Shira and Maxine Singing

Disabled visitors left high and dry on area beaches

Disabled visitors left high and dry on area beaches

Brad Fisher pushes daughter Shira’s stroller-wheelchair up an uneven pathway from Willows Beach.

Emma Prestwich/News staff
By Emma Prestwich - Oak Bay News
Published: August 02, 2011 10:00 AM

Brad Fisher pants as he heaves his daughter Shira’s specialized stroller-wheelchair up the steep, uneven sand path from Willows Beach.
He enlists the help of caregiver Stephanie Davidson to pull the stroller up backwards, then pushes it the last few feet himself.
Several short stairwells can be found along the length of the beach, but the rough-hewn little trail is the family’s only access down to the sand, where six-year-old Shira, who has the genetic disorder spinal muscular atrophy, loves to play.
Fisher and his daughter have tried out many beaches in the region. Even with its bumpy
path, Willows is the only one that comes close to being friendly to Shira’s specialized wheelchair. It’s also ideal because of the hard-packed sand, rarely found on other shorelines.
Fisher is angry that public beaches are so difficult to access for people with disabilities, and doesn’t think adapting them would be hard.
“They might say ‘well, we could make a ramp there, but what are they going to do when they hit the sand?’” he said.
“You leave (manoeuvring a wheelchair on the beach) up to us, but at least we’ll be able to get down to the sand.”
Tamara Lohner’s daughter, Charlotte, has the same condition as Shira. Even though they live downtown, they usually head to Thetis Lake Park because it is the only lake with stroller-friendly access.
Visits to Willows in the past saw Lohner forced to carry Charlotte and leave the stroller behind.
“It’s hard; she’s like a really heavy, wet noodle,” Lohner said.
Having a full-time job and a son with Down syndrome leaves her little time to worry about beach access. “I just don’t think about it that much.”
There are currently no plans to improve accessibility at Willows Beach, said Oak Bay parks manager Lorne Middleton. He noted a low, sloping path leads to the water at the base of Estevan Avenue.
But Fisher said large logs washed up on the path during the winter, making it a two-person job to hoist Shira’s chair over the logs. Middleton said he wasn’t aware the path was blocked and would address the issue.
The Capital Regional District website (www.crd.bc.ca) lists three beaches with accessible elements: East Sooke Regional Park, Elk/Beaver Lake Park and Island View Beach. Only Beaver Lake has a path leading to the water, and has an accessible fishing float and boat launch.
There is also a ramp leading to the beach near Clover Point.
Many Capital Region municipalities consider accessibility when planning for parks and public spaces.
But it’s hit or miss when it comes to specific guidelines.
The City of Victoria doesn’t have any official policy relating to beach accessibility, said
Todd Stewardson, acting assistant director of parks.
“We’re not discouraging it, but we’re more focused on maintenance of the natural area,” he said.
Beaches are dealt with differently than parks, he said. Many factors come into play when adapting them, such as the specifications for the slope of a ramp and measuring its impact on the surrounding environment.
Saanich parks manager Rae Roer says Gyro Park beach has gentle trails that lead from the parking lot to the sand. He tries to ensure there’s always a clear route to the beach. But he also doesn’t want changes to “sanitize” the beach. “We tend to manage beaches in a fairly natural, wild, West Coast style,” he said.
Joanne Neubauer, president of the Action Committee of People with Disabilities, said improvements don’t have to ruin a beach’s natural charm. “There are so many ways to achieve accessibility that wouldn’t necessarily change the overall atmosphere of the park,” she said.
Many beaches, such as the inlet by Mount Douglas and the stretch along Dallas Road, have steep approaches and would be harder to adapt. But Neubauer, who uses a motorized wheelchair, said there are several that are fairly flat.
“I know there are geographic constraints, but there are other beaches where that’s not the case, and they still haven’t made any effort to make sure everyone can access the beach.”
She suggested a ramp could be cut out of the concrete walkway that runs alongside Willows Beach. “It doesn’t always involve rocket science.”

The Bridge Of Life by Brad Fisher

The Bridge Of Life by Brad Fisher
Lech Lecha. G-d said to Avraham, “Go (further) away-for your (own benefit)-from your land, your birth place and your father’s house, to the land which I will show you.(Parshas Lech Lecha)” I’m not the most religious Jew but I am spiritual and I identify deeply with my culture and heritage and religion. I’m the father and main caregiver to a 6 year old beautiful little girl who was only given 2 years to live. There are moments I deeply identify with Avraham being sent by G-d to an unknown land.

I just returned from a Canada Day celebration that we were not able to attend at the last minute because our daughter Shira lost her vital signs and went into respiratory arrest as I was parking at the event. It’s moments like these I feel I’ve been sent to an unknown land. I don’t have time to think of anything at times like this but work on our daughter and make her live. I suctioned the secretions from her mouth like mad with one hand while pulling the thick secretions out of her mouth and nose with my other hand. I was standing on the edge of the abyss again watching our beautiful little girl slip away into G-d’s hands as I desperately worked on her to keep her with us.

Our daughter suffers from the number one genetic killer of infants and toddlers called SMA Type 1 or Spinal Muscular Atrophy Type 1. 1 in 40 people carry the gene and one in 5000 babies are born with it. It is considered such a serious threat that it’s included in the updated version for the genetic screen for Ashkenazi Jews even though the rates aren’t higher for this population over the rest of the population. At 4 months of age Shira was diagnosed with SMA because we noticed she was not meeting regular physical developmental milestones. Days like today (reviving our daughter) are not uncommon and our lives are governed by the 24 our multi-disciplinary intensive care we deliver to our daughter Shira.

I’m crying as I write this, tearful, fearful, shaken to the core of my soul once again. I hear our daughter’s beautiful little voice echoing from the other room as she lays on the floor playing with her bubby. When Shira was diagnosed we were told, “There is nothing you can do just take your child home and love her until she dies.” In some ways this statement echoes true for all of us even those of us with regular healthy kids. We all take our children home and love them until they or we die don’t we. What a thing to say to a patient and her family. Shouldn’t the more appropriate response after diagnosing anyone with a terminal illness be we can’t cure your daughter of this disease but we can support you in caring for her and help you deliver a high quality of life to her? What drives doctors to be so HOPELESS? In our world we believe there is only endless HOPE not a hopeless end. There is also a big difference between delivering daily care to a person with a life threatening illness and just diagnosing them with an illness. Most doctors have never had to care for a sick human being they just advise other’s on what to do and I’m sure 50% of the time the advice is riddled with personal biases and no experience to base the advice on. We have only met one doctor who said, “I have no experience with this illness but together we will find out what to do, create a plan and implement it.” We have realized that doctors are people to and suffer from the same daily afflictions the rest of the population suffers from and so great care must be taken when prognosis and advice is given. You must be on your toes and advocate for your child at every moment, due diligence becomes second nature and conventional wisdom must be locked away.

When death is near and has been fended off again you can’t help but feel you are walking on the Bridge Of Life (Gesher Hachaim). It’s thought that the bridge of life is a bridge between past and future. When people ask me if I have received any gifts from my experience caring for our daughter or learned anything I respond with, “ I have never been so present, I don’t have time to think of the past and I certainly don’t think of the future because the future almost always does not include being with our beautiful daughter. Being completely present, in the moment, aware of our daughter’s breath, this is the gift!” “ (Tehillim 34:13) If someone wants to live (a true life), he must “love days” – love his days to the extent that they should not be lost, but remain preserved for him(Gesher Hachaim by Rabbi Tucazinsky).” Another soul shaking lesson I have learned is visit the sick. Compassion without Action is empty and one must act and actually do something that will benefit someone in need. Bikkur cholim or Visiting The Sick has become a passion of mine. Until our daughter was born I’m afraid I did not do my share of visiting the sick. If I could pass on any advice from our families experience it is to reach out, visit and help out those in need after all it’s a Mitzva (good deed). We have felt so lonely at times, abandoned, and left out. Yes illness is scary but you will find when you get to know these people, children, with life threatening illness you will increase the joy and remove a lot of the oye from your own life.

Another question we are often asked is what keeps you going? Why do you do this? Well we love our daughter and we would do anything for her is our reply. The second answer I give is a quote from a Hasidic Sefer (from a book titled Wrestling With The Angel Published by Schoken) I read when our daughter was about a year old that gave us comfort, peace and hope. “How will we recognize those we loved when we meet them after 120 years in the world-to-come? If they died young, will they have grown old? If they were hurt or wounded, will they have healed? How will we know them, how will they know us if we have changed or aged? The answer is that we will know them, we will recognize them because they will be clothed and cloaked in the mitzvahs we do in their name.”

Three new screening tests for diseases available as Jewish Genetic Disease Consortium trains more rabbis, reaches out to intermarried.

Published on The Jewish Week (http://www.thejewishweek.com)

Home > ‘It’s Not Just Tay-Sachs’


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‘It’s Not Just Tay-Sachs’
Three new screening tests for diseases available as Jewish Genetic
Disease Consortium trains more rabbis, reaches out to intermarried.

Amy Spiro

Editorial Assistant

Tuesday, May 17, 2011




Brad and Maxine Fisher with son Sam, 8 and daughter Shira, 5, who suffers from Spinal Muscular Atrophy.



When Shira Fisher was just 4 months old, her parents already knew that something was wrong. She had frequent problems with choking and serious “physical developmental delays,” said Brad Fisher, Shira’s father and full-time caretaker.

Her parents brought her to several different doctors, before she was finally diagnosed (with the help of Brad’s Internet research) with Spinal Muscular Atrophy, Type 1. SMA affects the motor neurons that control voluntary muscle activities, like walking and swallowing, and causes their degeneration. Cognitive abilities are generally not affected.

At the time of Shira’s diagnosis, “we’d never heard of it,” said Fisher, who was told by doctors that Shira had little chance of surviving past the age of 2, and that he — and mom Maxine and big brother Sam — should “take her home and love her.” The family refused to give up, pursuing experimental treatment and therapies, and Shira will turn 6 next month.

The Fishers did no genetic screening before getting married or having children. “It wasn’t on our radar,” Brad said. But today SMA is one of three new diseases recently added by the Jewish Genetic Disease Consortium to a growing list of conditions for which Jews should be genetically tested.

The JGDC now recommends that any couple in which one member has an Ashkenazic Jewish grandparent be tested for 19 separate conditions, up from 16 last year. When the organization was launched six years ago (though Jews had been tested for years before it began), it recommended testing for 11 disorders. Over the past six years, that number has grown as new screenings and tests became available. New to the list are SMA, Joubert Syndrome Type 2 and Walker-Warburg Syndrome. These diseases are not newly discovered, or recently linked to the Ashkenazic population. But since screenings for the genetic mutation for all three only became widely available this year, the JGDC is now cautioning Jews and others to take advantage of the tests.

While the medical advisory board “has known about these diseases” for a while, said Randy Yudenfriend-Glaser, chair of the JGDC, “unless you can screen, there’s nothing to do.” She also noted that couples who have been screened in the past, and are planning to have more children, should get re-tested for these disorders. And she wouldn’t rule out the list of recommended tests growing in size. “It’s never going to shrink because the diseases don’t go away,” she said. “People think we’ve wiped Tay-Sachs out of the population, but we haven’t; we’ve tested it out.” Carriers of the disease are still common in the population. The medical advisory board of the JGDC bases its decisions “on carriage rate, but also on the severity of disease,” said Yudenfriend-Glaser.

Randi Chapnik Myers knows firsthand just how devastating Walker-Warburg Syndrome can be. She and her husband were screened for Tay-Sachs before having children. When their first pregnancy, in 1995, ended after doctors said the baby would not survive beyond birth, they thought it was a fluke. And after having two healthy children, the Toronto family never imagined the problems that would ensue. Chapnik Myers lost twin babies in 2000, and then a third the next year. Doctors made a diagnosis of Walker-Warburg in two of the three fetuses. “Not only had I never heard of it,” said Chapnik Myers, “I was told after my first loss that it wasn't genetic.”

She hopes that the new availability of screenings can help other women avoid her pain. “Having to wait until 20 weeks to be diagnosed, having a formed fetus growing inside of you, a human being, and moving in to maternity clothes and planning for a child” was incredibly difficult, said Chapnik Myers. “Going through seven deliveries for three children [the third child was born after all of the losses] was a strain emotionally and very physically.”

Since 2005, the JGDC has been reaching out to families, through its community outreach program, and to physicians, with its Medical Grand Rounds Program, hoping to spread awareness of the need for genetic testing in the Ashkenazic Jewish community. Last fall, as reported in this paper, the JGDC launched its Rabbi Education Program, aimed at urging rabbis to discuss genetic screening with couples during pre-marital counseling.

“[It] was the missing link which really rounds out our program,” said Yudenfriend-Glaser. To date more than 150 rabbis across the tri-state area have attended a seminar run by JGDC, and signed a “rabbi pledge,” promising to discuss genetic diseases with young couples.

Recent training events include sessions in Buffalo and Monroe, N.Y., as well as at the Jewish Theological Seminary earlier this month. JGDC is working on developing an online system for rabbis to be trained. Those who already took part in the sessions will receive updated information about the newly added diseases.

The JGDC’s goal is to combat the major misconceptions about Jewish genetic diseases — one of which is that intermarried couples don’t need testing. To that end they are also developing means to reach interfaith couples to raise their awareness.

One of the messages the JGDC deems most important is, “It’s not just Tay-Sachs,” said Yudenfriend-Glaser. Even as she tours synagogues and Jewish centers today, Yudenfriend-Glaser encounters people who are unaware of the dire necessity for genetic testing. She met with a rabbi this year “and he told me, ‘we don’t have any of that in our lineage.’ I did a double take,” she said. “You hear this from educated people; they really don’t realize how much is out there.”

Most importantly, she said, “people need to be advocates for themselves. They should go in armed with a list of the tests and say this is what I want done.”

Read more:

Healthcare
Healthcare
Copyright 2010 The Jewish Week


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Source URL (retrieved on 05/17/2011 - 21:15): http://www.thejewishweek.com/special_sections/healthcare/its_not_just_tay_sachs





Shira's Web Site: http://www.asonginthisworld.com

Shira's Videos: http://www.youtube.com/Shira2



"This book is dedicated to the health care organizations that not only raise money for research to seek cures for neeruomuscular diseases but also train and encourage health care professionals to provide the high-quality care necessary to prevent mortality while cures are being sought." - Dr. John Bach (From the dedication in Dr. John Bach's book Management of Patients with Neruomuscular Disease)

"Non intervention in fatal illness becomes a self fulfilling prophecy." - Dr. John Bach

"The phrase is apt to cause disquiet. There have been those among us who have arrogantly judged, from a vantage point of power, the value of a human life. They have made decisions based on their assessment of a person's quality of life about providing supports to sustain that life. This attitude peaked in Nazi Germany, where such decisions were used as the basis for genocide. We like to think that we have moved well beyond this perspective, but important decisions about people's lives are still being made from positions of power. Such practice is difficult to combat, especially in a period when responsibility of government in the area of human and environmental services is being cut back."- Introduction by J. David Baker (Quality of Life in Health Promotion and Rehabilitation)

"The creative spirit is not indestructible, but a courageous few discover that when in hell, they are granted a glimpse of heaven."-Anthony Storr

"The opposite of Compassion is Indifference." - Jean S. Bolen MD

"He who preserves one soul is considered as if he had preserved a whole world." (Talmud, Sanhedrin, 37A)

"To serve, to strive and not to yield." - Outward Bound

"Those of us who deal in science, even the most enlightened of us, have a strong and objectionable tendency to hubris. Hubris for scientists comes from an inadequate knowledge and appreciation of the past. Discoveries are thus made and claimed that are really rediscovered - not new advances at all, but history lessons. I have to concede priority to people who came before me. Rediscovery is every bit as good as discovery, If what is rediscovered is important and was forgotten. It is better still when the rediscovered information has the capacity to improve the lives of those around us."-From the book THE SECOND BRAIN by Michael D. Gershon, M.D.

"Throughout human history, as our species has faced the frightening, terrorizing fact that we do not know who we are, or where we are going in this ocean of chaos, it has been the authorities, the political, the religious, the educational authorities who attempted to comfort us by giving us order, rules, regulations, informing, forming in our minds their view of reality. To think for yourself you must question authority and learn how to put yourself in a state of vulnerable, open-mindedness; chaotic, confused, vulnerability to inform yourself. Think for yourself. Question authority."
-Timothy Leary

"Palliative care works with - not instead of - other treatments. It can start as soon as the family knows the child is ill. Palliative care does not mean "giving up." Good palliative care can help all seriously ill or injured children, not only those who are dying." -
When Children Die: Improving Palliative and End-of-Life Care for Children and Their Families

"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

Shira Fisher, honorary Rink Of Dreams captain

Shira Fisher,
honorary Rink Of Dreams captain,
invites one and all to the
“Rock and Roll Rink Of Dreams” Family Tailgate Party!!

Saturday, March 19th
11am – 3pm
Bear Mountain Arena Parking Zone
The Event

Telus is setting the stage for a live rock band concert in a massive heated tent.

Jive with your family to the rhythm of:
Invasion 62
The Wharf Street Band, and
That 70’s Band!!

There will be also be tasty food stands, cool drinks, steaming hot chocolate, mascots, Telus Kinect playstations and playzones, and more!!!

Entrance is by donation only. All proceeds are for kids of Vancouver Island who - just like our honorary captain - face life-threatening conditions.

It’s all part of the 24 hour Rink Of Dreams
(March 18/19 at the Bear Mountain Arena) in support of the Help Fill A Dream Foundation.

For more information please visit:
www.rink-of-dreams.com
www.helpfilladream.com
www.asonginthisworld.com

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.

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/

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#

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

Death, not disability, is the end of the world

I've been up since 5:30 this morning because the roof on our back porch has almost been torn off by the high winds here. In between going outside in the freezing cold and placing 10 lb weights on the roof to hold it down I've been doing a little research into quality of life and came upon this article. I have to say after I saw Million Dollar Baby I was left with a bad taste in my mouth. Because the movie was so well received without any critic about disability and quality of life shows what the general public thinks about the disabled. It's the old NIMBY or Not In My Back Yard approach to life. If I don't see it it's not my problem. Well we were a normal family before our daughter was born with a life threatening disability so I guess now were not normal. But as Groucho Marx said, "I don't want to join any club that would have me as a member!"

Death, not disability, is the end of the world
CBC News Viewpoint | February 03, 2005 | More from Disability Matters

This column will feature three writers, each with a different disability. They all have something to say about living with a disability and how they view awareness and attitudes toward disabilities in Canada. The column will deal with the rights of people with disabilities, eliminating inequality and discrimination, and issues of self-help and consumer advocacy. Our plan is to rotate among our columnists to have a new column each month.
Ed Smith Ed Smith is a retired educator and full-time writer. His humour column runs in several papers and magazines and he has had eight books published. He has been quadriplegic since 1998. Ed lives in Springdale, Nfld.


Clint Eastwood just lost me as a fan, something I'm sure will keep him awake nights.

His latest movie, Million Dollar Baby, has won praise from everyone who's seen it, and perhaps a few who haven't. As a person with quadriplegia I see it as nothing more or less than a scurrilous attack on people with spinal cord injury specifically, and those with disabilities generally.

A couple of years ago I gave a keynote presentation to a conference on disabilities. It was meant to be an upbeat and "go get 'em" type speech and from the standing ovation at the end it seemed I had succeeded admirably. Less than an hour later one of the delegates to the conference (we'll call him Jack) button-holed me in the hotel lobby. He looked me up and down and then spoke in confidential tones.

"When I see you now," he said, "and remember what you used to be like, I think 'twould be better if you were dead."

Jack and Clint would have hit it off well. Million Dollar Baby, which Eastwood both directs and stars in, is the story of a fight manager with a promising young boxer. The fighter gets a spinal cord injury in a fall and at her request the manager (Eastwood) kills her as she lies in a nursing home. The film will likely win all kinds of awards.

Not from me, even if I had them to give. Eastwood has hardly been a friend of people with disabilities. He was sued in 1997 for refusing to include $7,000 worth of accessible bathrooms in his $6.7-million resort renovations. Caring chap, Clint.

So it's what the boxer wanted, right? It's what I wanted, too, when I discovered I was paralysed in almost 90 per cent of my body. I pleaded with my wife to have me shot or put down in some merciful fashion. At the time, I didn't even care if it was merciful. That was for the first two days. Now, six years later, I'm rather glad she didn't.

Actor Christopher Reeve had a similar experience. So did many people I know who have suffered from catastrophic injury.

Incredibly, a preponderance of the population, even in our "enlightened" Canadian society, agrees with Jack that we're better off dead.

Some years ago I did a short editorial segment for CBC radio in which I defended Robert Latimer's second-degree murder conviction for having taken the life of his multiple handicapped daughter Tracy. I disagreed strongly with those who wanted him pardoned and set free. The producers told me later that calls and e-mails were running five to one against my position.

It's no fun being quadriplegic. We have to battle attitudes that believe us to be incapable of speaking for ourselves, unworthy of any accommodation of our physical needs, and something less than "normal." We wait in the snow or the rain outside hotel and restaurant doors that don't have automatic openers, hoping some kindly soul will see us and come running before we perish. We endure while waitresses and store clerks talk over our heads to our spouses or caregivers about what kind of pie or size shirt we want.

We get stuck in cubbyholes in the back of theatres and movie houses. I have almost been physically attacked while asking an able-bodied motorist to remove his vehicle from a handicap parking space so we could get close to an entrance.

While I was in a rehabilitation centre in Toronto my wife called several churches to see if they were accessible. Perhaps we chose the wrong churches or the wrong denominations or the wrong religions. She didn't find one that wouldn't have put me at the front of the church to be stared at, or in the middle of an aisle to be an obstruction.

Despite all this, the vast majority of spinal cord injured persons have a good quality of life. We have family and friends, hobbies and pursuits, occupations and pleasures. Life has changed irrevocably, but it has not become a hell so tormenting that we'd be better off dead. On good days it's downright bearable!

Million Dollar Baby sees no opportunity for any quality of life with spinal cord injury. Its premise is that unless you can walk and/or use your hands your life has no value.

I know several people with quadriplegia who swear up and down that they are really happy. Some of them have even said they're glad they had the accident because it changed their lives for the better. A friend who is also quadriplegic assured me not long ago that he didn't have a complaint in the world.

Although we all admired Christopher Reeve for many reasons, some of us had problems with his obsession that the only goal in life worth pursuing is to walk again. That just isn't going to happen for many of us so we get on with the task of making a contribution to our community in whatever way we can, and being as happy as the next person while doing it.

Being physically disabled is no picnic, God knows. But, Clint and Jack, it isn't the end of the world.

Beckman Oral Motor Therapy-Sometimes It All Comes Together

A few months ago I discovered a new oral therapy while reading through posts on http://www.smaspace.com The therapy is called Beckman Oral Therapy http://www.beckmanoralmotor.com/. Because SMA Type 1 patients get jaw contractures and have very limited range of motion in their jaws I have always been concerned about what the future holds for Shira's ability to keep speaking. I knew I had to take some kind of action before it was too late and this video appeared http://smaspace.ning.com/video/romansspeech-therapy-oral Today the head speech pathologist came over to improve the techniques I had learned by watching the video. I will post a video soon as well as the instructions I received from Phil Harmuth on Beckman Oral Therapy. I can't say enough about Shira's progress from our daily Beckman routine. Shira's jaw has more movement than ever before and she can actually seperate her upper and lower jaws. If you aren't already performing oral therapy on your child I strongly suggest doing some form of oral therapy to help prevent contractures and keep some range of motion in the jaw. This therapy also has relieved a lot of pressure in Shira's face. Sometimes everything just comes together and this is one of those days. Thanks Phil Harmuth and the Queen Alexandra Centre!

2 Great Books By Christopher Reeve--Advocacy

"So many of our dreams at first seem impossible, then they seem improbable, and then, when we summon the will, they soon become inevitable. If we can conquer outer space, we should be able to conquer inner space too--the frontier of the brain, the central nervous system, and all the afflictions of the body that destroy so many lives and rob our country of so much potential."

While Christopher Reeve did not suffer from a degenerative disease his catastrophic accident which left him a quadriplegic and vent dependent changed his life forever. Christopher Reeve's insight into living with paralyzes and how it effects your life is profound. Christopher wrote 2 books and I strongly suggest everyone read them as they give great information on family dynamics, hope, and the books are heavily weighted on advocacy. Without Chistopher Reeve stem cell research wouldn't be where it is today as he really increased funding for stem cell research as well as political will to a whole new level while he was alive creating the environment or stepping stone for scientists today.

Christopher Reeve "Still Me": This book deals with Christopher before and after his accident. This is his first book and obviously an inspirational story of his entire life. You get an eyeful of his child hood through adult hood, his injury, emotional and physical trauma and how he rebounded into one of the most effective advocates for the disabled in the United States ever!!

Christopher Reeve "Nothing Is Impossible": This is a must read for us advocates!!! The book is not deep but it goes into Christopher's thought process's one goes through after a catastrophic diagnosis or injury:
1) The First Decision
2) Humor
3) Mind/Body
4) Parenting
5) Religion
6) Advocacy
7) Faith
8) Hope
Christopher Reeve was an avid sailor and the lighthouse does not have religious meaning here. Here is an excerpt from his book:

"When the unthinkable happens the lighthouse is hope. ONce we find it, we must cling to it with absolute determination, much as our crew did when we saw the light of Gibb's Hill that October afternoon. Hope must be as real, and built on the same solid foundation, as a lighthouse; in that way it is different from optimism or wishful thinking. When we have hope, we discover powers within ourselves we may have never known---- the power to make sacrifices, to endure, to heal, everything is possible. We are all on this sea together. But the lighthouse is always there, ready to show us the way home."

4 year-old Shira Fisher is terminally ill in the news

CLICK ON ME TO WATCH VIDEO OF NEWS CAST

VICTORIA - 4 year-old Shira Fisher is terminally ill with Spinal Muscular Atrophy or SMA. Her parents were told she had months to live, when Shira was first diagnosed four years ago. But the young girl continues to defy the odds.

Her parents, Brad and Maxine, have become advocates for Shira and other SMA kids. Last week, they were told their application to get Shira a vaccine to fight out RSV (a virus that attacks the respiratory sytem) was denied. Shira has twice beeen hospitalized with RSV and both times nearly died.

Her parents believe the vaccine can keep Shira out of the intensive care unit this winter. The Fishers contacted A News and within 48 hours, BC Children's Hospital announced its vaccine protocol for the province would include children with SMA who are under 15 kilograms. Shira now qualifies and was given her first set of anti-RSV shots today.

The Fishers believe Shira's original rejection was over money - the vaccine costs about $8,000 and they worry about other families with special needs children won't fight the system to ensure quality care.

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