Methemoglobinemia is a rare blood disorder caused by inherited or acquired conditions, exposure to toxins, or dehydration. It causes excessive amounts of methemoglobin, which cannot transport oxygen, leading to anemia and organ failure. Treatment involves reducing methemoglobin through intravenous drips of methylene blue. Newborns and young children are especially vulnerable to the condition.
Methemoglobinemia or Met hb is a rare blood disorder that can be caused by an inherited or congenital condition, exposure to toxins, particularly nitrates, or dehydration, particularly in children. In this condition, methemoglobin, a form of hemoglobin, is present in excessive amounts. This particular type of hemoglobin cannot transport or bind oxygen, which means that the number of oxygen-containing red blood cells is reduced. Poor supply of oxygen containing red blood cells causes anemia and can be disastrous for organs, and when methemoglobin is present in large quantities, organs can begin to fail. Then the condition can be fatal, causing total heart failure when left untreated.
Usually, methemoglobinemia is noticed before organ failure, especially in fair-skinned people. It will cause cyanosis, distinctly blue skin. People who have this disorder look not just a little blue, but often very blue. Other symptoms include mental status changes, confusion, shortness of breath, dizziness, and headache. Continued accumulation of methemoglobin can cause unconsciousness, coma, and ultimately death. The diagnosis can be confirmed by checking the blood for methemoglobin, which should be less than 1%. Larger amounts confirm methemoglobinemia. Also, blood from the arteries looks brown instead of bright red.
The condition is treatable and may need to be withdrawn in people who have congenital or hereditary methemoglobinemia by attempting to reduce the methemoglobin so that the iron molecules are once again carrying oxygen. This is normally achieved through intravenous drips of methylene blue. As the treatment progresses, the skin begins to redden and the main organs of the body are better oxygenated.
While methemoglobinemia can be inherited, the incidence of heredity is low. Instead, the disease usually occurs after severe dehydration or exposure to toxins. In a 2007 episode of the Grey’s Anatomy spin-off show Private Practice, several sisters developed the condition after long exposure to nitrate fertilizers. This is actually a perfect example of how certain toxins can create the condition.
Newborns, especially those younger than six months of age, are also very vulnerable to methemoglobinemia if they contract diseases that cause dehydration. Young children are especially prone to dehydration from diarrhea or vomiting because their body weight is so low. Unlike adults, they can become dehydrated within hours of becoming ill, which can quickly create excessive methemoglobin levels. This is why it is extremely important to treat young children with severe diarrhea or vomiting immediately, especially if the condition does not clear up within hours and the child cannot tolerate or retain fluids.
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