The inaugural fundraiser to help the MPN Research Foundation.

Tuesday, February 21, 2017

Inaugural Raffle: Overview

Myelofibrosis: The Cancer, Disease, and Treatment

Myelofibrosis (MF) is a classified as a blood cancer in the MPN class. The cause is unknown. It is thought to be present in about 20,000 people in the USA; since this number is so low, the World Health Organization has classified it as a rare disease. However, we are starting to learn more about it, and realizing that it might impact many more patients.

MF typically presents with a couple different symptoms, and is very difficult to diagnose correctly. The two symptoms that usually present as definitive are a massively enlarged spleen, and wildly variable blood counts. The patient will suffer from a variety of secondary symptoms (fevers, sweats, chills, debilitating fatigue, cognitive dysfunction) and may well experience significant and severe organic damage (heart attack, stroke, cirrhosis of the liver, internal bleeding); if left untreated, the patient will typically die. 

Unlike most cancers, MF can kill the patient through it's secondary symptoms or through the cancer itself. Since the cancer impacts the blood manufacturing system, which is a most critical system for life, all other systems and organs are impacted.

There is no remission phase for this cancer.

There is only one FDA approved medication that was developed specifically to treat this cancer; other drugs might be used to treat some symptoms, but this approach does not treat the cancer itself.

There is only one treatment at present which might lead to a cure: a stem cell transplant from a closely matched sibling. Such transplants involve very lengthy and difficult recovery periods; compared to treatments for other cancers, they have a low success rate; they are suitable only for a small subset of patients; and finally – they are very, very expensive.

The MPN Research Foundation

The best way I know to help others afflicted by this terrible cancer is by supporting the MPN Research Foundation ( Awareness of myelofibrosis and other similar disorders must be raised at all levels of society, and basic scientific research must be conducted. These are the two areas in which the MPN Research Foundation specializes. The foundation regularly receives a high (3 star and 4 star) rating from Charity Navigator for their ongoing commitment to maintaining low administrative overhead and spending a high percentage of funding on awareness and research programs.

Awareness of treatment protocols is lacking.  Not only are patients told (as I was) to go home, take it easy, and let death come peacefully, but hematologists don’t realize that this type of cancer requires vastly different treatment protocols from other cancer, including hematological cancers such as leukemia. For example, when taking the one FDA approved drug for myelofibrosis, the patient will, 99% of the time, see a dip and subsequent trough in certain blood counts happen nearly immediately.

In every other blood cancer, this is an indication of bleeding, typically “hidden” internal bleeding and possibly even a stroke, and requires specific treatment to identify and/or just stop the bleeding. However, in our case, this dip and trough are normal side effects of the way the drug works in the body, and treatment is not required. Within a few months, the patient’s counts will climb back to their normal on their own.

Most hematologists are unaware of this.

This lack of awareness is a direct result of current educational practices in medical school and residency. Hematologists and oncologists receive, at best, two days of classroom training on these cancers in all medical schools. This is not nearly enough for doctors to know techniques to best treat patients; none of the standard hematological practices apply to these cancers. The MPN Research Foundation has programs in process and in place to address this very issue.

Awareness must also be raised among the "average persons".  When I was diagnosed, it was by complete accident; I was rushed to the ER because I thought I was having a heart attack. My case is not an outlier: most patients who I meet are diagnosed secondarily, because some other symptoms suddenly arose. In the case of one young man I know, he was finally diagnosed because of persistent erectile dysfunction!

Of course, raising awareness is not nearly enough. In 2015, I joined a clinical trial for a new, cutting-edge drug called pacritinib. This drug, if approved, would only be the second drug approved to treat MF. When I was part of the trial, the FDA temporarily halted it; that left quite a few patients who were responding to the new drug in a bad position. Suddenly, their treatment was gone. Remember: this is life or death.

MPN Research worked with doctors and the FDA, and managed to convince the FDA on behalf of the patients who were doing well to continue taking the new drug. Nobody else considered doing this.  These therapeutic exemptions saved lives.

MPN Research has established new guidelines for education at all levels. They have conducted pivotal research studies, including research that led to the first drug available to treat MF. They are currently involved in gene editing research (CRISPR), genomic sequencing, and research into how inflammation impacts the bone marrow niche where new blood cells are born.

Founded in 1999 by patients of these cancers, the MPN Research Foundation has funded to date a total of $11M in 56 institution independent research projects, and provided support to 53 researchers. Corporate sponsors include Janssen Oncology (a Johhson & Johnson Company), Lilly, cti Biopharma, Incyte, and many others.

Not only is MPN Research doing all this, but they are the only organization driving these initiatives. Their work is sorely needed, and it’s a travesty they don’t receive more support. They maintain full transparency, with all financial records and reports published on their website.

Blades Against Myelofibrosis Raffle

To do my part in supporting the foundation, I have created the "Blades Against Myelofibrosis" raffle. The twin blades of power (knives) and knowledge (light) working together will provide us with the means to battle and ultimately defeat this cancer. 

The inaugural 2017 raffle will start on 3/15/17. I am actively seeking sponsors.

I currently have the following sponsors signed up for the blades of power portion of the raffle:

-Razor's Edge
-Sketchen Scales
-Spyderco Knives
-BladeFlick Knives

I currently have the following sponsors signed up for the blades of light portion:
-Iron Emotion Design
-PK Design Lab
-Avant Systems, Inc

For this inaugural event, in addition to all ticketholders receiving a t-shirt with sponsor names, I have five prize packages

-1st place is a highly customized Spyderco Gayle Bradley 2 knife, with custom scales, Cerakoting, Mirror Edge Sharpening, and other modifications. Value is $700.00.

-2nd place is a customized Spyderco Manix 2 DLC, with custom scales, and Mirror Edge Sharpening. Value is $300.00.

-3rd place is a WorkSharp Ken Onion Edition with the Blade Grinding Attachment, mug, shirt, and cap.

-4th place is an Emerson CQC 8, valued at $165.00.

-5th place is a WorkSharp Guided Sharpening System, with the optional diamond stone upgrade, mug, shirt, and cap.

All prize packages include a PK Design Labs PK-FL2-LE flashlight.

Please note that I am limiting ticket sales to 110 tickets, which gives ticket purchasers 1-in-22 odds of winning a prize package of greater value than the cost of their ticket. These are decidedly good odds.

In order to keep administrative costs low so that the vast majority of the proceeds goes to the foundation, I am performing much the work myself, with no compensation. I am also paying for certain items out of pocket. For example, while I want to keep costs low by taking on most of the work myself, I also want to make sure that I have a competent “backup” person in case something happens to me – like an unexpected and extended hospitalization. I am therefore paying somebody out of my pocket  to learn the processes I am putting in place, and that person will be fully capable of substituting for me should the unforeseen take place.

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