and most widely studied injection technique. The Desferal dose is prepared in a syringe which is then "loaded" into the pump. The mechanical pump then slowly (over 8-12 hours) injects the medication via a needle inserted just beneath the skin. This is probably the safest way to inject Desferal. Most patients prefer to "wear" the pump at night allowing it to inject the medication when they sleep. The biggest hurdles in this technique are the incidences of local injection site side effects (swelling, pain, redness, itching…etc) and the need for about 10 hours on average for the injection. Some people more than others have serious injection site problems that hinder regular use of Desferal. Even considering all this, pump is perhaps the safest, easiest, most effective and most widely accepted injection technique for Desferal. I would recommend this technique more than any other (exceptions: read below).
• Subcutaneous as short infusions:
  Again the medication is prepared in a syringe. It is attached to a tubing at the end of which is a needle. The needle is inserted into the space just below the skin and medication injected slowly over 20-30 minutes.Although originally developed as a 10 minute "bolus" injection, I support the slower injection over 20-30 minutes and prefer to call it "short infusion". The total daily dose is divided into a morning and an evening dose. This is increasingly becoming popular with patients who weren't finding "the 10 or so hours" needed for the pump. They have one injection before going to work and one before going to bed. It is effective, generally safe (but less so than the pump) and less time consuming. The local injection site problems are similar to the pump technique. I would recommend this technique for anyone who is unable to use pump.
• Intra muscular injection:
  Painful and unsafe. I have met people who were on this injection technique in the late 70s (the then commonly used technique). I don't recommend this technique. I believe this technique to be only of historical significance.

Now that I have written all that, I would like to say that it really is not my choice what technique a patient uses. I would certainly recommend one or more techniques depending on circumstances, but it is ultimately the choice of the patient which technique he or she wishes to use. There are advantages and disadvantages to each of these techniques and individualised plans have to be the right way to go.

I would like to encourage everyone to discuss with their doctors before making changes to their medications. There are ways in which we can help minimise and control side effects or the severity of it. In case of a medication like Desferal, which I consider to be an essential life-saving medication for patients with Beta Thalassaemia major, it is important to do everything possible to help patients optimise their regular use.

It is not easy for the patients to adhere to this difficult treatment. But when it is an essential treatment, we must find ways and means to persevere.

Permision obtained from the reader for posting question.

Interesting question: Iron tablets.

Readers' question:
I am a Beta Thalassaemia carrier. I saw a doctor who said that I need to take iron tablets. I have been told by a friend that Thalassaemia carriers can’t take iron because it can cause iron overloading. Could you help me sort this out?

My answer:
Let me start by noting down some facts:

• Iron does not cure Beta Thalassaemia! It does not reverse the changes in the appearance of the blood cells caused by Thalassaemia carrier states.
  
• Anyone who is shown to be iron deficient should be given iron! This has to be shown by blood tests that actually measure iron levels and not through indirect tests.
  *Indirect tests (like demonstrations of smaller sized blood cells and pale looking blood cells) are not specific to iron deficiency. What is important for us is that these same changes can be seen with iron deficiency, Thalassaemia carrier states or both occurring together.
  *The direct tests measuring iron level are available in the Maldives.
  
• Patients with Beta Thalassaemia major have excessive iron in their bodies. This happens in 2 ways.
  Through blood transfusions and by excessive iron absorption from food.
  
• It can be dangerous for someone with iron overloading to take more iron (as with iron tablets).
  
• Beta Thalassaemia carriers, unless there is some other problem, generally do not have increased iron in their body. If they do become iron deficient, and they can if their diet is iron depleted or because of other problems, they would indeed need to be given iron.

I would suggest that you confirm from your doctor whether you have been shown to be iron deficient by blood test that directly measure iron level. If this has been established then you will need to take iron. Don’t hesitate to ask questions from your doctor. Doctors are legally and ethically obliged to answer any questions that you have related to your health.

I think it is important to known a person’s iron status before starting iron therapy. This is especially important in a country like the Maldives where the incidence of Thalassaemia is so high.
There are documented cases (not from the Maldives) where people with some forms of Thalassaemia (mostly alpha Thalassaemia- the genetic mutations for which have now been shown to exist in Maldives) have had their condition worsened because of secondary iron overloading resulting from use of iron.

Permision obtained from the reader for posting question.

My Project: Study of Desferal use.

My study enters data collections stage.

I have begun collecting data for my study on Desferal (Desferrioxamine) use among patients with Beta-Thalassaemia major in Melbourne. I will be looking at different aspects of use of the medication especially the adherence to prescribed regime and difficulties patients have adhering to the regime.

I hope to collect a similar set of data from the Maldives at a later stage for comparison.

I was asked to read this:

This URL from the Green Imperative, community outreach program by Banyan Tree Maldives was sent to me by a reader. http://www.banyantree.com/greenimperative/outreach.htm

I would like to thank Banyan Tree, Maldives for their efforts in helping Maldivian patients with Beta Thalassaemia major.

There are a few things that I MUST correct/ammend (or add comments to) on this webpage:

1. In the end of the first paragraph it reads "there is also a dire lack of iron...". This information is incorrect. In fact there is an excess of iron in patients with Beta Thalassaemia major. This is the reason why they need to use Desferal, the iron removing injection.

2. While it is true that many Maldivian patients with Beta Thalassaemia major die due to complications of the disease by around 20-30 years of life, this is not the case in most centers around the world. Patients indeed do live "generally well" into their late 50s provided that they receive adequate transfusion AND iron chelation therapy. Our patients need to be aware that they can live a relatively healthy life well over 20-30 years by being more compliant with their treatment.

I have sent an email to the web editor of the site regarding these errors on the page a few weeks earlier.

Thalassaemia publication

Alpha Thalassaemia in the Maldives.
Prevention of thalassaemia and haemoglobinopathies in remote and isolated communities—The Maldives experience
Author: Firdous, Naila Source: Annals of Human Biology, Volume 32, Number 2, March–April 2005, pp. 131-137(7) Publisher: Taylor and Francis Ltd
Click on the article title to read the abstract of the published article.

Read how Beta Thalassaemia is inherited

Thalassaemia is an inherited blood disorder. People who are affected by this illness are unable to make enough normal haemoglobin (Hb) in their red blood cells (RBCs).Haemoglobin is the part of the blood that carries oxygen to the cells of the body where it is used to release energy for the vital processes. All tissues of the body need oxygen. Hence without enough haemoglobin, and therefore reduced oxygen carrying power, tissues are starved of oxygen and energy, and cannot function properly.So how does someone get Thalassaemia? Read full article.

New URL for Dr Niyaf's supportGROUPS

I have moved the supportGROUP page to http://www.niyaf.com/supportGROUPS.htm. There have been minor changes to the appearance of the page but the overall content and feel of the site is unchanged.







The Thalassaemia supportGROUP URL is http://www.niyaf.com/supportGROUPS/thalgroup.htm.

Welcome to the Thalassaemia Support Group's BLOG

Here is an easy discussion board for everyone to post their thoughts.
As this is just the beginning, I would like to ask interested people to join the group. I would encourage people to use their own names. This will enable people to identify you and take your comments and suggestions seriously.

Lets start talking.