As a practising specialist in internal medicine with 34 years of professional experience at the San Fernando General Hospital, and as a past president of the Council of the Medical Board of Trinidad and Tobago (2004-2010), I am calling on the Medical Association of this country, through its president, to set the record straight with regard to the utterances of the Minister of Health, Terrence Deyalsingh, who continues to insist that the hereditary haemoglobinopathy called beta thalassaemia major, which is what has been reported in the media that the child Shannen Luke suffers from, is not a life-threatening illness in this country.

His statement is patently untrue. The minister has stated in the Parliament that he has obtained five independent professional opinions from doctors in this country and Barbados in support of his position. Further, he has insisted that he has formed his opinion based on the recommendation of the board that advises the Children's Life Fund. I am also, therefore, calling on this board to publicly outline their position regarding the advice given to the minister as it pertains to this specific issue.

Having not been a party to the details of this child's diagnosis, I am guided solely by media reports. All of the reports that I have read concerning the issue of this unfortunate child's illness consistently attest to the fact that she has been diagnosed with beta thalassaemia major.

Beta thalassaemia major is a hereditary haemoglobinopathy that results in variable phenotypes depending on the extent of the patient's inability to synthesise non-alpha globin chains. Based on media reports, including an excerpt of a referring letter from the child's attending haematologist taken together with what the Minister of Health has said in the Parliament, one can reasonably conclude that this child suffers from beta (0) thalassaemia which refers to mutations of the beta-globin locus that result in the absence of production of beta globin. This is to be distinguished from beta thalassaemia intermedia and beta thalassaemia minor.

Only the last of these mutations can truly be considered to be non-life-threatening in the context of the present state of development of the health services in this country.

Beta (0) thalassaemia is a rare illness with a reported annual global incidence of just one in 100,000. Such children usually present with severe anaemia during the first two years of life. These children require repeated and regular transfusions of red cells which, in turn, if not managed properly with commensurate parenteral iron chelating therapy, e.g., deferoxamine (Desferal), administered through a continuous infusion pump device, will lead inevitably to a systemic iron overload with resultant organ failure.

Are infusion pumps routinely and reliably provided by Mr Deyalsingh's ministry today?

Can he say that these patients reliably receive a supply of deferoxamine?

How often are they transfused and how safe are these red cell transfusions? Inadequacy in any one of these aspects of treatment poses a threat to life!

Furthermore, poorly transfused individuals with this disease suffer from obligatory resultant conditions that reduce their life expectancy considerably. On the other hand, regular transfusions in the absence of addressing iron overload similarly lead to a range of pathologic entities, e.g., diabetes, hypothyroidism, hypopituitarism, adrenal insufficiency and most frequently cardiomyopathy.

How on earth then, can the Minister of Health insist that this illness, such as it is reported to be, is not life-threatening?

See the article here:
Shannen's illnesslife-threatening or not? - Trinidad & Tobago Express

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