By Julie DeVito
Five year old Greta Baier zooms by in her sleek new power-chair, curly blond ringlet curls bouncing, and a smile broad across her face. It’s a weekday morning at New York University’s Gramercy Green Residence Hall and Greta is waiting for the bus to school.
Greta exhibits warmth, friendliness, and a sense of hope that is hard to shake when you first meet her. She likes Play-Doh, coloring, dress up, puzzles, and cooking. She also likes to sing and her favorites include Lenny Kravitz, The Killers, and Adele.
She exhibits a curiosity and caring about the people she knows, stopping one day to ask me about the man I was talking to on the street.
But behind the door of 1705 Third Avenue, Greta, who suffers from a form of congenital muscular dystrophy, lives in a world of nurses and medical equipment.
Greta, the daughter of Kate Baier, and her partner Lyn Krueger, suffers from a form of muscular dystrophy called nemaline myopathy.
Baier, Senior Director of Residential Life at NYU, explained that with neuromuscular disorders like nemaline, you’re balancing the strength of the muscle, against the size of the body. Baier said that Greta, who is confined to a wheelchair will likely never walk .
“Her muscle strength won’t decrease, but as her body gets bigger, it takes more strength, so her muscle strength and her body mass might not always be in alignment,” Baier said. “We see her growing all the time, but if she has a growth spurt, she loses the ability to do something until her body catches up with it again.”
Nemaline myopathy is a genetic disorder that affects about 500 people living in the United States. While there is no treatment for the disease, occupational and physical therapy help increase functioning. Although the disorder is rare, there are specialists and resources available, especially in the major cities.
Greta does not interact with many other children with her same disorder, but interacts with other special needs children in school and play groups.
David McDougall, of nemaline.org, organizes resources for those who suffer from nemaline. According to McDougall, nemaline presents itself in many forms and severities and each one can have a variety of symptoms.
“The prognosis is usually good with today’s standard of care and medical equipment,” McDougall said. “But the more severe the NM, then the more care is required. The milder forms often just hinder a person’s life and abilities, sometimes only vaguely.”
The most severe cases can mean complete paralysis and the patient is unable to communicate, he said.
“We do know that [Greta] gets stronger all the time, so we are optimistic that she might be able to be off the vent for periods of time in the future,” Baier said.
Baier said that most children with this type of Nemaline don’t live past the age of two because of respiratory complications, but Greta survived respiratory failure.
“I think that people are really inspired by her,” Baier said. “So often, kids with her level of disability also have other communicative disorders or developmental delays, so I don’t think people expect her to be as smart as she is. People are impressed with how motivated she is. She has a real drive to be stronger and independent.”
In 2008, Kate and Lynn agreed that they wanted to conceive a child. After considering a variety of options, they decided on artificial insemination, with an anonymous sperm donor, and Lynn as the carrier.
“We felt like we were ready and we wanted to have a family,” Baier said.
They met at Bell State University in Muncie, Indiana where they were both employed as Hall directors. The couple, who was not able to be married at the time through New York State Law, went through a complicated process of second child adoption to have shared parenthood of Greta.
Baier said that they knew almost immediately that something was off. Babies that have neuromuscular disorders are floppy, almost like a bag of jelly. But as first time parents she said they didn’t grasp the magnitude of it until they held another baby with more defined muscle tone.
“For me, I’m neurotic, I worry about crazy stuff,” Krueger said. “It made me realize how strong we really are. You’re presented with a challenge and you figure out a way. You live for the moment.”
When Greta was born not breathing and with mild brain damage, doctors originally diagnosed her with cerebral palsy.
In 2010, at the age of 18 months, Greta went into respiratory failure while at home. She was in the ICU for a month, where she was diagnosed with nemaline and put on a tracheostomy tube, that she remains on today.
“We don’t think of her as being sick, but she’s definitely not like other kids,” Baier said.
The first shock for Baier and Krueger was that Greta had to be put on a ventilator, which she relies on to breathe.
All of her medical equipment is stored in her power chair and bags when she is at school. She goes to pre-school at the Roosevelt Children’s Center which is a special education school. Her classmates have varying degrees of Down’s Syndrome, autism, and development and communication delays. Baier said that at school, the teachers create a curriculum specific to Greta’s needs. For one hour at school, Greta uses a stander, which is a machine that positions her in a standing position to allow her to bear weight on her body, promoting bone and muscle development. She also receives occupational, physical, and speech therapy at school.
They said that the second shock was that there would be a nurse with them 16 hours a day. They have a nurse with them for most of the day, even on the weekends. This is paid for by insurance, but only if the nurses are there enough hours for them to be considered needed. Thus, the couple has nurses there on the weekends. Although it was different at first, Baier and Krueger said that they adjusted.
They can’t have anyone watch Greta other than a nurse or a trained family member who is familiar with her disorder.
“You’re mourning the loss of everything you envisioned about your child,” Baier said. Very strong “And then you’re just so happy to be home. It’s easy to get used to having a nurse living with you when you were living in the hospital.”
The two interact frequently with parents of kids with disabilities, but Krueger said some parents don’t get over the mourning stage of having a child with a disability.
“I think there are two groups of people,” she said. “There are parents who are past the kind of feeling sorry for yourself and for your child and are just going to do whatever you can to advocate for them and help them to have the most normal typical life that they can have. And then you have the parents that are constantly feeling sorry for themselves.”
They also said that there is a high separation rate for parents of children with disabilities
“When something like this happens, you’re either going to step up and make the best of it or you’re going to feel sorry for yourself for the rest of your life,” Krueger said.
Krueger worries that Greta will be shy but Krueger and Baier are focused on instilling in Greta the need to be outgoing and advocate for herself. When kids ask about her equipment, Baier said that they let Greta decide how to respond.
“We try to empower her and teach her how to handle people who are intrigued by her. A lot of people approach her and don’t know what to do.”
New York City has an early intervention program where kids are evaluated in order to determine need and services that they are eligible until the age of three. When Greta was in the program, she received Occupational, Physical, and Speech therapy.
Greta is currently in a special education program good that provides her with the therapy services that she received in the early intervention program. She goes to school through coordination from the Committee for Pre-School Special Education, part of the Board of Education.
The city provides them with all the resources that they need for Greta, Baier says. She also receives Medicaid through the Care at Home waiver program that overrides the income considerations to provide medical services for children who would otherwise be permanently hospitalized or institutionalized.
Where most children at age three are exploring their environment, Greta was exploring virtually, mostly on the iPad and doing creative play. Baier said that Greta will pretend to read books, making up the story as she goes along. Greta has adapted to her limitations in mobility by challenging herself intellectually, her parents say.
A day in the life of Greta is busy with school, therapy, and social outings, much like any other girl her age.
“She’s a child that likes structure. We do things in very patterned ways because there’s a lot that needs to happen.”
Her day begins with a four-phase nebulizer treatment to clear out her lungs to help her breathe and cough up the mucus blocking her airway.
When her parents are not with her, a nurse monitors her day and night.
“She is the epitome of needing a village to raise a child but we have a really great village,” Baier said.
Although Baier and Krueger do not attend support groups, they have connected with other families of kids with nemaline, some in the city and some that they communicate with via Skype.
For McDougall, the chat group and Facebook page “Nemaline Myopathy Global Awareness” have been invaluable sources for the people he interacts with.
He strives to put families in touch with others who have nemaline.
“The NM Conventions have been a vital part in creating face-to-face opportunities between families and research scientists and support staff,’’ he said. “The meetings have made a huge difference to self- confidence and breaking down the feeling of ’being alone’ with such a rare disorder, which so many people do not understand.’’
Since Greta does not have a degenerative form of nemaline, she can get stronger in time. Krueger believes that there’s something innately special about Greta. Greta is motivated to walk one day and tells Baier that when she’s older she’ll be able to run or have her trach out. When Baier responds telling her that if she works hard to get strong, maybe she will walk, Greta responds, “Well, maybe I will.”
“There is something about her that tugs at people’s hearts,” Baier said. “I just think there’s she’s destined to do something really special. She’s inspiring. She inspires you to be a better person and not give up because she hasn’t.”
Her parents have chronicled her life in a blog since her birth. Through her blog, Greta’s story is shared with family members, friends, and other families with special needs children.