About kenlininger

Parent of Savanna, author of the blogs, husband to Rebecca, learning about all things epilepsy and autism.

4 Months Seizure Free and Counting…

Posted on August 1, 2013 by kenlininger

4 months since complete TPO resection. No seizures that we know about.

This is great news. 6 months is the industry bellwether. Seizure freedom 6 months after a surgical treatment to remove a seizure focus, generally indicates an upcoming long Honeymoon period if not permanent cessation of the seizures. Specifically, it means the dysplastic lesion was completely resected.

Savanna’s blog has a new page titled “Savanna’s Story“. I have re-written her story from start to finish. It is not a compilation of the posts. Those of you who have met Savanna along her journey or have known her from the beginning, I invite you to read her entire story here. “Like” it, if you choose.

Also, I have upgraded the “About Savanna” page to an abbreviated timeline of events, with a little commentary at the end.

Thanks for all your prayers, support, and comments,

-ken

Sunshine After the Rain

Posted on July 17, 2013 by kenlininger

There is nothing like a 3400 mile road trip to bring out the best behavior in everyone – all stuck in the van for what amounted to north of 64 hours. My apprehension taking Savanna on such a long road trip was high. Her demeanor the past three months tempered my expectations. In addition, results from the Bronchoscopy and EGD scope of her esophagus showed nothing was wrong. Rebecca took her that day, and I was home with the other kids. I remember getting the call, and actually feeling depressed. I desperately wanted something found to be affecting her that could explain her lack of eating and near constant agitation. Despite the fact she is not having seizures, we cannot live like this. For the first time, I actually thought maybe we made a mistake with the brain surgery. It was like a dark cloud was following her (and me) and the rain just wouldn’t let up. I said a prayer that day for God to give me strength to carry on.

Yes, there was some less unhappy time, even some photo opportunities in the past 3 months. But, I remember no discussion prior to surgery other than the common complications and rare unintended consequences, such as infection, hydrocephalus, and paralysis. I did learn how to help her regulate her behavior, but it required a lot of heavy sensory input from me, and it was exhausting. I just could not do it for more than 2 or 3 hours at a time. At which point, I just had to put her down. She could not just be held without a lot of stimulation. Here is a short video of what she looked like during most of her time awake since the surgery (just before the trip)

Travelling in the car proved easy really, as Savanna really enjoyed the constant motion and movement which is known as vestibular sensory input. Knowing how to regulate her behavior externally through my actions, I actually expected an easy car ride. But she wasn’t just easy to manage, she was really happy at times. It was refreshing to witness. At the hotel, the alternate personality emerged, (and so did the Ativan®). An hour or two was all we could endure at that point, and we had to intervene.

First stop on the trip was my Dad’s place in VA. They live in a modern log cabin on Lake Anna, a man-made nuclear power plant cooling reservoir. Lake Anna is nearly 17 miles long, populated by people who love being near the lake. The obvious enjoyment of lake living overflows from their property onto elaborate docks complete with second story living areas, slides into the water, dry docks for several different types of watercraft, duck blinds, and even an airplane hangar or two. It seems more developed each time we visit. Rebecca had to work in DC, so I took all four kids out on the pontoon boat with Grandpa and Grandma 2 days in a row. We pulled them around on the tube until they just didn’t want to tube anymore. I even took Savanna out on the tube with me, and she really enjoyed it. Before, after, and sometimes during, was challenging with Savanna as usual.

Then on the third day there, a miracle happened. Her morning was typical. Inconsolable for about 5 hours, then nap time, and then she awoke and didn’t cry! Like the sunshine after the rain, Savanna was happy. It was like a new beginning that day. I didn’t know what to think or even how to appreciate it? It was a relief at first. She wasn’t screaming and crying constantly and I assumed that she would start crying again soon.

She became a Mommy’s girl instantly. She wanted little to do with me, especially when Rebecca was within sight or earshot. How selfish was I to be a little depressed by this particular behavior, being the only one who could seem to regulate her for any length of time in the past few months. The coming days saw another leg of the trip (550 miles to Louisville, KY) where the other grandparents and family live. She could not have been happier once at Rebecca’s parents’ house. She smiled. She laughed. She was very deliberate with her actions and movements. Her cry changed to one of ‘look at me!’ rather than the boo hoo of ‘why is this happening to me?’ The shallowness of my thoughts during those first days would soon be overshadowed by the change we saw.

I really think God was watching us and was acting. I tried to explain to those around her our first night in Louisville that this had just happened. My words felt like they became awkward, as none of them have really seen her in the last 3 months. I should have just kept quite. Looking back, what did the past really matter anyway?

The joy of peace and happiness filled the air. All seemed right in the world. I learned on this trip not to disturb such joy with unnecessary conversation. I am still learning how to choose the right words to formulate responses to questions from many different types of people. I am still learning how to exude happiness, elation, and even satisfaction about how great she is doing compared to where she was less than 1 year ago.

I have immersed myself in her every detail of her condition and care, delving deep into how persons like Savanna ‘recover’. I have results from Early Intervention testing, advanced genetic testing, detailed clinic notes from many types of doctors, and feedback from therapists who spend most of their time with kids like Savanna. I spend time helping others work through similar situations, providing support, just trying to listen. All of this activity brings gravity to Savanna’s situation. Yes she looks great. Yes, only Savanna knows what she is going to do in this world. Yes to all the anecdotal advice we are given. But my vantage point gives my a different perspective.

A broken bone healing is how I think many see Savanna’s situation. During this trip, the cast came off, function resumed, and now she is ‘normal’. Just put her back with the other kids, right? What is your problem Dad? What, she won’t eat? Well, you need to learn how to feed her – don’t you know? Okay, maybe. Maybe everyone is right and I am over thinking some things [but I don’t think so].

Most professionals would agree that the broken bone example is over simplification, despite how she is acting at the moment. On this journey with Savanna, I am learning how to converse with people about her situation at various levels. Numerous times on this trip I found myself misguided in conversational direction. I was too argumentative with my know-it-all attitude, and too late to change course aside from shutting down the conversation. Live and learn.

Character flaw aside, I really enjoyed also watching Tristan, Brandon, and Austin spend time with their cousins and especially Savanna’s happiness with her Mommy. I felt blessed by the end of the trip. We let Tristan have a camera during the trip and just let him go to see what he would capture. Attached are some of the photographs he captured. Each day I see a little man emerging in Tristan. I wish I could just freeze him just how he is so I could have more fun with him at this age, but life goes on.

Our trip back to Houston was without incident, and Savanna has remained for the most part happy. She seems much more engaged with her surroundings when she isn’t just wondering around on the floor crying. She is exploring, learning, and having fun.

We accepted the arrival of our Au Pair, Ruth, from Australia. Ruth is a great addition to our family and the kids have warmed up to her quickly.

I am learning each day how to better appreciate a little sunshine after the rain.

-ken

Recovery… in the Shadows

Posted on June 13, 2013 by kenlininger

74 days since complete TPO resection.

In the shadows, we see great things from Savanna. She responds like never before to the world around her. Strength, she is gaining every day. Finding the blessings in each day are easier as she smiles now when she sees me and then deliberately crawls towards me and wants me to pick her up. Amidst the unending housekeeping, I see a toddler in the shadows exploring furniture and a house she has never seen on her own. Instead of seeing only the neighbor’s little girls play, I see mine play. I see things that scare me when she climbs something on her own the first time. Through teary eyes, I see the innocent love from her brothers who don’t really know what is going on yet. I see a toddler nearly 21 months old, functioning emotionally at a typical 6-9 month level. I see a toddler we are just getting to know. From what I have read, she is just getting to know us as well. I see all of this in the shadows of very short moments throughout the day.

: Brandon will at anytime, even if she is crying, jump in the swing and start to do his thing to make her laugh. I have to restrain myself at times, as it almost always works out great. She laughed here from her belly, it was a moment to to shut off the faucet, put the dish rag down, and just watch and absorb.

: The clarity and symmetry in her eyes now is stunning. It was never like that if you look back through her pictures.

: She now surprises me with what she can do sometimes.

: Austin with Savanna on the swing. She is happy here, but definitely in her own world.

Out in the open, she is in pain. It is inexplicable and no drug helps. Most feedings devolve into an act of futility within minutes. She is in pain. She veins out like a devoted weightlifter. She exhibits stridor when upset and is still retracting a little in her tracheal area of her throat even when not upset. She has failed a swallow study, but we were told “it was mostly normal”. A recent clinic visit with a Dr. GI planted a new seed in my mind for differential diagnosis: Candida in her throat below the esophagus. What you say? A yeast infection in her throat, really? Why yes. And I understand it can persist over long periods of time. If I play Dr. House on TV and populate my white board with symptoms and probable causes, Candida wins over re-flux handily (especially since we are already treating for re-flux in an effort to affect the situation – Dr. GI didn’t care about that). This day, like so many others, Savanna seemed ‘okay’ in the clinic at that moment. Why is that? It is because that is the way it goes. I know you were expecting a scientific or smart-a$$ statement, but no, ‘that is the way it goes’ is my answer and I am sticking to it. As soon as we left, all hell broke loose. In fairness, treatment of the patient depends a lot on presentation at that moment in time – in most cases.

Oh great, let’s treat the Candida, right? Wait, treating it without formal diagnosis would be ‘sloppy’ according to Dr. GI. We have to scope her in the OR under GA to confirm. But, even though we don’t know if it is re-flux or not, we will treat that right away. What? What kind of fuzzy ethical line is this I ask myself? I my heart, I know if this were Dr. GI’s kiddo suffering, the treatment plan would be different, or at least on a different timeline. Sometimes, it is what it is. I have accepted that for Savanna, suffering is just part of her life. As her caregiver, I work hard to learn how to help her, but sometimes you have to accept that there is some suffering still to be had by all. In His plan, I think this suffering helps reduce the mediocrity toward life when there are good times. Without doubt, it has changed the way I see the world in terms of special needs people. For starters, I see them now, which is in and of itself a big step for many people like me with virtually no background whatsoever.

“Oh don’t worry…” says Dr. GI, “If the situation gets worse, call us.” And, you will do what, exactly? Gosh, can it get worse? For a split second, I forgot. Yes, it most definitely can get worse. I count my blessings along with each day she seems to be seizure free. So for now we wait. We wait for the day she goes under the cloud of anesthesia and away from us – again. To a place where helping her as her parent, we have to let her go. From there, we are actually helpless. It is a dark feeling. It feels like you are at the bottom of something where no one understands, where you are unimportant despite great lengths that have been taken to educate yourself, despite the emotion surrounding the sacrificial, endless love and care that is required each day to survive. I will wait for something miraculous to take place yet again on that day. I will wait for that moment in the waiting room, where I hope to get good news. And like past experiences, hope a problem is identified that can be repaired with restorative measures. I just wait.

Tristan ‘graduated’ pre-school after only 4 weeks of enrollment. I did this so I could have a little break while Rebecca was travelling. The lady in the very middle in the white runs the school and could not have been nicer to me. Then I learned she had a daughter with special needs who has since gone to heaven. I am not sure how much harder it can be on this earth to watch your child slowly succumb to a rare, horrible syndrome. Even with what we have been through, I can’t imagine what she had to handle as a parent.

On a lighter note, Tristan, Brandon, and now Jackson have continued the pirate gold hunting activity started when Grandpa Squiz was here last (thanks….) Our neighborhood is still being developed which includes a lot of lake digging. So, we head when the weather is good and the equipment is resting to ‘look for pirate gold’ that might have been uncovered while they were digging. These are fun times indeed.

In the excavator bucket!

How many people can say they have been on a D6 dozer…

With friends, exploring the new sections of lake being dug in our neighborhood.

: What color is my tongue?

: It’s summertime!

Having Rebecca working from home has been ~~well, interesting.~~ great! She traveled away from home the first 6 of 8 weeks (6 consecutive weeks) which was very difficult. The last day of the last week of this travel marathon, the kids were bound and determined to say up to meet mommy, not scheduled to come home until around 1am. The last one asleep was Tristan who made it until 11pm. This was a moment I took to low light photography and got a cute picture with a 30 second exposure. It came out great with wonderful color and detail. Austin is passed out face down on the floor, and Tristan and Brandon who are best buds, were inseparable as usual. Savanna, where else, in the sensory swing.

We have wonderful neighbors who help me with the kids. They are willing to let me nearly kill myself on their boats trying to learn how to wake-board. (I am forecasting the need for services from adult doctors at Memorial Hermann one day if I keep trying.) They too have little ones and we are having fun teaching them all how to ski. Tristan and Brandon have skied now several times and are loving it.

Tristan skiing on a small arm of our lake very close to our house.

We have entered a contract with another Au Pair agency to accept an Au Pair, so full-time help is on the way! Her arrival will be early July and we can’t wait. There are projects around the house that need attention, things to paint, things to fix, things to change, just things to do. None of which I can do while spearheading Savanna’s care and keeping the other three kids ~~alive~~ entertained, well fed, and clothed. Somewhere in the middle of all the therapy, appointments, and ‘entertainment’, there is endless daily cleaning and housework that in spite of the effort, still leaves the house in a somewhat embarrassing state at pretty much all times while I am on duty. I am sure Rebecca can expound greatly on this issue.

I feel like I am on an island, far away from land, but close enough to see it. I am walking a fine line, tip-toeing on eggshells, carefully navigating freshly frozen ice on a pond. I am deliberately trying to starve her just enough to stimulate the desire to eat, but also making sure she is healthy for recovery from major surgery. Some people might read this and have differing opinions on what we should be doing to help her. Help her we are though, as she continues to improve. In retrospect, had Savanna not already have a G-tube placed, this situation might have been resolved a long time ago. Dehydration would surely have set in within a week or so of being discharged, and she would have been back in the ER. We might not have been discharged at all looking back. What can I say? It is a recovery in the shadows.

-dad

Field of Laughter

Posted on May 28, 2013 by kenlininger

I can’t ‘add’ anything (other than I know what a field is and how to make a round bale of hay….), please read this post from this mother….

Hello Butter

These four of mine, they trudge out to the middle of that hay field to satisfy a mother’s need for remembering moments. My sister looking through the viewfinder seeing us all laugh at posing just so, and smiling on cue.

She is Kaptured by Kelly and capture she does. Capturing moments this mother’s heart needs. Not just for the picture to be framed, but for the laughter of the day to sink deep into my soul, so that on days we are apart I can remember those special smiles we had out there in that field.

Small moments made big in my search for simple joy. Simple pure joy that fills a heart that overflows love. A kind of love that spills out when there are tough days. On days that we are separated by space or days when there is an empty house quiet and still.

On those days…

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Each Day there is a Climb and a Summit.

Posted on May 23, 2013 by kenlininger

Savanna is 53 days post op from a TPO resection.

Many people have commented on how well they think Savanna is doing, and how whenever they see her she looks so good, happy, and healthy. They are right. As her primary caregiver, I have different perception of the situation. Each day she experiences a sinusoidal track of mood and behavior. But this is not your normal roller coaster of emotions. As a result, our house has become a compound for Savanna. Not just a home base, but a safe zone where I am comfortable at all levels. Rebecca feels differently I am sure, as she is such a free spirit even after all we have been through with Savanna.

I am a planner at heart. I thrive on knowing the what, where, when, and who about every outing. Free spirited, unplanned activities outside the compound are few and far between for me when Savanna is in tow. Instead, each day is backwardly planned, managed for maximum pleasantness for Savanna when we leave the compound for a scheduled outing. Some would say this is a character flaw on my part. At some level I agree, and am trying to live more freely now that her seizures seem to be controlled. That doesn’t stop the overwhelming desire to make sure I have Diastat®, Ativan®, water, syringes, extra clothes, extra food, extension sets, sensory toys, etc, etc, etc. I pack light (relative to previous years), but I pack strategic items only with survival in mind if faced with a crisis with Savanna.

Even something as simple as a play-date with the mom’s group in our neighborhood is carefully managed. I can ill-afford to have Savanna in complete breakdown mode when we leave the compound. Austin is too mobile and vulnerable to a big fall on most of the playgrounds we attend. And, trying to hold Savanna when she is having a bad day is like trying to hold a 26lb mealworm on steroids. (For those of you fish, you get the idea.) And, even if it is ‘just a play date’, it is the summit of my day. It is a personal goal just to be able to attend, aside from my daily goals for Savanna’s progress. Once upon a time, I like to think I had significant impacts on not only companies I worked for, but their customers. It seemed very important at that time. Now, my primary job is to keep Savanna alive and make sure she can thrive. The contrast from then to now in my daily activity is stark. There was a time for me though, like many reading this, I took my kids for granted. Work was the most important daily task due to not only the sense of loyalty to the company and those I worked with, but also our family’s financial well being. My, what I have learned in my 40 years on this planet. Work is still important. As all parents of special needs kids know, having access to good health insurance is key in surviving. But the roles in our family have become more defined as the dependence on others to raise our kids has lessened substantially. It has been a difficult adjustment, and still a point of contention at times in our house.

Rebecca has been travelling almost every week in May and it has been a quite an adjustment. I have had to step back at times and make decisions about what has to get done during the day. Some days, the list is very fluid as Savanna is on a new path now and needs a new diet of sensory input. Learning how to feed her this input so she can thrive takes time, some days more than others. I am hoping some of the unhappiness is her expressing herself and needing additional and different kinds of input. The sensory swing I built many months ago has finally emerged as a worthy project. She can be completely inconsolable by any means and you put her in that swing and give her a big push and she stops crying and starts laughing. In many ways, we are starting over with her from a parenting standpoint.

I must give a shout out too, to Tristan, our 5 year old. He has been a great helper and guardian of Savanna when I am not looking. Brandon helps too, but he is 3 and his level of awareness is on and off, depending on what mischief he is currently undertaking. And, these 4 kiddos have really become closer in the last few weeks. I have tried to make it a point to do something with each one of them every day, though I spend a lot of time working with Savanna.

Savanna has a lot of therapy in the compound too, and those days too are carefully managed the same manner. This allows maximum benefit from the therapy. If Savanna is really upset or asleep, it can nullify the therapy visit. When the therapist is an hour late, it can really void the effort as an hour awake for Savanna is a long time. I have come to realize right now that her wake cycle is about 4 hours. The first hour now she seems happy, and it goes downhill from there. The kind of input she receives makes a difference and I am learning how to help her help herself.

When I have to leave for an extended period of time, I take the nebulizer in the car and run it while on our way to wherever we are going. If she is sleeping it is easy. If not, I probably look like a drunk driver. The extra inhaled steroids and ipratropium bromide really help with her stridor and wheezing and general upper airway congestion. There is something still going on in her throat. I am not sure, nor is the ENT. Savanna has stopped eating again almost entirely. The past 7 days, I managed only about 1 container of yogurt, and 1 container of baby food – that is it. So, she back on tube feeding which is really demoralizing. No one is really helping us with this situation, so I feel like I am on my own in figuring out what to do. She will put anything in her mouth and chew it, but won’t swallow it for nothing.

I have noticed now too that she is spitting up randomly throughout the day and smells like vomit a lot. She sometimes stops playing and starts to make that sound like she is going to vomit, but doesn’t. Not sure what to think. Doctors don’t think she is ‘hydro’ as they call it, short for developing hydrocephalus. But, as a parent, I just think something is not quite right with her, pardon the pun. I started her on Zantac to see it helps with the reflux.

In reflection, I have looked at my time spent with the kids lately and realize it is about a 50-50 split. 50% with Savanna and 50% with the other three total. Unfair for the older ones, but I sure hope one day they understand and can forgive me.

I couldn’t get this posted in time for Mother’s Day, but for the mom’s out there this is for you. You can tell something is bothering her, but it is indeed a great little clip.

For Savanna right now, everyday is climb. It is a challenge. God is showing us the way, one day at a time knowing we can’t handle much more than that.

Take care,

Ken

Exome sequencing in epileptic encephalopathies – a classification of de novo mutations

Posted on May 21, 2013 by kenlininger

I don’t often reblog, but this article was particularly interesting as a parent of child with epilepsy. What does it mean? My summarized thoughts are twofold. One, support networks could be established based on findings. Despite what is available now, we as a family feel very alone. Making the connection is the key. Two, maybe additional research along with clinic trials could guide therapy decisions. The more I learn about this topic, the more I understand that very little overall is known about why some epilepsies occur.
-Ken Lininger

Beyond the Ion Channel

Trio-sequencing your clinic. From the perspective of a child neurology clinic, I often wonder how much information we would gain if we performed trio exome sequencing for de novo mutations systematically in all our patients with difficult-to-treat epilepsies. Many of these patients have epilepsies that are difficult to classify and they have not been included in our existing EuroEPINOMICS working groups on defined syndromes. Now, a recent publication in Epilepsia gives us an idea what we will find if we perform family-based exome sequencing in patients with unclassified epileptic encephalopathies. Basically, you will find SCN1A and CDKL5 plus mutations in several genes that are likely pathogenic. But there is much more to this issue, which motivated me to come up with a classification scheme for epilepsy-related de novo events.

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Milestones: Savanna progresses while Daddy falls over the hill…

Posted on May 2, 2013 by kenlininger

32 long days since Savanna’s elective multilobar resection. What can I report? Well, it has been really difficult, but I think she may have finally turned a corner with all the crying and pain. And, it probably had more to do with two of four incisor teeth finally breaking through the gum line. One thing I have learned is that life’s normal challenges for young children are just that much harder for Savanna. Unfortunately, she doesn’t know any other way. Just before the grandparents left, we were able to get a few good pictures where she wasn’t sleeping and wasn’t crying.

Grandma Lou and Savanna

Mama Barbara and Savanna

I have come to realize that these periods are core training for parenting special needs kids. It is a bit like that darn underwater board in Super Mario Bro’s Wii, (…need some special thumb exercises or something). It is really difficult, but necessary to master (or at least pass) in order to progress through the game. With faith in God, living through these periods and not losing your sanity is actually possible. And, in turn, it helps me see the happy times more clearly and not to be so callus in social situations. Otherwise, I would probably go bonkers with typical conversation with people around me at any given time. We did document the good times when they happened and here are some pictures of the kids doing fun kid things. Tristan and Brandon are two pees in a pod and they do everything together.

Tristan and Brandon being silly at lunch.

Tristan in 'Time Out', Austin thought it was cool and wanted to join in.

Tristan in ‘Time Out’. Then Austin thought it was cool and wanted to join in. I couldn’t help but laugh, and that didn’t help the ‘time out’ situation, as Tristan knew what was going on – head down and laughing at me laughing at the them…. lol.

Play time after a bath with twin brother. Austin and Savanna.

Back yard fun with a planter box on a Saturday

Savanna in a moment of happiness, with Mommy.

Pre-school and then playing at the splash pad all afternoon is tiring. This is 6:15 at night, and Brandon falls asleep at the table.

Warning: yucky image forthcoming. One morning Savanna was having a particularly difficult time. She cried and fussed for about 5 hours straight starting at about 5 am. At 10 am, I decided to just buckler her into a car seat on the sensory platform swing I built and let her swing. She was worn out from all the fighting. She got quiet. I left her to deal with some laundry. I was at the putting away stage with about 4 loads, so I grabbed what I could and walked back to put it away. The smell hit me like a wall. It was obvious what had happened and who did it. She wasn’t just quiet but laughing. I already knew I had a big mess to clean up, but I was still amazed at what I saw when I turned the swing around to see her. Savanna was happy and laughing and I realized this was worthy of a photo. It pretty much speaks for itself.

Savanna happy about poop in swing

As Savanna navigates this unpredictable sea of difficulty, Daddy turned 40 this month. Yes, I feel older. No, I can longer just decide to do the P90-X Plyometrics workout at will and expect to make it through even half of the routine. No longer can I just decide to go run a few miles, with or without the kids in the stroller. Yes, I have a lot more grey hair – but no need for ‘Just for Men’ yet. Yes, I have another hernia in need of surgical repair. But, there is good news: I don’t need the little blue pills yet! The kids and mommy felt it necessary to make a cake and actually put 40 candles on it. Nice.

During all of this discomfort, Savanna has made strides in development. This has a somewhat calming effect for me, as I know this would not be happening if seizure activity was occurring in the manner it was prior to surgery. Right now, we have to stay focused on that fact. We have obtained the compression vest to help her with the awareness of herself as she moves around. It is like a custom wet suit that is adjustable in terms of tightness of fit. Savanna now has custom AFO’s (ankle-foot orthotic) that helps prevent hyper-extension of her knees when she stands. She is much more responsive when she sees you in the room and when you call her name. This is really a feel-good reaction from Savanna that both mommy and daddy needed to see finally after this surgery. It could not feel better when while not looking at you, you call her name, she turns to locate you, makes eye contact, smiles, and proceeds to crawls toward you and going to tall kneeling with her arms out and up to hold you. For some reason, I never thought that was actually going to happen with her. Then comes the inconsolable crying and fussing. Her movements and behavior when you hold her sometimes is like trying to hold a 25 lb mealworm. She is just everywhere, every which way, and really low tone at times.

Savanna decked out with a compression vest, AFO’s, and a medical walker.

She is crawling all over the house now. This is amazing to see, and is causing me to have to put things up off the floor in area’s she never bothered to explore or care about. She has climbed 2 steps unassisted (…and then… fell backward as I knew she would, so I caught her before she landed on her head. “Good daddy, good!” as Brandon says.)

She is standing now on her own with the help of furniture – meaning she can transition from sitting to standing on her own. This is a milestone for sure. I have attached a video showing her standing on her own. For me it was breathtaking while also very rewarding. This particular clip was about 5 am, and I heard her active in her room. Austin (her twin) was still asleep, so I flipped the light on to capture this sequence. Because of her visual field cut, I don’t think she saw me until I said something.

Going from laying down to sitting up, to standing up, all have their mechanics of motion. And those mechanics are broken down in movements or elements. The training includes correctly positioning the correct limb, at the correct time, with proper weight transfer, etc., etc. Learning to dance is similar when you really break down the movements. We have to teach Savanna each element and how to put together the movements to accomplish the overall goal, sitting up, standing up, walking, etc. It is amazing how normal kids just do it naturally and without any trained guidance. It is a good outward expression of how Savanna’s brain is different from a normal brain. Learning other skills require a similar approach.

Speaking of normal kids, we have been able to witness some normal twin activity that was never really seen in our house in the past. It has been great to witness even if it only lasts a minute or two. Austin sometimes mimics Savanna’s bunny hop crawl, and she gets the biggest charge out of that. Wish I had some video of one of those moments to share, but it starts and stops very abruptly and I just haven’t been quick enough on the draw to record it yet. Here are a couple of pictures of a rare during the day bath time for just the twins while the big boys were at school.

Austin, close up…

In the tub fun, without the big kids. A rare moment worthy of a photograph for memory.

She really likes tilting her head toward the resected side, and actually putting it on toys and such. She continues to do this a lot and we are not sure how to interpret this activity, especially when she is usually so happy when she does it. She also is increasingly insistent on banging her head against walls, mirrors, floors, table legs, etc. She does it repetitively, so she will crawl over to a door with a low glass panel, move to tall kneeling, and just start bumping her head against the window. It progresses to the point I feel the need to intervene. I think this may be sensory related, but are not sure. A protective helmet is on order as she is getting really aggressive with this activity.

Since her second surgery, Savanna gets pleasure from putting her head/ear on whatever is around her.

Unfortunately, Savanna is still battling something in her throat – or at least I think she is anyway. She makes funny sounds when she is sleeping, kind of whistle-like. When she gets really worked up, she has significant retraction in her throat. Her swallow sound is really labored and noisy. It is almost like when you have a really sore throat, and all seems well, then you swallow – ouch. A swallow function study recently indicated Savanna is in fact aspirating thin liquids, what a surprise. I had already ordered the infusion pump as a last resort option, and had been bolus feeding her through the G-Tube since surgery because my daddy instinct indicated she was having problems. Plus she refused to suck after the second surgery and that to date has not changed. So bottle feeding is over and done with for her. She has a follow-up scheduled with her ENT soon that will shed light on the situation I hope.

She is otherwise very healthy and just generally really unhappy about half of her awake time. It is somewhat concerning for us as parents, and we are really hoping her situation is just circumstantial regarding her teeth. We have already asked for guidance once from her doctors. Thus far, I think this just may be the way it is for some time yet. I noticed during the swallow function study that her 2 year molars are right there too. (This is a study done with x-ray ‘always on’, so there is plenty of time to see a lot of things in her head.) I don’t see bulges on her gum in those areas, but the teeth are right there. So, this could be a long six months or so. God watch over her.

-dad

Our baby is home….

Posted on April 8, 2013 by kenlininger

Savanna was discharged early Friday morning and we made it home by lunch time. It was so nice to be home, feeling like we were back in the driver’s seat again with her care. Savanna is doing better, but still has a long way to go in terms of recovery. She is in a lot of pain, and the best we have to manage the situation is ibuprofen and Tylenol.

The past few days have been rough trying to manage her pain. It comes and goes. We seem to get good moments all of a sudden, and then as quickly as it became good, it deteriorates into crying and fussiness. She has absolutely no interest in taking a bottle, so we are back to bolus feeds only (through her feeding tube). This is tricky when she is just agitated beyond consolation, and squirming like you would not believe.

I decided to make some adjustments to her medication to see if we could level out her mood without too much sedation. I split her onfi back to TID (tri-daily) versus morning/bedtime only. And I went ahead and started scheduling .5mg of ativan, TID. This has helped tremendously. She is much happier and playful, with a lot less ibuprofen. Once we get completely off the dexamethasone (steroid for her stridor), that will help this situation too.

She used to crawl by doing a bunny hop, versus alternating opposite leg/arm movements and now she is crawling like a normal baby. Albeit not much, but when you see it, it takes your breath away at first.

She is eating, like a horse. She is chewing well. Against normal intuition as a parent, dicing food into incredibly small pieces enables babies to simply swallow food versus chewing. One piece just the wrong way and she chokes or her gag reflex kicks in full force. So, the O/T (occupational therapist) we have recommended giving her larger pieces, so she would not naturally want to swallow the food, but rather naturally realize she has to chew it first. Even if she doesn’t swallow it at first, that’s okay, she is getting over the aversion to having food in her mouth. This has been a long process with Savanna. A process that honestly, we almost didn’t even notice with our other kids – that is how different they are from a developmental standpoint. It is all good.

One step at a time.

These guys are three pees in a pod. They are the best of brothers, and worst of enemies when it is time for ‘warfare’ – aka ‘playing wrestle’.

Tristan, Brandon and Austin are such good helpers. They love to do things for Savanna to help us. Most of the time, it is a good help. Sometimes, like when Brandon wants to spin Savanna on the sensory swing, things get a little out of control. I picture in my mind puke spraying out from Savanna splattering the walls all around her as she spins wildly…. and then… well, I slow down the swing of course. We have yet to see the puke, but I have to admit, she likes to spin.

They just crack me up with their antics and how Tristan (the oldest) can interact so well with Austin, (Savanna’s twin and the youngest). He is so protective and makes sure Brandon does not hit him too hard, or ‘wrestle’ him too hard. They drive me to my wits end, especially when Savanna is having a really difficult time. Then they turn right around and do things that are so touching and so thoughtful.

Sometimes, I have caught myself making parenting mistakes with Tristan (oops! did I say that out loud?) Yes. When my patience is the thinnest, I act my least thoughtful. I am so focused on something going on with Savanna, that I have said things in a voice that was not called for, but was heard. These situations are difficult.

Like seeing a picture you shouldn’t see…you can’t ‘unsee’ it. Once I hurt his feelings, it can take a long time for him to get over it no matter what I do or say.

In the beginning, he (Tristan) would ‘forgive’ (more like forget) pretty quickly, but not now. He is older and smarter. His feelings are genuinely hurt, and most times it goes into the next day.

I am learning as a parent and person in this situation. It is still a work in progress, but this ‘stay-at-home’ situation is far more difficult than I thought it would be when I signed up for it. With that, of course, goes the reward far beyond what I could ever achieve in any company, at any level.

Seeing Savanna learn and progress, knowing that I am big part of that effort, just can’t be explained in words. There are not words that I can put together to characterize the emotions involved, especially since this last surgery.

So, overall, Savanna is progressing and doing well. We are really anxious for the next few months to see what happens. We continue to learn as a family what it takes to care for someone like Savanna. More challenges are forthcoming. Just as I have said before, God is preparing us for the future with the situations of today.

A special thanks goes out to Grandpa Squiz, Grandma Lou, Mama Barbara, and McKenzie and Eric for being there when we really needed help. They allowed us to devote 100% of our time to Savanna during the operation and recovery period in the hospital. Their help was a blessing.

Breathe Eat Poop

Posted on April 4, 2013 by kenlininger

Savanna is three days post-op since her second resection for epilepsy. It is too early to tell about her seizure activity, but we see activity that is very concerning from our perspective. We have been told to give it another 3-4 weeks before sounding the alarm.

Savanna is moving around now, and her demeanor is dominated by discomfort and irritation versus acute pain and rage. This is good.

Still in the hospital, here we sit. Like waiting in the in the airport terminal and the boarding status just keeps getting delayed. Then delayed again. Then delayed again. Ugh! The reasons to continue to keep Savanna in the hospital seem convoluted and mired in bureaucracy. We know our daughter. We understand their genuine concern. But life in the PICU for someone like Savanna (and her family) is corrosive. She is ready to go play, roll around, and be a slightly-delayed toddler. The PICU was supposed to be a pitstop for her for the first 12 hours and then she was to be moved on to a more outpatient facility for monitoring and released 12-24 hours later. What a disaster ensued when we were admitted.

The unexpected respiratory situation confined Savanna to the PICU for 3 days, tipping the balance of the our emotions resulting in a more combative relationship with the staff than one that needed to be harmonious. She was having problems I admit, but looking back, the initial 12 hours of care should have been in the PICU and then Savanna should have been moved to the general floor for care. Maybe some of the nurses/doctors out there who follow this blog can elaborate on what I am saying. Or, maybe I am off base too, and by all means let me have it. Comments are not moderated. But, the PICU staff is used to seeing a certain type of clientele. Being the #1 trauma facility in the region, most patients here are completely different than Savanna (and so are the parents). By day 2 in the PICU, we had scuffled with 3 doctors and 2 nurses. Looking back, I am not sure why it took 2 whole days to get in that much trouble – it could have been accomplished in less time.

The doctor overall in charge (the neurosurgeon) had been called twice in the middle of the night during the first two days too. These unfortunate actions create an environment of ‘us against them’. The heat from the friction generated at times is tremendous. And the sad part is that it didn’t have to be that way. The thought that any one person knows 100% of what a baby like Savanna needs is naive. I say that without callus and arrogance, but rather a humble realization that the above statement includes me (the parent) too. Near the end our crime and punishment in the PICU, I asked the neurosurgeon, “What is required for Savanna to be discharged?” He replied simply “Breathe, Eat, Poop.” He elaborated, but I thought it was a great title for a blog post.

I was ready to take Savanna home now, but I also knew a brief stay in the EMU was more or less required. I tried to wiggle out of it, but the sentence was handed down and time served was not enough punishment. Savanna’s journey continued in the EMU. Luckily, the EMU is like ‘Celebrity Hospital for the Rich and Famous’ when you are there for observation without EEG equipment installed. It can be a desolate place too with little to no activity at night – very unlike the PICU. What is odd though, is just when you think it is safe to pick you nose, you realize you are being watched through the video system. You are reminded by the sound of the discretely placed camera panning and zooming under remote control of the EMU nurses. Haha.

Breathe.

No it is not a call for us as parents to take a deep breath, as though the person holding us underwater decided to let go and allow us to surface. Savanna has to maintain Oxygen saturation level of at least 90% on her own. Savanna is still struggling somewhat with stridor, which is due to the swelling in her upper airway. This is a potential result of being intubated for nearly 12 hours. It is not permanent, and will take some time to dissipate. It has to be remedied or markedly improved for discharge. As of now, the ENT doctor and neurosurgeon are happy with how she is breathing and feel this is not holding her back from moving forward. We checked this box.

Eat.

So, with her right vocal chord not closing all the way, there exists significant risk of aspiration if she were to spit up or vomit. It presents a delicate situation with respect to feeding her given the nausea from the pain medications and anesthesia. If she is enraged and hyperactive due to pain, she could aspirate while feeding. If she is sedated too much, she could aspirate while feeding. Dilemma. The neurosurgeon does not want her sedated post-operative as it could mask presentation and symptoms of complications. But, if she can’t be safe in a crib that looks more like a cage more than a place to sleep, what is the point. His answer is just to try to console her and redirect her pain with magic. We need her to be manageable and not in so much pain. We have four children, I can’t just sit with her for hours at time and try to keep her from being in pain with a little tylenol. There should be some common ground here, and I feel like I am being fought tooth and nail about keeping my daughter healthy while not in a lot of discomfort. And, the weird thing as this was not the case at all after the first surgery. Maybe I am missing something as ‘just the parent’. I don’t feel like an irresponsible parent, though there is some innuendo in the conversations with the different doctors. I have to say it is not appreciated after all we have been through with Savanna.

How does this rant apply to feeding? Okay, so if she really is in pain, it is really difficult to feed her. Bolus feeding is difficult to impossible as well. She has a G-tube, why can’t we just use that you might ask? Savanna does have a peg style g-tube, but not a fundo. So she can still eat normally as the stomach doesn’t have a ‘reverse flow’ limiter – if you will. So even bolus feeds are in question now, which I think is reaching too far in maintain Savanna’s admission. Even during Savanna’s most difficult times, sedated far more than now, she never had a problem with reflux with bolus feeds. But my historical experience as a parent is being ignored for the most part. I know why, but it is still frustrating, especially when I am right.

So, I still have a speech therapist consult scheduled tomorrow to prove I can feed my child safely. Amazing. An empty box that I have to get checked.

Poop.

I wore my lucky ‘poop’ shirt to the hospital and Savanna had an extra dose of miralax this morning. This box is checked, a couple of times over I might add.

Breathe, eat, poop. Breathe, eat, poop. Kind of sounds like a meditation phrase. Let’s go Savanna, find your happy place!

-dad

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

Posted on April 3, 2013 by kenlininger

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.

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.

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.

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

-dad

Savanna's Journey

Infantile Spasms. Partial Epilepsy. Surgical Treatment. ASD.

Author Archives: kenlininger

About kenlininger

4 Months Seizure Free and Counting…

Sunshine After the Rain

Recovery… in the Shadows

Field of Laughter

Each Day there is a Climb and a Summit.

Exome sequencing in epileptic encephalopathies – a classification of de novo mutations

Milestones: Savanna progresses while Daddy falls over the hill…

Our baby is home….

Breathe Eat Poop

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