How does porphyria affect the blood?

Porphyria (por-FEAR-e-uh) refers to a group of disorders that result from a buildup of natural chemicals that produce porphyrin in your body. Porphyrins are essential for the function of hemoglobin — a protein in your red blood cells that links to porphyrin, binds iron, and carries oxygen to your organs and tissues.

What organs are affected by porphyria?

Porphyria occurs when the body cannot convert compounds called ‘porphyrins’ into heme. While all tissues have heme, those that use it the most are the red blood cells, liver and bone marrow. Porphyria can affect the skin, nervous system and gastrointestinal system. More women than men are affected by porphyria.

Does porphyria make you crave blood?

People with porphyria experience the desire to drink human blood to alleviate their symptoms (the genetic disease causes abnormalities in a person’s hemoglobin, a protein found in red blood cells), declared biochemist David Dolphin.

What color is urine in porphyria?

The terms porphyrin and porphyria are derived from the Greek word porphyrus, meaning purple. Urine from porphyria patients may be dark or reddish in color due to the presence of excess porphyrins and related substances, and may darken further after exposure to light.

What makes the blood red in porphyria patients?

No matter what kind of porphyria you have, it has the same root cause: a problem with how your body makes a substance called heme. Heme is part of the “hemoglobin” in your blood — proteins that carry oxygen from the lungs to the rest of your body. Heme has iron and gives blood its red color.

Where does heme come from when you have porphyria?

All types of porphyria involve a problem in the production of heme. Heme is a component of hemoglobin, the protein in red blood cells that carries oxygen from your lungs to all parts of your body. Heme production, which occurs in the bone marrow and liver, involves eight different enzymes — a shortage…

How can you tell if you have porphyria?

Porphyria is diagnosed through blood, urine, and stool tests, especially at or near the time of symptoms. Diagnosis may be difficult because the range of symptoms is common to many disorders and interpretation of the tests may be complex. A large number of tests are available, however, but results among laboratories are not always reliable.

How many types of porphyria are there in the human body?

The porphyrias are a group of disorders in which there is a problem with the production of haem (also spelled heme) within the body. Haem is used to make haemoglobin in red blood cells. There are seven different types of porphyria and in most cases they run in families (are inherited).