In September, 2015, my wife was diagnosed with a fatal illness called Lewy Body Disease. Like all brain wasting diseases, there is no treatment and no cure. Our situation seemed hopeless—we were doomed to watching this intelligent and vivacious young woman fade away into the oblivion of dementia. We prayed for a miracle, and I researched every article I could get my hands on. To my surprise, I found a ray of hope.
It seems our bodies have three circulatory systems: a vascular system driven by the heart, a lymphatic system driven by muscle movement, and a glymphatic system driven by the brain’s glial cells. It is not entirely clear to me how the three interact, but I have concluded:
1. Cerebrospinal fluid is a clear liquid surrounding the brain and spinal cord. It moves through the brain along a series of channels that surround blood vessels and helps remove a toxic protein called beta-amyloid from brain tissue. That is the glymphatic system. Beta-amyloid accumulates in the brains of patients with Alzheimer's. In the past, the blood-brain barrier was believed to keep the brain separate from other systems; however, there must be some exchange with outside systems because radioactive beta-amyloid injected into a brain can subsequently be found in lymph glands in the neck.
2. Beta-amyloid protein is natural; everyone has it. With Alzheimer’s, the protein gets folded incorrectly into tiny “U” shapes that clump together to form plaques. The plaques are not removed by the glymphatic system. Instead, the masses continue to grow and eventually begin to kill brain cells by clogging the synapses. Tau tangles are another form of improperly folded and clumped protein that kills brain cells from within the cell. Both are present in Alzheimer’s patients.
3. While there is some question whether tangles and plaques are a result of Alzheimer’s or the cause of it, the protein clumps are not prevalent in healthy brains. There has been no way to eliminate the clumps from the brain, and no one has ever survived the accumulations…until recently…maybe.
4. A new drug called aducanumab (where the “mab” stands for monoclonal antibody) is being tested by Biogen Corporation. There are two ongoing worldwide studies—EMERGE and ENGAGE. Each two-year, placebo-controlled study has 1350 patients. Qualifying patients might also be enrolled in an additional two-year follow-on study where they are guaranteed to get aducanumab.
5. Aducanumab is a human antibody derived from elderly patients who did not get Alzheimer’s. The antibody apparently works by attaching itself to a single improperly folded protein in such a way that the glymphatic system can recognize the protein as waste and dispose of it.
6. I applied online for one of the slots in the EMERGE study, and, after rigorous testing, my wife was conditionally accepted into the study. We only have one more test before the drug (or placebo) will be administered to her—a PET scan. PET scans are very expensive so they are the last step. The psychological testing, blood work, urine tests, genetic testing, MRI, and PET scan will form her baseline, assuming her PET scan is acceptable to study doctors. In either case, we have submitted the paperwork to be considered for the follow-on study.
7. Assuming she moves on with the rest of the study, my wife will receive one IV infusion every month for approximately 24 months as part of the EMERGE study. It is her only hope of survival.
I am not an overly religious person; I have my share of faults and do not claim to be anything I am not. However, being faced with the death of my spouse, and not being able to do anything about it, forced me to the reality that I am not in charge of anything. One learns to pray under such circumstances. So, now I ask myself, is the study a miracle…an answer to prayer…or the natural consequences of science’s inevitable march forward? As I see it, it’s not just that the drug might actually work and that it is available exactly when my wife needs it, but that, out of the 7,484,325,476 people on earth, only 1350 can be in the study. Was it just luck that we might get a shot at being one of those? Clearly, I am re-assessing my place in the Universe.
As I said in an earlier blog, we are also pursuing my wife’s bucket list with gusto, in parallel with trying to find a cure. We have taken grand kids to Disney World, enjoyed a cruise, visited the Smokey Mountains twice, toured Dollywood and the Biltmore Mansion, spent some time in Charleston and Jekyll Island, roamed art galleries in Albuquerque and Santa Fe, and purchased a home for some of our grand kids in Houston (an early bequeathment). We will continue to do those kinds of things within the bounds of the study requirements. I might also begin writing again as I have a killer plot hatched up for a fifth Miss Havana paranormal comedy novel.
So why am I writing this particular blog? Please be assured I am not trying to convert anyone to anything. My intentions are simple. First, I wanted to update those of you who are following my wife’s story. Second, I believe everyone needs to know that Alzheimer’s and Lewy Body Disease are both lethal AND becoming more common—by 2050, the prevalence will quadruple. By that time 1 in 85 persons worldwide will be living with the disease. The impact on caregivers and the health care systems across the globe cannot be overstated.
If you have someone suffering from dementia, especially if diagnosed early, please check out the Biogen EMERGE and ENGAGE studies. They could save the life of your loved one.
Thanks for reading,
James L. Hatch