(Today's post is a bit disjointed. it has been several days since I have slept)
Sunday was a picture perfect autumn day for a long ride and Spike was a far better passenger than can be expected of any four year old. Maintaining his ketogenic diet is a challenge while travelling and certainly slowed the trip down bit he handled it well.
One the highlights of the drive from Raleigh to Cleveland is a tunnel through the mountains near the Virginia/West Virginia border. Spike had never been through a big tunnel so he waited with great anticipation as we drove along the eastern side of the mountains. Every time we came upon a particularly big hill, I asked him if he thought we could go over it and he always replied, “I think we’d better go through that one!”
His remained seizure free the whole morning and enjoyed the trip. We finally came around the last bend and faced a solid wall of color. Perfect timing for him to finally see the mountain with the tunnel, It just could not have been prettier. Unfortunately, that’s right when he had his first seizure of the day. The seizures generally last only 10-15 seconds but can put him out of it a bit longer. I could see him struggling to shake it off so he could enjoy the final approach to the tunnel. He finally said, “It’s very pretty“ before giving in and closing his eyes. I admit I was crushed by the timing of the seizure as I had really looked forward to sharing the experience with him. A little while later we came to the second tunnel but he was well into a seizure cluster by then and could no longer enjoy it.
A couple hours later, he had recovered his spirit and we pulled off to make his dinner at the Ohio welcome center. I had stopped at this rest area before and knew that it had a nice grassy hill that he could run on to burn off some energy. Little did I know that he would have laser accuracy in finding every low wet spot. Within minutes he was covered with smelly, slimy mud. Of course, that’s a four year old boy’s dream so he was very happy.
We arrived in Cleveland around 9 PM. Spike was especially hyper and that’s never a good sign. Sure enough, the seizures started around midnight and didn’t stop until 5 AM. Watching your kid have one seizure is bad enough. Watching as they continue every five minutes for five hours is indescribable. Fortunately, he slept through them. I did not.
Monday morning, he checked into the Cleveland Clinic and promptly introduced himself to all of the nurses as “Mr. Smooth.” He had come up with that name during his last stay at Duke Hospital. He asked me to show him his EEG tracings and then pointed out different points and asked if they were called spikes. He then identified the seizures as particularly spiky. He lay back on the bed, thought a bit and then declared that from that point forward he wanted to be known as “Smooth,” not Spike.
His new doctor quickly found out that he has not yet earned the nickname, at least as far as brainwaves are concerned. During her very long interview with us, she asked for the characteristics of the typical seizure. Being a good little patient, Spike promptly had two seizures so she could see for herself. To te doctor’s credit, she did not recoil in horror as so many others have done. She took a very clinical view of the activity and narrated the seizures. I certainly appreciate that as shock is not a trait I am looking for in his medical team.
The doctor went through the usual questions and laid out the likely test that be run during his stay. However, unlike others, she was also willing to give an early read on him, subject to the test results. She feels that he is likely headed to brain surgery in the very near future. She also tentatively believes that he has a reasonable chance of full success. Reasonable really means something greater than 50% but never all that much greater. That’s both hopeful and sobering.
The staff at the Cleveland Clinic, for the most part, seems capable of putting together a plan and then executing on it efficiently. We’ll see as the test schedule unfolds if that holds up but the indications are good for now. They seem ready to test and treat him promptly without the usually multi-month delays you can experience at other hospitals.
There was a bit of a mix up regarding his ketogenic diet. I was told very clearly last week that the kitchen was fully prepared to administer the diet and would simply deliver appropriate meals all ready for him to eat. That certainly did not happen as food service seemed caught completely off guard. To be fair, the dietician that normally handles the ketogenic patients is on vacation. Nonetheless, the system just doesn’t seem setup to handle the diet. It was hard to even find raw ingredients they could supply. Cooking the food proved difficult as well as the kitchen wouldn’t touch it and there was no refrigerator or microwave for patient use. The nurses could and did store our food in a refrigerator and microwave things for us but we had no direct access and that just makes the whole situation extremely difficult.
For those you of you not familiar with the diet, it is both simple in concept and difficult in execution. The general idea is to have 4 units of fat or protein for every 1 unit of carbohydrate. Sounds simple but that translates into eating a lot of butter, drinking heavy cream mixed with even heavier oil and then having tiny bits of meat and fruit. To make matters worse, everything has to be measure to the tenth of a gram. If you waver from the diet, you risk falling out of ketosis and thus causing even worse seizures. You cannot simply stop the diet, you must wean yourself slowly off of it.
By ten o’clock Tuesday morning, we still couldn’t get food service to give us anything we needed (and we needed very little as we had come prepared with almost everything). Even getting a single raw egg proved improbably difficult. The staff, to their credit, tried to accommodate us but were simply prevented from being effective by the way the whole system was set up. For instance, the reason we couldn’t get an egg was that the hospital kitchen never cooks raw eggs! You had to go to some mysterious special kitchen across the street where those always dangerous eggs were kept in hiding.
In the midst of all this, our savior stepped forward . The nurse practitioner for the juvenile epilepsy unit is an efficient bundle of energy and, more importantly, knowledge and she has the full authority to make just about any decision. When she heard what was going one, she simply said “Stop the diet. Now.” Well, that seemed to go against everything we had read or heard from others EXCEPT for the one scholarly article that found on the topic. A Johns Hopkins paper published through the NIH found no difference in seizure activity related to slow vs fast vs very fast termination of the diet. The nurse practitioner said, “He had 70 seizures yesterday. Are you afraid he’s going to have a couple more? Just stop the diet because it’s not working for him anymore. We do this all the time and it’s not a problem.”
Erring on the side of slight caution, she suggested a modified Atkins diet for all of one day. Basically, don’t give the kid a donut first thing. We chose menu items that were low in carbs but gave up all the micro management involved in the ketogenic diet.
When lunch arrived, with a flourish, I announced to Spike that the diet was over and he was going to have a cheeseburger. Now you should understand that this trooper never once complained about the diet. He drank his cream, ate his butter, swallowed that vile oil, turned away candy offered to him by other kids, watched people around him eat all the good stuff and simply endured his fate without a whimper. Ever.
When Spike pulled the cover off his lunch plate and saw that steaming burger with melted cheese, he immediately said, “This isn’t a cheeseburger! Where’s my bun? And I want French fries on the side!” He then grabbed an apple and did his best to stuff it in his mouth whole. He ate the burger without complaint. Heck, he ate most of it without swallowing! When there was nothing left, again from a abundance of caution, I asked him to eat a couple pats of butter to keep his ratio from sliding too fast. “No WAY! No more butter!” was his reply. I guess he had been internalizing all of his frustration these many months. What a guy.
Following his lunch, we girded for an onslaught of seizures as his ketone levels plummeted. Mr. Smooth refused to cooperate with our expectations and spent the afternoon completely free of seizures. I think he did it out of spite for having endured the diet so long after it was no longer effective. Of course, that happy result could not persist and the seizures did return after dinner. They appear slightly more severe than before but only someone who has watched nearly every one of 2,200+ seizures could discern tha change. I expect the abrupt termination of the diet will have no lasting ill effect and it will make him so much happier and easier to manage.
Speaking of ease of management, the neurology team here listened carefully to our concerns of medication side effects (more on that tomorrow) and is working to eliminate medications that seem to have no effect. It can take awhile to safely eliminate them but the process has begun.
Tomorrow he will have a PET scan and then Friday he will have an amazing scan called a MEG. More on that tomorrow as well.
It looks like Spike will be hospitalized at lest through Tuesday and there is a possibility that he could proceed to surgery without being discharged first. More on that tomorrow as well. Too many hours without sleep to explain it properly now.