Persons having inherited methemoglobinemia type 1 or hemoglobin M disease may be able to carry on their normal lives.
However individuals with hereditary type IIb5 cytochrome reductase deficiency have shortened life expectancy due to severe multiple physiological complications.
In persons with fair skin complexion, the diagnosis is easier requiring fewer tests as the bluish skin discoloration is apparent.
However in individuals with dark skin complexion and hyperpigmentation blemishes symptoms have to be studied and elaborate tests may be necessary for diagnosis and treatment.
Cyanosis and low O2 saturation reading around 90% may indicate methemoglobinemia.
The diagnosis of methemoglobinemia can be confirmed by test with multiple wavelength co-oximeter or multi-wave length pulse oximeter to directly measure methemoglobin.
Methylene blue in methemoglobinemia treatmentMethylene blue (methylthioninium chloride or C16H18N3SCl) is used in the treatment in severe cases of Methemoglobinemia.
Methylene blue 1% solution (10 mg/ml) 1 to 2 mg/kg is usually administered by the intravenous route.
This has to be done slowly for about five minutes followed by intravenous flush with saline. The IV administration of methylene blue may be repeated if necessary.
Oxygen supplementation is also done simultaneously.
Methylene blue functions as electron acceptor and makes the methemoglobin reductase enzyme to perform at five times the normal levels.
However in patients with the G6PD (Glucose-6-phosphate dehydrogenase) deficiency, an X-linked recessive hereditary disease, methylene blue is contraindicated.
In G6PD patients it is very dangerous to use methylene blue as it can lead to severe hemolysis.
Methylene blue, being a potent oxidant, can actually cause methemoglobinemia when administered at levels greater than 7mg/kg.
Ascorbic acid in methemoglobinemia treatmentVitamin C, being a potent antioxidant, reduces the level of methemoglobin in the blood.
Oral administration vitamin C at 500mg/d may be done on an ongoing basis when methemoglobin levels rise to more than 30%.
Oral methylene blue at about 200-300mg/d or riboflavin at 20mg/d also can be used instead of vitamin C.
Exchange transfusions and hyperbaric oxygen treatmentIn severe life-threatening situations exchange transfusions are resorted to save the life of the patient.
In G6PD patients and in individuals in whom there is lack of sufficient progress with methylene blue treatment, exchange transfusion becomes necessary to save the life.
In exchange transfusion, basically, abnormal hemoglobin in RBC is replaced with normal hemoglobin.
In hyperbaric oxygen treatment, by use of plasma dissolved oxygen, tissue oxygenation is achieved.
In infant metabolic acidosis, methemoglobinemia resolution lies in reversal of acidosis with intravenous hydration and bicarbonates.
Methemoglobinemia treatment must done by a qualified professional preferably in a clinic or hospital.
What is methemoglobinemia?
Causes and symptoms
Current topic: Methemoglobinemia treatment