2 Years Ago, It Was Christmas 2011, and…

2 Years ago today, I was in a hospital with Savanna on Christmas Eve, a few days out from the life-altering news Infantile Spasms diagnoses bring.  Like nearly all parents blindsided with this situation we followed doctors recommendations for a treatment plan without question.  In reality, we were just trying to survive.

2 years ago, we didn’t know our baby had focal cortical dysplasia type I and IIa resulting in a lesion spanning the temporal, occipital, and part of the parietal lobes in the left hemisphere of her brain.

2 years ago, we didn’t know our baby would experience refractory complex partial seizures that would leave her severely developmentally delayed.  The seizure activity was so frequent, her potential development was largely unknown.

2 years ago, we initiated advanced genetic testing for which we are still waiting on results today.  Off-the-record phone conversations have revealed no definitive genetic cause, for reasons outlined in this post.

2 years ago, we had no idea our baby would (or even could) undergo radical surgery to rescue her developing brain from the intense seizures.

2 years ago, I had no idea that Savanna was in a small sorority of patients  eligible for surgical treatment and how it is such a blessing.

2 years ago, I thought I was good father.  I thought I was a good husband.  I thought I was pretty smart.  During these last two years, Savanna’s condition has cleansed me of many of these misconceptions.   I am now better at all three.  Absolutely nothing can prepare you for this situation as a parent.

2 years ago, I never dreamed I would stay at home and raise children, lead the care of a special needs child, attempt to manage refractory seizures; learn about epilepsy.

2 years ago it was Christmas 2011.  I brought our baby home from a 9 day hospital stay that included her first big ‘diagnosis day’.  You can read about the emotions of that day here (not well written, sorry!)  I thought it was ‘over’; meaning, finish the ACTH and then she is okay right?  For some yes, not for Savanna.

Today, I marvel at the progress modern medicine has allowed her to experience.  Faith in God, following his guidance, and trust in his leadership has proved righteous.

Today Savanna continues to impress us with her abilities that advance daily.  Delays are still present, but far less noticeable.

Today, Savanna still has challenges, but not seizures.  That is nearly 210 days!

Today Savanna is not cured but, her epilepsy is in remission.

Today, we as a family thank the Lord for his guidance and support.  I thank myself for listening.

Today, I help others blindsided with this situation.  Savanna’s outcome thus far is not the same for all, and this knowledge is humbling.

We are in the middle of a trip to Kentucky and Virginia.  The usual sicknesses have afflicted our family somewhat.  It happens when you transplant an entire family to a new location.  Overall, we are healthy and thankful for the journey.  Being around family is great at Christmastime.

Have a Blessed Christmas everyone and safe travels!

-ken

Guest Post – Mixed Up Mommy

Infantile Spasms are not diagnosed early in many cases due to a lack of knowledge, even by physicians.  I am not putting any blame on physicians, but the fact is that this is so rare, most practicing pediatricians simply won’t even see a case in their career.  Parents are almost always blindsided by such a diagnosis.  In some cases, a family has warning of a symptomatic onset of this epilepsy.  All scientific evidence indicates early diagnosis and aggressive treatment gives the patient the best chance at the not only stopping the seizures, but the best developmental outcome as well.

The only thing I have obsessed over more than my son’s tuberous sclerosis complex diagnosis was the possible onset of infantile spasms. Since we had Connor’s diagnosis shortly after birth, we were in an uncommon position of knowing to be on the lookout for this rare and catastrophic seizure type. We were fortunate about that, though it certainly didn’t feel that way at the time.

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Connor was born with TSC, a genetic disorder that causes benign tumors to grow on the organs. The tumors can wreak havoc with the functioning of the organs. He was born with several brain growths called tubers that caused him to have complex-partial seizures from the time he was a few hours old. He spent five weeks in the NICU as his seizures proved difficult to control with medication. We thought he would undergo brain surgery in his first month of life. One (of many) 48-hour EEG recorded 82 seizures, the majority of which were subclinical (not visible to the eye). Eventually they decreased enough that doctors felt he could go home — on a cocktail of three seizure medications — and return for surgery when he was bigger and it was safer. He underwent a resection of the right frontal lobe at four months and we entered a new seizure-free life.

For a month.

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Then it happened. The moment I had lain awake dreading, watched countless YouTube videos in preparation for and prayed a whole lot not to happen(for someone who has never been particularly religious). That jackknife movement, arms flying up, knees up ever so slightly — the spasms were here.

Infantile spasms occur at a far higher rate in the TSC population than in the regular population. I believe the statistics said that 40 percent of children with TSC are estimated to develop this complication. I was so sure we could be part of the 60 percent. This wasn’t based on any sort of logic, just grasping at straws of hope.

I knew what I was seeing immediately. I had feared this moment for so long that the shock of it brought on a panic attack. I became dizzy and had to back into the chair in his room. My husband asked me if I was sure. I was, but I still — grasping at those desperate straws — said that maybe, just maybe I was wrong. Let’s wait and see if it happens again.

I took Connor into our room and we both fell asleep, him drained from the seizures, me from the panic attack. We awoke a couple hours later and went to the kitchen. I laid him on his playmat and sat down to eat. He was hanging out peacefully when it happened again. And again. And again. No more time for denial. I got on the phone to his neurologist. We already knew what the plan was.

I should mention that when I worry about stuff, I like to get really detail oriented. So one of the things I had obsessed over was that the spasms would start on a weekend when his doctor would be unavailable. But it was only a little after noon on a Friday. Thank goodness.

The office message began to play. Oh. My. God. They close at noon on Fridays. How had I forgotten that? Would I have to go to the ER? Wait, I could page him, the message told me. Relief washed over me.

The partner of my son’s primary neurologist called us back. He’s great too, and he was actually the one that originally diagnosed him. Ironically, Connor had an office EEG earlier that week as a follow up to the surgery. He didn’t sound optimistic when I describe what I saw, but he told me he would take a look at Connor’s EEG which hadn’t been read yet. When he called me back, I heard the dreaded word — hipsarrythmia, the chaotic brainwave pattern associated with infantile spasms (I want to note that many TSC parents report that their child presented with infantile spasms, but no hips on the EEG. So if you see something suspicious but the EEG is clear, push the issue!)

The hips was there before the spasms started. Well, at least the definite spasms. There were a couple strange movements he had done in the couple weeks prior that didn’t repeat and weren’t so obvious, that I now questioned.

An emergency prescription for Klonopin was called in to get us through the next couple days until we could get him started on vigabatrin. Vigabatrin/Sabril is the front line recommendation for TSC and is only available through mail order specialty pharmacies. ACTH has been effective for some, but at lower rates than in others with infantile spasms. The spasms stopped within a couple of weeks, though he persisted to have some strange eye rolling episodes that never showed up on EEG and were never defined.

Connor was already pretty delayed, but we were lucky that we saw no regression. He was subdued and giggled less, but the day after we saw the last spasm, he woke us early shrieking and laughing happily.

For many kids, spasms are the first sign of a problem so they go undiagnosed or misdiagnosed due to how rare they are. As much as my obsessing drained me, it likely allowed us a better outcome. We didn’t have to wait for a diagnosis — we had it within hours and were able to start treating it immediately. Many aren’t so lucky and the spasms cause irreversible damage as parents struggle to find out what is wrong with their child.

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I wish I could say Connor was seizure free, but unfortunately his complex partials have returned. In spite of that, we have seen an incredible amount of progress in the last few months. His first 14 months were extremely slow progress. But just since June he has started crawling, pulling to stand, cruising and climbing the stairs. He’s happy, opinionated and in love with Click Clack Moo and Super Why.

The blog, Mixed up Mommy, is a wonderful and inspirational blog about all things TSC and life in general.

Here is a YouTube video of his spasms which I have uploaded in the hopes of helping other parents identify them quickly.

Recovery from a Second Resection for Focal Cortical Dysplasia: Day One

Having taken care of Savanna after her first epilepsy surgery, I thought I knew what to expect.  I was misled by my own experience about the potential difficulty immediately post-op.  My bewilderment is like that of parent who’s first baby sleeps through the night in 3 weeks while child number 2 doesn’t even come close to such behavior.

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Savanna was very healthy during the procedure, but it still took nearly 12 hours to complete.  The craniotomy (opening in her head) was basically the same as her previous except one small relief cut around the vertex of the incision at the top of her head to allow more access.  The neurosurgeon told us that Savanna had significant scaring in the dura underneath the cranial bone which resulted in a longer procedure time than planned.  After surgery Savanna was extubated and began to experience difficulty breathing known as stridor (a wheezy, whistle sound when breathing due to constriction).  This resulted from her larynx and retinoids below it being very inflamed, which constricted airflow.  Imagine trying to run or workout while breathing through a straw only.

 

Throughout the night she was really agitated as the anesthesia wore off and the stridor kept her from breathing well.  Finally, after many doses of sedative and painkilling medicines, she really went downhill – so I thought.  I worried about her safety that night one time in particular.  Rebecca was right there and even though an RT was working on Savanna, things spiraled out of control – or so we felt.  Rebecca ran down the hall to find the critical care Fellow that was managing her case, and forcibly brought them back to the room.  I think there were some expletives used, as she too was really scared.

Savanna was in tachnycardia.  This can be really scary, but also normal presentation with a very agitated child.  For sure, with the stridor, the crying hurt as her throat was sore, and this increased her agitation, which results in more crying.  Add the constriction to the windpipe and you have a recipe requiring action.  With respiration rates in teens, heart rates in the 220-230 range, and bp north of 130, she was really unhappy.  They would administer a mg of morphine and she would settle down for about 10 minutes and then awake as though someone was cutting her leg off with a butter knife.  Then they tried something else, same result.  Everyone involved was amazed after several doses of versed, morphine, norco, the addition of precedex drip, there was still only minimal control of the situation.  Rebecca and I could not have been more relieved that we had clear contact with the doctors overseeing her neurological care.  They called the appropriate people in the PICU and we felt (even if it wasn’t the case) this made a difference in her care that night.

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Perspective:  Most likely, the Attending Physician, Fellows, Residents, Interns, and Nurses had not seen more than a few cases like Savanna’s in their lifetime.  And guaranteed, the only thing similar would have been the type of seizure disorder.  The etiology and child would likely be very different.  Some statistics for those interested in my rant on ‘perspective’ – (based on aged data, but the best I could find)

  • 4,000,000 live births in US according to census bureau.
  • 1:4000-6000 live birth rate for babies diagnosed with Infantile Spasms.
  • Result: 1000-2000 total babies are born that will be diagnosed with Infantile Spasms, total in the US.
  • The gender ratio is 60:40, boy:girl  ==  There is only 400 to 800 female babies per year born in the US like Savanna.
  • There are ~ 22 babies within 1000 square miles of Houston that are born like Savanna each year:
  • Ratio of 10-county (1000’s sq miles) population versus national population
    • 6,100,000 : 350,000,000 = 1.7% x total births = ~68,000 people born in this area
    • Factor in 1:5000 average, ~ 13 girls born that will develop IS.
    • Factor in 70% symptomatic diagnosis, 9 girls born who will develop like Savanna in Houston area (1000 sq miles) each year.
    • There are 2 locations qualified to treat this condition in the Houston area.

Even if you add to the number of children with FCD (focal cortical dysplasia) that did not develop IS (infantile spasms), it is a small number in any given year.  So from my perspective, Savanna is very special, even if some of the doctors who care for her don’t see her situation worthy of study when dropped into their lap.  I am not suggesting they ‘waste’ a lot of time understanding the her history, but more than a look at her current chart might give some perspective to her situation and our potential knowledge as parents.  But nonetheless, I find myself ‘just the dad’, with a perceived  ignorant and irrelevant opinion not worthy of anything except keeping to myself.  A situation that would seem to warrant 60 seconds of briefing by looking through her most recent neurology clinic output note devolves into one that they are forced to go back and look or face a difficult road.  I know other parents out there have felt the same way at times.  I can’t understand why the situation has to be so difficult.

The following day, we were assigned a nurse who deemed herself all-knowing as to what Savanna wanted, although she stated the opposite.  Her hypocritical actions combined with her statements didn’t sit well with me and we decided not to stand for it.  Rebecca took on the confrontational role and initiated a nursing change.  This particular day was stressful enough already and we did not need the attitude from the nurses treating us like we have never taken care of kids before or that it was our fault the night shift crew did not do the job just as she would have done it.

So here I am, shortly after the first 24 hours in the PCIU, realizing that my baby is probably going back to the OR in a couple of days to deal with the airway restriction that is hampering her recovery while facing the fact that I am confined to this location against my will.   To top off our sour experience, Dr. V arrives around 5pm to check on Savanna.  We had never even seen the attending physician that day even though they, ’rounded’ on Savanna.  No doctor or nurse even told us what ‘the plan’ was despite several attempts to find out ourselves.  Dr V, says she is going to contact the ENT she wants involved, then leaves the room to make the call.  Magically, the attending physician comes in to speak with us, and said he had already set up the consult.  Ironic.  Not sure exactly happened outside the room during those few minutes, but it just seems odd that all that happened within about 10 minutes.

Savanna Hospital Second Resection-3

Savanna Hospital Second Resection-2 Savanna Hospital Second Resection

But, here we are, Second Resection in Recovery – Day 1

-dad

Sometimes, Less is More: Less Brain Tissue with Focal Cortical Dysplasia Is More Freedom From Seizures.

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This gallery contains 7 photos.

We have been blessed with witnessing a profound new beginning for Savanna after her first resection at 13 months of age. Today, God willing, we are expecting a new child to emerge from the operating room again as she undergoes … Continue reading

The Neurosurgeon Asks: “How bad is it?”

On January 31st, Savanna had a clinic visit related to her 23 hour VEEG performed on January 16/17.  I couldn’t understand at that time why the language seemed so vague about her EEG results.  When I finally got my hands on the EEG report, it became clear.  It wasn’t processed until February 14th.  So, they had to scan the EEG themselves in the office to provide any news on the findings (thus the extra 3 hours of waiting).  Very disappointing, especially, when I learned she didn’t just have one seizure lasting only 30 seconds.  She actually had 4 seizures, none shorter than one minute and one just shy of three minutes.  I was really taken aback at the time and really felt somewhat mislead as to Savanna’s real condition.  But, somewhere deep inside me, I knew the truth.  This time, this day, was coming and it has arrived.

hmmm...  we don't know either....

hmmm… we don’t know either….

I had sent a follow-up email with more questions about Savanna’s care.  I knew all the answers.  Dr. V knew that I knew all the answers, because have been through this in the past.  But, I needed some moral support and reassurance that we were doing the right things with her care.  Dr V was simply too busy to answer such questions and brushed aside the email despite multiple requests for answers.  Instead, we were pushed to perform another MEG study and MRI.  Then we learned her case would be presented in the surgical conference a short week later.  It became clear Dr V was guiding us in the right direction.  During all of this activity, Savanna has been having a really difficult time.  We undoubtedly observed seizure activity.  Some related to teething pain, pain with constipation, and the typical unprovoked seizures during the sleep/wake cycle.  Intervention was necessary on several occasions, though no EC visit was required.

We had reached the target dose on the Trileptal (36mg/kg/day), her new AED.  We titrated the Sabril down and when we were nearly at end of that period (only 20mg/kg/day), she became out of control.  We honestly thought the IS had come back.  Her demeanor according to Rebecca was identical to the time when the IS was in full force prior to the ACTH therapy.  Intervention was necessary, and calls to Dr V were at times frantic.  Panic set aside, we knew what to do, and did what was needed to keep Savanna safe.  Maybe more sedated than she needed to be, but safe from seizures while the time passed.  We have since increased the Sabril back to around 60mg/kg/day and added scheduled Onfi at 15mg/day.  This cocktail seems to be keeping her from pulling her hair out, and us too.

come on dad, no more pictures....

come on dad, no more pictures….

During the surgical conference I get a call from Dr T’s office that they want to see her next week.  “Wow, this is moving fast” I thought to myself.  To get a call during the conference is not the norm.  The final days before we were to have the clinic visit with Dr T were filled with lots of seizures for Savanna, and inconsolable agitation.  Our APRN met us at the clinic visit which also was not normal, so we knew this was serious.  That morning, we got the MEG report which Rebecca and I reviewed on the drive to TMC.  There was a lot of epileptogenic activity, more than we realized.  That particular report didn’t show seizures, but rather, a lot of focal spikes.

We talked with the APRN for quite a while before Dr T came into the exam room.  Then Dr T burst in, greeted us, then just sat down and said “How bad is it?”  Rebecca and I didn’t know how to answer.  We fumbled through a few jumbled sentences, before he stopped us and proceeded to ask us other questions to try to figure out the answer for himself.  The rest of the conversation was about philosophy of the deciding on the surgical option and the procedure itself.  In the end, he explained the procedure plan which is a multiple lobectomy.  The intent was to remove the entire temporal and occipital lobes and the parietal lobe posterior of the motor strip.  It is a much larger resection than she underwent last year.

He proceeded to explain the procedure and what deficits she will have.  Then the conversation turned to more philosophy involved regarding making the decision to operate or not.  There was some pause on my end, but not from Rebecca.  She wanted to move forward and now.  Dr T felt if the Sabril was restarted and Savanna was doing better he would prefer her to be larger in size as she would have less stress during the procedure.  He also felt that there was a high probability that Savanna would have this procedure in the near future, even if we decided to wait now.  We left the visit with a surgery date of April 1, 2013.  Dr T felt we needed to think about the procedure some more and let him know if we still wanted to proceed.  I thought a lot about what he said and the conversation Rebecca and I had that day.  Then I turned to a story I came across from a member of an IS support group on facebook.  The mother’s son, in a slightly different situation to start with, underwent nearly the same procedures.  I read her blog on his story start to finish.  I cried.  The similarities were chilling.  Their son is about 6 months ahead of Savanna in terms of surgical timeline and twice her age.   Then I sat down and wrote an email to our doctors confirming our desire to move forward with the surgery.  I think it really summarizes our feelings, although Rebecca expounds below about what emotions go into a decision like this. While answering the question, Here is what I sent the doctors  (less the grammatical errors I found when transposing the text here):

….

How bad is it?  The first question you asked was the one we least expected, thus the fumbling responses.  Yes, she was crying and fussy for the 15 minutes you saw her.  Until you have lived with a child like Savanna, explanations to your answer are difficult.  Many of us chosen parents attempt to portray this fact/emotion blend through blogs and so forth.  But, until you have lived it as a parent, you cannot understand.   Separating the facts from the emotions is the key to your answer.  You are in a unique position as a highly trained professional dealing with kids like this frequently, and a parent yourself of I presume typical children (my apologies if I am mistaken).  I have given up my career (for the time being) to take care of Savanna.  Rebecca has assumed a role out of necessity that supports our basic requirements financially and from an insurance standpoint.  This role is slowly corroding our life as the job sucks – simply put.  (Yes, we can and will change that, but when you are in the middle of a battle, sometimes strategic decisions have to be made to win the war and that was one of them.)  We moved our family closer to your place of business and our family for her sake.  It has nearly broken us financially, and for sure wrecked our future financial planning.  She is developing, but at a snail’s pace.  And, now complex partial seizures are back and clustering.  Do we have diagnostic proof of the existence of the complex partial seizures, no.  We are willing to run more tests if you need to see more data.  I am using ativan to intervene occasionally and now onfi is scheduled TID.  It was just a personal choice to use ativan versus rectal valium.  You are not hearing about idiotic ER visits now, for several reasons.  Dr Von Allmen is not in France and unreachable.  Now, we are armed with experience, knowledge and access to medications to help her quickly.  It is not because she is not seizing.  Yes, I agree and admit the seizure frequency is lower than prior to the first resection.  But, how bad is it you ask, it is bad.

Yes…we can probably optimize medications to help her with the complex seizure control, but it will be at the expense of becoming non-participatory in life.  We already see that happening now.  We also feel confident this will over time degrade her state of health and make the procedure more difficult for all parties.  And, she is still having lots of electrographic events.  We are ready to act now.  We say that without the benefit of years of experience you have seeing patients like Savanna and knowing that we are making a dramatic decision that cannot be undone later.  We are making the most informed decision we can based on the collected data, opinions expressed by you and Dr. Von Allmen, the stories of other children in similar situations, and our faith in your God given understanding of the science. 

Savanna needs your help sooner rather than later if you feel she can safely withstand the procedure.  You can help her.  We trust you and Dr. Von Allmen, and your team members, to the extent that we are prepared to hand her life over to you for a short while why you all do what you do best knowing the outcome has lifelong effects, some good and some bad, and some risk of unintended permanent effects.  We trust your judgment on the intended procedure plan.  

….

there are no words....

there are no words….

Many of you may wonder (as do we), what will she lose when these sections of her brain are removed?  She will lose her speech, attention to her right side, and short term memory.  Those functions will move to the healthy side of the brain and she will learn to compensate accordingly.  She may have some weakness on her right side, particularly in her right leg.  If it does not go as planned, she may have paralysis on her right side.  This can be overcome with therapy, as it will plasticize to the right hemisphere.  She will lose her vision in the right half of both eyes, resulting in her loss of her natural peripheral vision on her right side.  This will not change, as it is a function that cannot move to the right hemisphere.  She will learn to compensate by scanning her right side every so many seconds.

(Commentary from Rebecca)  We are living now for this opportunity that has given many other parents hope for seizure freedom, and hope for a near-normal life for our daughter.  If successful, Savanna has a 40% chance of becoming seizure free after surgery without long-term medication.  Seizure freedom is what is required to give her the best chance to develop “normally”.  The odds may not sound good, but when we started our journey with infantile spasms (IS), her odds of even having IS, were less than 1 in 10,000.  Her odds of averting severe mental, physical, and emotional handicaps were only in the 5 to 10% range, so to us, 40% sounds very good.  It is “cause for celebration” as our first epileptologist put it.   As a conservative gambler, an engineer, and a statistician, I never thought I’d be one to go “all in” on 40% odds, but today those odds mean everything.

The risks involved in a second surgery are higher than with the first.  Savanna still is barely above the minimum 10 kilograms at which our surgeon will agree to operate.  Our surgical team will also need to navigate the prior resected tissue in her brain which presents it own set of challenges versus virgin tissue.  She could experience too much blood loss.  She could have a stroke during the operation.  The surgical team could have to abandon the surgery prematurely, requiring us to have to wait many more months before a third attempt can be made.  We could lose her.  Most of these risks are very very small according to the surgeon.  It is beyond terrifying to agree to these risks for your child, when they themselves have little say in the matter.  We remain focused on the 40%.

We are trying to balance the risk of moving forward with surgery now, to the risk of waiting too long, and having her lose milestones or go back to the weeks and months of constant seizures and near-coma sedation.  Some days, we watch her, and see how far she’s come since her 1st surgery.  Her hair has grown back, and you don’t notice her scar.  Maybe a few seizures aren’t so bad…She smiles at us.  She loves her brothers.  She’s getting so close to starting to crawl.  She gets excited when we walk in the door…then like so many other times, she’s just not there…  She’s staring off.  Her eyes are rolling, drifting cross-eyed, or pegging in an unnatural direction just for a few moments longer than what can be considered normal.  She goes limp.  Her breathing becomes labored.  Is it a seizure?  Is it just the way her brain and eyes function?  Ok, we think, we just noticed this…how long has it been going on?  Our minds start the mental count 1,2,3,…24,25,26… ok, this is really a seizure…Is it the first one she’s had today, or just the first one we’ve noticed?  We don’t really know.  In the middle of all this, there is life with the other kiddos…

Brandon in the sandbox

Brandon in the sandbox

Saturday morning with powdered donuts!  mmmm!

Saturday morning with powdered donuts! mmmm!

We have to give her this chance.  We pray that we are not selfishly doing it for ourselves, to have a chance to have our normal, healthy baby girl back, a child without lifelong harsh sentence of unknown special needs.  Is it really possible?  How terribly arrogant that sounds as I put my thoughts on paper.  We will love her no matter what, fiercely, and always, but we will do anything and everything within our power to take this burden away from her…away from us…to give her a chance…even a glorious 40% chance…  We pray it is the right decision…(back to Ken)

How bad is it?  Well, it can be worse.  As we have learned, it can always get worse, but we are so fortunate to have this option to hope for.

The Blowout Diaper – and somehow, despite the odds, we managed not to get poop all over us…

So she is doing well prior to March 12th, and not well now.  But, just getting around to this post and in the middle of authoring the next which will go out March 17.

Savanna has presented a number of challenges lately, but few are as familiar to most parents as the ‘blowout diaper’.  Constipation is part of Savanna’s normal panel of challenges which is attributed to several factors. Compounding the problem, she has Hypotonia and hasn’t been very active for more than a year.  Sometimes, she would not have a bowl movement for up to 6 days during the worst periods.  So when it happened, ‘Ho-moly!!!’ as our newly crowned 3 year old Brandon would say.  (He has learned the phrase is really Holy Moly!  but it was funny while it lasted.)  When the momentous moment arrived, usually everyone involved with the event needed new clothes, baths or showers.  Without actually dealing with it first hand, it is difficult to explain.  My experience is that newborns usually do this a few times before they become regular (at least ours did anyway).  But, when she is more than 12 months old, the amount of excrement is significantly greater.  There is a visual for you.  Just so you don’t miss the moment, she would do it about 3 times in a row within 2 hours.  More than a few times, plans changed – even doctor visits were rescheduled due to the collateral damage done by her explosive poops.

He scoots things like the activity table to the counter to reach his goals...

Austin scoots things like the activity table to the counter to reach his goals…

At our new friend's House, Zane, who also has IS

Austin in the doghouse, at our new friend’s House, Zane, who also has IS

Bathtime!

Bathtime!

At the playground in the swing...

At the playground in the swing…

Tristan 5th birthday, these were his gifts.

Tristan 5th birthday, these were his gifts.

at the playground

at the playground

Look! It's a bird! It's a plane! No, it's just daddy trying to take a cool picture...

Look! It’s a bird! It’s a plane! No, it’s just daddy trying to take a cool picture…

Tribute to our doctor, Dr Von Allmen

Tribute to our doctor, Dr. Von Allmen

After the second ‘event’, it was as if a God had spoken and said: “Okay, Okay I have had my laugh.  You can have a break now, carry on then.”   Happiness returned to Savanna and life was good!  And then, we start the process over.  About three days later, the constipation would return.   We double and then quadruple the miralax trying to help her, but it just takes time.  Usually her threshold was about four days and then she was no longer happy no matter what we did, and I don’t blame her.  We tracked it on paper, changing the miralax dose as needed.  We would double dose, then double it again, it just seemed like nothing helped.  The GI doctor felt a stimulant such as a laxative wasn’t prudent, so we were just stuck dealing with this unpleasant cycle.  Looking back, we should have tried the OTC stimulants for adults at a properly reduced dose for her weight.  It may have helped.  The GI doctors see even fewer patients such as Savanna than the epileptologist.  Hindsight is always 20/20 right….?

Part of being a parent of special needs child like Savanna is that you learn that when the brain is not healthy, many autonomous functions of the human body experience some dysfunction.  She is not special from that perspective.  So we roll with the punches, and continue to search for therapies can help her the most.

After her first lobectomy, everything was much improved.  We stopped tracking when she had movements as it seemed she had movements almost every day.   She still had the occasional ‘blowout diaper’, but it was becoming very infrequent.  I didn’t carry extra clothes and car seat covers everywhere we went.  Then her eating really picked up as the solid foods were introduced.  We noticed she seemed to be having pain when she urinated.  All signs pointed to a UTI on numerous occasions.  The most recent one thought had no indication of any pathogen in her urine after a culture as well as a clean stick-dip check.  A couple of abdomen x-rays, and renal ultrasound, and VCUG test later, pointed to constipation.  Apparently, she wasn’t completely emptying her colon when having a normal bowel movement, leaving stool in the colon which can cause pain during urination.  For all of you ladies out there who have had UTI’s, I feel for you.  Our daughter screamed bloody murder when she urinated and it became a scream with other vocalizations that were very, very specific.  We knew right away when she urinated versus falling over on the living room floor (probably from a seizure).

So we find ourselves increasing the miralax dose to help her complete the bowel movement by softening the stool.  And, I now find myself taking extra clothes where ever we go, leaving earlier than normal, and taking the extra car seat cover too.  Ho-Moly!… as Brandon once said.  We are back to the blowout diapers!  She only needs size 4’s but I think today I am going to buy size 6’s and use them when she is due for a movement.

Meeting new friends…

So here we are, living about 2 miles from a family with a child with IS, and did not know it.  We did not connect until after Savanna’s first surgery.  We decided to get together and they invited us to their house for dinner.  It had been almost 30 hours since Savanna’s last movement.  We had given her 30+ grams of miralax.  I knew when it happened it would be significant in nature.  All day we watched for her movement, and nothing.  Time to go to our new friend’s house, and halfway there, there is the smell.  Not just any smell, it is thick, warm, and cannot be mistaken for a simple event – (like a fart – there I said it!)  We arrive, and the first thing our new friends behold, is Rebecca lifting Savanna out of the car seat and liquid poop oozing out of our of her outfit and dripping off onto their floor.  What a great entrance.  We went to work already knowing it ends messy.  Yet somehow despite the odds, we managed not to get poop all over us.  The night went well otherwise, and we now have some new friends for life.

-ken lininger

Savanna Sitting Up and Then In Her Walker

Hi everyone,

A follow-up to the previous post.  Our friend Lisa from California is visiting and Rebecca and her took the kids to Galveston today.  Admist the help in getting them out the door, I ran across some video taken recently in preparing the cameras for an outing.

Taking a break from the deep cleaning, I wanted post this video.  It shows her sitting up and then later that day in her walker.  She isn’t walking yet, but we use it to help her put weight on her legs to start getting her used to it.  These two segments were both captured on Sunday, February 10th.  The last 30 seconds really summarize what it is like in our house right now when Savanna is happy.  Brandon is in rare form!  I watched it a couple of times and just laughed out loud!  A fun memory worthy of sharing.

-Ken