Paula from Poland - Dx 2009 - RIP 31/08/2011

[Fictional]

What a Blessing!

This is wonderful news. Get well soon, Paula!

'F'

[jcs2007]

Wonderful news regarding Paula's surgery.
Wishing her a quick recovery too.
Peace and Blessings to all who are in this courageous battle against ASPS,
Cindy

[Bonni Hess]

Dear Mania,
Thank you for sharing this wonderful news regarding the very successful outcome of Paula's surgery. I am so grateful that everything went so well. I hope that her post-op pain has been well controlled, that she is healing well, and that she will have a very speedy recovery. Please give her a gentle hug from me, and give yourself one also for being such a faithful and loving sister. Take care and keep us updated on Dr. Butrynski's treatment recommendations once Paula has recovered.
With special caring thoughts, healing wishes, and continued Hope,
Bonni

[Amanda]

Dear Mania and Paula,
I am so glad that the surgery is now over with and that it went so wonderful!
Please, post soon how you are both doing and what the next step is!

In healing hopes and *cheers* for Paula!

AmandaR

[MANIA]

Hello
Paula is doing so great during her recovery. She was home on 6th day after surgery! Started to walking with a walker, I'm so proud of her! She's such a fighter!
I was willing to post with some info after pathologic tests of the tumor margin arrive, but it seems we have to wait till we get to know them.
Tests results were souposed to be known yesterday. What we were told is that our surgeon wanted to sit with us to discuss results and future plan - we were told it was easier than phone and email to be able to answer all our questions. So here we are waiting for the appointment date. But my heart is pounding - what does it mean? I was expecting that they would say over the phone: 'everything is great' simply come to remove your stitches' and have a talk with doctors.
Ofcourse I will share when we have this appointment.
best to all of You!

[Bonni Hess]

Dear Mania,
Thank you for your once again very faithful update. I am so very happy to hear that Paula's recovery is going so well. I know that her positive determined attitude and strong fighting spirit, coupled with your strengthening love, support, and encouragement are a huge part of her ability to recover so quickly. I'm so sorry for your worry about the delay in receiving the pathology reports, but Hopefully the appointment will be scheduled very soon and your concerns will be alleviated after meeting with the surgeon. In the meantime, please take care, enjoy beautiful Boston (our favorite area was Little Italy which has wonderful ambiance, countless authentic Italian restaurants, and fun shopping when dear Paula's recovery allows), and keep this Forum updated as you are able.
With special hugs, caring thoughts, and continued Hope,
Bonni

[Amanda]

Dear Mania,
They are probably going to talk about Chemo and things like that to help her prevent more cancer i would think :/
If not look at how she is feeling now *today* and walking this is to me a marker of how she and her body are doing
After my surgery they said the same thing to me and i was also frightened! Tell her to look right now at only what she is feeling because today she is recovering and this is wonderful and to hold on to that more then what they will be saying. Though i kinow it is hard to do i think she can do this she has been thru a lot and she is strong and positive! If she feels bad have her read thru the survivor stories in this forum it helped me a lot

Hugs to you both!
An healing hopes and for a cure!

AmandaR

[MANIA]

Hi there. thank You for your posts, and strong support! You are just wonderful!
So, here comes: postrugical update.
There is one area where pathologists found some tumor cells on one margin. They are talking about microscopic scale. Surgeon said he didn't feel it should be removed surgicaly. But futher treatment is needed. Paula is a canditate for protonbeam radiation, we will know for sure tomorow. Meaning we have to stay in Boston a bit longer. Now we are of for MRI. Paula is in great condition, recovery going very well, in good spirits.Re Lungs state - there is 4 things/changes in there, radiologists after looking over and over are sure that 1 is a nodule. Of course no one says is tumor for 100%. Paula will have additional chest scan in 2 weeks. Doctors suggest after few additional weeks Paula goes back to sutent, since she responded so well to it.
Very important thing - primary tumor was mostly necrotic!!!! which is very motivating!
Greetings to all of You.

[MANIA]

Paula had her chest post surgical CT done. THere are 25 nodules in both lungs, the largest 4mm. We will discuss what should be done now, as further treatment. It's sad news, since in august there were 8 noduls visible.
Now the big question I have is: what is better? To start farmacological treatment and see how nodules are doing? OR to get rid of them at dr Rolle as soon as possible? I know it's up to her and doctors. But what seems better approach in ASPS. Our surgeon thinks treatment first, surgery after treatment. But what usualy is done with sarcomas doesn't have to be a good choice in ASPS right?

As I understand there are 3 options: 1. going back on sutent 2. cediranib trial if available 3. chemotherapy
We will see what our medical oncologist from Dana Farber suggest today.

Paula feels kind of down. Mixed emotions. She is 6 months after diagnosis, tired with presence of ASPS in her life. Days like today, when she hears there are some nodules present still make her more concious that she would have to deal with this difficult disease for a long time. She always said she needs to fight with it. Now I start to understand it's maybe not about fighting but accepting facts and living with it, and trying to have the best life with it.

She is staying in Boston with her fiance for 12 days of radiation. They are still deciding if it will be proton or photon radiation.

It feels really comforting being able to write this here. Please post your comments if You have any, I'll be happy to read Your opinion.
Sending sunbeams to Your hearts!

[Fictional]

Hi Mania,

This is a similar situation that we were in with 'K'. It is a difficult business. There is a suggestion that VEGF-type drugs won't help for the small (less than 1.5 cm) lung nodules - because the tumor cells can grow without an external blood supply. ASPS seems to bloom these small metastases in the lungs for 1-2 years after the primary is removed although it is difficult to know whether early surgery or some of the new agents might help reduce metastases.

The evidence is fairly strong that pulmonary metastasectomy can induce an immune response against the tumor and prolong survival and potentially induce long-term remission. Late metastases can occur, but the highest number of metastases seems to occur in the period where the primary is still in and in the first few years after its removal.

The first drug 'K' was put on was Sutent - and that was before the primary was removed. The primary turned black and didn't grow - but didn't die either, but her new pinpoint metastasis grew from just barely visible to about 4-7mm. We went for thoracotomy, but realized new pulmonary mets might continue to bloom after the surgery was completed. I don't think we regret it, and it looked as if thoracotomy slowed the progression of disease almost immediately after, but when you deal with small mets, even Rolle and his laser will miss them.

'K' went on ARQ197 and now R1507 and has had 3 thoracotomies total (1 on right, 2 on left - conventional thoracotomy for one one the left...never do that again) in addition to her primary being removed. She is now heading toward 2.5 years after diagnosis. Her largest remaining nodules are 1.5 cm (one on each lung) - both were missed by Rolle and his laser. They are not in dangerous places, but of course we wish they weren't there. Still, if they hadn't been there, she would not have been allowed to get into an ASPS trial (need to have a metastasis at least 1 cm) for R1507. Others are a few millimeters.

'K' is a candidate for more surgeries, but we don't want to do it now - because some are very tiny and inevitably could be missed if she has to go to surgery soon. We like the idea of Cediranib, but have pursued agents that might inhibit the formation of metastases (ARQ197) and R1507 because they have a different mechanism of action - and are not just VEGF. We typed 'K''s pulm met and the small ones (less than 1cm) didn't have VEGF or PDGF. We did try metronomic chemo due to anecdotal reports, but were disappointed in the results.

We are keeping Cediranib in mind - for later if we need it. We are thinking Cediranib may be commercially released by a year or so for now as it is in the pipeline for FDA approval for glioblastoma and colon CA. It is a little risky on our part not pursuing Cediranib when it has worked well for others, but with the typing results and our observation that Sutent couldn't stop the smallest ones from growing, this made sense in her situation. Also 'K' has no side effects on R1507. She is having a great time being physically active, on volleyball team, etc. and because Cediranib does have significant side effects, we wanted to hold off if we can. If she progresses on R1507, we might also consider Afinitor which is commercially available and an mTOR inhibitor, if that is the growth is not too fast - and we think we still have time with other agents. Pazopanib apparently works in ASPS by anedote, but it is phase III which means it is possible you could get randomized to placebo. There is a site in Bethesda where she could get Cediranib on the peds trial, but that would take her out from school, all the side effect business, and disruption.

[Amanda]

Hello Mania,
The Cediranib trial is as far as i know open and your doctors at the hospial should be able to contact them at the trial and get her on this. Try and get on it as soon as you can! There is a thread here and there is a phone number of a very loving lady that you should contact at the trial. She will help you

I am very sorry that more things showed up on the scan and that she is feeling down She is having a hard time also because i am sure she feels that now she is not in controle of her life and she needs to depend on others. Tell her that she is still in controle and to be active still in all treatments. She did the hardest thing already that surgery was tuff! An i think that she is amsing!
This is not a fun ride ASPS nor for that matter is any form of cancer! It takes the life you have created and diverts it in another directon and it is a fight to also be you thru this. I send her a *huge hug* I hate this also very much!
How is she feeling though? If she feels good that is what is important and she needs to hold onto that and hope! I know it is hard i am there with her and i hate that this has taken over my life for the most part also. But, we need to keep pushing on and try to be positive as best as we can. I was talking to someone the other day and i said that i now live my life trying to dance thru life on the tip of a pin in the wind. My friend looked at me and said that everyone lives like this and having ASPS just makes it a bit harder. I didnt know how to respond to this because untill you stand in the shoes of the paitent going thru a diagnosis like this though others try with all there hearts they just cant get what this feels like and i hope that they never do! I feel that the only ones that do understand more are the parents of are ASPS children.
I am fighting to still be me and i know that everyone is also thru this still trying to the person that they were before they found out that had ASPS. Tell her if she can sit in the sun if she can and walk if she can and see things in the area if she can do that will make her smile do it. Hold onto the hope that seems at times to flee and bring it back. There are many new things out there that will help and look very prommising for us. Have hope and smile and be her!
Durring every doctors appointment someone other then her needs to right down every word that the doctor says that is positive! Even if to the person righting this it seems a small thing it is not. Then she she feels down she is to take this list and read it over and over. I did this also and though it seemed a bit silly when i was told to do this it worked for me and i hope it also does for her <3

There are things that come to us as being a paitent of a diagnosis of cancer and that is are eyes are now open to see the things in are lives and the world that are beautiful much clearer. I told my doctor that in a very odd way i am thankful for getting this because i feel love deeper and see things muct better and for every minute i am blessed enough to walk this earth it is multiplied in happyness the things that i and many take in every day life for granted. He laughed and said "You have no idea how many paitents i hear this from" and he smiled.

After reading this forum and also talking to my and other sarcoms specialists there is no dead line for are lives we are as individual as are diagnosis. No ones experience with ASPS is the same way.

I am sorry that i went on so long with this But, in at least some small way I hope my rant helps her or anyone else that reads this to know that they are not alone in feeling like this.

In lovinging and healing hopes for all!

Amanda R

[Bonni Hess]

Dear Mania,
Thank you for your update. I am so very sorry about the increased number of lung mets which were found on Paula's most recent CT, and I know how very discouraging this must be for her and you. It is of course very difficult to accept that having ASPS usually involves a long and challenging battle, but it is very important to try to remain as positive as possible, to be relentless and aggressive in researching and pursuing promising new treatments, to hold tight to Hope that an effective treatment and cure will be found soon, and, as you and Amanda have so wisely and well expressed, to live Life to the fullest and the best that one can while still battling this insidious disease. Because of Paula's relatively large number of lung mets, I personally think that it would be best to pursue a treatment like Dr. Rolle's to try to remove as many mets as possible, followed by a systemic treatment like Cediranib to try to prevent any new ones from growing. It seems that reducing the tumor load by removing the tumors first would help strengthen the immune system and allow the systemic treatment to work better. This is of course my personal opinion and is not intended to be medical advice, but I do think that it is vitally important that all treatment approaches and options be thoroughly explored and discussed with the oncologist before Paula proceeds with treatment. Please keep this forum updated about Paula's treatment decision, and know that my continued most special thoughts and best wishes are with Paula and you.
Heart to heart with special caring and continued Hope,
Bonni

[Olga]

Dear Mania - I think that regardless of what decision will be done now for Paula's treatment to follow you should take her latest CT scan on a CD and contact Dr.Rolle and with his permission send him this scan for a review. He does the review free of charge (although it takes a lot of his time to go trough it) and after that you get his opinion on this chess game with the lung mets - if they are all in the OK locations there might be a time to try something else, but in some cases some of them are happened to be in the location that if they grow more then a certain size they can not be removed by laser as they get in the contact with the central blood vessels and Dr.Rolle will not be able to help her, I know the case when it happened. He usually prefer to see the dynamic - to compare the next scan to this one. It is often that after the surgery to remove primary tumor dormant metastases start to grow faster for awhile but it might slow its pace later.

[MANIA]

Thank You all for Your immidiate response.
Amanda, I always enjoy reading what You write, thank You for Your caring spirit.
'F', I look up to 'K''s case a lot, and I think it is so happy You are a doctor Yourself and have all of the medical knowledge about chemistry of medicines. I'm total amatour in it,but guess I have to keep on learning.
Bonnie, thank You for sharing your opinion with us, it is very important that we hear different approaches patients, and they families have.
Well, without this forum we would never found dr Butrynski at Dana Farber, who we visited yesterday, and our moods got a lot better after that. First of all, he didin't feel the 25 nodules were panic situation, actually looking at ct scan he found radiology reprote too dramatic (it is something weird with counting nodules though - every one sees different number of them)! We decided Paula will go back on sutent 2 weeks after radiation. In the meanwhile we want to send recent scans to dr. Rolle, so we get his opinion and see what he says, just like You suggest Olga, for future. But according to dr Butrynski (and his not the only doctor to say so) doing a surgery (wheter it's laser , or classical thoracotomy) would be a wrong move right now. Since there are other options that may work. Most likely after removing nodules surgicaly, the new ones from under them would pop out quite soon. We should consider farmacological treatment, and watch what is happenings. Paula's nodules are very small, and accoridng to his opinion they won't cause her any problems at the moment or in the new future.
Of course having them causes psychological stress, but she is beliving Sutent would help it. At the same time we will think of cediranib trial, but have to do more resarch on it, and apply for a trial, hopefully in europe. IT would be much easier then.
Any way our situation looks like this -we will have dr Butrynski for main oncologist, and he will cooperate with polish doctor on Paulina's case. We love the way he speaks with us, giving so many information, and making things look clear. At the same time, we know he had patients with ASPS - which makes him special.

Have a good day everyone

[Bonni Hess]

Dear Mania,
Thank you for your kind words and for being so faithful with your updates. I am so glad that you had a very positive meeting with Dr. Butrynski and that you found him to be as caring, compassionate, calming, and knowledgeable as we did when we were fortunate enough to have him for Brittany's oncologist in Seattle before he moved to Boston to work at Dana Farber. We really miss him , but are in occassional contact with him by e-mail to discuss Brittany's situation and to seek his highly respected professional advice. Please tell him "HELLO" from the Hesses when you meet with him again. It sounds like you have developed a good plan of treatment for Paula, and my best wishes for a very successful outcome to the treatment will be with her. There is a 21 year old ASPS patient named LCMA who was diagnosed two years ago with a primary tumor on her psoas muscle in her abdomen and lung mets. Following radiation and resection of the primary tumor, she began treatment with Sutent, and thankfully thus far she has had stabilization of her disease. Since her situation is similar to Paula's I thought that you might be interested in following her journey and her Sutent treatment experience through her Blog which is LCMA.blogspot.com Take care Mania, and keep in touch with this Forum as you are able.
With special caring thoughts, healing wishes for Paula, and continued Hope,
Bonni