Waking Up

I wanted to share that our big week of treatment is over. Hans received 5 days of radiation and 5 days of Irinotecan/Temodor. You can already SEE the results! The bump on the top of Hans' head is almost FLAT!

His vision is improved (his eyes are less crossed and he is tracking the doctor's finger tests better), but he still says he is seeing double.

Hans pretty much spent last week asleep and not eating at all. He would actually throw up several times a day, after each of his little meds. He already looks better, awake to watch Saturday morning cartoons at least, but the appetite may take a day or two to come back.

He has only had one complaint of a headache (no more back pain) all week, so we have been weaning him down and off the IV Methadone. He should be on an oral pain med by Sunday or Monday. I am hoping the back pain doesn't come back once he is off the Methadone. He is also being weaned off the Decadron and will go back to his Hydrocortisone tomorrow.

Hans has his MRI of the spine on Monday morning (with Sedation). We must be inpatient to get that slot! Sooo... we will have a mellow hospital weekend. Today and tomorrow will be his first two days that Hans won't have to have any treatment or tests in TEN STRAIGHT DAYS, so we will look forward to it, and hope to be fetching food requests far and wide as his appetite returns.

My mom is going to go ahead and go home for now. Her BFF Pat flew down to make the road trip back up with her. Thanks, Pat!

This week Elle got registered for school, and she is trying out with a couple of different soccer teams. Kevin was able to take three days off this week and has been knocking out our major DIY flooring project and spending time between CHLA and Bakersfield. This is a crazy time, for sure!!!

Mid-Week Update

I'm sorry to have kept you waiting.

There is good news and bad news.

The good news:
Hans' bone marrows were negative for Neuroblastoma!
Hans' kidney function is normal!
Hans' CT scan was "unrembarkable" (Yay - no bulky disease!)
Hans has now had three days of radiation and chemotherapy (thank God we are giving this kid some treatment!!)
They are radiating two spots on his head and they are giving him IV Irinotecan and oral Temodor. His Radiation therapy is scheduled for 12 sessions m - f, which he can do on an inpatient or outpatient basis. His Irino/Temodor is on a 21 day cycle, he gets chemo the first 5 days and has 16 days off. At this point we haven't planned any farther out than the first cycle. We will make our additional plans shortly.

The bad news:
THe MIBG looked worse than the one we did in Philly on July 14th.
There are more spots on the head (consistent with MRI and what we anticipated to see).
The spot is still there on the hip - this is the new one that showed up in Philly on July 14th.
There are a couple of question mark spots on the spine.
We will do another MRI to confirm these or rule them out.
In all honesty, with Hans' pain, and with the rapid growth of what we can actually see on the skull, I was bracing for worse news on the MIBG.

Hans is having a rough week! He is just wiiiped out. The pain team actually switched him out to IV Methadone for the pain in his back:( With the combination of all three (methadone, radiation, and chemo) he is just sooo sleepy and has no appetite. He has had nothing to eat since Sunday night. He is being very closely monitored by the endocrine and pain teams. The good news: we are weaning him back to a lower dose of methadone and a lower dose of his super high dose steroid, decadron. We are all hopeful that we will have home/oral dosing in place for a possible weekend discharge.

Our Outlook: We are treating all of the NB with the combination of Radiation and the Chemotherapy.
We are optimistic that Hans will respond. In fact, I am putting all of my eggs in that basket. I don't have another basket.

Emotionally we are pretty fragile. This has all happened so fast and it's terrifying. I am sure I take out my emotions on the staff here. When something goes wrong, and for some crazy reason we have had at least a half dozen communication errors, I freak! I actually sat our attending and fellow down and gave them a little presentation on those errors. Since then, I have been treated like the Duchess of York! Oh, I also fired one resident from coming into contact with Hans. We are sort of fragile, in a war zone. A little PTSD going down. I'm trying to chill out!

But, I have to say that we have found in Dr. Marachelian everything we could need or want from a local oncologist. I am satisfied beyond my wildest expections. We are in communication with Dr. Maris and Mosse, and the two teams continue to communicate about their decisions for Hans.

Thank you for checking up on Hans and thanks for your thoughts and prayers! Please put all your eggs in one basket along with us.

Monday Can't Get Here Soon Enough

We are looking forward to tomorrow because Hans will finally start on a therapy to treat this new NB! Tomorrow Hans will have a simulation for XRT (radiation), and he will begin both Radiation Therapy and Irinotecan/Temozolomide (chemotherapies). They are scheduled together because Irino/Temodor is a radiosensitizer and they work better in conjunction with radiation. It seems like so very many days have somehow passed us by since we first got news of progression back on July 14th. We can only charge full speed ahead and move forward from tomorrow.

What has been happening? Hans had an opthamology exam on Friday. They believe that the pressure from the mass on the skull is causing the double vision. They also believe that as soon as the mass is treated the pressure will be relieved and the symptoms should go away with no long term effects! We also had a consult with radiaiton. The Radiation Oncologist believes the mass should respond well to this therapy as "neurobalstoma usually does" to radiation.

Our oncologist consulted with Neurology and Neuro-opthamology and determined a lumbar puncture shouldn't be necessary at this time. A lumbar puncture would detect NB in the Cerebral Spinal Fluid (if I understand this correctly). At this point, that is not indicated, this does not appear to be a CNS (central Nervous System) replase. If it did, our path would likely take us to a well known treatment in NYC, but at this time we do not have to go down that road. Our oncologist also ultimately decided against an biopsy of the skull lesion. She felt that it is pretty clear that this is NB, and that he could face the risk of infection or complications as he is about to go into radiation of this area.

Yesterday he had a CT of the neck chest abdomen and pelvis. We do not yet have those results back. They are doing a full work up of Hans this week. Today he is doing a urine study to test his kidney function. Tomorrow he will do another Bone Marrow Biopsy and Aspiration. Tuesday/Wednedsay he will do another MIBG. We will be inpatient until at least Friday so that Hans can be monitored for his first five days of Radiation and Irinotecan/Temodor. I am not sure how many days total we will be here for radiaiton, but it could be anywhere for 10, to 12, to 20 on an out-patient basis after the first five days.

Hans has been having some back pain in the last 36 hours:( He is getting IV morphine to control this pain. There is a bump on his neck that is bothering him. We are hopeful that the CT, bone marrows, and MIBG won't bring us more bad news.

Hoping and praying for a complete response to these therapies for our beautiful boy!

MRI

So we met with our onc on Wednesday. The mystery symptoms, specifically, those bumps reemerged on Hans' skull, and a vision problem of seeing double or a little bit crossed eye, persisted. The onc took a look at him and ordered a STAT CT of the head. Wednesday evening we were relieved with the news that the head CT was "relatively preliminarily okay". However, yesterday's MRI proved the CT very wrong. The bumps along the top of Hans' head are NB. What they suppose happened is that the skull lesion that has been under control on the Millennium for so long has grown up and out. There doesn't appear to be any brain involvement at all. There are a couple of other little spots indicated on the outside of the head as well. The lesion(s) that are there are outside the skull.

This is a really big, unfortunate deal. We are going to get started on radiating these spots as early as we can, today or Monday.
We are inpatient once again (our fourth inpatient stint in 5 weeks) to expedite some additional tests that need to happen including a neuro opthamology exam, possible biospy, radiation consult, possible lumbar puncture, CT of neck chest and abdomen, and another MIBG scan. We hope that all of these tests will be completed by Wednesday of next week. However, treatment can start as soon as possible since we are not enrolling on a study at this time. We will do radiation in combination with some chemotherapy such as Irinotecan/Temodor, (to which Hans has responded before). We'll find out shortly, but the radiation will likely be on an outpatient basis, approx 20 sessions, or four 5-day weeks here in LA.

This is awful, terrible news in comparison with our hopes to do something low dose and oral and from home for Hans now.
I am just hoping and praying that Hans will continue to be the great RESPONDER to therapy that he has really always been. We can get these lesions under control over the next 4 weeks, and eventually find that great, low dose therapy we can do from home... For now, we are glad to be here at our home hosptial, even if it is a 2 hour drive from home! My mom brought Elle back home early this week. They are here with us now - they are staying at the beautiful nearby Ronald McDonald house and Kevin is able to work and come and see us and be here for important meetings. We have our sweet friend Aimee who has proven to be at our disposal!

Thanks for your thoughts and prayers for our beautiful boy. We could really use them now.

Love, Lara

Long 5 Days

Check out this thought provoking lil speech by an NB survivor, I just did and I think I needed to hear this tonight! Thanks, Tash!

We are having a calm and pleasant time at home since discharge, but a few of Hans' mystery symptoms are making these five days excruciating.

Right now, we are just hoping that they are able to book him for an MRI early this week. We need to get that done to inform Wednesday's meeting. You never know how a new hospital operates, but I had heard that one of three MRI machines is down and that appointments are scarce and hard to come by.

I wanted to put up a quick post to let you know that we are still at home and "okay", however loosely we are defining that these days. In truth we are pretty worried about our little guy and are anxious to have a plan to take care of him.

Thanks for checking up on us, Lara

Change of Plans...

Hans is starting to perk up! Getting a few dark eyebrows too:)

So, yesterday was a rough day. We kept looking at Hans and having huge anxiety about his capability to enroll on a trial all the way across the country, that would require him to travel not once, but five times in the next four weeks, either that or stay for a month in Philly. Our doctors helped guide us and advise us that it's not in Hans' best interest to go that route at this time. Maybe the universe or the hand of God is leading Hans away from this option for a good reason. It's important for me to restate that it's not Hans disease burden that seems to be getting him down right now. It's some virus or infection that we've been unable to label. I keep repeating to myself Dr. Marachelian's proclamation after reviewing his scans..."He has a TINY amount of disease!"

For now, we are gonna stay put and get treated in our new home state. That'll be a change up. Hans is looking forward to it! We hope to get discharged from CHLA today. Hans has continued to have his headaches, but his Sodium came up, and he actually needed a bag of blood yesterday. The fluids has has received all week have likely diluted his red blood count...

We are going to go home and enjoy our weekend of just being at home! And, then, on Wednesday we'll come back to LA and finally knock out a treatment plan with Dr. Marachelian. I have also heard they might do an MRI of Hans' head, which I'm sure will make me quite nervous ! We have three top options already lined out, and we'll discuss those and more next week.

The upside of this inpatient stint was finding a gem of an Endocrinologist to follow Hans here. I couldn't be more thankful. She is adjusting Hans' two steroids and she believes it is possible to help Hans avoid getting sick this often by proper steroid replacement. Without any adrenal glands, he just gets beaten into the ground when he is sick. Our new Endocrinologist is already trying to think preventative dosing... it's a very good find for us here!!!

Thanks for your thoughts and prayers for Hans. He really looks okay this morning. He is up and eating a little, watching his shows, talking about what he hopes to have for lunch on his way home---chicken! I guess now we just pray that the best possible treatment plan will fall into place for our boy!

More... Fever! Headaches! Inpatient! Again!

OH boy.... what a mystery this kid is this summer. We had a great day on Sunday AND THEN... Sunday night Hans started to have some pain in his HEAD. YIKES! There were bumps on the top of his head, causing him pain enough to take Tylenol w/ Codeine. Kevin and I were up for hours worrying about what that might mean. Monday Hans slept all day on and off while Keiran chilled and I made an attempt at picking up our place, doing laundry, etc etc. I was waiting all day long for the thumbs up from CHOP. AND THEN... at about 4:30 he told me to take his temperature... 102.6! OMG - did I fall apart. Here we had poor Keiran, we're waiting to hop on a plane to start a trial and now this.

So, off to CHLA. I left Keiran with Kevin in Bakersfield and hit the highway to LA. We spent all night in the ER, and then were discharged and released to Aimee's. Come morning he started brewing another 102.0 temp. At the same time I found out they were ready for us at CHOP. Kev had sent Keiran off on an early flight! Poor kiddo - he was sure a good sport about his shortened vacay.

Anyway, we reported to urgent care in the clinic where he continued to have fever and we were admitted. Mysteriously and wonderfully, those bumps on his HEAD went away and stopped hurting. Can you believe that???

Now the 'plan' is: Get Hans cleared for air travel, get to Philly on Sunday, RE SCAN (just found this out: need to re-test marrows and MIBG) and start the trial Tuesday.

Some of our sweet friends have asked us what to pray for. Oh, that is a good question! At some points during this month I've only been able to utter the little prayer "God help us." But I think that Hans is actually just fighting some virus or infection. He actually has a very light disease burden!!!! He just needs to get better and get on a treatment plan.
If you are moved to say a little prayer for Hans, please ask that he gets well enough to get home for a couple of days, stays healthy for a safe flight to Philly on Sunday, that his MIBG scan shows no progression from the 7/14 scan, and that if there really is NB in the marrow that they catch a sample of it so that we can test it for the ALK mutation, and ultimately, that he can start the ALK trial and that it starts working a little miracle soon.

Now, is that too much to ask for??? I absoluetly think not!

Hollywood and Highland

Kevin had a good fast trip to Seattle for some important family time at his grandma's service. I was soooo glad that he and Elle were able to attend. He needed a lift home from the airport today, so after another trip to LAX, we squeezed in a fun stop at Hollywood and Highland, the Walk of Fame, and a nice lunch.

Hans is feeling pretty okay I think. I forgot to mention that we have our pal Keiran with us this week! This plan had long been in the works, to bring Keir back with us from this past Philly trip to keep Hans occupied while Elle was off at camp, we just bumped his trip back along with ours.

We are having fun. I just need to pace our day so that we don't try to do too much. A lot of downtime and relaxing time is pretty much always key for a happy healthy Hans. (Thanks, Aimee for the guided tour).

Going Back to Cali

We learned yesterday that the new arm of this trial will not start today as hoped. There is basically a paperwork gridlock/logjam. Every i needs to be dotted and t needs to be crossed before all numbers are crunched and the new phase is approved. I guess some of the data still needs to be submitted before the data can be analyzed. That's what I get out of it, anyway.

We have been advised to catch a flight and have a nice long weekend at home. We hope to get a call Monday, telling us to come back out to start Wednesday. For the time being...I'm okay with it. As long as Hans starts a trial by Wednesday of next week, he won't need to be rescanned. If we go beyond that point we are looking at Plan B, C or D...

Hans is really going with the flow here. He is looking forward to getting back into town to see his dog, swim in his pool and hit our two favorite chicken restaurants...Popeye's and El Pollo Loco. We won't have Kevin til Sunday. He is flying out to Seattle today to attend his Grandma Lorraine's memorial. Elle gets out of camp today. My mom will pick her and Greta up from their special week at Camp Robbinswold on the Hood Canal. I went there as a girl too. It's gorgeous and beautiful! I can't wait to talk to her on the phone! I'm also ready to skip out on this East Coast heat wave. I'm sure it's gonna be hot in Bakersfield, though.

Thanks for checking up on us.

Love, Lara

Hans and the "ALK" Trial

More news and a recap. I may have misled most of you with last night's post, although Hans' CT scan was clear, it doesn't negate or override the progression noted on the MIBG scan. Hans is still considered to have progressed on Millennium, so now is the time to Change Up. We got more wonderful news today that Hans' bone marrow sample was negative for NB. Whoa, we didn't see that coming, bone marrow biopsies can be hit or miss. We'll probably never know if we just missed sampling some tumor in there. We have consented to sign onto the ALK trial at CHOP.

Here's the scoop. There has been a lot of buzz and excitement about a trial widely known as the "ALK Trial" which takes advantage of a certain genetic mutation present in some Neuroblastomas. If the mutation is there, the drug tends to work well, if the genetic expression is not there, the drug has still been shown to work in a smaller percentage of patients. The drug can"block or inhibit two proteins called c-met and Alk. These proteins may be important in the growth of certain types of cancer cells. " (taken from the patient consent form)

To this point we haven't been able to determine if Hans has this genetic experssion. Why? First off, because when Hans was first diagnosed back in 2006, researchers weren't even yet aware of this genetic link. Our team stored samples of the tumor from the inital biopsy. However, those samples were routinely stored in paraffin wax at that time, rather than frozen. By Hans' 2009 relapse, Dr. Mosse here at CHOP had discovered this genetic link! At that time we had a tissue sample of Hans' initial biopsy sent to CHOP for testing. They were unable to use the sample to run the test due to the way it had been stored. Dr. Maris assured us not to get upset by this, he said that Neurobalstoma can actually change its genetic make up from inital diagnosis to relapse anyway. We wanted Hans' tumor tested at that second relapse, but Hans presented with only a bony lesion (the skull) and no marrow disease. It was impossible for us to get a biospy of his disease because it wouldn't have been safe for Hans.

Since we have a new positive spot on the MIBG scan, we made an effort to do a biopsy of that area (the right hip) for that testing. We found out just this afternoon that the bone marrow biopsy is negative. So, there is nothing to test.

Regardless, we are hopping on the trial in the hopes that he does have the expression, or that it will work for him even if he doesn't.

A new phase of this trial is just about to open. It was so successful and showed so few side effects that the investigators are opening up a new higher dosing level as we speak. Hans will be among the first kiddos to enroll at this new highest dose level. No side effects are really anticipated. Since it is so targeted, there should be no need for blood transfusion, no hair loss! Yay. I can't wait for those hairs to pop back up.

BUT the biggest problem is that they need us back at clinic in CHOP on each of the next four Wednesdays!!! I can't quite figure out how to wrap my mind around that one. With such a long cross country commute, we're considering staying out here and maybe only making a couple of trips back home, poor Kev:(

Anyone got a beach house in Jersey they can give up for a couple weeks;)

Unofficial Good News

I have to update to share some unofficial good news! Both Dr. Maris and the attending here have eyeballed Hans' CT scan to say it looks good (aka clean, clear and free of NB or any other tumor or cancer!). Preliminary results of the bone marrow aspirate look good as well. The actual bone marrow biopsy will take a couple of days. Dr. Maris and I both suspect it will be positive on at least the right side. However, the onc who did the biopsy didn't see any "large clumps of tumor" when he spread it on the slide...

All good. All I can say is Hallelujah! Thanks for checking up on this little dude. Thanks for your calls texts emails and notes. I haven't been too chatty so sorry if I haven't shot a note back. I'm just using every drop of my energy and emotional reserves to do what we've gotta do here. This just sucks out of you about everything you've got. I was shaking like a leaf througout the CT scan was as nervous as I've ever been about anything. We meet with our team formally tomorrow. Still inpatient tonight as we continue to deal with low sodium,low potassium and headaches. Dr. Maris attributes Hans' current condition to a bug that has zapped him, and this is how Hans looks getting through it sans adrenal glands. I'll take that explanation to the bank.

CT Complete

Hans and I had a long and arduous day today. The poor kid had to drink 720 ml (24.5 or so ounces) of Blue Kool Aid with IV contrast. This is bad enough when you're at your best. It is usually a pain that Hans gets through. But Hans is still fighting nausea, vomiting and headaches. He has needeed the IV morphine for the headaches three times since 4 am. He's been on an IV drip so he's had no thirst. Sooo... it took us SEVEN HOURS...from 6:30 am until 1:30 pm to get all of the drink in him. He did vomit once, but only lost about 30 mls. I was surprised they allowed him to take so long, and I was alost surprised they 'made' him drink all of it. I thought they'd give up on him in the normal 3 -4 hours and let him get by on 50% - 75% of the drink. Once you get through the contrast, the CT is very quick and Hans does it without sedation. Today he got a head, chest and abdominal CT.

We are waiting. We'll get all these results by tomorrow. I am very very nervous right now.

Thanks for your thoughts and prayers. I know everyone is worried about Hans right now.