Coping with a Chronically Ill Child

BY: Jeanne Muchnick

 
I have this recurring dream. We're in the middle of some terrorist act, some crazy event where we can't find our loved ones and somehow, my l2-year-old daughter, Corey and I have become separated. She's with someone -- a neighbor I think -- but I can't find her. I keep checking my watch. If I don't get her next drink to her in time -- the "medicine" she needs every two hours to keep her blood sugar stable - she could die. Somehow, miraculously (this is a dream after all), I find her and am able to give her the simple Argo cornstarch and water mixture that she needs to stay alive. But it's the last solution I have, meaning Corey has about two hours more to live unless I can find more. I'm drenched in sweat and riddled with anxiety as I scour desperately through my war-torn town searching for a store that hasn't been decimated. I'm trying not to show Corey how frantic I am as I clasp her hand tightly and lead her through the rubble. She starts to complain that she is tired and I know time is running out. And then I wake up.

Corey has Glycogen Storage Disease (GSD) type la, a rare metabolic disorder which means her body is missing a liver enzyme necessary to convert the stored form of sugar (glycogen) into the usable form (glucose). Glucose is the essential source of energy for the brain and is the necessary fuel for every cell in our bodies. Because this enzyme is missing, she must be fed constantly in order to get the sugar required for her body to function. It's a genetic defect, and my husband and I both carry the recessive gene. At the time I was pregnant, in l992, there was no testing for this disease. Nor would we have even thought to be tested since there is no history of GSD in our families.

We spent six weeks at Columbia Presbyterian in NYC when she was seven months old trying to determine what was wrong with her after she had vomited nearly every formula on the market. Then another three weeks at Boston Children's when she was l8 months to get her on the cornstarch regime. Not to mention various hospitalizations in between -- first for a liver biopsy, then to put the gastrostomy tube in her stomach, as well as emergency visits due to the stomach flu. We tubed her for three and a half years, until finally we went to the Kennedy Krieger Center in Baltimore, where we spent another six weeks teaching her behavior modification techniques so she would drink her cornstarch, though we still tube-fed her at night. It was our first step towards a "normal" life.

The gastrostomy tube is our life-saver. It's a plastic tube that's been surgically attached to her stomach so that we can open and close it at will. It's how we can feed Corey when she won't take things by mouth --which happened a lot when she was little. Or when she's sleeping or sick.

Having GSD also means adhering to a restricted diet: no sucrose, fructose, lactose or glucose, so Corey can never chew an apple, taste a carrot, or lick an ice cream cone. Because she gets so much cornstarch during the day, she is never hungry, which makes mealtimes frustrating. And as with any other chronic disease, there are additional complications: physical and occupational therapy, a learning lag, self esteem issues.

There will never be any marathons for GSD or benefit concerts or celebrity-studded black tie events. It's an orphan disease -- just like others you've never heard of like progeria or alpha-l antitrypsin deficiency or neurofibromatosis. And like most rare conditions, there is no cure.

Despite the strides we've made in 12 years, I'm always focused on what's to come. It's become part of my internal makeup. I have no idea what kind of mother I'd be if Corey were "normal." As it is, I sit on the sidelines of many a "mom" conversation. It's difficult to concentrate on homework concerns, playdate escapades or sports accomplishments when I'm consumed with triglyceride levels, uric acid, heart function and cholesterol. The fact is, I'm scared all the time for Corey.

Corey calls me a worrywart, but I can't help it. Unlike other mothers who can fly out the door to run an errand with nothing more than their wallets, I can't leave the house without Corey's next drink -- plus a few extras -- in case of spillage. When the clock struck midnight on the year 2000, I had already stockpiled cases of cornstarch "just in case" something happened to cornstarch deliveries at my local grocery store because of millennium glitches. I keep an emergency backpack in my car for those times when I might get stuck in traffic or my car breaks down.

I worry when we fly about what the security screener is going to say when he sees my bag filled with vials of white powder, syringes and tubes. (I carry a note from my doctor but still.). I worry that Corey will never see the world after college. Backtrack: I worry about her going away to college and envision her home forever.

Corey's like an old-fashioned hour glass that runs out of fuel when the sand disappears. At night, we've been able to stretch her time limit to four hours, which means there are feedings at ll: 30 p.m., 3:30 a.m. and 6:30 a.m. I do the 3:30, which means I sleep really well between l0 p.m. and 3:30 a.m. but after 3:30 a.m. I'm usually up worrying about all that I need to do the next day. When I finally drag myself out of bed at 6:30 a.m., I feel like I haven't slept at all.

There are also the psychological issues, which are harder to "fix." Because Corey's liver is distended, it pushes out her other organs, making her stomach large in proportion to her body. Corey already thinks she's fat -- the "you have a medical condition" explanation doesn't really work on a budding teen who only wants to fit in with the crowd. It's these times -- often in The Limited dressing room after she's thrown a handful of shirts in my lap that our eyes lock and I want so badly to switch places with her, to say "Give me the missing enzyme. Let me deal with this disease," especially since we often leave the store empty-handed, as she tries to hide her tears.

I worry about boys -- how her first date might not understand why she needs to carry her cornstarch with her. Or if she'll even get a first date. I worry about her experimenting with alcohol and drugs -- hardly unusual for teenagers but deadly for her. I worry when she plays soccer in the hot sun that she'll collapse on the field, rather than tell the coach she's tired -- because no one else is.

I'm scared all the time for Corey, and for me and my husband and our marriage which is frayed at the edges due to sleep deprivation, financial stress and the anxiety of living with a child with a chronic disease. I try not to think of how our lives revolve around Corey, and how a part of me is angry that I no longer get to make my own choices: so many have now been made for me.

I also try to remind Corey (and myself) that everyone has challenges in their lives. Some people have them when they are younger, some when they are older. Some difficulties are easy to see, others are not. In the cracks, I remind myself of our accomplishments, small steps I never imagined possible. My mom would remind me to look at the glass as half-full, as opposed to half-empty. She battled breast cancer for years - always with a smile on her face and a sense of courage in her heart (though unfortunately, she lost her battle eight years ago). She would tell me the fable of the man who complained about his lot in life because he had no shoes. Until he looked over and saw a man with no feet. That story always helps me put my life in perspective. Whatever I have to deal with, there is always someone else out there who has it worse. Or different. Or just as challenging. And so I try to keep the hand I've been dealt with in perspective. And I try to keep the faith and optimistic spirit of my mom in my head at all times. I never thought, for example, that we'd be able to move away from the City (where Corey was born) to the suburbs, a good 45-minute drive away from our pediatrician who's been there since the beginning, and gives me his cell phone and beeper numbers every time he goes on vacation. But it's been seven years now and we've made it work. I also never thought Corey would be able to go to a friend's house for a sleepover, but a few special parents have been willing to be trained in the G-tube and have gotten up at night to feed her. I also worried about her independence and wondered how I'd be able to empower her to take responsibility for her own body. And indeed she's risen to the occasion with the same fortitude and determination that my mom had, cheerfully going off to sixth grade with an alarm watch and her drinks (which she can mix herself) in her backpack. She even takes the bus on occasion and checks in with me regularly from her cell phone.

And then there are those golden moments, when Corey surprises me with her maturity and sense of self. When she says "Mom, that's shirt too stretchy, forget it," at the mall, or when she says "no thanks" to a cookie offered to her at a bakery. Or when she doesn't cry at her annual visit to Boston Children's, where she's hooked up to an IV and for 24 hours they take her blood every hour on the hour to see how the cornstarch is holding.

I try to hope. I like to hope. Because though I can't control the nightmares that invade my sleep, I can hold on to my dreams.

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