Tok from South Africa - Dx around 2004

[Olga]

Marietjie, it is very typical that the hair are starting to fall out after the second cycle of the traditional chemotherapy. Tell him not to shave the head in attempt to look better. If he does that, the remaining roots are left inside and people say it is very itchy when they die from the chemo and need to go out, it is much easier for them to do so when the hair continues to outside. Tell him also pay a very close attention to the amount of the liquids he consumes, the regimen is very toxic and needs to be flushed out, it is a frequent occasion when the patients on chemo are given an IV liquids when there is a toxic build up.

[MarietjievdMerwe]

Olga,
We were worried about him shaving his head and will advise him not to do so, thanks! It does make sense if you think about it. In a weird way we are looking forward to seeing dr Cohen tomorrow. Is this a normal reaction? I wonder.
I will post an update tomorrow evening when we got home.
Thank you for caring.
Regards.

[MarietjievdMerwe]

Hi everybody!
News on Tok is that his bloodworks this morning was very good and he is busy with round 2 of chemotherapy. He is in very good spirits and is so positive. We saw dr Cohen but...... he is not keen on using anything else except the MAID treatment for the moment. It seems like there is Sutent (?) available in SA, but he is a little bit apprehensive about using it at the moment. Tok will have a CT scan after the next round of chemo and depending on the results of the CT, dr Cohen will "alter" the meds. And he is also not considering a brain MRI at the moment. We will again discuss this at a later stage.
Unfortunately there isn't much to tell, except my brother is the most positive person alive!
Keep well and let Hope and Faith guide you.
Kind regards.
Marietjie.

[Ivan]

Mariejie, is it possible for you to get another doctor? This one doesn't seem to know what he's doing.

[MarietjievdMerwe]

Dear Ivan,
Unfortunately this is Africa my friend. I contacted a dr in Cape Town who is currently doing clinical trials, but not for ASPS. Almost any other cancer, except ASPS. There are 8 oncologists in their practice, but as ASPS is so rare, so also couldn't give me any additional info.
At the moment it feels like we just have to bite the bullet and go with the MAID treatment. There is just no other alternative.
I am as frustrated as you are, but this is what we are stuck with.
I wish I had better news.
Thank you for caring.
Kind regards.

[Ivan]

Well, I really think that a brain MRI is very important. We've had a number of people die early because of brain mets which are otherwise very treatable - we also have number of people who had their brain mets successfully treated when found when still small.
There are some supportive articles that you can print out and discuss with an oncologist:
1.Brain metastases in musculoskeletal sarcomas.
http://www.ncbi.nlm.nih.gov/pubmed/10379335
"Patients with alveolar soft part sarcoma, Ewing's sarcoma, rhabdomyosarcoma and pulmonary metastases have a high risk of brain metastasis."

2.Sarcoma metastatic to the brain: a series of 35 cases and considerations from 27 years of experience.
http://www.ncbi.nlm.nih.gov/pubmed/20039192
"The authors conclude that surgery is more effective in treating selected patients with sarcoma metastatic to the brain, and that patients with metastasis from ASPS have good prognosis when submitted to surgical treatment."

3. Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults.
http://www.ncbi.nlm.nih.gov/pubmed/16410131
''Metastatic sites included lymph nodes, lung, and brain."
Good luck.

[MarietjievdMerwe]

Ivan,
Thanks a million, I will print this and will discuss this with dr Cohen when Tok sees him again.
Again, thank you for caring.
Marietjie.

[Bonni Hess]

Dear Marietjie,
I am very sorry and concerned about Dr. Cohen's unwillingness to schedule a brain MRI for Tok. It seems very irresponsible of him to refuse to consider this vitally important diagnostic procedure. If he has any knowledge about ASPS at all, he would know that early diagnosis of ASPS brain mets is critical to a successful treatment and outcome, which I can personally attest to. Since he is so determined to continue with the MAID treatment rather than at least considering other systemic treatment options, has he provided you with any documented data regarding treatment success for ASPS patients which would justify his inflexible attitude on this issue?
I share your great frustration with the limited access to Sarcoma specialist care for Tok in Africa, but given that this is unfortunately the situation, I encourage Tok and you to remain as pro-active and as well researched and informed as possible, and to continue to reach out to this Board for information, input, and support. Take care dear Marietjie, give Tok my very best wishes, and know that you are both held very close in my heart and most caring thoughts.
With deepest caring, healing wishes for Tok, and continued Hope,
Bonni

[MarietjievdMerwe]

Dearest Bonni,
It does my heart of hearts good to experience all the care and Hope from you guys. I will most certainly discuss this with the oncologist again next time. If, God forbid, there are mets showing on the brain MRI, what then? What happens then?
He doesn't even consider using any other treatment. Sad. I am a bit concerned about Tok's lungs. After the third day of chemo, he starts coughing a lot. Nobody seems to be concerned about this but it still worries me. Could this be from him lying down the whole time, which is also frustrating for me as we know that he must be moving around, but he seems to just want to lie down?
He was also very very sick after this second chemo. Fortunately his bloodworks are ok again.
What I also want to know is: what happens after the sixth chemo session and the mets are still alive and well?? What do we do then?
Thank you for caring and keeping in touch.
Hope you have a wonderful day.
Regards.
Marietjie.

[MarietjievdMerwe]

Dear Bonni, Ivan and Olga,
I just have to say that despite all that has been said, I still have the utmost respect for dr Cohen. He is a very kind and gentle man and I'm sure he is doing the best he can for Tok. It is just unfortunate that we do not have any clinical trials for ASPS at the moment, but just maybe there will be a light at the end of the tunnel in future! I do believe that anything's possible, even in Africa.
Be sure that we will discuss the brain MRI next time. Will keep you posted.
Kind regards. Groete (it means "regards" in my homelanguage Afrikaans:) )
Marietjie.

[Bonni Hess]

Dear Marietjie,
I am grateful that Dr. Cohen is a kind and gentle man who you respect which is very important. He is probably doing the best that he can within the constraints of the medical system that he is working with, but it is still very important that he be as knowledgeable as possible about ASPS so that he can try to better tailor Tok's treatment to the unique aspects of this extremely rare disease which requires specific diagnostic tests, such as a brain MRI and not just a brain CT, and which unfortunately has not been shown to have a very successful response to traditional oncology treatments such as MAID. Olga posted an excellent recently published article on this Board today ( May 11th) in the Medical Publications forum under the topic New General Review article about ASPS.If you have not already done so, I would strongly encourage you to read this article and to share it with Dr. Cohen. It provides invaluable information about ASPS. The conclusion of the article states " At this point, surgical methods are the most efficacious means of disease treatment; there are no convincing data in support of conventional chemotherapy or radiation therapy. New molecular therapies targeted to tyrosine receptor kinases and antiangiogenic agents have yielded promising data thus far, and these next-generation therapies may soon comprise first-line treatment for this tumor type." This conclusion is very relevant to the use of conventional chemo like MAID, and is certainly something that Dr. Cohen should at least be aware of. Of course, there are always exceptions to the rule, and each patient may respond differently to the same treatment, so I am VERY Hopeful that Tok and Dr. Cohen will prove the data wrong and that Tok will have a very successful response to his MAID treatment with stabilization of his disease and tumor shrinkage. Like you said, anything is possible. It is important to keep believing that and to hold tight to continued Hope, but it is equally important to be aware and as informed and knowledgeable as possible.
I am also VERY Hopeful that when Tok has a brain MRI that it will not show any brain mets. But if the scan does heartbreakingly show brain mets, there are a couple of possible treatment options depending on the location and size of the mets including resection ( surgical removal) of the mets, or radiosurgery such as Gamma Knife which is most effective if the mets are smaller than one centimeter which is why early diagnosis is critically important. Whole Brain Radiation (WBR) has unfortunately not proven to be successful because ASPS is so radiation resistant and the radiation can't be delivered in high enough doses with WBR due to the risk of destroying brain tissue. Hopefully,Tok will not develop any brain mets which would necessitate the use of this information.
I am so sorry that Tok is suffering from the negative side effects of the MAID. Is Dr. Cohen prescribing any medications to help alleviate some of the negative symptoms? Medical marijuana or prescribed marinol may help with the nausea if this is available in South Africa and if Tok is amenable to using it. Regarding your question about "what happens after the sixth chemo session and the mets are still alive and well?? What do we do then?", Hopefully this will not be the situation, but if the MAID is heartbreakingly unsuccessful, then you will need to discuss different treatment options with Dr. Cohen and have information available to present to him regarding the best possible options based on the status of Tok's disease in terms of rate of growth and size of his mets and development of any new mets. In the meantime, I continue to hold Tok and you very close in my heart and most caring thoughts, and to send my most positive thoughts and very best wishes your way for treatment success and healing.
With special hugs, deepest caring, healing wishes for Tok, and continued Hope,
Bonni

[Olga]

Marietjie - I am glad to hear that Dr. Cohen is a kind and gentle man so I hope that he is able and willing to educate himself on ASPS, so you can bring this latest review to his attention. I actually disagree with Ivan re. "change the oncologist". There are very few dedicated sarcoma oncologists since sarcomas are much rare than cancers and between them are very few that are knowledgeable in ASPS as the usually catch up as the need arises and most of them do not get to see any ASPS patient at all. It is not only in South Africa that there are no sarcoma doctors and ASPS dedicated trials but in many other places and we have to find a way to optimally deal with it with what we have. In Vancouver, Canada where we live, we also do not have a dedicated sarcoma oncologist and no clinical trials for ASPS either, we just were lucky to get a very open minded oncologist and an advanced interventional radiologist, that's all.
How is his cough? Is it better? How big the remaining lung mets are? How long ago did he have the lung surgery? In some cases there might be a pleural effusion as a result of the chemotherapy, so if he still coughs, he might need to have an X-Ray. Did he use the breathing training device after the surgery - the plastic one with the level in it to see the volume? If he had it (and still keeps it) he could do a test to see if his breathing volume is OK.

[MarietjievdMerwe]

Dear Bonni and Olga,

I have printed all 9 pages of the article and will give it to dr Cohen for reading, thanks a million! Olga, i am sure he is a very openminded person and i do believe he will read the article. Maybe it will only take one patient's family to change his mind and use another method of treating ASPS.

Tok's cough is much better, thanks. He did have the device, but i am not sure if he is still using it. Will sure be following that up on that one. He does have a oxygen system at home and we do encourage him to use it. I am sure he's feeling better after using the oxygen. Unfortunately i only see him every three weeks and cannot be with him during the day. Things would have been much different.

Olga, he had the lung op in Jan 2012 and only had the CT in March (?) just before his first chemo. By then the mets have grown from grains of sand to ,9mm. That was the biggest one dr Cohen meassured and will be using as his reference (?). Still not sure how many there were as he did not tell us.

I am also thinking of opening an Afrikaans (our homelanguage) forum on Facebook, just to hear other people's stories and again just maybe, something good will come from this. If there is enough pressure from the families of ASPS patient's, maybe our government will also take note and do more for our loved one's.
You are of great encouragment to me and i cannot thank you enough for your input and support.
I will not give up on Hope.
Groete.
Marietjie.

[Olga]

Marietjie - is the lung mets size 0.9 mm or 9 mm? Anyway, it is not the size that could affect his breathing, usually people with the mets like this are able to breathe normal. And he should be able to not use the home oxygen for now - most of our patients breath on their own in a few days after the surgery even the extensive one, they have to exercise good use the breathing trainer frequently and correctly - gently elevating the ball to a proper level, walk around and increase the lung capacity. His coughing might result from overusing the oxygen, but of course I am not a doctor and not aware of all details of his surgery and his condition, but it is unusual to use an oxygen for so long after the surgery. Sometimes there are some specialized doctors or respiratory rehab clinics that address the conditions connected to a lung surgery.

[Feng zhou]

Usually, prolonged time cough is due to lung inflammation in patient with lung lesions. you can do X ray chest film examination and routine blood test, to determine if there is a presence of the pulmonary infection and whether to use antibiotics.