Our Beautiful Day

Lately we have all been having a really great time. Shira is changing in leaps and bounds developmentally. Our days are filled with walks, therapy, play dates, beach time, walks in the woods and up the mountain, watching videos, taking walks in the stander. Today was an especially wonderful day. Have you experienced those days when you do a wide variety of activities, unplanned and it was totally effortless? Today was one of those days a day of total synchronicity. After caring for Shira all night and getting little sleep I was surprisingly full of energy this morning. Shira was in a great mood and Sammy was full of beans not wanting to do his home work. It’s not that Sammy is behind in his studies it’s just that we want to keep his mind engaged so that when he returns to school everything will be second nature rather than relearning what he learned last year. Every day Sammy surprises us with what he figures out on his own on the computer or around the house by reading and following directions. I have to say that the only struggle of the day was getting Sammy to sit down and do his math, reading and writing exercises but he did it and the day moved on from there. Sammy attended gymnastics camp today from 1-4pm and he’ll be there all week. Shira and I hung around the house playing, practicing letters and pre reading, singing songs and playing. Maxine was out in the morning working at an assisted living home doing her music therapy with a group of seniors. So it was Sammy, Shira and I at home which can sometimes be challenging but today was a joy. We interviewed a potential employee for nights and tomorrow we will be training her and trying her out for a couple of days to see if she can care for Shira. Our help is really here to support me in shira’s care and I don’t expect any of them to perform any emergency procedures on Shira or even put her on bipap or suction her at night. I have found that while its easy to teach someone how to perform most of Shira’s care I can’t teach them what to do when Shira crashes and this is why I am always close at hand!

The afternoon was wonderful. Maxine spent a few hours with Sammy and Shira singing and playing and then Sam went over to the neighbours behind us to play with their son. I had a nap and Maxine and Shira sang and danced. Shira has really taken to her stander lately more than her power chair. Shira loves to stand up and walk around the neighbourhood in her stander. I’m used to seeing Shira in the device but I’m sure she looks like quite the site coming down the road. I had better wheels and handles put on our stander so we can get out and about in it. It is a little awkward so smooth roads work best with it. Shira loves to be spun in it! We went out for about an hour and a half walk in the stander tonight which is a feat for Shira. Us regular people take standing up for granted but SMA kids have to be braced to stand up and their bodies compress with gravity because of their lack of muscle.

We walked by one of Sam’s friends grandmother’s house from school. The grandmother always takes her grandson to school so Sammy knows her. We rang the door bell and she came out and I told her Sammy wanted to come by and say hello. She held out her arms and Sammy walked up and gave her a great big hug! Then Sammy introduces Shira saying, “this is my sister Shira.” He is so proud to have a sister! I’m so proud of my son. Sammy is such a collected centered person with so much confidence! He has such a big soul. So tomorrow Sammy is going over to her house for a play date with her grandson from 10-12 before he goes to camp.

We then walked over to the park and played there for about 20 minutes because it was getting late. On the way back home from the park Sammy starting begging to be carried on my shoulders but I can’t carry him that way when pushing Shira in her stander because it’s a two handed job. You would think the poor guy was severely injured the way he was walking and begging to be carried and a woman I didn’t know was walking up behind him going I don’t know you and I’m going to chase you which got him moving. She was saying watch out for the crazy lady in the big floppy hat. She seemed decent enough and Sam caught up to me quickly!

About a block from our house a neighbour we met while picking blackberries the other day runs over to us and hands us a plate of home made blackberry tarts which were fantastic! It was late when we got home and I removed Shira from her stander we went into the living room to watch Maxine and Sammy dance free form to some beautiful Israeli singer. Sammy can really move and Maxine and Sammy were taking turns mirroring each other. Maxine has had a lot of training in Dance and it was really beautiful and moving watching her and Sammy dance together. Sammy takes it very seriously and has no inhibitions what so ever. Sam is really into Michael Jackson and the Lion King which he just saw on Broadway in NY a couple of weeks ago. The boy is seriously artistic! After the show we performed Shira’s chest physio, Maxine gave Shira a bath, then bipap and bed and that was our day.

Ventilation After Breathing Tube Removal May Alter Outcomes

Ventilation After Breathing Tube Removal May Alter Outcomes
Published: Thursday, August 13, 2009 | 7:15 AM ET
Canadian Press
(HealthDay News) - People with chronic respiratory disorders who receive early non-invasive ventilation after a breathing tube has been removed are less likely to suffer respiratory failure or die, a Spanish study has found.

The study included 106 people on mechanical ventilation. All of them had high levels of carbon dioxide in their blood, a condition known as hypercapnia. After their internal breathing tubes were removed, in a procedure called extubation, 54 people received non-invasive ventilation for 24 hours and 52 were given conventional oxygen treatment, the according to the study.

Respiratory failure after extubation occurred in 15 per cent of those who received non-invasive ventilation and in 48 per cent of people given conventional oxygen therapy. Non-invasive ventilation was associated with an 83 per cent decreased risk for respiratory failure after extubation, the researchers found.

They also found that the death rate after 90 days was much lower among people in the non-invasive ventilation group (11 per cent) than among those who received conventional oxygen therapy (31 per cent).

"Early non-invasive ventilation after extubation diminished risk of respiratory failure and lowered 90-day mortality in patients with hypercapnia during a spontaneous breathing trial," concluded Dr. Miquel Ferrer, of the Hospital Clinic of Barcelona, and his research colleagues. "Routine implementation of this strategy for management of mechanically ventilated patients with chronic respiratory disorders is advisable."

The study appears online this week and in an upcoming print issue of The Lancet.

More information

The U.S. National Women's Health Information Center has more about http://womenshealth.gov/faq/lung-disease.cfm lung disease.

SOURCES: Kathryn Schmitz, Ph.D., M.P.H., associate professor, epidemiology, University of Pennsylvania, Philadelphia; Wendy Demark-Wahnefried, Ph.D., R.D., professor, department of behavioral science, University of Texas MD Anderson Cancer Center, Houston; Aug. 13, 2009, New England Journal of Medicine

Time Flies When Your Not Having Fun

The past few days have been tough. On Saturday night at 1:30 in the morning Shira’s SAT level crashed. I was in a deep sleep with the monitor beside me but as you all know I sleep with one eye open and both ears on alert. I flew out bed and into Shira’s room and she was not doing well! I started frantically working on her performing CPT and using cough assist. Shira’s mucous was thick like glue but clear so the first thing that went through my mind was she might have RSV (Respiratoy Synctal Virus) again. I kept working on her with Maxine hooking up the oxygen to the ambu bag so we could give Shira some restful breaths with O2 in between the chest physio and cough assist. Once I got Shira stabilized I looked at the clock and it was 2:10 a.m. The time flies by when you are so focused trying to make your child breath on their own. You work and work and work on them while you watch them slip away then come back, then slip away then come back. I was just telling Maxine that day that I needed to de stress a bit and relax then that night Shira crashes.
Once I got Shira stabile I started listening to her lungs with the stethoscope and her lower left lobe sounded like a door on really creaky hinges. When I turned Shira onto her right side with her left in the air she did not do well and I immediately turned her to her opposite side. Usually I would stay home after removing a mucous plug but the creaky lung coupled with her desating while on bipap made us go in for an x-ray and cultures to make sure Shira wasn’t sick! I was so focused on Shira while working on her I didn’t realize Maxine had called the ambulance. Once the ambulance arrived it was the same old questions as to what Shira has and how it affects her etc. which I don’t have time to go into. Maxine and I are gong to send some information to the ambulance service and see if we can educate them a little. I am thankful they sent an ambulance with power this time even though we travelled on our battery back up system.
Once we arrived at the hospital we had to go into the ER where our Dr. met us to check Shira out. By this time Shira was in a great mood but not able to come off bipap without crashing so we decided to stay until morning. We were moved into a room of our own in the ER (thank g-d). Shira was hilarious calling out to our nurse Craig, “Craig, where are you, help me Craig!! I think the ER enjoyed having Shira there as a welcome break from all the adult problems they have to deal with.
All of you know how we try and bypass the ER because of germs and go straight to the pediatric intensive care unit and get placed directly in an isolation room to avoid germs; well I was freaking out washing down the room and everything. We had a wonderful night in the ER listening to a woman scream out in pain for 5 hours straight and people vomiting endlessly. I was nothing less than horrified that Shira will catch something.
In the a.m we were moved up to the PICU and into an isolation room. All of the tests for influenza and RSV came back negative and after the consultation with the nurses and doctors we determined that Shira was dehydrated. Shira’s food was put on continuous which cut the rate in half and I started adding more water and she started to recover! It was great seeing everyone in the PICU but of course we prefer running into our nurses and doctors around town rather than in the hospital! We arrived back home at around 3:30 and Maxine let me sleep for a couple of hours as I was up giving Shira her treatments in the hospital through the night while her secretions were starting to loosen up.
I made good use of our time in the hospital this time having our dietician weight Shira, measure her and perform a skin fold test on her. Shira is doing well nutritionally and still growing well! I have plans to have a DEXA Scan done on Shira and with those results see if we can optimize her diet further based on our findings. I’m also arranging for Shira to be looked at by the department of physiology at the University of Victoria and have extensive testing on her physical make up done there also to cross reference with the other tests done on Shira. I really want to understand Shira’s physical makeup and take her nutritional requirements to the next level by being able to deliver nutrition based on more science rather than a lot of conjecture. So while it is always a horrible and very stressful experience dealing with plugging and desat episodes we used our time well in the hospital and got out of their as quickly as possible!

Thank you for all the thoughts and prayers it really helps knowing so many are behind us when the going gets tough!! Brad and Maxine.

Sammy Is Fundraising Please Give!!

Hello everyone. Our son Sammy is helping to raise funds for Chai Lifeline an organization that helps terminally ill children and their families. Camp Gan Israel the camp Sammy is attending is holding a jump a thon where the kids do jumping jacks to raise money for sick kids. Please sponsor Sammy as this is a front line organization that truly helps families and children in crisis when they are dealing with Life Threatening Illness; we know, they have helped us tremendously! Please give what you can by going to Sammy’s Chai Lifeline Web Page and donating here http://www.chailifeline.org/chai-a-thon/mypage.php?myid=2325 !!!! Thinking of all of you! Brad.

Fun In The Sun with Horses, water park, Fan Tan Alley

U.S. stem cell funding rules finalized

U.S. stem cell funding rules finalized
Last Updated: Tuesday, July 7, 2009 | 8:54 AM ET Comments4Recommend3
The Associated Press
New rules unveiled Monday allows for research on older stem cell lines to qualify for U.S. government funding.New rules unveiled Monday allows for research on older stem cell lines to qualify for U.S. government funding. (Paul Sancya/Associated Press)

The U.S. government issued final rules Monday expanding taxpayer-funded research using embryonic stem cells and easing scientists' fears that some of the oldest batches might not qualify.

The administration under former president George W. Bush had limited taxpayer-funded research to a small number of stem cell batches, or lines, already in existence as of August 2001.

Current President Barack Obama lifted that restriction in March, potentially widening the field — there now may be as many as 700 stem cell lines around the world — but letting the National Institutes of Health set its boundaries.

But the final rules issued Monday settle a big question: Would new ethics requirements disqualify many of the stem cells created over the past decade, even the few funded under the Bush administration's tight limits?

The NIH came up with a compromise, saying it deems those old stem cell lines eligible for government research dollars if scientists can prove they met the spirit of the new ethics standards. Further, NIH will create a registry of qualified stem cells so scientists don't have to second-guess if they're applying to use the right ones.
'A reasonable compromise'

"We think this is a reasonable compromise to achieve the president's goal of both advancing science while maintaining rigorous ethical standards," acting NIH director Raynard Kington said Monday. "We believe that judgment is necessary."

He wouldn't speculate on how many old stem cells ultimately would qualify, but scientists welcomed the change.

"I expect that most existing lines will be found to have been ethically derived," said Dr. Sean Morrison, director of the University of Michigan Center for Stem Cell Biology. "This will eventually make hundreds of new stem cell lines available for use."

Researchers hope that embryonic stem cells — master cells that can morph into any cell of the body — can be harnessed to one day create better treatments, maybe even cures, for ailments ranging from diabetes to Parkinson's to spinal cord injury.

Culling those stem cells destroys a days-old embryo, something many strongly oppose on moral grounds. Once created, those cells can propagate indefinitely in lab dishes.

Federal law forbids using taxpayer money to create or destroy an embryo. At issue here are rules for working with cells that initially were created using private money.

NIH sifted through 49,000 comments from the public in finalizing the rules, which take effect Tuesday. The draft changed little, as stem cells created solely for research in whatever manner, including cloning, won't qualify.

Diet and Advocacy Spinal Muscular Atrophy. Use this to fight for your kids health!! CDC Growth Charts for Children with Special Health Care Needs.

http://depts.washington.edu/growth/cshcn/text/page1a.htm


The CDC Growth Charts for Children with Special Health Care Needs.

Diet is one of the main factors of an SMA patients daily therapies. Without good nutritional support SMA patients don’t thrive well. Diet creates a lot of problems in our doctor patient relationships also because there is so little information as to how diet affects SMA Patients. Doctors hold fast to conventional wisdom and the BMI index when calculating an SMA patients needs.

The above site will steer you health care professional, doctor, nutritionist in the right direction. I suggest carefully reading this site, copying it into hard copy and saving the link in case you need it to train your health care provider as to your childs special needs!

Here is a quote from Section 5 of the site:

“ in bone size and fat and muscle distribution make the use of the reference data for many estimators of body composition inappropriate for many children with special health care needs. For example, BMI-for-age may not identify overweight in some children who are "overfat" because of decreased muscle mass.
Since the body composition of a child with special health care needs may differ from that of the reference population, skinfold measurements, along with BMI-for-age, may provide additional critical information about a child's body composition. This information can be useful for monitoring changes in an individual's body composition and growth pattern.”
When it comes to diet health care professionals just guestimate and I say its time for them to start taking notice and performing skin fold tests etc to more accurately come to conclusions and recommendations!
I hope you have found this information useful! Brad.

http://depts.washington.edu/growth/cshcn/text/page1a.htm