Keeping Vigil

It is late night as I write this, sitting in Harvey’s ICU room. Harvey is sleeping his induced coma. I think he can still hear me sometimes when I talk to him, but perhaps I am just imagining it for my own consolation. I wonder what dreams he is dreaming as I keep vigil in his room, waiting and worrying about what new crisis the next hour, the next day may bring.

Writing is a form of catharsis for me, a way to clarify my own thoughts. I come from a small family, and both Harvey and I are first generation immigrants with all the emotional loneliness it implies. I want you to know your feedback gives me immense solace. What nature did not do for me, Harvey seems to have corrected by way of our very extended CLL family. I have not met most of you – but we walk down the same paths and that is enough for kinship of the heart.

Dangerous Waters

Harvey is swimming in dangerous waters, alligators to the right of him and alligators to the left of him. Making therapy decisions is very hard when there are so many conflicting needs and risks.

Harvey’s latest medical crisis has been diagnosed as ARDS (acute respiratory distress syndrome). Think of it as your lungs turning into a bloody mess so that it becomes a challenge just to get enough oxygen into your body. Some of you may remember the SARS epidemic scare a few years back. In that case, the viral infection attacked lung tissue and in advanced cases lead to ARDS. But it is not necessary to have a pathogen such as SARS to cause lung damage. It can happen as a result of autoimmune disease as well, the body attacking itself. So far, we have not identified any pathogen causing the lung damage in Harvey, the assumption is that the damage has been caused by sudden and devastating inflammatory cytokine storm that pulped his lung tissue. In otherwise perfectly healthy young people ARDS carries a mortality risk of 30-50%. I am sure you can imagine how much more scary that percentage becomes in the context of a newly transplanted patient.

Plugging the Holes

For a while, Harvey was losing blood so fast that we were not sure we could replace it fast enough to keep his blood pressure from sinking too much. We now seemed to have turned that corner, almost continuous infusions of plasma, platelets and red blood cells have patched most if not all the leaking holes. Hemoglobin, RBC and platelet counts have stabilized. The ventilator with its enriched oxygen content is keeping the oxygenation level in decent shape. But that is just the start. It will take time for the damaged lung tissue to heal. Until that happens, he will have to have ventilator assistance just to breathe. The question boils down to this: will he get that time? Will he be able to dodge all the other alligators in the water long enough for his lungs to heal?

Between The Devil And The Deep Blue Sea

Harvey is on massive doses of methylprednisolone in an attempt to halt the inflammatory spiral that got so dangerous so quickly. What initiated the process? No one knows for sure and there is not much point in speculating right now. Steroids are good at controlling most forms of inflammation. But they are also dangerous in suppressing the body’s ability to fight infections.

That brings us to the second big alligator trolling in the water: uncontrollable infections in a deeply immune compromised host. During times of deep inflammation the body’s first line defenders (neutrophils, macrophages) rush to the site of the inflammation and throw themselves into the fight – often making things worse by shooting off their weapons in random manner, causing even more tissue damage. A close analogy of this process is “keystone cops”, killing friends because they cannot tell the difference between friend and foe.

This misguided zeal of neutrophils is also suicidal – white blood cells die in droves as they exhaust themselves fighting an enemy that they cannot identify. Plugging the holes has stabilized the red blood and platelet parameters. Restoring the white blood counts to healthy levels is not that easy, since it is not possible to give white blood cell transfusions.

GCSF (Neupogen, Neulasta etc) shots are routinely used to goose white blood cell production. But did you know that GCSF (and its sister growth factor GMCSF) are quite inflammatory in themselves? How much GCSF shots can we give Harvey in an attempt to speed up the recovery of white blood cells, before the old inflammatory cascade gets out of control once again? Can we afford not to give him GCSF, if that means longer to neutrophil recovery? Will he survive the inevitable infection that will come by sooner of later, if he has no fighting troops? How much steroidal drugs can we give him to keep a lid on the risk of renewed inflammatory cascade, before we cross over the line in ability to fight infections? We are in a damned-if-we-do-and-damned-if-we-don’t mode here.

“Slim” is Still Alive

We did get one piece of good news. A bone marrow biopsy done earlier in the week shows the graft is still around and doing OK! “Seattle Slim” is one tough kid, he is still 100% in charge in the bone marrow, still trying to produce cell lines in these trying times. Comparison of most recent PET scan with one done a month ago shows GVL happening at a good clip, CLL remnants in full retreat. Way to go kid. May you live long, and may you help bring Harvey back to health.

Crisis Overdose

I oscillate between hope – forlorn though it may be, and unmitigated terror and grief as I contemplate the future. Maybe my best friend will beat the odds, dodge the double alligators of uncontrollable infections / inflammation – along with all the other smaller beasts such as kidney overload, liver toxicity etc. But maybe his lungs won’t get enough time to heal and he won’t ever be woken from the induced coma – what is the point of waking him up, just so he can experience the terror and pain of slow suffocation?

I wanted to spend a life time with my buddy. But on Thursday afternoon I would have settled for even just a couple of minutes together before he had to go into his present long sleep. There were so many people milling around in Harvey’s room in those frantic hours as they tried to intubate him, stabilize the bleeding and get him into ICU. I barely had time to explain to Harvey what needed to be done, and for him to scrawl “Agreed” on a piece of paper – he could not talk – and that was that. Now I sit here watching over him as he dreams away the hours, remote in his coma. Tomorrow will be here soon. Another day, another crisis – I will keep my vigil for as long as it takes, as long as he has a chance.

Be well,

Chaya