Lifting the Shadow

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In one of my oldest essays, on the problem of evil, I wrote about a rare genetic disorder called epidermolysis bullosa. EB is like the paradigm example of the worst disease you can have:

…in which a mutation disables the ordinary activity of a protein crucial to maintaining the strength and elasticity of normal skin. The result is extremely fragile skin, sometimes referred to as “butterfly” skin because it is so delicate, in which even slight pressure or friction can produce severe blisters akin to second-degree burns. The disease manifests itself from birth, so the majority of sufferers are infants and children.

With diligent wound care, people with the less severe forms of EB can live almost-normal lives. With the more severe kinds, it’s one horrible symptom after another: scarring and disfigurement, blistered fingers that fuse together as they heal, blistered feet too painful to walk on, greatly increased risk of malignant skin cancer. Children with the worst varieties of EB live short lives of unbearable pain and die from massive systemic infection.

Diseases like EB should make everyone doubt the existence of God. The idea that a benevolent being would choose to torture people in such a cruel way, would cause this to happen when he could prevent it, ought to strike anyone with a conscience as a sick absurdity. Since EB is genetic and manifests from birth, there isn’t even the thin excuse that the sufferers must have done something to deserve it. The fact that babies and children suffer the worst is an extra dollop of sadism.

Belief in God offers no meaning or comfort in the face of a disease as cruel as EB. But where theology is no help, science can step in. When I wrote about it originally, I said there was no cure. That may no longer be true.

The latest issue of Nature has an article with an attention-grabbing title: “Regeneration of the entire human epidermis using transgenic stem cells“. It was written by German and Italian doctors who treated a 7-year-old Syrian boy with junctional EB, one of the worst kinds. He’d suffered blistering all his life, but several weeks before admission, he’d contracted a skin infection that severely exacerbated his condition.

By the time he got to the hospital, he’d already lost 60% of his skin, and was suffering from massive infection and a raging fever. The Nature article details the horrible, heroic measures they took to keep him alive, including daily iodine baths (mercifully, under general anesthesia).

Remarkably, his doctors got the fever and infection under control. Nevertheless, their treatments were futile when the boy’s body had almost no ability to heal. He was in such bad shape that the doctors asked his father for consent to palliative care, to give him enough painkillers so he could drift off and die peacefully.

But the boy’s father pleaded for his life, and since he had little to lose, the doctors decided to try an experimental treatment. They removed a small patch of intact skin from his body and injected it with a retrovirus carrying a good copy of the broken gene. They sequenced the cells to make sure the new gene had been integrated. Then they grew those modified cells into large sheets of skin, the same technique used for burn victims. When they had enough new skin (nine square feet!), surgeons grafted it onto the boy’s body.

And it worked.

The skin grafts took root and thrived. Because they came from the boy’s own stem cells, there was no rejection. But with the repaired gene in place and functioning, they no longer had the deadly fragility of EB. In a series of operations over several weeks, the doctors replaced over 80% of the boy’s skin. 21 months later, he’s healthy and free of pain, with no sign of blisters. He’s able to play soccer with his father.

This isn’t a complete cure – EB sufferers can still get blistering in the mouth and throat, for example – but those symptoms are easier to control. More importantly, it’s a sunbeam of hope to thousands of people who suffer from a lethal disease that used to have no good options for treatment. Religion has done nothing to lift the shadow of this scourge from humanity, nor will it ever. Meanwhile, from vaccinations to genetic engineering, science is always giving us new powers to push back the darkness.

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