Wednesday, November 24, 2010

The Beginning.

I was only seventeen years old when I suffered my first attack of porphyria. The attack commenced with mild pain in my lower abdomen and quickly intensified to the unbearable point. When my parents realized that this was no normal illness, they raced me to the nearest emergency room. By the time we arrived, I was so ill that I had to be helped from the car into the clinic and was placed ahead of the other patients as the number one patient on their triage list.
The physician and several nurses rushed to my cubicle and initiated an emergency examination. After the doctor determined that I was not dying, he began taking the perfunctory medical history. I tearfully whispered that I was in far too much pain to respond to his lengthy list of questions. He was quite sympathetic and asked if I could at least describe my symptoms. I was embarrassed to sound like a melodramatic teenager, but I proceeded by telling him that my pain felt as if someone had pierced me with a thousand flaming swords and had left them deeply imbedded in my abdomen. I continued by explaining that I was also experiencing such weakness that it was difficult to breath or lift my arms and legs.
Despite what others would have thought were youthful histrionics, the emergency room doctor did not dismiss my seemingly exaggerated complaints. Instead, he listened intently and continued with a more thorough physical examination.
The only outward evidence of my agonizing inward state was that my abdomen was extremely distended and hard as granite. After the doctor felt my huge abdomen, he advised me that I might require emergency surgery. The pain was so severe that I cried out that I was ready and hoped a surgeon could operate right away.
As soon as he finished my examination, he explained that he would have to leave for a few minutes to order a few more tests. True to his benevolent nature, he reassuringly added that he was requesting that the reports be returned to him immediately so that he might be able to diagnose my condition quickly and treat my pain as soon as possible. Before he left my cubicle, he gave me one of those pats on the arm that are supposed to signify that everything will be fine soon. His gesture was kind and sincere, but I was so overwrought with pain that I knew then that whatever ailed me was serious business and that the compassionate doctor would find out soon enough.
The doctor had just returned to my curtained off cubicle when the clerk brought him my lab results. He reviewed them carefully and appeared perplexed at what he read. He showed me the report and pointed out that the results of the tests were normal in every category and, therefore, did not provide information that could help him determine what was causing me to suffer such severe symptoms.
I gaped at him in desperation, terrified that he and his colleagues would not be able to relieve my horrendous pain. Seeing my anguish, he comforted me by assuring me again that he would first handle my pain and then admit me into the hospital to seek an answer ot it’s cause. In the meantime, he promised that he would contact the best gastroenterologist on the hospital staff and that he would surely shed some light on my ailment. He also promised to advise the physicians in charge of my case that I would need adequate pain medication and adequate meant more than a hefty dose. Astonishingly, he did not mention exploratory surgery again and neither did I.
Before finding myself in this new health predicament, I had never experienced a sickness that required much more than aspirin, antibiotics and mother’s cold rags. At that point, those measures would have been like using a using a tricycle to go around the world. They were far from what I needed to relieve the horrendous pain. Nothing took precedence over pain relief. I was more than insistent about needing the pain medication to the point of begging every person in a white uniform for help.
Fortunately, I was admitted into the hospital promptly. As I was being wheeled to my hospital room, the reality of my situation hit me full force, and I started crying uncontrollably. I was convinced that I was going to die from an undiagnosed ailment before I ever experienced life on my own as a “grown up.”
“I’m sure it’s not possible for a human being to suffer this kind of horrific pain and live through it,” I sobbed. The nurse beside me offered the same assurances as the emergency room doctor. “Don’t worry,” she said, “We’ll find out what is wrong with you, but until then, the shot I am going to give you in a few minutes will take over, and you’ll feel much better.”
One big hypodermic later, I was no longer writhing in pain, but I was still conscious of it. The horrendous agony clung relentlessly to my lower abdomen like an imaginary demon hanging on for dear life and wrecking havoc in the process. The rest of my body ached much like a bad case of the flu. Although the weakness continued for weeks, the fierce pain and feeling that I had influenza abated within the next few days. At the time, I thought that the whole ordeal was over never to return---or was it ?.

Tuesday, November 23, 2010

The APF welcomes YOU

The American Porphyria Foundation ( APF) welcomes you to our new blog, Purple Light . The APF website is an educational explosion of information. The Blog will be the place where we weave pertinent information within the context of a human interest stories, yours and mine.

My name is Desiree Lyon Howe. I have been with the APF since day one when the APF began as an idea of mine and my friend, Jim Young. In the early 1980's, Jim and I met through several porphyria experts, namely, Dr. Claus Pierach and Dr. Karl Anderson. Just meeting another person interested in porphyria made such an impact on both of us that we felt that starting a foundation for people to learn more about the disease from experts and have the means to communicate with one another would be an important service to others who suffered with porphyria or had it in the familyt.

We discussed the concept further and settled on a name , the APF. Then we set about creating a plan that incuded selecting a Scientific Advisory Board as a first step. Without the most esteemed porphyria doctors in the country as our guides, we would not have the kind of recognizable standard we needed to have patients and their physicians pay close attention.

It was not hard to locate the best experts in the country. There were only a handful but they were all brilliant and world renonwn physicians and researchers. Imagine !!!!! That same group is still with us today.

Next we set up an office, which was my kitchen table and created our membership list which was comprised of two people, James and me. We are a long way from that now with a staff of five in Houston, one in Chicago and several more throughout the country. Plus, our two members has expanded to four thousand members and sixteen hundred doctors who want to be part of the APF. Our educational programs and services for patients and physicians are award winning. Our Protect the Future program to train the next generation of experts has become a model for other foundations and institutions , as as has our our In Touch support system. Our research projects are increasing , our govenment funding has increased and our social networking has helped spread our message tremendously. All of these services are a result of us working together.

Next week, I will begin the APF blog by sharing a bit of history and a few very intersting stories of the 15,000 people I have communicated with via the APF. Until then...Keep in Good Health !!!



The APF is all of us.

Important EPP Survey URGENT ASAP!!!

Important EPP Survey Dear EPP Community, As you know, the diagnosis of EPP is often delayed for many years. Researchers at the Icah...