Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Síofra passed away at home 2 months ago, almost 6 years after receiving her diagnosis of ASPS; an amazing little girl who grew into a woman on her journey with this disease. I have no doubt but she is an angel now.

She had a 7cm primary tumour in her left calf with lung mets across both lungs upon presentation in August 2008 (we were told the lung mets were non-resectable). Following excision of the primary tumour and the muscle in which the tumour was growing, in 2008/2009 Síofs had traditional chemo (IVAD and ICE alternating cycles) and subsequent radiotherapy - the disease remained stable for some time, but we read now that it may have done so anyway as ASPS is an indolent cancer, and therefore the year of gruelling chemo and recovery she endured at 16/17yrs of age may have been of no benefit to her - who knows..............?

In July 2012 a routine scan showed that the lung mets had been growing and she started Sutent in September that year (with no great difficulties once she adapted to a handful of side effects she described as "nothing" when compared with previous treatments). Then in March 2013 we found out from reading this website that her lung mets may in fact, be operable outside Ireland, contrary to everything we had been told in Ireland (on many occasions by many different medical professionals when expressing a willingness to travel and ability to privately fund/fundraise for whatever might be available and have proven efficacy). Oh the excitement! We headed to London for an appointment at the Royal Marsden a few weeks later, but to our dismay we discovered from CT scans there, that the disease had, by that time, spread beyond the lungs into the chest cavity and cervical lymph glands so we had missed the window for curative resection, a window that likely had been open for many years after diagnosis - but we had been given incorrect, or to call it by its given name, "protocol" information only. If only I had found your website sooner - or been less trusting as to the completeness of the information provided to us by the medical teams here. If only I had kept up the manic search for information, even when her disease remained stable......

On the advice of her team, Síofra remained on Sutent following this progression, and we were told subsequently that her condition improved significantly - she had CT scans in December 2013 that showed "dramatic shrinkage" with some mets no longer being visible on the scans. We were overjoyed!

But not for long: As we all know, the course of ASPS can be so erratic..................

In March this year (on the day of her engagement), Siofra, for the first time, complained of a pain in her back (and complaining was not usual so we all took heed). Following 3 weeks of being humoured with laxatives, ultrasounds, x-rays for "injuries" and a suggestion that maybe she was suffering from "anxiety", in April, in her routine CT, her team discovered bone mets. Subsequent radiotherapy and trabectedin did little to mitigate progression.

Nonetheless, thanks to an unfaltering positive outlook, immediately following her course of radiotherapy in May, Síofra enjoyed 8 fabulous nights in Las Vegas with her fiancé, and thereafter, thanks to an amazing palliative team in our home town, she had the smoothest journey possible to her untimely end, at home among her loved ones, virtually pain-free.

If only there was a way that every patient could ascertain, upon diagnosis, the full extent of their options all over the world and not be herded in panic into a protocol treatment that can do more harm than good, while missing out on knowing about what is available elsewhere and has worked for many patients.........

Here's hoping your website will do that for many people. xxx

Hello, welcome to our forum. It is so sad to hear that one more young and beautiful life was lost to ASPS. I am very sorry about your loss and I feel your pain. it is very comforting to hear that the palliative team in your town managed to keep her virtually pain free toward the end of her journey.
Also thank you for sharing Síofra story with us, it is very important to us to collect all the information possible in hope to help to some other ASPS patient to make a right decision and working as a community find an optimal path to manage this rare disease. As I understand the bone mets she developed were in her spine? It reinforces our recommendation for all ASPS patient to have a bone scan once a year. It is often a case that the treating oncologists do not request all the scans we recommend unless the patients insist on it. Also the very important piece of information is the increased response to continued Sutent intake after initial progression on it and heterogeneity of this response - some mets shrinking and some are appearing and growing at the same time, we already had some signs of this effect in other patients but this is all very vague and every single report adds its bit to a bigger picture.

Síofra passed away at home 2 months ago, almost 6 years after receiving her diagnosis of ASPS; an amazing little girl who grew into a woman on her journey with this disease. I have no doubt but she is an angel now.

I am so very sorry to hear, as Olga so graciously stated, that another young person has been taken by ASPS. I am so sorry for your loss.
I get the feeling by reading your story of Siofra's life and experiences , that Soifra got her angel tendencies from you.

You are truly an angel to rise above the loss and ,as I can only imagine as , the incredible sadness to share with us in detail of Soifra's journey from the beginning of ASPS to the end and more.

Never question whether you could of done things differently. Hind site takes away from the good memories and the effort isn't worth it. It sure sounds to me that she had your tremendously unconditional love and that in itself I am sure was the world to her. That gift was large.

Thank you so much for all your input. It is priceless to us all
All our condolence's to you and your family,

Much love
The Josh P family
Friends forever*

Dear Siofra's loving mother,
I am so profoundly sorry and saddened about, and so deeply share the immense pain and grief of your beloved daughter Siofra's devastating loss. It was so gracious of you to find the emotional strength and energy to write to share the information about Siofra's courageous battle with this insidious disease which has taken so many precious young Lives. As Olga has said, the information which you so thoughtfully provided will be invaluable in helping to guide those of us who continue to fight ASPS. It sounds like you did everything possible to help Siofra fight her increasingly challenging battle, and all that love could do was certainly done by you. Because there are no definitive answers for treating this extremely rare and poorly understood disease, all that we as parents can do is what you did which is to try to make the best treatment decisions possible based on what little and very limited information is available. I Hope that everyone who reads your thoughtfully shared story about dear Siofra's ASPS journey will heed your wise warning and heartbreakingly learned knowledge and insights about the need to be "less trusting as to the completeness of the information provided to us by the medical teams" as unfortunately, doctors and radiologists can and do make mistakes and they don't always have the most up to date treatment information available, nor do they always make the best treatment recommendations for ASPS. This is why I and other Board members continue to so strongly encourage the active participation and sharing of anecdotal treatment information by all ASPS patients/family members because that information is truly one of our strongest weapons in fighting this very challenging, little known, and unpredictable disease, and the information which you have so generously shared is invaluable.
I am grateful that your precious Siofra was able to receive excellent palliative care that controlled her pain and allowed her to be at Home surrounded by the comforting warmth and love of family during the final part of her difficult ASPS journey. Please know that she, you, and your family are being held very close in my aching heart, and feel the embrace of my gentle hugs, my deepest shared sorrow and sympathy, and my love from one mother to another. Bonni Hess, mother of now 32 year old Brittany, diagnosed at age 19 in July 2001

All,

Thanks so much for your kind words. Is there a facilitator that gathers medical information?

if so, there are some additional twists and turns on her journey that I would be happy to share by PM and that could, in turn, be shared with the larger group on an anonymous basis.

Yes, Siofra's bone mets were in 2 different vertebrae and in her scapula. Thankfully she didn't suffer spinal cord compression at any time when she would have been aware of her condition, and she was fully aware and alert when awake, up until a day or two before she passed.

Eleanor

Eleanor, I will be glad if you share the additional details of Siofra clinical story with me PM using messaging service of this board - just click on my name and send me a PM - and then I'll figure out how to post them, or alternatively you can post the detailed story in the Anonymous Patient Updates http://www.cureasps.org/forum/viewforum.php?f=60
thank you again for your offer of the additional information, it may help down the road to someone with ASPS tremendously in their decision making and treatment planning.

Dear Eleanor,
I echo Olga's gratitude for your gracious willingness to share additional anecdotal medical information about your beloved Siofra's courageous ASPS journey which may help to provide invaluable insights for other ASPS patients and their families as they continue to battle this insidious disease.
I am so deeply grateful that dear Siofra did not suffer the pain and debilitation of a spinal cord compression from her spinal mets, and that she was able to remain alert during the final days of her journey before peacefully passing from this World.
Having suffered the devastating loss of our beloved oldest son in a tragic vehicle accident nine years ago, I know far too well the profound pain and sorrow of your grief journey, and would like to share information with you about a wonderful international resource and support group for parents/families of children of any age who have heartbreakingly lost precious children from any cause. The organization is called
Compassionate Friends and you can obtain information about it and locations of parent/family meetings in your area at the Web site which is CompassionateFriends.org. I Hope dear Eleanor that you will find Compassionate Friends to be a helpful resource for providing shared comfort, strengthening support, and special understanding as you make this difficult journey which no parent should ever have to experience.
Heart to heart with continued deepest sympathy, shared sorrow, and special caring,
Bonni

From the hospital I send you love! Yes she is now a angle an will be standing next to a child cancer an you.
Her new area is free and no fear pain or open questions of what next
She is beside, inside, next to you an surrounding you. The feeling while you sit quietly and feel a whips of love is her.


She I believe is in a state of bliss a place that you would love

She is you an you her

I have family and friends there also

I'm so sorry for your loss!!!

I need to lay back down.

And your question about the.
Chemo, you both knew it was a hopeful try an worth that try