Gifts to Spencer’s Fund are tax deductible and can be sent to:

Spencer Jarrett Bendien Fund Children's Hospital Foundation
PO Box 16810
Columbus, OH 43272-5117

Spencer recently passed from this life, or as one of his friends wrote, Spencer has ‘graduated to a higher life.’ Though excelling in every way, at the age of 21.5 months, Spencer was tragically taken from us on January 26, 2005.

Spencer was one of the youngest Jeune’s patients to successfully undergo LTE when the left side of his rib cage was expanded at the age of 5.5 months. He spent just 10 days in Columbus and returned to the Virginia PICU where he remained for an additional 6 months, largely due to the unavailability of home nursing. To the joy of his parents, Spencer was able to come home in March of 2004 with his portable ventilator. Through out the spring and summer that year, Spencer enjoyed playing at home, reading books with his Mom and Dad, playing in his ExerSaucer, watching the Wiggles on his TV, splashing in his kiddie pool on the back porch on a warm summer afternoon, going for stroller rides outside, or any number of other fun activities. Spencer left home in August 2004 to have LTE on his right side. His pre-surgery testing showed a marked increase in his lung capacity, which made us all the more excited to see his second side expanded. He sailed through surgery with flying colors and was returned to the Virginia PICU for a mere 24-hour stay before coming home again.

Through out his entire life, Spencer would greet everyone with his trademark brilliant smile and twinkling eyes, and just seemed to radiate joy and happiness. His jubilant personality was intoxicating and you couldn’t help but feel your heart soar anytime you were around him. Our hearts, and those of everyone he touched, feel great sorrow at his departure. But, we take comfort in knowing that he is now with God and the loved ones that have gone before us. And, without a doubt in our minds, you can bet that he’s now happily running around with that big smile on his face, laughing, singing and continuing to radiate love and joy.

Kristin & Tom Bendien
bendien_family@yahoo.com 

On August 1, 2002, Dominique had her first chest expansion on her left side. It was absolutely amazing what they had done in a matter of 2 1/2 hours of surgery. Dr. Davis and his team provided us with x-rays and images of Dominique's lungs and chest wall prior to the surgery. After the surgery, through x-ray, we were able to see the expansion that had been done. Dominique responded wonderfully after surgery. I believe she was more upset about breaking her third tooth than having her ribs broken and fused together!

Since the surgery, she has been at RWJ and a rehab facility in New Jersey and has thrived immensely. Her ventilator settings are at the minimum. She is on room air and is happy as can be. She can now sit up by herself for hours at a time, playing with all her toys and interacting with people. She can point objects in her books when asked, still continues with her clapping, waving hello and goodbye and snapping her fingers. She loves to play peek-a-boo and share her toys with you. It is amazing how far she has come with her therapy in such a short period of time. They have built her a special wheelchair and other devices to build up her upper body. She is very smart. As soon as you show her something, she immediately picks it up. She is mouthing words even though she can make no sound. They are also teaching her to sign so she can communicate with us.

Granted, we have our good days and bad days, but the good days are outweighing the bad. Dominique is growing by leaps and bounds and has surpassed every doctor's expectation. We are anxiously awaiting her second chest expansion surgery sometime in 2003 with the hopes that soon after she will be off the ventilator and will be teaching her new little sister all her tricks.

I cannot express the gratitude we have for Dr. Davis and all who participated in Dominique's surgery.

Hope everyone is well and keep the prayers going for Dom.

Smiles,
Kristina, Joe, Dominique & Vanessa

Contact information:
Kristina and Joseph
732-316-9556
kraba@att.com

Birthday: 1/23/89
Favorite Color: Red
Favorite Food: Hot Dogs
Pets: A dog named Rex
Siblings: Three Brothers

Josh is now 14 and doing fairly well. This is the third year for him to be schooled at home from a program called ECOT out of Columbus. It is online. He has not had any illness in this time. Knock on wood!!!! He does experience what CHMC-Cincinnati calls fractures of the ribs of "shifting." Some are mild and some can be severe. They generally have very minimal warning prior to occurring. So, of course, this can make pain management very important. The only doctor he sees now is pain clinic.

Please feel free to email me if you would like to at parentforchildren@hotmail.com.

Birthday: 2/07/00
Siblings: 1 biological brother
who shows no signs of Jeune's

Katy weighed 8 lbs. 6 oz. when she was born 3 years ago. Prior to her birth, an ultrasound had shown that her extremities were short. At birth, she demonstrated signs of respiratory distress and was intubated and placed on a ventilator. She was diagnosed with Jeune's Syndrome. She was in the NICU for one month and then moved to the PICU. She was taken to surgery for a trach and a G-tube.

She had many "blue spells" while in the PICU. She received aerosols, chest vibrations, suctioning and g-tube feedings around the clock. She also received OT, PT and Speech therapy services. Katy was less than a year old when she made her first trip by ambulance to Columbus for surgery with Dr. Davis.

Titanium rods were placed in one side of her chest to allow for better lung growth and expansion. She returned to Toledo Children's and remained in the PICU while she grew and became stronger for a second surgery, which was performed in Columbus when she was about 16 months old. At that time, rods were placed in the other side of her chest.

One month after her second surgery, her brother was born without signs of Jeune's. A month later, we became involved with getting to know Katy and learning how to care for her. After securing home nursing help, since she required care 24/7, we took her home as a foster child at 20 ½ months of age. She was walking with a reverse K walker and some added assistance. She was on CPAP and oxygen around the clock. She was on continuous g-tube feeding 20 hours/day. She was weaned off CPAP, oxygen and g-tube feedings while awake. She reluctantly began to take some soft foods by mouth. During her first winter home, she had a couple of respiratory infections, which were treated successfully with oral antibiotics. She continued to receive speech, OT and PT at home. She began walking without the walker or assistance from us. She was weaned off the CPAP and oxygen at night. Her trach was removed a few months later, only to be reinserted after three weeks of obstructive sleep apnea and oxygen desats. She had her tonsils taken out. It was nearly a year before she was hospitalized for an infection. In late October 2002, Katy moved to an adoptive home. A couple of weeks later she had another infection and has since been placed on the ventilator while asleep and is on oxygen continuously. I've been told that Dr. Davis is hopeful that the ventilator and oxygen will be discontinued this spring. If she has a healthy winter next year, perhaps another attempt will be made to get the trach out.

Besides all the work that is involved in taking care of a child like Katy, the hardest part for us was the "not knowing." How successful have the surgeries been? When will she be off the vent? When will the trach be out? Why is she back on the vent after successfully being off for over four months? What does the future hold for her? These are questions no one can completely answer at this time. So, you live your life one day at a time and help your child to live each day to its fullest! And you pray that God continues to bless your child with each new day!

Contact Information:
Debbie
sk8rmom57@aol.com
419-874-0824

Birthday: 12/27/97
Favorite Color: Blue & Red
Favorite Food: Mac & Cheese
Pets: Dog -"Sydney" & 9 saltwater fish
Siblings: Evan - 7 & Josh - 2
Favorite Book: Power Rangers
Favorite Sport: Soccer/Hockey

My son Matt was approximately 2 months old when he was diagnosed with Jeune's. Prior to actually knowing what was wrong, we spent the first couple weeks in and out of the ICU unit at Children's Hospital in Columbus, Ohio. The concerns during that time were mainly respiratory related issues. Our family had gone through some very hard times not really knowing from day to day what the outcome was going to be. With one sibling at home, we did not have the normal "new baby" bonding period.

Fortunately, we live near Columbus, and it was comforting to have the "experience" of the hospital and its staff to consult with. Matt was followed very closely by a small handful of doctors and nurses. He has been, and will continue to be, followed with visits to measure his progress.

We were blessed with Matt having a mild case of Jeune's and thus far he has not needed any surgeries. I really cannot say enough about Children's Hospital and its staff. There are a number of doctors there who seem to have a lot of knowledge and experience, what little there is, with Jeune's. I know they are trying diligently to collect data of children around the country so we are all able to learn more about this disease.

Emotionally, Matt does very well for a five year old. My wife and I are, however, concerned about this once he starts school. So we feel we must keep his life just as structured and fulfilled as our other children. Therefore, we try not to make excuses for him or treat him any differently.

I have included my contact information, so feel free to let me know if I can be of any help.

Contact Information:
Steve and Trina
(740)548-0832
steveg@hotdipgalv.com

Birthday: 3/26/90
Favorite Color: Red
Favorite Food: Chicken/Shrimp
Pets: A dog, a cat, 5 finches, 4 turtles, a beta fish, a rabbit,
and a pacman frog
Siblings: Katie, age 11

Michael was diagnosed with Jeune's Syndrome two weeks after he was born. One doctor told us he would not live out the weekend, then he would not live out the month, then to take him home and enjoy him, but he would not live out the year. In March, Michael turned 13. We can only and continually thank his pediatric pulmonologists for never giving credence to that prediction! Michael was never on a vent except for 2 weeks, I believe, when he had his g-tube placed and a hernia repaired and had trouble coming off after the surgery. He was on oxygen 24 hours a day until he was 2.5-3 years old. He was in the hospital continually (it seemed) those first years with recurrent bronchitis and pneumonia.

He had his g-tube until he was around 7, I believe. Michael had the right side of his rib cage expanded when he was 5 years old by Dr. Davis and has not had the other side done as of yet.

We noticed a big difference after the surgery in his need for oxygen and his stamina. I believe he was 6 or so when he was diagnosed with having the liver problems that can sometimes come with Jeune's, however it has not caused any problems and is not symptomatic.

When he was 8, he had his last severe case of pneumonia. A hematologist was brought in when his blood counts could not be brought under control. She is fantastic and pulled all his blood work from birth, and we discovered he has had chronically low white count since birth...this, of course, contributed to all the colds, bronchitis, and pneumonia. After that time, as soon as he would get sick, we would give him shots (GCS-F) to up his white count and he became much healthier. He still has a very low white count, a condition called Severe Chronic Neutropenia and we are struggling with that right now. He was on shots just "as needed," but we have recently had to change that to continually every other day.

When he was 2.5 years old, his sister Katie was born and does not have Jeune's. He is a wonderful older brother, and Katie is a little mother with him. In many ways they are more like twins. Katie has her own struggles being the healthy sister of Michael, but they have both learned a lot about compassion and are friends as much as siblings.

Michael is an incredible boy and we love him and are so proud of him. He was able to go to New York City through Make-A-Wish last year. His wish was to visit the Pokemon Center in NYC, and to visit the dinosaurs' skeletons at the museum. We had an incredible trip and he kept saying over and over that there were some good things about having Jeune's...one, he got to make a wish for anything he wanted, and two, he is closer to the ground when he gets older and is hunting for dinosaur bones! Michael has a great love of animals and bugs and a general fascination for the world around him.

The last 13 years have been long, and filled with joys and frustrations, setbacks and triumphs. There is no one who has had to deal with Jeune's, or any other similar dystrophy, who has not at one time or another felt great fears and anxiety about what the future holds. We have learned that the old cliché "one day at a time" is the only way to live. Looking forward too far becomes overwhelming, but each day, each decision, each step is manageable when taken one at a time.

Contact Information:
Kelly & Mike Bowron
bkellyaf@yahoo.com

Favorite food: chicken/carrots
Favorite color: green
Favorite pet: small puppies
Siblings: Ray & Sarah

Taylor is doing well. Thank God! She has gained over 20 lbs. and now weighs 38 lbs. She looks good. She's been completely weaned off oxygen for close to 9 months and is off the vent from 7am-9pm and weaned from 1am- 3am. We're looking to remove the trache for Spring. She maintains her sats at 94-95 and sometimes requires very little oxygen when weaning.

She's able to eat almost anything orally and feeds and dresses herself. She's potty trained and extremely bright. She's now able to walk up and down the stairs but gets tired easily. I am able to take her outdoors tube and oxygen free. 

She's getting stronger everyday thanks to her wonderful occupational therapy and is also able to fight colds and virus on her own without being hospitalized. We had a rough winter, but she pulled together.

She knows when she needs care. She tells me, "Mommy I need suction" or "I need the vent" or "I am dry I need a treatment." She tells me she wants to be a doctor; you won't believe how bright she is. She gives her own treatment, on the nebulizer under supervision, she checks her sats, and she turns the vent on and connects herself. I have to keep reminding her she's not the doctor! One day she asked, "Can I suction myself?" I said, "Now you're pushing it." LOL. She tells the nurses, "You can't leave. You didn't change the vent's water." Taylor is very sweet. I have nurses from out-of-state coming over to visit. We love her dearly.

Our most difficult time was having Taylor transferred from Momomides in N.Y. to Ohio. It was just devastating not knowing if she was going to make it. Just being in another state alone, I didn't know anyone and missed my kids. When my husband had to go back to N.Y. to take care of the other kids, I had to stay in Ohio for almost 4 weeks. Waiting in the recovery room when she went into surgery was just devastating. Staying by her bedside through her recovery was very heartbreaking. I cried every single day. But thank the Lord; he gave me the strength to carry on.

We had to prepare her siblings, and I was just so concerned how they would handle Taylor's trache and G-tube. I remember coming home to N.Y. for one day to explain to the others about the surgery. I came home with a trache. Calmly, I explained this was all temporary. The trache would help her breath better until she gets stronger. The G-tube was just for gaining weight. I even explained about the PediaSure and how it had all the vitamins and supplements she needed to gain weight. Now it's just for night feeding. My children were so understanding. Sad, but very supportive. Today I am amazed!!! Ray, who is 18 years old, does the trache change. I am still fragile in that area. He monitors all her equipment and operates the vent and oxygen when needed. Sarah is 12 years old and she suctions, gives medication through the G-tube and does trache-G tube care. My children have developed nursing skills.

I remember when Taylor came out of surgery. The first time I saw a trache and G-tube, I almost fainted. I couldn't stop crying. Now I am unsure about removing the trache. I feel so safe with it. Her doctor said it's coming out whether I like or not. The G-tube I am not removing, she's gaining weight nicely. I supplement only at night. I am really astonished at everything I have accomplished dealing with Jeune's Syndrome. In the beginning, nurses couldn't even get me to suction, and now Taylor says, "Mommy, you be my nurse tonight." My friends and family cannot believe how I managed the pain and her care.

I would like to tell other families with Jeune's to not give up and don't lose faith or hope. What got me through this ordeal were prayers. I pray, pray and pray. The Lord has performed a miracle on Taylor. The doctors told me Taylor didn't have a chance. Taylor is my miracle baby, and I thank God everyday. I also thank the Lord for Dr. Davis and his wonderful team. "THANK YOU!!!! for saving my baby's life." Thanks to the ICU at Children's Hospital in Ohio for the loving and care they gave Taylor. I would also like to take this opportunity to thank her N.Y. pulmonologist, Dr. M. Marcus, for his interest and the care he has given Taylor. I would also like to thank the Staten Island Hospital and Children's Hospital in Mountainside N.J.

Contact Information:
Judy & Irving
BBGJr1035@aol.com