What medications are contraindicated?
In principle, all drugs that increase the viscosity of the blood or impair the oxygen supply should be avoided. For example, sickle-cell patients should refrain from taking contraceptives containing estrogen, as these increase their risk of thrombosis. Drugs that act on the autonomic nervous system and narrow the vessels (vasoconstrictive drugs) or reduce the oxygen exchange surface in the lungs (parasympathetic mimetics, sympathetic colony drugs; e.g. certain beta-blockers) should also be avoided.
What should be considered during anesthesia?
A heart–lung machine is used in surgical management when the heart and/or lungs have a malfunction requiring surgery and have to be operated on or even transplanted. This means that it can be assumed that the affected body was already in a relative oxygen deficiency state before the operation, ergo the diseased erythrocytes have taken their sickle cell form under oxygen deficiency. The greatest danger is the hemolytic crisis: After connection to the heart–lung machine, damaged blood cells are removed through a filter.
It is important to note that the circumstance of sickle cell anemia therefore filters more blood cells than a healthy person. Strict monitoring of the balance between oxygen carriers (erythrocytes) and other components (proteins, plasma) is necessary. This can be achieved, for example, by transfusions (erythrocyte concentrates, EK’s for short).
It should also not be neglected that the blood cells suffer minor mechanical damage during their flow through the heart–lung machine. This fact is also taken into account in healthy patients (i.e. patients who do not suffer from sickle cell anaemia) and the viscosity of the blood is specially monitored anyway. Under strict surgical management, sickle-cell disease patients can also be connected to a heart-lung machine, but this should be supervised by experienced transfusion and intensive care physicians and should only be carried out in the absence of other treatment options.
All articles in this series: