Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi Everyone, I soooooo wish I didn't have to be on this ASPS Forum. However, since life isn't fair & I am here, let me tell you how incredibly grateful I am that you're also here sharing your knowledge, experience & compassion with those of us who are just beginning this horrific journey.
My name is Bridget Agnello & I’m the single mother of 13 yr old Joseph. Joe was diagnosed with Stage IV metastatic ASPS on June 27, 2015 when he was 12 years old. We live in Rochester, Michigan (USA), 30 miles north of Detroit. He’s a patient of pediatric oncologist Maxim Yankelevich, who’s affiliated with Children’s Hospital of Detroit.

Joe had a 7.5cm tumor excised from his right, posterior thigh on June 19th. With less than a 1mm margin of clearance, his pediatric surgeon took him back into surgery on June 29th & in all, removed approximately 35%-40% of Joe’s right quadriceps. His pediatric surgeon is Dr. Nathan Novotny, an extremely gifted surgeon as well as a genuinely supportive, caring person! He is the reason that Joe did not have to have his leg amputated nor did he have to have any radiation therapy to this primary site. Joe still has some residual weakness in the leg at the end of the day, but otherwise you can’t tell from seeing him walk what happened to his leg.

On June 18, the night before Joe’s first emergency surgery, he underwent the following tests:
1. Lung - X-ray
2. Femur – MRI
On June 30, after his second surgery, Joe underwent the following tests:
1. Full body PET scan
2. Brain CT scan
3. Lung CT scan
At the time of his Dx, he was a patient at Beaumont Hospital in Royal Oak, Michigan. The Chief of Pediatric Oncology told us Joe has multiple bilateral lung lesions (approx. 15), with the largest ones being a 6mm in the right upper lobe & a 4 mm in the left upper lobe. Beaumont Hosp. has never treated a case of ASPS, so we were referred to St. Jude Children’s Research Hospital in Memphis, Tennessee. Joe was evaluated by Dr. Alberto Pappo, who is touted as the world’s authority on ASPS. (St. Jude has treated only 11 cases of ASPS in the last 30 years I found out later.) Pappo said St. Jude couldn’t do anything for Joe that we couldn’t get done back in Detroit with Dr. Yankelevich. So he recommended Joe begin 37.5mg of Sunitinib.

Pappo & Yankelevich both told me that there was nothing we could do about the tumors in Joe’s lungs. Apparently the amount of radiation his lungs would need to kill or shrink his tumors would also destroy the tissue. Joe not only has surface tumors, but he has them deep in each lobe. Upon receiving a PET CT from Children’s Hospital on October 16th, Dr. Yankelevich corrected Beaumont Hospital’s diagnosis of approx. 15 pulmonary tumors, and told me that there were too many to even count.

Even though I’ve been researching ASPS since the moment of my son’s diagnosis, I realize now that I let myself get lulled into a fall sense of security with the Sunitinib use. Four months after his Dx, he doesn’t appear to have any disease progression. But who knows if that’s because of the ASPS is in a stable state right now or if the medication is working. I have to say that I hate what the medication does to Joe. He’s had bouts of uncontrollable nausea, vomiting, diarrhea & migraine headaches (spent Sept. 2nd -4th in the hospital on Zofran & Morphine). The palms of his hands and soles of his feet have peeled, cracked and bled; his hair has begun to fall out while the remaining hair follicles on his entire body are turning albino white; he is retaining fluid and is perpetually puffy; and he’s developed acute arthralgia (arthritis) of his knees, ankles and hips. Five weeks ago I had him at the ER in a wheelchair because he couldn’t walk. He was in tremendous pain and he said it felt like he had 50lb weights attached to each leg.

After the arthritis, the edema & the hand/foot bleeding side effects all presented at once (the ER visit I wrote of), Dr. Yankelevich D/C’d the Sunitinib for 8 days. The side effects for the most part disappeared, except Joe hasn’t lost all the all-over edema. Dr. Yankelevich started Joe back on 2 pills instead of the full dose of 3 pills, and so far so good. Yesterday was the first day Joe began taking 3 pills again. I’ll be keeping a close eye on his symptoms. If he goes toxic again, he’ll either go to 2 pills and remain there or be switched to Pazopanib.

In all of my sleepless nights since this started, I found Dr. Rolle and all of you and Dr. Novotny has reached out to him. He’s preparing all of Joe’s scans to send to Dr. Rolle next week. Most of our doctors are telling me that Joe’s tumors are so small, that I shouldn’t put Joe through more surgery. But I feel as if I need to lessen his tumor load. Do you think this is too hard of a surgery for a child? Joe has no other medical conditions except for controlled Asthma and I worry about his lungs’ ability to heal.

Has anyone on this forum researched Non-Invasive Ultrasound Surgery? It was pioneered in Israel for brain tumors and has now been adapted for other parts of the body. Joe’s pediatrician is researching for me where else it’s being performed and its success rate.

Are there any other children on this forum with ASPS? I haven’t been able to connect with anyone else in Michigan who has this god-forsaken disease. I’ll write more later… too sleepy to continue for tonight. Thank you again for being here and listening and offering advice!

Love....Bridget )

Bridget, hi,
We are glad that you have found us? and I am very sorry to hear about one more kid diagnosed at the such young age. About 1/3 of the patients here are kids - mostly teens, the rest are young adults. My son was Dx in Nov.2003 when he was 17 years old, he is 29 now, he had the surgery with Dr.Rolle *times and yes, his drs here were also telling me not to go for it - offering nothing really instead.
I am very sad to hear that sunitinib treatment is very tough for Joe, with his body weight he may be needs to be on the reduced dose? 37.5mg is the dose that our young adult patients are having. You should find out exactly if it is working - wait till the next scan and ask them to compare it to the base line scan done before he started Sutent (not to the scan before). Wait to hear from Dr.Rolle, make sure he knows about asthma. Then you can decide what to do - after you get all the info.

Olga thank you for responding and thank you for making me chuckle! Joseph may be a "kid" in age and behavior, but certainly not in physical appearance. He's 5'8" tall and he has a size 13 shoe (sorry - don't know how to convert that). He was a stocky, muscular kid into tennis and baseball before this all started. He began 2015 at 5'3" and his growth plates indicate that he's got another 4"- 6" to grow.

I wish I could give him his childhood back, but that flew out the window in June. He's actually handling all of this pretty well. I'm the one that feels like I'm flying apart at the seams. I'm terrified that I will choose the wrong course of treatment for him and shorten the amount of time that I'll have him with me. I'm not sleeping well (I tiptoe into Joe's bedroom to check on him), I've lost 28lbs since the end of June, I have anxiety attacks all the time and my hair is falling out in gobs right along with Joe's. Yes, I know I can hear all of you telling me I have to get a grip...and I'm trying. Joe never sees me fall apart, but I'm SCARED TO DEATH ALL THE TIME! I cannot conceive of a life where my only child is not with me.

How are all you parents coping with this? Medication? Yoga? Therapy? Apologies for rambling off into the weeds....

Bridget (who's for real going to bed this time)

Hi Bridget

My name is Debbie and our son Josh was dx'd in 2012 at the age of 32. His daughter was 4.

We are all so sorry to hear of Joes becoming part of an ASPS life group . However in having ASPS , the forum brings so much to the patient and families table pertaining to this rare cancers information


I agree with Olga on talking to your oncologist about the idea of reducing the dose and maybe working your way back up with sutent.

Also you might want to perform a brain MRI at some point to have a better base line picture , if you will , for future comparisons as this is a lives commitment and scan commitement is a must. The scans are, most of the times, the way we discover ASPS as it normally has VERY LITTLE if any symtoms.

Wow Joe sounds like a growing machine
The growth platelet study is interesting

I hope your nights sleep brought you in a better place
In regards to anxiety , rest or sleep . It's VERY important. Also eating well
When the boipsy came back ASPS the first week in September after the removal of Joshuas left leg 6.8 cm primary , and I hung up from talking to him. I went outside I screamed and screamed
Life sure sucks on some days. No other way to put it

You can always reach out to any of us anytime.
Hugs and love
Debbie and family

Hi Debbie,
Thank you for your very supportive reply--I appreciate it! I've been following Josh's story along with several others since I discovered this website. The bravery and the fortitude and the perseverance of all of you in the face of this disease year after year simply blows me away. I pray every day that I have the strength to help Joe endure whatever comes our way over the course of this journey.

I forgot to mention that Joe did have a brain MRI on September 3rd when he was hospitalized for 3 days with uncontrolled vomiting and an unrelenting migraine. No mets to the brain at that time.

Good news Bridget on brain scan !
I'm not meaning to scare you but never under estimate the headaches. Brain tumors can and will grow very fast .

Thank you for your insight and comments !

The bravery and fortitude are the results of our families and friends support, and have been God sent .
I hope your family is there for you too.
Love
Debbie

Bridget - all parents here can identify with what you are saying about constant panic and fear for the kids - keeping the brave face on and screaming driving alone in the car. As the time goes it does not get any easier as the battle with ASPS gets more and more challenging - it is like a survivor game when passing each level just means that the conditions get more complicated and you move to a next level with more of everything flying into your face and you need to react fast and fight harder and harder. Use every moment of you "close to normal" life between the treatments as a celebration of life, appreciate the moments of the regular being as this is the best you are going to have. I do not use any medication or therapy as I am afraid that it will reduce the level of urgency and attention I give to all of this ASPS situation and I would became compliant with what drs offer - they often are trying to steer as toward the Quality of life and watchful waiting approach - the things they say when they do not have anything to offer. In our experience it just means that you have to rapidly expand your field of search for the best solution outside of the local team, may be outside of the country, may be change the approach, may be look in other areas of medicine overall. There is usually no time for the depression - you are ether in the relatively OK situation and frantically trying to enjoy life, going on vacation, concert, some events - small gifts of life - until the scans comes and all of the sudden something new awful discovered and everything collapses and you are having a jump start - get the copy of the scans, look for the drs who are the experts in the field with some unique approach, read what was done by whom on the Pubmed to beat this problem, contact them - better to contact 3-5 experts all around the globe to hear all the opinions. Then come the treatment, the travel, the pain and the recovery - which is equally important - to gain the QOL back and to be prepared to meet whatever comes next in a best shape - for everyone, not only for the patient. We took on cycling as a regular exercise 3 years ago - Ivan does lots of sports, other people run, do yoga, play tennis - you pick the activity that suits you best, but this is what you need. The mechanism of coping is different for everyone.

And Bridget
In thinking more on your comment of perseverance etc
I forgot to include the kindred spirit that is offered by folks like Olga and others on the iCureasps forum .

Well put ,Olga.
Debbie

Dear Bridget,
My name is Bonni Hess, mother of now 33 year old Brittany diagnosed 14 and a half years ago in July 2001 at age 19. I am so deeply sorry for your dear young son Joseph's ASPS diagnosis and all that he and you have already been through physically and emotionally, but I am very grateful that you found your way to this Web site and that you have now reached out to those of us on this Discussion Board for shared information and support. I am very grateful too that you have been so pro-active in your aggressive approach to fighting this very challenging and extremely rare disease, being as knowledgeable as possible, and seeking medical opinions and advice from several different doctors, all of which is critically important. Olga and Debbie have offered you some excellent advice and insights which I concur with. As Olga has said, we sadly have several other young ASPS patients on this Forum. The youngest patient is Brinley, who was age two when first diagnosed, is now six, and thankfully remains stable with no disease progression or metastasis after having had her primary pelvic tumor removed. Our most recent pediatric patient is nine year old Prairie from Nebraska who was diagnosed in August and you can read her story on the Personal Updates topic. Sunitinib treatment is also being considered for Prairie and I am sure that her mother Mary Jo would like to communicate with you about your shared experiences. Has Cryoablation been considered/discussed to treat and destroy any of Joe's largest or most concerningly located lung mets? My heart breaks for all of the harsh side effects that dear Joe is experiencing with his Sunitinib treatment, all of which are very similar to the side effects that our daughter Brittany suffers with her Cediranib treatment (Cediranib is another Tyrosine Kinase Inhibitor (TKI) similar to Sunitinib). Was Cediranib discussed/explored as a possible systemic treatment option instead of Sunitinib? When Brittany began her Cediranib Clinical Trial six and a half years ago, she was on a 45 mg. daily dosage which caused extremely severe side effects so her dosage was reduced to 30 mg. per day which did help to reduce the very debilitating symptoms, but unfortunately it did not completely resolve or alleviate them. Because of the chronic nausea and resulting dehydration caused by the Cediranib, Brittany was developing severe headaches and uncontrollable vomiting which required emergency hospitalization and treatment with re-hydrating IV saline as well as IV Zofran and Dilaudid every two to three months. To try to prevent these episodes which were taking a huge toll on Brittany's dear body, we requested that she receive outpatient IV rehydration every two weeks which thankfully has been very successful and she has not had any of the emergency hospitalizations since beginning the preventative rehydration regimen a couple of years ago. It is important to note that it was US instead of the doctors who made the ( seemingly obvious...) observation about the advisability of scheduling preventative hydration instead of waiting for severe headache and vomiting symptoms to begin. This clearly illustrates why it is SO important to be proactive and vocal because the doctors don't always know everything or provide the best medical direction or advice. If Joe is experiencing severe headaches and nausea from dehydration caused by his Sunitinib treatment, I encourage you to pursue and request regularly scheduled preventative outpatient re-hydration. Regarding coping mechanisms for parents of ASPS patients, I Hope that actively participating on this Forum will provide you with a source of strengthening support and encouragement, and that you will continue to reach out to those of us who share this difficult battle with you and so deeply know and understand your immense pain, sorrow, anguish, stress, anger, and fear in addition to having invaluable researched and anecdotal information to share with you. Please know that you are not alone dear Bridget and that we are all here to try to help in any way that we can with shared information and support. Feel free to write me ( BonniHess@aol.com) or call me (425-829-3210) any time that you may need to talk or just have someone listen who truly understands what you are going through, and knows your journey. Stay strong, let the bright light of Hope lead you through each day, and keep in touch as you are able.
Reaching out mother to mother with special hugs, caring thoughts, healing wishes for dear Joseph, and continued Hope,
Bonni Hess
P.S. Olga,who is our very dedicated and hard working Web site administrator as well as the mother of an ASPS patient, will probably move your post to the Personal Updates topic where it can be more easily followed by everyone

Dear Bridget,
I am so grateful to have been able to personally meet you and share some special hugs and time together at the recent very informative ASPS and Sarcoma Days of Learning Workshops in Miami. I wish that the full speaker schedule and busy days would have allowed us more time to talk with each other, but I am so glad that we have this Discussion Board on which to continue our discussions and sharing of information. I know that you have been very busy with the demands of Life and your precious Joe's ASPS battle, but since it has now been almost a year since you last posted on this Board, I Hope that you will be able to post and update about dear Joe's current situation sometime soon when your busy schedule, limited time, and the situation allow. Since our time with each other was so limited, I am not clear as to whether or not Joe is still receiving Sunitinib treatment and what the current status of his disease and treatments are. You told me that Joe is heartbreakingly emotionally struggling with the harsh realities of this insidious disease, and I Hope that he will be able to connect with and communicate with some of the young men on this Board who are also battling ASPS since the boys/young men and girls/young women seem to cope with it differently and boys/young men are usually less open and communicative about their feelings than the girls/young women. Please take care dear Bridget, stay strong, keep in touch as you are able, know that there is strengthening support and encouragement available for both you and Joe on this Forum as we are all sharing this difficult journey together, and as your wonderful shirt so appropriately said, "F*** Cancer", and may I add, "F***ASPS"!!
Sending special hugs and reaching out heart to heart to share caring thoughts, healing wishes, much love, and continued Hope,
Bonni

Hi Bridget
Could you give us update on Joes dendritic procedure and follow up determinations ?
Even tho maybe not great news , it maybe helpful for someone later?
Hugs and love
Debbie