Where's Molly?

We have a few documentaries at home and Sammy is at an age where today he asked me if he could watch Where's Molly http://www.wheresmolly.net/ Years ago we were on the news advocating to get all the medical equipment we needed to keep Shira at home and care for her, get her injections to ward off illnesses etc. When we were on the news we had many calls from people that wanted to take Shira off of our hands and care for her. Yes caring for people is a business and when a person is extremely disabled the caregivers get even more money. This documentary came into my life a year after we started advocating hardcore for Shira and sometimes you just need something to grasp onto that justifies your actions and re affirms you are doing the right thing. This video is one of them. We realized watching this video that our "regular" son would be a better person having his sister at home. Today Sammy watched this movie and had a lot of questions he couldn't understand why a family would give up a child just because they had a disability. We are so proud of who our son is, that he doesn't categorize and separate people. Our hearts are constantly challenged by Shira's ongoing struggles and we'd be lying if we said we didn't suffer from moments of anticipatory grief . We live deeply in the present experiencing the joy our two amazing children give to each other. We've watched this documentary countless times and believe it is important to watch so that the confused can become enlightened and the knowing can be justified in their actions and life.

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.

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.

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!"

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

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#

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

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.”

CanAssist Swing-Enabling System

Shira Fisher loves the thrill that comes from soaring through the air on a swing.


The four-year-old girl first tasted the freedom of swinging when her dad rigged a special swing for her in the backyard of their Victoria-area home. But because Shira has Spinal Muscular Atrophy, a genetic disorder that has severely affected her muscular development, she has never been able to swing anywhere else.

Shira longed to be able to swing alongside other children at some of the numerous parks in her neighborhood. While there are swings on the market for children with disabilities, none worked for Shira and could be attached and removed from a standard park swing.

"Shira's father, Brad, had an idea of what he wanted," recalls Brandon Fry, an engineering co-op student at CanAssist who was assigned to work on the request. "He already had a supportive seat designed for kids with special needs. He asked us to modify it so it could be attached easily to any park swing."

Brandon attached a seatbelt-like system to the back of the store-bought Tumble Form Chair. The chair's firm foam material safely supports a child in a reclined position, an important consideration for Shira. Then Brandon added a metal back support with two metal rods that link at both ends to a swing's two chains.

When Shira and Brad met CanAssist staff at a local park, Shira was so anxious to begin swinging that she was almost in tears. Being four, patience is not a readily available virtue at this point. But once Brandon buckled her into the new device, Shira was humming softly and smiling brightly.

"She wanted to get on it pretty quickly," says Brandon. "And once she was in, she wanted to go higher and higher."

Apparently Shira is a real adventurer, much to the chagrin of her protective dad.

Shira stayed and swung while the CanAssist crew packed up to go. Finally able to swing just like other kids, she didn't want her first adventure in the park to end too soon.

"Some people wish they could fly, some people wish they could simply swing," Shira's dad wrote to CanAssist later. "CanAssist made my daughter's wish to be able to swing with other children in any park come true. Thank you CanAssist."


Brandon ensures the seat
attaches securely to the swing. Shira's father, Brad, gives
Shira a push.

Shira Driving Her Power Chair Despite The Cold