Heart Attack in sickle-cell disease and possible role of alcohol and over-transfusion?

Sickle-cell anaemia, “SS”, is just one of the 4 common phenotypes comprising sickle-cell disease, defined as possession of 2 globin gene variants at least one of which is the sickle gene.¹ The others are sickle-cell Haemoglobin C disease, “SC”, sickle-cell beta-thalassaemia, “Sβ-Thal”, and sickle-cell hereditary persistence of Fetal Haemoglobin, “SF_hereditary”. Ghana abounds in beta globin gene defects (qualitative and quantitative), which allowed me to study the clinical epidemiology of these 4 common phenotypes contemporaneously.² For non-technical readers I use the term AcheAche for Sickle Cell Disease in all its forms, ie the possession of an ‘Ache’ gene from both father and mother.

Dr Pavlü and colleagues wrote an article about a 50 year old lady (Jan 20 2007, p 246)³ which is inadvertently misleading because although sickle cell anaemia ‘SS’’ (which their patient has) is sickle-cell disease, not all sickle cell disease is sickle cell anaemia. In over one thousand consecutive sickle cell disease patients that I have personally followed up, although ECG changes occurred frequently during sickle crises in all 4 phenotypes it is my “impression that signs of acute coronary occlusion and myocardial infarction, reversible after crisis, are more a feature of Hb SC disease where blood viscosity is greater than Hb SS disease (Figs 25.4 and 25.5; Case Histories 84 and 85)”.²   

     One reason their 50-year-old woman developed myocardial infarction could be because of increased red cell mass from “intermittently needed exchange transfusion”.³ What was the low-level haematocrit that determined need for transfusion, and what was the upper level attained afterwards? In my opinion the 110 g/L Hb level that many haematologists in the UK and USA feel “SS” patients required for their steady state does more harm than good^{1 2 4}. I have both a sickle-cell anaemia (“SS”) patient who required to be bled to keep Hb level below 90 g/L otherwise he had thrombotic episodes² (Case history 21, page 482) and a “Sβ-Thal” adult whom I once sent to Professor Lucio Luzzatto for a second opinion, and who demanded periodic venesections (removal of blood) to get Hb level below 850 g/L to abort incipient sickle crisis. Therefore, when was this 50-year-old last transfused before her myocardial infarction? What was her serum Ferritin level?

     While I totally agree with the authors “myocardial infarction should be included in the differential diagnosis of chest pain in sickle-cell disease despite normal coronary angiography”³ I want information that can help prevent myocardial infarction in my patients. Circumstances are crucial in the welfare of sickle-cell disease patients.⁴ What, then, were the circumstances of this particular episode? What was this lady’s alcohol intake like? A 44-year-old Hb SC man I once described (Case history 85, page 503)² “drank Campari and brandy continuously for about 8 hours having had little to eat before going to bed. At 4 am the next morning (12.6.67) he was woken up with severe retrosternal pain ..”². Cardiologist Professor Silas Dodu not only confirmed myocardial infarction (ECG’s on pages 504 to 508 of my book)² but also made the correct phenotype diagnosis of Hb SC disease even before electrophoresis report arrived.² Warned off alcohol, he abstained for 12 months but “the patient died at home after a history of drinking spirits”.² Alcohol effect on the heart and lungs has been described in sickle-cell states⁵ so we need to know more about this lady.

Incidentally, insisting on raising transfusion levels of sickle cell disease patients to 110 g/dL (ie 11 grams per deciitre) as sometimes happens in the UK, continental Europe, and the USA can cause what I have come to call “hyperviscosity encephalopathy” which manifests itself with severe headaches, disorietaition, fits, and sometimes such mental symptoms as make doctors call in a psychiatrist. Not necessary. Just quickly remove a substantial amount of the transfused blood, to get the haemoglobin down to between 8 grams and 9 grams per decilitre. Watch the patient, more than you watch the patient’s blood.               

I declare that I have no conflict of interest except that I have a book ‘The Sickle Cell Disease Patient’ (Home Page) and I had 2 brothers and 1 sister with Sickle Cell Disease. My conflict of interest lies in the fact that any time I read about patients given Heroin or Morphine, or over-transfused, and causing complications, I assume them to be my own brothers and sisters. 

Felix I D Konotey-Ahulu