What is porphyria? Did George III really have porphyria?

If you’ve been avidly reading Queen Charlotte: The Bridgerton Tales over the past week, you’re familiar with the complex (and steamy!) love between Queen Charlotte and King George III. But more than romance, power and gossip are revealed in this high-society “Bridgerton” prequel. The film tells a story of class and marriage, while also exploring King George III’s agonizing illness, which at least some experts believe to be porphyria.

that’s right. “Queen Charlotte” is a fictionalized retelling of the story of Charlotte and George, but the real King George III has medical problems and at one point had porphyrins, a very rare genetic disease announced by Cleveland. It was suspected that illness was the cause. Less than 200,000 people in the United States are estimated to be affected, according to the clinic. (However, some people are asymptomatic, so some people may not be diagnosed.) The disorder is usually inherited from a parent and caused by high levels of naturally occurring chemicals in the body known as porphyrins. increase. This causes negative symptoms that mainly affect the skin and nervous system.

However, at this time, the idea that George III had porphyria is fairly widely disputed. The diagnosis first surfaced in his 1960s, when he claimed that two psychiatrists said his medical records indicated he had the condition. But other studies over the years have shown that porphyria may have been a misdiagnosis in George III’s case, and he may have had bipolar disorder instead, reports the Journal of Clinical Medicine. . But still, the show reignited interest in porphyria. Here’s everything you need to know about liver disease, including symptoms, how it’s diagnosed, and whether it’s treatable.

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What is Porphyria?

According to the Cleveland Clinic, porphyrias refer to a group of eight rare genetic disorders that affect the skin and nervous system as a result of the buildup of natural chemicals called porphyrins. Porphyrins are found naturally in the body and are needed to make heme, the molecule needed to make hemoglobin (the protein found in red blood cells). The Mayo Clinic points out that too much porphyrin in the body can be harmful.

There are also two main types of porphyria. Acute porphyrias have a rapid onset and primarily affect the nervous system, whereas cutaneous porphyrias (such as late-onset porphyria cutanea, the most common type of porphyria) primarily attack the skin. To do. Some types of porphyria can affect both the nervous system and skin, but they are less common.

What are the symptoms of porphyria?

Porphyria symptoms vary from person to person and type, but symptoms can range from mild to severe, according to the Cleveland Clinic.

People living with the cutaneous form of porphyria (which primarily affects the skin) are often hypersensitive to the sun, causing itching, blisters, abrasions, and scars on the skin when exposed to the sun. You will experience rashes, skin fragility, extreme swelling and irritation.

Acute porphyria usually causes a sudden onset of symptoms, usually lasting hours, days, or weeks, according to the Cleveland Clinic. Common symptoms include pain in the abdomen, chest, arms, legs, and back, urinary retention (inability to completely empty the bladder), constipation, nausea, vomiting, muscle weakness, seizures, confusion, and hallucinations.

In some people with porphyria, the presence of excess porphyrins, whether acute or cutaneous, may cause reddish-purple or brown urine.

How is porphyria treated?

According to the Porphyria Foundation of America, diagnosing porphyria can be difficult because many of the symptoms resemble other diseases and disorders, but it is usually diagnosed with a blood, urine, or stool sample, or DNA genetic testing. increase.

There is no cure or prevention for porphyria, but there are treatments and symptom management techniques based on the type and severity of symptoms. Sun protection is important for people with cutaneous porphyria, and doctors may recommend eliminating substances that are likely to cause symptoms, such as alcohol. Some people benefit from frequent blood draws known as therapeutic phlebotomy, which can reduce the amount of iron in the liver and minimize symptoms.

According to the Cleveland Clinic, treatment of acute porphyria usually includes infusion of heme and/or glucose by IV, which reduces the number of porphyrins produced by the liver. Treatment in more severe cases may include blood transfusions, surgery to remove the spleen, and liver and bone marrow transplants.

Some research suggests that maintaining a balanced diet and reducing carbohydrate intake can minimize symptoms. Smoking should also be completely avoided.

celebrities with porphyria

The exact number of people with porphyria is unknown, but some studies suggest that as many as 1 in 20,000 people may suffer from some condition. It is also not always confirmed whether any historical person was formally diagnosed with porphyria, although (apart from George III) a case was reported involving Prince William of Gloucester, a cousin of Queen Elizabeth II. It is

Mary, Queen of Scots and her father, King James V of Scots, are also believed to have suffered from acute porphyria as they were ancestors of George III and inherited the disease.

Some studies suggest that Vincent van Gogh had acute porphyria. This is due to all the signs and symptoms of his underlying physical and mental illness.

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