My little sister, Gail, died of MLD (infantile-onset metachromatic leukodystrophy) when she was two.
I was four at the time, and my older sister was seven. MLD is a rare, terrible, genetically-inherited disease that a child is born with only if both of their parents are carriers. My older sister and I were born normally, without the disease, and Gail seemed completely normal for the first year of her life.
She met all of the first-year developmental milestones: smiling, sitting, crawling, standing, forming words. But just before her first birthday, she began exhibiting the ominous symptoms of MLD. Up until then, she was a healthy, happy baby -- with extraordinary beauty:
One day seemingly out of nowhere, she had a seizure. This was my parents' first alert that something was very, very wrong. It took some time for them to find out the final diagnosis since MLD is so rare, but when my parents finally did understand Gail's illness, they discovered there wasn't any cure for it, that it was progressive, and it was fatal.
During this time, my mother was pregnant with her fourth child, a boy. The doctors told her that the baby she was carrying had a 25% chance of having MLD. Although abortion was illegal at the time, she made the extremely difficult decision to terminate the pregnancy. She was able to do this legally "for medical reasons" and "with the recommendation of a doctor" because the doctor certified that if she were to have the baby with MLD, she would have a nervous breakdown (which is what ended happening anyway). For some reason that I'm not clear about, when she requested the abortion she also had to agree to having a full hysterectomy.
This whole terrible thing -- enduring a late-term abortion, a hysterectomy, watching her youngest daughter suffer and not being able to stop any of it -- pretty much did her in, emotionally.
Gail began having seizures more frequently and little by little, losing whatever functions she had. She eventually lost her ability to eat, so they had to insert a feeding tube. She started losing movement in her body until finally she was paralyzed and unable to even turn herself over in her crib. The medication they gave her to alleviate various symptoms such as the seizures gave her a bloated appearance and a fine coating of hair all over her little body. And finally, she went blind. My parents didn't tell me these details. When I grew up and was about to have my own children, I contacted the hospital and got copies of her medical records. Reading these records -- what my parents went through, details about how Gail really suffered -- gutted me.
After the abortion, my mom had to go away to a mental institution because she was so broken up. I don't know what happened to her there, because she never once talked about it, but it was there that she developed a hatred of psychiatrists and therapists. I suspect they asked her to tell them how she was feeling, and she could not even fathom dipping into her sadness at that time, or ever, because she would've drowned in that sadness. It was also in that hospital that she began her lifelong addiction to prescription drugs, especially sleeping pills -- anything that would knock her out, basically.
Finally my parents admitted Gail to the children's ward at Sonoma State Hospital because they could no longer take care of her at home. It's not a choice I'm comfortable with -- but I am not my parents, and attitudes and medical technology were different then. Sick children were hidden away in Draconian hospitals.
Credit: Sanfranman59 at Wikimedia Commons.
My father would visit her -- apparently my mother was too distraught to see her. I do not agree with many of the choices they made -- the termination of my little brother, who -- like me -- had a 75% chance at not having MLD, signing Gail into Sonoma State Hospital, my mom not visiting her, and I'm not sure about their choice to end life support, either -- but these were different times, and they were different parents than I am. My dad told me that once, he and his friend brought bagpipes into the children's ward and his friend played a soft lullaby beside Gail's crib even though they weren't sure she could hear them anymore. I remember accompanying my father on these trips and since I wasn't allowed in: I remember the waiting. I remember the terrible sadness that had moved in to our family; all of us were crushed beneath the weight of it.
Children who have MLD can live for perhaps 10 years as long as they have a feeding tube and survive the infections that commonly kill them such as pneumonia. My parents felt strongly that they didn't want to prolong her life; they felt they had already lost her and since the doctors told them that she had brain damage, was paralyzed, and was now blind, they wanted to end her pain. My father now had to make his own Sophie's Choice, and this was long before the infamous Karen Ann Quinlan right-to-die case: He asked the hospital to let her go.
They said no. They told him that he was trying to "play God" with his little girl's life.
He left, distraught, and when he returned he brought our family priest with him. (After Gail died, my parents became atheist-agnositcs, but before that we were active members in the Episcopal church.) Our family priest informed the hospital director that he was the one who was trying to play God, not my father, and that we needed to let go and let God make the decision.
After listening to our priest, the director agreed to let her go. My dad was with her when they discontinued her life support and feeding tube. He was alone with Gail when she died. He told me that right before she died, she squeezed his hand (for the first time in a long time), as if to say: Goodbye, Daddy.
"I like to think it was her way of saying goodbye, Dad, and thanks," he told me.
He wasn't able to talk about her very much because every time he did, he cried.
My parents didn't have a funeral for Gail. Probably because they were decimated. So my sister and I never really had closure on any of it. We thought she died of "ammonia." We never forgot about her. I still think of what life would be like if she was here. I remember her on her birthdays; at every holiday, I set a place in my heart for her. To be honest, I still cry when I think about her. It's a pain that never will heal.
When I grew up, I had a proper funeral for my beautiful little sister, and a reception in her honor. I put her name on a gold plaque in the memorial garden at St. John's Church in Ross, California, where my parents are now, to let the world know that she once was here, if only briefly. That she mattered then, and she matters still.
For more information on MLD:
- My Baby Reese: An MLD Awareness Video
- Eliza's Wish
- New Hope for MLD: Experimentation Has Begun!
- The Progression of MLD: Eliza Kate Williams
Ado (rhymes with Play Doh) from The Momalog
"Good enough parenting, one day at a time." I write about imperfect parenting from the perspective of a recovering ACOA.
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