Harley
an Angelman
Thursday, 24 May 2012
Wednesday, 25 April 2012
Collin Farrell
Angelman Syndrome does not discriminate; it can affect any family. Collin Farrells son James has AS and Collin has talked openly about his support for a cure for Angelman Syndrome.
Check out some of the links below to watch Collin talk on various talkshows about Angelmans
http://www.youtube.com/watch?v=8ui4SzW22rc
http://www.youtube.com/watch?v=g1jRPIrrqh4
http://www.youtube.com/watch?v=TLtx2dj-FHw
Check out some of the links below to watch Collin talk on various talkshows about Angelmans
http://www.youtube.com/watch?v=8ui4SzW22rc
http://www.youtube.com/watch?v=g1jRPIrrqh4
http://www.youtube.com/watch?v=TLtx2dj-FHw
Monday, 23 April 2012
A small film from The night for the Angels
Check out the following link. Upstairs studios has made a very wonderful little film about The night for the Angels
http://upstairstudios.com/
http://upstairstudios.com/
Wednesday, 28 March 2012
Harley frangipani
Harley got stuck in the worst seat on our way home from byron. To make it worse mum had frangipani cuttings in the back of car. Hes didnt seems so bothered except jasmine and I couldn't stop laughing at him
Research Insights and Updates from FAST australia
I know a lot of you have been interested in the scientifics of discovering Angelman Syndrom and hopefully its cure in the future. I can not adequately explain this so I have attached an article from the FAST website and hope you will take the time to read it and other great articles on the page
http://www.cureangelman.org.au/content/2946
http://www.cureangelman.org.au/content/2946
By Edwin J. Weeber, Ph.D. (From FAST Australia newsletter, June 2010)
What makes AS research different?
A good analogy for understanding how hundreds of laboratories around the world conduct their respective research is the tale of the three blind men examining different parts of an elephant and trying to describe to others what they think it is. In many ways this is what we do in science; each researcher looks at a different aspect of the condition and tries to see how their findings fit in the larger context. However, even if you fill the room with blind men, no consensus will ever be reached if there is a lack of open communication between the observers. This is why collaboration, synergy, and sometimes serendipity are paramount for quick advancement of a field of study. In 1998 Dr. Arthur Beaudet developed a mouse model for AS. This represented an expensive endeavor both in time and money. Can you imagine the stifling consequence to scientific discovery and the progression of AS research if Dr. Beaudet had kept this mouse model solely for his own research? Instead these animals were openly shared with whoever wanted to investigate them. I believe the collaborative spirit among the community of AS researchers today is a direct result of Dr Beaudet’s decision to share his valuable resources with other colleagues. This spirit is evident in the authorship of many AS-related scientific papers that list scientists from multiple academic institutions world-wide. Today, the community of AS researchers has remained in close communication and is continually growing.
Where is AS research now?
There have been a number of major advancements in the past year. Use of the mouse model has revealed structural changes in the cells of the brain. Changes have been identified in mitochondria, the energy suppliers of cell, and in the spines of neurons where synapses are formed and maintained (1,2). These studies suggest that morphological changes at the subcellular level play a role in the overall change in synaptic function. In two independent studies, changes in synaptic strength are seen in a specific area of the sensory cortex (3,4). These changes indicate that the dysfunction in how synaptic connections are maintained and strengthened during sensory inputs is more wide-spread throughout the brain than was previously believed. This is supported by the discovery that the AS gene product appears to be absent throughout the brain in the AS mouse model. Recent genetic research has shown that another gene mutation in the TC4 gene located on chromosome 18 were found in a small number of patients clinically diagnosed with AS, but with no identifiable genetic alteration in UBE3A (5). These identifications are important for two basic reasons. First, individuals with a clinical, but not genetic diagnosis represent approximately 12 % of all individuals diagnosed with AS. Identification of additional genetic mutations also associated with AS symptomology will reduce this percentage of unknowns. Second, identification of genetic disruption that results in AS symptoms may shed light on molecular mechanisms and down-stream biological consequences of UBE3A maternal deficiency. Finally, a recent study identified several new targets of the AS gene product, Ube3a (6). One of these targets is a protein called Arc. This protein regulates receptors at the synapse and is known to be involved in the processes that underlie the strengthening of synaptic connections and memory formation. The culmination of the studies listed above, and those not discussed due to brevity, will serve to help shape ongoing and future research.
What is the future of Angelman Syndrome research?
This may be an opportune time to talk briefly about the terms “cure” and “therapeutic”. A cure is defined as a method or course of medical treatment used to restore health completely. In essence to make a person healthy as if no malady had ever occurred. For example, a “cure” in the context of AS may represent a medical intervention after birth, or in utero, that would be given before the onset of symptoms. The “cure” in this definition for individuals with a current diagnosis of AS is extremely doubtful. With that said, the development of a therapeutic, defined as a treatment of a disease or disorder, is reasonable. However, the efficacy of therapeutics tends to be only as good as their targets. Thus, a “magic bullet” treatment for all of the symptoms associated with AS is easy to imagine given the one gene etiology, but may be more difficult to realize. This should not discount that the development of therapeutics to treat specific aspects of AS have the potential to profoundly impact individuals with a current diagnosis of AS. The development of novel drugs is an expensive and risky endeavor, but clearly not an impossible task.
Are there new drugs on the horizon?
Conclusions?
The past year has seen impressive progress of AS-related research across multiple disciplines. Increased awareness of AS in the scientific community and high profile scientific publications will continue to attract more basic and clinical scientists. The recipe for this is simple and often under-appreciated. For example, there were nearly 2000 scientific publications associated with Arc research in the past 2 years. The study mentioned above will introduce a multitude of new scientists to AS and will likely persuade some of those researchers to include Angelman Syndrome in their respective research programs. As I mentioned earlier, scientific research is a painstaking and slow progression, but a progression nonetheless. There is a deliberate and relentless march forward toward a better understanding, and one day a treatment for, Angelman syndrome.
What makes AS research different?
A good analogy for understanding how hundreds of laboratories around the world conduct their respective research is the tale of the three blind men examining different parts of an elephant and trying to describe to others what they think it is. In many ways this is what we do in science; each researcher looks at a different aspect of the condition and tries to see how their findings fit in the larger context. However, even if you fill the room with blind men, no consensus will ever be reached if there is a lack of open communication between the observers. This is why collaboration, synergy, and sometimes serendipity are paramount for quick advancement of a field of study. In 1998 Dr. Arthur Beaudet developed a mouse model for AS. This represented an expensive endeavor both in time and money. Can you imagine the stifling consequence to scientific discovery and the progression of AS research if Dr. Beaudet had kept this mouse model solely for his own research? Instead these animals were openly shared with whoever wanted to investigate them. I believe the collaborative spirit among the community of AS researchers today is a direct result of Dr Beaudet’s decision to share his valuable resources with other colleagues. This spirit is evident in the authorship of many AS-related scientific papers that list scientists from multiple academic institutions world-wide. Today, the community of AS researchers has remained in close communication and is continually growing.
Where is AS research now?
There have been a number of major advancements in the past year. Use of the mouse model has revealed structural changes in the cells of the brain. Changes have been identified in mitochondria, the energy suppliers of cell, and in the spines of neurons where synapses are formed and maintained (1,2). These studies suggest that morphological changes at the subcellular level play a role in the overall change in synaptic function. In two independent studies, changes in synaptic strength are seen in a specific area of the sensory cortex (3,4). These changes indicate that the dysfunction in how synaptic connections are maintained and strengthened during sensory inputs is more wide-spread throughout the brain than was previously believed. This is supported by the discovery that the AS gene product appears to be absent throughout the brain in the AS mouse model. Recent genetic research has shown that another gene mutation in the TC4 gene located on chromosome 18 were found in a small number of patients clinically diagnosed with AS, but with no identifiable genetic alteration in UBE3A (5). These identifications are important for two basic reasons. First, individuals with a clinical, but not genetic diagnosis represent approximately 12 % of all individuals diagnosed with AS. Identification of additional genetic mutations also associated with AS symptomology will reduce this percentage of unknowns. Second, identification of genetic disruption that results in AS symptoms may shed light on molecular mechanisms and down-stream biological consequences of UBE3A maternal deficiency. Finally, a recent study identified several new targets of the AS gene product, Ube3a (6). One of these targets is a protein called Arc. This protein regulates receptors at the synapse and is known to be involved in the processes that underlie the strengthening of synaptic connections and memory formation. The culmination of the studies listed above, and those not discussed due to brevity, will serve to help shape ongoing and future research.
What is the future of Angelman Syndrome research?
This may be an opportune time to talk briefly about the terms “cure” and “therapeutic”. A cure is defined as a method or course of medical treatment used to restore health completely. In essence to make a person healthy as if no malady had ever occurred. For example, a “cure” in the context of AS may represent a medical intervention after birth, or in utero, that would be given before the onset of symptoms. The “cure” in this definition for individuals with a current diagnosis of AS is extremely doubtful. With that said, the development of a therapeutic, defined as a treatment of a disease or disorder, is reasonable. However, the efficacy of therapeutics tends to be only as good as their targets. Thus, a “magic bullet” treatment for all of the symptoms associated with AS is easy to imagine given the one gene etiology, but may be more difficult to realize. This should not discount that the development of therapeutics to treat specific aspects of AS have the potential to profoundly impact individuals with a current diagnosis of AS. The development of novel drugs is an expensive and risky endeavor, but clearly not an impossible task.
Are there new drugs on the horizon?
An interesting development in new drugs for AS is the investigation of a novel compound developed by Ardane Therapeutics (www.ardanetherapeutics.com). Their new drug called CN 2097 is currently in a Phase I feasibility study for the potential use as a therapeutic to treat the cognitive impairment in AS. This novel drug targets a sub-type of neuronal receptor that is well known to be involved in learning and memory. In fact, a mouse genetically designed to produce more of these receptors was found to be ‘smarter’ then genetically unaltered mice. This ‘smart’ mouse was termed “the Doogie Mouse” with deference to the 80’s TV child prodigy, Doogie Howser M.D. CN 2097 is shown to reduce the threshold for synaptic plasticity; a measurable defect found to exist in the AS mouse model. While these studies are in the first stages of drug development, it indicates that these types of drugs are becoming of interest to the business-minded pharmaceutical development companies.
The past year has seen impressive progress of AS-related research across multiple disciplines. Increased awareness of AS in the scientific community and high profile scientific publications will continue to attract more basic and clinical scientists. The recipe for this is simple and often under-appreciated. For example, there were nearly 2000 scientific publications associated with Arc research in the past 2 years. The study mentioned above will introduce a multitude of new scientists to AS and will likely persuade some of those researchers to include Angelman Syndrome in their respective research programs. As I mentioned earlier, scientific research is a painstaking and slow progression, but a progression nonetheless. There is a deliberate and relentless march forward toward a better understanding, and one day a treatment for, Angelman syndrome.
- Mardirossian, S., Rampon, C., Salvert, D., Fort, P., and Sarda, N. (2009) Exp Neurol 220, 341-348
- Su, H., Fan, W., Coskun, P. E., Vesa, J., Gold, J. A., Jiang, Y. H., Potluri, P., Procaccio, V., Acab, A., Weiss, J. H., Wallace, D. C., and Kimonis, V. E. (2009) Neurosci Lett
- Sato, M., and Stryker, M. P. Proc Natl Acad Sci U S A 107, 5611-5616
- Yashiro, K., Riday, T. T., Condon, K. H., Roberts, A. C., Bernardo, D. R., Prakash, R., Weinberg, R. J., Ehlers, M. D., and Philpot, B. D. (2009) Nat Neurosci 12, 777-783
- Takano, K., Lyons, M., Moyes, C., Jones, J., and Schwartz, C. Clin Genet
- Greer, P. L., Hanayama, R., Bloodgood, B. L., Mardinly, A. R., Lipton, D. M., Flavell, S. W., Kim, T. K., Griffith, E. C., Waldon, Z., Maehr, R., Ploegh, H. L., Chowdhury, S., Worley, P. F., Steen, J., and Greenberg, M. E. Cell 140, 704-716
The tampa twenty-four
http://www.firstgiving.com/fundraiser/lendahelpingwing/lendahelpingwing
The current article was copied from the above website link. It higlights that clinical trials are very much underway in the USA.
The current article was copied from the above website link. It higlights that clinical trials are very much underway in the USA.
Lend A Helping Wing
Fellow Angelman Community Members:
As you know, the Minocycline Clinical Trial is about to begin. Twenty-four children with Angelman Syndrome, now affectionately known as The Tampa Twenty-Four, have been selected to participate in this potentially life changing trial. Each family participating in the trial must travel to Tampa, Florida, USA three separate times, at their own expense, as a part of their commitment to the trial. While many of us applied to participate and were not selected, and many of us could not apply due to the expenses involved, we can all contribute to help defray the significant costs to the families who were selected and were willing to make the sacrifice for the good of the whole community.
While each family's costs will vary, our goal is to raise a minimum of $3,000 per family, or $72,000.00. $120,000 would be even better. This may sound ambitious; but it is the Angelman community, and their friends, family and loved ones, a formidable and supportive force, that have brought us all to this point in time. We can all participate in our own way in this groundbreaking trial, by making it possible - and less expensive - for those who will actually be taking part. These raised funds are in addition to the $1,000 provided to each family by FAST. One organization can only do so much: We, the Angelman community, through this grassroots effort, can do the rest!
Please give generously and ask those around you to give generously. This could literally be a historical moment in Angelman Syndrome history and YOU can help make it happen.
For more information, including disbursement schedules, details on fund management and other specifics, please click here.
Sincerely,
Marc Bissonnette
Dale Jackson Van Hal
Tony Vidray
As you know, the Minocycline Clinical Trial is about to begin. Twenty-four children with Angelman Syndrome, now affectionately known as The Tampa Twenty-Four, have been selected to participate in this potentially life changing trial. Each family participating in the trial must travel to Tampa, Florida, USA three separate times, at their own expense, as a part of their commitment to the trial. While many of us applied to participate and were not selected, and many of us could not apply due to the expenses involved, we can all contribute to help defray the significant costs to the families who were selected and were willing to make the sacrifice for the good of the whole community.
While each family's costs will vary, our goal is to raise a minimum of $3,000 per family, or $72,000.00. $120,000 would be even better. This may sound ambitious; but it is the Angelman community, and their friends, family and loved ones, a formidable and supportive force, that have brought us all to this point in time. We can all participate in our own way in this groundbreaking trial, by making it possible - and less expensive - for those who will actually be taking part. These raised funds are in addition to the $1,000 provided to each family by FAST. One organization can only do so much: We, the Angelman community, through this grassroots effort, can do the rest!
Please give generously and ask those around you to give generously. This could literally be a historical moment in Angelman Syndrome history and YOU can help make it happen.
For more information, including disbursement schedules, details on fund management and other specifics, please click here.
Sincerely,
Marc Bissonnette
Dale Jackson Van Hal
Tony Vidray
Monday, 26 March 2012
FAST- Foundation Angelman Syndrome therapeutics
Get Involved
FAST is focused on one goal: developing treatments for, and ultimately curing, Angelman Syndrome. Your involvement is critical in helping us reach this goal as quickly as possible. Whether you are looking to donate or fundraise, there are countless ways you can help us cure Angelman Syndrome. Below are just a few suggestions:Click on this link to find out all you can
http://www.cureangelman.org/
A night for the Angels
Last week I attended my first ever fundraiser benefit; it was not the kind of fundraiser hosted by the local RSL, where you win a meat raffle and a bottle of the finest chardonnay. It was a very classy event where you dressed up in fancy clothes, sipped lovely champagne and bet in the silent auction, and it was all for a very good and close cause to me, The Foundation Angelman Syndrome Therapeutics.
One of my closest friends accompanied me knowing that this was something she wanted to support not only for me but for my brother too. I was disappointed that my Mum didn’t want to attend but I can understand she is very guarded and sceptical when it comes to discussing progress in the world of Angelmans. Before attending the event I was extremely nervous. I did not know what to expect and I did not want to talk about Harley with these strangers. Harley is one of the oldest Angels I have come across in the community so far, and I didn’t want to answer a question truthfully in case it wasn’t what they wanted to hear. What if I broke the spirit an AS parent? I wanted them to still have hope but I didn’t want to provide false hope. How wrong could I have been? My perspective changed as soon as we arrived at Miramare Gardens.
I ran into my brother Rhys in the car park, I had forgotten he was going to do the photography for the event. As we walked into the lobby we were overwhelmed by the assortment of silent auction items. It wasn’t just a couple of out of date items but hundreds of great things ranging from cases of wine, to surf lessons, to holidays, paintings and even a gorgeous diamond ring. I turned to my friend “whatever you do don’t let me bet on anything”, she nodded in agreement. As we made our way through the throngs of gifts we spotted my vice, a brand new GHD. Well both being woman we decided that betting on one item was ok and well we’d get our money’s worth out of a GHD. We also purchased two cute yellow paper Angels, excitement boiling over when we discovered that our little Angels came with prizes and were not just the decorative wall features we thought they were.
As we were ushered to our dining chairs, I couldn’t believe that there were four hundred and seventeen people in this room; the most important being Michaela Townsend who organised this grand event. I also realised I was sitting at a table of people who had AS children. At first I didn’t let them know I had an AS brother but I listened as they spoke about their sons and daughters and then without nerves I admitted my older brother had AS. Instead of bombarding me with questions they did none of the sort, we obviously discussed AS but I discovered that I was being overactive before. We were all just trying to expand our knowledge and find a supporting community; it was great to be able to discuss with a group of people that understood you instead of trying to understand what you are going through.
Not a dry eye was seen during the formalities when a video played highlighting all the young angels in the community. My friend gripped my hand; I don’t think she expected this reaction from either of us. Then Meagan Cross was introduced to the stage. I had informally met Meagan through our cyber networks and it was great to see her. She gave a wonderful and hopeful speech about finding a cure for AS. In the USA AS therapeutics was on the forefront, they had found a cure for AS in mice and that human clinical trials were on the Horizon. Then Meagan spoke of her daughter with AS. It was a lovely illustration of her daughter who like Harley has so many difficulties and so many outstanding qualities. My friend gripped my hand a second time, as my eyes unforgiving gave me away again. I needed to meet Meagan in person.
I decided to keep a distance as I saw everyone wanted to talk with Meagan but finally she made her way to our table. I sat next to her and introduced myself and she knew instantly who I was. At the same time my brother had joined our table and we talked and talked and talked. I was slightly star struck, filled with hope that in Harley’s lifetime there might be a cure, or some kind of therapy. We talked about how we can get involved more in the community, my brother and I walking away with a new sense of purpose. I could tell you about all the other AS families I met but theres too much to tell and too many people to talk about.
Back to the auction, the event raised over $70,000. This far exceeded the generosity I thought people we were inclined to give. Two people bet $4500 on nothing, an extremely generous donation for something that does nothing and needs nothing. As the night the night concluded I had my new GHD under one arm (it was a lengthy battle but I beat out a man named Kevin to claim my prize) and under the other a bottle of won Vodka. I felt elated I never thought people would care so much about a relatively unknown syndrome but I was proved wrong.
Sunday, 8 January 2012
A night for the angels
For the last six months my blogs have been very infrequent as I was out exploring the world. I have now returned to Australia and will continue to post my ever lasting stories of Harley.
As for now, I would like you to all go to this website
http://www.anightfortheangels.org/
If you are in Sydney at the time, please buy a ticket and come along to the fundraiser and support families affected by Angelman syndrome.
As for now, I would like you to all go to this website
http://www.anightfortheangels.org/
If you are in Sydney at the time, please buy a ticket and come along to the fundraiser and support families affected by Angelman syndrome.
Wednesday, 7 September 2011
Been away
Its been a long time since I posted my last blog. This is mostly due to the fact that I am away from home right now travelling.
Its when I am away that I realise how much I don't miss home but I miss Harley. There are certain aspects of him I will never miss such as cleaning up nappies, showering him and trying to get him to grocery shop. However Harley always reminds me of home and the craziness of my life. I guess when I am away I don't have that full on-ness that comes with Harley- I don't know how to relax. I crazily miss the spitting and the cups being thrown at my head, I miss his outrageous laugh and his personality.
I always think about Harley and wonder does he understand where I've gone and what I'm up to. I wonder if he knows what he's missing. Then I think wouldn't it be great if he could on a holiday with me, but this is impossible as he is just too difficult to take on holidays let alone a plane.
My Mum is away at the moment with me too and I have been watching her struggle to adjust with out Harley around. For the first time in 34 years she is having a holiday away from Australia, away from work and away from Harley. For the first couple of days I watched her wake up and walk around my flat completely confused as to what to do with herself. She would make toast undisturbed, she could shower undisturbed, she could watch television undisturbed, she could even walk down the street without Harley dragging on her- a new concept to her. After a week of adjustment, she's coping fine though a little restless still.
I guess it's a good thing that my niece and her grandbaby was born this week, it will give her a whole different kind of nappy to change
Its when I am away that I realise how much I don't miss home but I miss Harley. There are certain aspects of him I will never miss such as cleaning up nappies, showering him and trying to get him to grocery shop. However Harley always reminds me of home and the craziness of my life. I guess when I am away I don't have that full on-ness that comes with Harley- I don't know how to relax. I crazily miss the spitting and the cups being thrown at my head, I miss his outrageous laugh and his personality.
I always think about Harley and wonder does he understand where I've gone and what I'm up to. I wonder if he knows what he's missing. Then I think wouldn't it be great if he could on a holiday with me, but this is impossible as he is just too difficult to take on holidays let alone a plane.
My Mum is away at the moment with me too and I have been watching her struggle to adjust with out Harley around. For the first time in 34 years she is having a holiday away from Australia, away from work and away from Harley. For the first couple of days I watched her wake up and walk around my flat completely confused as to what to do with herself. She would make toast undisturbed, she could shower undisturbed, she could watch television undisturbed, she could even walk down the street without Harley dragging on her- a new concept to her. After a week of adjustment, she's coping fine though a little restless still.
I guess it's a good thing that my niece and her grandbaby was born this week, it will give her a whole different kind of nappy to change
Harley wearing a smock by Lois
This is harley wearing the bib that Lois made for him. Thanks Lois I think these are a great idea!
Saturday, 23 July 2011
Harleys afternoon tea
Harley enjoying his yummy arvo tea at my parents restaurant. This is just before I got covered in coleslaw and red fanta.
Wednesday, 20 July 2011
lets spit on it
When Harley was younger he had a terrible spitting problem. He would spit to get your attention. He would spit to annoy you. He would spit just for the hell of it. Thankfully he grew out of this sociably unacceptable behaviour. Until now. Since having his wisdom teeth out he has developed the habit again, rising out of the depths of his glands with a vengeance.
We try to ignore it with the idea that he’ll grow tired and stop doing it. So far he’s not bored. He spits day and night. He spits at the dog. He spits at his carers. He spits at strangers. He spits at Mum and Dad. And he spits at me.
So today I snapped. Why not fight fire with fire? Or in his case saliva with saliva. I was standing in the kitchen and swung around face to face with Harley. In a clear instant and with no time to protect myself, he shoots a giant loogie into my eye. How he got me with such precision is commendable however the fact that it was my eye breaks that little bit of patience I have remaining. With the thick slobber dribbling from my lashes, I dig deep into my throat drawing out as much moisture as I can. Before he can run away I grab both his arms and hold him so we are once again face to face. I purse my lips and spit. It sails through the air in one complete blob and smacks into the side of his cheek. He looks at me stunned. I jut my chin at him and think victory is finally mine.
As I turned to walk away triumphantly, I hear the familiar pfft of his lips and feel the warm watery spray. Furious and defiant I turn and spit again. He retaliates. I retaliate. Him. Me. Him. Me. With Mum watching in the wings, no one is backing down. Back and forth it flies. He mocks me by laughing hysterically. Maybe he is laughing because he knows I won’t last longer than him. I start to weaken. I’m a rookie at this spitting business, Harley is a pro. The pro. I’m disgusted and defeated. I walk away, knowing I haven’t felt the last of this.
Now as I write this blog, Harley is perched around the corner waiting for me to emerge. Almost primordial he leans his head into my room and spits asserting his alpha dominance. He shuffles back toward the banister of the stairs, I know he waiting for me to walk underneath it so he has his chance at an aerial attack.
Wednesday, 29 June 2011
Dead rising
I have an overactive imagination. I often sit and concoct theories as to why Harley is the way he is. I know it’s genetic but is there a philosophical logic as to why he is the way he is?
I have concluded that Harley is a serial killer. Well maybe he was a serial killer in a past life. Now before you start judging me, I will enlighten you with evidence that Harley could be the new restoration of Ted Bundy, Charles Manson or even Aileen Mournos.
It all began several years ago when I spied Harley watching a horror film with my brothers. It was particularly gruesome and at the moments he should have been turning away and squirming like any sane person, Harley was laughing his head off. I didn’t think anything of it at the time.
A couple months later we sat down to watch Shaun of the Dead. The film begins and at the first bloody decapitation Harley is pissing himself laughing... Literally. After I clean him up and we resume watching the film, Harley is hysterical from woe to go. The bellowing laughter peaks at the sight of hanging limbs, slashing guts and through the climax of flesh eating to the tune of Queen. This has become Harley’s favourite movie of all time and he attended my 21st dress up party as a zombie.
His fascination with the macabre doesn’t stop there. The more B-grade and grisly the film the more he enjoys it. Dog Soldiers I believe is in the top 10 along with Planet Terror, Dawn of the Dead, Day of the Dead, and Resident Evil. He likes it when the film is a straight up gore fest and hates watching horror films that try to have a substantial story line. Forget the thrillers give him the slashers: Harley never turns away and watches with acute attentiveness for those cringe worthy moments. He rips apart laughing at the sight of some imagined creature ripping apart the body of its prey.
This is where my imagination takes over. I often see Harley trapped in his body. I imagine that he is a serial killer trapped in their and forced to see the world from a passengers perspective. Is he unable to talk and do anything for himself so that he is forced to experience love and compassion without being able to run away? Is it ironic that he is referred to as an Angel? Maybe this is his Karma. Or maybe it is just a genetic mutation. We all have our theories, mines just a little less romantic and a lot of far fetched.
Thursday, 16 June 2011
A message from Lois
This is a message from Lois who contacted me,
When my growing granddaughter, who has AS, couldn't be fitted with large enough bibs, I took out my sewing machine to solve the problem. I showed the resulting smock to friends, who loved it, and my project, Sophie's Smocks, named after my granddaughter, was born so that many kids with AS could have a free smock.
I buy new and gently used turtlenecks and trim. Supporters donate their time to cut and sew, and their money for postage and materials to keep the project ongoing.
All the smocks are FREE for children or older with AS. I make child size 6 to adult XL.
To get a smock, all you have to do is ask by emailing smocks@cox.net. Give me your child's shirt size plus a name and mailing address including zip. I'll send one as soon as I can.
There's no catch and no obligation.
Lois
When my growing granddaughter, who has AS, couldn't be fitted with large enough bibs, I took out my sewing machine to solve the problem. I showed the resulting smock to friends, who loved it, and my project, Sophie's Smocks, named after my granddaughter, was born so that many kids with AS could have a free smock.
I buy new and gently used turtlenecks and trim. Supporters donate their time to cut and sew, and their money for postage and materials to keep the project ongoing.
All the smocks are FREE for children or older with AS. I make child size 6 to adult XL.
To get a smock, all you have to do is ask by emailing smocks@cox.net. Give me your child's shirt size plus a name and mailing address including zip. I'll send one as soon as I can.
There's no catch and no obligation.
Lois
Wednesday, 15 June 2011
Stiring the pot
I was making pasta today for dinner. The first time in history Harley walks up to the stove and tries to stir the bolognese sauce, then he turns to me and tries to lick the spoon. I have never ever seen him do this and its really exciting to see him trying to do something different. I squealed with delight and luckily had my phone in hand to capture the moment. I know to some people this might not seem like a big deal, but it is to me. It gives me hope that he is understanding everything that is going on and trying to develop his abilities. Now if only he'd learn how to do the dishes and the world would be complete.
Sunday, 12 June 2011
Its all in the head
Last night I bounded out of bed at the speed of thousand gazelles. That slick feeling of dread rushing over me, adrenaline surging as I burst open Harleys bedroom door. He’s laying there just he had been when Mum tucked him into bed. He raises his head grumpily and looks me in the eye as I flick on his light. Thank god, I think to myself, He’s ok. I can read his thoughts and know he’s saying, piss off woman stop disturbing the little sleep I get.
When Harley was younger he had terrible epilepsy. I mean seizures every day, and they were horrifying. Epilepsy is very common among “Angels” and it is so complicated that it is often difficult to regulate. Anyone who’s ever witnessed an epileptic seizure will understand me when I say it is the hardest thing to ever sit through. And that’s all you can do. Sit. Watch and wait for them to come around. Be there for them when they are conscious and the pain hits. Thankfully Harley grew out of his epilepsy as he got older. Sporadically he’ll have a seizure but they are very few and far between.
I remember one evening I was brushing my teeth in the bathroom opposite Harleys room, I heard him. I thought he was dreaming or rolling around his bed. It sounded odd though. Unhuman. I decided on a whim to check on him. I pushed open his slightly ajar door and there he was gripped in the throes of a seizure. His back was arched and his hands twisted like that of an old tree root. I jumped on the bed; I knew I had to get him in the recovery position but how? I’d seen Mum do it before, it should have been easy. Instead panic took over and I sat there holding him. I screamed for Mum, at first I couldn’t get the words out and on the second attempt I didn’t even recognise my own voice. Mum and Dad run upstairs and Mum efficiently took over. I was eleven.
A few years later, I’m in bed and I hear an odd noise. I think nothing of it. About thirty seconds later I hear that panicked screech for Mum. I leap from my bed. There in Harley’s room, my brother is scrambling to roll Harley into the recovery position. I recognise that same fear and panic in his eyes. I get there before Mum and help roll him into position. Mum gets there, and then Dad and we all sit together. We sit and we watch. We watch the confusion cross Harleys face every time he thinks he’s coming out of it but then gets dragged back in. We hold his hand and we sooth him. We try to hold back the tears. Finally it stops. He cries and that’s the best sound in the world. Mum sits with him all night, while he cries from the headaches. No one sleeps that night.
For the next year I checked on him every night. At the slightest sound I would soar out of bed and run to his room. Nothing was ever wrong. Yet every night I’d go through the routine of checking on him. Eventually I learned that it was ok but every now and then I feel that same sense of panic and I just can’t help running to his room.
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