Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hey all,

This is Connie, Kevin's girlfriend. Kevin was diagnosed with ASPS in February of this year (2012). Much love to all of you who are fighting this disease, as well as to your loved ones who are in it with you. First off, I can't even begin to say how grateful I am for the existence of this site. Yesterday, we found out that Kevin's ASPS has metastasized to his lungs and we're still reeling from what this all means. I've been camped out on this site since then, trying to learn everything I can. I've read some of the linked studies before this, but I think hearing from actual ASPS patients is the most valuable information of all.

Kevin noticed a lump in his chest last fall and went to see our family doctor. She was concerned, one thing led to another, eventually Kevin was recommended to seek the opinion of a surgeon at UCLA Medical Center. The surgeon believed the tumor to be a desmoid tumor (a relatively benign cancer) and it was removed in February of this year (2012). Only when they biopsied the tumor, did they realize we were actually dealing with ASPS. Luckily, they were able to remove the primary tumor with negative margins and he is currently finishing up local radiation for the area.

However, yesterday we met with our oncologist and he delivered the bad news that it appears from Kevin's latest CT chest scan that the disease has indeed metastasized to his lungs. The only good news is the nodules appear to be less in number than they expected with this disease, so they provided us with a ton of information (including this website) and put TKI drugs and Dr. Rolle's laser-aided surgery on the table as the best possible options.

We're kind of scared. Kevin just turned thirty, I'm twenty-eight. I don't think either of us know what to expect and our doctors tell us because of how rare this disease is, it is difficult to predict what Kevin's day-to-day life will be like living with this disease. At present, the nodules in his lungs are too few in number and small enough that they have no impact on his quality of life, but we're trying to prepare ourselves for if and when that changes. I read long-term prognosis is not favorable and it scares the daylights out of me, so any information on what we should expect and prepare for would be a godsend.

Hope and love,

Connie

Connie, hi,
I am Ivan's mom, Olga. Welcome here. I do not have a time to write a lot right now, but read my son's story first. I strongly advocate the surgery with Dr.Rolle before of going into TKI as they only work temporary and the surgery can fix this if Kevin gets lucky and the mets stay low in number. It is better to do it early as Dr.Rolle may retire at some point soon and the other thing is that try to keep the tumor load low as possible. You can send Dr.Rolle a CT on a disk. He will let you know his opinion. He kept Ivan mets under the observation for about 6 month before said that it is a time - to get all the mets visible. More later.
The quality of life is 100% normal with the surgery after the recovery is over.

Dear Connie,
My name is Bonni Hess. I am the mother of now 30 year old Brittany who was diagnosed with ASPS almost eleven years ago at age 19 in July 2001.
I am so very sorry for Kevin's recent ASPS diagnosis, but am grateful that you found your way to this site and that you have reached out for information and support from our ASPS Community members. I applaud you for being so pro-active in your search for ASPS information, and for being such a caring and supportive advocate for Kevin, both of which are so extremely important in fighting this extremely rare disease.
It sounds like the oncologists at UCLA are providing you with the most up-to-date and promising treatment options currently available for ASPS, and I am glad that they also provided you with a referral to this CureASPS Web site which is an invaluable source of shared researched and anecdotal treatment information and strengthening support and encouragement for ASPS patients and their families/loved ones from all over the world. Who is Kevin's primary oncologist? Hopefully Kevin has already had complete scans including chest/abdominal/pelvic CT scans, a full body bone scan, and a brain MRI (NOT just a brain CT which is inadequate to diagnose ASPS brain mets at the smallest most treatable size) because all of these scans are critically important and necessary in order to have an accurate diagnosis of the status of the disease and to plan the most effective treatment. Barring the diagnosis of any other mets than the ones currently seen in Kevin's lungs, I agree with Olga that the best treatment option at this time is laser resection of the lung mets to reduce tumor load which helps to strengthen the immune system to better fight progression of this challenging disease. Systemic treatment with a TKI like Cediranib, Sunitinib (Sutent), or Pazopanib ( Votrient) seems better reserved for ASPS that is aggressively progressing and/or tumors that are widely dissemnated and unresectable or untreatable with surgery,laser resection, radiosurgery, or ablation as was the situation with Brittany in 2009 when she began her Cediranib treatment. As Olga has said, TKI's have thus far unfortunately only proven to be temporarily successful for most ASPS patients. Thankfully Brittany has now had three years of disease stability and dramatic shrinkage of her multiple and widely disseminated mets since beginning her Cediranib Clinical Trial in April 2009, but her longer and more sustained response has been the exception to the average length of patient response before disease progression has heartbreakingly occurred in most of the ASPS patients taking Cediranib. We are painfully aware of this and continue our relentless search for a permanent treatment and cure. In the meantime, all of the current research data documents that removing or destroying tumors through surgery, laser resection, radiosurgery, or ablation remains the most effective way to treat and manage ASPS. Vigilant scanning with chest/abdominal/pelvic CT scans every 3-6 months, full body bone scans once a year, and brain MRI's once a year (if there have not been previously diagnosed brain mets which then requires brain MRI's every three months), is critically important to find and diagnose any new mets at their smallest and most treatable size.
I know that you and Kevin must be numb with shock and fear having just received the very difficult news of Kevin's lung mets diagnosis, and overwhelmed with all of the information which you are receiving and the treatment decisions you are facing, but please know that you and Kevin are not alone in this battle. I and the other ASPS Community members on this Board are here to help you in any way that we can with shared information, support, encouragement, special caring, and a unique understanding of what both of you are going through. Through our almost eleven year journey with Brittany's ASPS, we have found that shared information is one of our most powerful weapons in fighting this extremely rare and little known disease. This CureASPS site and those who participate on it are an invaluable source of that information for you and Kevin.
Please take care Connie, let Hope lead you and Kevin through each day of this journey, and keep in touch with this Board as you are able. Olga and Ivan who are the Web site administrators will probably move your post to the Personal Stories and Updates thread on this Board where Kevin's story can be more easily followed and your updates responded to by the other Discussion Board members.
Reaching out to share special caring thoughts, healing wishes for Kevin, warm friendship, and continued Hope,
Bonni Hess

Thank you all so much for sharing your stories and information with me. We reached out to Dr. Rolle via email but it looks like he's on vacation right now, so we'll ping him once he gets back.

As for Kevin's medical team, he was operated on by the Dr. Eilbers (father and son!), who removed his primary tumor. His oncologist is Dr. Noah Federman, and also includes Dr. Roach. I'm going to have to go back and track what scans he's had, but I do believe he's had a number of scans and MRIs since his primary tumor removal in February. I will cross-check your list with his paperwork to confirm, possibly email Dr. Federman to make sure. I will keep everyone here updated on our process as we go, will continue to go back and read through the boards for past information.

Olga, I read through Ivan's story and I can't tell you how great it was to hear firsthand account of the laser-aided surgery procedure. If you don't mind me asking, how is Ivan doing now?

Scratch that last question, just read Ivan's latest post from this May. So glad to see that he's doing well!

Connie, while you are waiting to hear back from Dr. Axel Rolle in Germany, you might send him a CD disk with the latest CT scan on it (and the scan before if he had 2), so he will see that in the mail when he is back from the vacation.

Good suggestion! I will do just that.

With that CD, you should include a page with the short description of his clinical history - name, age, address, when was Dx, where was the primary and what size, what surgery was done and outcome (complete/no), and if he had any other treatments or distant mets anywhere. Dr.Rolle generally takes only people with no other mets except lungs - although I know that he has done some exceptions - when the other mets were treated, controlled or there was a concrete plan in place that it is going to be done after the lung mets resected or ablated - sometimes the other doctors say:" well, if the lung were done, we might treat your other mets etc."

Great news all! We've heard back from Dr. Rolle and he has agreed to operate on Kevin's lung mets. Both our UCLA oncologist and Dr. Rolle recommended a staged treatment, so we will only do one lung this trip and schedule another after he has recovered.

It is a good news. I suggest you to plan to spend a day or two in Dresden before of the surgery, there are few very interesting museums and building in its historical center to visit.You can ask me or Ivan if you need to know anything before you go or alternatively there is a computer at the hospital to keep connected when you there.

Hey all,

This is Kevin and I have finally made the time to participate in this forum!

First I just wanted to say thanks to Olga, Bonnie and Ivan for posting such useful information about all things ASPS including the Dr. Rolle visit. Reading his journal has set up my expectations for my trip.

Dr. Rolle asked to see me on July 16th so we are currently planning on heading to Germany next Wednesday and trying to get a mini-vacation in before the procedure. Connie will be staying in one of the guest rooms.

My only concern was the possibility of collapsing my lung after the procedure. I usually run and weightlift a few days a week so I might have to cut that down for a while after i get back home. Is that a common problem with this type of surgery?

Kevin, it is hard to say how common it is - there are not enough people that had it in the world to make any stats. I think - ask Dr.Rolle though - that laser assisted surgery scars are weaker and easier to tear than the one that are made by the traditional stapler when the edge is rolled and stapled. It seems to me that the airleaks are longer after the laser assisted surgery so I would try to keep it safe for at least a month after the surgery - there are other types of activities you can do during that month that do not involve the sharp breathing movements - cycling, walking. Be very careful after the first lung surgery because having the other lung intact masks the limitations after the first surgery and gives you an impression that everything is almost OK in 2 weeks. Some denial always stands in a way of being carefull too.
If Conni is staying in the guest rooms above the admin floors - it is a very good choice. They are very basic but very convenient with the full kitchen avail. for the common use - there are only few rooms so the refrigerator and the stove are hardly used. The supermarket is in about 10 min walk so I did not rent a car - the area is extremely quiet and laid back with the hospital being the biggest employer in the area. The town is very small and remote to Dresden as it was set as an upscale mental institution a century ago - in a big park for a walk.
Some random tips for a trip:
- walk a lot as soon as possible, it is the best you can do there in addition to all the rehab they will force on you starting the next day after the surgery;
- ask the doc on the fllor for a few pain killer pills for a flight home;
- remember that you can request the Frankfurt airport assistance when going home at the airport of Dresden when checking in. There are long security lines in Frankfurt and long intra airport transfers so it might be easier if they wisk you away in that small electric car, just do not tell them that you had a lung surgery - only that you had a surgery in general;
- the internet in the hospital's lobby was coin operated when we went. If it is still the same you can get the coins at the reception, just give her the bills and say internet and she will break it down for you;
- most of Germans have at least some Engl skills, doctors are generally better speaking that the nurses. Ivan's favority male nurse was Matias and he speaks fairly well, ask if he is still there, he was great.

Great advice Olga, thanks. I will be extra careful and walk whenever I can. I do tend to over exert myself with false confidence.

I just ordered a TEP wireless portable wifi device to use while we are in Germany. It can connect up to 5 unique devices ( our laptops, iphones/ipads ....netflix! ) The idea of coin-op internet scares me more than the recovery itself.

I am almost sure they won't have any wifi internet at the hospital, if anything it interfers with the stuff they use there...Coin operated internet will make you to walk downstairs few times a day...I hate it too but there is nothing you can do! We used a laptop in the room to watch movies though, take headphones for it. Are you going to pay for the private room? The semi-private will give you a German speaking patient watching the movies in German, nothing bad with it:( We had the private room everytime we had to go there. They feed you 3 times a day at the hospital but I would always buy some add food at the supermarket or at the small meat/deli store on the right side by the entrance to a supermarket, there is a refrigerator for the common use on the floor as well but you have to mark the food by the name and the date.

We will be thinking of you and keeping you in our prayers. My husband Tom's experience was a very good one. It was some of the best care he has had! We loved the area. The walk to the store was not bad at all. It is a nice campus and town in general. The coin operated system was not so bad. It got us out of the room and enabled us to communicate with all of your loved ones. We set up a caring bridge site in order to keep everyone up to speed on how he was doing.
We loved 2 of the nurses - Marcus and Susie - If you see them, please tell them that we said hello!

Best wishes,
Kathy