dudden22
06-13-2005, 01:33 PM
This is so sad! There are a few FM mentions
"It's about a little girl who sings along to Fleetwood Mac and plays a mean game of Uno, working hard to grow up."
She can play outside only at night
Sunshine darkens world of 4-year-old
CHRIS ROSENBLUM
BELLEFONTE, Pa. - Sunshine can be friendly to Kasey Knauff.
It greets her in the morning with a rainbow on the ceiling of her parents' room. It bathes a special teddy bear that doubles as a night light. Her bedroom mirror is shaped like a sun, and little orbs join moons and stars on her quilt.
Mostly, though, the rays are Kasey's enemy.
Instead of caressing her skin, they burn. They can blind. They keep her inside her Bellefonte home during the day, watching the world from her living room window. She can play outside only in darkness. Fluorescent lights are mortal dangers.
This is what it means to have congenital erythropoietic porphyria.
In the four years since her birth, Kasey has struggled with CEP, a rare genetic disorder that makes her skin extremely sensitive to light. Fewer than 200 cases have been reported worldwide.
Because of an enzyme deficiency, natural chemicals called porphyrins build up in her bone marrow, red blood cells and teeth. Exposure to strong light can cause severe blistering and infections.
Kasey already has suffered a lifetime of hurt.
Scars from burns suffered when she was an infant cover her abdomen, face, arms and legs, despite plastic surgery and several skin grafts. Five times her left leg has fractured, the result of brittle bones from her condition.
Yet, Kasey's story has more than pain.
It's about a little girl who sings along to Fleetwood Mac and plays a mean game of Uno, working hard to grow up.
It's about a family persevering through confusion and fear.
And it's about the village of therapists, nurses and friends in Kasey's corner, making sure the sun doesn't ruin a childhood.
"Isn't she special?" Jenn Miller, one of Kasey's nurses, said. "I'm thankful for every day I get to spend with her."
Hospital lights burn the baby
Doctors didn't know. Nobody knew.Born April 10, 2001, Kasey went under the normal nursery lamps. That was bad enough. But she looked jaundiced and complications developed. Mount Nittany Medical Center sent her to Geisinger Medical Center in Danville, Pa.
With nothing on but a diaper, she was placed under stronger bilirubin lights.
"That, of course, made everything worse," said Kurt Knauff, her father. "She went from bad to worse in the next 12 hours."
Brenda Knauff, Kasey's mother, checked herself out of Mount Nittany to be with her baby. Family members arrived.
Meanwhile, unbeknownst to everyone, Kasey was burning.
The Knauffs received an early-morning call from the hospital. Kasey was getting worse. Come immediately. "She was ballooning up before our eyes," her father said. "And they didn't know what to do."
Kasey was turning blue and red. Her heart rate jumped. The hospital suspected a viral infection but scrambled to solve the mystery.
Blood transfusions and morphine dripped into Kasey. Her family sang to her and prayed.
After more than two weeks, a sunburn finally provided the answer. A dermatologist noticed the similarity between Kurt Knauff's redness and Kasey's. Underneath monitor patches, her skin was white. From medicine's obscure corners, the dermatologist came up with a diagnosis of CEP.
Kasey came home covered in purplish blisters that became thick, red scar tissue.
In no way, the Knauffs said, do they blame the hospital.
"The doctors, the way we feel about it, they gave her life," Kurt Knauff said.
Scar tissue deforms Kasey
They were afraid of light.
How much was OK? They weren't sure. But no further harm would come to their baby. Kurt Knauff pulled the blinds, checked for escaping glimmers. At night, he pushed lampshades down.
Eventually, he, Brenda and their older daughter, Kylie, relied only on candles and small battery-powered lights. They changed Kasey's diapers and dressings in the television's glow.
"It looked like midnight in the middle of the day in the house," Miller said.
So rare is CEP, doctors could scrounge up only a pamphlet for the Knauffs. They needed more information. One by one, they tracked down the pamphlet's sources: a physician in San Francisco, one in Cincinnati, a researcher in Boston.
The Knauffs found out about a possible cure, requiring a bone-marrow transplant. And through the Cincinnati doctor, they met two families whose children had CEP. Among other things, the Knauffs learned that 40-watt lights were safe. They didn't have to live in a cave.
They had hope.
Donor tests yielded no matches from the family. Kasey joined international bone marrow and stem cell donor registries. Then a more immediate problem cropped up. Kasey's scar tissue wasn't growing with her, deforming her limbs and torso. Her thumb was pinned to her forearm.
The family couldn't find anyone willing to do such delicate surgery on a tiny body. One Philadelphia surgeon said a finger would have to go to save the thumb. The Knauffs balked. At a friend's suggestion, they contacted the Lehigh Valley Hospital's burn recovery center. A surgeon agreed to do the operation without amputating. But the Knauffs' insurance wouldn't approve the facility.
Kurt Knauff vowed to pay himself, whatever it took. He packed up his family for Lehigh Valley after threatening to tell their story to a local TV station.
En route, his cell phone rang. Their insurance would pay.
Kasey got her thumb back.
"We gave her a rattle so she could rattle," Brenda Knauff said. "That was a big deal for us."
Dreaming for nightmare to end
Kasey looks forward to the garbage man.He'll drive by her living room window, tinted like all panes in the house to screen out ultraviolet light. Even so, Kasey usually dons protective goggles. She kneels on the couch and waves. He always waves back.
When she grows up, she'd like to be on his side of the glass.
"Kasey wants to be the driver so she can wave and blow kisses to all the kids watching in the window," Miller said.
For now, it's just a dream for a girl who must hide under a special ultraviolet-resistant blanket or step into the kitchen whenever the front door opens, throwing unfiltered sunlight into the living room. Kasey rarely ventures outside during the day.
She has fed a nearby park's ducks and fish at night. She has gone sledding in the dark. Some restaurants are fine, if the lighting is dim. Darkened movie theaters are safe.
`She has amazing skills'
Usually, Kasey's eyes twinkle in merriment. But sometimes, they appear grave beyond her years, pooled with knowledge no child should possess.
"She's an old soul trapped in a young girl," Walsh said.
Few children have had a body cast, skin grafts and several operations before kindergarten. Even fewer have a medical port flushed out.
It goes straight from her chest to her heart, installed last year because her scars make finding a vein for intravenous tubes difficult. Every six weeks, a nurse cleans it with a saline solution.
The nurse arrives one morning as Kasey is playing in her favorite yellow Sesame Street chair. Her smile dissolves into a frown. Tears appear once the procedure starts.
"Hold me," she whimpers in Miller's arms.
She utters the same plea when the bandage around the angry splotches on her stomach is changed.
Getting dressed and playing are more fun. Kasey's nurses, paid by the state, are both caregivers and friends once her mother goes to work at Penn State, her father to nursing classes and her sister to school.
Miller brushes Kasey's hair one day at a toy vanity.
"Hold on, let me chase the rats away," Miller says. "You don't have any rats today. They must have hopped out in the tub."
Down the hall, a Fleetwood Mac album thumps softly. Kasey wasn't even 2 when Miller first played it and swayed with her to help her walk. They still dance to it.
Other times, Kasey makes crafts and plays card games or with her doll house. But lately, she also has had to work.
Therapists from Central Intermediate Unit-10 preschool services visit weekly. Her scarred fingers need exercise. Her legs, weakened from her breaks, need to be strengthened. Her speech, because of her facial scars, sometimes slurs.
By all accounts, she's an ace student.
"Kasey would be the star of any preschool class," Shawn Franks, an occupational therapist, said as Kasey deftly snipped a heart from construction paper. "She has amazing skills."
As long as the therapists are there, the happy-go-lucky side of Kasey that loves answering the phone and baking cookies with her sister disappears.
Franks offers help with one exercise. No, Kasey replies.
"Oh, Kasey, you can do anything," Franks says.
"Yep," Kasey says. "I can do anything."
Isolation and uncertainty
Kasey has Oreo, her hamster. She has a tiger cat, Lucky, and plenty of stuffed animals.
What she lacks are friends her age.
"Name one kid who's at the park when we're there at 9 o'clock," Kurt Knauff said. "She doesn't know how other kids act."
Brenda Knauff hopes Kasey will make friends once she's in school. To go, she'll need a few things -- tinted classroom windows, covered lights and an outfit made from material used to clothe astronauts. A Maryland woman, through a nonprofit foundation that helps children with genetic disorders, is sewing Kasey a pair of full-body suits to protect her from light and let her go outside for brief periods.
There are other challenges. While walking, she still needs her hand held to keep from falling. As she grows, her scars will require more operations, which in turn will produce scars. This summer, her right leg -- almost 2 inches shorter than her left from scarring -- will be lengthened.
Much remains uncertain. Without a transplant, she could live at least to middle age. Or the stress on her spleen, liver and other organs could shorten her years.
All the Knauffs can say for sure is this: They're thankful for all the help so far and for the joy their little survivor brings.
Says Kurt Knauff: "She bounces right back. She's the greatest."
http://www.charlotte.com/mld/charlotte/living/health/11881164.htm
"It's about a little girl who sings along to Fleetwood Mac and plays a mean game of Uno, working hard to grow up."
She can play outside only at night
Sunshine darkens world of 4-year-old
CHRIS ROSENBLUM
BELLEFONTE, Pa. - Sunshine can be friendly to Kasey Knauff.
It greets her in the morning with a rainbow on the ceiling of her parents' room. It bathes a special teddy bear that doubles as a night light. Her bedroom mirror is shaped like a sun, and little orbs join moons and stars on her quilt.
Mostly, though, the rays are Kasey's enemy.
Instead of caressing her skin, they burn. They can blind. They keep her inside her Bellefonte home during the day, watching the world from her living room window. She can play outside only in darkness. Fluorescent lights are mortal dangers.
This is what it means to have congenital erythropoietic porphyria.
In the four years since her birth, Kasey has struggled with CEP, a rare genetic disorder that makes her skin extremely sensitive to light. Fewer than 200 cases have been reported worldwide.
Because of an enzyme deficiency, natural chemicals called porphyrins build up in her bone marrow, red blood cells and teeth. Exposure to strong light can cause severe blistering and infections.
Kasey already has suffered a lifetime of hurt.
Scars from burns suffered when she was an infant cover her abdomen, face, arms and legs, despite plastic surgery and several skin grafts. Five times her left leg has fractured, the result of brittle bones from her condition.
Yet, Kasey's story has more than pain.
It's about a little girl who sings along to Fleetwood Mac and plays a mean game of Uno, working hard to grow up.
It's about a family persevering through confusion and fear.
And it's about the village of therapists, nurses and friends in Kasey's corner, making sure the sun doesn't ruin a childhood.
"Isn't she special?" Jenn Miller, one of Kasey's nurses, said. "I'm thankful for every day I get to spend with her."
Hospital lights burn the baby
Doctors didn't know. Nobody knew.Born April 10, 2001, Kasey went under the normal nursery lamps. That was bad enough. But she looked jaundiced and complications developed. Mount Nittany Medical Center sent her to Geisinger Medical Center in Danville, Pa.
With nothing on but a diaper, she was placed under stronger bilirubin lights.
"That, of course, made everything worse," said Kurt Knauff, her father. "She went from bad to worse in the next 12 hours."
Brenda Knauff, Kasey's mother, checked herself out of Mount Nittany to be with her baby. Family members arrived.
Meanwhile, unbeknownst to everyone, Kasey was burning.
The Knauffs received an early-morning call from the hospital. Kasey was getting worse. Come immediately. "She was ballooning up before our eyes," her father said. "And they didn't know what to do."
Kasey was turning blue and red. Her heart rate jumped. The hospital suspected a viral infection but scrambled to solve the mystery.
Blood transfusions and morphine dripped into Kasey. Her family sang to her and prayed.
After more than two weeks, a sunburn finally provided the answer. A dermatologist noticed the similarity between Kurt Knauff's redness and Kasey's. Underneath monitor patches, her skin was white. From medicine's obscure corners, the dermatologist came up with a diagnosis of CEP.
Kasey came home covered in purplish blisters that became thick, red scar tissue.
In no way, the Knauffs said, do they blame the hospital.
"The doctors, the way we feel about it, they gave her life," Kurt Knauff said.
Scar tissue deforms Kasey
They were afraid of light.
How much was OK? They weren't sure. But no further harm would come to their baby. Kurt Knauff pulled the blinds, checked for escaping glimmers. At night, he pushed lampshades down.
Eventually, he, Brenda and their older daughter, Kylie, relied only on candles and small battery-powered lights. They changed Kasey's diapers and dressings in the television's glow.
"It looked like midnight in the middle of the day in the house," Miller said.
So rare is CEP, doctors could scrounge up only a pamphlet for the Knauffs. They needed more information. One by one, they tracked down the pamphlet's sources: a physician in San Francisco, one in Cincinnati, a researcher in Boston.
The Knauffs found out about a possible cure, requiring a bone-marrow transplant. And through the Cincinnati doctor, they met two families whose children had CEP. Among other things, the Knauffs learned that 40-watt lights were safe. They didn't have to live in a cave.
They had hope.
Donor tests yielded no matches from the family. Kasey joined international bone marrow and stem cell donor registries. Then a more immediate problem cropped up. Kasey's scar tissue wasn't growing with her, deforming her limbs and torso. Her thumb was pinned to her forearm.
The family couldn't find anyone willing to do such delicate surgery on a tiny body. One Philadelphia surgeon said a finger would have to go to save the thumb. The Knauffs balked. At a friend's suggestion, they contacted the Lehigh Valley Hospital's burn recovery center. A surgeon agreed to do the operation without amputating. But the Knauffs' insurance wouldn't approve the facility.
Kurt Knauff vowed to pay himself, whatever it took. He packed up his family for Lehigh Valley after threatening to tell their story to a local TV station.
En route, his cell phone rang. Their insurance would pay.
Kasey got her thumb back.
"We gave her a rattle so she could rattle," Brenda Knauff said. "That was a big deal for us."
Dreaming for nightmare to end
Kasey looks forward to the garbage man.He'll drive by her living room window, tinted like all panes in the house to screen out ultraviolet light. Even so, Kasey usually dons protective goggles. She kneels on the couch and waves. He always waves back.
When she grows up, she'd like to be on his side of the glass.
"Kasey wants to be the driver so she can wave and blow kisses to all the kids watching in the window," Miller said.
For now, it's just a dream for a girl who must hide under a special ultraviolet-resistant blanket or step into the kitchen whenever the front door opens, throwing unfiltered sunlight into the living room. Kasey rarely ventures outside during the day.
She has fed a nearby park's ducks and fish at night. She has gone sledding in the dark. Some restaurants are fine, if the lighting is dim. Darkened movie theaters are safe.
`She has amazing skills'
Usually, Kasey's eyes twinkle in merriment. But sometimes, they appear grave beyond her years, pooled with knowledge no child should possess.
"She's an old soul trapped in a young girl," Walsh said.
Few children have had a body cast, skin grafts and several operations before kindergarten. Even fewer have a medical port flushed out.
It goes straight from her chest to her heart, installed last year because her scars make finding a vein for intravenous tubes difficult. Every six weeks, a nurse cleans it with a saline solution.
The nurse arrives one morning as Kasey is playing in her favorite yellow Sesame Street chair. Her smile dissolves into a frown. Tears appear once the procedure starts.
"Hold me," she whimpers in Miller's arms.
She utters the same plea when the bandage around the angry splotches on her stomach is changed.
Getting dressed and playing are more fun. Kasey's nurses, paid by the state, are both caregivers and friends once her mother goes to work at Penn State, her father to nursing classes and her sister to school.
Miller brushes Kasey's hair one day at a toy vanity.
"Hold on, let me chase the rats away," Miller says. "You don't have any rats today. They must have hopped out in the tub."
Down the hall, a Fleetwood Mac album thumps softly. Kasey wasn't even 2 when Miller first played it and swayed with her to help her walk. They still dance to it.
Other times, Kasey makes crafts and plays card games or with her doll house. But lately, she also has had to work.
Therapists from Central Intermediate Unit-10 preschool services visit weekly. Her scarred fingers need exercise. Her legs, weakened from her breaks, need to be strengthened. Her speech, because of her facial scars, sometimes slurs.
By all accounts, she's an ace student.
"Kasey would be the star of any preschool class," Shawn Franks, an occupational therapist, said as Kasey deftly snipped a heart from construction paper. "She has amazing skills."
As long as the therapists are there, the happy-go-lucky side of Kasey that loves answering the phone and baking cookies with her sister disappears.
Franks offers help with one exercise. No, Kasey replies.
"Oh, Kasey, you can do anything," Franks says.
"Yep," Kasey says. "I can do anything."
Isolation and uncertainty
Kasey has Oreo, her hamster. She has a tiger cat, Lucky, and plenty of stuffed animals.
What she lacks are friends her age.
"Name one kid who's at the park when we're there at 9 o'clock," Kurt Knauff said. "She doesn't know how other kids act."
Brenda Knauff hopes Kasey will make friends once she's in school. To go, she'll need a few things -- tinted classroom windows, covered lights and an outfit made from material used to clothe astronauts. A Maryland woman, through a nonprofit foundation that helps children with genetic disorders, is sewing Kasey a pair of full-body suits to protect her from light and let her go outside for brief periods.
There are other challenges. While walking, she still needs her hand held to keep from falling. As she grows, her scars will require more operations, which in turn will produce scars. This summer, her right leg -- almost 2 inches shorter than her left from scarring -- will be lengthened.
Much remains uncertain. Without a transplant, she could live at least to middle age. Or the stress on her spleen, liver and other organs could shorten her years.
All the Knauffs can say for sure is this: They're thankful for all the help so far and for the joy their little survivor brings.
Says Kurt Knauff: "She bounces right back. She's the greatest."
http://www.charlotte.com/mld/charlotte/living/health/11881164.htm