Rare condition won't stop lively youngster

JOSEPHINE--A vivacious smile is the first thing you'll notice when you visit the home of Michael and Cheryl Ickes, and it's the last thing you'll remember when you leave.

The grin belongs to the Ickes' 5-year-old daughter Amiee, a charming youngster with long brunette locks.

'Hi...,' greeted Amiee innocently as she eagerly peeked with curious brown eyes from behind a child-size recliner.

However, a grin that radiates her courageous spirit isn't the only thing special about the child who will be a kindergartner at Blairsville Elementary in the fall.

Amiee is one of 900 people in the world who suffer from a rare, congenital vascular disease called Klippel-Trenaunay Weber Syndrome, or KT.

Golfer Casey Martin, who also suffers from KT, recently shed light on the disease when the U.S. Supreme Court ruled that the Professional Golf Association must allow him to to ride a golf cart during tournaments.

His right leg is badly disfigured from the medical condition which affects his blood circulation.

'KT manifests itself differently in different people,' said Judy Vessey, a Minnesota woman who serves as director of the KT Support Group to which Cheryl Ickes belongs. 'You could line people up who have KT, and they may all experience different kinds of things. Some might be dramatically disfigured, some may not. Others may experience internal problems or spinal problems. It depends on where KT strikes.'

Cheryl, who has extensively researched KT since her daughter's diagnosis three years ago, revealed that its symptoms generally include a Port Wine Stain, malformed veins and enlargement of a limb or other part of the body.

'Amiee has all three of those symptoms,' said Cheryl, adding that doctors 'do know that you have to be born with KT. You can't catch it.'

Experts have yet to shed light on the exact cause of KT.

'There is no cure and there will be no cure in Amiee's lifetime,' continued Cheryl. 'KT is thought to be genetic. Not in the hereditary sense, but rather where one single gene is mutated early in pregnancy.

'It's almost like if you run off 999 copies, and every one is perfect, but there's that one copy that has a letter missing.'

RESTRICTED BEHAVIOR

Amiee's diagnosis didn't come as a shock to Michael and Cheryl Ickes.

'Amiee was born with a Port Wine Stain,' said Cheryl of her daughter's red birthmarks which extend from her waist down. 'Up until she was two we thought that it was only a birthmark.'

It was during a routine visit to Amiee's dermatologist at Children's Hospital in Pittsburgh that Cheryl and Michael were told that Amiee had been diagnosed with KT.

Like most mothers attune to their children, Cheryl suspected for some time that Amiee suffered from more than just a birthmark.

'I noticed she had bulging veins and saw that one leg was fatter than the other,' said Cheryl.

She also noticed that Amiee would frequently walk on her toes.

However these differences don't stop young Amiee from playing with one of her favorite toys, a stuffed bear given to her by the Blairsville Elks.

'I like to play,' yelled Amiee as she happily tossed the plush toy into the air.

And like any child, Amiee plays a lot. She just has to be a little more careful.

'Amiee is a very outgoing vivacious, normal little girl,' but her activities will have to be restricted to an extent, said Cheryl.

Those who suffer from KT are at high risk for infection. Amiee is also prone to sudden falls.

Cheryl explained she and Amiee's doctors believe the falling is due Amiee's leg length discrepancy along with the swelling and heaviness affecting them.

'We do not put playgrounds on our agenda at all,' explained Cheryl. 'Playgrounds are considered dangerous for Amiee because with KT, you have bleeding problems.'

Bleeding, said Vessey, is believed to be caused by excess veins at the surface of the skin.

Cheryl said that any scrapes, bruises or bites can lead to serious infections for those with KT. Also, because of circulatory problems and leg length discrepancy, Amiee must do most of her play activity on her stomach.

'Amiee only recently learned to pedal a bicycle because her legs weren't strong enough before,' said Cheryl. 'She is allowed to jump if she wants to but she has to understand that she may have a lot of pain later.'

PAIN STILL A MYSTERY

'Things have always been different for Amiee,' said Cheryl. 'For example, when a baby gets a bellyache, they'll pull their legs up against their chest and scream. Well Amiee still did that at 2 and she will still do that (because of the pain).'

Cheryl explained that doctors still aren't sure exactly where the pain comes from.

'And Amiee is obviously too young yet to tell us what she is feeling,' said Cheryl.

'Lack of good circulation in the veins can cause swelling and may make the limbs feel very heavy and tight,' said Vessey, whose 20-year-old daughter has KT. 'Veins can be stagnant, and sometimes the blood is working very hard in certain areas to go where it needs to go.'

'We have refused so far to put Amiee on narcotics,' said Cheryl. 'Tylenol is a friend to her.'

Amiee's bed needs to be elevated. She is also required to wear special compression garments which are worn like pantyhose. Made from thicker material and much tighter, the compression garments support Amiee's affected limbs.

'I don't like them, they hurt me,' said Amiee, pointing to the suspenders which hold the garments up.

Like any parent of a young child, Cheryl said that you never know what to expect when you have a child with KT.

'Every day is a challenge,' said Cheryl. 'We have good days and we have bad days. We have days when nobody would ever know that Amiee has a medical condition. And then some days she'll scream and be unable to walk and she'll need to be in a wheelchair.'

Cheryl said that warm baths help alleviate Amiee's pain.

'Many times Michael has stayed up all night massaging her legs,' said Cheryl.

Aside from a pediatrician and a gynecologist, Amiee deals with eight specialists at Children's Hospital in Pittsburgh.

'The pain is something that Amiee will have to deal with the rest of her life,' said Cheryl. 'It's sad that somebody has to get used to something like that, so we try to take her mind off of the pain.'

Sometimes Cheryl and Michael will sing selections from Mary-Kate and Ashley Olsen's Greatest Hits, Amiee's favorite video, featuring the twin sisters who found fame from their roles on the hit television series Full House, or they'll talk to her about her favorite Barbies or Disney characters.

'We try to talk her through it,' said Cheryl. 'Sometimes it works, sometimes it doesn't.'

WHAT WILL PEOPLE THINK?

Amiee's condition does attract attention.

'People have a natural curiosity when they see something different so I don't mind at all if people come up and ask, 'What's wrong with your daughter?' But when people stare or giggle... I just don't tolerate that,' said Cheryl, adding that people have gone so far as accuse her and her husband of child abuse.

'They see the red marks on her legs and just assume,' said Cheryl.

'When people say things and make comments, I'm careful what I say because how I react is how Amiee is going to learn to react,' said Cheryl. 'I tell her that people stare at Mickey Mouse and Minnie Mouse because they are special so you must be special, too.'

Others' curiosity and how they express it worries Cheryl about the upcoming school year.

Amiee has primarily been around her parents and brothers, Michael, 26, and Matthew, 20. The family is close and has provided Amiee with all the support she needs.

'But I do worry...So far, Amiee calls her birthmarks her 'rainbows' and she's very proud of them,' said Cheryl. 'But at some point, that pride will change and maybe she won't be as proud of them anymore.'

Cheryl is hoping that parents will talk with their children and tell them that despite KT, Amiee is just like any child.

'I fear the people in the world that laugh or stare will strip away her pride in herself,' said Cheryl. 'She doesn't see herself as being any different than anyone else, so why should they?'

Cheryl contributes people's reactions to lack of education and understanding about KT.

'I really wish that more doctors were educated on KT,' said Cheryl. 'There's probably a lot more cases of KT out there that aren't being diagnosed or are wrongly diagnosed because people and doctors aren't educated enough about it yet.

'If they were, it would lead to earlier diagnosis and better treatment.'

According to Vessey, Boston Children's Hospital is currently conducting a study on what causes veins and arteries to grow.

'It might yield some something, but KT is such a multi-faceted problem that it's not like we're not going to have just a shot someday to cure it,' said Vessey. 'It will be more complicated than that.'

SCHOOL DAZE

Registering Amiee for kindergarten was a frustrating experience for Cheryl.

'They were very gracious to test Amiee for learning disabilities,' said Cheryl. 'I just wish the school would learn that just because someone has a physical disability, it doesn't mean that they have learning problems, too.'

'There's a whole gamut of people in our support group,' said Vessey. 'We even have a subset of the group that includes doctors with KT.'

Besides Martin, just one of the many KT sufferers proving that theory wrong is Vessey's daughter, Rachel, a history/literature major at Harvard University.

And Amiee herself is another bright example. Active and creative, she loves to draw, color and enjoys learning her A-B-Cs.

'She really likes being on the computer also,' said Cheryl. 'She has to elevate her legs a lot so we spend a lot of time watching movies, too.'

Cheryl explained that Amiee will probably need an aide to help her through each school day.

'Individualized Educational Program (IEP) is geared for children with learning, not physical, disabilities and they have to follow a contract,' said Cheryl. 'I want something like that for Amiee, too.'

Cheryl said that Amiee will need to elevate her legs during class to prevent blood clotting. She'll also need a special seat pad.

'She will only be allowed to sit for 10 minutes at a time, and she'll need to move around a lot since her medical condition puts her at risk for blood clotting,' said Cheryl. 'Amiee will also need assistance in walking up the stairs because she falls constantly.'

Although the school has an elevator, Cheryl doesn't want Amiee to feel that different from her classmates.

'Besides, walking up the steps will be good for her circulation.'

Since blood is present in Amiee's urine, an aide will need to monitor her to make sure she isn't hemorrhaging.

'And although she can't take part in any high impact exercises, I want Amiee to be able to take part in gym class with the rest of the children,' said Cheryl. 'There are a lot of things in kindergarten gym class that she could do.'

DARING TO DREAM

The Ickes' were ecstatic over the recent U.S. Supreme Court decision in favor of Martin.

'It will teach even other people with different syndromes that dreams can happen,' said Cheryl. 'Casey Martin can use his cart, and he can continue living his dream. Amiee even has her own set of golf clubs. She calls them her Casey Martin golf clubs.'

'Casey Martin,' smiled Amiee as she pointed to her special set of Fisher Price golf clubs. 'This is my putter, my chipper...this is a wood.'

'I try to teach Amiee that she can do anything she sets her mind to do,' said Cheryl. 'I teach her that dreams are for everybody, not just the able bodied.'

Right now, Amiee's dream is to visit Disney.

She also loves Snow White and Cinderella and her computer has a Lady and the Tramp screen saver.

'Unfortunately our budget won't allow a trip to Disney right now,' said Cheryl explaining that Amiee's compression garments cost $230 apiece for two pairs every three months.

'We are fortunate that our insurance does pay for one pair.'

NETWORK OF FRIENDS

Cheryl said that her family has been her greatest support system.

She often unloads on her mother, Helen.

'I also have a great support group over the e-mail,' said Cheryl. 'I can get on the computer and scream and holler as much as I want. There are plenty of times I fall apart, but I never let Amiee know that.'

Cheryl said that the KT Support Group has also provided an outlet for her to vent her frustrations.

'Since it's such a rare syndrome, the group's sole purpose is to offer a network of people to provide support and awareness to KT,' said Vessey, who founded the group 15 years ago. 'We keep on top of the medical advances and treatment centers that know something about KT.'

The support allows Cheryl to view the family's situation as a challenge.

'I've spent that last three years since Amiee's diagnosis leaning all I can about KT,' said Cheryl. 'I haven't had time to ask, 'Why me?' If I would've had time to ask that question, then I would ask God, 'Why did you think that I deserved such a wonderful gift?'

'Amiee has truly been a gift.'

WHAT DOES FUTURE HOLD?

Since little is known about KT, Cheryl said that it's difficult to predict what the future will hold for Amiee.

'A lot of people with KT end up with amputations because of the high risk of infection and swelling,' said Cheryl.

'Amiee has had cellulitis,' said Cheryl. 'We've been lucky that she hasn't had any blood clots. She does get surface blood clots but they are harmless. They break up and absorb into the tissue.

'I just want people to know that Amiee is the same as every other kid,' said Cheryl. 'The only difference is that she has purple legs, or 'rainbow' legs.

'And she is the most experienced five-year-old drama queen,' laughed Cheryl. 'She's so dramatic, she reminds me of Susan Lucci. She's a miniature Erica Kane.'

'We'll go to Wal-Mart and Amiee talks to everybody,' said Cheryl. 'She'll go up and ask the clerk for anything by the Olsen twins, Mary-Kate and Ashley. She'll point to them and say, 'They're my best friends.'

'She's absolutely sure that they'll show up on our doorstep to visit one of these days!'