ketogenic diet

For now the Keto is a no-no

Liam had a long slow burn of a seizure tonight. It started slow with a twitch and a full body spasm for only a few seconds every three or four minutes until it clustered about ten minutes after it started. The nasal versed we’ve been trialing to break larger events worked just fine though, and he drifted into his drug induced haze right at his normal bedtime. If he’s going to have a big one, that is just about the perfect time for it. So aside from his normal twitches that’s two for the day today,  this morning’s was much milder.

You may recall that in late July Karin and I elected to admit a healthy Liam into the hospital to transition him onto the ketogenic diet. Hoping that the high fat, no carbohydrate diet and the ketones his body would produce as a result would reduce the quantity and/or severity of his seizures. Within days we were seeing a change. Cranky and miserable while his body fought what instinct told him was starvation, by the end of that first week we saw a few seizure free days and returned home hopeful that this diet really would help Liam reduce the amount of seizures and as such, anti-seizure meds he has always been prescribed.

Just about 14 hours later Karin and I were admitting a very sick Liam into the emergency room for what would end up being a 26 day stay in the intensive care unit to battle septic shock, the ileus (intestinal obstruction) that resulted, a severe increase in seizures, the constant struggles in finding and maintaining IV access including two different central lines being put in (one of them by the surgical team), and the discovery of an enormous blood clot which is the result of the first of those lines. Oh and one other thing but we’ll get to that later.

For the first few weeks of that stay we stayed true to the diet, even when Liam was NPO. instead of the normal D10 or D5 for hydration, Liam’s maintenance fluids were specially made in the pharmacy to give him as little dextrose as possible. His IV meds were specially made because most solutions contain dextrose. The chlorhexidine wipes that hospitals use for sponge baths contain glucose so we went old-fashioned with good old soap and water. Basically, let’s just say that keeping a kid who was sick enough to stay asleep for days and days at a time on  this ketogenic diet was getting a lot more complicated.

While investigating some blood in his stool (I know some of these details are a little  “too much information” but they really do tell the story, ultimately it was another byproduct of the shock and resolved itself eventually) a bright circle appeared on one of his x-rays. A circle that seemed so symmetrical as to appear unnatural it was at first disregarded medically,  The assumption being that it was one of the leads to Liam’s heart monitor or a piece to the in-line suction tube of his circuit. A few days and a few more films later, the diagnosis surprised us all. Gall stones. A ton of them. Little crystals that when his gall bladder was tasked with breaking up such a high fat diet  rose up in revolt and clustered together into a mass to protest.

Freaking gall stones at three and a half.

Now I don’t think I need to explain how severe a gall bladder surgery on any three-year old would be nevermind a child as medically complex as Liam. Lucky for us, the medical consensus was not to do anything. remove the strain on the gall bladder and they should spread out again and not cause Liam any pain I was told. So I asked another doctor, and then another, not believing that finding a gall bladder full of tiny stones wasn’t a more major problem that would require at least some kind of intervention. Come to find out, no one was even all that surprised.

All told, by my very rough estimate, I think Liam has spent about 6 to 8 months of his life on TPN and Lipids, nutrition through an IV instead of his gut. Of course nothing is without its side effects and apparently gall stones is one of them so to find out that they were there was hardly a shock. They were there the whole time. The only reason that we know they are there now is because Karin and I took our healthy little Liam into the hospital to try the ketogenic diet.

A diet.

Adjusting for weight every half-year or so, day in and day out Liam eats the same amount of calories. Pumping through his g-tube exact amounts of protein, fats, and carbs in his formula Liam’s diet for his entire life had been designed to make it as easy as possible for his body to digest. He has grown so big and strong and since we hadn’t had a hospital admission in just about a year Karin and I were convinced that now was the time to try the diet.

Liam is no longer on the ketogenic diet. Until he is strong enough that we consider removing the gall stones (and with them probably the whole gall bladder itself) he will not be able to resume the ketogenic diet. Sepsis and blood clots, staying on seizure meds and giving injections of blood thinners, I feel guilty about trying this diet every single day.

I think I always will.

But even living with the constant guilt, I know that we made the right choice in trying.

We knew what the risks were going in. We were the ones who approached our neurologist to try. Seizures suck enough, the long and short-term side effects of pretty much all meds to control seizures suck just about as bad. To think that there was an option to control seizures and reduce the amount of meds Liam required simply by changing his formula? A case could be made that we would have been negligent in not trying it. Right?

That doesn’t make it hurt any less when I think about everything Liam went through for us to find out. I know he understands. I know he’s a fighter and wants to beat these seizures as much as any of us do. He tells me with his eyes. That doesn’t make me sleep any easier remembering what he had to go through in that intensive care unit. There was no reason that anyone could have predicted the events that transpired from this change. That doesn’t make the thoughts go away that I somehow should have seen it all coming.

*****

The universe works in amazing ways.

Liam’s NICU stay was 153 days long. In a teaching hospital that’s 5 months of residents rotating in and out every 30 days. Among them when Liam was born was Dr. S. just beginning her residency and in her first rotation. About 10 months later she started her rotation into the PICU. At the time Liam was about halfway through a 109 day stay there. The following year Liam was admitted for a virus that attacked his GI and who should arrive to consult when the GI team was called but Dr. S now onto that rotation. During his last few admissions though, Dr. S would just come by to say hi to her buddy Liam whenever she saw his name on the patient list.

Liam has a hematologist now. Cross another specialist off the list. He is now a patient of the hematology clinic at the Tomorrow Fund of Hasbro children’s Hospital. Last week we went in to asses the level of his blood thinner and go over any other concerns of the three to six month course. We weren’t there to meet with the attending of course but the fellow. The fellow, a year-long assignment instead of a month. The end of being in medical school and the beginning of being a doctor. (technically they are “doctors” the whole time but you catch my meaning.)

So it should not surprise anyone at this point when in walks the hematology fellow — Dr. S.

For us and for her it was coming around full circle. Liam had been a part of her entire med school career. She even remarked about it to the attending who popped in towards the end of the appointment.

“Yeah, you’ve been a part of Liam’s team his whole life.” I said.

The attending looked at Dr. S and then back down at Liam and said ” Oh she’s not alone. I’ve looked over his chart. I bet there are a whole lot of doctors out there who this boy has taught something to.”

She’s right. There are. And Liam is an excellent teacher.

The connections that we make with other people mean something. Something important. That is no less true for a person like Liam who can’t make a connection verbally. He can’t sign and for the most part he can hardly smile (with his mouth. Liam smiles with his eyes and anyone who has seen it can tell you that it has the power to change the way you look at the world.) but the connections and bonds that Liam can make above all those obstacles mean something, to him and to us.

During that last hospital stay Karin and I were able to meet a parent whose child had just been trached. We were able to give our experience in how we are able to manage a trach and vent at home. That means something. We were able to meet a doctor we hadn’t yet met and although Liam was not her patient she would visit him and us, and we would talk for long periods about what this life of ours is like. A strong bond of friendship not doctor/patient was quickly formed. That means something.

Any future patients that enter Hasbro Children’s Hospital to try to start the ketogenic diet will be getting an ultrasound of their gall bladders beforehand. Liam did that, and that alone is worth the risk. Maybe the next kid wouldn’t have been as strong as Liam is. He has helped to make sure that other kids don’t need to encounter this problem in the same way. That means something too.

Karin and I brought our healthy boy into the hospital for a week to try the ketogenic diet hoping it would reduce his seizures. A 33 day hospital stay, crazy complications, twice daily injections at home, and we are right back where we started. Only Liam won’t allow me to think that it was all for nothing. It’s his strength that gives me the courage to try whatever we can do to improve his medical needs. Along the way, connections are made that show me how much Liam means to nearly everyone he meets. It’s those connections that I hold on to when I wonder if I made the right move. If it was worth the risk.

It hurts like hell, but I still think that is was.

Until my knuckles bleed.

The text messages came to me at work just as the lunch rush was winding down.

He is vomiting badly — 1:49pm

and then

Need you — 1:53pm

Seven minutes and a run through the underground tunnel connecting the hospital I work in and the hospital Liam is currently admitted later I entered Liam’s room to find Karin and a respiratory therapist put the finishing touches on an emergency trach change with Liam stripped to his diaper and looking a little grayer than his normal pasty pale. (The nurse, who was in the room when it happened, apparently bailed out and left soon after the vomit, showing her inexperience with trachs and vents. This is why Liam should have been admitted to the PICU. For those first important minutes when the risk of aspiration was greatest Karin’s were the only capable set of hands in the room. But anyway.)

Vomit. I suppose that most parents become accustomed to vomit by the time their kids are three. Not us. Liam just isn’t a puker. Today marked the third time the Liam has ever vomited.

Third.

The first two were the result of major and I mean major intestinal blockage. Both occurred during long, long hospital stays. So, when a text message says vomit I go a running.

Thankfully this one was not as serious. No, today’s Technicolor yawn was brought to us by a poorly timed bolus of water that should have been pumped in slowly with his feeding pump but instead was pushed in with a large syringe in a matter of minutes (I don’t mean to harp on her but this was also the result of the same nurse not knowing enough about Liam’s medical needs.) and a lunchtime feed being pushed too quickly afterward. The acidosis of the transition to the ketogenic diet certainly didn’t help matters and the diarrhea he experienced from a med given to help an elevated potassium reading the night before left us with a very dehydrated, grumpy, and did I mention gray little boy.

I’ll admit to being nervous and anxious and then an overwhelming sense of guilt that what Liam was going through was the result of our decision to begin this treatment. If it ain’t broke don’t fix it we always say but Karin and I took a healthy, if somewhat twitchy, little boy into a den of germs and put him through this pain and discomfort. Had we really made the right decision?

It was only a few minutes later when Dr. G arrived. To try to explain the respect, admiration and trust the Karin and I have for Liam’s neurologist who first saw Liam when he was only three weeks old would make this post unbearably long so just know that he is a very calming presence at a time when we needed one. He was followed by three residents who stayed silent and observed while Dr. G, Karin and I discussed symptoms, tests and then the options for nearly every scenario of test result for a little while. Sensing the end of the consult as Dr. G’s beeper went off I asked a question I normally never do since I prefer numbers and hard data over impressions or feelings.

“so, uh…you aren’t . . .concerned about this?” I was pretty sure I knew the answer to the question before I even asked it since Karin and I have done our homework on the diet and this transition and I always knew something like this could happen. At that moment, with Liam lethargic and miserable lying nearly naked in front of me, I needed to hear him say it.

“No this is normal. Episodes such as this are what we expect to see as we make the transition into ketosis. This is the reason we start this diet with a hospital admission.” The words came so easily to him that they instantly neutralized the tension in the air.

Photo by Karin.

The neurology nutritionist working with us entered the room to hear what our new plan was and with the release of all of my nervous, worried energy I called out to her “Hey, this is all your fault! I can’t believe you told us how smoothly this was going just this morning in front of Liam!” I pointed over to Dr. G. “Ask him. Liam hears something like that he’s going to throw you another wrinkle. He doesn’t ever let us off that easy!” The whole room laughed out loud. More than one of us got up to get closer to anything wooden to give it a knock because any mention of things going well, or worse the unspoken H-word*, is only tempting fate. Karin resumed hugging Liam  and the team disbanded to put our newly formed plan to action.

I waited a little while to witness the blood draw and get a few of the results we were looking for before heading back through the tunnel to work confident in the knowledge that all of the people who needed to hear the plan did so (including the capable nurse for the next shift who has been assigned to Liam 3 of the 5 evenings of this admission). Hospitals on their best days are a scary and stressful game of “telephone” where information and orders can change ever so slightly between departments and shifts. I love it when we can have everyone together at once.

After my shift had ended 5 hours later I returned to Liam’s room to find a pink, awake little boy snuggling comfortably in the large hospital bed with his mom. A small iv drip that had already been stopped and the tolerance of two feeds in my absence and you’d find it hard to believe it was the same boy who earlier had struggled so just to open his eyes. Dr. G who is the on call neurologist this weekend just happened to stop by only a little while later. We discussed the plan moving forward and reveled in the reveal of acceptable test result numbers in all of the categories that we were looking for. This time we didn’t have to ask for his impression, Dr. G decided to give it anyway.

“I’m really encouraged with these results. He seems to be transitioning very well. Like I said we do expect little events like this one. How have the seizures been?” He asked.

The kid caught up on some serious sleep this week. Alas, his parents, did not.

“Well It’s been about 36 hours now since we’ve seen anything!**.” We were so excited with the news Karin and I responded in unison. It has been a long time since we could say that.

“That’s wonderful.” Dr. G said with a great big smile “Now someone better knock on some wood!”

And all three of us did. Now you should too.

* the unspoken H word for the uninitiated begins with an h and rhymes with gnome and shall not be uttered until all three of us are in the van.

** We are in no way expecting this treatment to eliminate all seizures and I am positive that they will occur but a stretch that long this early is an encouraging sign that as he continues on the diet we may see a reduction in their quantity and severity. We may even be able to ween from some of the dangerous seizure medicines he is currently taking. We’ll see. Right now I am hopefully optimistic.

Elective not Emergency

The loud thunk of an ambulance door as it closes sounds equally ominous from either side. Inside on the bench seat, struggling to hold Liam’s hand and stay balanced as the EMT keeps checking vitals, or in the car, speeding behind trying to make it to the hospital as quickly as the rescue unit does. Unfortunately, Karin and I have been in both postition a few times each. Right now though, with Liam due to be admitted to the hospital in less than 12 hours, I almost envy those adreneline fueled roles.

Emergencies don’t give you time enough to stress. To worry. To obsess.

Tomorrow morning, Liam is being admitted to the hospital to start the Ketogenic Diet (go here to learn more about the diet) which hopefully will help control his seizures. He will be admitted for 3-5 days but I’m banking on 5. Given our history with hospital stays, hell I’m crossing all of my fingers and toes and HOPING for 5. Let’s just say that we know how quickly things can change in the big house and having two weeks to plan for this stay also means we’ve had two weeks to imagine every possible worst case scenario (and some of the impossible ones too.)

The possibility that Liam responds to the diet and we see a reduction in seizure activity and maybe even reduce the amount of medications he needs to control them make this hospital stay more than worth the inherent risks involved.

Anyway, as strange as it may sound we’d definitely prefer to stay in the PICU as we have spent so much time there that at this point it feels downright comfortable but I suspect that even though the nurses on the floors are unable to change a trach if the need arises, we’ll be up in gen-pop hoping we don’t get shivved instead of downstairs in solitary.

Don’t worry, this is actually a very exciting time for us all and I am almost giddy at the prospect of this treatment working. I’m trying very hard not to let my hopes get too high. It’s just that the anticipation of returning the family routine into “hospital mode” can be a tough pill to swallow. We’ve had hospital stays that were expected to be days turn into weeks and yes even months before so choosing to spend a week in the joint can seem a little crazy.

As you know I’m not a day-to-day-update kind of blogger. When I find a topic or an event and can think of a way to twist it into an essay then up it goes, but the general details of our lives go mostly unmentioned until I can work them into something bigger. Hospital stays though, change all that. The overnights next to Liam’s hospital bed or the sleepless nights alone in a much-too-quiet-house, both tend to inccrease the blogging drive and increase potential material so,.. I guess there’s that.

Like I said, I generally don’t like to post about a thing until I delude myself into thinking I have it all figured out. It’s why I can’t figure out how to end this disjointed and awkward post. I really just don’t know enough about what will happen in the next week to explain to you how I feel about it. Right now, I have nothing figured out. Just a whole lot of worry and a desire to fast forward the next 5 days away so my family and I can all be home again.

By then, probably, I’ll have it all figured out again.