Royalty and Porphyria

{George III in his coronation robes}

King George III suffered from the genetic disease porphyria, which had also tormented Mary Queen of Scots, who passed it on to her son, King James I of England.

We now know that there are at least eight types of porphyria, and that the clinical manifestation of each type is not the same. A common feature of all porphyrias is the accumulation in the body of "porphyrins" or "porphyrin precursors." These are normal body chemicals, but they do not normally accumulate. Each type of porphyria is determined by deficiency of a different enzyme. These enzyme deficiencies are usually inherited. Symptoms of the disease can include (but are not limited to) photosensitivity, strong abdominal pain, port wine-colored urine, muscle weakness or paralysis in the arms and legs, and behavioral changes including anxiety, irritability, and confusion. The interruption of nerve impulses to the brain can cause the development of psychiatric symptoms such as depression or delirium. (For more information, contact the American Porphyria Foundation .)

George III had a particularly severe form of porphyria. His first attack occurred in 1765, four years after his marriage to Queen Charlotte. Further signs of the disease showed up in 1788-1789. From 1811 to the time of his death in 1820 the royal patient became progressively insane and blind. He was nursed in isolation, and kept in straight jackets and behind bars in his private apartments at Windsor Castle.

Other members of the far-flung royal family who suffered from this hereditary disease were Queen Anne of Great Britain; Frederic the Great of Germany; George IV of Great Britain--son of George III; and George IV's daughter, Princess Charlotte, who died during childbirth of complications of the disease.

In Sickness and in Health

Steve and I were married in June 2008. As customary, part of our vows were "in sickness and in health". We didn't know that sickness was just 3 weeks away.

I always thought of myself as healthy. Growing up I had colds, flues, ear infections... the usual. In my teens I had various times of stomach pain, cramping, migraines... all consistent of PMS... or so we thought.

However, there have been what my family calls my "medical mysteries". When I was 13, I woke in the middle of the night with terrible pain in my abdomen. It took me 4 hours to get out of bed and get help. I was rushed to the local ER, but by then was starting to feel better. After hours of testing and questions, the doctors never did find a reason for my pain and said I probably had a ruptured ovarian cyst and to follow up with an OB/GYN. So I did.

During high school I had more "mysteries" when I would wake up in the middle of the night and have just enough time to run to the bathroom or just sit up before vomiting uncontrollably. Stress. This is what we told ourselves. After all, high school is not easy.

At 19 I again had "mystery" abdominal pain for a few days. My mom figured I was constipated. I realized I was in trouble when I went to my college class and my professor asked if I was ok. She said I looked green and was rocking in my chair due to pain. She sent me home. That afternoon I was taken to the ER. The doctors couldn't find a reason for my pain, so figured it was my appendix. I was taken into the OR for an emergency appendectomy that night. Pathology reports show that my appendix looked normal.

Two weeks after my appendectomy, I was again at the hospital with abdominal pain. This time, since I didn't have an appendix to blame the pain on, they said it was my gallbladder. The only thing I had eaten out of the ordinary was avocado. Well that must be it. I was diagnosed with, get this, "gallbladder sludge". No real explanation of what it was. But I could have my gallbladder removed and the problem would be solved.... no thanks. How about you, Doctor, keep the avocado, and I'll keep my organs (or what's left of them anyway).

So that takes us up to age 23 when I get married. It was a lovely wedding and Steve and I were excited to vacation for 10 days on our honeymoon. Unfortunately, I got sick on day 3. Nausea and diarrhea with one bathroom is quite a way to introduce yourself to married life. I was sick the entire time and even 2 weeks after we returned to Atlanta. Within a month of our wedding, I got up for work one morning and noticed thousands of tiny red dots under both eyes. What are these and where did they come from? I didn't know, but I looked ridiculous. Even heavy concealer didn't cover them. They lasted 2 weeks. This was my first sign there was something deeper brewing.