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| July 15, 2008 Medical explanations, background, and how we got to cutting holes in her head... |
Truth be told, this entry is being written long after both temporal decompressions were
performed. While some of the information is generally ancient history, its all relative to how we
got to where we are today. This is the background garbage (read as gar bajjjjjj, it seems more
sophisticated) you need to know to understand what's been going on. And finally, it will
explain how Avalon came to be the proud owner of two new holes in her head.
I thought maybe I should do this section, before I plunged into yet another diatribe about life in
the hospital with Avalon. For any old-timers reading this site, some of this may be repetitive.
Then again, when you're not living it, I don't blame anyone on the planet for tossing this junk
to the curb, as soon as I say it. I know I'd like to!
For the people who've recently joined our merry little band of followers, well, by golly, this may
be rather helpful. So, sit back and get comfy - I feel one of my "books" coming on. Aw criminy,
who am I kidding? When don't I have a "book" coming along? Yeah, yeah, I'm wordy. Deal
with it.
------------
This site, and Avalon's medical life began with her diagnosis of Acute Lymphoblastic Leukemia
at the tender age of 17 months. Avalon completed a 26 month chemotherapy protocol, that
officially ended 12-28-06.
Protocol is the scientific word for a specific treatment plan. In the pediatric cancer world, the
doctors and scientists have banded together to try to improve our children's survival rates.
Hospitals work collaboratively, to be able to have enough children enrolled in a study, to show
statistical progress with new or altered treatments. What that means is, all Children's
Oncology Group affiliated hospitals, will treat children diagnosed with ALL within a given time
period, with the same treatment plan. Plans/protocols are very specific, and often will have
several different "arms", that test small differences in treatment. For example, Avalon's
protocol was called CCG-1991. CCG-1991 had 4 arms: standard - with 1 Delayed
Intesification and oral Methotrexate, variation 1 - with 2 D.I.s and oral MTX, variation 2 - with 1
D.I. and IV (intravenous) MTX, and variation 3 - with 2 D.I.'s and IV MTX. Avalon was
randomized into the standard arm of treatment.
The standard arm of any treatment protocol, is always the best known course of treatment at
that time. The different test arms are variations of the standard, trying to determine if any
change of the standard will produce less negative side effects, while maintaining or improving
survival rates. If, at any time, the researchers discover that a certain regimen is better than
another, it becomes the new "standard" and new variations are tested.
Why on God's green earth did I just explain all that nonsense? Two reasons. One, I think
knowing the scientific terms and processes behind anything you discuss is appropriate. Two, I
want it to be clear, I believe in the science behind Avalon's treatment protocol. I know that the
doctors did the best they could in curing her cancer - the resulting "fall-out" from the chemo
was nothing more than collateral damage - they had no way of knowing it would happen.
Also, there is something else worth noting here. The way C.O.G. hospitals work together is
nothing short of amazing. There are thousands of doctors who work as a team, sharing
information and consulting at a national level. Adult cancers don't work anything like this.
There are so many adult patients, that often - there can be several research projects going
within one hospital. Dr. A might be researching blah about breast cancer, while Dr. B is
researching bleck. Each doctor may prescribe entirely different treatment regimens. Within
our C.O.G. network, there is continuity of care. I email with people from around the world,
whose children have received the exact same drugs, in the same doses my daughter did. Its a
miracle of modern communication and cooperation. However, I also want to strongly note - the
C.O.G. is NOT St. Jude's. When you support St. Jude's, you are supporting that one
institution, and only the children who go there. St. Jude's is a money-making monster machine
that doesn't play well with others. If you support CureSearch - you're helping the hundreds of
hospitals that have chosen to work collaboratively.
OK, back to Avalon's issues. The most intensive part of Avalon's protocol was the two month
time period called Delayed Intensification. During D.I., the protocol hits the children hard and
heavy - trying to eradicate any and all hidden cancer cells. D.I. not only involves high doses
of several of the drugs they have already taken for 6 six months, it also involves several new
drugs that are so strong they are only used for a short period of time. D.I. is when most
leukemia kids lose their hair, when you can usually expect to do hospital time, and when our
kids are, generally speaking, the sickest. D.I. is followed by 18 months of Long Term
Maintenance chemo for girls, 30 months of LTM for boys. Yes, leukemia is a ridiculously long
battle.
During Delayed Intensification, the children receive several very toxic chemos, including the
heart-damaging Doxorubicin, the potentially stroke-causing Ara-C and Peg-Asparaginase,
and mountains of high dose steroids and oral and intrathecal Methotrexate. In other words,
they live through two months of chemical warfare.
Avalon had a pretty difficult D.I.. She was hospitalized with a suspected stroke one time, then
hospitalized several times over a two month period - trying to fight off an infection in her
subcutaneous infusion port. Eventually, she lost that port, and had to deal with having an
open wound packed - because she had necrotic (dead) tissue in her chest wall surrounding
the infected port. During this time, Avalon spent several weeks on and off of Vancomycin - an
extremely strong IV antibiotic.
Why does this "ancient history" matter to what Avalon's going through now? Because this is
when it all began to come apart...we just didn't know it then. During one of her early
hospitalizations for the fevers from the port infection, Avalon began holding her head. She
would cover her ears and grimace. We investigated oto-toxicity from the Vancomycin, excess
wax in her ears, and even somewhat attributed it to sound sensitivity that can be caused by
steroids. Basically, the doctors checked everything they knew to check - but her sudden
sensitivity was never really understood. We just chalked it up to one of the weirdo things that
chemo can cause. When a child isn't old enough to be verbal, its difficult to diagnose some
things.
Now, we believe those early episodes of holding her ears, were when her intracranial pressure
was first beginning to climb. Looking back, (oh the beauty of hindsight) we realized that the
head-holding coincided with the strongest chemos and IV Vancomycin time period. So exactly
what damaged Avalon's brain? Your guess is as good as any. It could have been any of the
D.I. chemos, the intra-thecal (in her spinal column) Methotrexate, the high dose steroids, or
the IV Vancomycin. Better yet, it could have been a combination of any of the above. We'll
never really know. And honestly, it doesn't much matter. It just happened. No sense in trying
to nail down the specific why, its the when that's germain to this discussion.
Knowing that Avalon's pressure began rising during D.I. is important, to help explain her
behavior now. Avalon was merely 22 months old when she first showed the signs I'm referring
to. She was definitely not verbal. We interpreted behavior by what we saw, she never said
anything about her head, or owies, etc. We merely saw sound sensitivity and those early days
of holding her ears.
In adults, normal intracranial pressure is 10-12. Avalon's has been so high its exceeded the
measuring capacity of the manometer - greater than 45. That's a pressure four times that of a
normal adult! Intracranial pressure headaches are some of the worst pain you can imagine.
They are so bad, nothing but strong narcotics can even remotely dull them. Adults with
pseudo-tumor cerebri often become drug addicts, trying to learn to live with the pain. There
are even papers published about suicide rates in adult PTC patients, because the pain is
unbearable. And yet, my tiny little girl lived with that pain as her norm - from so early in her
life, she came to accept it as her reality.
This has affected every other aspect of Avalon's care. Because Avalon lived with what adults
would consider bone-crushing pain as her daily norm from such a young age, it entirely reset
her pain parameters. Avalon doesn't "feel" pain in the way most people do. "Feel" isn't the
exact right word, "perceive pain" is perhaps more appropriate. Chronic pain patients often
have different perceptions of pain than the general population, they have to adapt to the pain
to live. Avalon is a chronic pain patient - whose pain developed so young - she really grew up
knowing nothing else. It makes her difficult to treat now, because she's not really capable of
explaining to medical caregivers when her body is in trouble. We are always playing
"catch-up", trying to explain to doctors and nurses - that just because she denies any pain - it
doesn't mean her body is not in turmoil. And by the time Avalon says something hurts....she's
in serious trouble.
While we now know that D.I. was the beginning of things unravelling, we didn't realize she had
a problem, until nearly a year after the fact. You see, high pressure behaviors - such as
irritability, anxiety, excessive sleep, sound and light sensitivity - are all normal chemo side
effects. I would mention Avalon's issues at each check up, her nurse would note them, and we
both presumed they were par for the course. Even Avalon's balance issues were very normal.
Vincristine, the main IV chemo drug for Leukemia, is well known for causing peripheral
neuropathy. Vincristine can cause tingling in the extremities, difficulty walking ("drop foot")
and balance problems. Avalon exhibited all of the above.
Actually, by the Spring of 2006, Avalon's balance and mobility issues were so bad, Heme-Onc
(Hematology-Oncology - her cancer department) referred her for a physical therapy
evaluation. The physical therapist was very thorough in testing her walking, climbing,
balancing, running, and mobility issues. The therapist's conclusion was that Avalon had an
"acquired laziness". She felt that when Avalon had been the most ill, we'd had to carry her
around often. She went on to say that Avalon had become accustomed to that, and that her
muscle development was substandard because we'd let her "get away with" being babied too
much. She said we needed to basically force Avalon to walk further, and to go up and down
stairs, and to stop giving in to her requests to pick her up. I bear no ill will toward this woman,
she's actually very kind. However...she was WRONG about everything she said. I carry a
mountain-sized load of guilt for listening to her....
Over the next several months, we tried our best to follow the therapist's recommendations.
We would make Avalon walk up and down the stairs, and do the exercises that we were given.
We would make Avalon walk through parking lots, and in stores, even when she begged to be
carried. I'm telling you...even writing this makes my heart hurt. We were so wrong...and we
were so needlessly cruel. Of course we did everything out of love, and hopes of making her
life better - we had no idea at the time that we were so very mis-informed about her.
By late Spring of 2006, Avalon's sound sensitivity was reaching epic proportions. She couldn't
tolerate anything loud, and various pitches would cause her to run from a room covering her
ears. She rarely ventured outside, because A) she was scared of a horrible neighbor who had
yelled at her, and B) she would tell us it was too loud or too bright outside. Sadly, we thought
the B) portion was just a response to the A) event. We even had her see the Heme-Onc
department's psychologist, to help her deal with the fear of the neighbor. While the fear was
real, (and very well-founded) again - we were missing the truth.
Finally, one day we were getting her ready for a monthly Heme-Onc clinic appointment. It was
one of her spinal tap/spinal chemo and IV chemo days, so Nick (daddy) was coming too, to
help with the other kids. We had an awful time trying to wake her up that morning. In fact, we
could barely keep her awake. When I took her out of the shower and sat her on the bed to get
her clothes - blink- she fell over asleep. When I sat her up and got her dressed, blip - she fell
right back over asleep. It was crazy! We were both glad we were heading to clinic anyway -
we'd have been calling and taking her in if we didn't already have a standing appointment.
During Avalon's physical exam, we explained to our nurse practitioner what had been
happening that morning. She, too, was concerned, even thought the exam and vitals
appeared to be perfectly normal. The nurse practitioner brought in our attending oncologist to
have him listen to our story. We explained her extreme sound sensitivity, her holding her
head, and about the sleeping thing. Dear man that he is, her doctor tried to explain to us that
sometimes kids pick something to complain about even though its not really a problem. He
thought Avalon was holding her head, because something else was bothering her that she
couldn't verbalize to us.
I explained to him that we very well understood that concept. At that time, our middle daughter
would complain of knee pain, if she had a runny nose, a cough, or diarrhea. Knee pain was
just her "thing". I was going on to tell him that I respectfully completely, utterly disagreed with
him... when Blip - Avalon just fell over in Nick's lap, asleep. She went from awake and active to
limp and asleep - no warning. You should have seen the attending's face! I said, "See?!
That's what we're telling you!" Suddenly, it was like watching someone scroll through old
computer files. You could see on the doctor's face as he was scrolling through memories of
previous experiences with Avalon. He suddenly thought back to the Spring of 2005 when
she'd first held her ears during the Delayed Intensification hospital stays... He clicked on the
same information we had.
Because Avalon was already scheduled into the surgery center for her LP (lumbar puncture,
layman's terms - a spinal tap), they decided to keep her appointment. They sent her down for
a CT scan first though - to make sure there wasn't an imminent problem. Also, they ordered
extra blood work, and told us they'd be removing extra fluid to check for a spinal viral or
bacterial infection. Good and worried, we headed down to the surgery center.
After Avalon's procedure, the doctor came out with an odd look on his face. He told us
Avalon's opening pressure (how they measure intracranial pressure) was "a bit higher than
what they normally see." After several direct questions, he finally gave it up, and said it was
30. It took a bit more prodding - but eventually he said they normally would expect to see
10-12. That was the beginning of a whole new world for us....
After OR, we went back up to clinic. Avalon's attending came out to talk to us. He was
obviously concerned, and said he was worried about something called pseudotumor cerebri.
He immediately referred us to the Opthalmology department to have them confirm the
diagnosis. They were able to get us in within a few days. However, sad to say, the Children's
Hospital ophthalmologists were/are very uneducated about pseudotumor in children. In
children, it presents very different from adults. In fact, Avalon was seen by the head of the
department and he not only missed the diagnosis, he argued vehemently that she couldn't
possibly have it - she was too young. He was dead wrong.
Thankfully, Avalon's oncologist is not only a brilliant man, he's a determined one. The
ophthalmologist's opinion didn't set well with him. There was too much evidence to the
contrary. Dr. Ranalli (the oncologist) referred Avalon to the neurology department for another
opinion. He even called and begged/bribed/cajoled his way into Avalon being seen within a
few weeks, rather than the typical 4 months. As with Dr. Ranalli, the neurologist, Dr. Zamel,
also felt the evidence pointed toward pseudotumor cerebri. To confirm it, he called in a favor
from a colleage from Ohio State, Dr. Susan Benes, a neuro-ophthalmologist. Dr. Benes is a
world-renowned specialist in pseudotumor - she's basically the best of the best. Dr. Zamel
begged Dr. Benes to see Avalon, explaining why he and Dr. Ranalli were worried - and why
they wanted her to take a look. Thank heavens and all that is good in the universe....Dr.
Benes agreed to take Avalon as a patient.
Dr. Benes discovered that not only did Avalon have pseudo-tumor cerebri (PTC), but she'd
had it long enough to cause a considerable amount of damage to her vision. At the time of
Avalon's first visit with Dr. Benes, Avalon had lost nearly 70% of her peripheral vision! Avalon
had no lower half peripheral vision. She didn't avoid going up or down stairs because she was
"lazy" - the poor child couldn't see them! She didn't lay on the floor to play with her toys
because she was being lazy - she laid down so she could see into her doll house, or see into
whatever she was playing with. She literally only had a very narrow tunnel she could see
through. Yes, the retroactive guilt was suffocating....
We spent the next several months discussing treatment options with Dr. Benes, Dr. Ranalli,
and Dr. Zamel. Most pseudotumor cases in children happen in pre-teens or teens. We
couldn't find any information about 2 year olds with it. The few published papers about PTC in
children were very anecdotal, and usually only covered a handful of children at a particular
institution. By and large, the cases were similar. PTC occurred after or during chemotherapy
- presumably as a response to the stress on the body from the chemo. In nearly all the cases,
once the chemo was fully out of the system, the PTC would resolve. We determined to try to
manage the PTC, trying to buy Avalon some time until she could complete chemo. In the
mean time, the governing body of the Children's Oncology Group held conferences to decide
what to do with Avalon. After much discussion, they decided to discontinue her steroids and
intrathecal methotrexate 6 months early - to prevent further damage to the brain, and to
maybe even reverse what had already happened. At the time, we were terrified. (OK, we still
are...) If research had shown that 26 months of chemotherapy was the ideal time period,
stopping two major elements of it 6 months early seemed insane to us. However, we took the
recommendations of the doctors, knowing that their primary concern was also her health. We
took a leap of faith.
We tried several ways of reducing Avalon's pressure. We tried two separate drugs that can
be quite effective in reducing CSF (cerebral spinal fluid) production. Unfortunately, she
couldn't tolerate either one. Both drugs caused severe tachycardia and GI distress. The one
drug made her so sick, she lost 6 pounds in a week - and she was only 3 years old! It was
obvious that drugs weren't the answer. While we tried to wait out Avalon's chemo time, we did
"serial" LP's, repeated spinal taps where excess fluid was removed - to temporarily lower her
pressure. The problem is, its a short-lived solution. Spinal fluid replenishes within 24-48
hours, so each "draw-down" (as we called them) only gave her brief respite.
Finally, in August of 2006, Avalon's sight reached critical levels. Her Left optic nerve
developed a bubble of fluid that threatened her vision. She went in the next day for an Optic
Nerve Sheath Fenestration, to keep her from going blind. The ONSF was performed by Dr.
Benes, in the Surgery Center of our Children's Hospital. It was part well-known procedure,
part stroke-of-genius by Dr. Benes.
An ONSF is an operation where the surgeon puts purposeful holes in the myelin sheath that
covers the optic nerve. The holes are to allow drainage of Cerebral Spinal Fluid (CSF)
directly behind the retina, reducing the pressure on the critical final 4 mm of the optic nerve.
ONSF operations have been used often in the treatment of adult PTC. They are very effective
in saving vision. However, a lot of specialists argue against their use, because they must be
repeated. Over time, the body will heal the holes, necessitating another operation to re-open
them. Dr. Benes' genius is in a procedure she has pioneered. She treats the fenestrations
(holes) with a small amount of a chemo agent - to retard their self-healing process. Therefore,
she keeps the fenestrations viable - long after most surgeries have had to be repeated.
Avalon's fenestrations are two years old, and flowing beautifully.
While the ONSF did improve Avalon's balance, heart rate, and vision nearly overnight - it
unfortunately, had very little effect on her ICP (intra-cranial pressure). By December of 2006,
all doctors involved agreed - we had no choice but to place a permanent V-P
(Ventricular-Peritoneal) shunt. Avalon needed the VP shunt to remove the excess fluid from
her brain and stop the degradation of her vision, as well as the other host of high pressure
behaviors she suffered from. Surgery was scheduled, and we entered into our next phase of
Avalon's medical life.
Avalon's first VP shunt was placed in January of 2007. I won't go into details of 2007 here - it
would be another 12 pages of info - and my fingers are tired. Besides, if all goes as planned, I
will eventually "back-date" the site - with all the gory details. Suffice it to say, it was nice to no
longer be ritualistically poisoning Avalon in 2007 (chemo ended in Dec 2006) - but her life
really didn't get much easier.
Moving forward to 2008, and Avalon's ICP was once again showing signs of being too high. I'll
leave those details for "back-dating" about the May 28, 2008 shunt surgery. Guess you'll just
have to trust me on this! Anyway, after the May 28th surgery...Avalon really began to fall
apart. She developed panic attacks, language issues (stuttering, ephasia), emotional control
problems, and a host of other symptoms of continuing high intra-cranial pressure.
A follow-up check-up with Dr. Benes showed that Avalon's vision was, once again, under
attack. The peripheral vision loss we had seen in April and May, was still increasing. In fact, it
had increased enough that it looked as if the shunt surgery had brought about no
improvement at all. Not only was Avalon's peripheral vision continuing to diminish, but her
"blind spot" (you know, the one we all have...) had again increased in size - by a rather large
percentage. Avalon was in trouble.
Dr. Benes recommended an immediate LP to check Avalon's ICP. Avalon's new
neurosurgeon, Dr. Kosnik, is not a huge proponent of checking ICP - he's more of a "watch
how they act" kind of guy. Unfortunately, he's learning that approach just doesn't work with
Avalon - she's too good at masking problems. (reference the earlier discussion of living with
pain for so many years) Dr. Benes didn't even bother asking him, she just called Heme-Onc
and arranged an LP for as fast as they could schedule it. The signs were all there for high
pressure.
Sure enough, the July 9, 2008 LP showed a pressure of 28. For someone with a shunt, that's
insane. We were scheduled in to see Dr. Kosnik within a few days after that, so we could
discuss options. Considering how much Avalon's eyes had progressed, and how awful her
behavior had been since the shunt revision, I whole-heartedly believed that the shunt would
have to be revised or entirely replaced by Dr. Kosnik. Oh my...did he have other plans.
Avalon was finally feeling better after a horrible weekend of painful headaches. When we saw
Dr. Kosnik, we explained everything that had happened since the LP on last Wednesday, and
how she was doing that morning. Although, it was silly to explain it. He'd been in contact with
his nurse and nurse practitioners - he knew everything that was happening as it happened.
Which is why the following took place the way it did.
After my timeline of her pain after the LP, Dr. Kosnik agreed with myself and the NP's, that
Avalon had most likely experienced a spinal leak from the LP. Dr. Ranalli (who did the LP) had
said it was an unusual stick, plus he had drawn off 12 cc's of CSF to temporarily reduce her
pressure. We had also gone up to the neuro-surgery unit and had the NPs turn Avalon's
shunt down to the final setting. Adding all of those fluid-reducing things together...and Avalon
had experienced a low-pressure nightmare. The interesting thing is, by the time we saw Dr.
Kosnik on the Tuesday following the LP, Avalon had already progressed from low pressure, to
a "honeymoon" period of good pressure, and back to high pressure. This poor kid just can't
catch a break.
So, Dr. K's news went something like this. He said obviously, Avalon doesn't handle low
pressure well, she's sensitive to it. He asked me why Dr. Elton had originally used a VP shunt
with her, then he said he agreed with the logic I reported to him. He said he did feel Avalon
had experienced a spinal leak, and that we would do an MRI tomorrow morning, before
surgery - to make absolutely certain the spine had healed completely. Then he said, he did
not believe that an LP shunt would be good for Avalon, they can easily over-drain. An LP
shunt could leave her with the possibility of the same bone-crushing headaches she had just
lived through...nearly every day of her life. We agreed, that would be awful. His answer?
Temporal decompression.
Dr. K introduced temporal decompression as a very old operation, one that isn't used very
often now, because of the popularity of VP shunts. However, he said that temporal
decompression can be helpful for kids with slit ventricle syndrome (which Avalon has), and
also kids who have odd pressures and extreme pressure sensitivities like Avalon. He
explained that he would be removing a piece of Avalon's skull on each side of her head, close
to her temples, to allow the excess fluid somewhere to flow, to reduce pressure.
All I could think to ask was about Anam - who loves to whack Avalon in the head. Dr. K
assured me that he had never had someone who sustained a serious brain injury from being
hit like that, and that, in fact, he'd never even read about it. Where they remove the piece of
skull, the brain is rather "deep" in the skull. Also, they remove it from behind the strongest
part of the jaw muscle. The jaw muscle then acts as the protective barrier, in place of the
skull. The difference is the muscle has a little more "give" allowing the fluid to press out on it.
Dr. K said we would be able to crudely check Avalon's pressure, by how "full" her holes feel. If
they feel tight and full - her pressure is up. If they're nice and squishy, she's doing well.
Basically, its like adding a baby's fontanel on each side of her head.
The only other thing I could think to ask about was why not check the shunt? I said, "I don't
think its working". His answer? I can't check it. He said there had been too much in and out in
a short time, he was afraid the skin couldn't handle being opened. He was afraid he wouldn't
be able to close it. What a horrifying thought... And yes, it makes my blood boil that the other
surgeon left her in such a state. But that's for other discussions....
About this time, Dr. K moved on to his next patient. I had a few minutes to process my
thoughts. I found myself in awe of the moment. I had sat there, listening as a man proposed
drilling large, permanent - holes in my daughter's head, and easily agreed with him. There
was no tight chest, no knot in my stomach. I accepted it all, as if someone asked to do a panel
of bloodwork. Not to mention, Dr. K had requested to see if Avalon could do the pre-op MRI
with NO sedation. He said he thought she would need to do a lot of MRI's over her lifetime,
and if she could learn to tolerate them, it would be easier on her in the long run. Again, I
agreed. I couldn't believe myself. I decided it was a sign, I not only trust Dr. Kosnik - I have
good reason to. I've learned a lot about listening to my "guts" in the past few months. In this
case, my gut instinct was actually OK. Who could have imagined?
In a few minutes, Dr. K's nurse, Margaret, came in. She came with pre-op consent forms, and
to discuss blood work, the MRI, etc. She asked me if I had any questions about the
operation.... Um....yes! Only about 1000 of them....but I was honestly too dumbstruck to come
up with a single one. I told her I was so flabbergasted I couldn't think of a thing...but I'd get
back to her, if I ever managed to concentrate long enough. She was reassuring and as warm
as always, and reminded me that we would see him before the operation tomorrow - if we
wanted to ask anything. Sure, sure...that sounded great. My mind had switched over to
slightly-freaked-out-oatmeal. I was good for nothing but set decoration...
Margaret and I then covered the pre-op blood work we'd need. We decided it would be much
easier if we just popped down to Heme-Onc clinic and had them do the stick. After all, we
know them and Avalon loves the PCA's (nurses aides) that do the labs. Besides, Heme-Onc is
always more than happy to do labs for any cancer patient. They always like to see the results
too!
So, a few shocking discussions later, we were off to Heme-Onc clinic. Margaret sent a lab
order with me, but it was blank. She cracks me up! She sent it with the instructions, "They
know way more than I do. Just tell them what she's having done - they'll be better suited to
figure it out." What a hoot! Sure enough, it took a hot second for someone to order the
appropriate labs. It only took a few minutes for them to be drawn. Meanwhile, there was the
chorus of "What?!" from everyone who heard why we were there. Avalon's nurse practitioner,
Diane, amused the britches off of me. (OK, its a figure of speech...no one wants to see me
with my pants off!)
Diane plopped down next to me in the waiting room and asked me what was up. Of course
she already knew about the LP, it was Avalon's Heme-Onc attending, Dr. Ranalli, who had
drawn it. So, Diane just wanted to know what Dr. K wanted to do. I'm here to report, she was
just as "icked out" as I was about the prospect of cutting out hunks of Avalon's skull.
Yep-a-rooney, the great and glorious Diane even gave a oooooh-gross shudder when I
explained that we could eventually check her pressure by pushing on the new squishy parts.
Ha ha! I managed to gross out the unflappable Diane. There ought to be a prize for that.
Actually, I was rather ornery. I told her I'd be hog-tying her and forcing her to touch the
squishies. After all - it was her chemo that caused the problem. Therefore, if she could drag
me into this mess - I was "taking her with me!" down the bleck path. You have to love a nurse
who not only admits to being "icked out" - but who loves you enough to joke with you about it.
She was the perfect prescription for me at that exact moment....
The PCA's were hysterical in their own way. Our dear friend, Ryan, was his typical goofy self.
No grossing out for him, he said he couldn't wait to feel it! Ewwwww....... Of course, they also
listened to my rather dis-combobulated ramblings..... Again, the perfect prescription for that
exact moment. I honestly think I repeated myself 20 or 30 times. I just couldn't wrap my pea
brain around what we were preparing for. Thankfully, the awesome Heme-Onc crew once
again stepped up to the plate. They met my worry and fear with the perfect combination of
humor, concern, love, and support. I left the clinic convinced I'd made a fool of myself, but
knowing it was OK if I did. Avalon left feeling loved, several dozen hugs will do that for you.
Both of us left better than we came in - you can't ask for more.
From clinic, we headed home and began the insane round of phone calls and preparations for
the coming week. "Hello, your daughter is having radical brain surgery tomorrow," will set a
family's collective rear end on fire like you can't imagine! Below the pink line is an email I sent
out to family and friends the night before the first temporal decompression. I posted a nearly
identical email to my online support group, ALL-Kids. My support group friends have become
a second family to me. Some day, I'll write an obscenely long entry about how much I adore
them.
The following email is included for one reason - honesty. I think its important that anyone
reading this site, gets a real sense of our perceptions of the events of Avalon's life. We can
only teach, if we are honest in sharing all of our feelings - the good, the bad, and the
probably-shouldn't-be-mentioned-in-public. I believe in the goodness in the heart of mankind,
and hope that somehow, Avalon's journey is meant to touch the hearts of the people around
us. At the very least, that's what I hold onto on the difficult days...so let me live in my
delusional bubble please.....
performed. While some of the information is generally ancient history, its all relative to how we
got to where we are today. This is the background garbage (read as gar bajjjjjj, it seems more
sophisticated) you need to know to understand what's been going on. And finally, it will
explain how Avalon came to be the proud owner of two new holes in her head.
I thought maybe I should do this section, before I plunged into yet another diatribe about life in
the hospital with Avalon. For any old-timers reading this site, some of this may be repetitive.
Then again, when you're not living it, I don't blame anyone on the planet for tossing this junk
to the curb, as soon as I say it. I know I'd like to!
For the people who've recently joined our merry little band of followers, well, by golly, this may
be rather helpful. So, sit back and get comfy - I feel one of my "books" coming on. Aw criminy,
who am I kidding? When don't I have a "book" coming along? Yeah, yeah, I'm wordy. Deal
with it.
------------
This site, and Avalon's medical life began with her diagnosis of Acute Lymphoblastic Leukemia
at the tender age of 17 months. Avalon completed a 26 month chemotherapy protocol, that
officially ended 12-28-06.
Protocol is the scientific word for a specific treatment plan. In the pediatric cancer world, the
doctors and scientists have banded together to try to improve our children's survival rates.
Hospitals work collaboratively, to be able to have enough children enrolled in a study, to show
statistical progress with new or altered treatments. What that means is, all Children's
Oncology Group affiliated hospitals, will treat children diagnosed with ALL within a given time
period, with the same treatment plan. Plans/protocols are very specific, and often will have
several different "arms", that test small differences in treatment. For example, Avalon's
protocol was called CCG-1991. CCG-1991 had 4 arms: standard - with 1 Delayed
Intesification and oral Methotrexate, variation 1 - with 2 D.I.s and oral MTX, variation 2 - with 1
D.I. and IV (intravenous) MTX, and variation 3 - with 2 D.I.'s and IV MTX. Avalon was
randomized into the standard arm of treatment.
The standard arm of any treatment protocol, is always the best known course of treatment at
that time. The different test arms are variations of the standard, trying to determine if any
change of the standard will produce less negative side effects, while maintaining or improving
survival rates. If, at any time, the researchers discover that a certain regimen is better than
another, it becomes the new "standard" and new variations are tested.
Why on God's green earth did I just explain all that nonsense? Two reasons. One, I think
knowing the scientific terms and processes behind anything you discuss is appropriate. Two, I
want it to be clear, I believe in the science behind Avalon's treatment protocol. I know that the
doctors did the best they could in curing her cancer - the resulting "fall-out" from the chemo
was nothing more than collateral damage - they had no way of knowing it would happen.
Also, there is something else worth noting here. The way C.O.G. hospitals work together is
nothing short of amazing. There are thousands of doctors who work as a team, sharing
information and consulting at a national level. Adult cancers don't work anything like this.
There are so many adult patients, that often - there can be several research projects going
within one hospital. Dr. A might be researching blah about breast cancer, while Dr. B is
researching bleck. Each doctor may prescribe entirely different treatment regimens. Within
our C.O.G. network, there is continuity of care. I email with people from around the world,
whose children have received the exact same drugs, in the same doses my daughter did. Its a
miracle of modern communication and cooperation. However, I also want to strongly note - the
C.O.G. is NOT St. Jude's. When you support St. Jude's, you are supporting that one
institution, and only the children who go there. St. Jude's is a money-making monster machine
that doesn't play well with others. If you support CureSearch - you're helping the hundreds of
hospitals that have chosen to work collaboratively.
OK, back to Avalon's issues. The most intensive part of Avalon's protocol was the two month
time period called Delayed Intensification. During D.I., the protocol hits the children hard and
heavy - trying to eradicate any and all hidden cancer cells. D.I. not only involves high doses
of several of the drugs they have already taken for 6 six months, it also involves several new
drugs that are so strong they are only used for a short period of time. D.I. is when most
leukemia kids lose their hair, when you can usually expect to do hospital time, and when our
kids are, generally speaking, the sickest. D.I. is followed by 18 months of Long Term
Maintenance chemo for girls, 30 months of LTM for boys. Yes, leukemia is a ridiculously long
battle.
During Delayed Intensification, the children receive several very toxic chemos, including the
heart-damaging Doxorubicin, the potentially stroke-causing Ara-C and Peg-Asparaginase,
and mountains of high dose steroids and oral and intrathecal Methotrexate. In other words,
they live through two months of chemical warfare.
Avalon had a pretty difficult D.I.. She was hospitalized with a suspected stroke one time, then
hospitalized several times over a two month period - trying to fight off an infection in her
subcutaneous infusion port. Eventually, she lost that port, and had to deal with having an
open wound packed - because she had necrotic (dead) tissue in her chest wall surrounding
the infected port. During this time, Avalon spent several weeks on and off of Vancomycin - an
extremely strong IV antibiotic.
Why does this "ancient history" matter to what Avalon's going through now? Because this is
when it all began to come apart...we just didn't know it then. During one of her early
hospitalizations for the fevers from the port infection, Avalon began holding her head. She
would cover her ears and grimace. We investigated oto-toxicity from the Vancomycin, excess
wax in her ears, and even somewhat attributed it to sound sensitivity that can be caused by
steroids. Basically, the doctors checked everything they knew to check - but her sudden
sensitivity was never really understood. We just chalked it up to one of the weirdo things that
chemo can cause. When a child isn't old enough to be verbal, its difficult to diagnose some
things.
Now, we believe those early episodes of holding her ears, were when her intracranial pressure
was first beginning to climb. Looking back, (oh the beauty of hindsight) we realized that the
head-holding coincided with the strongest chemos and IV Vancomycin time period. So exactly
what damaged Avalon's brain? Your guess is as good as any. It could have been any of the
D.I. chemos, the intra-thecal (in her spinal column) Methotrexate, the high dose steroids, or
the IV Vancomycin. Better yet, it could have been a combination of any of the above. We'll
never really know. And honestly, it doesn't much matter. It just happened. No sense in trying
to nail down the specific why, its the when that's germain to this discussion.
Knowing that Avalon's pressure began rising during D.I. is important, to help explain her
behavior now. Avalon was merely 22 months old when she first showed the signs I'm referring
to. She was definitely not verbal. We interpreted behavior by what we saw, she never said
anything about her head, or owies, etc. We merely saw sound sensitivity and those early days
of holding her ears.
In adults, normal intracranial pressure is 10-12. Avalon's has been so high its exceeded the
measuring capacity of the manometer - greater than 45. That's a pressure four times that of a
normal adult! Intracranial pressure headaches are some of the worst pain you can imagine.
They are so bad, nothing but strong narcotics can even remotely dull them. Adults with
pseudo-tumor cerebri often become drug addicts, trying to learn to live with the pain. There
are even papers published about suicide rates in adult PTC patients, because the pain is
unbearable. And yet, my tiny little girl lived with that pain as her norm - from so early in her
life, she came to accept it as her reality.
This has affected every other aspect of Avalon's care. Because Avalon lived with what adults
would consider bone-crushing pain as her daily norm from such a young age, it entirely reset
her pain parameters. Avalon doesn't "feel" pain in the way most people do. "Feel" isn't the
exact right word, "perceive pain" is perhaps more appropriate. Chronic pain patients often
have different perceptions of pain than the general population, they have to adapt to the pain
to live. Avalon is a chronic pain patient - whose pain developed so young - she really grew up
knowing nothing else. It makes her difficult to treat now, because she's not really capable of
explaining to medical caregivers when her body is in trouble. We are always playing
"catch-up", trying to explain to doctors and nurses - that just because she denies any pain - it
doesn't mean her body is not in turmoil. And by the time Avalon says something hurts....she's
in serious trouble.
While we now know that D.I. was the beginning of things unravelling, we didn't realize she had
a problem, until nearly a year after the fact. You see, high pressure behaviors - such as
irritability, anxiety, excessive sleep, sound and light sensitivity - are all normal chemo side
effects. I would mention Avalon's issues at each check up, her nurse would note them, and we
both presumed they were par for the course. Even Avalon's balance issues were very normal.
Vincristine, the main IV chemo drug for Leukemia, is well known for causing peripheral
neuropathy. Vincristine can cause tingling in the extremities, difficulty walking ("drop foot")
and balance problems. Avalon exhibited all of the above.
Actually, by the Spring of 2006, Avalon's balance and mobility issues were so bad, Heme-Onc
(Hematology-Oncology - her cancer department) referred her for a physical therapy
evaluation. The physical therapist was very thorough in testing her walking, climbing,
balancing, running, and mobility issues. The therapist's conclusion was that Avalon had an
"acquired laziness". She felt that when Avalon had been the most ill, we'd had to carry her
around often. She went on to say that Avalon had become accustomed to that, and that her
muscle development was substandard because we'd let her "get away with" being babied too
much. She said we needed to basically force Avalon to walk further, and to go up and down
stairs, and to stop giving in to her requests to pick her up. I bear no ill will toward this woman,
she's actually very kind. However...she was WRONG about everything she said. I carry a
mountain-sized load of guilt for listening to her....
Over the next several months, we tried our best to follow the therapist's recommendations.
We would make Avalon walk up and down the stairs, and do the exercises that we were given.
We would make Avalon walk through parking lots, and in stores, even when she begged to be
carried. I'm telling you...even writing this makes my heart hurt. We were so wrong...and we
were so needlessly cruel. Of course we did everything out of love, and hopes of making her
life better - we had no idea at the time that we were so very mis-informed about her.
By late Spring of 2006, Avalon's sound sensitivity was reaching epic proportions. She couldn't
tolerate anything loud, and various pitches would cause her to run from a room covering her
ears. She rarely ventured outside, because A) she was scared of a horrible neighbor who had
yelled at her, and B) she would tell us it was too loud or too bright outside. Sadly, we thought
the B) portion was just a response to the A) event. We even had her see the Heme-Onc
department's psychologist, to help her deal with the fear of the neighbor. While the fear was
real, (and very well-founded) again - we were missing the truth.
Finally, one day we were getting her ready for a monthly Heme-Onc clinic appointment. It was
one of her spinal tap/spinal chemo and IV chemo days, so Nick (daddy) was coming too, to
help with the other kids. We had an awful time trying to wake her up that morning. In fact, we
could barely keep her awake. When I took her out of the shower and sat her on the bed to get
her clothes - blink- she fell over asleep. When I sat her up and got her dressed, blip - she fell
right back over asleep. It was crazy! We were both glad we were heading to clinic anyway -
we'd have been calling and taking her in if we didn't already have a standing appointment.
During Avalon's physical exam, we explained to our nurse practitioner what had been
happening that morning. She, too, was concerned, even thought the exam and vitals
appeared to be perfectly normal. The nurse practitioner brought in our attending oncologist to
have him listen to our story. We explained her extreme sound sensitivity, her holding her
head, and about the sleeping thing. Dear man that he is, her doctor tried to explain to us that
sometimes kids pick something to complain about even though its not really a problem. He
thought Avalon was holding her head, because something else was bothering her that she
couldn't verbalize to us.
I explained to him that we very well understood that concept. At that time, our middle daughter
would complain of knee pain, if she had a runny nose, a cough, or diarrhea. Knee pain was
just her "thing". I was going on to tell him that I respectfully completely, utterly disagreed with
him... when Blip - Avalon just fell over in Nick's lap, asleep. She went from awake and active to
limp and asleep - no warning. You should have seen the attending's face! I said, "See?!
That's what we're telling you!" Suddenly, it was like watching someone scroll through old
computer files. You could see on the doctor's face as he was scrolling through memories of
previous experiences with Avalon. He suddenly thought back to the Spring of 2005 when
she'd first held her ears during the Delayed Intensification hospital stays... He clicked on the
same information we had.
Because Avalon was already scheduled into the surgery center for her LP (lumbar puncture,
layman's terms - a spinal tap), they decided to keep her appointment. They sent her down for
a CT scan first though - to make sure there wasn't an imminent problem. Also, they ordered
extra blood work, and told us they'd be removing extra fluid to check for a spinal viral or
bacterial infection. Good and worried, we headed down to the surgery center.
After Avalon's procedure, the doctor came out with an odd look on his face. He told us
Avalon's opening pressure (how they measure intracranial pressure) was "a bit higher than
what they normally see." After several direct questions, he finally gave it up, and said it was
30. It took a bit more prodding - but eventually he said they normally would expect to see
10-12. That was the beginning of a whole new world for us....
After OR, we went back up to clinic. Avalon's attending came out to talk to us. He was
obviously concerned, and said he was worried about something called pseudotumor cerebri.
He immediately referred us to the Opthalmology department to have them confirm the
diagnosis. They were able to get us in within a few days. However, sad to say, the Children's
Hospital ophthalmologists were/are very uneducated about pseudotumor in children. In
children, it presents very different from adults. In fact, Avalon was seen by the head of the
department and he not only missed the diagnosis, he argued vehemently that she couldn't
possibly have it - she was too young. He was dead wrong.
Thankfully, Avalon's oncologist is not only a brilliant man, he's a determined one. The
ophthalmologist's opinion didn't set well with him. There was too much evidence to the
contrary. Dr. Ranalli (the oncologist) referred Avalon to the neurology department for another
opinion. He even called and begged/bribed/cajoled his way into Avalon being seen within a
few weeks, rather than the typical 4 months. As with Dr. Ranalli, the neurologist, Dr. Zamel,
also felt the evidence pointed toward pseudotumor cerebri. To confirm it, he called in a favor
from a colleage from Ohio State, Dr. Susan Benes, a neuro-ophthalmologist. Dr. Benes is a
world-renowned specialist in pseudotumor - she's basically the best of the best. Dr. Zamel
begged Dr. Benes to see Avalon, explaining why he and Dr. Ranalli were worried - and why
they wanted her to take a look. Thank heavens and all that is good in the universe....Dr.
Benes agreed to take Avalon as a patient.
Dr. Benes discovered that not only did Avalon have pseudo-tumor cerebri (PTC), but she'd
had it long enough to cause a considerable amount of damage to her vision. At the time of
Avalon's first visit with Dr. Benes, Avalon had lost nearly 70% of her peripheral vision! Avalon
had no lower half peripheral vision. She didn't avoid going up or down stairs because she was
"lazy" - the poor child couldn't see them! She didn't lay on the floor to play with her toys
because she was being lazy - she laid down so she could see into her doll house, or see into
whatever she was playing with. She literally only had a very narrow tunnel she could see
through. Yes, the retroactive guilt was suffocating....
We spent the next several months discussing treatment options with Dr. Benes, Dr. Ranalli,
and Dr. Zamel. Most pseudotumor cases in children happen in pre-teens or teens. We
couldn't find any information about 2 year olds with it. The few published papers about PTC in
children were very anecdotal, and usually only covered a handful of children at a particular
institution. By and large, the cases were similar. PTC occurred after or during chemotherapy
- presumably as a response to the stress on the body from the chemo. In nearly all the cases,
once the chemo was fully out of the system, the PTC would resolve. We determined to try to
manage the PTC, trying to buy Avalon some time until she could complete chemo. In the
mean time, the governing body of the Children's Oncology Group held conferences to decide
what to do with Avalon. After much discussion, they decided to discontinue her steroids and
intrathecal methotrexate 6 months early - to prevent further damage to the brain, and to
maybe even reverse what had already happened. At the time, we were terrified. (OK, we still
are...) If research had shown that 26 months of chemotherapy was the ideal time period,
stopping two major elements of it 6 months early seemed insane to us. However, we took the
recommendations of the doctors, knowing that their primary concern was also her health. We
took a leap of faith.
We tried several ways of reducing Avalon's pressure. We tried two separate drugs that can
be quite effective in reducing CSF (cerebral spinal fluid) production. Unfortunately, she
couldn't tolerate either one. Both drugs caused severe tachycardia and GI distress. The one
drug made her so sick, she lost 6 pounds in a week - and she was only 3 years old! It was
obvious that drugs weren't the answer. While we tried to wait out Avalon's chemo time, we did
"serial" LP's, repeated spinal taps where excess fluid was removed - to temporarily lower her
pressure. The problem is, its a short-lived solution. Spinal fluid replenishes within 24-48
hours, so each "draw-down" (as we called them) only gave her brief respite.
Finally, in August of 2006, Avalon's sight reached critical levels. Her Left optic nerve
developed a bubble of fluid that threatened her vision. She went in the next day for an Optic
Nerve Sheath Fenestration, to keep her from going blind. The ONSF was performed by Dr.
Benes, in the Surgery Center of our Children's Hospital. It was part well-known procedure,
part stroke-of-genius by Dr. Benes.
An ONSF is an operation where the surgeon puts purposeful holes in the myelin sheath that
covers the optic nerve. The holes are to allow drainage of Cerebral Spinal Fluid (CSF)
directly behind the retina, reducing the pressure on the critical final 4 mm of the optic nerve.
ONSF operations have been used often in the treatment of adult PTC. They are very effective
in saving vision. However, a lot of specialists argue against their use, because they must be
repeated. Over time, the body will heal the holes, necessitating another operation to re-open
them. Dr. Benes' genius is in a procedure she has pioneered. She treats the fenestrations
(holes) with a small amount of a chemo agent - to retard their self-healing process. Therefore,
she keeps the fenestrations viable - long after most surgeries have had to be repeated.
Avalon's fenestrations are two years old, and flowing beautifully.
While the ONSF did improve Avalon's balance, heart rate, and vision nearly overnight - it
unfortunately, had very little effect on her ICP (intra-cranial pressure). By December of 2006,
all doctors involved agreed - we had no choice but to place a permanent V-P
(Ventricular-Peritoneal) shunt. Avalon needed the VP shunt to remove the excess fluid from
her brain and stop the degradation of her vision, as well as the other host of high pressure
behaviors she suffered from. Surgery was scheduled, and we entered into our next phase of
Avalon's medical life.
Avalon's first VP shunt was placed in January of 2007. I won't go into details of 2007 here - it
would be another 12 pages of info - and my fingers are tired. Besides, if all goes as planned, I
will eventually "back-date" the site - with all the gory details. Suffice it to say, it was nice to no
longer be ritualistically poisoning Avalon in 2007 (chemo ended in Dec 2006) - but her life
really didn't get much easier.
Moving forward to 2008, and Avalon's ICP was once again showing signs of being too high. I'll
leave those details for "back-dating" about the May 28, 2008 shunt surgery. Guess you'll just
have to trust me on this! Anyway, after the May 28th surgery...Avalon really began to fall
apart. She developed panic attacks, language issues (stuttering, ephasia), emotional control
problems, and a host of other symptoms of continuing high intra-cranial pressure.
A follow-up check-up with Dr. Benes showed that Avalon's vision was, once again, under
attack. The peripheral vision loss we had seen in April and May, was still increasing. In fact, it
had increased enough that it looked as if the shunt surgery had brought about no
improvement at all. Not only was Avalon's peripheral vision continuing to diminish, but her
"blind spot" (you know, the one we all have...) had again increased in size - by a rather large
percentage. Avalon was in trouble.
Dr. Benes recommended an immediate LP to check Avalon's ICP. Avalon's new
neurosurgeon, Dr. Kosnik, is not a huge proponent of checking ICP - he's more of a "watch
how they act" kind of guy. Unfortunately, he's learning that approach just doesn't work with
Avalon - she's too good at masking problems. (reference the earlier discussion of living with
pain for so many years) Dr. Benes didn't even bother asking him, she just called Heme-Onc
and arranged an LP for as fast as they could schedule it. The signs were all there for high
pressure.
Sure enough, the July 9, 2008 LP showed a pressure of 28. For someone with a shunt, that's
insane. We were scheduled in to see Dr. Kosnik within a few days after that, so we could
discuss options. Considering how much Avalon's eyes had progressed, and how awful her
behavior had been since the shunt revision, I whole-heartedly believed that the shunt would
have to be revised or entirely replaced by Dr. Kosnik. Oh my...did he have other plans.
Avalon was finally feeling better after a horrible weekend of painful headaches. When we saw
Dr. Kosnik, we explained everything that had happened since the LP on last Wednesday, and
how she was doing that morning. Although, it was silly to explain it. He'd been in contact with
his nurse and nurse practitioners - he knew everything that was happening as it happened.
Which is why the following took place the way it did.
After my timeline of her pain after the LP, Dr. Kosnik agreed with myself and the NP's, that
Avalon had most likely experienced a spinal leak from the LP. Dr. Ranalli (who did the LP) had
said it was an unusual stick, plus he had drawn off 12 cc's of CSF to temporarily reduce her
pressure. We had also gone up to the neuro-surgery unit and had the NPs turn Avalon's
shunt down to the final setting. Adding all of those fluid-reducing things together...and Avalon
had experienced a low-pressure nightmare. The interesting thing is, by the time we saw Dr.
Kosnik on the Tuesday following the LP, Avalon had already progressed from low pressure, to
a "honeymoon" period of good pressure, and back to high pressure. This poor kid just can't
catch a break.
So, Dr. K's news went something like this. He said obviously, Avalon doesn't handle low
pressure well, she's sensitive to it. He asked me why Dr. Elton had originally used a VP shunt
with her, then he said he agreed with the logic I reported to him. He said he did feel Avalon
had experienced a spinal leak, and that we would do an MRI tomorrow morning, before
surgery - to make absolutely certain the spine had healed completely. Then he said, he did
not believe that an LP shunt would be good for Avalon, they can easily over-drain. An LP
shunt could leave her with the possibility of the same bone-crushing headaches she had just
lived through...nearly every day of her life. We agreed, that would be awful. His answer?
Temporal decompression.
Dr. K introduced temporal decompression as a very old operation, one that isn't used very
often now, because of the popularity of VP shunts. However, he said that temporal
decompression can be helpful for kids with slit ventricle syndrome (which Avalon has), and
also kids who have odd pressures and extreme pressure sensitivities like Avalon. He
explained that he would be removing a piece of Avalon's skull on each side of her head, close
to her temples, to allow the excess fluid somewhere to flow, to reduce pressure.
All I could think to ask was about Anam - who loves to whack Avalon in the head. Dr. K
assured me that he had never had someone who sustained a serious brain injury from being
hit like that, and that, in fact, he'd never even read about it. Where they remove the piece of
skull, the brain is rather "deep" in the skull. Also, they remove it from behind the strongest
part of the jaw muscle. The jaw muscle then acts as the protective barrier, in place of the
skull. The difference is the muscle has a little more "give" allowing the fluid to press out on it.
Dr. K said we would be able to crudely check Avalon's pressure, by how "full" her holes feel. If
they feel tight and full - her pressure is up. If they're nice and squishy, she's doing well.
Basically, its like adding a baby's fontanel on each side of her head.
The only other thing I could think to ask about was why not check the shunt? I said, "I don't
think its working". His answer? I can't check it. He said there had been too much in and out in
a short time, he was afraid the skin couldn't handle being opened. He was afraid he wouldn't
be able to close it. What a horrifying thought... And yes, it makes my blood boil that the other
surgeon left her in such a state. But that's for other discussions....
About this time, Dr. K moved on to his next patient. I had a few minutes to process my
thoughts. I found myself in awe of the moment. I had sat there, listening as a man proposed
drilling large, permanent - holes in my daughter's head, and easily agreed with him. There
was no tight chest, no knot in my stomach. I accepted it all, as if someone asked to do a panel
of bloodwork. Not to mention, Dr. K had requested to see if Avalon could do the pre-op MRI
with NO sedation. He said he thought she would need to do a lot of MRI's over her lifetime,
and if she could learn to tolerate them, it would be easier on her in the long run. Again, I
agreed. I couldn't believe myself. I decided it was a sign, I not only trust Dr. Kosnik - I have
good reason to. I've learned a lot about listening to my "guts" in the past few months. In this
case, my gut instinct was actually OK. Who could have imagined?
In a few minutes, Dr. K's nurse, Margaret, came in. She came with pre-op consent forms, and
to discuss blood work, the MRI, etc. She asked me if I had any questions about the
operation.... Um....yes! Only about 1000 of them....but I was honestly too dumbstruck to come
up with a single one. I told her I was so flabbergasted I couldn't think of a thing...but I'd get
back to her, if I ever managed to concentrate long enough. She was reassuring and as warm
as always, and reminded me that we would see him before the operation tomorrow - if we
wanted to ask anything. Sure, sure...that sounded great. My mind had switched over to
slightly-freaked-out-oatmeal. I was good for nothing but set decoration...
Margaret and I then covered the pre-op blood work we'd need. We decided it would be much
easier if we just popped down to Heme-Onc clinic and had them do the stick. After all, we
know them and Avalon loves the PCA's (nurses aides) that do the labs. Besides, Heme-Onc is
always more than happy to do labs for any cancer patient. They always like to see the results
too!
So, a few shocking discussions later, we were off to Heme-Onc clinic. Margaret sent a lab
order with me, but it was blank. She cracks me up! She sent it with the instructions, "They
know way more than I do. Just tell them what she's having done - they'll be better suited to
figure it out." What a hoot! Sure enough, it took a hot second for someone to order the
appropriate labs. It only took a few minutes for them to be drawn. Meanwhile, there was the
chorus of "What?!" from everyone who heard why we were there. Avalon's nurse practitioner,
Diane, amused the britches off of me. (OK, its a figure of speech...no one wants to see me
with my pants off!)
Diane plopped down next to me in the waiting room and asked me what was up. Of course
she already knew about the LP, it was Avalon's Heme-Onc attending, Dr. Ranalli, who had
drawn it. So, Diane just wanted to know what Dr. K wanted to do. I'm here to report, she was
just as "icked out" as I was about the prospect of cutting out hunks of Avalon's skull.
Yep-a-rooney, the great and glorious Diane even gave a oooooh-gross shudder when I
explained that we could eventually check her pressure by pushing on the new squishy parts.
Ha ha! I managed to gross out the unflappable Diane. There ought to be a prize for that.
Actually, I was rather ornery. I told her I'd be hog-tying her and forcing her to touch the
squishies. After all - it was her chemo that caused the problem. Therefore, if she could drag
me into this mess - I was "taking her with me!" down the bleck path. You have to love a nurse
who not only admits to being "icked out" - but who loves you enough to joke with you about it.
She was the perfect prescription for me at that exact moment....
The PCA's were hysterical in their own way. Our dear friend, Ryan, was his typical goofy self.
No grossing out for him, he said he couldn't wait to feel it! Ewwwww....... Of course, they also
listened to my rather dis-combobulated ramblings..... Again, the perfect prescription for that
exact moment. I honestly think I repeated myself 20 or 30 times. I just couldn't wrap my pea
brain around what we were preparing for. Thankfully, the awesome Heme-Onc crew once
again stepped up to the plate. They met my worry and fear with the perfect combination of
humor, concern, love, and support. I left the clinic convinced I'd made a fool of myself, but
knowing it was OK if I did. Avalon left feeling loved, several dozen hugs will do that for you.
Both of us left better than we came in - you can't ask for more.
From clinic, we headed home and began the insane round of phone calls and preparations for
the coming week. "Hello, your daughter is having radical brain surgery tomorrow," will set a
family's collective rear end on fire like you can't imagine! Below the pink line is an email I sent
out to family and friends the night before the first temporal decompression. I posted a nearly
identical email to my online support group, ALL-Kids. My support group friends have become
a second family to me. Some day, I'll write an obscenely long entry about how much I adore
them.
The following email is included for one reason - honesty. I think its important that anyone
reading this site, gets a real sense of our perceptions of the events of Avalon's life. We can
only teach, if we are honest in sharing all of our feelings - the good, the bad, and the
probably-shouldn't-be-mentioned-in-public. I believe in the goodness in the heart of mankind,
and hope that somehow, Avalon's journey is meant to touch the hearts of the people around
us. At the very least, that's what I hold onto on the difficult days...so let me live in my
delusional bubble please.....
Sent: 7-15-08 (to family and friends)
Subject: Avalon in OR tomorrow
Because I have 1001 things to accomplish in the next few hours, I'm going to do my best to
make this as concise as possible. Yeah, heah...I know I'm rather rotten at that.
Yes, the subject line was correct - Avalon is headed back to surtgery tomorrow, July 16, a
mere 6 weeks after her last brain surgery. Here is how the past several weeks have played
out:
May 28 - botched, less-than-medically-ideal VP shunt surgery
- followed by numerous visits with Avalon's NEW neurosurgeon - to discuss her odd
behaviors
- Mom continuously said, "Avalon's pressure is still high!"
June 30 - neuro-ophthalmologist appt - shows large vision loss - indicators of high intracranial
pressure
July 9th - LP (lumbar puncture) - confirms high ICP - 28 (normal 10-12)
July 10th - Avalon in excrutiating pain - horrible headache - would have taken her to hospital,
but couldn't - Voldemort was on duty
July 11th - Avalon woke OK, by 3 pm - headache returned with a vengence
July 12th - headache/pain for 24 hours straight
July 13th - woke OK, headache/pain returned by 5 pm
July 14th - Yay! no headache
We saw Dr. Kosnik today. He agrees with our feelings that the weekend torment was from low
pressure from a possible leak with the LP. He's very concerned about the high pressure and
vision loss. However, he does not feel safe going back in to check the "new" shunt. He
doesn't like the look of her skin. He's afraid if he goes in, he won't be able to close her again.
(in last surgery she was closed, then re-opened, then closed again) Dr. Kosnik doesn't like
the idea of an LP shunt - fearing she would overdrain and live in constant pain from low
pressure.
The solution is icky. Dr. Kosnik will be permanently removing two "sizable" (his quote) sections
of her skull - near her temples. These "holes" will allow excess spinal fluid an escape route -
to keep from pressing on her brain. We'll be able to monitor her pressure (at least crudely) by
"touch". If her pressure is high - the holes will bulge slightly and be hard. If the pressure is
low -they will be squishy, like a baby's fontanel. This is a very old method of controlling
intracranial pressure. Sadly, it appears to be our best bet at the moment. We certainly can't
risk him opening her VP shunt - and not having the skin to be able to close again. And I really
don't ever want to see her in low pressure agony again - much less on a daily basis.
OK, did I sound calm in that last paragraph? Make no mistake....I'M NOT!!!! Truth be told -
I'm completely flipping out. C'mon....he's taking two hunks out of her skull...PERMANENTLY!!
I'm not quite sure if I want to get miserably, regretably drunk, or sit down and cry myself into a
migraine stupor. For the most part...I'm just totally freaked out. However, I want to be very
clear here. I DO NOT have the tight chest, flipping stomach, and screaming anxiety that I did
before the last operation. I'm calm...just really grossing out, and angry that its come to this. (it
didn't have to, thank you - Voldemort) I do trust Dr. Kosnik, and know that he's put a great
deal of thought into this decision. I know Avalon is in excellent hands - but I don't have to be
tickled pink over what she faces.
As for the next few weeks. Here's the scoop. We report to Nationwide Children's at 5:45 am
tomorrow. We first go to the surgical unit for check-in. Then, Avalon is going to MRI to scan
her spine - and make sure there are no residual leaks. (she already did pre-admit bloodwork
and pre-surgery X-rays today) Because the MRI is short - we're going to try to help her do it
with no anesthesia. Dr. Kosnik said it would be great if she could learn that - as he thinks she
may have several more.
After MRI - we go back to the surgery unit and wait. Avalon will be second on Kosnik's
schedule tomorrow. The nurse anticipates the procedure being at least 2.5 hours - maybe
longer. Tomorrow, Dr. Kosnik will only remove the piece from her right side. We'll wait a week
- then repeat all of this to remove the piece from the left side. Typically, patients are only in
for 2-3 days after the procedure, then they come back in for round two. Avalon's GI issues
(low motility) may keep her in-house longer. We won't/can't bring her home until her GI system
has fully awakened. Low motility kids don't do well being under general anesthesia for several
hours. It kind of shuts down the whole monkey-works. As soon as I hit "send", I'm calling her
GI doctor to give her the heads up about the two surgeries. Basically, only time and her gut
will tell when we actually get sprung.
In addition, all of this may simply be another bump in the road. There is no guarantee that this
will "fix" her problems. We may have to revisit the VP shunt later - or explore options we've
discussed previously. Its all a bit mind-numbing. (no pun intended)
Is she scared? Not really - more sad. Here goes even more hair. She, of course, is also
being typical Avalon. She insisted on stopping at the little gift shop on the way out so she
could scope out which Webkinz to try to hit me up for. Gotta love that kid...always working the
angles.
We are headed out the door in a few minutes to pick up some groceries, costumes for a
charity event, and hair dye. Oh yeah, we're going down the chemical path again. We're fed
up with those ridiculous colors though! They wash out too quickly!! This time, we're going
Pippi Longstocking Pink. Or, in Columbus, OH - they might call it "Wendy-red". Yep - I'm
going to make it look as if my red hair is actually natural (when it sooooooo isn't) and I've
passed it on to my short people. Goodbye dull, hello fire-engine red! Ha! You have to admit
- it will make for a great mowhawk on the little princess. I mean, after all, its only hair. We, of
all people, truly understand that.
So, my friends, please cast a loving, positive thought Avalon's way this week. I think cancer,
osteoporosis, nerve damage, vision and hearing loss, brain damage, and 4 brain surgeries in
5 short years of life is more than enough. Calgon....take us away.......................
I'll update as soon as I can.
LOVE TO ALL OF YOU - you help us just by being willing to read this.
- Alicia, Nick, Aurora, Ambrosia, Anam, and the brave, beautiful, funny, greedy-but-cute,
AVALON
Subject: Avalon in OR tomorrow
Because I have 1001 things to accomplish in the next few hours, I'm going to do my best to
make this as concise as possible. Yeah, heah...I know I'm rather rotten at that.
Yes, the subject line was correct - Avalon is headed back to surtgery tomorrow, July 16, a
mere 6 weeks after her last brain surgery. Here is how the past several weeks have played
out:
May 28 - botched, less-than-medically-ideal VP shunt surgery
- followed by numerous visits with Avalon's NEW neurosurgeon - to discuss her odd
behaviors
- Mom continuously said, "Avalon's pressure is still high!"
June 30 - neuro-ophthalmologist appt - shows large vision loss - indicators of high intracranial
pressure
July 9th - LP (lumbar puncture) - confirms high ICP - 28 (normal 10-12)
July 10th - Avalon in excrutiating pain - horrible headache - would have taken her to hospital,
but couldn't - Voldemort was on duty
July 11th - Avalon woke OK, by 3 pm - headache returned with a vengence
July 12th - headache/pain for 24 hours straight
July 13th - woke OK, headache/pain returned by 5 pm
July 14th - Yay! no headache
We saw Dr. Kosnik today. He agrees with our feelings that the weekend torment was from low
pressure from a possible leak with the LP. He's very concerned about the high pressure and
vision loss. However, he does not feel safe going back in to check the "new" shunt. He
doesn't like the look of her skin. He's afraid if he goes in, he won't be able to close her again.
(in last surgery she was closed, then re-opened, then closed again) Dr. Kosnik doesn't like
the idea of an LP shunt - fearing she would overdrain and live in constant pain from low
pressure.
The solution is icky. Dr. Kosnik will be permanently removing two "sizable" (his quote) sections
of her skull - near her temples. These "holes" will allow excess spinal fluid an escape route -
to keep from pressing on her brain. We'll be able to monitor her pressure (at least crudely) by
"touch". If her pressure is high - the holes will bulge slightly and be hard. If the pressure is
low -they will be squishy, like a baby's fontanel. This is a very old method of controlling
intracranial pressure. Sadly, it appears to be our best bet at the moment. We certainly can't
risk him opening her VP shunt - and not having the skin to be able to close again. And I really
don't ever want to see her in low pressure agony again - much less on a daily basis.
OK, did I sound calm in that last paragraph? Make no mistake....I'M NOT!!!! Truth be told -
I'm completely flipping out. C'mon....he's taking two hunks out of her skull...PERMANENTLY!!
I'm not quite sure if I want to get miserably, regretably drunk, or sit down and cry myself into a
migraine stupor. For the most part...I'm just totally freaked out. However, I want to be very
clear here. I DO NOT have the tight chest, flipping stomach, and screaming anxiety that I did
before the last operation. I'm calm...just really grossing out, and angry that its come to this. (it
didn't have to, thank you - Voldemort) I do trust Dr. Kosnik, and know that he's put a great
deal of thought into this decision. I know Avalon is in excellent hands - but I don't have to be
tickled pink over what she faces.
As for the next few weeks. Here's the scoop. We report to Nationwide Children's at 5:45 am
tomorrow. We first go to the surgical unit for check-in. Then, Avalon is going to MRI to scan
her spine - and make sure there are no residual leaks. (she already did pre-admit bloodwork
and pre-surgery X-rays today) Because the MRI is short - we're going to try to help her do it
with no anesthesia. Dr. Kosnik said it would be great if she could learn that - as he thinks she
may have several more.
After MRI - we go back to the surgery unit and wait. Avalon will be second on Kosnik's
schedule tomorrow. The nurse anticipates the procedure being at least 2.5 hours - maybe
longer. Tomorrow, Dr. Kosnik will only remove the piece from her right side. We'll wait a week
- then repeat all of this to remove the piece from the left side. Typically, patients are only in
for 2-3 days after the procedure, then they come back in for round two. Avalon's GI issues
(low motility) may keep her in-house longer. We won't/can't bring her home until her GI system
has fully awakened. Low motility kids don't do well being under general anesthesia for several
hours. It kind of shuts down the whole monkey-works. As soon as I hit "send", I'm calling her
GI doctor to give her the heads up about the two surgeries. Basically, only time and her gut
will tell when we actually get sprung.
In addition, all of this may simply be another bump in the road. There is no guarantee that this
will "fix" her problems. We may have to revisit the VP shunt later - or explore options we've
discussed previously. Its all a bit mind-numbing. (no pun intended)
Is she scared? Not really - more sad. Here goes even more hair. She, of course, is also
being typical Avalon. She insisted on stopping at the little gift shop on the way out so she
could scope out which Webkinz to try to hit me up for. Gotta love that kid...always working the
angles.
We are headed out the door in a few minutes to pick up some groceries, costumes for a
charity event, and hair dye. Oh yeah, we're going down the chemical path again. We're fed
up with those ridiculous colors though! They wash out too quickly!! This time, we're going
Pippi Longstocking Pink. Or, in Columbus, OH - they might call it "Wendy-red". Yep - I'm
going to make it look as if my red hair is actually natural (when it sooooooo isn't) and I've
passed it on to my short people. Goodbye dull, hello fire-engine red! Ha! You have to admit
- it will make for a great mowhawk on the little princess. I mean, after all, its only hair. We, of
all people, truly understand that.
So, my friends, please cast a loving, positive thought Avalon's way this week. I think cancer,
osteoporosis, nerve damage, vision and hearing loss, brain damage, and 4 brain surgeries in
5 short years of life is more than enough. Calgon....take us away.......................
I'll update as soon as I can.
LOVE TO ALL OF YOU - you help us just by being willing to read this.
- Alicia, Nick, Aurora, Ambrosia, Anam, and the brave, beautiful, funny, greedy-but-cute,
AVALON