I hate Pelizaeus-Merzbacher disease (PMD).
On March 21, 2003, I was told that PMD was the cause of my son Jacob’s many medical problems. I’ve hated the disease ever since. When I learned that it is degenerative and would continue to burrow into my son’s brain and nervous system like a voracious mole, until it ate away his abilities to think, to breathe and to live, I detested PMD. And when I witnessed my son on a ventilator two months ago because the disease had so weakened his immune system that a cold required him to have mechanical assistance to stay alive, my hate relationship was in full swing.
These days, I can’t think of PMD without my heart twisting with loathing. This is hate to the extreme. When Jake was hospitalized for the first three months of his life, I repeatedly said it wasn’t fair. Why was he, an innocent newborn with a whole life of possibilities ahead of him, suffering such excruciating pain and struggling so hard to breathe? It didn’t take long for me to realize that the saying “life isn’t fair”—often used half-heartedly by my parents when I complained as a teen about not being allowed to go to a party with friends—is 100 per cent accurate. Life isn’t fair and my son’s disease is proof of it.
Life’s cruelty and my abhorrence of PMD crashed into our house when I had to explain to my twin daughters that Jake’s PMD was worsening.
The fact that Jacob has PMD is wrong. The rapid change in his health is completely undeserved. And the fact that Jamie and Sierra have to experience all this, alongside their brother, is unreasonable in its heartlessness. At ten years old, Jamie and Sierra are forced to witness this injustice every day.
When Sierra and Jamie were six years old, they gave their friends an impromptu genetics lesson by explaining the inheritance pattern of the disease that affects their big brother. They’ve always known that Jacob’s care is difficult and there are nurses in his room every night to ensure his medical safety. But until recently, they didn’t realize that PMD is a fatal disease and that despite our efforts at researching a cure, their brother might not live long enough to benefit from medical breakthroughs.
I’ve learned that people adapt to changing conditions. I remember saying that I wouldn’t be able to stick a tube down my son’s throat and suction out the mucous that was blocking his airway and causing him to turn blue. Now, I do this every day and my hands remain surprisingly steady during the process. When Jamie used to hear the rumble of the suction machine, she would stop what she was doing and put her hands over her ears to block out the awful sound. But, last week, as I was threading the tube down Jake’s airway, Jamie held her brother’s hand and talked him through the entire procedure, even wiping away the tears that pooled at the edges of his eyes. There was so much injustice in that scene, but also a ton of love.
My daughters experience things on a daily basis that youngsters should not have to bear, and that is deplorably unfair. They are aware of the frailty of their brother’s lungs, they know how to read his oxygen monitors and understand what numbers indicate a dangerously high heart rate. They dissolve into tears when he needs to go to the emergency room because, in their experience, that is the start of a long hospitalization and a complete disruption of their lives.
Caring for Jacob is extremely challenging and scary at times. He can go from breathing quietly to sounding like he is drowning in his saliva in seconds. The level of oxygen in his blood can plummet from a safe 98 per cent to a dangerously low 77 per cent in the blink of an eye. The doctors and nurse practitioners have taught me how to care for him and when to intervene. So for the most part, I know how to “manage” my son’s precarious medical needs. I don’t like it, but I am adjusting to the new level of care that Jacob requires.
Late at night, when the house is quiet, save for the humming of the oxygen concentrator in Jake’s room, what keeps me awake is thinking about how PMD is affecting my twin daughters.
Prior to their births, I read a lot about siblings of kids with special needs. Everything I came across consistently stated that kids raised in homes with atypical children grew into empathic, sensitive and caring adults. I do believe that, and have already witnessed several examples of my daughters’ concern for the feelings of their friends. They display a level of compassion beyond their years.
Sierra and Jamie are forced to cope with things many adults are unable to fathom. My heart shatters when they come home from school crying because their friends don’t understand what it’s like to live with a disabled brother whose health is so fragile. I have to explain that most of their friends aren’t emotionally equipped to support them as they express their fears. And that, too, isn’t fair.
How do I hold it together when they ask questions like “Why doesn’t home feel like home anymore?” or “Why does my life have to be harder than everyone else’s at school?” or “Will a cure for PMD be discovered in time for Jake?”
These are questions about which little kids shouldn’t have to wonder, and issues from which I desperately wish I could shield my daughters. But I can’t. So we talk. In the car, on our way to an activity, we talk about why we don’t travel like their friends’ families. And at dinner, we talk about whether the auditorium where the girls are performing in a play is wheelchair accessible so Jacob can come and watch. And late at night, in the darkness of their rooms, we chat about how unfair it is that their brother has PMD, that he can’t tell us exactly what he’s thinking or feeling and that they are scared to be alone with him in case he stops breathing.
We love Jacob with every fibre of our beings. But we hate PMD.
This piece originally appeared in Her Magazine. You can follow Marcy White on her blog Cure PMD or read her book The Boy Who Can.