Finance Bob's War against cancer
Myelofibrosis (MF) is a classified as a blood cancer. 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.
I have many issues. I have severe cirrhosis of the liver; an enlarged spleen; portal vein hypertension; significant connective tissue morphing into bone; and the list goes on. This is just the organic damage caused by the cancer secondarily.
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.
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.
Many 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.
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 – and suddenly stopping this type of drug was known to cause severe side effects, including death.
Dear Dr. -----------:
I'm not sure if you remember me, but you saw me as a myelofibrosis patient in 2015 and the beginning of 2016. I repeatedly complained about abdominal pain, nausea, and excessive tiredness.
You insisted that none of these symptoms were related in any way to the cancer.
Well, in April 2016, many of my esophageal veins, which had become varices, burst. I started vomiting blood in profuse amounts.
It turns out that, in fact, the enlarged spleen was causing forward flow portal hypertension. I nearly died that week; the surgical team worked on me for 10 hours, placed me in an induced coma, and told my wife to call our priest for last rites. The bleed was so bad that I required 17 units of blood that day - yes, 17.
On top of this, my liver has been severely damaged by the excessive pressure of the blood the spleen is sending to it. I suffer from the beginnings of hepatic encephalopathy; my liver is so impacted by the cirrhosis that my blood ammonia levels, as described by my current hematologist "are typically seen not in patients who are walking, but in patients who are in a coma."
I've had to surrender my driver's license. I cannot work, because I cannot think or drive. My wife and I may well lose our house - that's really the only investment we have left; we could well be homeless barring a miracle. Speaking of the house, I've not left ours in about 2 years except to visit the hospital. Not being able to drive, and having myriad other issues, prohibits that.
I do hope you read and re-read the above note. I hope that it awakens your soul, and I hope that you stop dismissing your patients and instead treat them. Nobody, nobody, least of all somebody in the prime of his life, should ever have to go through what I have gone through and continue to experience on a daily basis. For you - you who are NOT a mental health professional - to diagnose me with depression, and strut around the hospital, telling all my doctors that I am mentally unstable is dangerously close to a gross violation of any reasonable code of ethics. I especially liked how you took it upon yourself to tell my pain management specialist that I was not feeling any pain, that I was simply depressed, and that I was only seeking drugs. I hope that you never experience pain that drops you to your knees, that makes you scream and sob, that makes you beg some unknown power to take your life so that the pain will end.
I did, as a result of your work.
Perhaps at another time you can tell me the formula used to derive the volume of blood the patient will vomit from the degree of depression (s)he is suffering.
You wield a great deal of power in your patients' lives. Please, sir, take that power seriously; please behave ethically; please listen to your patients.
If you have a patient who works hard to understand the cancer impacting his/her body, who spends hours studying, who dutifully reports symptoms to you, don't just ignore that patient.
Don't let another patient end up like me.
However: if your intent was to buy a ticket, this benefit ran last year. I did not close this GoFundMe down because of various issues I have with them.
There are, as I see it, a couple possibilities.
One, I bundle off your donation to the folks at MPN Research.
Two, I refund your donation.
Three, I keep your donation and use it for medical expenses. This lattermost course is attractive because I soon have to journey to Johns Hopkins, and it's going to take money I don't have. However, my personal values demand that you tell me how to proceed.
Financial update: well, it sucks. Social Security denied my claim. According to the rep, I'm perfectly capable of working. As I write later, that's not the case.
So I'm selling off all my belongings. I sold the bulk of my good record collection - that hurt. Tomorrow I sell my high-end turntable, and I keep selling the rest of the big music rig until it's gone.
I'm going to have to put on a skirt and start working the parking lot at the truck stop.
I'm trying to get my knife sharpening / regrind / repair business up and running and get customers. We are planning on selling the house and moving to something smaller. Not many other options.
Personal update: I'm not doing too well, and haven't been for about a year. I never leave the house, except for the hospital or ER. I've felt largely terrible, and can tell that my mental powers are impacted.
I complained about cognitive dysfunction, and essentially I was not believed - largely because even with a compromised brain, I could still carry on a technical conversation about this cancer and be right when the doctors were wrong. However, I finally got somebody to listen.
I have advanced cirrhosis of the liver, and the beginning of hepatic encephalothapy. This latter condition explains my cognitive decline, which is so bad that I've surrendered my driver's license.
As my hematologist/oncologist put it, he's never seen anybody with ammonia levels as high as mine not be in a complete vegetative coma.
On top of that, I'm suffering from edema - holding water. I know, what's a little water, right?
I normally weigh about 260-265lbs. One day I woke up, and my legs were tight, and I weighed in at 320lbs; the day before I was at 260. We started to treat this with diuretics.
I took one dose of diuretics, and essentially urinated once every 45 minutes for the next 36 hours. At the end of this period, I weighed 260lbs. I dropped 60lbs as easily as I gained it.
Again, this is undocumented in the medical literature.
There's not much to be done for treatment. I'm taking a special laxative which works by preventing protein from converting to ammonia in the intestine, with the hope that will lower my ammonia levels and I will be able to function again.
The standard course of treatment is a liver transplant. However, I will not be placed on the recipient list given my cancer. Normally, they would put me at the top.
There's more! I think, however, that you get the picture: my health is worsening. I would love to manage another round of "Blades Against Myelofibrosis" but I just don't have the ability to think that well.
Another symptom of the liver disease is sleepiness. The assdouches at the Cleveland Clinic might tell me that I'm not tired, but depressed. They are wrong.
I'm extremely tired from liver failure. If I forget to take my narcolepsy med, I sleep 20hrs/day. I can fall asleep standing up; while this is an impressive party trick, I would not recommend that you do it.
Thanks for reading. Please let me know how to handle your donation!
Bladeforums should be ashamed. I'm deleting my account.
So while I can see why BF may not want to support every cause every person brings their way, it's not the first time I've heard this "blowing up" complaint form the owner over there. I get that deciding when and how to do this can be hard, and it may even put BF in a tough position is some charitable thing turned out to be a scam. All that said, it doesn't seem unreasonable for them to at least get back to you. I'll go drop a note over there. They can slap my hand or boot me out if they feel it's warranted.