Hannah has a love for lyrics and so what better storyteller than the man in black himself, Johnny Cash. At a very young age Hannah was introduced to Johnny Cash and now has a love/hate relationship with his songs (thinks they're sad/thinks they're good). A perennial favorite with her and Max is Ring of Fire. With Max's increased exposure to skate culture, I decided to try out Social Distortion's version of the song on Max, which he loves (and I love that he loves). Supposedly Nic was chiming in, "ring of fire!" with Max but that wasn't caught on the video.Below from Sunday's trip to the San Diego Aerospace museum. San Diego has a long and colorful history of aviation, but Max is most enamoured with the P-40 Tomahawk, which I completely understand and support. However, like his Pinewood Derby car, it's long on looks and somewhat short on performance. Nevermind, he likes what he likes for the right reasons.
Finally, since Nic... wait, he's mentioned so little that maybe I should explain. In case you were wondering, Nic is our third child. He not only suffers from 3CS (3rd Child Syndrome meaning he can make his own toast at 2 1/2, watches way too much TV and way too much TV that is too violent for his 2 1/2 years but then what are you to do if you have a 6 and 8 year old and they want to watch Kim Possible and Battle 360 all the time?) but he also suffers from SHC (Sibling Has Cancer meaning his needs are frequently and consciously neglected in support of the greater good cause of taking care of Max). Below is Nic being allowed to get way to close to the railroad tracks that run through Del Mar, while throwing rocks from the trackbed that are coming dangerously close to people walking down the path below. Stuff his older brother and sister never got to do, but since he suffers from 3CS and SHC, something that he gets to do.
- CT Scan - to determine bony vs. soft tissue disease in comparison with the mibg scan
- Brain MRI - to see if the spot on his head is in the bone or tissue
- Urology appt - to check on the blood in his urine
- Clinic CBCs, urine, chemistry panel
Thank you for all your kind words and thoughts. You're giving us strength.
Andy & I were able to meet with Dr. Roberts, Max's oncologist on Friday afternoon to discuss treatment possibilities. Of course, there is no protocol for how to treat Max's neuroblastoma any longer - again we had "the discussion" as to whether or not we wanted to do anything at this point. And again, we agreed on a course of aggressive, but smart (ie, managed toxicity) treatment. Or to put it into the War in Iraq analogy, we're trying to kill as many insurgents while minimizing civilian casualties. Not an easy job either way. But whereas the value of fighting for Iraq is questionable, the value of fighting for Max is unquestionable. I can't see how a reasonably logical person could think otherwise. So, we're fighting.
Let's be clear. There is no cure, there is no established route to success. Our goal in this effort is time - time that extends our chances of finding something that is curative, or guaranteed to stop the disease for the long-term, and which his body can tolerate. So we have to keep him here, and we have to keep him healthy. In other words, we don't have to kill the enemy, just contain it. If we can do that with acceptable toxicity (such as we had for the better part of the last 16 months), we'll have succeeded in our goals.
The MIBG scan indicates both bony and soft tissue disease (soft tissue we think in lymph nodes near the heart and in his pelvis). A CT scan has been scheduled for 4/1 to hopefully give us a better idea of where the bony and soft tissue disease are. Max still has the urology consult appointment 4/1 after which we'll see if the doc there can make quick with the cystoscopy and tell us what's causing the blood in the urine.
So, what do we do?
First, we're taking Max off the topotecan because it appears that he progressed while on it. We'd like to put Max back on cyclophosphamide but can't if the bladder wall is damage by it.
For those of you interested, here is what Max is currently taking daily. Much of this is directed at stopping metastasis. IL-6/8/10, c-reactive protein, ceruloplasmin, VEGF - all are blood factors associated with angiogenesis and metastasis.
- Glutamine-gut protector
- Nifurtimox-trial drug; weakens NB cells
- Celebrex-cox-2 inhibitor; anti-inflammatory
- Skullcap-lowers IL-8
- Genistein-hormone blocker used in breast cancer
- Prevacid-stomach upset
- olive oil-fat & calories, omegas
- ImmunoKinoko-increases NK cell function
- Vit D-aids in calcium absorbtion
- Acidophilus-immune health
- Melatonin-G-MCF stimulator
- Bromelain-platelet stimulator
- Calcium Citrate-calcium boost for Zometa infusion
- Milk Thistle-liver function
- Tumeric (curcumin)-anti-inflamatory (also makes Celebrex more effective)
- Shark Liver oil-platelet stimulator
- Fish Oil-omega 3's, fat
- Zinc Citrate-lowers ceruloplasmin
- Taurox- lowers IL6 & C reactive protein
- Cellular Forte w/ IP6 & inositol-lowers plasma VEGF
Andy and another neuroblastoma Dad are using this weekend for some R&D on what Max's treatment possibilities are. Some clinical trials are a possibility, most likely we'd come up with our own mini-trial which is nothing other than a unique combination of agents suited to Max's individual needs. We are not limiting ourselves to facilities on the west coast and have told Dr. Roberts that we will do whatever takes, go where ever we need to go.
More on next post including possible agents/trials we might use, and which will be discussed during a conference call Tuesday with Dr. Roberts and Dr. Sholler.
Max went in for his MIBG scan this morning and there were spots all over the place. Spots = neuroblastoma.
His scan started with his head where I saw a V shaped spot enhanced. Text to Andy: "spot on his head" send. Then we go down to his check/back area where I see the original spot looking pretty good. Lightly enhanced, maybe a little smaller. Text that info to Andy. Move down to the abdomen/pelvic area. hmph, I've never noticed it being so enhanced in this area. Uh-oh, the tech is taking another picture of that area. Not good. Text info to Andy. Now we're at the legs and there's a vertical line on one thigh and a bright circular spot on one knee. Text to Andy. Response from Andy: F%*k F%*k F%*k. I concur.
So I'm sitting in the Nuc Med scan room, Max is watching a Seuss movie while going through the scanner, I'm staring at the computer screens with all this crap on them, and I'm trying my damnedest to keep it together while the tech, also named Melissa, is also trying not to lose it because she knows that I know what I'm seeing up there. She can't say anything as she's not a doctor - and not Max's doctor. Max is blissfully oblivious to the whole scene as Horton Hears a Who! is very entertaining.
After the scan Max & I had to walk over the clinic to drop off a urine sample, me fighting back tears the whole walk and Max pretending to be on a bear/deer/hare hunt with his new cap-rifle. All the nurses we see and know are in clinic and I just couldn't even tell them what I just saw because I knew I'd start crying and not be able to stop. So we hustled out of there and I took Max back to school. Max feels great right now, no apparent pain, aside from complaining about his mickey button in the evening.
We do not have the official results or reading from the radiologist yet. It will come sometime tomorrow. I think the shock of seeing the scans has worn off - a little - and I'm ready to hear what the plan will be to combat this invasion of Max's little body.
Max & Nicky in Idyllwild this past weekend running around shirtless outside, making owl hoots, in 50 degree weather. (that's freezy cold in So Cal, btw)
Max sportin' his new "real" cowboy hat courtesy Papa and the Pony Express Trading Post in Idyllwild.
He's one tough hombre.
So there we are at the hospital. Max had a 9:00am ultrasound of his kidneys and bladder, then came to clinic to have his CBCs checked and have the doctor decide what to do about his pee. He had to provide another urine sample and by this time his pee was plain ol’ yellow! (It’s interesting that with some good hydration his urine clears up.) Dr. Schiff - another wonderful oncologist - ordered a BK test on his urine that will tell us if he has a certain type of viral infection that occurs in the bladder. It takes about one week to get results. A positive result will require a special IV antibiotic. Max also has an appointment with the urology department on April 1 to review the ultrasounds and all the urine tests he’s had done recently. Due to Max not being in any pain when he pee’s, and his urine clearing when he’s well hydrated, this “blood in the urine” deal is not an emergency… and hopefully we stay in code yellow.
Max’s HGB was also low (7.6) so he got to stay for a blood transfusion! Time to pink up those cheeks and fuel up for the weekend.
Max and I were at the hospital from 8:50 ‘til 5:00. Believe it or not the time went by quickly as Max was shuffled from the radiology department to clinic, to the exam room, back to clinic… oh, back to radiology again, and his final stop: clinic! Don’t put off until tomorrow what you can do today!
Another excerpt from Winston Churcill's 1941 Harrow school speech:
The approach is called metronomic, low-dose or antiangiogenic chemotherapy. It was pioneered by a remarkable man, Dr. Judah Folkman. More than the ingenuity of his ideas, I am more impressed by the stories I've heard about how Dr. Folkman would listen to, and work with, any parent who dropped by his lab, desperate for a treatment that might save their own child's life.
It is that kind of hope and optimism that propels us in what we do with the MagicWater Project. And it is with researchers and oncologists like Folkman that we work with, who are never too busy to sit down with a desperate parent, and discuss how they can save a child's life.
Last summer and fall, we put Max on a modified version of the drug combination used in the trial mentioned. The trial consisted of low-dose, oral Cyclophosphamide, Etoposide, Celebrex, Thalidomide and Fenofibrate. Max was on the first three, but it was difficult for him to tolerate both chemos (first two) at the same time. Now, Max is on orally-administered Topotecan, Celebrex, and Nifurtimox. In addition, he is back to taking Curcumin which has been shown to display significant tumor properties in-vitro and in-vivo (but in one study it was contraindicated with Cyclophosphamide so that's why we stopped using until recently)
Where were we...? Oh yes, the article link:
CV father’s MagicWater Project dedicated to working with cancer researchers and oncologists to speed up discoveries of potentially life-saving new drugs
•Fundraising coordinator needed
By Catherine Kolonko
About three years ago, a little Carmel Valley boy, Max Mikulak, was diagnosed with a type of children's cancer that is difficult to cure. When the cancer returned after traditional treatment, his parents began an odyssey through the world of science and medicine in search of new drugs that might keep him alive.
Max was displaying symptoms of anemia, including a lack of energy when he was diagnosed in 2004 with high-risk Stage 4 neuroblastoma, an aggressive pediatric cancer that is diagnosed in about 600 children per year in the United States, said his father Andy Mikulak. The cancer is more prevalent among babies under the age of 1 but much deadlier in older children. After the age of 2 the survival rate dips drastically
“If you wait another year and it sticks around, it becomes a deadly killer,” said Andy.
Neuroblastoma is a disease in which malignant cancer cells form in nerve tissue of the adrenal gland, neck, chest, or spinal cord. By the time it is diagnosed, the cancer has usually metastasized, most often to the lymph nodes, bones, bone marrow, liver and skin, according to the National Cancer Institute. Usual treatment options for severe cases like Max's involve high doses of chemotherapy, radiation and stem cell transplant.
For about a year after his initial treatment, the cancer disappeared but Max, now 6 years old, had a relapse in 2006 and has been battling the return of the cancer for 16 months. He's had most of the traditional treatments for his disease and there are only a few options left, none of which are very effective, Andy said. That dearth of treatment possibilities led Max's determined father to reach out to other parents in the same situation, not only as a support system but also in a hunt for new life-saving drugs.
“We were kind of thrust into this new world where you're on your own as far as treatments,” Andy said.
“All of a sudden you start to become an activist and you start to think 'How are you going to save our kids' lives.'”
Almost 80 percent of kids with cancer are essentially cured and become long-term survivors but then there are the 20 percent that don't, said Dr. William Roberts, an oncologist at Rady Children's Hospital in San Diego who oversees Max's treatment. For children with relapsed neuroblastoma “The outlook is not good,” Roberts said. “The number of kids surviving is about 30 percent, which is pretty dismal.”
For that reason, Roberts said he understands the mindset of parents like the Mikulaks who are open to trying something innovative with the idea of “What can we do differently to try to gain some ground?”
There are “tons and tons of drugs out there that might work” but have no visibility because there is no business reason to pursue them, said a frustrated Andy Mikulak. It is unfair that approved treatment options are so limited just because of the relatively small number of children affected by the deadly cancer, he said.
Last year Andy and Neil Hutchison, a San Diego father whose son also has relapsed neuroblastoma, started the MagicWater Project (www.magicwater.org), a foundation dedicated to working with cancer researchers and oncologists to speed up discoveries of potentially life-saving new drugs for children with relapsed neuroblastoma and medulloblastoma. Their goal is to find innovative, low toxicity, new treatments through funding of clinical trials and other research.
The foundation name was derived from the explanation by the parents of an 18-month old girl about why she needed chemotherapy. “He just called it magic water,” Andy said.
“We all have our own mechanisms for dealing with this with our kids,” Andy said, referring to the challenge for parents who must explain cancer treatment to their very young children. Calling the foundation magic water seemed appropriate for the foundation because it symbolizes hope and pays tribute to that little girl who eventually lost her battle to cancer, he said. “It's kind of the inspiration for what we're all working for,” he said.
Funding for MagicWater is done mainly through networking and from friends and family. Since its inception, a couple of people have written checks for as much as $100,000 and another $25,000 was acquired through a fundraiser at Max's school, Andy said. He compares the MagicWater project mindset to that of a venture capitalist who seeks investments that are high risk and high return, and notes that it is much different from a traditional cancer foundation that is more conservative, like a bank, he said.
“It's meant to speed up the process by which an agent can get tested in neuroblastoma and medulloblastoma,” Andy said.
One course of treatment now showing promising results initially caught the attention of researcher Giselle Sholler, a Vermont doctor. She had heard about a case reported by physicians from Brown University who treated a child for a parasitic disease known as Chagas who also happened to have neuroblastoma. The administration of the antibiotic nifurtimox, long used in South America to treat tropical illness, seemed to have an unexpected benefit on the cancer which went into remission.
“She started looking at it and thought it might have some interesting anti-tumor properties,” Andy said.
Nifurtimox was not approved for marketing in the states but had been available for years in other countries. In further lab testing of the drug, Sholler found that it appeared to shrink or kill neuroblastoma tumor cells. Her findings eventually led to a phase 1 clinical trial that included Max as a patient. The trial was designed to test the safety and toxicity of the drug in combination with a regimen of chemotherapy.“Sometimes you just have to go on these Phase 1 projects and hope for the best,” said Andy.
The results were “very encouraging” and that trial has opened the door to getting the drug available for other children, said Dr. William Roberts, Max's doctor at Rady Children’s Hospital in San Diego. Although the trial's objective was to determine the drug's safety profile, “Clearly there's a whole world of information, so you don't want to ignore it,” he said.
A phase 2 clinical trial headed by Sholler is underway in Vermont to further investigate the use of nifurtimox alone or in combination with other drugs as treatment of relapsed or refractory neuroblastoma and medulloblastoma. Children’s Hospitals in San Diego, St. Louis, and Atlanta are also expected to participate. The MagicWater project donated $150,000 to facilitate the study.
Another project that MagicWater recently funded with a $100,000 grant is research conducted by Dr. Donald Durden, scientific director at the Aflac Cancer Center in Atlanta and a professor of pediatrics at Emory University School of Medicine. He is conducting research in in vitro and animal models to discover novel combinations of new drug agents that could slow or stop the progress of neuroblastoma and medulloblastoma. The goal is to find the right combinations of agents that could then be tested in clinical trials with pediatric cancer patients.
One compound resulting from Durden's work may soon be tested in a clinical trial, he said. The low toxicity drug, which appears to be well tolerated, will soon be submitted to an investigational review board that monitors U.S. drug trials and ensures the safety of participating patients.
Durden said he understands the sense of urgency felt by parents who want to find new treatments that might save their children.
“You might say we're not waiting around,” he said. “We're trying to push the envelope a little bit. It's not like they have a few years to be thinking about this because the kids are going to die.”
Meanwhile, Andy and others involved with the MagicWater project continue to search the Internet and network with other parents, researchers, and doctors to find possible drug candidates worthy of funding while Max and other children like him fight their courageous battles with cancer. Max recently started a new round of chemotherapy and his daily medications are adjusted regularly based on their toxicity and efficacy.
Andy Mikulak and Neil Hutchison, MagicWater cofounders, are working to build the organization and they are currently seeking someone who can coordinate fundraising for the project. More information is available on the Internet site, including blogs, with updates on Max and other children can be found at www.magicwater.org.
Tuesday Max was in for an MRI and next week he’ll have his bone scan. We are really hoping for some good results – a little smaller spot on his spine would be nice, wouldn’t it? My hopes are that the last set of scans we had taken may have been a little too close to the radiation he received and were still showing enhancement (cells DYING!!) I can be optimistic as long as I don’t overdo it.
There seems to be a lot of really good energy in clinic today. It could also be that I just finished a huge cup o’ jo… nah.
Max is feeling considerably better this week and seems to be completely over his stomach bug. He’s also gained a kilo back and is up to 20.2kg. He’s having minimal nausea and is not throwing up. Yay!
Max was able to start back on chemo this week. Thank goodness. It’s been almost a month since we had to stop his oral cyclo. It sounds funny to want your kid ON chemo, but when the alternative is that the cancer may make a breakthrough – chemo is definitely a good friend. Due to the blood in his urine we have suspended the cyclo and started him on oral topotecan, low dose, daily, skip weekends. He’s had three doses and his nausea is very minimal – hope it stays that way!
Speaking of the blood in his urine, Dr. Roberts has ordered an ultrasound of the bladder to see if there any blood clots that may have been formed when he had the stomach bug. If there’s a clot that could explain why his urine is sometimes clear, sometimes red, sometimes has little clumps (little bits breaking away here and there). This would be the best scenario I believe. They might be able to flush his bladder and clear it out a bit. Second scenario we talked about is he could have bladder wall damage either caused by a viral infection (going back to his stomach bug) or caused by the cyclo chemo. To figure this out he would probably have to have a visit to the urologist where they would use a scope to look around in there and see how bad the damage is. It could be just the top layer, or it could through a few layers. The latter needing much more recovery time, possibly months.
In the meantime, his blood counts are awesome today!
ANC 1988 !!
Tony telling Max that his son has the same shirt but has written all over the skull's head. What is it about little boys and skulls... oh, the pirate thing, right....
Tony doing a hand-plant, Max's favorite trick, right in front of him.
Bucky Lasek and Max.
Max and Sam were treated to another skateboard adventure on Saturday as Clash At Clairemont took place. Max's skate-n-destroy first-grade teacher Miss Sturt and her husband Daniel hooked us and the Hutchison's up with VIP tix to the event. Max originally didn't want to go, but that was just him being lazy. nce we got there, the music, the crowd and the tricks all got him going. The event was very family-friendly and it was mostly kids and families. Cool!
Tony Hawk at Clash At Clairemont. We were in the VIP area which gave us very close access to the skaters. The boys loved it... then we got to go up to the top of the ramp!
Max is finally starting to feel better, ie, back to "normal", normal being a kid with deadly cancer who doesn't eat but receives 98% of his daily caloric intake via a tube that empties directly into his stomach. But what's normal these days anyway??
Good trend, a bit more data than last Saturday's March 1 post:
LDH (normal range is 470-900)
VMA (normal range <8.5)
Max has lost over 2 kg since getting the intestinal bug (1 kg = 2.2 lbs), in other words, a lot given how little he has to spare. He's back to looking pretty skinny compared to the photo at the top of the blog, and he's pale as can be, and almost bald so he looks quite different again. I'll post a picture this weekend so you can see - we've been lazy/busy in other areas lately but I know a picture says it all.
Max will be starting again on chemo today. His counts are good: ANC: 1250 and platelets 110K (his minimum platelets to be on chemo is 50K, which we lowered from the previous limit of 75K otherwise he'd never get chemo). So it looks like his bone marrow has finally recovered from the whammy of the IV topotecan late January. Max will be starting on oral topotecan today, a decision made by the possibility that the cytoxan he's been taking orally since July '07 has damaged his bladder (he's had blood clots in his pee fairly consistently for the past 3 weeks). Which if turns out to be the case, he can't use oral cytoxan any longer which will be a challenge for us as it appears to have been well-tolerated and (as far as we can tell), effective against his cancer. However, looking at the most recent VMA and LDH numbers, maybe the topotecan really did a bang-up job and is what we should be focusing on using going forward? Unfortunately, there are no simple, definitive answers with relapsed neuroblastoma. Everything's a informed crap-shoot at best.
On the propoganda and advocacy front, this week was busy...
Yesterday, Max was on San Diego radiothon supporting Children's Hospital. He was on 95.7 and 94.1 I believe. Melissa said he did great, was interviewed twice, and got a toy aircraft carrier out of the deal so everyone's happy.
Next week, there will be a feature on the MagicWater Project in our local newspaper, The Carmel Valley News/Rancho Santa Fe Review/Del Mar Village Voice, which is a really good local newspaper combination.
The MagicWater Project is also having discussions with Amy Marcus, WSJ reporter who is on leave working on a Robert Wood Johnson Foundation project focusing on orphan diseases. She's interested in our model, and of course our outcomes. Who knows what may come of these discussions, hopefully some good visibility and credibility for what we're doing. Like Steve Jobs' 2005 commencement speech at Stanford, hopefully this will all make sense at some point in the future.
"Of course it was impossible to connect the dots looking forward... But it was very, very clear looking backwards ten years later."
These are the normal range for CBC (complete blood count):
WBC white blood cells 4.0-12.0
HGB hemoglobin 11.5-14.5
PLT platelet 140-440
ANC absolute neutrophil count 1500-5000
These are about normal for Max:
LDH 600-700 (we watch this as it can indicate NB activity)
In order for Max to begin a chemo round his blood counts need to be:
These numbers were 75 &750 in the past, but we've had lower them in order to keep Max on treatment. When Max goes below ANC 500 he in considered at risk of getting an infection (neutropenic). If he were to get a fever of 101.5+ he would need to be checked into the hospital to receive IV antibiotics for up to 10 days.
Urine markers we watch are called cathecholamines, aka HVA/VMA:
Max's fluctuate between:
Andy keeps a graph charting the points to watch for any steady rise in the numbers which would indicate NB activity.
Andy referred to Max's LDH spiking this past week which gave us a huge scare in conjunction with the HVA/VMA numbers being higher than usual. It turned out that because Max had a red blood transfusion on Tuesday it artifically increased his LDH in his CBC (complete blood count) on Wednesday. When you receive a transfusion there are LDH in the red blood and they can increase the longer the blood sits on the shelf waiting to be used. Which translated into Max's LDH being twice what we normally see and which put Andy & I into orbit until Dr. Roberts soothed our fears (and the LDH #s dropped as he said they would).
I hope this helps!
More good news. LDH is down. So that really was a false alarm this week but, regardless of the extra work it involves us, our friends and family, and the good folks at RCHSD, it's better to be paranoid and react immediately than wait. This cancer is so aggressive, so difficult to predict its behavior, that I'd rather over-react versus the alternative.
LDH (normal range is 470-900)