The Gameplan (Treatment)

As I've mentioned, scientists know very little about the disease at this stage. They don't know why this happens in people other than it is a result of a chemical reaction in the blood.

Also, because of the rarity of the disease, research has been slow. Progress is being made on a patient-to-patient basis. It is quite possible that during my treatment doctors will learn a new technique or observation about the disease that may help future patients.

Doctors, resigned to the fact that they do not know how to cure amyloidosis, shifted focus in the early years to stopping its spread throughout the body (task: save or prolong the life of the patient!). There are many, many different approaches that doctors are taking to combat this disorder, each with various rates of success.

Most treatment options involve oral or IV chemotherapy with or without steroids. (You'll note that treatment is not much different than for cancer.) However, most of these treatments do not give favorable odds for a complete remission (CR). You want a complete remission and not a partial remission (PR), for as long as there are amyloid produced in your body, they may continue to be produced and affect the organs.

Due to my relatively good health and young age, the team over at the Boston University School of Medicine will attempt what I call "the nuclear option".

"The nuclear option"

My treatment will consist of two parts: High-dose chemotherapy combined with stem cell transplant(s). Here's what will happen (in a nutshell):

• A catheter will be installed in my chest for stem cell collection.


• Over the next few days, I will be given growth factor injections to dramatically stimulate white blood cell production. This will literally burst my bones to the seams as stem cells are released from my bone marrow. They will then be collected through the blood stream via catherer.


• A little under a week later, I will be given high doses of IV chemotherapy for two days straight. When I mean "high doses" I mean a year's worth of chemo in two days. They will watch me for a day or two and send me home.


• Two days later, they inject me which the stem cells they collected in the previous week. These cells will not stop the destruction of my blood cell count as the these cells are too immature.


• A few more days pass while the chemo travels through my body, destroying all bone marrow and blood cells in its path.


• About 4 or 5 days after my stem cell transplant, I will hit the nadir, or low point. At this time my blood counts will be at or around 0, and I will no longer have an immune system. They will rush me to the hospital at this time.


• For the next week, I will be on a suite of 9 or more drugs: painkillers, anti-sickness drugs, antibiotics, growth factor injections to stave the body of infection as the stem cells deploy and start rebuilding the immune system. This is where I will be the most vulnerable, as the slightest infection can kill me.


• I remain in the hospital until blood counts return to normal. I am then sent home.


• I am checked every few days on progress. Sometimes a patient gets an infection and is sent back to the hospital for an extended duration.


• Six to eight-weeks after the stem-cell injection, I should be able to walk around. I will not have much energy and need to avoid germs. I will be able to walk outside again, but only with a mask, and cannot be around people.


• Three months after the stem-cell injection, I should be able to go back to work. I am still with a weakened immune system, and I will need to wear gloves and a mask when travelling outside. I should be able to get visitors by this time. Also, doctors will have a unoffical report on the success of the treatment.


• Six months after the stem-cell injection, doctors will perform additional tests on me and determine the success of the procedure. If a complete remission (CR) is found, all I will need to do is do semi-annual checkups at BUSM to make sure that the amyloids stay in remission. Otherwise, if only a partial remission (PR) is found, they will wait another six months for my immune system to rebuild, then they will do the procedure all over again.

Usually they give you two attempts on treatment to halt the advance. If they are unsuccessful after two tries, then I will be in a bit of trouble.

Odds

Being a math-oriented person and an occasional poker player, one of the first questions was, "What are my odds of making it through and defeating this disease?" I can't settle for a PR, I really need a CR. So, based on trial notes and doctor's discussions, these are my odds based on my calculations.

• Chance that I survive initial treatment:95%


• Chance of complete remission after one treatment: 50%


• Chance of partial remission after one treatment: 20-30%

If a partial remission is achieved, most patients have been known to live an additional 5-10 years.

If a 'complete remission' is achieved, there are unknown odds that I will get a relapse. There are also significant odds that the chemotherapy might weaken or destroy a body organ.

However, my doctors are confident that I will get through this.