Members & Followers

Tuesday, February 22, 2011

More King George III

On that same note about King George III, we had another recent occurrence. that gave the APF and porphyria more publicity. Recently, the movie, The King's Speech, was the story of one of the heirs of King George who became King after his brother abdicated, "for the woman he loved." He was the grandfather of the present Queen Elizabeth. Remember that story!!!

Several years ago , producers of the movie called us regarding the porphyria connection to the royal family. The King has a stammer, so they wanted to know if pophyria was a cause. I told them this was another family issue.

Regardless, they gave the APF credit in the movie credits. Which reminds me, we have had more television exposure than any other rare disease. If you watch HOUSE, you will have seen porphyria featured many times. To illustrate , here is a list of shows below, as well as two movies:
MOVIES
*The Madness of King George (1994) & (2005) Movie
* The King's Speech ( 2010) Movie

TELEVISION

*House, Honeymoon, Season 1: episode 22

*House, Fools for Love, Season 3: episode 5 (mentions porphyria)

*House, Finding Judas, Season 3: episode 9

*House, Don’t ever Change, Season 4: episode 12 (mentions porphyria)

*House, Guardian Angels, Season 4: episode 4 (mentions porphyria)

*House, Whatever it takes, Season 4: episode 6 (mentions porphyria)

*House, Itch, Season 5: Episode 7

*House, Emancipation, Season 5: episode 8 (mentions porphyria)

*House, Let them eat cake, Season 5: Episode 10



*Scrubs, My Ocardial Infarction: Season 4, Episode 13

*Scrubs, Kelso’s last stand,Season7: episode 9

*Scrubs, My Dumb Luck, Season 9: episode 7

*Scrubs: Outta here like Vladimir: Season 8, Episode 4



*CSI Las Vegas, Justice Served Season 1 Episode 21

* CSI Las Angles, Once Bitten , Twice Damned Season 2 Episode ?



*ABC News Specials, Medical Mysteries Series, episode 2*

*ABC News Desiree Lyon interview

*ABC Prime Time CEP segment



*Mystery Diagnosis, The Sickest Patient in the Hospital and Terrifying Tremors, Season 5

*Mystery Diagnosis, The Boy Who Kept Swelling, Season 6 episode 6



*Mystery ER, Seeping through the cracks/Purple Haze, Season 1: episode 3



*Secret History: Purple Secret in Search of Royal Madness, Season 6: episode 6



*Montel Williams: Porphyria with Lauren Warren

*Montel Williams, Rare Diseases, Leppert Family



*Travel Channel, Documentary Romania



*Learning Channel Dec 09 Porphyria, another myth in the making.



*Fox News Desiree Lyon interview

*Fox News Vampires , A Medical Myth Nov 2009

*Fox News King George, Madness or Arsenic July 2005



*BBC news Desiree Lyon



*Sanjay Gupta CEP patient, Kasey Knauf CEP



*Anderson Cooper 360 CEP



*CNN documentary on CEP patient, Kasey Knauf

*CNN interview Desiree Lyon (CNN HERO)



*Dr. Oz EPP patient, Craig Leppert



*National Geographic; Feb 2010 Six Ways to Stop A Vampire



*Court TV



*Gray’s Anatomy, Time Warp Season 6 episode 15



*Castle, , Vampire Weekend, Season 2 , Episode 6

_________________________________________________________________________________

Parade Magazine, Desiree Lyon Howe story

Parade Magazine, Porphyria, Rare Disease Feature

New York Time Magazine-Sunday, Perplexing Pain : Porphria Article

Tuesday, February 15, 2011

King George III and Porphyria and You

Since our last blog mentioned that one way to gain media attention is to enlighten them on the significance of Porphyria and the Revolutionary War. Namely, King George III, often known as Mad King George, was the reigning king of Great Britian and Ireland from 1760-1820. The APF has a letter signed “GR” to the Lord Chancellor, Kew, Feb. 23rd 1789 and is the one single document in English history in which medical and political history are joined. It is difficult to imagine a more important autograph letter marking a happier occasion in George III’s life-long battle with the illness that entirely unjustly, saddled him with the epithet “Mad King George.”

George III was born in 1738, first son of Frederick, Prince of Wales and Augusta. He married his beloved wife, Charlotte of Mecklinburg-Strelitz in 1761. The couple produced a prolific fifteen children: nine sons and six daughters. George III succeeded his grandfather, George II, in 1760 and became one of the longest ruling Monarch of the British crown. Sadly, George III died blind, deaf and mentally ill at Windsor Castle on January 29, 1820.

Since George III ruled during the American Revolution, he was thought by many historians to have had a significant impact on Britain's loss to the revolutionaries. His mental and physical lapses were blamed for much of the mishandling of the war.

King George lost his American colonies south of Canada by the Peace of Paris in 1783 and was notified by his Lord Chancellor, Edward Thurlow. During that period, George had a number of bouts of illness which made it difficult for the leaders to take timely action, including military decisions. His illness created enormous complications for his kingdom. Much pressure was also placed on the royal physicians, who were distressed over their utter inability to diagnose or alleviate his painful malady. The physicians meticulously recorded each attack and in some, described him as “arbitrary, capricious, given to fits of rage and to long periods of depression.” Occasionally he even had to be restrained physically.

Although he had his first attack at age twenty six, the illness began again when he as about 50 years old. Sir George Baker was the main royal physician at the time, so when King George began suffering another severe bout, he summoned Baker. The

king first experienced severe abdominal pain and constipation, followed by weak limbs, a fast pulse, hoarseness, fever and strange dark red urine. Over a short period of time, he began experiencing a multitude of other symptoms: headaches, visual problems, restlessness, delirium, convulsions, and insomnia. Because his attacks waxed and waned, Parliament was continually debating his ability to rule and continually threatening to wrest his kingdom form him. All of his symptoms were similar to those described by other porphyrics; severe abdominal pain, breathing problems, a rash and muscle cramps.

Part of the problem was that in George III's time, doctors were permitted to do very little to what they called the “royal body,” so they had to base their diagnoses on what the king told them. Dr. Baker prescribed the standard purgative of that day: castor oil and senna. Instead of the purgatives relieving King George of his symptoms, they made his condition far worse. When the pain intensified, he was prescribed laudanum, which also did not ease the pain. During later attacks, according to notes made by the physician, King George became so agitated and was talking so incessantly and violently that his physicians had to confine him to a straitjacket.

Quizzically, they also mentioned that he had dark reddish-purple stains in his bed clothes during these sieges of physical pain and what they referred to as periods of “madness.” Since the royal physicians were not permitted to conduct extensive physical examinations, they had to greatly depend on King George’s descriptions of his symptoms. On one particular occasion when he was suffering an exceptionally bad mental relapse, Parliament openly debated his ability to maintain his position as King. Shortly thereafter, he spontaneously recovered, further validating the idea in the minds of many that he was emotionally ill.

By the middle of November, 1788, the accumulated symptoms of the King’s illness had led to government-wide fears for his life and sanity. This forced the King’s first minister, William Pitt the Younger, to propose a Regency Bill, which would have transferred the King’s power to the improvident Prince of Wales, who was the enemy to both Pitt and the King. As this debate proceeded, George III’s ministers, convened in a cabinet meeting by the Prince of Wales, decided to move the King from Windsor to Kew, a luxurious place of confinement and attempted marginalization nonetheless. Through all this, Lord Chancellor Thurlow, who had been one of the King’s staunchest proponents of his absolute sovereignty over his American colonies during the Revolutionary War, kept his options open with both sides.

Months passed and the King remained at Kew Palace. While there, he endured a humiliating regime at the hands of Dr. Francis Willis, including confinement in a straight jacket and fixed restraint in a chair if he did not precisely follow Willis’ directions. Because of the nature of porphyria, George III recovered naturally from this attack, which had begun to recede noticeably by January, 1789. When the Lord Chancellor saw that the King’s recovery was likely to prove complete, he jumped fully into the King and Pitt’s camp, and moved to adjourn the Regency Bill’s third reading on February 19, 1789. Four days later George III decided that he could notify all his allies – and serve notice on all his political enemies – that he had fully recovered and was ready to resume full sovereignty.

He suffered a relapse thirteen years later, then again three years after that. The symptoms appeared in the same order, beginning with abdominal pain, fever, and weakness and progressing to mental difficulties. Finally, an attack in 1811 placed him in an apparently permanent stupor. That was the proverbial “last straw” for the Lords of Parliament. At that reoccurrence his son, George, the Prince of Wales had him dethroned. While he lived for several more years, he continued to experience further episodes of porphyria.

In the letter George III wrote to the Lord Chancellor, in which he relates the details of a meeting he had just held that very day with the Prince of Wales (with whom a successful meeting, in particular, implicitly testified to the King’s complete recovery of his nerves) and intimates that Thurlow should convey the same to “Mr. Pitt whom I propose seeing tomorrow Morning”. By conveying these and related details, and at the same time thanking “the Nation at large to whom I am so much indebted for the Support and anxiety shewn during my illness,” George III consolidated the support that saw him successfully through the Regency Crisis, and through his next two porphyria attacks (1801 and 1804), as well.

In other words, the letter, written from Kew, which was the scene of both the worst of George III’s ordeal, and his recovery, explicitly marks George III’s forceful, yet diplomatic re-entry into politics and the business of government. His final phrase of the letter concerns “supplies for the Current year,” i.e., the 1789 royal budget. Within days of his letter, George III had fully resumed his duties as King.

Medical historians Ida Macalpine and Richard Hunter first proposed that the King George had porphyria in their historical monograph, George III and the Mad-Business. It had long been suspected that King George III had porphyria, but this assumption was not proven until scientists exhumed the bodies of family members and performed DNA studies.

I also had a personal experience over this questions. A British Lord came to visit a friend of mine in Houston. He wanted to have a typical Texas experience, so she ask Dick and me to take him to the hill country of Texas where we had a lake home. He was a lovely guest and was thrilled when he we took him to the first dance hall, in Gruene . He had fund doing the two step and watching all the terrific dancers whirling in a circle.

The next morning over coffee, he told me he loved the dance hall and the Wimberly, Texas "pie social" better than all other experiences. I was so sick that I could not share his delight. He told me that he didn't share his delight and my sickness was porphyria.....not a hangover as I don't drink. He asked me what was wrong and I said my usual, "Nothing." But he insisted that something was wrong at which point I said it was a disease that he most likely had never heard of and I said, "Porphyria. "

" I know it well , " he said. " My wife was in the lineage of George III and her brother had pophyria. " What about that for a coincident !!!!!

You can Google the subject and even see paintings of King George and others in the Royal Family who are deemed to have the disease. It makes for an interesting story for the media so it is something to gain the attention of a local reporter.

Tuesday, February 8, 2011

There was Fun, Too!!!

I wanted to include in the materials we were developing for doctors and patients the issues that were key to patients. So I interviewed many of them and asked them their thoughts on the subject. I would like to take a moment to share with you a few special patients who were in the Rockefeller Research program with me.

Herta was another extraordinary Porphyria patient at Rockefeller. When Herta arrived at the hospital, everyone knew it. She walked down the halls with a bigger than life personality. Everyone was a friend. Herta was vivacious, smart, interesting and had such a commanding presence that she drew instantaneous attention. I liked her and her husband, Herman, from the moment we met. Unfortunately, Herta was also very sick with Porphyria at times and often had to visit the outpatient clinic at the hospital.

Herman drove Herta into the city from New Jersey for her appointments. Since he was making the round-trip to and from the city, he took on the job of bringing urine samples from other New Jersey patients to the research lab at Rockefeller. You can imagine all the names we created for his automobile and his new volunteer service, our favorite of which was The PeeWee Delivery Service. Laughter , the Good Book says , is the best medicine and in this case it was definitely a major factor. The arrival of the PeeWee Delivery Service, was a much awaited event that precipitated howls of laughter each time. Plus, Herta and Herman transported more than urine when they came to the hospital. They brought overflowing love and if we were lucky, they also brought oodles of goodies that Herta had cooked. I never asked, however, if they kept the food and urine samples in the same ice chest.

Herta’s son, Roland, was producing videos at the time and approached me about filming a video on Porphyria. I jumped at the chance and assured him that we would distribute it through our new American Porphyria Foundation. After retrieving the specific permissions from the patients and doctors who were going to participate in the film, we finalized all of the other details before starting the video. Interestingly , the basics are still true today, so although we a new DVD , Porphyria Live, the older version is a treasure, too.

I was so impressed with Roland that I told him, "You are such a special young man that I think one day, you will be a producer on the Today Show or a similar program. Sure enough, not too many years later, he became one of the one of the Today Show producers and is still in television production.

Roland first set up the camera in the recreation room at Rockefeller Hospital. Most of the patients in the hospital gathered to watch the filming, whether they had Porphyria or not. I was the film’s narrator and Herta and Frank were the key patient interviews. They conversed about their respective cases of Porphyria and other men and women in the New York area who were patients at Rockefeller discussed the photosensitive Porphyrias. In years to come, we have tried to keep this as a prime feature at the APF, namely, working together, making what patients have to say very important , and soliciting the opinions of our members.



Our APF members are the reason we are a foundation. Since I don't take a salary and never have , I can say it is not for funding on my part. Also, our porphyria experts have very limited research funds and don't receive any funds for consultation with doctors or patients, so no one can say they benefit either. What we do is because we know pophyria is rare and many patients need help desperately.

Thus, we all need to help each other. Certainly, your donations help. But also it is helpful for you to do whatever you can to help educate your doctor , other patients and , of course, your community. You may ask , "How can I accomplish this? "

Let me give you a few suggestions:
Contact your local media and offer your story, particularly if it is National Porphyria Awareness Week or if there is a very exceptional angle to it . For example, near the 4th of July, you might suggest a story on pophyria because of the historical significance with Mad King George III and the Revolutionary War as historians feel that his ignoring the colonies was partly responsible for the British defeat. The APF will provide suggestions, materials and other help you may need to garner media attention to your story or to porphyria.

Another suggestion is to advance awareness of porphyria in the medical community. gain medical attention at your hospital , like a seminar at your hospital and teach your own doctor.

Watch the next blog to learn about King George and what he has to do with porphyria.

Tuesday, February 1, 2011

The APF and You.

When the two of us decided to move forward to start an American Porphyria Foundaiton, we first discussed the fact that without a group of experts behind us, we would have absolutely no validation. Fortunately, we knew some of the porphyria experts in the country, so we began contacting them to discover the others.

We set the parameters to determine what consitiutes an expert and we decided on certain parameters. the following were a few of the parameters:

They wrote the medical text book chapters on poprhyria.

They did the porphyria research.

They already had a reputation in the medical world as the "pophyria experts."

They attened the national and international porhyria conferences .

They were known by their european counterparts as porphyria experts.

They had publications in the most prestigious medical journals, like New England Journal of Medicine.

They were known as porphyria experts amoung their peers.

They had many porphyria patients and not just a few .

With these very strict parameters in place, we were easily able to identify THE EXPERTS. They were an elite group and were amazingly knowledgeable about the porphyrias . Our next step was to contact them and arrange a meeting. This was not easy as they were spread across the country and we ha no funds to bring them together. So we investigated where the next medical meeting would be held that many of them would attend. Chicago was the place. The onew who were already attending were not a problem. Those doctors who were not attending the convention paid their own way.

The plan we devised that day turned out better than we could have imagined. Our first step was to invite a group of prominent Porphyria experts to gather in Chicago to discuss our objectives and to solicit their input and participation. We both agreed that the effectiveness of our future foundation was dependent upon the caliber of the physicians/researchers involved and the quality and reliability of the educational materials we would produce. The name we chose for the new foundation would be The American Porphyria Foundation.

The financial burden of starting such a foundation was on us, including gathering the names and support of Porphyria specialists around the country to start the plan of action. This would be a very costly but necessary endeavor. Fortuitously, the Digestive Disease Week convention was scheduled to convene in Chicago shortly after James and I started planning our mini-conference. Digestive Disease Week is the major convention for gastroenterologists, liver specialists and other related medical specialties, which meant that most of the Porphyria specialists who were involved with the acute Porphyrias would be in attendance. This was not only a huge financial break for us; it also saved us from the logistical quandary of trying to bring doctors from different coasts to one location.

We quickly contacted the list of specialists, introduced ourselves and invited them to join us to discuss our dream of an American Porphyria Foundation. They all accepted. At the time, I did not know the rigors of a medical convention, so I wasn’t sensitive to the great sacrifice of time and energy that the experts shared with us.

Our meeting was held in one of Chicago’s lakeside high-rises. The view was spectacular, which added to the ambiance of our first meeting with a group of strangers. James knew one of the specialists very well, but, like me, he never had met most of the other attendees. The Porphyria specialists were a wonderfully congenial group of men and women. When we presented our plan to start the American Porphyria Foundation, each of them was very receptive to the idea and agreed to be members of our scientific advisory board. Almost thirty years later, they continue to volunteer their time and expertise, a contribution not often duplicated in other educational or medical foundations.

Before I proceeed, I want to add here that this has been the way our experts have been for the next thirty years. They have volunteered their time , etc. This is why I am so bothered when I see commments about our board of experts making money out of porphyria or getting money from a drug company....... that is so ludicrious. No one makes money on a rare disease. They have consulted with thousands of doctors over the past thirty years and have never changed one red cent.....not one penny. So those people who ae saying differently are not only ill informed, i my book, they should be ashamed. You see we need to be appreciative of this group of doctors who are so caring and who have volunteered their time for 30 years. to enhance that thought, one of the original experts, donated the major funding for the APF to move forward.


To continue, when we met with Dr.s Anderson, Bissell, Bloomer, Desnick, Pierach, Pimstone, Shedlofsky, Tishler, Kushner, Bonkovsky and Sassa ( who has since died). they were glad that we wanted to start a foundation and heartily agreed to serve on our Scientific Advisory Board.

This gave us the impetus to continue. If your are seeking information from any group or organization, if they do not have experts at the forefront, then it might be best to find another information source.



With this group, we developed educational materials and even got names of patients from the experts and NIH. Unfortunately , however, shortly after we started the APF, NIH stopped their porphyria research program and the government almost completely stopped their funding of rare disease research. That brought important research to an almost stop. This was severely disappointing.

Until recently, for over 25 years, research funding was very difficult, if not impossible, to find. You can imagine how I felt and how hard it was for the porphyria researchers. Nonetheless, we pressed onward and upward. We grew each year and instituted major patient and physician educational programs. WE knew both were of ultimate importance. As many of you havc experienced, most doctors have NEVER had a porphyria patient and did not know how to diagnose and treat each of the porphyrias.

Therefore, we have put as much emphasis on the doctors educational program as the patient education program knowing that unless patients are diagnosed and treated properly,we are still at square one. We feel the same way today , so the APF makes these educational programs a very important funding priority.

How you can help us is to send in your doctor's name and address and we will send him or her a very comprehensive packet of info all written by pophyria experts and we will put the doctors on the database to keep them updated. We also attend targeted medical conventions like , The Liver meeting, where 8000 doctors attend to learn the latest in liver disease. You can volunteer to help man our APF exhibit booth and distribute our brochures etc to the attendees. What an opportunity to make a difference. So check the APF news letters and E-NEWS to find out when these conferences will take place.