Kate’s CaringBridge Introduction Post

Saturday, December 8, 2007 9:05 PM, PST

Here I am, writing my very first EVER blog post. I can’t believe I’m doing this, but heck, everyone and their dog has a blog nowadays and I don’t want to be behind the times, so I figured it was time to bite the bullet and just do it. Lol I suppose I should write a little “Introduction to My Kids” post for anyone who might come across this blog and decide to take the time toread it.

Megan is my oldest. She’s 16 and NT (neurotypical). Okay, if you want to get technical, she does have Tourette’s, but around our house, having *just* minor tics means you’re fine. Really. I know that some people view Tourette’s as a disability, but Megan and her father and I see the condition as a nuisance and nothing more. Megs is beautiful, intelligent, mature way beyond her years and a huge help around the house. She is a lovely young woman with a spiritually-sensitive heart and I sometimes step back and wonder, “HOW has she become this person? I wish I were more like her!” So many people gripe about their teenagers and I, thankfully, can not relate to their angst. Megan isn’t perfect (who is?), but she’s a pretty awesome 16 year old. She enjoys reading, horses, reading, dogs, reading, dragons, reading, reading and reading.

Adam is 15. He has multiple diagnoses, but the most-challenging ones are Asperger’s & Auditory Processing Disorder. Adam is very smart, VERY mechanically-savvy and he has a knack for making me laugh uproariously with his preference for physical comedy. He has a special bond with his baby brother, Joshua, and goes out of his way to help me with Joshua’s care every day. We recently had a doctor inquire whether Adam might have Marfan’s Syndrome, so Adam is getting evaluated for it, but I don’t think he actually has it. I think Adam is just a super-tall 15 year old (he’s a bit over 6’6″) with really big feet (size 18). Considering that my brothers are both over 6’7″, it isn’t a surprise that Adam is taller than average, too. Adam’s interests revolve around medieval things, computers and Legos.

Emily is 8. She has Asperger’s, Arnold Chiari Malformation type 1 (ACM) and Tethered Cord Syndrome (TCS). Chiari is a condition where the lower portion of the brain (the cerebellar tonsils, which is the bottom part of the cerebellum) doesn’t have enough room and gets pushed out of the skull into the spinal canal, thus compressing the brainstem and blocking the flow of cerebrospinal fluid (CSF). This causes multiple neurological symptoms and can lead to such complications as syringomyelia (CSF being forced into the spinal cord), hydrocephalus, brain damage and unrelenting pain. Tethered Cord is a condition where the spinal cord is either adhered to bone/muscle/scar tissue OR where the lowest part of the spinal cord is stiff rather than being flexible like a rubber band, so it’s unable to stretch and move freely as it should. When the cord is tethered (stuck), then normal movement causes unnatural stretching of the spinal cord, which can lead to permanent nerve damage (as well as pain, mobility difficulties, numbness and other neurological symptoms). Both Chiari and Tethered Cord are treated with surgery. A Chiari decompression involves making an incision in the back of the head, along the base of the skull & neck, to allow a neurosurgeon to remove some occipital bone and the backsides of 1 or 2 cervical vertebra, then open the dura (the casing that holds the spinal cord/brain/CSF) and add a patch to enlarge the area where the cerebellar tonsils are at so that CSF can flow freely again and the pressure can be taken off the brainstem. A Tethered Cord release involves making an incision on the lower back and releasing the cord from whatever it is stuck to. This procedure is pretty straightforward and easy for a simple tight filum (what Emily has), but it is much more difficult and involved for a person with Spina Bifida since the amount of scar tissue that typically tethers the cord is enormous. Emily hasn’t had any surgery yet because her Chiari is asymptomatic and she isn’t having enough Tethered Cord symptoms to make surgery worth the risk. However, we know that a TC release is in her future at some point because eventually she will grow enough to stretch her cord to the point where her symptoms require treatment. Emily is blonde, with big green eyes and a beautiful smile. She loves to read and do math, but her very favorite activity is anything involving crafts. I am so NOT a crafty person, but my Emmy is. She loves to create masterpieces from any material she can find and her room always has broken crayons and uncapped pens lying around (sigh).

David is 6. He was my first diagnosed special-needs child. October 25, 2002 introduced me to the world of autism and the phrase “special needs”. In addition to Autism, David also has Chiari and Tethered Cord. He had his Chiari decompressed on February 21, 2005 and his cord was surgically released on August 12, 2005. As long as he remains asymptomatic, he won’t require any more surgeries. David loves to play Gameboy games and watch movies. He likes “mutated lizards” (ie: Godzilla), Spiderman and SpongeBob.

Sarah is also 6. She has Chiari and Tethered Cord. She had her Chiari decompressed twice, on July 7, 2004 and again on February 21, 2005. Her cord was surgically released on February 21, 2005. Explaining Sarah’s experiences with Chiari would take a separate post, which I probably will write in the near future. Suffice to say that she’s currently asymptomatic but has some permanent side-effects from her condition that affect her learning abilities, but not her overall intelligence. Sarah is a talented artist, a mathematical whiz and one of the most-loving children I have ever known. She’s the kiddo who is always running over to give me hugs and kisses throughout the day.

Isaac is 4. He received a PDD-NOS diagnosis at 15.5 months of age, but I vacillate back and forth whether I think he’s actually on the spectrum or not. It can go either way. He *is* very rigid and he still has a severe articulation disorder, but he’s also very imaginative and social in ways that David and Emily and Adam just aren’t. Isaac likes guns (no, we don’t own any, but that hasn’t stopped him from being obsessed with ’em) and playing computer games and riding his scooter.

Joshua is 21 months old. He has a form of Spina Bifida called Lipomyelomeningocele (LMC), which is basically myelomeningocele (the Spina Bifida that most people are familiar with ~ where the baby is born with the spinal cord outside of the body) that is covered by skin and also has a benign tumor made of fat, muscle & nerves growing in and around the spinal cord. Joshua also has Chiari type 1 and is fed primarily through a gastrostomy tube (gtube). He has severe nausea that causes multiple bouts of vomiting each day as a ‘side effect’ of his second Chiari surgery. We give him Zofran around the clock to combat the nausea and he receives just 5 ounces of Nutren, Jr. formula per tube feed since larger amounts triggers more vomiting. Since he has to take in 30 ounces per day of formula, it can be a struggle to get him fed often enough to ensure good growth, especially on days when he is throwing up more than normal. He has a neurogenic bowel and bladder, which is a fancy way of saying that he needs help to pee and poop because there is nerve damage to those body parts ~ he gets cathed 4 times a day and takes Miralax to stay regular. Oh, and he’s allergic to latex, too, so  have to stayalert to what he’s around and/or touching when we’re not at home (the house is pretty much latex-free). In his first year of life, Joshua had just 1 surgery ~ his initial repair done at birth where the neurosurgeon removed as much of the tumor as she could, put his spinal cord back into the spinal canal where it belonged, closed the dura (with an artificial graft) and the skin. However, this past year has been one hospitalization after another. Joshua had 2 Chiari decompressions (April 20th & October 1st), 1 tethered cord release (April 20th), a gtube placed (August 13th), an emergency laparoscopy to retrieve a piece of the gtube that got lost in his abdominal cavity during a “routine” procedure to change the PEG tube for a Mickey button (both on September 25th) and then another GI procedure to change over the button once more (it was done in the OR just in case the same complication occurred again ~ on December 4th). He’s also had multiple MRIs, pH probes, endoscopies, CT scans, xrays, ultrasounds of various body parts, VCUG/urodynamics, and more doctor appointments than I can keep count of (over 100, easily). Joshua did have good speech for his age until he was 10 months old, at which time his Chiari became symptomatic and he lost what words he had. Unfortunately, he hasn’t regained much speech and relies mostly on pointing, grunting/squealing and a few words or approximations of words to communicate. Joshua generally is a good natured little boy (as long as the world is going his way) and despite his challenges, he brings our family a great deal of happiness and laughter. So… that’s the rundown of my kids and their respective “disabilities”. Truth be told, the only kids I think of as having true special needs are David and Joshua. Asperger’s and PDD-NOS are definitely a challenge and life can be really difficult with Adam, Emily & Isaac at times, but overall, they are all so high-functioning that anyone who casually meets them probably wouldn’t notice anything odd at all. Or maybe it’s more accurate to say that because they have siblings with problems greater than their own, high-functioning autism spectrum disorders don’t rank as high on the “disability” scale around here.



Tuesday, February 24, 2009 10:18 PM, PST

An online friend emailed to suggest that I look into the possibility that Joshua may have a condition called pseudotumor cerebri (PTC), which occurs when there is

increased pressure within the skull caused by a lack of proper absorption of cerebrospinal fluid (CSF). In reading about PTC as it occurs in conjuction with chiari

malformation, I learned that 20% of chiari decompressions fail (ie: symptoms return post-operatively). Of those failures, 42% are eventually diagnosed with

pseudotumor cerebri. In studies that were researching the connection between PTC and chiari, I found that the majority of children who were treated by having a

shunt implanted in the ventricle of their brain had “significant resolution” of their symptoms. This gives me some hope!

The symptoms of pseudotumor cerebri in a post-decompression chiari patient are as follows:

1. Chiari-like symptoms (headaches, nausea, tingling in upper extremities, dizziness, ataxia, etc ~ you know, Joshua’s usual constellation of symptoms which he

currently is experiencing).

2. Brain MRI is normal. There has to be normal flow of CSF around the base of the brain and at the skull-neck junction & normal ventricles. (Joshua has this)

3. Elevated CSF pressure as measured by a lumbar puncture (don’t know yet if he has this)

4. Symptoms were temporarily relieved after draining CSF & reducing the CSF pressure (I wonder if perhaps part of the reason Joshua improves for a short time

after each surgery is that he loses CSF when Dr. W opens the dura ~ thus lowering pressure ~ and then, within a month to 6 weeks, the pressure has increased so that

he again becomes symptomatic).

Perhaps he has PTC in conjunction with chiari and it is THAT which contributes to his “never staying fixed”. If he does have problems properly absorbing CSF, that

could explain why his head grew so quickly as an infant when his skull sutures were not yet fused but he never had hydrocephalus ~ the excess fluid & pressure are on

the outside of the brain, not within the ventricles, so a CT scan shows no ventriculomegaly. This link http://webeye.ophth.uiowa.edu/ips/IIH/PSEUDOTU.HTM

discusses PTC and how the headache associated with pseudotumor cerebri is extremely painful, often wakes a person from sleep & usually lasts for hours. This is

EXACTLY what happens with Joshua ~ he wakes with his head hurting on a daily/nightly basis! The article states nausea is common & vomiting less so ~ when Joshua

has headaches, he is very gaggy & retches, so we give him Zofran and that helps (would suggest nausea to me). It says there can be episodes of transient blurred vision

that may involve one or both eyes ~ Joshua frequently rubs his left eye & blinks rapidly & repeatedly for 30 seconds to a full minute. Perhaps he is having blurred

vision? The ophthalmologist did say that Joshua is favoring his right eye over his left, so maybe that’s the reason.

My plan is to contact Dr. W about this. I will ask her if she would please do a lumbar puncture when she is already in the operating room with Joshua to drain his

pseudomeningocele. If he has high CSF pressure, thus confirming the suspicion of PTC, she could place a VP shunt while he’s in the OR and we could go on with life &

hopefully Joshua would feel good more than 50% of the time. If his pressure was normal, then we would conclusively know that he does not have PTC & we’d be back

right where we are now in terms of not knowing what is causing Joshua’s symptoms, but at least I would know that we ruled out the treatable conditions before

jumping into the realm of degenerative disorders. I feel like I have to push for that, even if it turns out I’m wrong.

If you’re praying, please ask God to move on Dr. W’s heart so that she would want to check the level of Joshua’s CSF pressure. Pray that she would read my note to

her & that it would make her want to investigate the possibility of Joshua having PTC more closely.


Tuesday, March 3, 2009 10:20 PM, PST

Joshua’s next surgery is scheduled for April 6th. In addition to the pseudomeningocele repair, Dr. W will be doing the lumbar puncture I requested. The LP will assess

whether or not Joshua has high CSF pressure. If he does, he’ll receive a diagnosis of pseudotumor cerebri & a ventriculoperitoneal shunt for treatment. However, since he

may not have PTC, we’re moving forward with getting an appointment set up with the neurologist. I am hoping an appointment can be made for Friday, April 3rd so that I

can combine the two events in one trip north. It will be a “God thing” if the neurologist schedules Joshua for an appointment on the exact date that I want, but it sure would

be convenient, so it’s what I am praying for.


Tuesday, March 24, 2009 10:23 PM, PDT

Joshua is not wanting to walk much anymore. When we try to get him to walk to the car from the house, for example, he

cries & says he can’t; he doesn’t know how to. He wants to use his wheels or be carried for even short distances now.

Tonight, he was crawling through the house rather than walking. The skin around the scar on his back is caving in again, too.

Last week, it was perfectly flat, but now he’s got an obvious depression all along his lower spine. When this happened last

fall, I was told it was scar tissue attached to his skin that was pulling inward. I was surprised to see it again tonight.

He complains of back pain throughout the day. He’s lost sensation further up his legs ~ he fell & cut his right thigh and didn’t

notice it, and today he skinned the front of his shin & never reacted. It’s hard to see increased deficits and continued loss of

function. I am praying he’ll get it back post-operatively.

The interesting thing is that yesterday I got a copy of the MRIs from February 20th. According to the radiologist, Joshua’s

brain MRI showed diminished CSF flow through the foramen magnum (base of the skull opening into the spinal canal), which

would explain the chiari symptoms he’s having. Dr. W said she thought Joshua had adequate flow, so I’m not sure who to

believe. I mean, if Joshua were asymptomatic for chiari right now, I’d think the radiologist was wrong. But since he’s having

definite chiari-type symptoms, it’s easier to think that this time, Dr. W is wrong, especially since that would mean we could

treat the problem & potentially alleviate Joshua’s symptoms again. Of course, it doesn’t matter what I think because I can’t

force a neurosurgeon to change her mind (though I did put a call in to her P.A. yesterday, asking about the discrepency &

whether Dr. W had read the radiologist’s report, and if she had, did that change her opinion at all? Unfortunately, I haven’t

heard back from anyone yet).

The lumbar spine MRI showed the collection of CSF outside the spinal canal and under the skin, of course (the

pseudomeningocele that will be drained in April), but also that the bottom of Joshua’s cord is pulled down to L4-5 (it’s

supposed to be up at L1) and that there is no motion in the cord at all. So once again he’s tethered, which is to be expected

in a kid with spina bifida ~ the joke I’ve heard multiple times is that SB kids retether before they come off the operating

table ~ but to have zero movement in the spinal cord indicates severe tethering that is linked to symptoms like back pain,

loss of sensation in the legs & increased falling ~ you know, stuff Joshua currently is experiencing. I find it insanely difficult

to wrap my mind around the thought that he would be able to retether so much in just 3.5 months after surgery (as of the

day he had the MRIs done, February 20th). I know Dr. W will remove scar tissue outside the dura when she operates in April,

but I’m betting she is not going to remove the dural patch and attempt to remove the scar tissue inside the dura/around the

nerves of the cord because it’s so soon after his last detethering surgery & every time she goes in there, the risk increases

for more permanent damage. Besides, she’s going to be busy trying to patch the hole that is causing the CSF leak & doing

the lumbar puncture to assess Joshua’s CSF pressure (and putting a shunt in his brain if it’s high). I’m pretty sure she’ll tell

me we need to come up with a plan for pain management and put off doing yet another detethering until something serious

forces the issue, instead. I’m fine with not doing more surgery ~ it’s not like I enjoy putting my 3 year old through the

process repeatedly ~ but I want him to not hurt. Physical deficits are not a big deal (to me ~ it’s all about perspective

nowadays), but daily pain that gets worse over time? That has to go. There must be something we can do to give Joshua

relief for longer than 4-8 weeks. I just pray we stumble upon the answer soon because this cycle of him getting just 1-2

months of being “good” before becoming symptomatic again is very draining/stressful, not only on him & me, but all of the

other kids & Charley, too.

Through all of this, God has been quite gracious to give me daily reminders that He is present and that I’m not walking this

road alone. I’m grateful to have Him & my treasured friends who lift my spirits, listen & offer advice/support/reasons to

laugh and are always there for me, be it via a phone call, an email, a card in my mailbox or getting together for lunch or a

“girl’s night out”. I am so blessed to be surrounded by friends and to never have to feel alone or defeated by the challenges

that I face with my kids’ many medical issues. Thanks to all of you who read this site & care about my son. Your prayers and

well-wishes in the guest book mean so much to me!

P.S. For those who want to know about the other kids ~ Adam’s blood pressure is still hovering in the high 140’s/low 150’s

over 90’s. His echo & bloodwork came back normal, but he’s still dumping protein in his urine, which would indicate that

regardless of the normal kidney function tests, something is not working properly there. He is scheduled to see his

cardiologist in April after we get home from Portland. Please pray for this to get figured out.

Isaac had his echo today & I am still awaiting the results. I asked the tech if she could tell where his murmur was originating

from & she said she could, but she couldn’t tell me anything more (liability and all that). I’m assuming the results were okay

since no one called today with bad news. He has his audiology test in April after I get back from Portland & he’ll have his

speech evaluation soon thereafter, as well. No answers as of yet, but the ball is rolling in the right direction, so that’s


Packing is going well. We’ve moved over all of the garden ties, decorative bricks, dirt that we bought to put in the raised

flower beds, roses & lilac bushes. We’ll pick up the U-Haul on the 1st, load it, drive to the new house, unload everything and

start getting things set up. Charley has to work on the 2nd, but he has the 3rd & 4th off, so we’ll have a couple of days to

get things all situated before I leave to take Joshua up north. I’ll be offline for a few days, but I’m hoping our internet

provider can get us reconnected in the new house on the 2nd or 3rd. Say a prayer that our internet blackout isn’t for long

(‘cuz our internet people also supply our phone & I want that hooked up quickly, too)!

I have to admit, even though I’m fairly accustomed to hectic stuff happening in my life, I’ll be happier when Joshua’s surgery

is over & we’re moved & settled & life goes back to what is normal for us.


Thursday, July 2, 2009 3:56 PM, PDT

Hello from home! We had a safe drive home & were hit by 102 degree temps. Yuck! Portland was a good 20 degrees cooler, which was NICE!

Thanks for your prayers regarding Dr. Wehby ~ they worked! She was the friendly & kind doctor that I’ve known her to be in the past (before she got so utterly

frustrated by my kid). When I brought up the possibility of pseudotumor cerebri (PTC) again, instead of getting irritated with me (which is what has happened the

past two times I mentioned it), she calmly told me that Joshua didn’t have the typical symptoms for PTC. I agreed with her that he didn’t, but then explained that

there had been a study done showing that 50% of kids who don’t have resolution of their chiari symptoms after decompression have PTC and their symptoms are not

what is typically seen (ie: no swelling of the optic nerves ~ called papilledema, and no change in the size of the ventricles). She gave a little snort & asked, “Yeah? And

who did this study?” I pulled out a copy of the study (just “happened” to have it, complete with highlighted important parts, LOL) and handed it to her as I answered,

“Dr. Frim.” She seemed shocked and told me, “David Frim in Chicago? He and I are good friends!” She took the 2 page article from my hand and… surprise of all

surprises… she sat there and read it! Then she half-muttered to herself, “Okay yeah, I see what you’re getting at…. now… what did he do with these kids? Aw

crap… they were shunted… okay, was it in their backs or brains? Crap.. VPs….” (VP is ventriculo-peritoneal, which is a shunt in the brain, versus an LP, which is

a lumbar-peritoneal shunt in the back).

Dr. W agreed that Joshua could have PTC, but said she does not want to put a shunt in him unless she absolutely can’t avoid it because with his connective tissue

disorder & propensity for scarring so badly, she can guarantee we’d be in her office ~ and the operating room ~ on a really regular basis & it would be horrible. I

would be inclined to think Dr. Wehby was exaggerating or “Oh, she can’t possibly predict that happening,” but I know of a girl (online) with spina bifida who also has a

connective tissue disorder and she started out a very bright little girl, but after 27 shunt revisions & a bunch of scarring within her brain, she is now mentally

retarded and has multiple learning disabilities & her doctors had to do some specialized mapping of her brain to try to find a place they could put her shunt and they

found nothing available that hadn’t been tried or was a scarred-up mess. If we can avoid that scenario with Joshua, I (obviously) want to. I know what Dr. Wehby

fears is very real, and I agree with her that if we can treat Joshua for PTC without having to put in a shunt, it’s the best option.

Dr. Wehby told me that she fears if Joshua had a shunt, a malfunction could be fatal. She also said (again) that if has to get a shunt, we’d have to move up to

Portland because every time Joshua got one of his killer headaches & was vomiting, we’d all wonder if it was his shunt and he’d end up having to come in to get

checked. Basically, the picture she painted was a scary one and not a road I want to go down unless we have no other choice. I told her that I’m in agreement that we

should treat him with medication, but if he ever gets to the point where he absolutely needs a shunt, I want him to get it even though the risks are high. Dr. W said

she agreed with that, as well, but reiterated that she does not want to have to do it. She finished off by saying, “I don’t wanna do it; you don’t wanna do it. If we

have to do it eventually, we will, but I know you’ll end up regretting it and so will I.” Not the most comforting statement, but I appreciated her honesty & being

straightforward with me.

She agreed with Dr. Huffman’s choice of Topamax as the medication to try first, so that’s what we’re going to do. We will hopefully see a reduction in headaches &

vomiting without an increase in coordination difficulties and/or other side effects. As long as the benefits of the med outweigh the side effects, we’ll keep Joshua on

As far as his MRIs go, Dr. Wehby said she doesn’t need to do anything surgically at this time (yea!) because she isn’t sure how much of Joshua’s symptoms are due to

his cord being tethered and how much are due to whatever underlying condition he’s got. She said she wants to see what the muscle biopsy shows first and then make

a decision for her part of Joshua’s care, which makes sense to me. I’m not interested in putting him through unnecessary surgery, that’s for sure! She said she

wanted to see if he has a form of muscular dystrophy, and she confirmed that his reflexes in his legs/feet are absent & they’re weak in his arms.

As for the reduction of CSF at the back of his brain, she said that every time Joshua’s head is hyperextended backward, he’s going to have scar tissue being pushed

up against his brain & temporarily impinging the flow of CSF, so the easiest treatment for that was to NOT hyperextend his neck. lol Since his chiari is stable at this

time, the only neurosurgical procedure that might offer Joshua relief from his symptoms is a shunt, but as I already explained, that isn’t going to happen unless we’ve

exhausted every other option first (and even then, it may not happen… depends on what we find out from the muscle biopsy).

After the appointment with Dr. W was over, Adam, Joshua & I checked out of the Ronald McDonald House (such a blessing of an organization that deserves to

be financially supported by anyone looking for a worthy cause to donate to!) and got on the road for home, and 4.5 hours later, here we are. Charley is still not home

(thus why I have the opportunity to type this update), as he had to take Isaac to speech therapy & Emily went along so she could be dropped off at her friend’s house

for a playdate, but it was fun coming home to Megan, David & Sarah. 🙂

We’re happy to be home and I plan to enjoy the next 3 days before we go back north for Joshua’s 2 surgical procedures (we’ll leave on Monday). Tomorrow is Charley

& my 18th anniversary & then there’s the 4th to celebrate, as well.

I’m thankful for the prayers sent up on Joshua’s behalf these past 4 days by so many of you. He did exceptionally well this trip, which made everything so much

easier to handle.

Oh.. and a quick “p.s.” The geneticist’s nurse called just a minute ago to tell me that Dr. Anadiotis had contacted the lab to find out what was taking so long on the

collagen studies from Joshua’s skin biopsy and he was told there have been some “technical difficulties”, so they’re expecting the results some time next week. The

nurse told me that she thinks the technical difficulties involve people not wanting to work so they can leave early for the 4th of July. She also mentioned that Dr.

Anadiotis was interested in the mitochondrial studies more than the muscle fiber analysis portion of the muscle biopsy, so he’d be pestering the lab for those results

right away. I told her that that makes the score 2 for muscular dystrophy & 2 for mitochondrial disease, so what did she want to bet he wouldn’t have either?

She agreed that she could see Joshua being “one of those kids who defy a diagnosis,” but assured me Dr. A wouldn’t give up trying to figure him out, so that gives me

hope that someone on Joshua’s medical team will eventually find the answer we’re all searching for.


Saturday, July 4, 2009 8:57 PM, PDT

I’ve opted to not start Joshua on the Topamax yet because the package insert said to “talk to your doctor or dentist prior to any surgery, as you may need to stop

taking this medication”. Since Joshua is scheduled for surgery in 3 days, I kinda thought giving him a medication that might force the cancellation of his surgery was

not a great idea. I called the pediatrician to verify that this was an acceptable plan of action & she agreed that it was, so I’m comfortable with the decision. I figure

waiting a week to start it isn’t really going to make or break anything.

I put in a call to the neurologist on Friday and left a message asking if prescribing a medication that needs to be weaned prior to surgery is really the best drug of

choice for a child who, historically, has had surgery on a regular basis for the past 2 years (I worded it a bit more politely than that, though). I do think there’s a

good chance that Joshua’s frequent surgeries are a thing of the past since his chiari is stable & Dr. Wehby isn’t going to be detethering him multiple times each year.

As long as he doesn’t get a shunt, I don’t expect to be seeing the neurosurgeon very often anymore (wouldn’t that be awesome?!). However, I thought I’d pose the

question to the neurologist, anyhow, because if Joshua were to get an infection in his port & need to have it removed, we wouldn’t have adequate time to gradually

wean him off the Topamax and I don’t know how that would be handled. So I’ll hopefully connect with Dr. Huffman’s office on Monday to get an answer.

I’ve read a bunch about Topamax and will flat-out admit that I’m apprehensive about giving it to Joshua. If you go to

http://www.askapatient.com/viewrating.asp?drug=20505&name=TOPAMAX&page=1 this website, you can read about the side effects experienced by

patients who have taken Topamax. The overwhelming majority (1046 respondants thus far) have had cognitive problems as a result of taking the medication (trouble

with word retrieval, slow processing speed, losing their train of thought, not remembering how they got somewhere, spelling ability going down the tubes, etc), as well

as numbness/tingling/sharp pain in their hands, arms, legs & feet. A large number of people have weight loss because they have no appetite & food and drinks taste

terrible to them. There are other worrisome side effects, but those are the main ones that almost every single person who responded said they experienced.

The potential side effects concern me because Joshua is finally interested in eating food & I don’t want him to stop. Also, I personally would hate to have numb

hands/feet or tingling sensation in them & I don’t think my son will enjoy it, either. And the cognitive problems? Who would want the equivalent of Alzheimer’s for

their 3 year old? And what about loss of coordination? Like Joshua doesn’t fall enough on his own? So… yeah… it concerns me.

I am trying to give this big worry to God. I know He loves Joshua even more than I do and I want to believe that He will protect him as we trial this medication. I

know that increased CSF pressure that goes untreated will eventually cause damage to a child’s brain, so I feel as though we have to try something, and since I agree

with Dr. Wehby’s assertion that we should try to avoid placing a shunt in Joshua, medication is all we have left to try. Sooooo…. I’ll take a deep breath, pray for

God’s protection over Joshua, and when we get home from Portland and he is on the road to recovery, I’ll start the trial of Topamax. I would greatly appreciate

having others join me in praying that the medication would work well for Joshua without any side effects.

Monday, July 6, 2009 5:57 PM, PDT

The neurologist called and we discussed Topamax versus Diamox. She said she’d spoken today with Dr. Wehby and the neurosurgeon gave her conflicting information

which had her feeling stuck between a rock & a hard place. Basically, the neurosurgeon is saying that she doesn’t think Joshua’s problems are all due to high pressure

& she is unwilling to put in a shunt for pseudotumor cerebri, but she still fully supports conducting a trial of Diamox, which is the medication typically prescribed to

treat pseudotumor cerebri (when trying to avoid putting in a shunt). Dr. Huffman (neurologist) said she doesn’t know how she can be expected to prescribe a

medication for high CSF pressure without hard proof of that increased pressure. I told her I understood her dilemma, but I couldn’t force Dr. Wehby to do a lumbar

puncture in order to give her the evidence of increased pressure.

I believe that when Dr. Wehby told Dr. Huffman that she didn’t think Joshua’s problems were all due to high pressure, she was referring to the assumption that

there is an underlying genetic condition causing some of Joshua’s symptoms. I don’t think Dr. Huffman understood that, however. She’s relatively new to Joshua’s

team of doctors, though, so I can’t really blame her for not knowing everything about him yet.

I am a bit frustrated with Dr. Wehby, though, because she is not making this easy for Dr. Huffman and she absolutely could have done so by offering to do an LP

tomorrow when Dr. Bliss has Joshua knocked out in the OR. I told Dr. Huffman that I didn’t know what to do because as a parent, I have zero power whatsoever. She

replied that she wasn’t saying she wouldn’t do a trial of Diamox because she knows Dr. Wehby would like her to do one, but she could really use some hard evidence

that the medication is an appropriate one to try. To that end, she is going to contact Joshua’s ophthalmologist tomorrow and get his records transferred up. I told

her she wouldn’t find any evidence of papilledema (swelling of the optic nerves) since Joshua’s never had it, but the absence of papilledema did not exclude the

presence of PTC in kids who also have chiari malformation.

She also asked if we could get labs drawn tomorrow to get a baseline of Joshua’s electrolytes because Diamox has a serious side effect of messing up the

body’s electrolyte levels, as well as paresthesia (the same numbness in hands/feet that Topamax causes), fatigue, decreased sweating and more. I asked if it had the

same cognitive side effects that Topamax has and she said, “Not usually.” I told her that if she wanted labs drawn, I knew it wouldn’t be a problem to get them since

Joshua will be in the OR and they could get ’em then, but she’d have to write the orders because no one is going to listen to me.

The conversation ended with the neurologist saying she would touch base with me by the end of the week to discuss which medication she wants to start Joshua on.

I’m hopeful that she will choose Diamox because that is typically the medication used to treat pseudotumor cerebri and if I have to put Joshua on something that is

going to potentially have a lot of nasty side effects, I’d rather it be the one that ALSO has the potential to help him the greatest amount.


Friday, September 11, 2009 6:08 PM, PDT

We’re home! I’ll write more later, but the nutshell version of things is this: EMG/NCS results were normal (that doesn’t 100% rule out a problem, the

physiatrist told me, but he also said he thinks my idea of SB + PTC = “a home run” as far as diagnosis), I was able to talk to the neurosurgeon’s P.A.

about coming up with a long-term plan for what we’ll do once diamox isn’t working anymore (it loses effectiveness in time) since no one wants to put a

shunt in Joshua, he (J) is sore but in a decent mood now that we’re home, & I’m exhausted.

Will write more in-depth later, if not for your interest, for my own recollection in the future. 🙂

Thanks for the prayers ~ I think they definitely helped!


Sunday, September 13, 2009 11:13 PM, PDT

When I was registering Joshua at the hospital for the EMG/NCS (electromyelograph/nerve conduction study), I noticed the

paper said, “EMG/NCS with Versed”. That made me nervous because I’d been told Joshua would be sedated, which has

always meant “knocked unconscious” in the past, and I knew that Versed doesn’t work on Joshua at all anymore, but I

resolved to stay calm & see what the nurse/doctor had to say.

When the nurse came to get us, I asked her if the plan was to use Versed to sedate Joshua for the procedure. She replied

yes, then began to explain what Versed is & how it works. I interrupted her to ask, “Do you know that Versed is not

effective on Joshua anymore?” She didn’t. She asked why. I told her, “Probably because he’s had 16 surgeries, a dozen or

so MRIs & lots of CTs and he’s been sedated for all of ’em. Eventually it just doesn’t work anymore, I guess.” She was really

nice and discussed with me some potential options, including rescheduling the test with full sedation (ie: knocking him out),

using different medication to chill Joshua enough to do the test, or trying Versed to see if maybe a huge dose would work. I

shot down the last option since I’ve seen the anesthesiologist give Joshua 3 full doses within 30 minutes & get zero effect as

recently as July 7th, so I didn’t see any reason to waste time trying to make Versed work and I refused to hold him down

throughout this test (they stick electrode needles into the muscles and send an electric current through them to measure

how the nerves are functioning) and traumatize him. I wanted him unconscious or totally gorked out on something. Period.

I expressed my wishes (politely) and the nurse said she’d call Dr. J to see what he wanted to do.

About 5 minutes went by and then in walked Dr. J. He hadn’t made up his mind what to do about the sedation, but he

wanted to discuss Joshua’s symptoms & whatnot, instead. During our discussion, I asked him if he was going to

test Joshua’s arms and legs and he told me he just wanted to do the legs. I told him that I didn’t think that was going to be

useful and he wanted to know why I thought that (asked as he was almost rolling his eyes at me), so I said that since Joshua

has spina bifida, his legs were obviously going to have abnormal nerve conduction and the test results would not tell us

whether the abnormal nerve function was due to spina bifida or something else…. which was the same problem we had with

the muscle biopsy, since the surgeon took the sample from his thigh rather than his deltoid (muscle in the backside of the

upper arm). The physiatrist looked at Joshua, who was sitting in his wheelchair playing a video game with Adam, and asked

in a loud, VERY shocked voice, “He has SPINA BIFIDA?!?!?”

Yes, folks, this doctor who was minutes away from doing an invasive test on my 3.5 year old didn’t even know he had spina

bifida!!! The man had not looked at one page in Joshua’s 6-inch thick chart. I did not manage to hide my disdain as

I answered Dr. J’s question with the response, “You don’t know his primary diagnosis?” Be proud of me, though, because I

didn’t add, “You moron!” to the end of that question.

Seriously, is it expecting too much to think a doctor would look at the FIRST PAGE of a kid’s chart to learn a little something

about him ~ you know, like WHY he’s coming to have an EMG done ~ before he walks in the room to treat him? Sheesh. I

was incredulous. He tried to blame the geneticist for not telling him that Joshua had spina bifida, at which point I bluntly

reminded him that Joshua has both a large paper chart and an e-chart accessible by any computer in the hospital and that

every report by every specialist he’s seen clearly states at the top of the report that he has SB, so he (Dr. J) could have

made himself aware of my son’s primary diagnosis prior to walking in the room. He was busted & he knew it. I was less

than impressed that he didn’t own up to it, but instead tried to blame someone else.

There was a lot of intense discussion for about 20 minutes before Dr. J finally agreed to knock Joshua out for most of the test

and then have the anesthesiologist half-wake him for the last part of the test where they needed to have him contract

muscles, AND he agreed to test Joshua’s arms since testing his legs would give confusing results. I was allowed to hold

Joshua on my lap while he was gassed to sleep and then Adam & I left the room (we weren’t allowed to be in there;

otherwise we wouldn’t have left).

The test was normal, which means that Joshua does not have a degenerative condition, he doesn’t have spinal muscular

atrophy, and his problems are probably all related to his spina bifida, connective tissue abnormality & pseudotumor cerebri.

So that’s good, of course, but also concerning since the neurosurgeon won’t put in a shunt (he’d scar over it and need a new

one put in over & over & over, which would lead to brain damage from scar tissue being deposited in his brain and then it

could eventually kill him, per the neurosurgeon’s explanation for why she doesn’t want to put in a shunt). Yet a shunt is the

proper treatment for PTC (pseudotumor cerebri). We’ll use the Diamox, which works, but he can’t be on Diamox forever because

it loses effectiveness, which necessitates a higher dose, but higher doses wreck the bicarb level (ie: kidneys), so

then he won’t be able to take it anymore. I ran into Jenny, the neurosurgeon’s P.A., and she said she’d chat with Dr. Wehby

about coming up with a long-term plan for what we can try when Diamox isn’t working anymore. When I take Joshua to

Portland this upcoming Thursday to see his neurologist, I’ll pop over to Dr. Wehby’s office (she’s next door to Dr. Huffman,

so it’s convenient) and see if she’s come up with anything yet (or remind her to start thinking about it).

Dr. Wehby hasn’t wanted to concede that Joshua actually has PTC, but in light of all the testing he’s had done & the fact that

everything has been normal, there isn’t really anything else that explains Joshua’s symptoms OTHER than Pseudotumor

Cerebri plus Tethered Cord as a result of Spina Bifida. Sooooo… I’m praying that the neurosurgeon will come around and be

moved to WANT to investigate the connection between Chiari & PTC so that she can prove to herself that yeah, it’s possible,

and yeah, Joshua has every symptom in the book, and yeah, this is something that she is gonna have to help

manage because it doesn’t fall under anyone else’s “jurisdiction,” so she can’t farm Joshua out to some other doctor (as

much as she may wish she could). 🙂

Oh! Another thing! It turns out that even the abnormal muscle biopsy results were actually “normal” for Joshua since severe

chronic denervation atrophy (what the muscle biopsy showed & what led the geneticist to mention Spinal Muscular

Atrophy) is something that is seen in muscles affected by spina bifida as well as conditions like SMA. But no one from

around here bothered to tell me that! My Australian friend had her neurologist friend look at Joshua’s muscle biopsy results

and it was that doctor who said the denervation atrophy wasn’t anything to worry about since Joshua had Spina Bifida. If it

weren’t for those two Australian women, I would have continued to wonder if Joshua had SMA, and I would not have thought

to ask the physiatrist to test Joshua’s arms rather than his legs, which then would have led to a false diagnosis once the EMG

showed abnormal nerve conduction results. So I am thankful that God provided the intelligence of my Australian friend &

HER friend to guide me in the right direction so that we (Charley, myself & the doctors) could get an accurate picture of

how Joshua’s nerves are working & save us from going down the wrong road.


Monday, October 26, 2009 6:21 PM, PDT

Joshua had an impromptu visit to the pediatrician this afternoon after speech therapy. He moaned “ow… ow… ow…” throughout the entire appointment, his head

resting on my shoulder. It was pretty pathetic-sounding.

The ped called the neurosurgeon’s P.A. to tell her what is going on and was told that the docs in Portland “feel pessimisstic”. I’m not entirely sure what that means,

but it didn’t sound good. The ped said that it may be time to start thinking of taking Joshua to a big medical center like Stanford or UCSF (Univ. of California, San

Francisco). There is no way that Joshua is the only child in the United States who has intracranial hypertension + spina bifida + chiari + connective tissue problems

that make getting a shunt out of the question, so what we should start looking for is someone who knows how to treat these kids. Because really, there HAS to be

someone out there who knows how to treat this, right???? Right? Okay, I HOPE there’s someone and that God will lead us to him/her. And in the meantime, I hope

that our neurosurgeon will be able to help get Joshua out of pain again to provide him with another chunk of stable time.

Charley and I agreed with the pediatrician on everything she said, and she said she wish she knew what to suggest to us for how to help our little boy. I told her it was

all right… and at least he’d had a pretty long good period this go-round. She looked at me and said, “Ten weeks is not a long time.” I guess she’s right. It’s kind of

sad that 2.5 months seemed like a long time, though.

She sent us over to the hospital infusion clinic to get labs drawn, and for the second time in a row, the EMLA (numbing cream) did not work, so Joshua had another

traumatic blood draw through his port. The nurse & I couldn’t figure out why he’s not getting numb, but the nurse suggested I get a new script for EMLA in case

what I’m using is too old to be effective.

As we were leaving, another nurse was following us and listening to Joshua cry the whole way from the infusion clinic to the parking lot. She approached me and

handed me a small paper bag that contained two cupcakes, telling me to please take these for my little boy and she hoped he would start feeling better soon. That was

such a sweet gesture, I thought.

I am currently waiting for the results of the lab draw. Instead of checking just Joshua’s bicarb level, Dr. S ordered a full metabolic panel to make sure his kidneys,

liver, blood counts & the like are all okay. She said she’d call after hours to give me the results.

We’re going to give Joshua pedialyte for 24 hours to rehydrate him. He’ll get 50ml/hr (that’s 1.67 ounces per hour) and then we’ll switch back to giving him his

regular formula again. We can’t run his formula on a continuous feed because he doesn’t tolerate it for more than a few hours before he starts throwing up, which

pretty much defeats the purpose of the continuous feed.

I’ll post another quick update if the results of the bloodwork show anything concerning. If I don’t post anything new tonight, it means that nothing worrisome showed

up (that’d be good!).


Thursday, November 12, 2009 5:25 PM, PST

I’m sitting here in a Howard Johnson Inn partway between Portland and home, extremely tired and thinking about the visit

we had with Dr. W today. The Ronald McDonald House was full because the west house (there are two in Portland ~ one on

the east side of town and one on the west side) is getting renovated, so those families that normally would stay in the west

house got shunted over to the east house. That’s why we’re in a motel rather than our usual digs across the street from the

The appointment with the neurosurgeon went as well as could be hoped for. Dr. W will be detethering Joshua’s spinal cord

(5th time) and placing a lumbar-peritoneal (LP) shunt in his back. The placement of the shunt is not ideal; it should go into

a ventricle in his head. However, Dr. W said Joshua’s ventricles are so tiny as a result of the pressure of the cerebrospinal

fluid pushing down on his brain that if she tried to put a catheter into one of them, it would make the vents collapse and that

would be devastating to him.

As worrisome as putting a shunt into Joshua is due to his propensity for overproducing scar tissue (and the resultant

concerns about repeated obstructions & need for subsequent surgeries to replace the shunt), Dr. W said we’re at the point

where this is really our last option. There is about a 50% chance that the shunt will work to alleviate Joshua’s pseudotumor

cerebri symptoms and the neurosurgeon wasn’t feeling overly optimistic that Joshua will fall into the right 50% category. I

asked her what we’d do if it didn’t work and she said all we could do then is send Joshua to rehab and let the pain

management doctor find a combination of medication that would keep Joshua out of pain. She agreed that keeping him

pain-free would do nothing for reducing the pressure in his head (if the shunt doesn’t work), but again, said that there’d

really be nothing else we could do.

Soooooooo… we’re hoping the shunt works. We’re hoping really, really hard that the shunt works and that Joshua doesn’t

scar over it and that it reduces the pressure in his cerebrospinal fluid and that it gets the pressure off his brain and that he’ll

get some long-term relief.

Dr. W said that she talked with the geneticist & they still think Joshua has *something* going on, but the geneticist said he

didn’t know what else to test for at this point in time. I heard that and kind of shrugged a little. I mean, really… what’s the

point of saying, “We think your kid has a problem but we can’t test for it or tell you what it is.” ??? That seems pretty

pointless to me. Considering all of the testing we’ve done already (bloodwork, skin biopsy, muscle biopsy, EMG), I’m not

going to worry about a potential *something*. Sure, it might be the clue that answers the question of why Joshua keeps

having problems and doesn’t ever stay “fixed” for a long period of time, but since there’s no way to figure it out, I am

refusing to dwell on it and expend mental energy stressing about the possibility.

The surgery scheduler was not in the office at the end of our appointment, so I’m supposed to call tomorrow to get the date

set for Joshua. I was told that it’ll be after the beginning of the year since Dr. W is completely booked until January, which is

kind of the pits since we’ve had to increase Joshua’s dosage of diamox again and he’s having breakthrough pain in-between

some doses, so in addition to being a bit concerned about his bicarb level (which will need to be checked as soon as

possible), I’m nervous about our ability to keep him comfortable until January. Dr. W’s P.A. gave us a script for more tylenol

with codeine and said we’ll probably have to deal with withdrawals when Joshua is in the hospital since he’ll have

been getting the narcotic for over 3 months by the time he has surgery. I guess that’s something else to look forward to

(yes, I’m being sarcastic).

I’m sure I’ll have more to say later, but for now, I think this is enough. For now, I’m going to go cuddle my little boy and

watch some NCIS with Adam & Emily. Today has been a very long day.


Friday, November 13, 2009 6:03 PM, PST

Surgery will be November 30th. Thankfully, Dr. W wanted it done soon, so Joshua won’t have to wait until January. I’m SO relieved!!!

We’ll celebrate Isaac’s birthday a few days early on November 28th (his 6th b’day is December 2nd, but we won’t be home) and then Charley, Adam, Joshua & I will

head north on the 29th. Charley will come home the day after Joshua’s surgery, leaving Adam to help me like he always does. He (C) wants to come up for this

surgery, which I appreciate. It will be nice to have his support. He won’t be able to stay long since he has to work, but I’m glad he’ll be there for the day of surgery.

I’m off to read up on LP shunts. I know that they have the same risks as VP shunts (a shunt that goes from the brain to the abdomen) ~ infection & occlusion &

malfunction ~ but I want to see if there’s anything different about an LP shunt that might be useful to know. Besides that, I don’t really know the symptoms of shunt

problems yet (never needed to know until now), so I’d best get educated in the next couple of weeks.

We’re doing labs on Monday to check Joshua’s bicarb, but even if it’s dropped below 17, we’re not taking him off the medication until after his surgery. No way, no

how. He’s currently getting 2.0ml three times per day plus one or two 1.0ml ‘boluses’ when he’s having breakthrough head pain. The tylenol with codeine is for his

back pain and is still effective for keeping him comfortable.


Wednesday, November 18, 2009 8:13 PM, PST

I called the neurosurgeon’s office on Monday to ask if it would be possible to give Joshua something other than versed at pre-op since that med does nothing for

relieving Joshua’s anxiety. After so many surgeries & other procedures that he’s been sedated for, Joshua knows what is happening as soon as he sees us heading for

short-stay (where we go prior to going downstairs for him to head to the operating room). I was feeling worried about having another horrible hand-off like we did in

July, so I thought I’d see if some other anti-anxiety medication could be given. The neurosurgeon’s P.A. called me to say that yes, absolutely we could give Joshua

something that will hopefully lower his anxiety, which is a huge relief to me. She said she’ll have the anesthesiologist call me to discuss everything and that he’ll

decide what my little guy will be given. Probably something like valium or ativan.

The P.A. also talked with me about the two procedures that she & the neurosurgeon will be doing. This will be Joshua’s 5th cord detethering, so I know what to

expect with that. Dr. W will remove the accumulated scar tissue and regrown lipoma so that Joshua’s cord can float freely within his spinal canal (until it gets

retethered yet again, because that is the nature of the beast with his constellation of conditions). Because of the problems we’ve encountered with getting his dura to

heal post-operatively, Joshua will be corsetted again ~ wearing the black compression brace that he has worn after the past two back surgeries ~ and he’ll keep that

on around-the-clock for 3-4 months. That will hopefully prevent a large CSF leak from developing, which is why the compression brace is used.

As for the shunt, the P.A. explained how it is inserted ~ one end of a catheter is placed in the spinal canal and the other end exits the dura & attaches to an

adjustable valve, and then another cathether that is also attached to the valve will be tunnelled under the skin around Joshua’s side and a tiny cut will be made in his

belly so the doctor can pull the end of the catheter into his abdomen and tack it down. Excess spinal fluid will drain out of the catheter in his spinal canal and into his

peritoneal cavity, where it will be resorbed by his body.

The P.A. told me she and Dr. W aren’t worried at all about the surgeries, themselves. It’s the aftermath that has them concerned (not in recovery, per se, but longer

term). She told me LP shunts generally have more complications than VP shunts (ventriculo-peritoneal ~ shunts that are inside the head) and that they worry that even if the shunt works, Joshua’s connective tissue problems will cause him to scar over the shunt tubing & obstruct it on a repeated basis. The odds are 50/50 that putting in the shunt is going to work to control the pressure in Joshua’s spinal fluid and they predict the odds of complications to be much worse than that, so that wasn’t very encouraging to hear (again, since the doctor had told me the same thing). She said that after surgery, Joshua will probably have symptoms of low CSF pressure, which would include headache, nausea and/or vomiting, sensitivity to light & a desire to lay flat since that will be what makes his symptoms improve a little. These symptoms can be expected to occur in some form of severity for up to 4-8 weeks post-operatively, though he could feel better sooner or he might have continuing troublesome symptoms for longer (there’s no way to predict this). I asked her how long I should expect him to be in the hospital and she laughed. Yep, she did! I knew why, actually, and I chuckled right after asking the question. You see, thus far in his life, Joshua has only had 1 “typical” hospitalization where everything went the way it was expected to; he averages 2 weeks in the hospital after each surgery, so no one expects him to do the expected anymore. However, IF everything went perfectly, he’d have surgery on Monday (the 30th) & we’d come home on Saturday (Dec. 5th). It’s a nice goal to pray for, I think.

Joshua’s lab draw on Monday showed his bicarbonate has dropped to just barely above the level at which the nephrologist said he’d have to be taken off themedication. As such, he’ll have another blood draw on Friday to assess how things are progressing. As long as his bicarb doesn’t drop to a dangerously-low level, Ihave no plans to take him off the diamox no matter what the nephrologist wants (the neurosurgeon doesn’t want me to take him off of it, either, as it is the only thing keeping Joshua’s CSF pressure at a tolerable level). We need his kidneys to ‘hang in there’ for 11 more days!

Friday, November 27, 2009 11:42 PM, PST

Now that Thanksgiving is over, there’s not a lot to detract my thoughts from focusing on Joshua’s upcoming surgeries on Monday. I’m finding myself more

nervous this time. Not about the spinal cord detethering. That doesn’t stress me since it will be the 5th time Joshua’s had the procedure done, so I feel

like I pretty much know what to expect. I know something surprising could come up, but I don’t expect that to happen at all. As for the shunt, however,

I’m feeling less certain. Oh, I’m not worried about the surgery to put it in. I have full confidence in our neurosurgeon & I expect no complications during

the surgery itself. It’s what happens afterward that has me feeling uneasy.

I know I need to have faith that the shunt will solve the problem of his increased intracranial pressure, but with the odds being just 50/50, it’s not so

easy. The words, “this is our last option,” reverberate in my head & don’t do much to instill a sense of optimism.

I’m reading my Bible & praying, of course, and I do find comfort in the promises of God. Despite that, however, I still have times when I am scared. I

want so badly for the shunt to work. I want an end to the roller coaster that my little boy & the rest of my family have been on for the past 3 years.

Damn. Three years. I typed that and thought, “Is that right? Has it been three years, really?” Sadly, it has. Joshua first started showing symptoms of

chiari & tethered cord when he was 8 months old and he is now a few days away from being 3 years + 9 months. Since then, it’s been a pattern of

surgery leading to improvement & stability, which leads to increasing symptoms & an overall decline starting up again which eventually leads to more

surgery. On average, Joshua has gotten 4 to 6 weeks of feeling good (ie: no pain, vomiting or other symptoms) before symptoms begin again. It’s a sad

way for a child to live. His therapists are amazed that he’s not further behind in development than he is. After all, it’s difficult to learn new things when

you’re puking a couple dozen times every day or when you’ve got excruciating headaches or moving makes your back hurt. The fact that Joshua functions

as well as he does is a testament to his strength, resiliency & high tolerance for pain.

I desperately want him to be one of the people for whom a shunt is successful in treating pseudotumor cerebri. I want this to be the answer to my

prayers ~ the solution that allows Joshua to get more than a couple months of relief. I don’t know if my hopes are realistic, though. I know he’ll continue

to produce too much scar tissue & that will cause his cord to tether & become symptomatic more rapidly than normal. I know he will face surgery to

address bowel issues eventually. His days of having surgery are not going to end after Monday’s procedures unless God steps in and miraculously heals

Joshua of all his conditions. Do I believe that could happen? Yes. God can do all things. Do I think it’s going to happen? No, not really. Perhaps that will be

construed as a lack of faith on my part, and maybe it is, but 18 surgeries in 3 years (and 19 total ~ the first was done when Joshua was 7 days old) have

a way of convincing a person that God is allowing this all to continue for a reason and doesn’t have plans to step in and wipe it all away any time soon.

I’m not saying He can’t or won’t. I’m just admitting that it doesn’t seem likely to me.

I wish I had the kind of faith that would have me proclaiming in all sincerity that I was completely at peace and whatever God wanted to happen, well…. I

was fine with that! I don’t, though. I know that Joshua belongs to God. All of my children are on loan to me and I get the privilege of raising them, but

they ultimately belong to God, so He can do in their lives whatever He wants & I can’t control that. I understand that basic truth. But I still am wrestling

with the idea of acceptance in the form of saying, “Your will be done, God. No matter what.”

The truth is, I want God’s will to be done if the outcome is a good one. But if the road for Joshua is not going to be long, or if this pattern that he’s been

in for the past three years is not ever going to end (or is going to get worse), then I’m not at a place where I can accept that graciously. I want my son’s

suffering to end, but not with his death. I want it to end & for him to be able to live & learn & laugh & enjoy life like every little child should get to do. I

am extremely well aware that there are a multitude of children who suffer due to chronic illness, dire living conditions, extreme poverty, etc. My heart

aches for them, but it cries for my son because I love him & it is brutally painful to watch him hurting but know there is nothing I can offer aside from

some medication & being held.

There has to be an end to this cycle, right? Eventually a time is going to come when he either gets better or he doesn’t. Joshua’s neurosurgeon believes

we are at that point. The shunt is our last option (this opinion is supported by several other reputable neurosurgeons around the country). Either it’s

gonna work or it’s not. If it works, he could still wind up in the operating room on a regular basis due to the shunt becoming obstructed with the scar

tissue that everyone expects he will continue to overproduce as a result of the connective tissue disorder he has, but at least he would not be screaming

or moaning in pain for hours every day. If it doesn’t work…. well, if it doesn’t work, then we’re going to be in a world of suckitude. Hardcore. I can’t really

fathom living the way we’ve been with no end in sight, or having to keep my son on heavy-duty narcotics in order to control the pain caused from having

his brain & spinal cord compressed by spinal fluid that is under high pressure. Merely contemplating it makes my nose burn & my eyes fill with tears &

then I have to tell myself, “Nope, that’s not gonna happen,” in order to regain my composure. I just hope & pray I’m not lying to myself. This has to

work, right? Because, really, the alternative would be unbearable. I know I can do all things through Christ who strengthens me, but I don’t want to be

strengthened to walk that road & I definitely don’t want my son to have to walk it, either, or any of my other children or my husband (because this is

something that affects all of us). I’m praying that God will stop this roller coaster and tell us that we’ve ridden long enough & can finally get off. WITH

Joshua, who will be happy & headache-free for good.

Is that too much to ask for?

I guess time will tell. All I know right now is that when it comes to the shunt, we have just two options: it will work or it won’t.

I’m pulling for WILL.

Monday, November 30, 2009 9:49 AM, PST

Update from the OR: Joshua is stable. Dr. W has Joshua under the microscope & is working away. It’s slow-going, as he’s got a lot of scar tissue. Once he’s

detethered completely, then she’ll start the shunt placement. No idea how much longer it will be.

Monday, November 30, 2009 1:57 PM, PST

He’s out of surgery & doing well. Dr. W said his spinal cord was pulled to one side by scar tissue & twisted, which is why he was hurting so much.

The shunt was placed at the T12 level, just above where his previous scar ended. The tubing goes around his right side, just above his hip, and he has a tiny incision in

his belly. Overall, he looks really good.

Thank you for praying for Joshua.

Tuesday, December 1, 2009 12:41 PM, PST

Last night, not long after I posted, Joshua projectile-vomited bile despite having his g-tube venting continuously (it was draining into a diaper) & receiving

IV zofran. A few hours later, he vomited a few more times and then had some thick greenish gunk mixed with blood that poured out of his g-tube when

he cried/vomited. No one knew last night what it was. No one knows today what it was. It’s been saved in the diaper it drained into so that the

gastroenterologist can examine it. It continued for a couple hours & then tapered off. The blood is assumed to be from irritation caused by being

intubated. The green color is bile, but the big question is what is the creamy substance that the bile was mixed with. It’s a little mystery no one has yet

figured out.

There was concern this morning because he’s not making a lot of pee (only got rid of 155ml in 12 hours) & the question of whether diamox might have

affected his kidney function was raised. A bedside bladder ultrasound showed a majorly-distended bladder full of urine & a kinked foley catheter. HOW it

got kinked inside him is anyone’s guess. What they told me was, “Well, Joshua does do things his own way.” Not the most-reassuring answer, but really,

the only answer for how it happened is, “I don’t know. It just did.”

It took 2 hours to get a new catheter in. We tried straight cathing him with no success. We just could not get the catheter in since he was so overdistended.

I tried a few times, 3 nurses tried, and finally the charge nurse got a tiny (6 french ~ it’s the same size as a feeding tube used in NICU for

newborns) foley catheter in, so now he’s got pee draining very slowly, but at least it’s coming out.

Labs have been drawn to check his bicarb level & other kidney function tests, the IV team will come later to change his port dressing (he HATES that),

and both GI & neurosurgery will be in later, as well.

I clamped his g-tube at 11am and we’ll see how he does ~ whether he moves the gastric juice down through his intestinal tract or if he starts puking it

up. It’ll give us an idea as for when we’ll start trying to feed him again.

The neurosurgeon’s P.A. came in and reprogrammed Joshua’s shunt, which was an interesting thing to watch. It’s a special magnet that’s used to set the

shunt to the drainage level they want. She said it will probably take a few adjustments to find Joshua’s “sweet spot” ~ the perfect drainage level for him.

All in all, it’s shaping up to be a typical recovery for Joshua, but maybe things will turn around for the better and surprise us all.

Wednesday, December 2, 2009 3:59 PM, PST

Joshua’s morning has been rougher than it needed to be and I’m a bit frustrated by it.

We have a competent but uncaring nurse this shift. She thought Joshua should go down to just tylenol with codeine today simply because “it’s been 48

hours since surgery & we always move detethered kids to tylenol after 2 days” (nope, she’s never met Joshua before), and when I told her that wasn’t

going to cut it for my kid, she thought I was just trying to keep Joshua on narcotics unnecessarily. That led to me telling her to go call the neurosurgeon

NOW and find out what HER orders are for my kid. She did and found that Dr. W wants Joshua to still get morphine & fentanyl, not just T3. I hate it

when a nurse who doesn’t know my child refuses to LISTEN when I’m trying to explain how my child is different from the norm & why we do things

differently with him and, as a result, my child suffers for her pigheadedness.

Moving Joshua causes him tremendous pain ~ he can’t roll from his side to his back by himself yet, even ~ so when his diaper leaked (he was leaking

urine around the tiny catheter they had in him) and we had to change the chux pad & towel he was laying on, it really hurt him. We had just got him

situated & calmed down when a CNA came in to give Joshua a sponge bath. I told her I thought he was okay, but she insisted, so I relented and told her

she could wash his legs, but leave the rest of him alone. Long story short, she ended up getting the clean chux pad & towel soaking wet, which

necessitated moving Joshua again so we could lay down clean/dry stuff again, and THAT led to his intense crying because his back hurt and then he

started throwing up, which made him hurt worse. He got into a cycle of retching and crying and because his pigheaded nurse thought he didn’t need

anything more than tylenol with codeine & hadn’t yet called the neurosurgeon to confirm that I’m not a moron & yeah, my kid could still get

morphine, Joshua had to wait longer than necessary to get the pain relief he needed to help him to settle down.

So… the morning was frustrating.

We’ve given him a couple tubefeeds (2 ounces pumped in over an hour) and he threw up the first, then kept the second one down but retched a lot and

WANTED to throw up (but couldn’t). We vented his tube & gave zofran to stop the dry-heaving, but it continued for about 20 minutes after there was

nothing coming out of his g-tube. What we surmised was that the pressure of the fluid draining from his shunt into his belly may have been pushing

against his stomach, which was contributing to him feeling nauseated. The right side of his stomach and around his side are pretty squishy and swollen,

which the nurse told me is from fluid draining and not being resorbed quickly.

The foley catheter is out and I’m back to straight cathing 5 times a day, which is fine because it’s one more step closer to normal (for us).

One last thing for now ~ a clown came by & gave Joshua a red nose, some crayons & a peacock feather, which my little guy is using to poke the

nurses when they come in to bother him. LOL He is feeling pretty feisty when he’s not hurting.

Sunday, December 6, 2009 9:27 PM, PST

Another up & down day. Joshua had a lot of nausea after his 6 ounce feeds today, but no vomiting (still have one more feed to give him at 10:00pm, so that status

could possibly change). He sat at 30 degrees for most of the day & was able to sit upright with Adam’s help for a few minutes, too.

He fell asleep a bit before 7pm and then woke suddenly, wailing with head & back pain & retching. After not having any pain meds for 36 hours, he absolutely needed

morphine to deal with the level of pain he was having. He also got zofran to stop the retching. The nurse put the head of the bed back to laying flat and said we

needed to keep Joshua down for the rest of the night.

I know he’s making progress, but any setback is still a disappointment. I’m not accustomed to Joshua needing morphine once he’s been off of it for over 24 hours, so

this was a new thing for him. The neurosurgeon said that the shunt would cause some pretty intense headaches for a few weeks & I now see what she was talking

about. I feel so badly for him when he’s hurting and there’s nothing I can do aside from buzz the nurse to come drug him up. I am so hopeful that Joshua will soon

not need any pain meds at all. I pray I’m not being unrealistically optimistic.

Wednesday, December 9, 2009 6:55 PM, PST

We’ve been sent on a 48-hour pass to the Ronald McDonald House. On Friday at 1:30pm, we’ll see the neurosurgeon and she’ll reprogram Joshua’s shunt

(if necessary) and then we will get officially DISCHARGED to go home!!!

It feels really good to be here at the RMH and out of the hospital environment. We have to be here for 48 hours to ensure that Joshua can maintain his

fluid levels adequately since that has been a problem for him in the past. The neurosurgeon also wants us to stay close to the hospital for a few more

days to make sure Joshua’s shunt is set at the right level ~ we’ll be watching for headaches & nausea/vomiting & pain that isn’t relieved by tylenol with

codeine. As long as that isn’t a big problem, she’ll leave his shunt set where it’s at & send us home.

I don’t foresee any problems ~ Joshua is happy & feeling good. He’s in his wheelchair and has only needed one dose of pain medication so far today. I will

upload some pictures tomorrow to show you all just how good he looks. I know you all think you’ve seen Joshua looking happy, but wait until you see him

now. The absence of pain always makes him almost euphoric. He’s giddy and giggly and it’s such a HUGE relief to see him this way again. We’re hoping it

lasts a really long time.

So… we’ll be home on Saturday (Charley will get up here Friday late afternoon and we’ll leave Saturday morning). I can’t wait to sleep in my own bed,

but it’s going to be really wonderful to sleep in the double bed in our room tonight without any nurse visits or beeping to disrupt our slumber.

Adam, Joshua & I are all happy tonight!


Joshua’s Walk

  • I was asked on Topix to post this post about Joshua, Adam and Kate going for a walk. This post was made the day before Kate Estes put up a post asking fir more fundraising monies.
  • Written August 5, 2013 2:41am by Kate Parker

I don’t know how I am going to survive this. I look at him sleeping next to me and think of the last thing he said before he fell asleep (“I love you forever & ever.”) and the thought of not hearing that every night anymore makes me feel sick inside. I don’t want to have to do this… I don’t want to have to live without my little boy. I’m thankful he is still here for me to hold. I never want to let him go even though I simultaneously do not want him to suffer a moment longer than he is forced to. My head asks God to come quickly and free Joshua from the pain. My heart begs God to let me have a little more time because the rest of my life seems way too long to have to live without my son & I don’t want that journey to begin.

I still don’t have an idea about how much longer he will hang on. I emailed Dr. S (ped) and flat-out asked if she has any idea of a time frame that she could give me. It would be helpful to know if this is a days versus weeks type of situation.

Joshua ate a little more today than he has in the previous days, but the total number of calories he took in totaled about 300. He drank about 6 ounces of fluid. He wanted me to take him on a walk around the block, just the two of us, and he shared things that were important to him as we went. He told me “The angels are so shiny & bright that you can’t really see them here, but you’ll see them in heaven,” and how “God loves us so much He is letting us live in His home forever.” He said he is ready to go Home and clarified that he meant heaven, not our house. He said that when the angels come for him, he is going to go with them and then make sure they come back for me. He pointed to a flower (weed) he wanted for his collection and the blue lid to a milk container that was lying on the ground that he wanted “because it is one of my favorite colors.” He spoke to me of his love for me, telling me I have been a very good mommy… the best mommy in the whole world… and that he loves me forever. He asked if I remembered the times we cuggled (cuddled) and I said I did and he told me he loves to cuggle with me because I am the best mommy and when I told him I love to cuggle with him, too, because he is the best Joshie in the world, he responded that he knows he is the cutest Joshie in the world (I did not disagree with him on that). He told me his leg likes to cuggle with my knee (he drapes one leg over mine at night as he is falling asleep… how will I go to sleep without that??? Oh, there are a million things I am going to miss about this little boy!).

In the evening, he asked if Adam & I could take him out for a walk so he could tell us something important. As we walked, Joshua was quiet at first, so I asked, “What did you want to tell us?” He looked at Adam and said, “I need to tell you that I love you. I love you SO MUCH. I will love you forever, Adam.” Then he told me he needed to tell me the same thing… he loves me SO MUCH. Forever. Both Adam & I assured him we felt the exact same way. We talked about other things… silly little things like what colors Joshua likes and we tried to find leaves in those colors. He found a white rock and a red one and I picked them up and handed them over to be added to the collection of ‘nature things’. We talked about heaven and God and angels. We spoke of love and how much we mean to each other and how that will never change. It was a light-hearted, yet intensely serious, walk as Joshua seemed to be settling things in his head and tying up loose ends by making sure he said all the little things he wanted to say. Adam videotaped the entire 25 minute walk. I am so grateful he did.

Joshie wants me to get a mine so that I can get him some gold so that he can make a trophy for Megan and he wants to write on it, “You are the best sister in the world.” He wants to try going through a car wash (he doesn’t remember ever doing it, though he has). He asked to go to the mini mart yesterday to try a Laffy Taffy because he never has had one (actually, he HAS… he loves them… he just didn’t remember). It’s odd how he randomly thinks up things he wants to do. I indulge every whim that I can. It is enough to make him happy.

He is still pushing Emily, David, Sarah & Isaac away more often than not and I don’t know why. Please pray that he will soften and let them love on him. Pray, too, that Joshua will respond and love them back. He has always adored his siblings and Isaac has been his best friend. It is breaking their hearts to be pushed away at this time, especially when they know Joshua won’t be here for a lot longer. Please pray that he will be able to connect in a way that is meaningful for Emily, David, Sarah & Isaac before he dies. They need at least one more happy moment with their baby brother and to hear him say, “I love you.”

Tears are filling my eyes as I type and feel my little boy’s leg pressed against mine and glance over to watch him sleeping. I don’t want him to suffer anymore, but I don’t want this time with him to end. When the angels come to take him to heaven, I will not beg him to stay (though I will desperately want to), but I am never going to be ready for this. I have said I’m as ready as I will ever be, but that doesn’t mean anything when it’s a situation no mom can ever be ready for.

Make A Wish

Someone on topix had asked about Kate talking about Make A Wish on CaringBridge. This is all the Make A Wish things I could find.


Monday, December 27, 2010 12:06 AM, PST

Things haven’t gotten better. In fact, they’ve gotten worse. Joshua is having episodes of low oxygen levels evidenced by his lips turning purplish-blue in color, the skin around his mouth and over the bridge of his nose turning a dusky blue-grey and formation of dark purple circles under his eyes during “episodes”. He also gets extremely tired & develops a headache. After I told our pediatrician about what we were seeing at home, she loaned us a pulse oximeter to use over the holiday weekend. I picked it up on Friday and began actively paying attention to see when Joshua’s color was “off” so I could hook him up to the pulse ox and measure his O2 saturation level. He had readings down to 82-86% and many episodes where his O2 was 92-94% (normal is 95-100%). What does this mean, exactly, and what will we do about it? I don’t know yet. I emailed the results to the pediatrician tonight and will wait to hear from her tomorrow or Tuesday (she’s out of the office on Mondays).

I never heard back from Dr. W’s assistant, which is a first since they typically call back within a day of my leaving a message on the P.A.’s voicemail. I don’t know if that means there’s nothing they can do (which is what I expected) or what. Either way, their silence is not terribly reassuring to me.

I was informed by our ped’s nurse that the ped would be happy to fill out the paperwork to apply/nominate/refer (whatever the appropriate word is) Joshua for Make-A-Wish. I asked if he actually qualified for that program since the primary eligibility requirement is the child must have a life-threatening illness/condition, and I admit I was shocked when the nurse quietly nodded her head. I still am struggling to wrap my brain around the idea that he doesn’t have a prognosis for a normal life expectancy. This is one more “new normal” that I never saw coming & it’s the hardest one for me to accept, by far.

Joshua has a new prescription for pain medication ~ Lortab ~ and that is working better for him than tylenol with codeine was. He’s still not tolerating bolus feeds, so he’s back on a continuous feed of 2 ounces per hour for 18 hours a day & he’s doing well with that, which is something I’m thankful for. His weight was down to 37.5 pounds last Thursday, so we’re hoping to get a few pounds back on him & having him NOT puking is a good start to achieving that goal. He dropped from the 75th percentile in weight to the 25th percentile in about 6 weeks, which is concerning since he’s above the 75th percentile for height (44 inches). He’ll see his physical therapist tomorrow to assess his foot positioning & gait and perhaps determine whether he needs to be braced again (I don’t know if she’ll make that decision tomorrow or not), and I’ll be calling the ophthalmologist to make an appointment so the double vision/fuzziness that Joshua is experiencing can be evaluated. While it’s suspected to be “central,” we’re hoping maybe we’re wrong and the ophthal can do something to help.

It is heartbreaking for me to realize that I subjected my son to a surgery that did nothing to help him and that we’re now at a place where no one really knows what to do.

“Fixing” him seems to be the impossible dream and that’s a hard reality to accept.

Thursday, February 24, 2011 5:08 PM, PST

Joshua has had increasing headache pain that isn’t controlled by the combination of Lortab on top of his Neurontin. I called our ped yesterday for advice & she asked me to contact the neurologist & neurosurgeon, so I put calls in to the nurses of both of those doctors. Dr. W (neurosurgeon) said there was nothing she could do at this point since it hasn’t been long enough since she last went into Joshua’s head (which I knew… the first thing I told Dr. W’s nurse was that I was not asking Dr. W to *do* anything & I had zero expectation that she could help at this point, but I was advised to call & give her a ‘head’s up’ about how Joshua was doing). I asked Andrea (Dr. W’s nurse) if we could increase Joshua’s dosage of Neurontin & she came back with the information that Dr. W wanted that order to come from neurology rather than her since it was that specialty that put Joshua on the medication in the first place. Soooo… Andrea played phone tag with Lisa, Dr. H’s nurse, & the end result was Andrea calling me at 6 o’clock last night to tell me that Dr. H had left without checking her messages & she (Andrea) didn’t have a dosage increase yet. Sigh. Andrea assured me she would call today to give me the new dosing information, though, and after I thanked her for calling & not leaving me hanging, we hung up.

This morning, I headed out with Joshua, Bethany & Hannah for therapy appointments (B & H), a port flush (J) & labs (J). When I got home at 2pm, I asked Charley if Andrea had called yet & was surprised when he answered that she hadn’t. I called the neurosurgeon’s office & got their answering service telling me the office was closed due to inclement weather. Apparently the storm that our weatherman had predicted would drop 6-10 inches of snow ended up in Seattle & Portland, instead. Bottom line? I’m still waiting to find out how much to increase Joshua’s medication as we try to manage his pain in other ways.

Two “Wish Granters” from the Make A Wish foundation came to our house this past weekend to talk to Joshua about his wish. We’re waiting to hear if the corporate MAW agrees to grant the wish & once we know for sure, I’ll share what Joshie wished for. The whole MAW experience is an emotional one. It’s really fun & exciting to think about being able to do something for Joshua that we would not have been able to do without MAW, but it’s painful to know that the reason he’s receiving a wish is that he has a life-threatening condition. Yes, we know there are LOTS of kids who have had a wish granted through MAW & then gone on to live into adulthood, but even more children have not. Life-threatening means exactly that. Whether or not Joshua lives into adulthood remains to be seen; all we can see right now is the day-to-day stuff, which isn’t very encouraging, and as such, MAW is a double-edged sword from an emotional point of view for my husband & me. That said, we do believe seeing Joshua’s wish granted will be a wonderful experience for him & the rest of our family & we are looking forward to it.

Monday, April 25, 2011 5:11 PM, PDT

Surgery is over and went as well as it could. Dr. W said the inside of Joshua’s head was a mess, with regrown bone and a bunch of scar tissue everywhere. She cauterized his cerebellar tonsil way up and off his brain stem, which should help him. I am relieved surgery is over… now to get him recovered and out of here so we can get ready for his Make A Wish trip onJune!

I will update more after I get to see my boy. Thanks for the prayers and well-wishes.

Monday, May 2, 2011 12:50 PM, PDT

Joshua is essentially recovered from surgery. The only changes I’ve seen are that he now has apnea episodes while lying down, which is not good, but he can pop himself out of them without intervention, which is good. He’s having pretty intense headaches, too ~ severe enough to make him vomit. I don’t think it helped that the neurosurgeon decided at 5:05pm on Friday that it was time to wean Joshua from his PCA pump (continuous fentanyl) but didn’t bother to get any other form of pain relief arranged. She did say she’d call pain management, but again, at just after 5pm on a Friday, it wasn’t like anyone was going to come on the weekend. And they didn’t. Which meant Joshua had some rough patches on Saturday & Sunday. Which resulted in my increasing frustration because I don’t like seeing my child in pain & completely miserable. As of now, 1pm Monday afternoon, we’re still waiting to see if anyone from pain management shows up. He was weighed last night & we were surprised to realize he’s lost 2.3 kg (a little over 5 pounds) in the past 17 days. Yipes. I knew he wasn’t eating or drinking much of anything, but it didn’t occur to me that he might be losing a significant amount of weight. We’ll be starting tube feeds sometime today, but again, it’s a matter of having to wait for a doctor to order formula for Joshua & who knows how long that will take?

I think it’s time for him to be moved off the neurosurgeon’s service & back to the pediatric service. When Joshua was first flown up here 2.5 weeks ago, he was admitted on the pediatric service, which meant he saw a pediatrician every day & they took care of whatever need came up. Last Monday, when he had surgery, he got moved to the neurosurgical service, which made sense. But now that he’s a week out from surgery & there isn’t really anything more that Dr. W can do, I am thinking maybe he should be switched back to peds so that things like phone calls to the pain management doctor & formula for tube feeds can be taken care of right away versus at the end of the day when the neurosurgeon gets done with all of her surgical cases for the day.

Joshua still can’t sit up for more than a few seconds, but I’m not sure if that is related to surgery (low-pressure headache, perhaps) or the generalized headache pain he has. Truth is, I don’t know why he’s acting the way he is. All I really know is that his Make A Wish trip is coming up & I would like him to be able to enjoy himself, which isn’t going to happen if he’s hurting. As such, I want the pain management doctor to figure something out soon & get things underway so that Joshua can feel better & then we can get out of here.

Friday, May 13, 2011 6:25 PM, PDT

Joshua was discharged from the hospital in the late afternoon & we are now at the Ronald McDonald House until tomorrow, when we will leave to drive home! Yeaaaa!!!!!

I was taught how to mix his TPN & inject medications into it before hooking him up to it. I had a friend ask me what the difference was between TPN & gtube feeds, so here’s my ‘layman’s explanation’: formula that gets pumped through the g-tube goes into the stomach, where enzymes & gastric juices begin the digestion process, breaking food into smaller bits before it moves to the small intestine. Once in the intestine, the partially-digested food gets further broken down into basic substances ~ protein, carbs, fats, vitamins, minerals ~ and those get absorbed into the bloodstream, where they can be used by the body for energy & growth. TPN is a mixture of the basic substances that gets pumped through a central line into the veins to be used by the body for energy & growth. TPN bypasses the first stage of digestion completely. Joshua’s TPN will infuse for 16 hours each day, running from 5pm to 9am (or thereabouts). I’m happy that most of his waking hours will not be spent hooked up the pump.

All things considered, Joshua is doing great! If you compare him to a typical (ie: normal) 5 year old, then his being in a wheelchair, unable to walk right now (too weak after a month lying in bed), throwing up roughly 1/2 of what he ingests, being fed through a central line 16 hours a day, & needing a fentanyl patch & lortab after having a nerve block to control his pain probably doesn’t sound so wonderful. But when you consider that one month ago, he was admitted to the hospital in acute respiratory failure & right now, he’s not needing to be on oxygen, not needing continuous monitoring via pulse ox, and he’s not having episodes where he doesn’t breathe as long as we do not let him lay on his stomach (and yep, we have doctor’s orders saying he is not to lay on his stomach or hyperextend his head backward), that is pretty darn fantastic! I learned years ago to not compare Joshua to his peers because it results in my feeling sad; as such, I can feel sincerely happy that he’s doing so well compared to where he was a short time ago.

I have no idea what the future holds, aside from a trip to Give Kids The World through the generosity of the Make A Wish Foundation (yippee!), but as of tonight, Joshua is in the best shape possible to face whatever comes his way, which makes me one happy mama. I am so thankful for the doctors & nurses at Legacy Emanuel who worked so hard to get Joshua to this stable place ~ they are incredible & I hope I was able to adequately convey to them how VERY much they are appreciated. God has truly blessed us with a superb medical team to take care of my little guy & I feel so fortunate that they care so much about him. As we hugged nurses & thanked doctors before leaving today, it was with a measure of sadness because even though we’d rather not be in the hospital, saying ‘goodbye’ means we won’t see the people who have become like a second family to us for (hopefully) a long time.

Maybe it seems crazy to care so much about hospital personnel, but I don’t know how a parent avoids it when these hard-working people make such a profound difference in a child’s life. I’ll always have a soft spot for our medical team at Legacy Emanuel. They’re the best!

Saturday, June 11, 2011 5:38 PM, PDT

Today was a very special day ~ Make A Wish granted Joshua’s wish by giving him a game room! The original plan had been to send our family to Give Kids The World in Florida, but when Joshua was admitted to the hospital just 8 days after being discharged from Portland, his doctors deemed him not stable enough to fly across the country. It was just as well, since the trip was supposed to take place June 1st – 7th & Joshua was in the hospital until June 5th. Once we knew Joshua couldn’t go to Florida, we thought about what he could still do & what he really enjoyed and came up with an alternate wish for him.

At noon today, I took Adam, David, Sarah, Isaac & Joshua out for two hours so that the Wish Granters (Amanda & Michael) could come in & set up a 42″ flat screen television, DVD player, Wii with 4 controllers, drums & guitar (for the Lego Rock Band game) & 4 gaming chairs in what is now our game room (was the kids’ computer/play room). When we came home at 2pm, there were bunches of “Make A Wish” helium balloons at the end of our driveway & on our porch, welcoming Joshua and everyone who came to his party to celebrate his wish. He was happy to see his Grandma & Grandpa, our pastor & his wife, his beloved physical therapist & occupational therapist, one of his honorary Aunts & her son, the fabulous Wish Granters & our family, but his attention was primarily focused on the large pile of gifts sitting in the corner of our living room (we kept him from the game room until he’d opened the presents). 🙂 He had a blast tearing into the gifts, opening each one &periodically asking, “Why am I getting all of this Wii stuff? We don’t have a Wii!” After he’d opened the last gift, the Wish Granters led Joshua into the game room, where he saw the huge tv & the Wii and immediately hustled out of the room to go retrieve all of the Wii games & other paraphernalia he’d just opened. Once he returned, he couldn’t contain his excitement & was basically bouncing around, laughing & saying, “Ya hoo! We have a Wii!” He’s been in there ever since, taking only a short break to blow out the candles on his SpongeBob cake (we sang, “Happy Make A Wish Day to you,” instead of “Happy Birthday) & eat a little of the dessert (with ice cream, of course).

It was a magical day for our family. To see Joshua SO happy & having SO much fun as he was surrounded by people who love him & have been an important part of his life… well… I don’t have words to tell you how good it felt, but it really was special. No, it wasn’t a week-long trip to Florida, but in the end, I think this wish is even better, because playing games & watching movies are two activities that Joshua does every day & both make him happy. He will be able to spend time interacting with his siblings & memories will be made for so much longer than just one week, which completely warms my heart.

Friday, December 30, 2011 7:15 PM, PST

This is the medication it takes to keep Joshua “stable” for 36 hours. I drew them up for our trip to Portland. These will cover him for today & tomorrow until 4pm. Adam, Joshua & I drove to Portland today for Joshie’s photo shoot for the Community Hero exhibit. It was rainy most of the way up, but traffic wasn’t too bad. Joshua slept 90% of the way. We had to wake him when we arrived at the photo studio. Thankfully, we were 45 minutes early, because it took Adam & me a good 20 minutes to get Joshua awake & ready to go inside. He wanted to continue his nap!

The photo shoot went very well. Michael, the photographer, was amazing with Joshie & some of the pictures he showed me were tear-inducing. I told him, “Stop showing me these! You’re going to make me cry!” He gave me a side-hug & said, “Good! I want them to have that effect on everyone!” Joshua had a lot of serious pictures & then Michael got him giggling & smiling. Interestingly, the pictures the photographer preferred were the serious ones because he said Joshua has extremely expressive eyes & while he has a fantastic smile that lights up the room, his eyes disappear when he grins. He wants the exhibit picture to be one that shows Joshie’s contemplative side with his eyes open. I told him I’m sure whatever picture is chosen will be beautiful. He asked if we’d driven up from southern Oregon just to do the photo shoot & when Adam & I told him, “Yep!” he seemed shocked, then told us, “I should tap-dance for you or something to make it worth your while!” I told him that the pictures would make it worth our while. He ended up doing a 45 minute shoot instead of 30 minutes since we’d arrived early and he took extra pictures of Joshua with both Adam & me and some of Joshua with Adam & me separately. I’ll receive a CD of all the pictures taken except for the one that will be used in the exhibit, so I won’t know what picture they chose until the exhibit is unveiled at the end of May. I can’t tell you how valuable that CD will be to me. I am SO glad we got to do this. It’s an honor for Joshua to have been chosen as one of the 25 Heroes, and to be able to “memorialize” him in this way is so meaningful to me.

The CCA representative who was at the photo shoot asked me if Joshua had been granted a wish through “Make A Wish”. I told her he had & described what they’d done for my little guy. She said that was great & then told me that her organization wanted to offer Joshua a wish, too. I was speechless! The woman asked Joshua what he wanted if he could have anything & he very enthusiastically replied, “A laptop! I’m saving my money for one!” She smiled & told him, “We can do that!” Then she looked at Adam & me and asked, “Is there any specific kind you want for him?” We gave her some preferences & she jotted down notes, then told me she’d be in touch about getting that to Joshua. So… wow! I was completely shocked. The kindness of people never ceases to touch my heart, especially when it’s kindness aimed at Joshie. After the photos were taken, the boys & I headed out of town toward home. We drove 1.5 hours (Joshua slept for an hour), then checked into a motel for the night. And that is where we are right now. Joshua is very, very tired. The day’s activity has wiped him out. He is cozy & warm under two of his fleece blankets as he finishes up a tube feed & dozes on the bed beside me. Actually, now that I look over at him, he’s sleeping again. It’s sad that a day like today, where all he did was ride in the car & attend a photo shoot, could exhaust him the way it has, but that’s the reality of life. Tonight I am choosing to be happy that he was able to enjoy himself for those 45 minutes & that we were able to make this trip at all.

Let Others Think She Had Lost A Kid

This was on MWOP! She totally let everyone believe she had “lost” one of the kids. Instead of saying we gave one of the girls away she just didn’t say anything even after other told her they were sorry for the loss.

A lot of MWOP did know about her situation. She even had a button for the adoption at one time on MWOP.

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