Ellie from England - Dx Sep 2016 at 12 yrs old

[D.ap]

Sorry I should add Olga that Ellie is having a 20mg dose daily which she is fine with. The recommended dose is 17mg. So far there has been no need to reduce dosage. Ellie is currently on week 6 of treatment. Her bloods, blood pressure, weight and urine are good.

Melanie
Is the 17mg doseage by weight or clinical trial effincey determination ?
As I understand Ellie is not a part of .
So is Ellie on a higher dose than known to be working on patience’s of her age? Height and weight ?

[Disco76lover]

Hi, thank you for your caring responses. Ellie is scanned 3 monthly. I did ask about scanning her elsewhere but her brain scan was in May and the last bone scan was in Sep 2016. The Oncologist said she would do full scans again if there were signs of anything or if there was growth on her lungs. I will keep asking though, especially for the abdomen as I have read and understand the importance of trying to keep ahead of the game.

Ellie is on 20mg (the higher dose for her age) as I understand it because we can always reduce it to 15mg. I believe the meds are available in either 15mg or 20mg in our case, so we ageeed with the Oncologist to start her on the higher to see how she copes. Fortunately, so far, no complaints really. Ellie has been on cedarinib 6 months now and is on the drug through compassionate means and not trial. Thank you all. Xx

[arojussi]

I would ask for brain mri. As asps most commonly metastases to lungs, brains and bones. Blood brain barrier can limit the amonunt of Cediranib in brains. Also treating brain mets is easy when they are small and asymptomatic, but treating bigger brain mets is very difficult and expensive, so finding brain mets as smallest possible size would actually save money. Brain ct cant detect small mets.

[Disco76lover]

Thank you Jussi. They scanned her brain 6 months ago so will ask for another in 6 months time. X

[Bonni Hess]

Thank you for the additional shared information dear Melanie. I strongly disagree wirh your oncologist not wanting to do scans other than the chest CT unless there "are signs of anything" or "growth in her lungs". You DO NOT want to wait for any possible new mets to become symptomatic because they need to be found and treated at the smallest possible size for the most successful outcome, and once they become symptomatic they may be too large to resect or treat with ablation or radiosurgery. Also, based on our personal experience with Brittany's liver met 13 years ago, the chest CT may appear stable but there can still be metastatic development in other areas of the body. We and the oncologist were very surprised to learn that the chest CT alone is not adequate to determine disease progression!! Also, since the bone scan was done over a year ago in September 2016 it is time for another one as a full body bone scan should be done once a year. If the May brain scan was an MRI, it only needs to be done once a year unless brain mets were found, but it is critically important that it is a brain MRI and NOT just a brain CT which is inadequate to find ASPS brain mets at the smallest most treatable size. I am holding very tight to Hope that the Cediranib is stabilizing the progression of dear Ellie's disease, but it still remains essential to maintain a very vigilant and thorough scan schedule to adequately monitor the disease. I Hope that you will be successful in pro-actively demanding and obtaining every 3-6 month abdominal/pelvic scans (preferably MRI's to prevent the concerning radiation exposure of CT scans) as well as an annual full body bone acan for dear Ellie in addition to an annual brain MRI. Heart to heart with deepest caring, healing wishes, and continued Hope, Bonni

[D.ap]

Melanie
The need to scan is ongoing .Bonni is correct .

We sure aren’t here to scare and or make you feel on edge.
Doctors need to understand the schedule of scans as being not when symtoms accure but to show when we need to address them when discovered . Plan for surgeries and or ablations . Like the lower tumor that is 10mm. I bet your oncologist is targeting and following with your 3 month CT scans.

Jussi was saying one in the same about the brain Mri , pertaining to the brain.

Asps is asymptomatic ALOT of the times. It becomes incrediably large and travels without notice . As a primary tumor and can and will as a metastatic disease .

So scanning the primary , the lungs , the brain and the bones are a life long regiment and are absolutely necessary .

Hope we didn’t overwhelm you with info .

Love
Debbie

[Disco76lover]

Thank you both for your advice and concern. I am going to keep asking for the scans as I am concerned about it spreading to other areas also and will contact the oncologist to discuss this with her.
Again, thank you both very much as you yourselves have your own worries and you are all in my thoughts too. X

[Bonni Hess]

Hello again dear Melanie, I am grateful that you are aware of and understand the critical importance of adequate and appropriate scans with this unpredictable disease. Inexplicably and inexcusably too many doctors take a naiive or complacent approach to pro-actively managing and monitoring ASPS, and unfortunately too many patients/ patient's families assume that the doctor knows best which is not always the case. Throughout our precious Brittany's very challenging 16 and a half year ASPS journey we have often had to argue against the doctors' inadequate scan schedule recommendations and some questionable treatment recommendations based on our extensive research, networking, and sometimes actually being more knowledgeable about this extremely rare disease than some of the doctors are. It is sometimes a battle which it shouldn't be,
but the doctors seem to respect our knowledge and with invincible determinarion and perseverance we ultimately succeed in obtaining the necessary scans. Stay strong in your resolve and insistence to get the appropriate scans for your dear Ellie. She deserves to be given the best opportunity to fight this insidious disease with every available weapon of which regular appropriate scans are critically important. With special hugs, caring thoughts, healing wishes, and continued Hope, Bonni

[Disco76lover]

Thank you Bonni, you are very kind. Can I ask a question regarding blood brain barrier? What does this mean especially when on cediranib as I have read some posts discussing this? X

[Bonni Hess]

Dear Melanie, The blood brain barrier can prevent some chemo drugs from entering the brain to prevent the development of and shrink/destroy existing brain mets which is a critically important issue in fighting a metastatic disease like ASPS. It is thought, but not yet conclusively proven, that Cediranib can cross the blood brain barrier which was a significant factor in our choosing Cediranib treatment for Brittany since she had a history of continuing and increasing development of brain mets and she needed a drug which could cross the blood brain barrier to shrink/destroy her existing brain mets and prevent any new ones from developing. VERY thankfully, the couple of Gamma Knifed brain mets which she had when she began the Cediranib Trial in April 2009 have completely disappeared and are no longer visible on her brain MRI"s, and she has not had any new brain mets for eight and a half years since starting her Cediranib treatment which seems to indicate/validate that Cediranib DOES cross the blood brain barrier. I Hope that answers your question. With special caring thoughts, healing wishes, and continued Hope, Bonni

[arojussi]

As far as I know Cediranib can most likely cross blood brain barrier, but unfortunately amount of drug in brains is most likely smaller than amount of Cediranib in rest of the body as bloodstream takes cediranib everywhere, but some amount of cediranib molecules will most likely be lost during transporting it through blood brain barrier. I think Cediranib trials with gliomas support this idea.

[Disco76lover]

Thank you both so much for explaining. There is so much that I am still learning about ASPS and this site is invaluable thanks to people like yourselves who take the time to post and explain things. Thank you so much. I am so pleased that Brittany is having ongoing success with cediranib and I hope she continues to do so. Our oncologist doesn’t think it will reduce Ellie’s mets in size and number but I keep hoping as Brittany is an inspiration to us all. Xx

[Bonni Hess]

Hello again dear Melanie and thank you for your very kind words, thoughts, and good wishes for Brittany. I don't understand why Ellie's oncologist doesn't think that the Cediranib will reduce her mets in size and number because that along with disease stabilization is the treatment goal with Cediranib, and that is what it has VERY thankfully done VERY successfully and dramatically for Brittany. Although I am not personally aware of any other ASPS patient who has had as significant and as long term sustained Cediranib response as Brittany,, there have certainly been some patients who have had some met shrinkage, and of course every patient may respond differently to the same drug. It is my great Hope that dear Ellie will have a VERY positive, significant, and sustained response to Cediranib, and that her pessimistic oncologist will be proven wrong. Heart to heart with special caring, MOST positive thoughts, healing wishes, and continued Hope, Bonni

[Olga]

To add to what Bonni said. Most of the ASPS patients who had cediranib were treated in a clinical trial setting. It is a frequent situation when people on trial have a progression in one or two mets only while the rest of the mets are shrinking or stable - i.e. the location and the make up some mets might be different from the rest and they became non-responders. When Brittany had this situation with the soft tissue met, her excellent trial oncologist just decided to resect the non-responding soft tissue met and continue on cediranib - because he was may be less limited by the trial rues as it was a phase 1 trial where the efficacy not the primary goal but the drug is tested re. its metabolic properties in the body/dose finding. If she was on phase 2 or had a different oncologist, she would have been taken off trial with the progressive disease documented. I am wondering how many of the patients that were taken off trial would have an excellent response later, after their unresponsive mets were ablated/resected/irradiated etc - locally destroyed...

[Disco76lover]

Thank
You Olga and thank
You Bonni for your always caring and kind words. I must say our oncologist is an amazing lady and I wonder if she says that to not get our hopes up in case there is no shrinkage.

We Keep hoping for a reduction but I worry that the last scan showed 1mm increase in a couple of mets which is just a nightmare as I now keep thinking the next scan will show a bigger increase. I hope not.