Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Tanja,
thank you for reaching out.
After the last conversation we had with Saskia here, I felt that she might be offended by the negative comments re. complimentary treatments she is having. For us it is not the new subject and not the first time we have to say that again - nothing should be assumed as beneficial for ASPS treatment if not proven. Anything that is done can affect in 3 ways - do better, do worse and do nothing, and if you do not have a proof if works in any particular way, there is a risk. Most of the alternative medicine treatments have no proof of action, and the science there is only limited to the dish experiments.
I am very concern that the alkaline infusions are given with the immunotherapy trial going on. Do you know what the alkaline infusions consist of - what is given/dosage and how they affect the mineralocortical hormones (aldosterone, cotrisol) and sodium-potassium balance, plus the red-white cells counts - lymphocytes, neuthrophils? I suspect it should affect that a lot since it is blends into the blood. Keytruda is her best chance for now and I would not risk loosing it for some non-proven benefits. Immunotherapy is a very gentle treatment and basically the person just have to be in its best help and I do not think the IV infusions are such an innocent thing during the immunotherapy clinical trial - the doctor leading the trial has to be notified about that for sure, as it can change the response to both drugs and their toxicity profile. I have seen that alkaline IV are used in emergency rooms for the hyperkalemia situations so for sure it can and will modify the bloodwork.
Saskia is a young person and her immune system should be good if just supported with the good food, sleep and exercise. IV is to rough of an intervention in my opinion, the alkaline cocktail she is having is good enough. Besides some people use the alkaline IV for the weight loss which you do not want.
About the FGFR4 mutation. There are some studies re. different genes mutations in ASPS, I will find that later. There are studies of the genetic alterations in ASPS but the up regulated growth factors are different between the patients and using the own patient tumor to analyze and find the better suited therapy is an interesting option but not always works. Besides there is usually a resistance develops to any growth factors inhibition when the body finds another pathway to go around this point, developing accessory pathways. There is usually a good response rate in TKI but even with the very active ones it is not uniform. As I understand, you are asking if it makes a sense to use the result of gene expression profiling that you got for the FGFR4 positivity. It is hard to say when the FGFR4 inhibitors are in the clinical trials - if any of them will work as intended, you should look at the Pubmed.gov for the articles
there was a pretty recent article about the gene expression profiling in ASPS, I have the PDF, but in general they say:
Different approaches including gene expression profiling, western blotting and immune profiling have revealed robust expression of angiogenic associated molecules including vascular endothelial growth factor receptor (VEGFR)1, VEGFR2, VEGFR3, epidermal growth factor (EGF), MET, RET, platelet derived growth factor (PDGF)B, PDGFRB and innate immunity related receptors such as toll-like receptors (TLR)2 and TLR9 in ASPS
https://www.sciencedirect.com/science/a ... 2218314306
they do not mention FGFR4 but we have seen that there might be difference between the patients especially based on the age as innate factors change as we age.
So in general, we do not know if it makes sense to seek the enrollment into the FGFR4 clinical trial. Right now she is on the best clinical trial as it shows by far the best results in ASPS by now, just try not to interfere with it. It is good that the antibiotics were avoided. What are the blood work results - any deviations from norm, any problems, what is the neutrophil/lymphocyte ratio (NLR)? I keep the table with the results Ivan get to watch for the changes and problems.

And just to clarify the weight issue - it is very good that she managed to add some on, but also it is important that specifically the muscle weight is added. I realize that It is difficult to add that with the primary tumor site issues, but she might want to look into sitting exercise of some sort, using small weights perhaps. Plus make sure she gets enough fiber in the diet (whole unprocessed fruits and raw vegetables are better sources than a juice where the fiber is mostly removed) and elemental Zn has to be present no less than the recommended daily intake - it is 8 mg for adult women and I do not know if it may be has to be more for the growing person? Zn is needed for the maturation of the lymphocytes. Some diets or IV may actually remove the Zn from the body.

Hello
We were at the hospital today for 5th keytruda. Unfortunately Saskia s port is somehow growing out and it is the case that it might come out completely the next days. Therefor she needs to have another surgery tomorrow to fix it and maybe try to Implantate a smaller one iff the existing connection works smoothly. They will also use antibionase which I'm afraid of because we tried to live without any antibiotics. But they told us it's important :(

So to be quick now: the alkaline infusion she gets at the other doc is: Natriumbicarbonate 8,4%, and Procain in NaCl only.

I did also ask today for specific bloodtest for leucocytes.
Neutrophils are at 62
Lymphocytes are at 22.
I think the NLR is fine so far. Unfortunately we can't compare as we don't have figures or values from before.
Do you agree?
Shall we let this be checked always?? For comparison reasons?
Zn wasn't on the list.
Natrium 138
Kalium 4.1
Calcium 2.4

Hugs
Tanja

Tanja - does the oncology doctor on a clinical trial know that Saskia is getting the alkaline infusions? Both, Natrium bicarbonate and NaCl change the acidity balance and as the effect the overall drugs metabolism could be altered. Perhaps, it is not the right time for the additional complimentary treatments that can interfere with this important treatment.
The blood work has to be done before every K infusion with the blood cells and electrolytes count plus the kidney and liver function, I have seen somewhere the new guide. NLR is a bit to high but it is probably because of the recent infection and the port complications. Ivan does not have a port and it is just fine, but he is getting Keytruda alone.
It looks like the units they use for the absolute blood cells count are different from the ones that are used here. What is shown in the table? in our test is is measured in 10*9/L

Thx Olga for the quick reply.
NaCl is also given with Keytruda everytime. We will check the other ingredients.

Subtypes of leucocytes are measured in %, leucocytes (8) is mrd/l and common value given as 4.0-9.0

Ref.to the surgery tomorrow and the antibiotic : any suggestion for afterwards to compensate in a good state? Like drinking a lot or digest something special to bind it or get it out of the system in time (when it did its job)?

Tanja

P. S. Yes we have bloodtests for sure everytime before K but we didn't have the Subtypes of leucocytes. I asked for it.
Liver and kidney is done, metabolites, electrolytes, glucose, AST/GOT, lipids, blood (Edta)

Saskia wrote: Wed Nov 13, 2019 1:09 pm P. S. Yes we have bloodtests for sure everytime before K but we didn't have the Subtypes of leucocytes. I asked for it.
Liver and kidney is done, metabolites, electrolytes, glucose, AST/GOT, lipids, blood (Edta)

Hi Tanja,

Would the alkaline infusion been adjusted , had you had leucocytes factored in when prescribed prior to infusion ? Or are the alkaline infusions a standard prescription .

NaCl is a saline medium that is used to dilute Keytruda. 0.9% Sodium Chloride is used, NaCl=Sodium Chloride. I guess it is also used with Procaine for the same purpose. Procaine is a local anesthetic drug, to make the IV with it less painful. Is it Natrium bicarbonate or Sodium bicarbonate IV?
In any way, having an anesthetic drug every week is not that an innocent thing. We have discussed recently that some of them have an immunosupressive effect.

Hello all,
We have bad news. Saskia had her MRI of the leg yesterday and the primary tumor grew despite the fact she had her 6th Keytruda treatment yesterday. Additionally a 2cm bone met was visible in her leg as well and we guess 2 skin Mets. One on the right of her tummy and one at her neck.
The docs discuss to have radiation on her leg as soon as possible and start soon with chemo treatment with Trabectedin to stop the process. We are confused and devastated by those news as we really thought ICI Keytruda should be the best. Lung CT will be on 16th but nobody expects good results now for that as well. I still pray for it.
Her leg hurts a lot and for sure we are not confident for sports and exercises now not to cause any fracture.
Can you give advise on Trabectedin or do you have any other ideas at the moment?

Lovely greetings with hurting hearts
Tanja and Saskia

Tanja and Saskia,

It’s truly hard to hear and see reports of growth however as I’m sure you are aware of , ICIs can produce pseudo progression as well.Also our son had what turned out to be a benign growth removed on his neck . Sebaceous cyst.

Consult the radiologist and maybe get a second opinion on the skin growths being seen ?
Hang in there as I truly understand your distressed feeling .
Love

Thx Debbie for your quick reply.
The MRI was discussed on the local tumorboard and with other specified sarcoma clinics in Munich, Berlin and will be discussed with Essen as well. As the tumor grew 2cm in every direction plus new Mets developed they are of the opinion not to speak of pseudo progression. We hope for the lung results to prove better but it's not expected, we've been told.
But if so, I'd love to continue with ICI Keytruda plus radiation.
Do you agree or is it better to have a break of the DNA by chemo Trabectedin plus radiation?
Were kind of lost and so devastated by this quick growth in total.

Tanja

Hi Tanja and Saskia,

Is there any chance to stop the treatment temporarily to give the body rest for primary resection?
From my experience and another person told me on this forum that primary removal could give the body less tumor burden and could probably slow the met growth speed.
However, if they think that other mets shall take first priority I do not against that systemic therapy first.
Send my prayer to you,
Daniel D.

danieldew7 wrote: Thu Dec 05, 2019 5:56 pm Hi Tanja and Saskia,

Is there any chance to stop the treatment temporarily to give the body rest for primary resection?
From my experience and another person told me on this forum that primary removal could give the body less tumor burden and could probably slow the met growth speed.
However, if they think that other mets shall take first priority I do not against that systemic therapy first.
Send my prayer to you,
Daniel D.

I agree
Maybe next scan ?

Skin mets are unusual in ASPS. Can they biopsy these new things? There are known numerous cutaneous adverse reactions to immune-checkpoint inhibitors.
Is the primary tumor resectable overall - what is the size?
There is a dr in Spain that performs intratumoral injection of the immunotherapy with the following cryoablation. The primary tumor is to big for this though, and probably for everything else except the surgery.
There is an article about melanoma patients undergoing treatment with immune-checkpoint inhibitors, supporting the idea why the surgery to remove the large primary tumor would be beneficial:
They say:
"the initial tumor burden influences the response to treatment: small tumors respond better to treatment than larger tumors"
Response of patients with melanoma to immune checkpoint blockade - insights gleaned from analysis of a new mathematical mechanistic model.
https://www.ncbi.nlm.nih.gov/pubmed/31580835
we do not have a reason to expect that ASPS tumors would react differently.

Dear Olga
This is really calming us down to hear that skin Mets are unusually with asps. Biopsy was not considered for it right now which I thought is good because also this forum suggests to decrease any biopsies if not absolutely necessary.
Right?
We will try to get other opinions about. Surgery of the primary and will speak to the surgeon personally at our hospital in Frankfurt why he denied. Oncologist said that he was of the opinion that it is too big, too many muscles involved and so he will not be able to make a R0. 14x8,4x6cm tumor with a little serom surroundings.
But as far as I understand all your statements it would be good and reasonable to decrease the tumor load anyway (also if this means to maybe have another surgery after a year or so again?) to give any medication and treatment the chance to do its job on the smaller tumors/Mets?!
This is absolutely logical to me as we have several tkis plus the Keytruda which didn't do a thing on the primary yet.
We pray it worked for the lungs. Let's see on 16th.

Is there only the clinical study on melanomas you sent? Unfortunately nothing for asps patients? What is the hint to compare melanomas and asps to have an argument?
Our oncologists is only convinced by clinical studies.
Or can you let me know the percentage here at the forum of all patients where treatments worked much better when primary was removed?

What do you think of radiation which we start next week and if lungs are stable to first remove primary(if somebody is able to do so) and then decide on further treatment? Going ahead with Keytruda or trabectedin....

Thx
Tanja