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Since Panhematin has beeen on the news for the last month, I thought I would tell you my first expereince with the drug. When I took it so many years ago, it had not been FDA approved and subsequently becem the first ORPHAN DRUG.

That story will come at a later date.

. My doctor at NIH, Dr. Lamon , arrived at my room . I was wide-awake and ready for what I hoped would be good news. Dr. Lamon said he thought that I might benefit from treatments with infusions of hematin; however, the FDA had not yet approved heme therapy for the acute Porphyrias. Evidence from years of prior research indicated that hematin was effective and might enhance my recovery.
Since I had been very ill for quite some time, Dr. Lamon warned me that hematin was sometimes not as effective when given far along in an attack as I was then. Having never been one to stand and wait for anything, I certainly didn’t want to think more than that moment to tell him that I was willing to try the hematin as soon as possible. Participating as a research patient was also an intriguing proposal. I concluded that having the hematin infusions would provide me with the chance to benefit from the use of an experimental drug while being able to help someone else in the future. I liked that idea, too, so I was ready to begin the treatment.
I later learned that the rationale for administering heme therapy is to correct a heme deficiency in the liver and repress production of porphyrin precursors. Since the first report in 1971, numerous patients were given heme therapy and clinically benefited from the treatment. Although there have been no controlled studies, the reported results were impressive enough to advocate the early use of heme therapy for porphyric attacks. When heme therapy is given late in an attack, no substantial benefit may occur; but if given early, the heme therapy almost always normalizes ordinary porphyrin and porphyrin precursor values.
If a patient with signs and symptoms of a Porphyria attack is not stabilized within a day by glucose and other care, most specialists recommend the administration of heme therapy. Usually, a dose of 3-4 mg/kg, given once daily for four days, produces a highly beneficial effect in most patients. A decrease in pulse rate, blood pressure, abdominal pain and decreased levels of urinary porphobilinogen are commonly noted and can occur within a day. At the time, I didn’t know what would happen, but I was certainly ready to try.
The following morning Dr. Lamon walked into my room holding an extremely large syringe full of hematin for me to see. I gasped and remarked that the concoction looked like swamp water, a black/green color. Some patients have since told me that they thought that hematin looked like crankcase oil or a prop in a Frankenstein movie. Regardless, I was ready to receive the infusion. At that time, Dr. Lamon infused the remedy into a vein in my arm with a butterfly needle and a very large syringe. He repeated the process daily for five days.
The hematin may have looked like swamp water or crankcase oil, but it was 24K gold to me. After only a few infusions, I improved dramatically despite the fact that I had been ill for a number of weeks. Relief from my constant pain and nausea occurred quickly, but the extreme weakness remained for months thereafter. (Hematin was the first drug approved by the FDA for rare diseases under the Orphan Drug Act many years ago. It became available from Abbott Laboratories in Chicago under the trade name Panhematin. It has since been sold and is now available through Lundbeck, Inc , which purchased Ovation Pharmaceuticals ..)
My doctor now gives me Panhematin when I have an attack. . Since many Porphyria patients asked how my doctor arranged for the treatments, I have included the following description of the usual scenario of the infusion process. The IV infusion is much different now than it was when Dr. Lamon infused the hematin by hand with a large syringe and a butterfly needle.
Now when symptoms of an attack of AIP begin, my docotr immediately orders Panhematin through the hospital pharmacy. Some people take it at local infusions centers , in their doctors offices and soem who are on home care take it at home...Most of the time, the hospital pharmacy or whatever the source does not maintain Panhematin on hand. Therefore, it is ordered from Lundbeck and is shipped immediately from their emergency supply. In fact, it can arrive within a matter of 12 hours since they keep a supply neaar the airport in Chicago.

More to come .


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