Saturday, September 30, 2006

Got A Little Bug

Well Chan had it and then Moni had it, then Chan got it again... and now our little bugs got it. Just a cold, so far nothing to big and bad, you can just tell he feels a little crummy. So were holding off on our 2 hour reprieve until Mr. Man is over his cold. Moni and Chan had it for 2 days, so hopefully this will be a quick one.

Wednesday, September 27, 2006

Labs Are In

Well I just got Skylars labs back and man did he do GREAT!!!! His bi-carb is 19, normal is (18 to 27) His blood oxygen level is 94 and his saturation level is 97. In all it just means Skylar is doing incredible!!!! We have room to make adjustments, he is now at 14 on his V-pap and starting Saturday will be getting 2 hours off. And his doctor said if he does really good with the time off all I have to is give him a call and he will up Skylars time off and write a new order. So the next few weeks and months are going to be full of change, but change is good in this case. I cant wait to see how it all goes and how my love bug does.

Monday, September 25, 2006


Id like a pepperoni with extra cheese and sausage please.... What??? You need to speak to my mommy.... No no I want a pizza...

GOOD Blood Gas

Well today was absolutely GREAT!!!! Skylars blood work is excellent in almost every way other then his low Co2's. He is still at 34 and normal is 40, so I talked to our pulmonary doc today and he agrees.... We are going to lower his settings to 14 over 5 and we are increasing his time off up to 2 hours a day. For the first few days or week its up to how he does with it all, but if it is anything like the past few weeks, he is going to do great!!!! So other then that we are all very very happy and excited.

Special Time With My Daddy

WOW I never noticed how much Skylar looks like his papa, until I took this picture last night!

There they sit like old times, snuggled in dads chair watching football.

Today is a big day, were off to Everett in a little while to have Skylars CBG drawn, this will tell us what his venues blood gas is.... I know its going to be great, he is doing so awesome!!!! But we wont know what the results are until Wed-Thursday.

Monday, September 18, 2006

Ahh Some Sissy Love and Some Peek A Boo

Can you believe this is the first time in 6 months that Monica got to hold and love on her little brother, WOW thats a long time!!! It was so cute seeing Skylar peek up at her in amazement and to see Monicas face light up when I put him in her lap. It was a GREAT day!!!!!! Monica was on the move, but Sky was holding on to waht ever posture he could get a hold off. Skylar is trying to get use to not having that darn hose hanging off of his neck, he likes the freedome, but its still a bit to get use to.
Wheres the baby???? Where is my Skylar????
Oh I see you peeker..... I see you......
There you are silly boy!!!!!!

Saturday, September 16, 2006

New Pictures

No tubes no wires, just free!!!!! And loven every second of it.....

Friday, September 15, 2006

Oh A Parents Joy

Ok I didnt wait for Monday to give Skylar his time off the V-pap, I did it today and he did so GOOD! He loved being off and I have to say it was so wonderful to see my baby with no hoses, no wires, just my darling little man. He is so amazing, and I and dad are just so excited about this, we know it will only be a mater of time before he is off the V-pap for most of the day. I was and is a great day! Ill post some pictures of our free Sky on Saturday....

Thursday, September 14, 2006

Back From Clinic With Good News

Well Skylar is doing great! He hasnt gained weight, but he has grown in length. Im not concerned, because his sister was the same way. I mean he weights 23.98 lbs and he is 85 CM long, I think thats just great. He looks good and he is active again. The good news is that we are giving him time off starting on Monday, 30 mins at a time and we will build from that 30 mins more as he tolerates it. (YEAH) Mommy and Daddy are so happy and we are so proud of this little man, he has been through alot. But maybe now its Skys time to shine and show everyone out there I can do this!!!! I almost forgot Skylars endtitles (CO2) were very very good, they were 30 to 34. Normal would be 40 so we now know his little machine is doing way to much, so Saturday Im lowing it again and on Monday we have our first time off.... YEAH :)

Monday, September 11, 2006

Trail Update

Ok I guess because of Skylar being on the V-pap he does not qualify for the trial in a few months. But if we can get him off of the V-pap within a year he will be able to do the trial with hydroxyurea. So I guess we are not out of the game just yet. I just have to keep this little man in pristine health and get him off of the V-pap. I think we can do, but its also going to take the ok from our pulmonary doc. So hope and pray that we can get this done and move on to what is going to improve his life. Thank You Sheila

Stanford Trials

Well I have been in contact with DR Wang from Stanford. Good news and bad, they were approved to do the clinical trial but didnt get the funding in there grant. So that trial is on hold for a while, possibly a year. But they are starting another trial with a drug called phenylbutyrate, it works the same way as the hydroxyurea. In current test they have found that the motor functions increase in strength in 3 to 9 days. So this is all good news. Skylar will be doing the trial with the phenylbutrate in a few months, so Thank God... I know with this my little man is going to do great things.

Thursday, September 07, 2006

Just A Few New Photos

Its been an exciting week for Skylar, we have been swinging our hips and moving our upper and lower legs more this week. The movements may be slight to someone who does not know him, but to us it BIG!!! These maybe little things, but they are incredible things.... Inch stones.....He is such a happy little man. I couldnt ask for a more perfect baby. Ive been in contact with Stanford, Im just waiting for a call back in regards to getting Skylar into this trial. We can only hope and pray, that this is something that soon becomes reality!!!! This week we have managed to lower Skylars V-Pap settings, and he did so wonderful with it. Next week I will lower them again, and if he tolerates it then we will have to see what his doctors want to do, they want him at 16 but I think he can go lower, he is pretty tuff..... This week we have a check up at Childrens with pulmonary, so hope and pray that all goes well. They will be checking his blood gases this time to see how he is doing with the changes, so pray for low numbers, mid 30 to low low 40's. Other then that Skylar is doing very very well, we couldnt be more proud of him!

Wednesday, September 06, 2006

Must READ This One!!!!!

As many of you know Skylar was to do a clinical trial at Stanford, we never did hear from Stanford so we were unable to go. This is an artical I found online, its about a little girl with SMA and its about the clinical trial at Stanford. I cryed when I read it, its so amazing and wonderful to know there is something that helps these little ones. Well it fired me up and tomorrow I am calling Dr Wang at Stanford and Im enrolling Skylar, regardless. I wil lnot take no for an answer. So PLEASE read the artical!!!

Girl with a fatal genetic disease making progress in a clinical trial
By Bonnie Miller Rubin
Chicago Tribune
PONTIAC, Ill. - When Elizabeth Lee Hallam was born on Sept. 29, 2003, she was a textbook example of the perfect baby. But by 8 months of age, she was battling a fatal genetic disease her family had never heard of.
Elizabeth was diagnosed with the most acute form of spinal muscular atrophy, a motor neuron disorder similar to Lou Gehrig's disease that afflicts one in 6,000 babies. With no cure or treatment, the doctor predicted Elizabeth - like 95 percent of all SMA babies - would not live more than two years.
"I screamed when we got the news," said her grandmother, Jeanna Huette, 48. "I could not understand how such a beautiful child could just die. I cried for a few more days ... and then I knew I had to save her."
As Elizabeth approaches her third birthday, she cannot sit, stand, crawl or walk and relies on machines to swallow, get nourishment and even cough. But she is alive and improving.
Her family enrolled Elizabeth in a trial designed to test the safety and effectiveness of the drug hydroxyurea against SMA. It is one of only a few clinical studies on the disease approved by the Food and Drug Administration and is run by Dr. Ching Wang, a scientist affiliated with Stanford University who has made conquering the disease his life's work.
The research is in the initial stages, but if the treatment succeeds, Wang says it could give new hope not just to children afflicted with SMA but those with other genetic illnesses and other motor neuron diseases.
Elizabeth, who has grown into an engaging child with an ever-present smile, is one of Wang's star patients.
"This little girl is defying every rule, every possible odd," said Wang, director of the Neuromuscular Disorders Clinic at Packard Children's Hospital in Palo Alto, Calif. "She's going to turn 3, and she shouldn't even be here."
Dr. Mary Schroth, a pediatric pulmonologist at the University of Wisconsin Children's Hospital who treats SMA patients, including Elizabeth, from all over the Midwest, said the research is promising.
"These are just tremendous gains," said Schroth, who is not involved in the trial.
Clinicians won't know everything until the data are published, Schroth added. "But it's a huge breakthrough for those afflicted with a horrible disease."
"The state of the field is much more encouraging than it has ever been in the past for SMA," said Dr. Kenneth Fischbeck, chief of the neurogenetic branch at the National Institute of Neurological Disorders and Stroke.
SMA is caused by a defect in a gene that codes for a protein called "survival motor neuron." Its precise role is not understood, but the absence of survival motor neuron protein results in degeneration of the lower motor neurons, preventing muscles from receiving commands from the brain.
Over time, the nerve cells in the spinal cord waste away, causing unused muscles to shrink and severely compromising other vital functions, including breathing.
Wang first became interested in the illness in the early 1990s as a pediatric neurology fellow at Columbia University.
"A baby with SMA died in front of my eyes, and I could not do anything to help her," he said. "It stuck with me ... and I vowed to study this disease until there is a cure."
At Columbia, he joined other scientists in trying to tease out the molecular cause of SMA. French researchers eventually identified the culprit gene in 1995. After arriving at Stanford in 2002, Wang zeroed in on the potential of hydroxyurea, a drug originally used as a chemotherapy agent that helps boost the body's protein production.
"We found out that in a much lower dosage, this could have other benefits ... because it can modify the way the human gene works," Wang said. For this reason, he added, the approach may be applicable to other genetic diseases as well.
Elizabeth was a month old when her grandmother noticed she couldn't hold her head up and had floppy limbs. A doctor advised the baby's mother, Christen Huette, then 20, that some babies are just lazy and she should be patient.
By 5 months, however, no one was dismissing the suspicions.
SMA is not one of the 34 disorders routinely included in the state's screening program for newborns, though people with a familial link can ask for prenatal testing, according to the Illinois Department of Public Health. No one in Elizabeth's family had ever been diagnosed with SMA, so the family was caught off guard when a pediatric specialist delivered the grim news, based on a blood test.
Two days after the family was thrust into this strange, new world, Jeanna Huette took action. After stumbling across a blurb on the Internet about the Stanford University study, she confronted her daughter.
"I followed her into the bathroom and said, `Either we're going to California or I'm taking her myself,'" said Huette, jaw set, wagging her finger. "My daughter was grieving for this baby, and she wasn't even gone yet."
In June 2004, the three of them - none of whom had ever been on a plane - made their first trip to Packard Children's Hospital.
Now, every month, they haul six machines that help keep Elizabeth alive - including a cough-assist device the size of a vacuum cleaner - on a 120-mile trip to Chicago's O'Hare International Airport to fly to California so Elizabeth can be treated by Wang.
The Huettes also pack a special stroller, other adaptive equipment and containers for her special amino-acid diet, which includes breast milk, green beans, bananas and several supplements all delivered via feeding tube directly into Elizabeth's stomach every two hours.
The 14 patients enrolled in the hydroxyurea trial come from all over the U.S., Wang said.
A few have even relocated to Palo Alto - a move the Huettes briefly considered, since Elizabeth won't complete the trial until May 2007. "But when I saw the price of real estate, I just went like this," Christen Huette said, dropping her jaw.
As it is, finances are tight. The airfare for Elizabeth and her mother is picked up by Mercy Medical Airlift, a charitable program, but Jeanna Huette must pay her own travel expenses, about $750 a month. That's over and above the regular medical costs, which are daunting enough for Christen Huette, who earns $6.75 an hour at the local Kmart and lives with her parents.
The baby's grandfather, Herb, is a mechanic, and Jeanna Huette provides full-time supervision for Elizabeth. The baby's father does not live with the family.
In August - two months after the trial started - all the hassles paid off.
Instead of being a rag doll, Elizabeth moved her head. Next came her limbs and stronger muscle tone. Now, Elizabeth is chattering away, repeating her ABCs, counting to 10 and even managing a lip-curling, hip-swaying Elvis imitation.
The odds are still long, but the trips to California have given everyone something they didn't have before: hope. Family members slip easily into talking about Christmas, preschool and other future milestones.
"Whatever time we have has been a gift," said Christen Huette. "She's not giving up, so neither can we."

Tuesday, September 05, 2006

Moni And Chans First Day of Middle School

Skylar is none the wiser as he sleeps like the baby he is, so peacful and sweet. Not knowing the journey his brother and sister are about to take. Good luck guys, I Love YOu and Ill see you when you two get home!!!! Big Bug Hugs Sky

There they go.... Our Big 6th graders!!!!!!
Bye Mom.... we will be fine.... you can go now.....
Moni all excited and ready to go!!!!
Chans racen to go.... woo hoo lets go!!!

Saturday, September 02, 2006

First Haircut

Look at this cute little man, he dosent look like a little baby, he looks like a little man. Skylar did great durning his haircut, with the help of Monica and Daddy, Mommy was able to give Sky his new look in about 10 mins.