JD and I met with Clay's doctors yesterday to go over the MRI. The results were "good" in that the main tumor is slightly smaller than in the December MRI. Not "great" since Avastin typically gives the biggest shrinkage at the beginning of the protocol, and therefore the doctors' expectations were high. The tumor appears to have reconfigured, and pulled back from the optic nerve, which has allowed Clay to regain his vision. For now the plan is to continue the bi-weekly protocol and rescan in another two months. Next week the PICC line will come out. We are going to give it a go with doing future treatments with an IV. This will spare Clay another surgery, and the worry associated with having another foreign body in him (the port).
Please take a minute to cast a vote for Rex Burkehead, a running back for the Nebraska Huskers, for Uplifting Athlete of the Year Award. This award goes to the college football player that has made a lasting impact on a rare disease. Rex has befriended a boy named Jack, who suffers from the same disease as Clay. Their friendship has received some media attention already, which is helping raise awareness of both the disease, and PLGA -- the only group that is funding the research for low grade pediatric gliomas. If Rex wins, he will be featured on ESPN and numerous other national sports shows but MOST IMPORTANTLY he will receive his award directly from the National Institute of Health. This would be great for our cause and getting PLGA on the research agenda for the NIH and NCI.
The final award decision is based on total number of votes (there are 4 competing athletes). Between now and February 26th, you can cast your vote for Rex by going to http://www.upliftingathletes.org/take-action/rare-disease-champion (scroll to the bottom of the page to register your name and vote...It takes ONE MINUTE). Please spread the word and ask friends and family to do the same...)
As always, thank you for your support.
Thursday, February 9, 2012
Sunday, February 5, 2012
Feb 5
The past few weeks have been good ones, with Clay going to school most days. He has made some nice friends, and his teachers could not be more wonderful. Here he is with a school buddy working on his latest project. He is learning all sorts of things, and this week reported to me that Pluto is no longer a planet, but a "dwarf planet." When I asked what that meant, he told me that "you used to be able to drive there, but now it is made of gas, so you can't." Hmmm. I wonder why Wikipedia is missing this important detail.
We enjoyed a visit from Aunt Amy and Grandma Inez this past week. The kids favorite activity was fondue dinner at the Melting Pot. Really, what could be better than dipping marshmallows and cheesecake into hot pots of chocolate? We also started a 1,000 piece puzzle that kept us all incredibly focused, however, there is a good chance the puzzles completion may require another visit.
Clay's next MRI is tomorrow. We will give an update after our consultation next Wednesday. Please keep Clay in your prayers tonight.
Sunday, January 15, 2012
Jan 15
Two doses in and so far so good. I took Clay on Wednesday to the clinic so Dr. P could see him. He said "Wow, who are you? You look fabulous, and there is so much more of you!" Clay has gone from 39.5 lbs to 48 lbs in about 3 weeks. We continue to taper the steriods, but it is a slow road. Clay was down this week for a few days with the stomach bug, but today when he woke me up at 7:30 he reported that at 3 a.m. he had been downstairs to have a bowl of cereal and a corn muffin. So his appetite is back. His vision seems to be holding steady -- we don't know where it is compared to what was his normal, but he can see colors, can color, and can get around pretty well. We aren't done holding our breath since we saw how quickly his vision deteriorated last month, but we are hopeful. The plan is to have 2 more doses of Avastin, and then do an MRI in the middle of February. If we see stable or shrinkage, then we will remove the PICC line and schedule another port placement. This will put me out of a daily job flushing his line, but it will mean Clay can swim and shower again.
Wednesday, January 4, 2012
January 4
Clay's vision has improved over the past ten days. Before Christmas he was able to detect only shadows and contrasts while today he is able to discern most images and colors. He is able to recognize letters and numbers on his kindergarten work book. While none of these observations are clinical measurements the improvement is unmistakable. Does Clay have "normal" eyesight or even "normally bad" eyesight? No. He suffered permanent damage after his first trauma and this latest episode has further degraded his capabilities. However, the recent improvement has moved him from "clinically blind" to a state where he can function under his own power. For this gift, we are truly grateful!
To what do we attribute this trend? The steroids have had an impact right from the start, but Clay's Doc feels that most of the improvement is indication of the Avastin/CPT11 doing its job. Dr. Packer, not a very effusive person, wrote to us that this news "made his New Years." Packer feels that in the situations in which Avastin proves effective that it shows efficacy very quickly. Mary and I are being very cautious in this stage; we are guarding against "false positives" and are aware that even when the drugs are working against the disease they make Clay very susceptible to life-threatening brain bleeds as a side-effect. But, as we have outlined in previous posts, there are few options left for Clay--- his disease is non-operable, radiation therapy is a final-ditch effort that would need to be so comprehensive that he would be severely damaged by it, and there is no known chemo or biologic cure. Our goal is to "buy time" in hopes that medical science can out-run the disease.
Finally we do not underestimate the POWER of positive energy and prayer that has been directed at Clay. Mary and I are convinced that the support is not just comforting but impactful. Whether it be the nightly reminder from the McDowell children, the prayer groups of neighbors and teachers, the Pizzano family's gift of water from Lourdes, or the daily half-hour devotions of my Aunt Roni, we know these and many other signs of faith and love are helping. Thank you all!
As far as daily life, Clay continues to succeed in having an upbeat attitude. There have only been a few times during these "dark" hours when his frustrations and fears have overcome him. For instance, after Christmas eve mass our family tradition is for each person to open one gift from under the tree to take a little pressure off the wait till Christmas morning. This year Clay unwrapped his selection and tossed onto the ground sobbing: " I hate this, I can't see my presents." In addition to the vision problems, Clay is also tolerating discomfort of an IV line (temporarily implanted in his left arm), along with side-effects of a massive steroid dose----the most noticeable being his ravenous appetite. He is eating something close to 5,000 calories per day---awakening at 3 am to eat bowls of cereal for what is the first of three breakfasts. He has gained about 20% of his body weight in 10 days. Even so, yesterday Clay returned to kindergarten where the excellent teaching team guided him through a successful first day back.
-- JD
To what do we attribute this trend? The steroids have had an impact right from the start, but Clay's Doc feels that most of the improvement is indication of the Avastin/CPT11 doing its job. Dr. Packer, not a very effusive person, wrote to us that this news "made his New Years." Packer feels that in the situations in which Avastin proves effective that it shows efficacy very quickly. Mary and I are being very cautious in this stage; we are guarding against "false positives" and are aware that even when the drugs are working against the disease they make Clay very susceptible to life-threatening brain bleeds as a side-effect. But, as we have outlined in previous posts, there are few options left for Clay--- his disease is non-operable, radiation therapy is a final-ditch effort that would need to be so comprehensive that he would be severely damaged by it, and there is no known chemo or biologic cure. Our goal is to "buy time" in hopes that medical science can out-run the disease.
Finally we do not underestimate the POWER of positive energy and prayer that has been directed at Clay. Mary and I are convinced that the support is not just comforting but impactful. Whether it be the nightly reminder from the McDowell children, the prayer groups of neighbors and teachers, the Pizzano family's gift of water from Lourdes, or the daily half-hour devotions of my Aunt Roni, we know these and many other signs of faith and love are helping. Thank you all!
As far as daily life, Clay continues to succeed in having an upbeat attitude. There have only been a few times during these "dark" hours when his frustrations and fears have overcome him. For instance, after Christmas eve mass our family tradition is for each person to open one gift from under the tree to take a little pressure off the wait till Christmas morning. This year Clay unwrapped his selection and tossed onto the ground sobbing: " I hate this, I can't see my presents." In addition to the vision problems, Clay is also tolerating discomfort of an IV line (temporarily implanted in his left arm), along with side-effects of a massive steroid dose----the most noticeable being his ravenous appetite. He is eating something close to 5,000 calories per day---awakening at 3 am to eat bowls of cereal for what is the first of three breakfasts. He has gained about 20% of his body weight in 10 days. Even so, yesterday Clay returned to kindergarten where the excellent teaching team guided him through a successful first day back.
-- JD
Saturday, December 24, 2011
Christmas Eve Update
JD here
It has been 2 years and 8 months since Clay's first trip to the emergency room with what we thought was a severe concussion. 24 hours and three hospitals later we learned that Clay was suffering from a slow-growing cancer called Juvenille Pilocytic Astrocytoma (JPA) which is part of a class of cancers called low-grade gliomas (PLGA's)that account for roughly 30% of pediatric brain tumors. While less is known about pediatric conditions than adult disease relatively less is known about PLGA's due to a set of factors including physical, institutional, financial and legal. What is known about PLGA's is that while there is no cure for the disease many children survive the disease by using a combination of chemo and radio therapies to contain its growth until maturation to adulthood when the cell mysteriously stops mutating. Evidence of the lack of medical understanding of PLGAs is that the first line of defense is a chemo protocol that is over sixty years old.
It is not uncommon for PLGA kids to undergo chemo therapy for a period of time and then to experience multi-year periods of stability. Renewed growth is countered by a new chemo protocol. The trick in SUCCESSFULLY managing the disease is to contain the growth in a way that the cumulative impact of the disease AND the therapies does not severely impair the child.
Unfortunately for Clay his disease has not followed the rules of PLGAs. This tumor-type rarely bleeds; Clay's first presented itself through a hemorrhage. The tumors grow but rarely spread; Clay's disease had gone beyond the large mass in his brain to include a sheathing around his spinal cord when first diagnosed. Clay has another confounding genetic condition called NF1 that which can interact with the disease and/or chemo in unknown ways.
It took 2 years and three different chemo protocols to bring a period of stability to the disease which lasted a mere 3-4months. We shared with you Clay's six-month scan showed new growth and increased pressure on his spine resulting in "fraying" of spinal cord which the docs surmised was from blockage of spinal fluid from the disease. As you know Clay already has a brain shunt to relieve the cranial pressure from the main tumor; there is an equivalent surgical implant for the spine which Clay may need to endure.
At this time we did another round of consults with the few experts on this disease and found that there was no consensus on next step for Clay. We decided that we would enroll Clay in a clinical trial at Johns Hopkins for a biologic agent, Sorafenib that has shown some real treatment promise. Sorafenib had been developed and approved for a form of melanoma and was being studied for its safety and effectiveness with PLGA's because this type of melanoma and PLGA's share a similar cellular mutation. Sorafenib was designed to disrupt the "signal pathway" for this mutation which in theory should disrupt the growth of the cells. After two weeks of paper-work, diagnostics and a full day at Hopkins with the drug in Mary's purse ready to walk out and begin the protocol, the lead institution of the trial, NYU called Hopkins and pulled Clay from the trial due to his history of hemorrhage. At the eleventh hour Mary and the Hopkins docs switched Clay to another experimental agent (RAD 001) which has similar qualities as Sorafenib.
At this time Clay described his eyes as being "fuzzy" followed a week later by nearly 90% loss of vision. Suddenly Clay was able to distinguish only the largest contrasts; his ability to navigate the bathroom or his dinner plate was gone. He was literally smacking into walls and going down stairs on his bottom. The "hope" was that the vision loss was due to the new drug --which if the case would allow the vision to return after cessation of treatment. This proved not to be the case and the latest MRI---taken a mere six weeks later brought us very disturbing news. The disease had progressed roughly 25% since the last MRI with advance in both the main tumor area, the spinal column and possible two new spots in the brain.
Clay's coordinating physician, Dr. Roger Packer (truly one of the top 5 authorities on this disease) did not spare us his concerns. The speed of the advance was "highly uncharacteristic". It is "rare" for this type of cell-type to mutate into a "more aggressive form" but not "unheard-of". Packer used the terms "proving to be chemo-resistant" and "on the verge of snow-balling out of control". While he was clear not to present this as his prognosis at this time, he did suggest we contemplate many new scenarios such as massive radiation(which would most certainly leave Clay significantly damaged) and "end-stage situations".
Dr. Packer recommended and we agreed to a chemotherapy protocol combination of Avastin and CPT11 which has shown some very good results in providing a quick punch to the disease. The down-side to the drug is that the shrinkage does not last all that long and when growth returns it is usually fairly strong. In addition, one of the side-effect risks is especially dangerous to Clay. Avastin is effective because it "goes after" the blood supply to the disease. For a kid with a history of hemorrhage this increases the danger 100-fold. With the enemy making such inroads we felt we had no choice but to throw everything at it we have at it in hopes to stop the momentum . So on Thursday Clay began this new treatment.
Now for some good news. First, Clay did not have an allergic reaction to the new drugs and the side-effects are so far manageable. This is HUGE because it means we can continue the treatment and give it a chance to work. And on his vision----he was prescribed a powerful steroid to give this blood vessels and nerves a "boost" and that alone has produced some vision improvement. What this means is that Clay's vision "machinery" still has some capability so if the Avastin/CPT11 succeeds in pushing back the disease he could regain greater vision function.
Along with the steadfast support of you, our family and friends, this bit of good news has cheered us greatly. Through it all Clay has been in remarkably good spirits and there is no way you could tell from his attitude that he was not your typical 6-year old excited about Christmas. We are hopeful for more good news over the coming weeks and Clay's docs think it reasonable to be hopeful. It is not lost on us the powerful symbolism of this time of year and we look forward to re-telling the story of Clay's Christmas Miracle in the many years to come.
From the pure of heart:
Clay to the nurse as she is preparing him for his latest MRI (which includes full, intravenous sedation): "Don't worry, Mouse-y and I are excellent at this!"
Grace, when asked what she wants for Christmas: "What I really want is for Clay to see again."
Clay, while strapped to the drug infusion machine at the clinic: "Daddy, when you and I are in heaven will you spread your blanket next to mine?"
May you all have a blessed Christmas and holiday season.
It has been 2 years and 8 months since Clay's first trip to the emergency room with what we thought was a severe concussion. 24 hours and three hospitals later we learned that Clay was suffering from a slow-growing cancer called Juvenille Pilocytic Astrocytoma (JPA) which is part of a class of cancers called low-grade gliomas (PLGA's)that account for roughly 30% of pediatric brain tumors. While less is known about pediatric conditions than adult disease relatively less is known about PLGA's due to a set of factors including physical, institutional, financial and legal. What is known about PLGA's is that while there is no cure for the disease many children survive the disease by using a combination of chemo and radio therapies to contain its growth until maturation to adulthood when the cell mysteriously stops mutating. Evidence of the lack of medical understanding of PLGAs is that the first line of defense is a chemo protocol that is over sixty years old.
It is not uncommon for PLGA kids to undergo chemo therapy for a period of time and then to experience multi-year periods of stability. Renewed growth is countered by a new chemo protocol. The trick in SUCCESSFULLY managing the disease is to contain the growth in a way that the cumulative impact of the disease AND the therapies does not severely impair the child.
Unfortunately for Clay his disease has not followed the rules of PLGAs. This tumor-type rarely bleeds; Clay's first presented itself through a hemorrhage. The tumors grow but rarely spread; Clay's disease had gone beyond the large mass in his brain to include a sheathing around his spinal cord when first diagnosed. Clay has another confounding genetic condition called NF1 that which can interact with the disease and/or chemo in unknown ways.
It took 2 years and three different chemo protocols to bring a period of stability to the disease which lasted a mere 3-4months. We shared with you Clay's six-month scan showed new growth and increased pressure on his spine resulting in "fraying" of spinal cord which the docs surmised was from blockage of spinal fluid from the disease. As you know Clay already has a brain shunt to relieve the cranial pressure from the main tumor; there is an equivalent surgical implant for the spine which Clay may need to endure.
At this time we did another round of consults with the few experts on this disease and found that there was no consensus on next step for Clay. We decided that we would enroll Clay in a clinical trial at Johns Hopkins for a biologic agent, Sorafenib that has shown some real treatment promise. Sorafenib had been developed and approved for a form of melanoma and was being studied for its safety and effectiveness with PLGA's because this type of melanoma and PLGA's share a similar cellular mutation. Sorafenib was designed to disrupt the "signal pathway" for this mutation which in theory should disrupt the growth of the cells. After two weeks of paper-work, diagnostics and a full day at Hopkins with the drug in Mary's purse ready to walk out and begin the protocol, the lead institution of the trial, NYU called Hopkins and pulled Clay from the trial due to his history of hemorrhage. At the eleventh hour Mary and the Hopkins docs switched Clay to another experimental agent (RAD 001) which has similar qualities as Sorafenib.
At this time Clay described his eyes as being "fuzzy" followed a week later by nearly 90% loss of vision. Suddenly Clay was able to distinguish only the largest contrasts; his ability to navigate the bathroom or his dinner plate was gone. He was literally smacking into walls and going down stairs on his bottom. The "hope" was that the vision loss was due to the new drug --which if the case would allow the vision to return after cessation of treatment. This proved not to be the case and the latest MRI---taken a mere six weeks later brought us very disturbing news. The disease had progressed roughly 25% since the last MRI with advance in both the main tumor area, the spinal column and possible two new spots in the brain.
Clay's coordinating physician, Dr. Roger Packer (truly one of the top 5 authorities on this disease) did not spare us his concerns. The speed of the advance was "highly uncharacteristic". It is "rare" for this type of cell-type to mutate into a "more aggressive form" but not "unheard-of". Packer used the terms "proving to be chemo-resistant" and "on the verge of snow-balling out of control". While he was clear not to present this as his prognosis at this time, he did suggest we contemplate many new scenarios such as massive radiation(which would most certainly leave Clay significantly damaged) and "end-stage situations".
Dr. Packer recommended and we agreed to a chemotherapy protocol combination of Avastin and CPT11 which has shown some very good results in providing a quick punch to the disease. The down-side to the drug is that the shrinkage does not last all that long and when growth returns it is usually fairly strong. In addition, one of the side-effect risks is especially dangerous to Clay. Avastin is effective because it "goes after" the blood supply to the disease. For a kid with a history of hemorrhage this increases the danger 100-fold. With the enemy making such inroads we felt we had no choice but to throw everything at it we have at it in hopes to stop the momentum . So on Thursday Clay began this new treatment.
Now for some good news. First, Clay did not have an allergic reaction to the new drugs and the side-effects are so far manageable. This is HUGE because it means we can continue the treatment and give it a chance to work. And on his vision----he was prescribed a powerful steroid to give this blood vessels and nerves a "boost" and that alone has produced some vision improvement. What this means is that Clay's vision "machinery" still has some capability so if the Avastin/CPT11 succeeds in pushing back the disease he could regain greater vision function.
Along with the steadfast support of you, our family and friends, this bit of good news has cheered us greatly. Through it all Clay has been in remarkably good spirits and there is no way you could tell from his attitude that he was not your typical 6-year old excited about Christmas. We are hopeful for more good news over the coming weeks and Clay's docs think it reasonable to be hopeful. It is not lost on us the powerful symbolism of this time of year and we look forward to re-telling the story of Clay's Christmas Miracle in the many years to come.
From the pure of heart:
Clay to the nurse as she is preparing him for his latest MRI (which includes full, intravenous sedation): "Don't worry, Mouse-y and I are excellent at this!"
Grace, when asked what she wants for Christmas: "What I really want is for Clay to see again."
Clay, while strapped to the drug infusion machine at the clinic: "Daddy, when you and I are in heaven will you spread your blanket next to mine?"
May you all have a blessed Christmas and holiday season.
Tuesday, December 20, 2011
Please pray for Clay
We could really use your prayers this week. Clay has lost much of his vision, and the doctors feel it is due to tumor progression. We have stopped the oral medication and are switching this week to what the doctor feels is his best shot for regaining some vision and stopping the progression. Today Clay is having another MRI to rule out a hemorrhage. Tomorrow he will have a PICC line placed, and Thursday he will start this new chemotherapy. Please keep him in your thoughts as we pray for a Christmas miracle to restore our boy, who just turned 6 last week.
Sunday, December 4, 2011
New Protocol
Thank you for all of the e-mails wishing us good luck on Friday. It was a very long day at Hopkins -- we left the house at 8 a.m. and got home at 8 p.m. Things were moving along fairly well on schedule until about 3 p.m. The trial we were signing up for had been approved that day, and I was going over the potential drug side effects with the researcher when the final word came in from NYU (the lead hospital for the trial) that Clay was ineligible for the trial. Since his tumor hemorrhaged once, they decided it was too high risk to include him in the trial for fear that it could happen again (an unlikely, but serious side effect of the drug). At that point Clay had had an MRI, x-ray, physical exam, and blood tests done. So, we switched gears and starting discussing plan B. Another oral drug that is meant to switch off what is making this tumor grow. This drug is also in clinical trial, but the trial is closed, so we are doing the drug "off-trial." Enough kids have been enrolled in the trial and are doing well, and bleeding is not a potential side-effect. So for now this is the protocol for Clay and we pray it does what it needs to do. For now we will have weekly appointments to check his blood work, and if all goes ok, another MRI in three months. A special thanks to my mom for keeping Clay happy for hours while I met with the doctors.
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