Wrestling The Bear

On Moral dilemmas, Technical solutions, and the Problem of Sickle Cell

© Photo by Bertie

Every problem has two approaches: moral and technical.

Moral dilemmas ask us how to act in a given set of circumstances. They don’t fix a problem, or even change it, but rather require us to maneuver within and around it. Technical solutions, on the other hand, attempt to eliminate a problem - they “solve” it in the classic sense of making it disappear.

Most people will take a technical solution over a moral dilemma any day.

When I was a young resident, sickle cell disease was a primarily a moral dilemma. A patient would be admitted in vaso-occlusive crisis, hypoxic, in pain, their red blood cells twisting and rupturing. All because of a minuscule genetic defect, where one of three billion nucleotides in their genome was a thymine instead of an adenine.

From a technical perspective there wasn’t much to do: hydration and analgesia - that was about it. Finding a vein was always a challenge in these patients, but the real issue was the treatment: narcotics.

In those days they used to call pain the 5th vital sign, but it’s not - it’s the opposite. Vital signs are objective. Pain is subjective. The only way to measure it is to ask. Depending on the severity of their illness, patients with sickle cell disease could be admitted as often as every month. Typically, they would be treated with intravenous opiates and discharged with oral opiates.

Sooner or later many of these patients would become dependent on narcotics, and the ones we saw most often would be addicted - and whose responsibility was that, by the way, if not ours, who had been their suppliers since early childhood? Every sickle cell case begged the question: would the standard treatment ultimately make the patient better or worse?

What was the right thing to do? It wasn’t always easy to decide.

If the patient was either clearly in pain or perfectly comfortable, fine. But what about the many shades of gray in between? The somnolent patient rousing himself only to ask for escalating doses of morphine. Or the aggressive patient demanding fentanyl in a way that felt threatening to the staff? Or the patient who never felt ready to taper down? Or the patient whom we admitted with a long history of recurrent sickle cell crisis only to discover, after a week of struggling to find the right fentanyl dose, that he did not have sickle cell at all.

Add to the mix feelings of psychological manipulation, leveraged by the fear of doing medical harm, balanced against apprehensions about perpetuating medical bias in a primarily Black patient population, itself leveraged by the warnings from experts (not to mention from pharmaceutical reps!) about under-treating pain….

Little surprise that under such circumstances writing a simple medication order could feel, to a young resident, like a complicated moral dilemma.

Now I’m an older attending, and the problem of sickle cell disease has been technically solved. An article in the New England Journal of Medicine describes the process, a type of experimental gene therapy called Exa-Cel.

Here’s how it works: 1. The patient’s stem cells are extracted from a blood sample. 2. The stem cells are modified in the lab, using a gene editing technique called CRISPR, to reactivate the production of fetal hemoglobin. 3. The patient’s bone marrow is ablated. 4. The new stem cells are re-infused, engraft to form new bone marrow, and proceed to produce healthy red blood cells with fetal hemoglobin.

In other words, an autologous bone marrow transplantation with genetically altered stem cells.

How effective is this one-time cure? Reduction in vaso-occlusive crisis over a 12 month period: 97%. Reduction in hospitalization: 100%. Hemoglobin level (chronically low in sickle cell patients): normal. Overall quality of life: vastly improved.

Problem? What problem?

Not that the moral dimension disappears altogether. On the contrary, it morphs.  As you can imagine, Exa-Cel does not come cheap, and it will likely be hard to get for many Americans who need it. Even if it weren’t, consider that the 100,000 Americans with sickle cell represent less than 0.5% of the 20-25 million people in the world with the disease. It’s hard to imagine making any difference at all in their lives without solutions on a much bigger, more global scale than Exa-Cel. Is it fair to spend so much on so few and so little on so many?

That’s one moral dilemma. There are others.

But, as I like to tell my kids, don’t let the great be the enemy of the good. A technical solution with the potential to sweep aside at least some moral dilemmas seems like a step forward to me. Anyway, maybe that’s just the way it works - the flip side to the seductive appeal of technical solutions in general. Sure, they’ll take old moral dilemmas off your plate, but only to replace them with new ones of their own.

In other words, let me revise the first line of this post.

Maybe it’s not so much that every problem has two approaches, moral and technical, but rather that all problems exist in a kind of moral/technical dialectic, whereby one leads stepwise to the other. Moral dilemmas beget technical solutions beget new moral dilemmas beget new technical solutions until… until what, exactly, Master Shifu, until what?