Jo from Perth Australia - Dx 2011

[Mel&Jo]

Gosh it feels like a life time ago since I first made contact on behalf of my beautiful sister Jo.

Since Asps came into our lives (just a little over 2 years ago) we are no longer the same people. Asps forces you on a roller coaster ride and one you didn't want to take - but it has made us stronger as a family unit and for that part I am grateful. How naive we were 2 years ago...... we never thought we would have to leave Australia and send Jo to the UK but thankfully we found your website a godsend of information!

So where to start - after trialling sutent in WA the results just weren't there. We were told in July 2011 that there was no other treatment available here so finding this website And reading about clinical drug trials we stumbled on Prof Ian Judson in the UK & Dr Rolley in Germany though Dr Rolley was unable to accept Jo. Long story short Jo was accepted onto the cedirinib drug trial and she moved to the UK in December 2012. It hasn't been easy especially with Jo living so far away. I cant even imagine what Jo goes through. My dad flew back and forth and my siblings and I met Jo in Europe and we had the best European vacation together... One we will never forget. Stability for a year we are blessed and Jo moved to a 12 week programme which allows her to now fly home as physically she thinks she unstoppable! She even tried her hand at learning the trapeze - her medical team think she is insane but she LIVES each day.

Jo's latest results just came in - there not great. Today she has just flown back to the UK so we are now waiting to hear what her medical team thinks. I'm hoping there are new drug trials available?

Love & hugs to everyone affected by this terrible disease. Mel xo

[wangcns]

Dear Mel,

I'm so sorry to hear about your sister. My husband Kevin was diagnosed with ASPS in February of 2012, and I can totally empathize with you. Cancer has totally changed our lives. It really is a crazy roller coaster, and I'm still trying hard to take joy when and where I can get it, while being strong during the inevitable lows. I think the hardest part is seeing the healthy, happy person you love struggle to go through this terrible disease, but I'm so glad you found this website. Olga and Bonnie, not to mention all the wonderful people on this board have been my support group, helping me parse all the information and figure out what decisions to make in this cancer with so many unknowns.

I also share your frustrations with coverage for this disease. We were unable to get insurance coverage for both of Kevin's surgeries with Rolle and so we launched a fundraiser through a website called GiveForward.com. This is a great way to make it easy for friends, family, and even strangers to donate and is similar to Kickstarter, but for medical expenses. I highly recommend it, you write a bio and launch the page, and it becomes an easy link for anyone to click on and donate.

All my best thoughts and love for Jo, she sounds like a wonderful person. Take time for yourself too.

Best,
Connie

[Bonni Hess]

Dear Mel,
It was so very good to hear from you again, and your thoughtful update is deeply appreciated. It is always so very concerning when people lose touch with the Board. I am grateful that dear Jo had a year of disease stability on the UK Cediranib Trial which allowed her to enjoy a special European vacation shared together with your family as well as to pursue learning the trapeze!! , but I am so deeply sorry that her most recent scan results were not good. Did her scans show any new tumors, or just increased growth of her existing mets indicating developed resistance to the Cediranib? Did she have significant tumor shrinkage/disappearance while on the Cediranib or just stabilization of her disease? I will be anxiously awaiting your update regarding the outcome of Jo's meeting with Professor Judson and the medical team, and will be holding VERY tight to Hope that they will be able to offer some promising new treatment options for her. There is some encouraging data which indicates that if a patient has had a successful response to a Tyrosine Kinase Inhibitor (TKI) like Cediranib and then develops resistance to the drug and disease progression, they may be able to switch to another TKI like Pazopanib, obtain disease stability, and then return to taking the original TKI and regain the treatment success and stabilization of the disease that they experienced originally. Of course the problem with Cediranib is that it is only currently available in Clinical Trial, and unfortunately most Clinical Trial protocols will not allow the patient to return to taking Cediranib once disease progression has occurred and the patient has been taken off of a Cediranib Trial. Hopefully someday soon Cediranib will become FDA approved for prescription by an oncologist so that it would then be available without the constraints placed on its availability by Clinical Trial protocol. Please know that my most special thoughts and very best wishes are with Jo, you, and your family, take care Mel, and keep in touch with the Board as you are able.
With deepest caring, healing wishes for Jo, and continued Hope,
Bonni

[Mel&Jo]

We may have a little luck on our side - jo's latest scans were compared to her 2011 scans not her jan 2013 so what appears new maybe incorrect. Jo has arrived back in the uk and they are going to remeasure. That roller coaster comes to mind ....

[Mel&Jo]

So good news - Jo's scans were re-measured in the UK and she was in fact stable. 12 weeks have passed and her latest results (Monday this week) Jo is stable again.

[Amanda]

YAy for stable!!!

BTW i think her doing what ever wild and fun thing she can do is great! LOL

Tell her i am cheering her on!

<3

[Olga]

I am happy to hear that Jo is stable, but please have her to get the brain/bone scans at home - during the trial they only survey and measure the targeted nodules usually in the lungs and the rest of the normal periodic scanning remains to be done at home by the regular oncologist team, it is very important to realize and not to overlook.

[D.ap]

Mel and Jo

Hi. My name is Debbie. I am Joshua's mom.
My question to you girls is how the trial is going? Do the doctors have any plans to do surgery,surgeries, of any sort and are they keeping good tabs on the tumor close to the heart?
Are there plenty of scheduled scans, bone etc scheduled?

Given the choice, you and your family(ies) have such a great outlook that I really stand back and admire you all. God knows that it is hard to muster up some days.

Keep up the good work

Debbie

[Bonni Hess]

Dear Mel,
Thank you so much for your thoughtful update with the VERY good and encouraging news that Jo's most recent 3 month Clinical Trial status scans showed continued disease stability I am so happy for Jo and your family, and am holding very tight to Hope that the Cediranib will provide continued and sustained stability for her. Has any shrinkage/disappearance of Jo's mets been observed on the scans, and if so how much? I strongly agree with Olga's recommendation that Jo have additional periodic scans done by her local oncologist for those areas of her body which are not being scanned and followed by the Clinical Trial doctors. It is critically important to monitor all areas of the body, and not just the area with the targeted mets because this insidious and unpredictable disease can unfortunately develop and show up in other areas even when the targeted mets appear stable. Being vigilant and pro-active with complete scanning is vital in fighting and managing ASPS to ensure that any new mets can be detected and treated at their smallest most treatable size.
I Hope that Jo is tolerating the side effects of her Cediranib treatment well. When your time permits, it would be deeply appreciated if you could share information about what side effects she has been experiencing thus far.
Please give Jo a happy hug from me, know that I am sharing the relief and joy of her good scan results with great happiness, have a wonderful rest of the summer, and keep in touch with the Board as you are able.
With special caring thoughts and shared happiness, healing wishes for Jo, and continued Hope,
Bonni

[Mel&Jo]

2015 ... Hi Everyone!

Just an update on my beautiful sister Joanna. So Joey had been doing super well on the cedirinib trial in the UK but unfortunately had to have spinal surgery (t7 removed) due to a tumor (or an existing lung met pressing and attaching to her t7 - where still not sure?). Therefore she has had to come off the trial but will still travel to the UK in search of another trial in the next few months. Such a disappointment considering Jo has been stable for 3 years on cedirinib but as most of you know any surgery and the trial stops. Jo's now recovering and about to start 15 rounds of radiation she is also taking pazopanib. No scans until June. I'm am worried as I've been reading that once you stop cedirinib patients can experience rapid growth. Where hoping that being on pazopanib - this may help.

Bonnie - we did a head scan as you mentioned 6 months ago and found a 2cm lesion in the base of jo's skull. As they had never scanned jo's head it wasn't deemed 'new' and has remained stable. The surgeon here will remove once Jo has recovered. She is slowly getting back on her feet. She lost a fair amount of weight but we are working on this now. I think one of the side effects to pazopanib is weight loss? But like I said she's eating and dealing with this so hopefully Jo can put the weight back on.

I'm currently reading up on PD-1 - fingers crossed there is a trial that we can access.

Love Mel x

[Olga]

Hi Mel, thank you for an update. I agree that it is a big disappointment that Jo had to go off trial when the progression was only in one location - we just recently discussed with a new patient from UK here that some mets could be stable or even shrinking on cediranib and some could grow - in my observation the lungs are often stable and the soft tissue mets grow. Bonni daughter Brittany was on cediranib trial phase 1 that allows the patients do surgeries - the phase 1 trials do not study efficacy but find the maximum tolerable dose and side effects, so when she had a progression in the soft tissue mets she just had them resected. She remains on cediranib even after the trial was finished as she is receiving a clinical benefit and they have to continue to supply the drug...
anyways, since Jo responded to cediranib well, the chances are good that she will respond to pazopanib as well. I hope the surgery for the head met is a success - is it in a brain or in a bone?

[D.ap]

2015 ... Hi Everyone!

Just an update on my beautiful sister Joanna. So Joey had been doing super well on the cedirinib trial in the UK but unfortunately had to have spinal surgery (t7 removed) due to a tumor (or an existing lung met pressing and attaching to her t7 - where still not sure?). Therefore she has had to come off the trial but will still travel to the UK in search of another trial in the next few months. Such a disappointment considering Jo has been stable for 3 years on cedirinib but as most of you know any surgery and the trial stops. Jo's now recovering and about to start 15 rounds of radiation she is also taking pazopanib. No scans until June. I'm am worried as I've been reading that once you stop cedirinib patients can experience rapid growth. Where hoping that being on pazopanib - this may help.

Bonnie - we did a head scan as you mentioned 6 months ago and found a 2cm lesion in the base of jo's skull. As they had never scanned jo's head it wasn't deemed 'new' and has remained stable. The surgeon here will remove once Jo has recovered. She is slowly getting back on her feet. She lost a fair amount of weight but we are working on this now. I think one of the side effects to pazopanib is weight loss? But like I said she's eating and dealing with this so hopefully Jo can put the weight back on.

I'm currently reading up on PD-1 - fingers crossed there is a trial that we can access.
Love Mel x

Hi Mel and Jo,

Thanks for the update on Joey
I am sorry that Joey had to get off the trial when stability was there and on her side. But it sounds like you made the right choice
We are all praying that it comes available SOON for all of our friends to have available to them.

I was wondering what the PD-1 info that you had to read was saying when you get a chance.
I wish for Joto have a speedy recovery.

Love
Debbie

Another ASPS patient is looking into it too.
Jen

http://www.cureasps.org/forum/viewtopic ... =135#p8101

This is what I've found

pembrolizumab

Its an authorized melanoma med and is being looked at for advanced lung cancers.
http://www.medicalnewstoday.com/articles/292589.php

[Bonni Hess]

Dear Mel,
It is so good to hear from you again, and your thoughtful update is deeply appreciated. I share Jo's and your great disappointment and sorrow about the heartbreaking progression of Jo's disease after three years of sustained disease stability and a seemingly successful response to Cediranib. This sadly documents to me the suspected fact that even though a Cediranib patient may have long term sustained disease stability, the patient can develop resistance to the drug at any time and disease progression. Did Jo's scans ever show that she was completely scan visible macroscopically tumor free, or did she just have sustained disease stability and tumor shrinkage or disappearance of some of her mets? Although the two Cm. lesion at the base of Jo's skull that was found six months ago wasn't deemed "new" since the doctors had (inexplicably/inexcusably!!) never done a brain MRI, I am concerned that it actually was somewhat "new" because ASPS brain mets typically grow much faster than mets in other parts of the body, and I think that it probably did develop sometime recently during Jo's three years of alleged "disease stability"!! VERY frustratingly, it is the practice of Clinical Trials to only monitor and scan areas of the body that had exisiting mets and to follow selected target mets which were present at the time that the patient started the Trial. Status of the patient relative to treatment success or failure in stabilizing the growth of the existing mets and preventing the development of new mets in the scanned areas is then erroneously based on specific scans that don't include the entire body. This is why we insist on, and vigilantly schedule, Brittany having regular brain and spinal MRI's now every four months as well as an annual full body bone scan locally in Seattle since her Cediranib Clinical Trial in Edmonton, Alberta only schedules her for chest/abdominal/pelvic scans now every four months because she did not have mets in any other areas except her chest and abdomen when she began the Trial six years ago. Knowing that mets can and too often do appear in other parts of the body than the chest requires vigilant and regular scanning of ALL areas of the body to ensure that any new mets are found and treated at the smallest most treatable size. Unfortunately, the burden to request and insist on adequate and appropriate scans too often lies with the patient or the patient's family, but it is critically important to be aware of this and to be extremely pro-active in following through with requesting, obtaining, and having the scans despite any opposition that may be faced from the oncologist and/or insurance company. I am very perplexed and concerned that Jo's relatively large met at the base of her skull was not resected or treated with Gamma Knife when it was first diagnosed six months ago. Is the met in her brain, or in the cervical part of her spine? You said that it has fortunately remained stable for the past six months, which is rather amazing given the usual nature of ASPS brain mets to grow quite rapidly, but I Hope that it will/can be resected or Gamma Knifed as soon as possible when Jo is adequately recovered from her spinal surgery. I Hope that the Pazopanib will be successful in re-stabilizing the progression of her disease. I am not personally aware of treatment results for any ASPS patient who has been treated with PDL-1 because we don't have any patients who have posted any PDL-1 treatment information on this Discussion Board, but I follow a 15 year old ASPS patient in Oregon named Nathalie who is currently being treated with PDL-1, but unfortunately neither Nathalie's family nor her have shared any anecdotal treatment results on this site despite my repeated requests for them to do so. You can try to personally contact them to ask them about Nathalie's PDL-1 treatment experience and results with a private Facebook message to Nathalie's father Nathan or to Nathalie.
My very best wishes are with dear Jo for a speedy and full recovery from her spinal surgery, and a very successful response to the Pazopanib in stabilizing the progression of her disease and shrinking/destroying her mets.
Thank you for being such a caring, loving, and faithful sister to Jo. She is very fortunate to have your dedicated help and strengthening support in her courageous battle. Take care dear Mel and keep the Board updated as you are able.
With special caring thoughts, gentle hugs and healing wishes for dear Jo, warm friendship, and continued Hope,
Bonni