It's been a long time...

Since we've received news this liberating and wonderful.

Owen's infantile spasms (seizures) are "on their way out," according to O's neurologist and a fellow neurologist who also did a read of the EEG. They only saw a "couple" of seizures. Two. I carefully took notes, indicated times, gave detailed descriptions of Owen's "suspicious" posturing. I pushed the button. None are seizures. None of them. I feel as though I could both laugh and cry and I said that exact thing to the two neurologists who gave us the good news.

Trust me, the infantile spasms, as everything we read about them in October of 2007 indicated, have left their mark. At the time, Owen was having over a hundred seizures a day. Children with IS typically lose any abilities they had prior to these seizures (such as head control in Owen's case - I still torture myself by looking at video of him, on his belly, holding his head up) and gain very little in terms of developmental milestones. This also does not mean that Owen is not at risk for future seizures - new and different. He is. But the diet worked - it reduced his seizures to practically nothing and kept new ones at bay, for the most part. If the newest research on the Ketogenic Diet proves consistent and accurate, it may have even initiated permanent changes in Owen's brain, making him less susceptible to seizures as he grows older. Now, we get to slowly and carefully, under the guidance of a nutritionist here at Swedish, over the course of the next year, wean Owen off of the diet and slowly introduce whole foods. 

Owen's neurologist suspects involuntary muscle spasms (brought on by increased spasticity) are one of the causes behind his wakefullness and difficulty falling asleep and is going to suggest doubling the dose of baclofen (he indicated that we're using a "tiny" dose right now).

The other probable cause of Owen's lack of sleep... sleep apnea. His "slack tone" likely also creates an airway restriction problem We'll be meeting with the sleep specialist to discuss the results of the sleep study and talk about sleep positioning and a potential tonsil and adenoid removal to open up Owen's airway (a fairly minor procedure).

The neurologist attributed the rest of the arching and sleeplessness to "irritability." Actually, it's more so the case that he's stubborn, strong and opinionated and he's not afraid to let us know what he thinks... And this, I will celebrate.

The sun is up! Well... almost.

I'm looking out from the 9th floor at Swedish, across the east side of First Hill and out over Cherry Hill. It's a lovely view, classic Seattle, although the way the fog is settled in, between the houses and buildings, it looks like a cold day in late October. But judging by the people I see, walking the streets below, it is still a relatively warm day in September. This is how one gauges the temperature from inside a hospital room...

I just recevied my A La Carte breakfast from the cafeteria. Scrambled eggs, toast, bacon (!) and a LARGE coffee. Owen slept from 9:45 pm until 12:45 am last night and then from 5:20 am until 7:45 am. This is how it's been since late July.

Our neurologist is due for our in-room consult at 11:00 am to discuss the findings and, The Plan. Hopefully (please, please, please), the EEG technician will arrive around noon or so to start to wind things down and begin the hour-long process of removing the leads. My hope is that we are on our way home by 1:00. It never seems to go that quickly or smoothly, so I'll be elated if I can eat dinner, tonight, at home with my family.

Since the neurologist can view the EEG remotely, and does so throughout the twenty-four hour period, he has a good idea of what things look like - whether he needs more data or if we can be set free soon. We have been able to capture two of the "acute pain episodes," so will know definitively if these are seizures. This is a very good thing. 

The other possibility is that we stay another night. Long story, but we requested in June that a sleep study be done in conjunction with the 24-hr EEG as Owen's sleep is so poor and we absolutely must try to help him sleep better - so that he can learn and develop to his potential. My sense from our night and the data that was collected (the sleep study technician stays in the room all night to assess and fine tune the data and was forthcoming about what he saw) is that Owen may have sleep apnea - either induced by seizures or the one of the underlying causes of his seizures. We suspected this. 

Strange as it may sound, it is exciting to have all of this data - practically every square inch of Owen's body was covered in leads, sensors, and monitors assessing his breathing, body movements, fluctuations in muscle tone, respiratory functions, the data from all of which will be used in conjunction with the EEG tracings to create a picture of what his body and mind are doing and how this is affecting his sleep and seizure activity. I have developed a love-hate relationship with all of this medical technology. Beeping, bright lights, internal fans cooling computer processors... Regardless of my emotions, I also appreciate this technology and most oftentimes, it makes Owen's life better and his experience, clearer. What IS clear, and now confirmed, is that he's having increased seizures (the horrid and insidious infantile spasms) as he falls asleep. We noticed this increase a few weeks ago, some days the increase more pronounced than others.

So the combined studies should tell us why he's so wakeful. Pain? Seizures? Sleep apnea? Uncontrolled muscle movements? 

Then we make The Plan.

SkeletomuscularScoliosisFlexorsHamstrings... Oh my!

I should be napping right now. If I was able to nap (I've never really been able to sleep when it's light outside), I'm sure I'd be dreaming about warm, sandy beaches. It is a chilly, damp, cloudy sixty degrees. I have never, in my thirty-eight years, fifteen of them spent in the Pacific Northwest, experienced a Six Week Summer. I'm not a big fan.

So, since the boys are sleeping and my pineal gland is really the one in charge, I should probably consider relaxing with my headphones and new agey music. Giving myself quiet time, although sometimes difficult and, lately, almost impossible, is always - always, always - beneficial when the weight of navigating medical issues and clinic hallways seems overwhelming.

Instead. I'm blogging.

Owen is still in pain. A short break, three or four days, late last week when he seemed to be more at peace. That's all he got.

The AFOs (foot orthotics) helped to stop the spasming in his feet. The baclofen hasn't done anything just yet, but did improve his sleep for a few days. We're not quite at our target dose, but if this is the pharmaceutical solution to Owen's larger pain issues, I'll be very surprised. I do, however, think it is a good choice for long-term spasticity management.

Owen is back to only sleeping a couple of hours a night. He still has episodes of acute pain when in his stroller and is just generally, very, very uncomfortable. He arches in my arms, arches in bed, arches in his stroller. And cries. And cries. Those who know Owen, understand the significance of me talking about these behaviors outside of their normal context. The intensity, consistency, and tone are all very different. Cuddling or relaxing in his bed, listening to HIS new agey music, no longer works much of the time. Once again, the medications we have for calming him, are not working.

There is an underlying issue that we've not discovered yet. I know this. Whether or not this process of discovery results in insanity, remains to be seen, but some nights at two o'clock in the morning it feels like a real possibility. Fortunately, then, the sun comes up and for that, I am grateful.

We are now seven weeks out from when Owen became increasingly trapped inside a body that hurts and does not work.

So I have a (another) call into the orthopedics clinic at Seattle Children's Hospital. We are running out of possibilities but my hope is that we're finally getting closer to understanding what is going on since we've ruled out almost every other possibility that exists. It seems as though there are multiple issues, probably all brought on by increased spasticity and as a result of Owen's cerebral palsy. I've been spending quite a bit of time the last few days reading about skeletomuscular complications that occur as a result of cerebral palsy. Stuff I should already know intimately, but that's not "easy reading" in the evening when there's no acute issue and you just want some good fiction.

1. Right hip? Owen was diagnosed with mild subluxation of his right hip two years ago. Dr. W. in the orthopedic clinic two weeks ago was concerned about this hip, and after a thoughtful review of Owen's films from this August, suggested additional x-rays and bracing. In a subsequent follow-up visit with the orthopedic surgeon, Owen's hip was given secondary status, to the curvature of his spine and the growing rod surgery. No one is right or wrong - it's a tough issue. I was encouraged to "feel" the difference in this right hip, compared to his left hip, when at Children's Therapy Center last week (see below), while Owen was seated in his chair. Left hip - set within the socket and the musculature. The right hip - a bony protrusion. The PT there strongly believes that his hip might be the cause of significant discomfort, especially when seated. (The x-ray above is NOT Owen's but does illustrate what hip dysplasia looks like in a child.)

2. Tight hamstrings (hamstring contractures)? This is also a very common problem in children with spastic quadriplegia CP and can be both painful - even more so when the contractures are significant, the muscles are shortened (both due to CP) and when the child is in a seated position.

The hip flexors are connected to the hamstrings are connected to the calves are connected to the feet. And all of this, connected to the spine. Of course, then there is the neurology behind it all... Throw in spasticity and hypotonicity and you've got serious potential problems (or really good band names).

We decided to follow through with a recommendation we received in February to see the wonderful, compassionate, talented folks at Children's Therapy Center. There, on Wednesday, Owen was measured and casted for a TLSO (thoracolumbosacral orthosis) and a SPIO suit (the one generously given to us by our friends, the Leemans, does not fit Owen anymore). We are choosing this direction, external bracing, with the hope that we can keep Owen flexible, keep him "moving," and, when not actively moving or exercising, can keep him straight. Then, in his early teenage years, spinal fusion will most likely be necessary to stabilize the spine once it has stopped growing (around 11 or 12 years of age). We've been told by the surgeons that external bracing does not work. They may be correct, but we will explore this route first, with a careful eye to any increasing curvature.

Owen and I will be in for a two-day, inpatient stay at Swedish Medical Center (Seattle) on Monday and Tuesday. We scheduled a VEEG (24-hour video EEG) and a sleep study three months ago, for the 13th and 14th of September, to establish a baseline prior to Owen coming off of the ketogenic diet. Should he have any increased seizures as we wean him off of the diet, we'll have a "pre-wean" EEG to better understand any possible correlation. Parents are required to remain in the room at all times, 24/7, since monitoring is part of the process (I need to push a big, red button should he have any questionable episodes that might resemble seizures - no pressure, right?). Owen and I have been away so much these last few weeks and these stays are so far from home and far away from my two other guys, it's hard for me to think about doing this in just three days. Please feel free to send some cheer our way early next week. I'll be on Facebook, have my phone, access to email... I can even send out our room number if any locals want to join us for an all-nighter. Just kidding. I've got a button to push...

Xo,

S/K/O/E

A Lesson in Spasticity.

It was all about uncontrolled spasticity.

We have seen an increase in Owen's spasticity over the course of the last several months, mostly during periods where he was very uncomfortable - with illness, fatigue, constipation, teething, and reflux from the high fat ketogenic diet. We are fortunate to have a long-standing, wonderful relationship with Dr. M., an expert in spasticity management at UW/Seattle Children's Hospital, and this spasticity had been managed pretty nicely by him over the course of the last two and a half years with Diazepam. What we realize now, is that the pneumonia and GI distress quickly and acutely escalated Owen's spasticity to a level that was both painful and severely distressing to him. We now have an intimate understanding of how this type of muscle spasm/disfunction becomes both increasingly worse and more frequent during an acute phase like this when left untreated. We also learned that spasms in the feet - due to the number and complexity of muscles, tendons, etc - are especially painful. What Owen was experiencing was similar to a prolonged, intense charlie horse, most likely in both his calves and feet.

This is the issue that we suspected was at the core of Owen's discomfort, but not having experienced this type of acute escalation, and all of this on the heels of Owen having pneumonia AND the GI issues AND being on the ketogenic diet (which has it's own pretty serious, potential risks) AND being prone to seizures, it was absolutely imperative that we rule everything else out.

Botox injections are not an option for Owen's spasticity as they are really only effective in cases where there is very specific areas of spasticity. In Owen's case, they would have a difficult time determining whether to use the treatment in his hamstrings, calves or feet. And with "generalized dystonia/spasticity," Owen will probably never be a good candidate for botox both because of the nature of its (specific) application and the fact that it needs to be repeated on a regular basis. But never say never, right? So, Owen started oral baclofen three days ago. While oral baclofen does have side effects (which is why we've held off using it), we've been assured that it is "perfectly suited" to children like Owen (keeping in mind that no pharmaceutical is perfect) and the best thing for him right now. It will decrease the spasticity in his entire body but once he's adjusted to his dose, won't sedate him. His body will be free and his mind available. That is our hope.

In addition to baclofen, Owen was fitted yesterday for foot orthotics which will keep his feet in the neutral position. We have been trying out his "old" orthotics the last two days, which are much too small for him now, to see what effect they would have. They don't decrease the spasticity, which is to be expected, they do eliminate the extension of his feet into the downward flexed position. This has completely stopped the "episodes" of pain. The new orthotics will arrive later this week.

We were seen in the orthopedics clinic at Seattle Children's Hospital yesterday for Owen's spine and hip consult. The final read of Owen's spinal x-rays, the one from Fall 2008 being compared to the one from August 2010, indicates that his curve has increased from 41 degrees to 48 degrees. A decent increase, but certainly not terrible given the circumstances. The curve is in the lumbar region of the spine (curves in different locations affect different functions and organs). Growing rod surgery is most likely in his future, but we're not willing to say, right now, that it is a definite. But, thankfully, this procedure does exist. There are some risks, including fracture of the ribs where the upper screws and hooks attach, and dislocation and breakage of the rods. We will decrease the likihood of these happening if Owen's spasticity is better managed. MUCH better managed. It is obviously a long and complex surgery and they also prefer to see kids at a healthy weight in order to prevent any issues while under anethesia and to prevent infections. Plumper kids do better. Owen is far from plump. They indicated that he also has a good deal of flexibility in the area of the curve and if we are able to maintain this, keep his curve from worsening and maintain his very healthy pulmonary state, then there is no urgency to move forward with the growing rod procedure. These are challenging things to achieve in a child with no head or trunk control. What is most at-risk for Owen are his internal organs and the effectiveness of his pulmonary system - both of which can be compromised when scoliosis becomes severe. As a child with unsupported/untreated, significant scoliosis grows larger and heavier, the weight of the unsupported child compresses the organs and lungs. So we will keep on with stretching exercises, keep on with feldenkrais, keep on with cranial-sacral appointments, and with regular warm baths (he loves these so much, and relaxes so much, that a jacuzzi tub may be in our future - not a bad thing for ANY of us) and massage, massage, massage... Owen will have orthopedic follow-up appointments every six months and, in addition to the one we have scheduled in March, he will have a CT of the spine (under general anesthesia) to determine if, at that time, there is any deformity of the vertebrae which would make growing rods a more urgent issue.

After repeated blood tests, ultrasounds, several x-rays, numerous exams, consults, ER visits, in-patient stays and countless hours on the internet, what we have learned, and what is not at all surprising to us, is that Owen is actually very healthy. Aside from needing to gain significant weight (which we've been trying to advocate for for months now - calories are being limited by the ketogenic diet), and, of course, the curve of his spine, he's a sturdy little guy. He really is amazing.



I realized when I was forced to give up Owen's blog for several months earlier this year, that I blog not only for him, but for us, for our family, friends and for all of the families that we stay connected with. I always receive such valuable input and feedback when we go through these issues that are new to us - we feel like we're not going it alone. And we're not just receiving feedback and ideas from other parents with SN kids - which is always valuable in a very specific way - but also from so many of our friends and family members. We are so grateful for all of the support we've received these last few weeks. It makes such a huge difference. I only hope that these LONG posts, and the focus on Owen's life and complexities (as a family, we do tend to be pretty private people so this isn't always a comfortable process for me), assist other families in feeling more comfortable reaching out and finding solutions.

So now. We relax for a few days. Maybe even an entire week. Time at home. I need to get my hair cut. Elliot needs his hair cut. I need a massage. Elliot starts preschool, a Parent and Child Program, at Kingston Co-Op Preschool, on September 17th. Owen starts communication and motor therapy at Cascade Children Therapy on September 21st. I'm even more excited about this program now, knowing that Owen's spasticity will be better managed and his mind more relaxed and available (fingers crossed!). He and I will be in Seattle (Mill Creek) for his "school" every Tuesday.

Elliot and I read one of our current favorite stories this morning to Owen. Owen smiled and smiled. He is feeling better and, just as quickly as life became difficult, it has become easier.

We leave you with a little Moo Ba La La La.

S/K/O/E

Letter.

I just emailed a letter to the neurodevelopmental pediatrician who we adore and who we've worked closely with at Seattle Children's the last three years. This letter explains what we are certain it is that has been causing Owen's discomfort. I'm going to paste it below.

Tomorrow is our consult for Owen's hips and scoliosis and hence, our conversation with the orthopedic surgeon about the "growing rods." Will keep you posted...

Thank you - everyone - for your support via email phone, in person, via Facebook, Owen's blog... We've had offers of hugs, housing, food, hugs... It means so much. It gives us strength.

----


Dr. M.,

It seems as though, through various clinic visits, home observations, videos, ER visits (the first on August 16th, and a second one yesterday), in-patient stays, etc. over the course of the last three weeks, we have finally determined what lies at the heart of Owen’s overall discomfort AND his acute episodes of pain.

We are in absolute agreement that Owen’s spasticity has increased and needs to be better managed. This increase in tone, especially in his legs and feet, has been developing over the course of the last year and his recent pneumonia/GI discomfort, in late July, created a shift from what had been variable tone to consistent high tone (again, mostly in his legs and feet), leading to a great deal of physical distress.

We are also certain that Owen has experienced an acute stress injury in his left foot, as a result of the almost constant downward flexing of that foot during these periods of high tone and flexion. His left foot has consistently, for the last two years, been the point of initiation for his full-body extension, especially during periods of distress (when sick, when receiving his food, when tired). We have witnessed now, over the course of the last four days (after an intense episode late Friday night), that full, strong flexion of this left foot causes pain, which causes increased tone which then causes increased pain, and higher tone until he experiences a great deal of pain in that foot and has” the episode” of being “paralyzed” with pain. The “episodes” that we’ve been seeing for three weeks now. During this acute episode, his foot “locks” in this flexed position. Breaking the extension by flexing him at the knee seems to help some of the time, although there are times when he is so stiff and extended, that we cannot even flex him at the knee. If we are able to bend him at the knee and break this pattern, his left foot then quivers, slightly flexing upwards and downwards until it finally “quiets.” His loud screams shift to constant crying. His left foot, for minutes and sometimes hours following these episodes (the prolonged one he experienced on Friday night lasted 30 minutes and almost prompted me to call 911), is incredibly sensitive to the touch – especially at the arch and the lower part of his heel. You are obviously intimately aware of the fact that Owen has absolutely no control of these episodes. They are terribly distressing to him. As I’ve mentioned, these episodes are now occurring even when he is in a good mood, alert and well-rested.

I have a couple of questions that I would like to address, as soon as possible, to make Owen more comfortable and to avoid any additional injuries and pain. We do also have an appointment with you on November 10th, so will obviously follow-up on these issues, and current interventions, at that time as well. As I’ve also mentioned, we’ve been unable to get him out much of the time the last three weeks due to his discomfort and the unpredictability of these acute episodes. This has impacted Owen, and our entire family, to an enormous degree.

1. The increase in Diazepam, which we have been using in conjunction with you for two years to manage Owen’s spasticity, DOES indeed help to alleviate the acute episodes where Owen becomes stiff with pain after repeated flexing of his left foot. Unfortunately, though, it also heavily sedates him. It has been necessary to administer upwards of the additional 2 mL that you suggested last Tuesday (your suggestion was to use as little as possible, but as much as needed, of that 2 mL),the last couple of days, to avoid these acute pain/left foot extension episodes (in addition to the 5 mL used previously for spasticity management). The administration of this additional dose is oftentimes necessary even when his overall demeanor is calm and he is well-rested and happy. This is distressing to Owen, to myself, to other members of his family as he is mush of the time, happy and calm otherwise. That said, the Diazepam dose stops the flexing of his foot. Once the additional Diazepam is administered, Owen is then frustrated at being over-sedated, and in a state where he loses what clarity he does have, cannot sleep and just whines and whines in his bed. How can we calm his body without sedating him and also calming his “mind?” I ask this knowing that the two are virtually inseparable…

2. I would like to discuss the possibility of botox injections for his left foot (and possibly in both feet since his right foot is now also increasingly involved in the flexion episodes) in conjunction with fitted orthotics for his feet to decrease the muscle spasms and pain and to try to eliminate the flexion and extension through his feet and legs. My sense is that if we use orthotics exclusively, Owen will continue to flex his feet and apply pressure to the orthotic, further aggravating the pain in his foot.

Sincerely,

Stacy Marshall

Four weeks.

July 23rd: Owen diagnosed with his first bout of pneumonia. He's prescribed two, heavy duty antibiotics. Kelsey and I say between ourselves, "Wow, the fact that this is the first real episode of respiratory illness, bodes well for Owen. Hopefully, the antibiotics will knock it out quickly." We listen to him gurgle, crack and cough the next several nights.

July 28th: Severe GI distress and diarrhea from the antibiotics. SEVERE. This is also the start of severe sleep deprivation for Owen and his parents. Elliot sleeps perfectly. Twelve hours a night. Uninterrupted.

Late July: Owen begins having spells where he gets really fussy, arches, cries, goes rigid, gets red in the face and then screams and screams with a look of terror on his face. He becomes paralyzed with pain. Is it pain? Fear? This happens most frequently when he is upright in his stroller or in his car seat. So. We stop taking him anywhere unless we absolutely have to. We take video of the episodes and start looking for answers. Elliot has taken to running up to Owen, when he hears him crying out, saying "Owen, Owen," and then "Pat, pat." He pats him gently on the chest hoping that this will soothe him.

Early August: We are giving Owen probiotics, soaking him in epsom salt baths, trying to get him to his cranial-sacral and feldenkrais appointments. But inevitably, while in the car, he has a spell. I have to pull the car over, take him out of his car seat and try to soothe him. What ARE these episodes and why can't we think our way out of this one?

August 16th: Enough of this. Off to the ER. Pack up Owen. To Seattle Children's Hospital. Pack up Elliot. To Oma and Grandpa's house. X-rays, blood draws, oxygen saturation monitors. "We can't find anything wrong. If the episodes get worse or more frequent, come back." "No. You don't understand. I can't get out of the house with him. Something is wrong. He's horribly uncomfortable." They agree to admit Owen. We stay two days.

August 17th: Day of discharge. Owen is constipated. So much so that the attending physician, during rounds, says to the team while showing everyone the films of Owen's little abdomen, "Folks, that's a lot of poop." He also has eight teeth coming in and when he has these "episodes," clenches down on his erupting molars and surrounding tissue so much that they are a bloody mess. He opens his mouth and looks like a prizefighter. Miralax for the constipation. Tylenol for the teething. Home.

August 18th: At home the episodes persist. No sleep for anyone except Elliot. And Dutch. Lucky dog.

August 20th: Make calls and send emails to specialists' offices at Seattle Children's. "Owen is trying to tell us that something is wrong. We need help figuring out what is going on. Will you help us?" One is kind enough to see us the following week, even though he's booked out through 2010. Thank you, Dr. M.

August 24th: Dr. M. says, after viewing Owen and looking at the video. "We don't want to mask this with medication. We need to find out why he is in pain" Thank you. "Owen is trying to tell us that something is wrong." Thank you. More labs ordered. More blood drawn. Another abdominal x-ray. Abdominal ultrasound ordered.

August 26th: At Seattle Children's Hospital again, all day, except for an 8:30 neurology follow-up at Swedish. Owen and I have crashed at the Silver Cloud at Broadway and Madison Wednesday night (eight down pillows PER bed!) since trying to do an appointment while overnighting at home would have required a 4:00 a.m. wake-up call from Dutch. Nope. Neurology visit goes well. Owen will begin the year-long wean off of the ketogenic diet in mid-September. IF we can stabilize him. Owen has an abdomnal ultrasound to look for problems with kidneys, pancreas, gallbladder... At his orthopedic appointment, after a review of the spine and hip x-rays, we learn that his scoliosis is bad. Really bad. Fifty-five degree curve. They recommend that a "growing rod" be attached to his spine. A metal rod that is surgically inserted and screwed in at the top and the bottom of the spine. On the ferry, on our way home, Owen has another episode.

I say, "enough already!" Geesh.

Happy Birthday to you...

Wow. Three years...

Joy. Heartache. Wonder. Courage. Hurt. Fear. Amazement. Awe. Uncertainty. Doubt. Growth. Change. Adoration. Love. Reflection.

Being a parent. Universal, yet each experience, totally unique.

Happy Birthday, sweet Owen.


Homemade cupcakes c/o Grandma Janet.



Elliot and I giving Owen a long, birthday-eve massage.



Alba Botanica Hawaiian Coconut Milk Body Cream. Nothing but the best... Do you think he likes it?



Catching the cool breezes on vacation last week on the Oregon Coast (Thank you, Great Aunt Connie!).


With Dad and Elliot at Girabaldi Fisherman's Terminal.