KRISTIANS STORY
On the 30th 0f September 2002, Kristian Matthew was born. Kris was born with joint contractures and very little body fat.The doctor at the hospital advised us to make an appointment with a geneticist. When Kristian was four weeks old he had a great appetite but wasn't gainning weight and started throwing up a lot. We brought Kris to the geneticist when he was six weeks old. The doctor noticed that Kristians' skin on his abdomen was very shiny and tight. She called in a dermatologist and they did a skin biopsy.
Kris was admitted to the hospital because of his low weight and not being able to hold his foods down.They put an ng-tube in to feed him very slowly. After Kris spent a week in the hospital we got the results of the biopsy. The doctors told us Kris had restrictive dermopathy. We thought they could do something to help this condition but they couldn't. This was a very rare syndrome and they thought our beautiful little baby might live for another three or four weeks. We were devastated and very sad to think we wouldn't have much time to share with our newborn son.
We read and searched for information on restrictive dermopathy everywhere but couldn't find any treatments or much information to help him. The doctors told us to take him home and make him as happy and comfortable as possible.
At home we continued Kris' feedings 24 hrs a day and he gained a little weight. Kris continued to improve slowly. At that time we were involved with an early intervention program and we had a physical and occupational therapist, they both gave us hope for Kristian.
We enjoyed every day that we had. Kris began eating regular food along with his tube feedings. He started playing, learning and talking but because of his contractures his mobility was limited.
When Kristian was about eight months old, the doctors decided to sent off a blood sample and they were checking to see if Kris had progeria,an aging disease. Three months later we were told he did have progeria. We learned everything we could about progeria. At least the children with progeria usually survived into their early teens and this is better than what the original diagnosis was but we couldn't believe this was happening to our son. Kristian was a very happy and determined little boy. His physical and occupational therapies were helping with strength and development.
When Kris was one he went into the hospital for an operation to place a feeding tube directly into his stomach. On the morning of the operation we noticed our little boy getting very sleepy. At first we just figured it was from getting up at 5:00 a.m. When we arrived at the hospital the doctors delayed Kris' surgery because of an emergency surgery on another patient. Kristian's surgery was scheduled for 9:00a.m. but now it was 11:30a.m. We were finally called down to the prep waiting area at 12:00p.m. As soon as we got there I told the nurse that Kris wasn't normally this sleepy and he started sweating. I told her it was the first time he fasted and maybe his blood sugar was low. She came over a few minutes later and checked his blood sugar. As soon as the machine beeped and registered his blood sugar she ran. About 10 seconds later the anesthesiologist ran and put in an iv. He gave Kris a concentrated dose of glucose (his was 26 and normal was about 85-110). He began to wake up just as the surgeon came in and the doctor said this baby is to sick for surgery!
After talking to some of his other doctors we realized Kristian had no fat and therefore no reserves. The surgery was rescheduled for three days later. We gave Kristian his formula until 2:00a.m. then clear liquids (sugar water) until 5:00 a.m. and his surgery started at 9:00 a.m. His blood sugar was fine this time but I asked the nurse to check it after the surgery and now I knew that if Kris was going to survive we needed to insist that he be treated very delicately as he had no reserves.
After the surgery another problem arose. Kris was very swollen and red (we barely recognized him). We were told that was normal for children to be swollen and red from the fluids and the anesthesia. Later when Kristian went in for a cardiac mri and he needed general anesthesia we found out the swelling was because Kris' heart couldn't handle the "normal" amount of iv fluid they gave him. He ended up in the icu for three nights with pulmonary edema (fluid in his lungs).After many doses of lasix he was off the oxygen and we went home! Kris was almost 3 when he went through that ordeal.
Now without the feeding tube in his nose Kris did a lot more talking. His legs were getting stronger everyday. Kris loved running in his walker, chasing after the cats.While his physical therapist was working with Kris he stood up and walked over to our coffee table (about 4 steps!!!) He was one and a half and weighed ten pounds and was now chasing the cats on foot. Kristian liked to eat a lot of different foods including waffles, cereal, eggs, cheese,chocolate, ice cream, tunafish sandwiches, spaghetti & meatballs, and M&M'S. He was still having trouble holding his food down. The doctor prescribed Zantac and raglan but it didn't help.
Kristian was just having a lot of fun now, so independent, telling us what he wanted and being able to walk and play with his toys. Kris was also taking growth hormone which helped give him the energy he needed to be like other twenty month olds. We noticed a lot of muscle growth after two months of taking growth hormone. At two, Kris was strong enough to start attending a play group through early intervention. All that Kristian accomplished physically and weighing just twelve pounds was just amazing! Kris also began HIPPO therapy which is riding a horse with a physical therapist walking alongside the horse and guiding the patient through some exercises while riding. This therapy also helped to build Kris' self esteem. He loved telling his friends and family that he rides a horse named "Rocky".
A couple of months before Kris turned three, he started on prevacid slow release tabs, one in the a.m. and two doses of the Zantac in the p.m. to see if it would help stop the throwing up. Six days after starting them he stopped completely. After two months of being able to hold his food down Kristian gained one pound, and normally he gained one ounce in two or three months. It was great, he could eat, (which he loved to do) and hold it all down.
Kris really enjoyed the playgroup he attended through early intervention. He eventually felt comfortable playing there without mom by his side. His favorite thing was snack-time, especially when it was someone's birthday and they would get cupcakes with lots of frosting! I can't say enough about the early intervention program, and all of the wonderful therapists, that helped not only Kris for his first three years but our family as well.
In October 2005, Kris started preschool! He was very excited to ride the bus and kept asking "a yellow bus?". We were happy that first day of school to see a "yellow bus" heading towards our house. At school Kris had an aide, "Miss Laurie" who would be there for him if he needed help. As Kristians' parents we were very excited and nervous about sending him off to preschool at just three years old. Kris could walk very well but moving around a classroom safely, with twelve other young children, wasn't going to be easy. The teacher prepared the other children before Kris started school. Kris' classmates learned quickly that Kris was a lot smaller than themselves, but he had a large personality. Everyone at preschool helped make Kristian feel very welcome and just like one of the kids. This new experience was so important for Kristian to be as independent as possible. After his first couple of weeks at preschool, Kris was feeling very comfortable and opening up.
At home, Kris loved to play hide-and-seek. He had a favorite hiding spot in the large antique food cupboard. Even though we could see his skinny legs sticking out of the cabinet door we would call out to each other, "where's Kris?", "have you seen Kristian?". He would laugh and laugh, and finally push the door open and say, "here I am!". Kris loved to run around after his nephews and niece, who were all just months older than himself. He would get so excited and enjoyed playing with them. Although Kris was shy around most people, at home he was very feisty and playful.
Our family was planning a trip to the National Institute of Health on March 26, 2006 for Kris to undergo tests for one week. This study was the start to a possible treatment for children with progeria. The Progeria Research Foundation (www.progeriaresearch.org) has helped us and our doctors to care for Kristian, providing information on physical therapy and nutrition. We have enjoyed attending the road race/ walk that the Progeria Research Foundation organizes yearly in Peabody Ma. We are so grateful to help in some way to find a treatment or cure, for these very special kids.
In November of 2005 Kris' feeding tube was falling apart and there was a painful granuloma around the g-tube. We had been taping up the small leaks for months. His doctors were nervous about putting Kristian through an operation to replace the g-tube. This was the original tube, which for two years worked well for Kris. On Dec. 21, 2005 Kris had the operation and now had the "button" feeding tube. Because Kris' skin on his tummy was very tight, he needed another surgery on Jan.28, 2006 to repair a prolapsed mucosa (the lining of the stomach). By the beginning of March, the mucosa prolapsed again, and Kris had emergency surgery. The surgeon told us he had to remove the g-tube due to necrotic(dead) tissue on Kris' stomach. He told us he was worried that Kris might not recover from such a huge surgery. Kris' stitches might not hold or he could get a very bad infection. We prayed a lot and by the fifth day following surgery we were feeling hopeful for Kristians' recovery. The doctors removed the ng-tube,(a tube going to his stomach through his nose to keep stomach juices off the stitches), on the forth day. Early on the sixth day the pic line, feeding Kris nutrition through a vein during recovery, was clogged and stopped working. Shortly after that Kris started a fever, even though he was on Tylenol. His oxygen saturation levels were going down and they told us he needed to be intubated in order to get enough oxygen. We just could not believe all that was happening to Kristian. We thought he was recovering well. When they tried to intubate Kris his stomach was very full of dried blood and stomach juices but they didn't know this. He threw it all up and most went into his lungs. We found out after that only one percent of people who aspirate this amount into their lungs survive. Kris died on March 23rd, 2006.
Kristian will forever be in our hearts and our daily thoughts. Kris was curious, playful, loving, independent , thoughtful,and his determination was unmatched for such a young boy. He was a joy to be around. We feel priviledged to have been the parents of such a special person. He is sadly missed by his family and friends.