Medical Suppliers Profitting Off Terminally Ill Chidren - The Rotten Vermon Stench Of The Medical Supply Business

THE COST OF BEING SICK by Brad Fisher

Most healthy people do not realize or understand the perils caregivers and patients must deal with on a day to day basis. You think that if you get sick you will be cared for. Here in Canada we have a socialized system of medicine. Don’t think for a minute we aren’t paying for this medical because we have very high taxes and these taxes pay for our medical. While our system is socialized patients with very complex needs are not having these needs all met. Our daughter has a life threatening condition called Spinal Muscular Atrophy Type 1. While our daughter is physically immobile she has 110% cognitive function and can communicate but this article is not about my daughter’s illness as much as it is about medical suppliers and the system.

Since our daughter’s diagnosis I have noticed the incredible cost of medical devices for children with life threatening disease and other children with disabilities. What gets me is how prices of equipment and supplies don’t get any less expensive even while our dollar keeps rising close to parity with the U.S. dollar. I’ve noticed just looking on line that the cost of items is in many cases 30-60% less than suppliers are selling them for here in Canada. Because I was once in the retail business I completely understand how retail operations purchase from manufacturers and distributers. I know that items a retailer purchases cost less when they purchase in bulk or just have an account with a supplier or manufacturer. Then retailers turn around and sell items at the manufacturers suggested retail price which can often times be 40 to over 100 percent more than the retailer paid for these items. I understand people have to make money that is what business is all about.

What about these businesses that supply the government or in our case our socialized medicine? Why is it that the government who routinely does not cover 100 percent of the cost of items for our daughter, that have to be additionally funded by charities, cost more than when our dollar was 40 percent less than the U.S. dollar. Why does our government or socialized medical prefer to pay a local supplier sometimes 50% more for an item because they want service vs. paying 50% less from a supplier in the states thereby not forcing families to seek additional funding from charities to purchase equipment for their children!

If we look deeply into the business practices of our provincial government purchasing from local medical suppliers I guarantee we will uncover graft, over billing, and cheating on every level. I see medical suppliers as vermin feeding off profits made from sales to families with terminally ill children not caring if the item exceeds the programs allowable funding knowing full well charities will cover the rest. Yes I believe in business and profits but when government health programs are not paying the full share of the cost of equipment thereby making the public pick up the tab over and above the taxes we pay through donations to charities you have to stop and ask why? Who is in charge? Why is there no accountability? Why is the government denying equipment and other services or only funding portions of them? Why is the government paying too much for products?
I have done some comparison shopping recently on a piece of medical equipment our daughter needs and found the government could save almost $2000.00 purchasing the item in the U.S. The argument the government has is what if we need to service the item. Even if the item had to be serviced and paid for it would still be less expensive than buying from local retailers not taking the high dollar into account. Government medical contracts do not create competitive pricing. The lack of competition in Canada has bred an environment of suppliers feeding off the people that need the most help. It’s time for our government to step in and oversee purchases made and what these suppliers are doing so that children with life threatening illness are not denied equipment needed to improve their quality of life. It’s just not right that people need to go to charities to raise money to buy medical equipment while medical suppliers laugh all the way to the bank on the backs of children with life threatening illnesses, the elderly and the challenged. Shame on you medical suppliers; How do you look your children in the eyes knowing your pricing may get a child denied a much needed piece of equipment that has a life threatening illness and shame on the government employees not standing up to bad policy!! Shame, shame, shame on you!

Shira Is The Face Of Help Fill A Dream

Dear family and friends. Please vote for Help Fill A Dream. Help Fill A Dream is a front line organization that fulfills children's dreams that have life threatening illnesses. Help Fill A Dream aslo helps with medical expenses, purchasing medical equipment and more. Please vote for Help Fill A Dream. This costs nothing but 3 minutes of your time and lots of money is being given away that we could win for this incredible organization. Help Fill A Dream purhcased, delivered and erected the playground in our back yard!!!! go to : If you go to www.helpfilladream.com and click on the Aviva banner, at the top of the page it takes you to the HFAD idea....With apologies for their less than intuitive site. and please every day until the competition is finished , often and pass this around. Thank you from the bottom of our hearts!

Think For Yourself, Question Authority, Create Your Own Reality!

Living with a child that was supposed to have died before the age of 2 is a wild ride. Every day I pinch myself because Shira is here and thriving against all the local doctors expectations. What made my wife and I disbelieve what the doctors were saying to us. Reliving the early journey we took with our daughter diagnosed with a terminal illness went something like this. At 4 months of age after many months of knowing something was wrong with our daughter, seeing 4 doctors and 1 chiropractor, all of them blaming us as knew anxious parents. We were told to take drugs, drink wine, get counselling everything under the sun as help for our daughter except actual help for our daughter. Until I read Dr. Jerome Groopman’s book How Doctors Think I really thought medical doctors really knew what they were doing. It was not clear to my wife and I that biases also crowd doctors minds making them misdiagnose or not diagnose properly like in our situation. Luckily for me my wife has the ability to think way outside the box, research and ask very tough questions in a linear manner as not to offend.

When Shira was finally diagnosed at 4 months of age clinically and then genetically with Spinal Muscular Atrophy type 1 and we were given the horrific statistics of this disease our sister in law informed us there was a doctor who specialized in treating SMA patients. Upon hearing about Dr. John Bach we felt we had to take Shira to see him as there might be hope but Dr. Bach was in New Jersey in the U.S. As my wife made a phone call to Alaska Airlines to make reservations she struck up a conversation with the ticket agent. My wife told the ticket agent we had to get to New Jersey to see a doctor because our daughter was sick and there was a specialist there. The ticket agent asked Maxine if our daughter had SMA. We were blown away. It turns out the ticket agent had a son now living in Denmark that also had a grandson with SMA. The ticket agent kindly gave our phone number to his son. The next day I got a call from Josh Mortenson who told me his entire story about his son’s diagnosis and how the NIV Protocol of Dr. Bach had saved his son’s life from the medical system and all the conventional thought around SMA.

The truth in the words Josh told me, his experience so close to ours and many others spurred us on to seek out what Dr. Bach was doing. The next thing that happened is we joined smasupport which confirmed that our story and experience with the medical system was not unlike every one else’s experience. Everyone else’s experienced also included being told there was nothing they could do, there was no hope, take your child home and love them. But this was not the case.

There are things you can do to help patients whether they are very sick or just have a broken leg. Our kids are very sick and they need a lot of support. I read everything I could voraciously and also compiled and libraried every single bit of data I could find on the treatment and care of SMA patients. I realized early on that all the doctors advising us had absolutely no information on the treatment and care of SMA patients other than end of life care (just let your child die) or tracheostomy and conventional treatment with it.

We realized early on that there were 2 camps of thought in the SMA world and that was 1) There are treatments which can insure your child does not die an untimely death while treatments are being sought.
2) Let your child die.

All of the doctors we ran into before Dr. Bach were of the philosophy “Your child will have no quality of life you should let them go.” Who are these people? Do these doctors have sick children themselves? Do they have children? What are their religious beliefs? How many SMA patients have they worked with on a daily basis? Do they know of any treatments for SMA? Do they know of any doctors that treat SMA patients as a specialty?

We had a lot of questions and the more we asked the less answers there were. So why didn’t people know about the NIV Protocol of Dr. Bach? Why do people just follow what a doctor says to them without questioning?

There seems to be 2 camps of people. I have met people that love to care for children with disabilities and think the world of these children. I have met people that struggle horribly with regular children and can’t seem to keep their lives together even thought they have 2.5 healthy children, a mortgage, a career, cars and take holidays and their lives are hell.

Is life hell if you have a disabled terminally ill child or is life opened up for you? I was certainly a different person before Shira’s diagnosis letting myself be controlled by people in positions of power believing education, money, position etc. proved they should be listened to. But the reality is these things are fabricated, created. If these people in these positions go home and close the door and stop doing what it is that put them there in the first place those positions would cease to exist.

What doesn’t cease to exist is our child’s disability and what it takes to care for these children. I have had people tell me to let my child die because it will ruin my real estate career. We have had people tell us that our child is a gift from God and God gives you what you can handle. There are many ways to perceive your reality and many ways to deal with one’s own reality. To tell you the truth trying to justify and figure out our reality based on what other people thought started to become very confusing. We realized that no matter how much we struggled with what was happening with us we had to look past ourselves and start to deliver the NIV Protocol to Shira. As Dr. Bach says, “Non intervention in life threatening illness is a self fulfilling prophecy.” We had to get past our pain, our suffering, the words of others, the lack of others experience, the distorted views of others with no experience in these matters and start doing something for Shira.

My father used to tell me all the time, “If you want something done right do it yourself.” Well this was it. Here was Dr. Bach and his 2 published books “Management Of Patients With Neurological Disease” “Non Invasive Ventilation” and our doctors with no experience, no publications nothing. It was obvious what we wanted to do. If we wanted our child to live as long as possible in hopes there is a cure while she is alive then we had to intervene. We couldn’t wait for a system that was so hung up on policy, doctors personal biases, lack of resources, lack of knowledge etc. etc. we had to do something now.

As soon as we got in touch with other parents on smasupport we realized our experience was not our own it was endemic and attached to any disease with a bleak prognosis. Doctors give up when there is no cure. There was a huge chasm between what doctors told us and the reality of what parents were doing and the results we saw. The internet is Shira’s life saver. Because of the internet we were able to seek out doctors that do care, that believed there was a quality of life to be delivered and to be lived. We realized that doctors while being trained basically the same way its how doctors think that make them different and set them apart. Some doctors are totalitarian and its there way or the highway while some doctors give you many choices, help educate you, work with you and support the families wishes and desires. It would be wrong to lay the whole blame for the terrible journey families have to take through a valley of misinformation and horrible support. Many families themselves have serious internal strife among family members which does not allow for an environment of high level care.

So what it all comes down to is perception and the creation of what reality you want and then advocating for it. There is no one reality that has to be adhered to there are choices. We as parents can choose to look at our children and put labels on them like normal, disabled, terminally ill what ever you wish. The label I have chosen is the label human being. Shira is as much a human being as anyone else and we will deliver what she needs to live as good a life as she can the same way we do for our other child and each other. This journey called life has no one way and nothing is guaranteed. We only have our perceptions and biases created by our early socialization of how we were brought up and all the life experiences we have gone through. In the end our perceptions of our reality have to be let go to be able to support another human being. In simpler terms it means, “taking a walk in someone else’s shoes.” Caring for children with Spinal Muscular Atrophy type 1 is an emergency situation. It takes dedication. You have to deliver Multi disciplinary intensive care to your child daily and become adept at doing medical procedures you never thought you would have to do. It’s a choice to do these things. I have watched people put 100 percent of their energy into fundraising and choose to let their children pass. I have watched people choose not to do anything and let nature take it’s course. I have watched parents trache their children. I have watched parents do what we are doing following the NIV Protocol of Dr. Bach. I have seen everything in between.

It’s my hope that by reading what I have written here you will questions authority, gather information, speak with other parents and create your own reality based on perceptions created through experience not solely on other people opinions. The worst opinions we have heard are from those in the medical field while the best information, life saving information has also come from the medical field. As they say in business Caveat Emptor (Buyer Beware). Don’t believe everything you hear and don’t compare yourself with others as it will lead you down the wrong path. Look past yourself and see your child/patient and do what you know is right based on fact not fiction. Research, research, research and speak with lots of parents with children living passed the biases and misinformation of uncaring health practitioners who don’t care.

Diet and SMA ~ Our children have different baseline "NORMAL" so calculate for it stupid!

The problem is is that the dieticians working with doctors (even the good doctors) calculate diet to the 10th percentile using the CDC growth charts for regular children. Doing this is NUTS!!! if you take into accounte the physical makeup of an SMA patient based on Dr. Kelly's findings i.e. that "that sma type 1 patients have muscle mass as low as 5-10% that of a regular child and that muscle mass makes up around 40% of your body mass" if you calculate to the 10th percentile the patient would be obese as far as fat to muscle ratio. Dr. Kelly also states that only the Age/Length charts should be used to insure the child is growing properly. When a child stops growing properly is anyone's guess when it comes to SMA Type 1 there is no data at all let alone even clinical or anecdotal there is none. The main thing to remember is that if a dietitian recommends you do something without doing skin fold tests and other tests to properly estimate the bodies physical makeup they are only guessing. And because they have guidelines set out by the government they have to follow they have to calculate to the 10th percentile to avoid liability problems. This is why the Kennedy Krieger CP Quadrilgia chart is so important becasue at least its a population with closer physical traits than regular children and if you are going to use a chart it just makes more sense. Here is an explanation below from Dr. Kelly to Jeanna Huette years ago. This information is basically what the AA Diet is based on as far as calculating the diets. I advise getting skin fold tests done on your child by a qualified dietitian that does it a lot and is good at it. Shira has muscle mass around 15% that of a regular child. it's important to note the information below should be applied to what ever diet you deliver to your child and that the diet should be calculated optimally and re calculated often. The goal is to deliver the optimal nutrition without excessive volume to prevent reflux, and retain as much movement as possible. Our children are sedentary and have very low muscle mass so there needs are far different than regular children. The trick is to find the balance and deliver exactly what they need and like the rest of us they need a healthy diet not just processed food which I see all the time building diets for people. The type of tube and placement are also important when calculating diets but in my opinion the crap most dietitians suggest these children to eat is nothing short of ridiculous with very little thought put into it.



, “For such an "average" child, muscle constitutes 40% of body weight, whereas for the average SMA-I child, muscle is at most only about 10% of body weight, and often only 5%. Similarly, because about 40 to 50% of caloric expenditure is from muscle metabolism, a child with SMA needs far fewer calories, often only 60% of that recommended for age. For nutrition recommendations, physicians are taught to go by the book. However, unfortunately, there is no nutrition book written for SMA, and dietary recommendations made using standard scales are just not appropriate. For example, when a child is very small for age (below the third percentile), as some SMA children are, physicians are taught to use the "weight-for-height" chart to specify an ideal weight for a child's size rather than age. However, again, the weight-for height charts were developed for children whose body composition is normal or at least potentially normal when better nourished, which never is the case for SMA. Thus, the published weight-for-height charts are not valid for SMA and should never be used.



Although what I have written here explains the basic principles behind the special weight and nutrition goals for SMA, in practice I usually look only at the length chart to make sure a child's linear growth has been steady. If so, then the rest of my recommendations are based on what a child looks and feels like, not a number that I calculate. However, for physicians who are not familiar with SMA and muscle disorders of similar severity, the calculations I have presented usually help them approach the problem correctly and avoid the almost universal problem of overfeeding in SMA.” 



I also want to point out that just because an SMA Type 1 child is alive passed the age of 2 doesn't mean they are healthy for an SMA Type 1 child it just means they are alive. Many dietitians and parents can't look passed this fact and overload the children and fall back on the knowledge just that the child is alive so in the SMA Type 1 world must mean they are doing well. our children have a different baseline normal than regular children and most doctors and dietitians do not want to believe this. Food has many psycho social dynamics attached to it. Some parents have serious eating challenges themselves which directly affects their children and the same goes for doctors and dietitians and their own problems directly affect what they see before them instead of using science, logic, observation within our community etc. basically science. It's pathetic!

Modified Running Stroller

CLICK HERE FOR LINK TO ORIGINAL ARTICLE

Four-year-old Shira Fisher loves to play, sing, and most of all, go on runs with her dad.

Shira was born with Spinal Muscular Atrophy Type 1, a genetic disease that causes muscle weakness and affects voluntary muscle movements. Her condition requires that she be kept in a supported, reclined position.

Scott (left) with Brad and Shira at the UVic track testing the modified running stroller.

Scott (left) with Brad and Shira at the UVic track testing the modified running stroller.
When Shira was a baby, her father, Brad, would take her for runs in her stroller. She loved these outings and even participated in marathons with Brad. But as Shira outgrew her stroller it became increasingly difficult for the two to continue this activity. Brad even purchased an EASyS running stroller, but it was not meeting their needs.

The EASyS stroller had a fixed front wheel that made turning difficult. The design of its rear suspension made Brad tire quickly. And the tray beneath the stroller, meant to hold Shira's medical equipment, was too low to the ground and would frequently bottom out. Running had become more of a burden than a joy, so they stopped the activity altogether.



Yet Brad still wanted to run with his daughter. After all, running gave Brad some much-needed exercise, a break from his full-time care duties, and an activity to enjoy with Shira. For Shira, having her dad push her around the track was a blast!

As former CanAssist clients, they knew CanAssist could help. So Scott Lagadyn, a member of CanAssist's mechanical engineering team, set about modifying Shira's old stroller.

A close up of the new pivoting front wheel.

A close up of the new pivoting front wheel.
Scott removed and then completely rebuilt the front structure and wheel, which was making the stroller so difficult to turn. He replaced it with a pivoting wheel taken from a BOB brand stroller. This high-quality unit includes a wheel "lock-in-place" option.

"Brad now has the option of a pivoting wheel, which allows for easy turning, or a straight wheel," Scott explains. "By locking the wheel in place, the stroller is able to remain stable at higher speeds."

Scott eliminated the rear suspension, replacing it with the rear axle from Shira's brother's old running stroller. The added rigidity means easier running for Brad.

Lastly, Scott raised the medical equipment tray several inches so it no longer bottoms out and interrupts Brad and Shira's run.

When Scott delivered the stroller, Shira was ecstatic! She wanted her dad to run with her right away. Brad ran up and down the street to test the stroller with his little girl. It was clear that Shira was in her element; when Brad stopped to chat with Scott, Shira impatiently pleaded "again, again."

Shira was not the only one delighted with the modified stroller.

"Running around the University of Victoria track with Shira was incredibly emotional and brought tears to my eyes," says Brad. "Once again Shira and I are able to enjoy the freedom of running together!"

With the little girl safely nestled in her modified stroller, Brad and Shira are back in training mode and are preparing to run the Victoria marathon this fall.

Happy New Year 5771

Wishing our Family and friends a happy and sweet new year.
L'Shana Tovah Tikotevu V'techataimu- Shana Tovah Umtukah. May all our prayers pierce the Heavens, especially our prayers for Peace in Israel and around the world.

Summer Time Fun

I don’t know where to start as we have been having so much fun. Sammy has been in many different camps this summer. Besides the camp he has been going to since he was 4 years old the Victoria Conservatory Musical theatre camp was a big highlight for Sammy and for Shira. As many of you well know I am a huge advocate for staying away from social gatherings of any kind with Shira. Whenever you are around more than your own family your chance of infecting Shira with something goes up exponentially. Being summer and knowing how slow our local pediatric intensive care unit is we decided to take Shira to Sammy’s recital. Sammy had been practicing the songs from Annie for many days so of course Shira also learned them. Where ever we go Sammy and Shira sing It’s A Hard Knock Life and The Sun Will Come Out in unison. It’s the cutest thing you’ve ever heard and seen. I was walking down the street the other evening with Sammy and Shira in her stroller. The streets were very quite and you could here their voices ringing out singing It’s A Hard Knock Life. Shira in her stander certainly makes those words ring a little true except for the fact we don’t torture her at home like Ms. Hanagon. Anyways Shira was blown away by the beautiful stage and of course the recital. Shira screamed when the young girl playing Ms. Hanagon appeared on stage. It was an amazing production and Sammy was the only boy to get a part out of the chorus. Sammy played the policeman chasing down Annie and he did a great job!

Yesterday we took Shira to her first movie and it was an IMAX movie no less. The movie opened up with a 6 story Orca Whale jumping at us. The movie was about whales and it was absolutely beautiful of course. Most of the scenes were from South America and it just made me want to get on a plane. Shira absolutely loved it and she kept singing Aerial’s little theme song from The Little Mermaid all the way through the movie. When the movie finished Shira said, “Yeah!” After the show we walked through the whole B.C. museum which she also enjoyed.

On Thursday I took Shira to Sammy’s outdoor camp but I have to say it was not all that exciting and I signed Sammy out and Shira, Sammy and I took off together. We made a trip to Shira’s school www.sides.ca to see if anyone would be there. Shira will be attending Kindergarten this year at home but the school will be sending a teacher to our home. I of course took Shira and Sammy to the wrong campus and I guess they called where we were supposed to go because when we got there they were all waiting for us. Shira kept saying, “I’m so excited, I’m so excited!” The whole staff was so welcoming and loving towards Shira it was a beautiful thing. It was really like someone was on our side, really understood Shira and could see how much potential she has. While we were at the school Sammy felt the need to push Shira in her wheel chair. Sammy is so proud to know his sister is going to school. The staff really treated Sammy well and really understand the dynamics between siblings and the role they play in the family unit with a child with challenges. We had the greatest time and it was very energizing to be there.

Today we are off to the Johnson Street Market then maybe a visit with the goats at Beacon Hill Farm.

Spinal Muscular Atrophy~ Our Day At India Day

Here is a video of us at India Day Victoria, B.C. It was too hot but the kids had fun! http://www.youtube.com/watch?v=j0sBpvn8LNQ

Shira Watches Mommy Perform At Hillside Mall

Shira is now 5 years old and believe it or not she has only seen her mom perform in public 2 times. The reason for this is we keep Shira away from social gatherings. 90% of children with SMA Type 1 are dead by the age of 2. These statistics are based on no intervention and high levels of exposure to other people which lead to death through disease. Shira has had relatively few hospitalizations because of our disease protocol (can be viewed here http://www.asonginthisworld.com/images/client_pics/DISEASE%20PREVENTION%20FOR%20OUR%20HOME.pdf

But being summer and cold and flu season slower this time of year (but not totally gone you still need to be vigilant)we took Shira to the mall to watch Maxine perform her children's show Let's Make Music And Move. Here is the video: http://www.youtube.com/watch?v=pXkxtvJdke8 Shira absolutely loves watching her mom up there and all the children reacting to her. Shira also loves singing and performing herself and really wants to get up on stage and sing which we might do next time.

As you can see Shira has a little glass bead neckless with a lion bead on it. We were at a Pow Wow last 2 weeks ago and Shira chose the beads and the whole family pitched in and helped her make it. Sammy also made a wonderful bone bead neckless with beads he bought there.

Shira is really doing great and has so many interests like regular kids. She just needs more support when doing things. Today we are off to an Indian Festival downtown. Shira and Sammy are both excited to dress up in their authentic Indian clothing from India and going to the festival. d

Tonight Shira said, "I have SMA. I want to be grown up!"

I'm not sure but I think Shira is thinking when she is grown up she will be able to walk and be like everyone else. Now i'm thinking how do I deal with these questions. I've just started searching for answers to these questions and found this article on www.fsma.org web site:

How do I explain SMA to my young child?



Dear Parent,
You asked how to tell your son about his SMA without damaging his soul. I am 26 years old and have SMA. As I recall, my parents were a lot sadder about my disability than I ever was as a child. Under no circumstances did I want my parents to be sad about me, and I still don't. Your son probably does not want you to be sad about him either.

As far as what to tell him, I think it is generally best to answer his questions as he asks them, not before. At his age, I remember I wanted to know what the reason was that I could not walk, and by that I mean I wanted to know what the malfunction of my body was, but everybody usually gave a sermon every time I asked. It took a long time for me to get the answer I was looking for, that my nerves (which were like phone lines) were not able to get enough messages from my brain to my muscles, and so all of my muscles were very weak because they didn't get my brain's messages enough. So listen carefully to what your son asks and don't make his questions bigger than they really are. You say you are worried how to tell him that he won't walk again. It is possible that he doesn't believe he will walk again and just wants to know whether or not he will. I would tell him that he will probably not walk again, and then ask him how that makes him feel. Talking to him about how he feels, however he feels, is more important than trying not be optimistic in telling him the news. Your son will react how he will react. All you can do is be there to support him through his reaction.

He probably will ask you himself whether or not his illness is life threatening. If he ever watches the MDA telethon, gets hospitalized with a bad pneumonia, or meets others with neuro-muscular disease, trust me, he will one day ask you if he is going to die young. You must tell him the truth, which is that some people with SMA die young and others do not. Whether or not he dies young is up to how healthy he tries to be and up to whatever God has in mind for him. Tell him this is true for all people, not just people with SMA. Tell him that his job on earth is to live as fully as he can, not to wait around for death. And then ask how he feels. If he feels scared or sad, let him know how you feel when you think about dying young. It's a difficult idea for everyone, after all.

I would recommend you introduce your son to Stephen Hawkings' movie, A Brief History of Time (not the book - it's incomprehensible) when he's a little older. Stephen Hawkings has ALS, a neuromuscular disease that it almost always fatal. Stephen has lived more than 20 years longer than predicted, and his doctors are forever saying that he only has a little more time left. He is married, has kids and has made a huge contribution to the world through his work. He's a good role model for children who are worried about the life implications of ALS, not because he has survived, but because he lives the life he has.

Sincerely,
Jennifer Rellick

Soul and Giving

On Wed morning Shira, myself and our care worker went to a mall to watch Maxine perform for children. Maxine is a music therapist with a masters degree in psychology but she also has a show created specificly for toddlers. It's not easy to keep a toddlers attention for 45 straight minutes but Maxine is a master at it and really understands what children need. Shira has only seen Maxine perform 2 times because we have been so afraid to expose her to germs in social environments. Shira of course loved the concert and watching mommy and all the children responding to mommy. After the concert the mall gives out balloons to children. Shira was given 2 balloons and when we walked away I heard a little girl start to cry hysterically because she didn't get a balloon. I told Shira a little girl was very sad because she didn't get a balloon and would she like to give one of hers to the little girl. Shira being Shira said, "that's not good daddy! We can give her a balloon!" We walked over to the girl and her mother and I said my daughter would like to give her little girl one of her balloons. The mother looking over at Shira laying in her stroller drooling with her oxymeter and food line going under her shirt, her orthotics and wrist brace said, "no it's ok you don't have to she'll be alright" probably thinking what is this I couldn't take a balloon from this girl. Shira in her very low volume voice was saying, "don't cry have a balloon!" I held Shira's hand in mine because she can't raise her own arms and moved her arm in the direction of the little girl with the pink balloon in her hand. Shira gave the little girl the balloon and the little girl started to settle down but was still hyperventilating. I almost lost it (cried) when Shira did this. Shira is my song in this world, my mitzvah girl (good deed).

Below is an explanation about soul by a chassidic master Rebbe Nachman. I found it interesting.

The differences between the body and the soul are vast. Yet their combination is what makes "man." They can work together in perfect harmony.
Rebbe Nachman taught:
A person must care for his body, so that it shines with each spiritual advancement he achieves. for the soul perceives and understands extremely lofty levels, while the body remains ignorant of them. A person must therefore purify his body, to enable it to share in the soul's perceptions. The soul will also benefit from a body which is finely attuned to spirituality, for fi the soul falls from its spiritual level, the body which has experienced Godliness will enable it to regain its previous level of holiness.

This can occur because the body has attained a corresponding level of purity.
For a person to attain this level of harmony and cooperation between body and soul, he must break the brazenness of the body's lusts and desires by countering it with "holy brazenness, " the stubborn will to bring himself to spirituality, come what may. In doing so, he allows his soul to blend completely with his body.

The means through which the body blends with the soul is the performance of the mitzvot (charity or good deed). The more good deeds a person performs, the greater his body's subjugation to his soul, allowing the body to actually feel the soul's attainments. (Likuty Moharan I, 22:5, 8) from Anatomy of The Soul Rebbe Nachman Of Breslov

Probiotics cuts intensive care infection

Probiotics cuts intensive care infection
Published: June 17, 2010 at 7:01 PM

OMAHA, June 17 (UPI) -- Ventilator-associated pneumonia in critically ill patients in hospitals was cut in half after probiotics were given to the patients, U.S. researchers say.

Lead author Dr. Lee E. Morrow of the Creighton University says ventilator-associated pneumonia affect an estimated 30 percent of patients who are hospitalized in critical condition.

Morrow and colleagues chose 138 critically ill patients from a single hospital to receive either placebo or probiotic therapy. Patients who received probiotic therapy got Lactobacillus rhamnosus twice daily.

The researchers find the daily use of probiotics not only decreased ventilator-associated pneumonia infections by about 50 percent compared to the placebo.

The study, plus a meta-analysis of existing studies, finds an overall reduction in ventilator-associated pneumonia of 39 percent with probiotics, suggesting a novel, inexpensive treatment, but more than 90 percent of patients in the intensive care unit were deemed ineligible for the study.

"Larger clinical trials



with more liberal inclusion criteria are needed to establish the effectiveness of probiotics and to allow for extrapolation to a larger at-risk population," Morrow says in a statement.

The findings are published online ahead of print in the American Journal of Respiratory and Critical Care Medicine.

Justifying My Existence - Daniel Silveria

Justifying My Existence - Daniel Silveria
Hollee J. Chadwick
Published September 18, 2009 by:
Hollee J. Chadwick
More: La House Movers La Movers Quadriplegic Daniel Pearl Plate Tectonics
I was born with Spinal Muscular Atrophy and, as a result, have been a quadriplegic for most of my 33 years. My mother didn't know prior to my birth that I would be afflicted with this illness. I was diagnosed when I was
two years old. There's no way of knowing if she would have decided to terminate the pregnancy had she known in advance that her child would be disabled; if she'd have been overwhelmed at the prospect of maintaining an extremely dependent individual with a severely compromised immune system and a questionable "quality of life."

The thing about life is that it's a zero-sum game. In order for one's quality of life to be examined and taken into consideration, one must first have a life. My basic thesis here is that I'm glad that I do. If I'd have been aborted it would've made this far more difficult to write.

So I'm writing this from the perspective of the unaborted fetus, a not at all disinterested third party in this third rail debate. There are compelling arguments on both sides of the issue. In fact, I'm pretty sure I would be accused of riding the fence, since, though I am in favor of choosing life, when it comes down to it, I am reluctantly pro-choice. This is because I'm very much a proponent of states' rights, as opposed to a collection of any-way-the-wind-blows politicians in Washington deciding what's best for Joe Schmo in Idaho. The brilliant P.J. O'Rourke once compared the concept to being married, saying you can argue with the people (your wife) all you want, but inevitably it's just going to be, "Yes dear," and let democracy have the final say.

However, I'm just as opposed to some uninformed "it's my body, it's my life" able-bodied activist deciding for me and my ilk that my life, such as it is, is expendable and essentially not worth living. "To be or not to be?" is my question, not yours.

Life is worth living. When reduced to its simplest capabilities, when merely existing and drawing breath while being able to contemplate the sensation of that breath, life is worth the ride. Give me liberty, but first give me breath. And I'm not the smartest being to ever not walk the face of the earth, but having the ability to think at all allows me to appreciate the richness afforded me by the five senses. I can watch the nightly news and marvel at the human condition and all its long winded shortcomings, ubiquitous brilliance, and interwoven storylines. Despite my condition, the beauty is not lost on me. The sights and sounds all around provide more than enough motivation to get me out of bed in the morning. And even when I can no longer get out of bed, I'll find a way to supply myself with word of the movers and shakers and what they're moving and shaking.

If I am one day reduced to a coma, visit me in my subconscious Shangri-La with a bottle of something expensive, and we'll raise a coma-toast. If I'm alive, I'm not unconscious. My heart still knows where to pump the blood, my immune system still knows where to find the bacteria cafeteria. Consciousness is precious beyond any words I could put here in support of it. To say nothing of the visceral realm, which is sometimes background music, but sometimes makes the little hairs on the back of your neck stand in awe. Maybe it's a consciousness higher than consciousness.

You might be thinking that someone like me develops an exceptionally fortified wall of denial as a defense mechanism. Da Nile ain't just a river in Egypt, after all. I don't see it like that, though. The game has merely been simplified for me by way of removing some of the extras, and appreciation of what is replaces focus from what is lacking. It's all relative. Somebody that most people would agree has ideal circumstances in their life might be absolutely miserable because their focus is intensely on the few things that they lack. So the opposite is often true of someone who might be perceived by the consensus as having less than ideal circumstances. I'm not saying that I constantly see the world through the lenses of rose-colored glasses. I visit the doldrums every now and then. But there's no shortage of people pounding the doldrums. Misery loves company, so pity parties are all the rage. There are human interest stories as far as the eyes can see, where the humans of interest have a tale of woe. And the more morbid the tale, the more spectators.

It seems we are obsessed with constantly reaffirming that life is not fair. I have a very profound response to this presumption:

Duh!

We hold this truth to be self-evident - and freakin' obvious. You're perfectly entitled to your childish notions of entitlement, but reality has a funny way of shaking the plate tectonics of your paradigmbag. How many times do we lab brats have to run into the electrified walls of "life's-not-fair" and still be shocked and amazed by the maze? Pearls of wisdom are produced the same way actual pearls are: via friction and time. I have been pearl-lyzed, hallelujah!

It's my belief that depression's primary cause stems from the expectations and entitlement mentality running headlong into the Truth Train.

The brutality of reality gives its brand of tough love to the unsuspecting gamer. We all have a predetermined timeline arranged in our minds as to where we're supposed to be at a given point in the midst of this mortal coil. Kind of a biological clock, but applicable to endless other rites of passage we presume to be part of the grand tour. "I should have 2.3 kids by the time I'm 30 years old;" "I should be able to retire by the time I'm 65;" "I should have a house in the Hamptons with a gardener, chef, and personal misuse who doubles as my doubles partner and caddie, and drives my Cadi while I'm in the back, swigging gin & Jack on the cell with my broker, who's got me stalks of stocks socked away all by the time I'm sprouting grey hairs."

"Happiness never lays its finger on its pulse." - Adam Smith

You see, we have midlife crises, earlylife crises, and end-of-life issues. But what we really have is a beautiful collection of priceless moments. Moments are in the I of the beholder.

Two years following my diagnosis, my mother got pregnant again. Strangely, some highly motivated organizations caught wind of this and swarmed down on her like vultures, offering advice and support in preventing such a horrible misfortune from happening again. Because, you know, my sit-uation is genetic and there was a good chance that history would repeat itself repeat itself. This time she knew the risks, and come hell or sick toddler, was perfectly willing to accept the results. Baby brother Andrew came nine months later, happy and healthy.

No group elected or otherwise should have authority over another individual's life or death, or in any way feel justified in evaluating that individual's quality of life or value to the tribe. Social engineering cannot be acceptable ever. No matter how many pretty euphemisms you try to pin to it.

You might look at me, and compared to the rest, assess what you see as flawed. Look closer you'll find a soul and a mind. I am a living expression of God.

Physical Therapy: Flexibility, Fitness and Fun

Physical Therapy: Flexibility, Fitness and Fun

Advances in supportive care have altered the natural history of SMATherapeutic developments in spinal muscular atrophy Sage Pub. April 2010

Advances in supportive care have altered
the natural history of SMA
Any review of therapy for SMA would be remiss
without a discussion of the important and evolving
role of multidisciplinary supportive care.
SMA leads to predominantly proximal muscle
atrophy and weakness, and the potential for medical
complications such as scoliosis, joint contractures
and ventilatory impairment [Wang et al.
2007]. This latter complication is primarily the
result of respiratory muscle weakness, which
prevents the normal expansion and clearance of
the lungs leading to a restrictive defect. In recent
years, advances in pulmonary care and the
increasing application of noninvasive ventilatory
support has dramatically improved the morbidity
and mortality associated with pulmonary decline,
particularly among children with severe (type 1
and 2) disease phenotypes [Oskoui et al. 2007;
Bach et al. 2001, 2000]. Improvements in physical
therapy management and advances in surgical
approaches to scoliosis, including the use of
vertical expandable prosthetic titanium rib and
related ‘growing rods’ approaches [Hell et al.
2005], have allowed a more effective and timely
management of secondary musculoskeletal complications.
The optimization of nutritional management
to avoid potential complications arising
from both malnutrition [Messina et al. 2008] and
obesity [Sproule et al. 2009] has also emerged as
an area of increased attention in recent years
[Wang et al. 2007].
In the context of such advances to supportive
care, a notable improvement in the natural history
of SMA has been observed over the last two
decades despite an absence of efficacious therapy;
this is particularly true for children with SMA
type 1. Byers and Banker described 25 such subjects
in 1961 with a mean age at death (n¼23) of
10 months (range 17 days to 52 months), and a
mean age of 17 months in those who survived
(n¼2), range 10 24 months [Byers and
Banker, 1961]. A similar report by Zerres and
Rudnik-Schoneborn published in 1995 found a
survival probability of 32% at 2 years, of 18% at 4
years, of 8% at 10 years, and of 0% at 20 years
among 197 children with SMA type 1 [Zerres
and Rudnik-Schoneborn, 1995]. A recent analysis
of 143 patients with SMA type 1, comparing
those born from 1980 to 1994 (n¼65) with those
born between 1995 and 2006 (n ¼ 78) showed a
70% reduction in risk of death in the latter group,
likely associated with improvements in clinical
management, particularly with regards to noninvasive
ventilatory management [Bach, 2007;
Bach and Bianchi, 2003; Bach et al. 2000], and
a trend toward more proactive care [Oskoui et al.
2007]. While recent efforts have been made to
standardize clinical care with the publication of
a consensus statement of care for patients with
SMA [Wang et al. 2007], variability of clinical
care between centers and the evolving natural
history of the disease can both represent challenges
for trial design, as we discuss below.

Anyone wanting the full research paper send me an email to asonginthisworld@gmail.com and i'll send it to you.

My Take On The FightSma Conference Addendum

Here is something very interesting Sarah the dietician at the conference
really was negative about elemental amino acids and then mentioned
there are other choices like poly peptides like she Knew something we
didn't. here is some info from Dr. Bach which again is about 10 years
old

"Harpey et al. felt that there was a significant improvement in... "text_exposed");''>See More strength and function for 13 patients treated
with modified diets that provide high carbohydrate and elemental amino
acids and small chained polypeptides, such as Tolerex and Pediatric
Vivonex (Novartis, Minneapolis). (4) Although 90% of SMA type 1 patients
have been reported to die by 1 year of age, none of our 30 SMA type 1
patients using this diet have died and the oldest are now 8 years of
age. Therefore, I recommend prescription of Pediatric Vivonex for
infants with a diagnosis of SMA Type1."

I can't believe how totally unprofessional this woman is. She obviously hasn't done much research because if she is only coming up with this info now which Dr.
Bach published almost 10 years ago we aren't in very good hands are we!

My Take On The FightSma Conference

First off I would like to say thank you to FightSma for doing this as its very important to have lots of open dialogue on all of the subjects of SMA.

I do have to say that I shudder when a parent with a toddler with SMA type 1 starts to ask questions and through the questioning we find out they have no equipment and the child is having trouble sleeping among other things. This tells me we haven't come very far with the treatment and care of SMA and still have a mountain to climb to make the medical community understand that there are viable options of treatment and care. There is still a huge disconnect between the worlds of medicine and Quality of Life and Rehabilitation which cares more about the tools to live as well as you can.

Most of what was said was basic and i'm sure an eye opener for the newly diagnosed families. I disagree with one of the comments that there were many choices with the treatment of SMA. From my research which is extensive you only have 3 choices: 1) end-of-life-care. Just let nature take its course 2) NIV Protocol of Dr. John Bach 3) Tracheostomy/and all of the Bach protocol without NIV. I haven't heard of anything else beyond that?


I especially liked the fact that the PT recommends starting PT as early as possible. I would like to add that
Motor Oral therapy should also be started as soon as possible and thedisconnect between the PT's and SLP's have to stop so that we as caregivers can better treat our children from the tops of their heads to the tips of their
toes but otherwise excellent. Another subject on the same topic is speech. It would have been great if someone spoke about the fact that our kids need to speak through a microphone so they can better hear themselves and in turn their speech will develop. I think SLP's with SMA experience have to become more involved and we need more early intervention in the SLP department especially in the areas of jaw contractures, motor oral therapy, and speech development.


Diet: I have to say that Sarah Feasel, MEd, RD, CNSC comments regarding the elemental formulas ws very inaccurate, misleading and not professional. While it is true that elemental formulas were created to deal with allergies the reason why elemental formulas have been adopted by the SMA community is very different. Mary Bodzo created the AA Diet based on the findings of Dr. Kelly of John's Hopkins. Dr. Kelly found that SMA patients better metabolized elemental to medium chain amino acids more efficiently than full chain animal proteins. Below are some exerpts of conversations by parents with Dr. Kelly.


" Similarly, because about 40 to 50% of caloric expenditure is from muscle metabolism, a child with SMA needs far fewer calories, often only 60% of that recommended for age. For nutrition recommendations, physicians are
taught to go by the book. However, unfortunately, there is no nutrition book written for SMA, and dietary recommendations made using standard scales are just not appropriate. For example, when a child is very small for age (below the third percentile), as some SMA children are, physicians are taught to use the "weight-for-height" chart to specify an ideal weight for a child's size rather than age. However, again,
the weight-for height charts were developed for children whose body composition is normal or at least potentially normal when better nourished, which never is the case for SMA. Thus, the published weight-for-height charts are not valid for SMA and should never be used.

Although what I have written here explains the basic principles behind the special weight and nutrition goals for SMA, in practice I usually look only at the length chart to make sure a child's linear growth has been steady. If so, then the rest of my recommendations are based on what a child looks and feels like, not a number
that I calculate. However, for physicians who are not familiar with SMA and muscle disorders of similar
severity, the calculations I have presented usually help them approach the problem correctly and avoid the almost universal problem of overfeeding in
SMA."


On breast milk, " I unfortunately have not in recent years worked on the nutritional aspects of SMA or related neuromuscular disorders, so I don't have any more up-to-date thoughts than I have communicated in the past. I
suspect that the benefit of breast milk comes not from any special
"factor" present in the milk but from the lower protein and specific
fat content of breast milk, which better matches the nutritional needs
of someone with greatly reduced muscle mass. The efficiency of
utilization of the lower amount of protein also is enhanced because,
clearly, the amino acid composition of breast milk is better matched
to human amino acid requirements than any type of animal milk or
animal protein. Moreover, compared to animal milks and proteins,

breast milk fat contains a higher proportion of medium-chain fats,which, theoretically, should be more efficiently metabolized by SMA children than regular long-chain fat."


The dietician Sarah Feasel, MEd, RD, CNSC mentioned that the elemental formulas do not contain enough fat to promote proper brain development especially in a childs early and most important developmental stages.
This is true and this is the reason these elemental formulas are used. Because of the low fat content of
both vivonex (which contains more fat than tolerex) we are better able to optimize the diet by adding appropriate amounts of fat while still being able to deliver the correct amount of protein, carbs etc. Almost all other elemental formulas on the market do not allow this flexibility and you always end up with disproportionate amounts of fat to protein levels which works great for active children not sedentary hypotonic children. It is a misrepresentation of the diet to not explain that other foods must be added to enhance calorie intake and that other fats must be added to elevate the fat intake. anyone who is giving their child the amino acid diet and not calculating these other nutritional factors into the diet are not following the AA Diet of Mary Bodzo. The information Sarah Feasel, MEd, RD, CNSC has presented regarding the AA Diet was not science but a biased opinion unsubstantiated opinion. If she actually knew how to calculate the diet, what is added to the diet, why it was created in the first place (the history of the diet) then the AA Diet would have made sense. All that was said by the doctor at the podium and Sarah was innacuarcy and fear. I would hope that we have moved away from this type of discourse especially when it comes to our childrens health but old habits are hard to stop. In a nut shell these people do not truly understand the diet and until they do these types of opinions should not be tabled.


Growth Charts

Judging by the conversation on growth charts I suggest the professionals speaking on nutritional needs re visit the web site at the cdc at http://depts.washington.edu/growth/cshcn/text/page1a.htm ; All the way through this 8 page description of the cdc's growth charts the cdc warns of the inaccuracies of using traditional growth charts for children with special needs. While I use them only to insure there is nothing extraordinary going on with the child I am creating a diet for that is pretty much all they are used for. I take more notice of the Kennedy Kreiger Cerebral Palsy Quadriplegia charts as they have much more in common I think with SMA than regular children.


"And that's all I have to say about that!"

Please Help Us Fund Raise!!!!!

I’m asking for your assistance. I am joining thousands of Canadians who don’t take their muscles for granted, by participating in Walk For Muscular Dystrophy 2010 to raise funds for Muscular Dystrophy Canada.

You can help me by making a secure online donation using your credit card. Click on the link below:

http://my.e2rm.com/personalPage.aspx?SID=2518511

Your gift helps Muscular Dystrophy Canada fund leading research to find the causes, treatments, prevention and cures for over 100 different neuromuscular disorders. Your donation also provides information, costly medical equipment, support and hope to over 50,000 Canadians living with neuromuscular disorders.

It takes 48 muscles to lift a baby and only a few to lift someone’s spirits.

Shira Receiving Beckman Motor Oral Therapy

Shira receives Beckman Motor Oral Therapy every day right after her CPT session in the morning.

Beckman Motor Oral Therapy Handout with Diagrams and Directions
http://www.asonginthisworld.com/images/client_pics/Beckman%20Motor%20Oral%20Therapy.PDF

Beckman Motor Oral Therapy Part 1
http://www.youtube.com/watch?v=KKxbexuV804

Part 2
http://www.youtube.com/watch?v=mY6ZujX-o_4

Part 3
http://www.youtube.com/watch?v=1-jMGPhJYrM

Part 4
http://www.youtube.com/watch?v=SGu9wCxE63g

Shira Yesterday and Today

Only one week left until the NJ Premiere of Time of my Life! (Wednesday March 10th)

Dear friends. If you live in NY or NJ and need some inspiration please go see this movie. It is about the life of Simcha Esther a woman that contracted cancer and fought the bravest fight you can imagine. I am proud to say that Simcha was my friend and an inspiration to me. Simcha left 3 children and a husband, she was in her early 40's. While she was alive Simcha set out to make a documentary about her fight with cancer and her deep return into her faith. Please pass this along to anyone you know!!

Only one week left until the NJ Premiere of Time of my Life! (Wednesday March 10th)

You can now buy tickets online: http://timeofmylife.us

(Please get your tix early. Last time we sold out and had to turn folks away.)

Blessings,

Yoni

PS - Also, please help us promote this film by posting to blogs, your email lists, facebook etc.

You may wish to include the following links in your emails, postings etc:
Link to the trailer: http://timeofmylife.us/Trailer.asp

Facebook event page: http://www.facebook.com/event.php?eid=248412489589&ref=ts

Link to the website and online ticketing: http://timeofmylife.us

Great Videos Explaining What SMA Is

Here are some excellent videos by FightSma that explain what SMA is. Shira has SMA type 1.

An Introduction to Spinal Muscular Atrophy - Dr. Bob Leshner
http://www.youtube.com/watch?v=-DgUVrvrNfs&feature=youtu.be

Spinal Muscular Atrophy Type 1 - Dr. Bob Leshner
http://www.youtube.com/watch?v=QRJ2pxVxUQw&feature=related

Spinal Muscular Atrophy Type 2 - Dr. Bob Leshner
http://www.youtube.com/watch?v=w_hyHQUwhco&feature=related

Spinal Muscular Atrophy Type 3 and Type 4 - Dr. Bob Leshner
http://www.youtube.com/watch?v=-Geojki_Hn8&feature=related

The physician-scientist, the state, and the oath: Thoughts for our times

"What, then, constitutes medical humanism? I would suggest four core values: First is the preciousness (or sanctity) of each human life. The second value is respect for human dignity. The third core value of humanism is the celebration of human diversity. Finally, the fourth core value of medical humanism is a sympathetic appreciation of the complexity of the human condition- how difficult it is for anyone to meet all of society's idealized expectations regarding individual and interpersonal behaviors, and how history and circumstances have conspired to make it especially difficult for some."~ The physician-scientist, the state, and the oath: Thoughts for our times Barry S. Coller

Read The Full Paper Here: Top right hand corner full text pdf
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1578617/

Sophia Doebbert and Dr. Mary Schroth, Our American Family Children's Hospital

There are some doctors that are real heroes. Dr. Mary Schroth is one of these heroes. Rarely do you see this amount of compassion from a Doctor but Dr. Schroth really cares about all her patients so much. Maxine flew to Wisconsin to meet with her because we were advised by Doctors at B.C. Children's Hospital not to contact U.S. doctors because they wouldn't be there for us in our time of need. Maxine was met at the door of the hospital by Dr. Schroth and the social worker. Dr. Schroth (the for profit doctor) spent 3 hours with Maxine.

While we witness children failing extubation all over the U.S. we see them flown to Wisconsin to be succesfully extubated. Doctor Schroth sees our children as human beings that are valued members of our society.

This video is of Sophia Doebbert from Minnesota. Though we haven't met her we feel very close to this family. Doug and Andrea have been great support for us sharing their knowledge and just being there for us, to listen to us. Watch this video

http://ourkids.uwhealth.org/stories/entry/beyond-expectations-the-sophia-dobbert-story/

A Day With The Ducks

Click on the link below to watch Shira's video

http://www.youtube.com/watch?v=z1ETv6qIPLU&feature=player_embedded

What Is a Life Worth? Research on the cost-effectiveness of medical treatments pits our emotions against our pocketbooks.

What Is a Life Worth?
Research on the cost-effectiveness of medical treatments pits our emotions against our pocketbooks.

By Sharon Begley | Newsweek Web Exclusive

Feb 5, 2010

This is the kind of news that unleashes hysteria about "death panels" and "health-care rationing," but here goes: an analysis of genetic screening for an incurable, untreatable disease called spinal muscular atrophy shows that it would cost $4.7 million to catch and avert one case, compared with $260,000 to provide lifetime care for a child born with it. So here's the question: do we say, "Damn the cost; it is worth any price to spare a single child the misery of being unable to crawl, walk, swallow, or move his head and neck"—or do we, as a society, put on the green eyeshades and say, "No, sorry, we can't afford routine screening"?

Just to be clear, this is not about comparative-effectiveness research, which analyzes whether treatments work and which ones work best, without regard to cost. In contrast, studies of cost effectiveness, like this one, take something that works and ask whether the cost is worth the benefit. There is no question that screening for the genetic mutation that causes spinal muscular atrophy works, in that screening detects the mutation in 95 percent of those whose DNA has it. If a couple decides not to conceive (if they were screened before trying to start a family, they can instead use donor sperm, for instance), to implant only healthy embryos (if they are using in vitro fertilization), or to terminate the pregnancy, then a child with SMA is not born.

The study presented Thursday at the Society for Maternal-Fetal Medicine’s annual meeting starts from this "screening works" premise. What, asked Sarah Little of Massachusetts General Hospital and her colleagues, would be the costs and benefits of universal screening?

Here's the numbers-heavy paragraph. One in 50 Americans carries an SMA mutation. Because carrying one copy of the mutated gene causes no symptoms, people don't know if they're carriers (though having relatives with SMA should raise suspicions). If two carriers conceive a child, however, there is a 25 percent chance the child will have SMA, which is the most common genetic cause of infant mortality. About 1 in 10,000 babies are born with SMA. Of those children, 70 percent have a severe form of the disease, in which newborns are limp and floppy, cannot lift their heads, and have difficulty rolling over, sucking, and even swallowing. Universal screening detects 95 percent of carriers, and 2 percent of babies born with SMA have the disease even though neither mom nor dad is a carrier (the gene mutated after conception). Screening costs just over $400 and lifetime care for someone with severe SMA costs $260,000. For an individual, that seems like a great deal. But throw all of this into a calculator for society as a whole, and the answer is this: 11,000 women would have to be screened to prevent one case of SMA, at a cost of $4.7 million per case averted.

Since the $4.7 million cost of averting one case of SMA swamps the cost of having a child with SMA, the brutal conclusion is that "screening everyone for SMA is not cost-effective," concluded Little and her colleagues. Only in people with a high risk of the disease, such as those with a family history of SMA, might it make sense.

Screening for SMA has been controversial for years. In November 2008, the American College of Medical Genetics recommended making carrier screening for SMA available to all families. But last May, the American College of Obstetricians and Gynecologists came out against preconception and prenatal screening for everyone, largely because you would have to test so many couples to avert a single case of SMA. The appeal of screening for genetic diseases seems obvious, however, and new companies are springing up to give parents-to-be the assurance they want.

So here's the issue. What if an insurer or (horrors!) "Obama-care" refused to cover SMA screening because it is not cost-effective? Judging by the hue and cry over the very idea of using cost-effectiveness to make coverage decisions (something that was never in any of the health-care bills), millions of people would be appalled—especially once the first SMA baby was born after his parents' insurer refused to pay for a genetic test. Unconsciously or implicitly, then, many of us are in favor of paying $4.7 million to let a couple know that their fetus has SMA. But as we've seen in the public hostility toward health-care reform, there is deep ambivalence about how much of our hard-earned money we are willing to spend to avert preventable deaths in people who die because they do not have health insurance. For many Americans, that answer seems to be "not one dime." It's the old story: we respond to specific cases of tragedy when they come with a name and a face, but grow cold and uncaring when the unfortunate are nameless masses.

More such cost-effectiveness analyses of various medical treatments are in the works. The question is, how will American doctors, insurers, patients, and others use the information? Research has already shown that digital mammography, for instance, is not cost-effective compared to plain old film mammograms, but because of the power of breast-cancer groups, insurers have been loath to tell patients, "no, sorry, not worth it." And when Britain uses such analyses to decide what its National Health Service will pay for, Americans go crazy. (This fear is the basis for the false claim in ads featuring former surgeon general C. Everett Koop that he, as a 93-year-old, would not be allowed to receive a pacemaker or joint replacement under the British system.) At least the Brits apply the guidelines about what a life is worth consistently.

Sharon Begley is NEWSWEEK's science editor and author of The Plastic Mind: New Science Reveals Our Extraordinary Potential to Transform Ourselvesand Train Your Mind, Change Your Brain: How a New Science Reveals Our Extraordinary Potential to Transform Ourselves.

Find this article at http://www.newsweek.com/id/233123

Respiratory Care Options For Children With Neuromuscular Weakness by Dr. Mary Schroth

This is a very important video to watch if you want to have a deeper understanding of the treatment and care of SMA or Spinal Muscular Atrophy. Dr. Schroth goes through all the modes of care from daily care to intubation and extubation, non invasive vs. tracheotomy, and more a must watch!! click on the link below to watch the video

http://nch.dataserv.tv/NCH/Viewer/Viewers/Viewer240TL3Banner.aspx?mode=Default&peid=7c4f6f16-f662-46d3-b323-d41ca2851670&playerType=Port25&mode=Default&shouldResize=true&pid=b309b7a4-eb87-404d-805a-90e73efbaafd&playerType=Port25#

When Talking Is Not Enough

When Talking Is Not Enough

By Maxine Fisher
Posted: Monday, February 1st, 2010

"Music can name the unnamable and communicate the unknowable." -Leonard Bernstein

Music therapy, as defined by the Canadian Association for Music Therapy, is the skilful use of music and musical elements by an accredited music therapist to promote, maintain and restore mental, physical, emotional and spiritual health. Music has nonverbal, creative, structural and emotional qualities. These are used in the therapeutic relationship to facilitate contact, interaction, self-awareness, learning, self-expression, communication and personal development.

Humans hear and experience sound for the first time in the womb when they hear the mother’s heartbeat, breath and muffled voice. From the moment of birth, loved ones' voices are recognized through the musicality of their speech.

Most people have been deeply moved by a piece of music during their lives. Songs can be used to create a retrospective road map of those lives. Think back to those songs from childhood, teens, 20s, 30s and onwards; humming a tune can bring back old memories while particular songs are often associated with a person or place. Music is universally accessible to individuals as well as groups of people, and to experience music, one does not have to have formal musical training. Music Therapists bring music to clients to enhance and improve their lives. Music heals, relieves tension and energizes. Singing and playing instruments in a safe environment with others brings joy and a sense of community and allows people to express their feelings. Song lyrics often describe inner thoughts and bring about discussions.

For many years, Music Therapy has been successfully used to assist people who struggle with physical and emotional issues. In one case, music helped a stroke survivor who was unable to speak with words. However, he was able to sing words to familiar songs and even say a few words following the song.

Often, people with dementia (including Alzheimer's) experience moments of joy and clarity when singing familiar songs. Family members, at times, have joined in song and expressed how relieved they were to see their loved ones having a break from confusion and observed how much more present they were. Using familiar songs, Music Therapy can “facilitate reality orientation and stimulate long and short term memory," says Music Therapy Association of British Columbia's Vice-President Kevin Kirkland.

Live music has a different impact than recorded music. Singing a familiar song directly to someone, making eye contact, observing their breathing and matching it with the rhythms in the song, all contribute to bringing that person back into the moment and participating in the basic human interaction of sharing music.

Many people say they cannot sing well or do not have a good voice. One woman, who could only whisper due to an operation on her vocal chords, came to the music group and sang along. Although her voice was barely audible, her joy and enthusiasm while singing was extraordinary. It is not how we sound to others, but how we feel when we sing that brings a song to life. It brings joy to others and us when we join in song.

Some residents always stay in their rooms and do not participate in senior facility events. For these people, a visit to their rooms with the introduction of songs often brings them great joy and inspires them to join the music group, socialize and have fun.

In independent and assisted living, seniors have said they look forward to the music groups. Participants are encouraged to request songs and the members choose from a variety of new and familiar songs. As time goes on, many participants in the group sessions show a strong desire to try instruments they may not have chosen to play during the first few sessions. Learning new skills keeps brains functioning at a higher level. Everyone wants to continue learning and experiencing the best that life can offer.

Music can be shared in many different environments. Home visits allow people to experience the joy of music in their own familiar environments, while being offered opportunities to make choices and express themselves through musical activities specifically designed for them. Some of the seniors I have worked with in their own home have experienced a loss, are dealing with an ongoing illness, have suffered a stroke or have the onset of dementia. Music Therapy sessions offer relief to family and caregivers when they see their loved ones participating in and enjoying music.

Maxine Fisher is accredited by The Canadian Association of Music Therapy and has been working with seniors, families and children for over 16 years.



FEBRUARY 2010 SENIOR LIVING MAGAZINE VANCOUVER ISLAND

Gwendolyn Strong Foundation Comes In 6th and Wins $100,000

"Never doubt that a small group of thoughtful, committed citizens can
change the world. Indeed, it's the only thing that has." -- Margaret
Mead

We did it! We really, truly did! Together, we accomplished the
monumental. Together, we raised a TON of awareness of SMA. Together,
we are having a material impact on curing it. Together, we are
changing this cruel disease -- FOREVER! Out of more than 500,000
charities, the Gwendolyn Strong Foundation, striving to END SMA,
received nearly 52,000 votes in 8 days and placed 6th -- sixth place!
For that honor, we'll receive a $100,000 grant from Chase which will
be used to accelerate awareness and research toward a cure.

Never before have we been part of something like this -- a true
grassroots movement. Something so much bigger than ourselves. Baring
witness to a change occurring -- a shift in what has been before. We
are humbled, moved, and motivated by the extraordinary that has
happened this week.

We are so proud of the amazing SMA community. The families who
virtually stopped all else in their lives. The parents who ferociously
fought and bore their souls to expose the agonizing reality of this
disease. And did so with painfully beautiful poignancy.

We are humbled by the extraordinary acts of kindness, the grassroots
activism, the tireless persevering, the enormous favors called in from
those both impacted by SMA and not, but who regardless took it up as
their passionate cause -- because the health of all babies is
important to all of us.

The things we witnessed people doing, the energy and creativity behind
each effort, the pure passion to prop the Gwendolyn Strong Foundation
and SMA on our collective shoulders and compete against these much
larger organizations to remain on the leaderboard -- person by person,
family by family, company by company, organization by organization,
ask by ask, email by email, tweet by tweet, vote by vote. Each of us
refused to relent. And we know we are only aware of a fraction of what
occurred in this movement. Take a look at this incredible list (click
here - http://bit.ly/7jioKN) of celebrities, athletes, musicians,
organizations, publications, blogs, etc. that threw their support
behind our cause. Humbling. Simply humbling.

The Gwendolyn Strong Foundation doesn't have a fancy national
headquarters or hundreds of chapters or a big marketing budget or a
team of employees. No, we're just two parents in a house in Santa
Barbara, California with two MacBook laptops caring for our terminally
ill two-year-old daughter and fighting any way we can to save her
life. But, in this Chase campaign the Gwendolyn Strong Foundation was
much, much more than the three of us. It was also two parents in a
house in New York. And three people in a house in Spain. And three
people in a house in Ohio. And 25 people at a company in Mississippi.
And one family in a house in Australia. And one person in a house in
Texas. And five people in a house in Arizona. And two people in a
house in the England. The list goes on and on. And together --
collectively -- UNITED -- relentlessly -- all of our amazing
individual efforts and our enormous unwavering passion amassed into
this wonderful grassroots effort.

We should all be so proud of ourselves. We have done something that
has never been done before. We always knew this Chase Community Giving
campaign was an incredible opportunity for SMA, but it has become so
much more than we could have ever imagined. We are so very honored to
have fought for this with you. Together, we have helped change the
future of this cruel disease.

-Bill Strong

Vote for a cure for SMA and Shira!!!!!

Brad Fisher
Dear family and friends on facebook. Please vote at http://www.voteforsma.comBy voting for the Gwendolyn Strong Foundation this charity will donate 100% of the $1,000,000 proceeds to SMA
research. This research could save our daughter Shira's life. It is free to vote and only takes a moment. ... Please pass this along to your friends. Thank you from the bottom of our hearts. Please paste this on your face book page and pass it along to all your friends. Thank you. Brad

IF

IF.....


IF you can keep your head when all about you
Are losing theirs and blaming it on you,
If you can trust yourself when all men doubt you,
But make allowance for their doubting too;
If you can wait and not be tired by waiting,
Or being lied about, don't deal in lies,
Or being hated, don't give way to hating,
And yet don't look too good, nor talk too wise:

If you can dream - and not make dreams your master;
If you can think - and not make thoughts your aim;
If you can meet with Triumph and Disaster
And treat those two impostors just the same;
If you can bear to hear the truth you've spoken
Twisted by knaves to make a trap for fools,
Or watch the things you gave your life to, broken,
And stoop and build 'em up with worn-out tools:

If you can make one heap of all your winnings
And risk it on one turn of pitch-and-toss,
And lose, and start again at your beginnings
And never breathe a word about your loss;
If you can force your heart and nerve and sinew
To serve your turn long after they are gone,
And so hold on when there is nothing in you
Except the Will which says to them: 'Hold on!'

If you can talk with crowds and keep your virtue,
' Or walk with Kings - nor lose the common touch,
if neither foes nor loving friends can hurt you,
If all men count with you, but none too much;
If you can fill the unforgiving minute
With sixty seconds' worth of distance run,
Yours is the Earth and everything that's in it,
And - which is more - you'll be a Man, my son!

by Rudyard Kipling

One of those weeks when you can feel yourself age

This week was another one of those weeks when you can feel yourself age. People look at you and say, “you look tired.” 2 weeks ago Shira started to get fevers off and on and by last Friday Shira was definitely sick. Last Saturday Shira crashed hard (lost her vital signs) from a mucous plug. There is always a fine line when working on a child in this type of emergency situation. These crashes are also known as extreme bradychardia. One minute you look at your child and they are smiling at you and the next minute there pupils are dilated and they are struggling with all their might to breath. If you don’t get your child breathing chances are by the time the emergency services show up at your door and start CPR or intubate your child they are going to have brain damage so I have made it a priority to become an expert at resuscitating Shira when in respiratory arrest.

I have heard enough stories and paid close enough attention to the bad experiences of other families with SMA Type 1 children to really put some effort into becoming proficient in resuscitating Shira. I haven’t had to give chest compressions to Shira because her heart has never stopped from these episodes but her saturation levels have reached into her 60’s for her heart rate and as low for her oxygen which is all very bad. You have to work fast and I have to say it’s terrifying watching your child slip away before your eyes. We have been to this place with Shira many many times and I know how she is going to die, I understand what it’s going to be like I just want to be there holding her in my arms. I don’t want to be away from her when this happens. When Shira was in respiratory arrest this time we worked on her hard and fast and there is a fine line as to how hard you can work on a child before you create other complications like vomiting. Unfortunately my over zealousness in this situation caused me to make Shira vomit which meant I had to quickly open her g-tube turn her on her side and deep suction her orally. So the poor kid was now in respiratory arrest and possibly aspirating vomit which can lead to bacterial pneumonia and serious complications and or damaged lungs and possible death. All us SMA parents are scared of the complications vomiting can bring. I stabilized Shira quite quickly but needless to say the poor girl was wiped out.

Shira’s saturation levels returned back to baseline of 100% for O2 but her heart rate was in the 160’s. We chose to stay home until morning so we spoke to the pediatric intensive care unit and told them we would hold off until morning and see how things go. The next day (I believe Sunday) Shira was on day 3 of fever and still had a very high heart rate. We didn’t want to chance the high heart rate was brought on by complications due to aspiration of vomit so we called the ambulance and headed into the PICU. The PICU was totally empty which is nice so we didn’t have to worry as much that Shira could contract other diseases. Shira was tested for Respiratory Synctal Virus (she had this at 6 months and 3 years of age and both times it was very very bad!) H1N1 and influenza A and B and all tests came back negative.

While in the PICU we also had a lot of blood work done on Shira to check how her diet was affecting her as well as her blood gases to check how well she was being ventilated.
It turns out Shira’s diet is spot on but she was over ventilated which was causing extremely low CO2 levels. We also have made arrangements to meet with an RT that will be bringing over a bunch of new bipap masks so we can replace the mask Shira has used since she was 6 months old.
While in the PICU we give Shira Chest Physio Therapy sessions every 4 hours or as needed around the clock; sleep deprivation torture won’t work on me anymore so if I get captured by extremists of some kind they will have to tickle me or something instead. While I hate to be in the intensive care unit with Shira I do enjoy the staff there and the support they give us is second to none! You get the full attention of 3 nurses and 1 doctor and there is no messing about it’s serious business in the PICU. We are very grateful to all of the staff and Dr. Jeff and Dr. Amanda (as Shira calls them). It’s a good feeling to have a team behind you that when things get at their ugliest you have a place to go and know Shira will be well taken care of. It’s amazing the work the PICU staff does on a daily basis, real life and death work!

So after 5 tiring days we are back home and thankful that our Sweet Pea is still with us, laughing, playing, singing. Shira teaches us how fragile life is and we give thanks for this life and time we have together as a family.

Sammy is doing well but the signs of stress are sometimes evident but all in all he is doing quite well. His teacher at school is an amazing person and very nurturing towards Sammy and what he has to live with and she gives him extra attention which we are so thankful for.

We are still downstairs as Sammy just has a nasty cough that isn’t going away and we just can’t take the chance that he might catch something else and spread it to Shira so we are down in Chez Shira for a while.

Shira is able to come off bipap now after his illness so today we went out for about an hour to get some fresh air and play with Sammy and some other kids at the park. “And that’s all I have to say about that.”