The Mom gene
Maternal instinct kicks in for mothers faced with children's chronic illnesses

By Janet Simons, Rocky Mountain News
May 2, 2005

http://www.rockymountainnews.com/drmn/family/article/0,1299,DRMN_107_3744295,00.html

Mothers of children with rare disorders do more than soothe brows and sing lullabies. They're also diagnosticians who "just know" that something isn't right.

They're medical researchers who spend long hours on the Internet. They're physical, respiratory and occupational therapists, dietitians and the world's most stubborn patient-rights advocates.

It's a different kind of heroism, but it still saves lives. With Mother's Day coming Sunday, we talked to mothers who face much more serious challenges than most.

Dr. Daniel Hall, medical director of the newborn intensive care unit at Children's Hospital, has seen many, many mothers stay next to their children's beds night and day.

"It never ceases to amaze me how mothers who are stunned and grieving because they've just received bad news are able to rally to provide the care that their babies need most," Hall said. "They start by providing something as simple and necessary as breast milk."

And when a chronically ill child gets home from the hospital, a lifetime of medicating, analyzing and therapy begins. Often, Mom is the designated first responder in a care team that also includes Dad, grandparents, siblings, health-care professionals and child-care providers.

Hall said mothers and other family members are able to provide constant, loving commitment that medical professionals can't offer, and that he's consistently seen children who receive that kind of attention make stronger, more rapid gains than children who don't.

"It can be exhausting for mothers who may have other children and responsibilities and health issues of their own," he said. "But it makes a huge difference for the children. To my mind, that's every bit as heroic as running into a burning building to save a child."

Hall said when a rare disease doesn't manifest itself at birth - as with Rett syndrome and Moyamoya disease, for example - mothers are the ones who have to convince their children's pediatricians that, although the child is within normal standards, something is wrong.

"I think the mother's observation and intuition are most often right," Hall said. "These diseases can be fairly subtle, and the sooner they're recognized the better it is for the child. Most pediatricians learn to listen to mothers who tell them something just isn't right about the way their child is developing."

Here are four examples of children with rare, serious illnesses and their mothers, all of whom are thriving and learning from each other:

Chase Grey, 8 months, Lafayette

• Prader-Willi syndrome: Children born with Prader-Willi syndrome can't recognize that their appetite for food has been satisfied. They are identified at birth by their "floppy" muscles, have moderate developmental disabilities and can become very hard to manage as they enter adolescence.

• Cause: A defect on the 15th chromosome

• Treatment: Hormone shots and speech, physical and occupational therapy. Diet and access to food must be constantly monitored and controlled.

• Incidence: One in 12,000 to 15,000 births

• Prognosis: If morbid obesity can be prevented, people with Prader-Willi can have normal lifespans.

Chase Grey looks like any other 8-month-old as he sits in his infant seat on the living-room floor, but the games his mother, Robin Grey, plays with him have a larger purpose.

"This is a special seat," Grey explains. "It makes him use his stomach muscles to sit up and helps his muscle tone. What we're doing is disguised as fun now, but it's going to get a lot harder."

The current challenge is to get Chase to sit up by himself, something most normal babies do by 8 months. Then they'll work on walking. But the hardest job has yet to begin - controlling Chase's eating.

"For the first two years, we have to try to get him to eat because his sucking response is so weak," she said.

The hunger usually kicks in at age 2, she said, and from then on meals, snacks and exercise will have to be closely controlled. Grey has joined the Colorado chapter of the Prader-Willi Association and has been getting lots of tips from mothers of older children with the disorder.

"Managing Chase's weight will probably be really hard. But when you look at the big scheme of things, this isn't a very serious problem," she said.

• Kincaid Bimler, 10, Littleton

• Moyamoya disease: Moyamoya creates irreversible blockage of the main blood vessels to the brain as they enter the skull, which tends to cause strokes or seizures.

• Cause: Unknown

• Treatment: Surgical replacement of the blood vessels. Kincaid had this operation four years ago.

• Incidence: 1.2 cases per 100,000 people

• Prognosis: The outlook is hopeful for patients who have the surgery before serious brain damage occurs.

Karen Bimler watches Kincaid play basketball in the front yard and says she has been amazed by what she learned from her son's brush with death.

"I always wondered whether I'd have that mothering instinct," she said. "I learned that I did."

It came into play when Kincaid walked into her bedroom one morning "holding his arm funny." She called the pediatrician, who told her he probably had just slept on it wrong. Three months later, Kincaid had a small stroke that slightly crippled his left side. The doctors at Children's Hospital diagnosed Moyamoya disease and in 2001, Kincaid had surgery to replace the blood vessels leading to his brain.

She watches him closely for any sign of weakness in the new vessels, which were taken from Kincaid's jaw.

"I've always been a helicopter mom," she said, using a term developed at the Love and Logic parenting center in Golden to refer to overprotective, "hovering" parents, "but it's really hard not to be when any headache could be something more."

• Andrew Adams, 6, Aurora

• Plastic bronchitis: Patients with plastic bronchitis form fibrous "plastic" casts of the bronchial tubes in their lungs that can block their airways. Patients sometimes cough up these rubbery, branching casts.

• Cause: Unknown, although it is a rare complication of Fontan surgery, a procedure to correct a chronic heart defect that Andrew underwent in April 2004.

• Treatment: Andrew's family has been administering nebulizer treatments of heparin, a blood thinner.

• Incidence: Estimates vary widely, but it is quite rare.

• Prognosis: There's a high risk of life-threatening lung complications, especially among children with underlying heart problems in their first year after surgery. Plastic bronchitis also can go away completely.

Jessica Adams quit her job as nursery director at a fitness center shortly after Andrew was diagnosed with plastic bronchitis last winter.

"For about 21/4 months, he was in the hospital more than he was out, and I just had to quit," she said. "And I'm glad, because it's one less thing to worry about. Now I can focus on him."

She also focuses on Andrew's 8-month-old brother, Ryan. Both boys recently had influenza B, a bout that took Andrew back to the hospital for one night.

Sometimes just monitoring and administering Andrew's medication can seem like a full-time job. In addition to the nebulizer, his parents treat him with an airway clearance machine and monitor his blood oxygen levels.

"When we get to the hospital or the doctor's office, I'm the one who has to explain his condition, because it's so rare," she said. "The doctor asks me, 'What are we doing for this? What's the plan?' It's scary. I say, 'I don't know. I thought that was your job.' "

What an article. It was very refreshing to see something that gives hope instead of taking it away.

The word is getting out more and more.

DJ...my gosh...described me to the T !!!!! especially when it said when mothers have other kids to care for and health issues of their own.  Very interesting article especially when they used MoyaMoya  as one of the disorders....I thought the odds were like one out of 2 million ???  Maybe not as rare but not as well known yet.

Have a GREAT day.  CarasMOM

Thanks, DJ - that was a great article and a great Mother's Day gift, as well.  The incidence ratio was surprising.

I contacted the author of the article. The family mentioned only lives a few hours from me. I am looking forward to talking to someone in person about MM and meeting them.

Rena