For me years of chronic transfusion dependence went by and I counted my blessings: more than 530 units of blood in total since 2008, with a stable transfusion interval of 10-18 days since 2010.
My treating professor initiated an invitation for me to a special clinic, where I underwent an MRI in autumn 2017. They scanned my heart, liver, pancreas and my bone marrow. The results were devastating. My view on iron overload broke with the medical report. I still have not fully recovered from what I was told. I would be very lucky, the radiologist said, if my heart was still free of iron. But my liver and my pancreas show a five-fold iron overload, also my bone marrow is full of iron. Another obstacle for
haematopoiesis in addition to my blood disorder.
Where do we go from here? Heading towards diabetes, liver cirrhosis or even hepatic cancer? My heart is iron-free, yes, but the pancreas is strongly loaded. No reason for long-lasting joy as the pancreas takes up similar iron species as the heart, only earlier. Iron overload of the pancreas serves as an early and robust marker of prospective cardiac risk.
It is neither an option for me to increase the dosage of the iron chelator – this could be toxic for my kidneys for instance - nor to reduce the number of transfusions – this would destroy my quality of life and would send me into severe anaemia. I’m stuck.
As so often in the course of my disease there is a diagnosis without any therapeutic consequences. That’s frustrating.
On the medical report I more recently read ‘severe hemosiderosis of the pancreas and the liver’ amongst my other serious health problems. However, my liver values (GPT, GOT) are not yet elevated. Not yet? I am anxious and confused: that’s the patient’s perspective.
Only now I am finding out that for any one patient, the predictive value of ferritin is quite poor. Two patients with identical total body iron burdens can have vastly different serum ferritin levels. Many factors affect the relationship between iron overload and serum ferritin levels. But still the ferritin level generally correlates with both, total body iron stores and clinical outcomes.
Serum ferritin therefore remains the most commonly used metric to monitor iron chelation therapy. Mainly because its measurements are inexpensive in comparison to MRI. However, the biology of circulating ferritin is poorly characterised and hardly comprehensible for non-physicians and non-scientists.
The way iron is regulated by the body relies on a regulated network of systemic and cellular mechanisms for the acquisition, transportation and cellular utilisation of the metal. I very well understand that these metabolisms are completely disturbed in case of strong iron overload.
Chelation Therapy: Good or Bad?
At the end of this article I want to make clear one thing: the outcomes of chelation therapy in Myelodysplastic Syndromes are not bad. There is a positive impact on survival, without doubt. I probably would not be alive any more without Deferasirox.
There are retrospective studies clearly indicating a significant improved survival in patients who received regular iron chelation. Transplant outcomes are also far better after iron chelation therapy. And in case there is a cure, high iron stores can be drained or at least reduced by a most effective phlebotomy therapy.
So yes, there are definitely some solutions for iron overload, if you are in the right cohort. But we urgently need more innovative iron chelation therapies.
Bergit writes a regular column in ‘MDS Europe’, the online home of the MDS-RIGHT project and the future hub for all European MDS information and guidance.
This article appeared first in MDS Patient Support Group Newsletter May 2018