Q&A with Dr. Ellis Neufeld

October 16, 2014 – CAF periodically receives questions from patients on a wide range of topics related to thalassemia and its treatment.  We have shared a few of these questions with Dr. Ellis J. Neufeld, the Chair of CAF’s Medical Advisory Board and Associate Chief, Division of Hematology/Oncology at Boston Children’s Hospital. Dr. Neufeld has kindly answered these questions below.

Dr. Ellis J. Neufeld

Dr. Ellis J. Neufeld

1.  What is darker colored urine an indication of in thalassemia patients?
People have been fascinated by urine color for thousands of years.  There’s no single answer to this question, and as for many medical questions, it depends why one is asking. Here are some important things to know.

Generally for persons with thalassemia or without, concentrated urine is darker than diluted urine, and if something is coloring the urine more than normal, this effect will be magnified. Ordinarily the most concentrated urine is first morning void, after not drinking all night.

Many thalassemia patients are used to seeing different color urine from medications like iron chelators.  Deferiprone (Ferriprox) turns the urine orange.  Patients on deferoxamine (Desferal or generic) will have reddish urine when the drug (which is colorless by itself) combines with iron.

Darker yellow to light brown urine in thalassemia is from the bilirubin from broken down red blood cells.  This might be especially pronounced in thalassemia intermedia (non-transfusion-dependent thalassemia) or in transfused patients shortly before a next transfusion is due.

Urine that is truly the color of cola or very dark tea is not normal, and could signal increased breakdown of red blood cells (much more bilirubin than normal). Particularly in the few days after a transfusion, or if a person is feeling more tired than usual, this kind of dark urine should prompt a call to your healthcare provider right away.  A check of the urine and blood tests can be reassuring or point to a problem if one has arisen.

Red urine not related to foods (beets) or deferoxamine or laxatives  (ExLax, phenolphthalein) may reflect either bleeding  or hemolysis (breakdown of blood cells) and should always be investigated.

2.  If we know that orange juice can help in the absorption of iron, should we NOT be taking Exjade with orange juice?
Vitamin C intake and levels in thalassemia should be sufficient, but not excessive.  One glass of OJ a day, or ~100 mg of extra vitamin C a day, is just about right.  It’s fine not to take Exjade with orange juice too, but then there needs to be some other way of assuring vitamin C sufficiency, because chelators work better with adequate vitamin C present.

3.  Drinking tea is supposed to inhibit iron absorption; does that include caffeine-free teas?
TanninOnly tea with tannins (the brown bitter stuff in black tea) impairs iron absorption and one has to drink a heck of a lot of tea to have this effect.  Herbal teas, say, may have no tannins. But decaffeinated black tea has a normal amount, only the caffeine is removed.

4.  Have there been any studies that have shown that taking a B-complex vitamin daily can slow the breakdown of red blood cells?
Not to my knowledge.  I have not investigated in detail, but we get B vitamins from our food pretty well.  B vitamins like thiamine and B12 and folate are absolutely required to make blood, but that’s a different story.

5.  Can you address whether Exjade should be split into 2 equal doses taken every 12 hours (rather than one every 24), and how combining it with food can affect its effectiveness?
a. “Should be” is a discussion best had with your hematologist.   Exjade CAN BE split into two doses a day, and they don’t necessarily have to be equal, so that if you use three tablets daily, you might split 2 and 1.  There are several theoretical reasons why splitting twice daily might be advantageous.  First, there is less load of the drug all at once. This helps prevent stomach aches and loose stools in some patients on high doses. Second, although on the average, the drug can last up to around 24 hours in the blood, some patients have shorter drug “half-lives” in the blood, and splitting the dose means coverage through more of the day. Third, in a few published abstracts, and in the experience of many treaters, Exjade may be more effective split twice a day (or it may allow higher doses, which are more effective, if it allows less GI side effects).  So at our center we offer or urge twice a day in case of abdominal symptoms or not good-enough effect at higher dose.  Also, see the answer about combining with food (in part b of this answer). If it doesn’t need to be on an empty stomach, twice a day Exjade is much less of a burden.

b. In a study carried out at many expert centers around the world, sponsored by Novartis (the manufacturer), it was proven that Exjade is at least as effective takenExjadeFood with food as not.  As you know, the drug doesn’t dissolve well in water, that’s why it makes a chalky slurry. It dissolves better in fatty foods, and this may be why this is a good option. So we counsel patients  that they can crush the tablets and mix in most anything (but don’t cook or microwave it), and that’s fine. The DRUG LABEL still says to take on an empty stomach in liquid, but we believe this may be because the drug will soon go off-patent, and Novartis hasn’t gotten around to making this change on the label, perhaps for commercial reasons.

6.  Is clumping of platelets a side effect of thalassemia trait?
No.  Rare people have clumping of platelets that isn’t a disease but a lab artifact called “pseudothrombocytopenia” (pseudo as in not real), and your hematologist can sort that out by counting your platelets in a different anticoagulant (that is, a blue top tube instead of purple top).

7.  Are there tests other than creatinine levels to detect kidney damage early?
Yes. This is an important point.  The Exjade package label has specific information on testing for protein in the urine for example.  Exjade raises most everyone’s creatinine without causing significant kidney damage, and then does cause renal disease in a small subset of that group, not related to the creatinine alone.

8.  Is blood warming beneficial to decrease antibody reactions and/or antibody buildup?
In patients with temperature-specific antibodies to red cells, definitely.  In patients without such antibodies, no.  Your center’s blood bank would know this for you. There is no call for general blood warming for example.

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