Prairie from Nebraska - Dx 2015

[Bonni Hess]

Dear Mary Jo, I reached out to many of my family members who have Facebook accounts and I shared dear Prairie's story and your request for people to participate in your Facebook campaign for Prairie to be able to personally meet Taylor Swift at this coming Friday's concert. My wonderful niece who lives in Omaha wrote me to tell me that she has been following Prairie's story through all of the local media coverage, and through her work with the Red Cross and a couple of family stories she has worked on, including that of a former classmate of Prairie's. My niece sent me the video link to Prairie's and your Friday's radio interview where you received the very happy and exciting news that Prairie will have VIP seating at the concert and will get to personally meet Taylor Swift!! I am SO VERY happy for her and you dear MJ, and commend you for all that you did to help make dear Prairie's wish to meet Taylor come true I echo Olga's request that, if possible, in any further media interviews or reports, you can be sure to refer to Prairie's cancer specifically as ASPS to help raise desperately needed public awareness about this extremely rare disease, since my niece said she that had no idea Prairie's cancer was ASPS, and the television and radio coverage only refers to it as a rare cancer. Through your successful efforts to obtain media attention and support for Prairie to meet Taylor, you have also created a rare and excellent opportunity to raise awareness about ASPS and the critical need for funding for desperately needed ASPS research. Congratulations to dear Prairie and you, and special wishes for a MOST happy, wonderful, and magical Taylor Swift concert evening on Friday With happy hugs, special caring thoughts, healing wishes for precious Prairie, lots of love, and continued Hope, Bonni

[Bonni Hess]

Dear Mary Jo, I Hope that the yesterday's appointment with the radiologist went well and that you were able to reschedule Prairie's brain MRI for one month instead of three months to adequately monitor the suspected brain mets and ensure that they don't grow too large to have the best chance for successful Gamma Knife treatment. With ASPS it is always best to err on the side of caution in terms of frequency of scans, and based on our personal experience and observations, ASPS brain mets grow faster than mets in other parts of the body. With ongoing special caring thoughts, healing wishes for dear Prairie, love, and continued Hope, Bonni

[Vega343]

Friends, Appointment with radiologist on Tuesday was a tough appointment and the last few days have been hard. Very scary for prairie not knowing what was going to happen. They did CT and marked her body (pelvic region) with marker as set up to start radiation in a couple weeks (4 weeks post surgery). Schedule tbd, likely 5 days a week for 6 weeks. Side effects are likely and include diarrhea, burning urination and skin burns in the area. Ugh.
Adding to that, the radiologist pointed out there is still a 2x3 cm tumor in the lower right abdomen - shocking to me since surgeon told us only margins were left. Finally talked to surgeon today who verified he was also shocked, and determined it all must not have been connected as thought during planning, allowing him to miss it during surgery. Furthermore, he didn't notice it when we were in over the weekend scanning for abcess. I did feel that he was being honest and straightforward when we talked.
It is operable, but he wants us to consider leaving it there to learn how it responds to radiation and then remove any tumor remaining when radiation is done. I will be researching this, and we can decide at follow up appointments next Tuesday.
Thoughts?

[Vega343]

Btw, scans will be when I want them. One month for brain and 3 mo for lungs, pelvis.
I get u about saying asps to media and have done so. Thanks for the tip, there are a few interviews I was able to get that in!

[Bonni Hess]

Dear MJ, My heart is breaking for your additional heartache and challenges resulting from the new findings and revelations from the radiologist on Tuesday. I had been so very Hopeful that this would be a good week for precious Prairie, you, and your family as you excitedly awaited and looked forward to Friday's Taylor Swift concert and personally meeting her. I Hope the evening was very special and that it allowed you to emotionally escape from all of the angst and worry of the unexpected news you had received on Tuesday. Although I don't understand how the second abdominal tumor was inexplicably and inexcusably missed by the surgeon in reviewing the scans and planning and mapping the surgery pre op and during surgery, it has been our experience that doctors unfortunately can, and do sometimes make mistakes because they are Human and subject to making Human mistakes. Sadly, too often patients become the victims of these Human mistakes, some of which are irreversible, put the patient's Life at risk, and/or cause additional pain and suffering as was the case with Brittany's inexcusably missed large spinal met. Thankfully, Prairie's radiologist's thorough review of the scans found the second abdominal tumor before it grew any larger and so that it can now be addressed and treated. I personally have concerns about, and disagree with, the surgeon's recommendation to delay resecting the relatively large but still operable tumor until after the radiation is completed. ASPS is notoriously radiation resistant and I personally don't think the risk should be taken that the tumor may not respond to the radiation and could possibly increase in size and require a possibly more difficult and destructive surgery. I know that you are facing some difficult treatment decisions for which I encourage you to seek other medical opinions from experienced and knowledgeable Sarcoma experts as soon as possible. Take care dear MJ, stay strong, and know that my most caring thoughts, my healing wishes for precious Prairie, my love, and my continued Hope are with you, Bonni

[Bonni Hess]

Hello again Mary Jo,
Just a few additional thoughts and comments as my phone battery was almost gone when I wrote my previous response to you. I would like to re-emphasize my concern about the surgeon (who inexplicably/inexcusably somehow missed the concerningly large 2x3 Cm. second abdominal tumor...) now recommending " leaving it ( the tumor) there to learn how it responds to radiation and then remove any tumor remaining when radiation is done." To me this will just compound the initial mistake of his missing the tumor in his pre-op review of the scans and during the surgery, and it seems that he is gambling on an unproven treatment to shrink the tumor while taking the unnecessary chance that it might continue to grow. All of the ASPS studies that have been done in addition to our own personal experience with Brittany and 14+ years of extensive research, observations, and networking with other ASPS patients confirm that ASPS is extremely radiation resistant, and the documented best and most successful approach to fighting and managing this very challenging disease is resection, ablation, or radiosurgery if the tumor is resectable or treatable with ablation or radiosurgery. Since the surgeon has told you that Prairie's abdominal tumor IS operable, to me this seems to be the obvious treatment choice. It is heartbreaking and infuriating that Prairie will now be subjected to the pain and suffering of a second abdominal surgery that could/should have been avoided if the surgeon had done his job correctly and adequately and thoroughly reviewed the pre-op scans. I understand the rationale for the post op radiation that is being planned for the initial tumor resection site since good margins were not able to be achieved, but radiation instead of resection for the second tumor makes no sense to me. Radiation may be able to clean up surgical margins to try to destroy any possible remaining tumor cells to prevent tumor spread, but to my knowledge and in my experience, radiation to large tumor masses is seldom if ever successful because of the large toxic amount of radiation that would be required unless it is a targeted radiation radiosurgery such as Gamma Knife, Cyberknife, or SBRT. If there are concerns about the location and/or size of the tumor respective to resection, or if it would be too physically traumatic and dangerous for Prairie to undergo a second abdominal surgery so soon after her previous surgery, perhaps ablation or radiosurgery could be explored to try to shrink the tumor, but I personally think that radiation should be the last choice to try to shrink it given the chance that it could unfortunately be unsuccessful and put Prairie at risk of increased growth of an already large tumor.
On a more positive note, I would like to express my deep appreciation to you for accepting and following through with my and Olga's requests for you to specifically refer to and discuss ASPS in your media interviews in order to use the opportunity to help raise much needed public awareness about this little known disease and the critical need for ASPS research funding to help find a desperately needed treatment and cure before any more precious young Lives are tragically lost to it.
I Hope that you are able to obtain some positive and appropriate direction for your treatment decision at your Tuesday follow up appointment, and I will be anxiously awaiting your next update.
With special hugs, deepest caring, healing wishes for precious Prairie, much love, and continued Hope,
Bonni

[Olga]

To have an abdominal surgery after the radiation is a very complicated situation as the tissue is very impaired and its healing is very complicated. I would not do the radiation before of the surgery, that for sure. I think you probably need a second opinion again from someone. I am not sure the radiation to the resection borders or to this newly found tumor make sense at all now, probably just have the second tumor resected and watch for the local recurrences. If they happen, then the recurrence zone is usually smaller in size than the all surgical field or this second tumor, so the radiosurgery can be used - smaller field/higher dose. They made a mistake - why this second tumor was not discussed before of the surgery, was it in the pre-surgery report at all? Do not rush with the radiation to the margins or to this second tumor, the planning is done and can wait - you need to pick the treatments very carefully so no damage is done. May be to cryo the second tumor? The size is good for that.

[Bonni Hess]

Dear Mary Jo, I strongly agree with Olga's wise advice and echo everything she has said. Additionally, I think the highest priority right now needs to be resection or possibly Cryoablation of the second abdominal tumor before it grows any larger in Prairie's thin body, and then a followup scan and evaluation of the four brain lesions followed with resection or radiosurgery if they have increased in size and appear to be ASPS mets. As Olga has said, radiation to the margins of the first resected tumor can/should probably be delayed at this time since it is unknown/unproven if radiation can even be effective in preventing local reoccurrence. You need to be as well informed as possible before making any of these critically important treatment decisions, and don't just accept and go forward with the advice of one surgeon or radiologist. With more concern, special caring thoughts, healing wishes for dear Prairie, love, and continued Hope, Bonni

[Vega343]

Thank you Bonnie and Olga. You echo my thoughts, I was second guessing it from the beginning, but especially now, and not for am experiment of sorts.
To answer, yes, the ore plan assumed all was connected and it wasn't, so this mass was missed.
I have many more questions for radiologist, and don't be proceeding with radiation until after we meet with Sloan Kettering on October 27th if all. I hate thinking of going irreversible damage and causing chronic pain to her for the radiation that may or may not be effective. Thank u fir your valuable insight. We meet w local surgeon and onc tomorrow to discuss upcoming scan schedule, he was supposed to quantify her lung mets, have insight from his colleagues, and we are planning to have her drain tube from surgery removed as well.

[D.ap]

MJ and Prairie and family

Hugs, love and prayers to you all today.

Love
Debbie and family

[Bonni Hess]

Dear MJ, I Hope that the appointment with the surgeon and oncologist went well today, and that dear Prairie tolerated the removal of her drainage tube with minimal pain. If it has not already been discussed, an additional concern that needs to be addressed with the radiologist regarding the proposed pelvic/abdominal area radiation is the possible future infertility for Prairie that may be caused by the radiation.There are many serious and permanent side effects of radiation that need to be considered and carefully weighed in terms of known possible risks versus documented possible benefits.With deepest caring, healing wishes, much love, and continued Hope, Bonni

[Vega343]

P did get that dang tube out, thank goodness. The docs had already reconsidered the post radiation surgery and decided we had better proceed with resection by the time we had our appointment. We had the resection of the small 2x3cm tumor on Thursday morning with great success as reported by docs. The tumor was basically encompassed by the sciatic nerve, and was difficult, but they visualized ahead of time with ultrasound and they think he got it all grossly, of course residual tissue is still there. But... No damage to her sciatic nerve is noted yet as she is walking fine and the original pain she has had for about a year (that lead us to the original MRI) is gone! We were so thankful and cried and prayed together when p told us that. She was so afraid it would never go away.
Further treatment... Rescans of brain (MRI), lungs (CT w contract), and likely post surgery pelvic scan (type is tbd), are scheduled for Thursday 22october, promised to be on a disc that day for us to take to Sloan Kettering that Saturday. Our appointment there is 27oct, for oncology and surgical consult. This will be 1 month after initial brain scan noted 4x1mm brain mets, and 3 months post original lung scan showing unquantified lung mets. Knowing there is still cancerous irresectable cells in her pelvis and given lung mets, our onc is encouraging us to begin sunitinib systemic treatment soon, to avoid the occurrence of any unresectable mets and maybe help the rest. At this point, given your feedback and what I know from others doing radiation (for other cancers), we have all but decided not to do broad radiation now. I feel at ease about this, knowing that damage from that treatment never goes away and can be pain inducing. Still hoping the docs at Sloan Kettering can help check this recommendation and further expand on our lung treatment options. Also, been trying to get in touch with dr. Role unsuccessfully. If you have good email or phone contact info, I would greatly appreciate it. I did talk to someone, but the language barrier was too hard. I have emailed Rolle based on what we found online but haven't received response.
Outstanding questions:
Can dr Rolle help us?
Can anyone stAteside do the same thing as Rolle?
When us the right time to start systemic treatment? Seems like I should do any lung surgery or gamma for brain ahead of starting systemic?
What type of systemic treatment is right? P drug? S drug? Alpha drug? Clinical trial c drug?

Thank you, you are becoming such a huge part of my life! I also found out about someone named Karen in lincoln ne that I will get I touch with, apparently she is an adult ASPS patient. When I have the energy...
Will let you know.

[D.ap]

Mj

Huggsss tonight !!

Prayers for recovery and care over all involved
Prayers for you and family to get a good nights rest and face tomorrow refreshed to make some more descions with Prairies care

Love

Will write in morn

Love
Debbie and family

[D.ap]

Mary Jo

Dr. Rolle's phone number

http://www.cureasps.org/forum/viewtopic ... =865#p6424

If you go to lung section on forum there is a Q and A along with a whole host of information.
I suspect Dr. Rolle will need to hear whether there is resolution to Prairies brain scan as well as to her surgery.



Who will you be seeing at MSK?
Please say hello to Dr. Tapp for us as Josh and his wife are having Dr. Tapp help administrate Joshua's care here in Kansas.
Also I would like to share with you a link to lung met removal procedures available.

http://journals.lww.com/jto/Fulltext/20 ... omy.6.aspx
To answer your question to whether there is anyone state side that performs the laser procedure there was someone beginning to perform a similar procedure back in 2012 however I believe his funding ran out.

I hope you all were able to get a much deserved good nights sleep .
Love
Debbie

[Bonni Hess]

Dear Mary Jo, Thank you for somehow finding the strength and energy to update in the midst of everything that is going on. I am so sorry that precious Prairie had to go through the pain and suffering of another surgery, but am very grateful that the second abdominal tumor has now been successfully resected and that she has thankfully obtained pain relief from the tumor being removed from the sciatic nerve. I Hope that dear Prairie is recovering well and is now Home resting comfortably. I am grateful too that the doctors appropriately prioritized the prompt removal of the tumor and postponed questionably effective radiation, and that a brain MRI has been scheduled for Thursday instead of concerningly waiting 3 months. Regarding your question about systemic treatment, I think that the first and highest priority needs to be obtaining a definitive diagnosis of the brain lesions and immediate radiosurgery or resection of them if they are determined to be brain mets based on increased growth since the brain MRI was done a month ago. Following resolution of the brain lesion issue, if there are any large or dangerously located lung mets, I recommend pursuing cryoablation to treat a couple of the most concerning mets, or laser resection with Dr. Rolle to remove as many mets as possible. I personally think that this should be done prior to beginning any systemic treatment in order to reduce Prairie's body's tumor burden and enable a systemic treatment to be more effective. My concern about Sutent is that all of the ASPS patients who I am personally aware of who were treated with Sutent had only partial shrinkage of their mets ( about 30%) and developed disease progression usually within one or two years. It will be very important for you to research the various treatments specifically regarding results of the treatment with ASPS, and to request documented results of Sutent treatment for ASPS patients from the doctors if they continue to advocate it. I Hope that you will be able to connect with Karen the adult ASPS patient in your area, and obtain some helpful information from her. If you do connect with her, please let encourage her to visit and participate on this CureASPS forum if she is not already familiar with it. In the meantime, I Hope that you can get some rest and relaxation this weekend as you prepare for this week's Tuesday's scans and your upcoming trip to MSK on the 27th. My most special thoughts and very best wishes are with you, dear Prairie, and your family as you continue this difficult new journey. Holding all of you very close in my heart and reaching out to embrace you with caring hugs, healing wishes, love, and continued Hope, Bonni