Re-entry, part 2

OK, I ended my last post describing our Great Medical Adventure in between flights home to Albuquerque and promised to pick up where I left off on the second leg of my trip. In case you were concerned, we didn’t hit the Bermuda Triangle. Jila and I did in fact make it home and we’ve had a great week of getting back into our groove.

To be honest I struggle with how much to share about this whole saga. This is the kind of stuff I usually handle privately. And it doesn’t really have to do with self-employment or small business. But it has shaken up our lives like an earthquake and I feel I need to just share the basic facts of what happened before I can move on writing-wise.

So, to recap: My 6-year-old daughter Jila developed hepatitis (i.e. inflammation of the liver) last June, but the strange thing about it was that she tested negative for every typical hepatitis-causing virus: hep A, B, C, Epstein-Barr, etc. Another possible cause of hepatitis is ingestion of a toxic drug or poison, but Jila hadn’t taken any medications or been exposed to anything toxic that we could determine. (Believe me, I tried every crafty way I could think of to get her to come clean with me about anything she had ingested that she was afraid to tell me about. I promised her endlessly, for weeks, that she wouldn’t be in trouble if she told me about something she “just remembered” she ate, like something that she found at the park, or in a friend’s family medicine cabinet, anything. We came up empty.)

Another possible cause of hepatitis is autoimmune and metabolic disorders. With autoimmune hepatitis, the body’s immune system attacks the liver. With metabolic disorders, the body fails to metabolize things correctly leading to liver damage. Our family doc didn’t seem to think these sounded likely.

So with hepatitis diagnosed but no known cause, our family doc referred us to a pediatric gastroenterologist, and they jointly agreed the most likely explanation was that Jila had contracted a less common virus that causes hepatitis. They said there were hundreds or thousands of viruses that can affect the liver, and Jila probably just caught one of the more unusual ones. The plan was to wait to see if it would clear on its own, with blood tests every week or so to track things.

The GI doc also tested Jila for markers of autoimmune and metabolic diseases, which came back negative. But the doc told us that with autoimmune disease, negative test results aren’t 100% definitive. To paraphrase, you can never completely rule out autoimmune disorders. But so far, everything had been negative.

After a couple weeks of continued worsening in early July — by far the most stressful two weeks of my life — it finally all started to come down. By the end of July her bilirubin levels were normal, and the enzymes were much improved, but still had a ways to go until normal. She was acting and eating almost completely normally during this time, so our GI doc said just to keep getting blood tests, monthly at this point, to track the inflammation. But at every appointment she expressed concern that the levels were coming down too slowly, indicating something unusual was going on. Most non-chronic hepatitis viruses clear within just a few weeks and Jila’s inflammation was very stubbornly holding on even two months later.

Then in October the enzyme levels, indicating inflammation, started climbing again. They did a liver biopsy. The results showed mild hepatitis, mild scarring of the liver, and no signs of autoimmune disease. Still no indication of what was causing it. By mid-November, her enzyme and bilirubin levels were spiking and she was turning yellow.

While I trusted (and continue to trust) the competence of our specialist in Albuquerque, it had chronically been difficult to communicate with her office. The only way to contact her was to leave a message on a voicemail system and hope they called back, which they often did not. As Jila’s condition rapidly worsened in early November (including a very scary plummeting platelet count) the unreturned calls became unbearable.

So I did some research, learned that Cincinnati Children’s hospital had the nation’s top pediatric gastroenterology department in the country, and sent four doctors there an email with details about Jila’s case. All responded within hours. I had a phone meeting the next morning with one, Dr. John Bucuvalas. He offered to see Jila right away, as soon as medical records could be transferred, which his staff coordinated seamlessly. We were on a plane 2 days later, on Thanksgiving morning. We finally were cleared to come home last week.

It was a very intense two months. The team at Cincinnati Children’s hospital was incredible, particularly in their level of coordination and communication. Within a week of arriving Jila was admitted as an inpatient to get her started on anti-inflammatory steroids, which she tolerated well and which continue to help bring her enzymes back to normal. We have a little ways to go with the enzymes, but her bilirubin level has now been normal for a couple weeks.

Besides treating the hepatitis, there was a lot of work done — like, tons and tons of testing — to determine what is causing the hepatitis. Even though Jila had tested negative for markers of autoimmune disease, other blood test results were leading the docs to believe there was some sort of immune system dysfunction going on that might be to blame. Dr. Bucuvalas brought in immunologists who did an unbelievable number of blood tests, looking at genetic and immune system components that might explain the hepatitis. Many of the tests took more than a month to process, and some are still pending. They signed our whole family up for a research study and we all had blood tests (including Jasper who was a trouper) to see if there are any familial issues that could explain Jila’s symptoms.

So far, nothing. The immunologists said early on they might never find a “smoking gun” and so far, none has been found.

But the important thing is that Jila is doing great. We are adjusting her medication in preparation for tapering her off the steroids, which have nasty risks and side effects, especially when used for long periods. The main side effect is bloating and weight gain, and Jila has indeed gotten quite puffed up. But other than that, her energy is completely normal and she’s in excellent spirits, happy to be back home and back to school. Beyond having to get her blood drawn a lot (and the IVs in the hospital were no picnic) she never complains a bit about the whole thing.

I’m so incredibly thankful for the team in Cincinnati, especially Dr. John Bucuvalas and his RN/Care Manager Jennifer Willoughby. They and the other doctors and nurses were nothing short of heroes to me during some very long days in the hospital. Spending time in a children’s hospital is a life-changing experience and seeing some of the other kids there, day after day, really puts things into perspective. I also have been so grateful to have our amazing family doctor, Dr. Mark Unverzagt, at our side throughout this. I just feel incredibly fortunate to have been able to pursue the care we did and to have such amazing support from our family and friends.

So there you have it. I’m getting back into work and have a very cool venture that’s almost ready to announce. I hope to be blogging more regularly, and will likely continue to try to make sense of this experience and how it fits into my self-employed life.