Your Critically Ill Child: Life and Death Choices Parents Must Face - Chapter One

Chapter One
Running the PICU Marathon – A Parent’s Challenge

[ To accomodate a web display, Chapter One has been broken into six sections. ]

Section One of Six

Critical illness strikes children suddenly and few parents are ready when it does. We are not ready because our children are a complicated package of endearing vulnerability mixed together with resilient indestructibility. One minute we are overprotective of them and the next minute we are naively unknowing about what they are doing. The range of serious illnesses and injuries that can happen to children is terrifyingly broad. Yet in spite of this minefield of potential dangers, nearly all children somehow escape them to become parents themselves. So although we do fear for our children, we also take comfort in the knowledge, even the expectation, things will turn out fine--and things usually do.

Sometimes, however, serious illness and accidents strike, and children need help when that happens; they need parents who know what to do and where to turn in a crisis, and they need the experts and the sophisticated capabilities only found in a pediatric intensive care unit, a PICU. The PICU is a marvel of high-tech wonders, and this chapter’s story will show you those wonders. Robert’s story will also show you that, for many children and their families, the PICU experience is like running a marathon--a physical, mental, and emotional marathon. The great challenge is to complete this marathon successfully. To do so parents must be alert and active participants in all aspects of their child's care, from finding and obtaining the best possible care to deciding how and when to use it. Robert’s story, like all those in this book, will help you do that. At the end of his story, as with all the others in this book, I summarize some key points about how to learn from his experience.

Robert was an only child who lived in a small city with Gail, his mother. Like most children, Robert had been generally healthy during all his five years of life. Of course he had suffered a few ear-aches, experienced an occasional fever, and had a rash or two, but he was generally an active, healthy boy. All of that changed one Sunday when Gail did not hear her son up and playing in his room as he usually did first thing in the morning. She went to check on him and saw he was still in bed and appeared to be sleeping. When she aroused him he was groggy and did not seem to be himself at all. He was disoriented and confused, did not answer questions coherently, and did not even seem to know where he was. Something was clearly very wrong with the boy, so Gail brought him to the emergency department of her local hospital to find out what it was. He vomited several times in the car on the way there.

The doctor in the emergency department examined Robert and found him still incoherent and confused. The child also did not react normally to bright lights shined in his eyes and would not obey simple commands, such as “squeeze my hand.” The doctor noted a few other things besides Robert’s mental confusion. For one thing, the child’s skin was yellowish, jaundiced. In addition, his liver was larger than normal. In a child Robert’s age, one normally feels the edge of the liver just below the ribcage on the right side of the belly. The doctor felt Robert’s liver edge extending further down into his abdomen, making the organ about twice as big as it should be. Robert also winced when the doctor pressed down to feel his liver, indicating the organ was also abnormally tender.

Gail was confused and afraid, as any parent would be. Yet it is important at a critical time like this for parents, in spite of their fears, to understand what the exact problem is with their child. So what was wrong with Robert? Several blood tests soon showed, at least in a general way, what his trouble was; his liver was inflamed, a condition called hepatitis.

When most people hear the word hepatitis, they think of the common forms of that illness, typically caused by infection with a virus. By tradition, scientists have assigned these illnesses letters rather than names: hepatitis A is caused by contaminated food or water, and both hepatitis B (once called “serum hepatitis”) and hepatitis C are transmitted by contaminated blood or needles or sexual contact. But there are many other viruses besides these three that can cause hepatitis, a term that really means nothing more than “inflamed liver” in Greek. Many cases of hepatitis are not even caused by viruses at all, since a wide variety of other diseases, medicines, and toxins can inflame the liver.

Most forms of hepatitis are quite mild, and often affected persons do not even know they have anything wrong with them unless they happen to have a blood test done. Robert, however, clearly had a severe kind of hepatitis. Judging both by how sick he was and how deranged were the blood tests of his liver function, he had what we call acute fulminate hepatitis. This term is only a descriptive one. It does not really tell us anything about what was causing Robert’s illness.

The doctor in Robert’s home town hospital was very worried about the child’s situation, and he called me to talk over Robert’s case. I shared his concern and I suggested we transfer the child to our PICU. Robert’s mother agreed. We needed to do it quickly, since it appeared likely he would get worse, possibly far worse, before he got better. It was even possible he would not get better; Robert’s liver was failing rapidly, and severe liver damage can be permanent or even fatal. I arranged to have Robert flown from his local facility to my PICU.

This flight was Robert’s first encounter with high-tech medicine. He was to have many more during the ensuing weeks. Medical aircraft are equipped as a sort of mobile intensive care unit, and they are typically staffed by transport teams specially trained in the needs of critically ill children. Team members are in constant communication with the medical experts at their base of operations, so such teams are often able to begin sophisticated treatments even before the child reaches the PICU. Depending upon the needs of the child and the travel distance, the aircraft can either be a helicopter or a standard, fixed-wing airplane.

Robert’s condition had changed by the time he arrived in the PICU several hours later. Earlier in the morning he was listless and confused; when he arrived in the PICU he was agitated and combative. In fact, he was hallucinating and did not recognize his mother. I gave him a sedative medication to calm him down through the intravenous line running in his hand, and he dozed off in his bed. I then did some emergency blood tests, rechecking his tests that measure liver function, as well as adding some additional studies to see how bad his situation was. I also did some tests to see if Robert had inadvertently taken one of the several drugs or been exposed to one of the chemicals that can cause liver failure--he hadn’t.

Robert’s test results came back quickly, and they looked ominous. All his numbers were worse than they had been only a few hours before. Robert’s general condition was also looking increasingly ominous. His level of consciousness was deteriorating, meaning he was lapsing into a coma. It was clear I soon would need to use on Robert some of the high-tech medical life-support capabilities we have in the PICU. Before it all ended, my colleagues and I would need to use nearly all of them. But before you meet that technology and learn about the treatments, we need to go over a few things about what the liver is and what it does. This illustrates an important point of this book: parents need to participate actively in their critically ill child’s care, and to do so they need to understand their child's illness, particularly the parts of the body affected and how they function.

In Robert's case the organ was his liver, a crucial organ for the body. It has several key functions. One of these is to remove from the bloodstream the toxic things we may eat and drink, such as alcohol, as well as the waste products from the body’s natural processes, such as occurs with the natural death and recycling of body cells. The liver is also a factory for the manufacture of most of the proteins that circulate in our bloodstream. Chief among these are the blood coagulation proteins, which are key components of the delicate system that both makes our blood clot normally in the right places and prevents it from clotting abnormally in the wrong places. A third vital function of the liver is to maintain our blood sugar in the normal range between meals. Our livers do this by steadily releasing sugar (in the form of glucose) into our bloodstreams as our other organs need it. Our hearts and brains in particular need a predictable and constant supply of glucose. A fourth important function of the liver is to make bile and to secrete this vital substance from the liver into our intestine via a tube called the bile duct. We need bile in our intestines in order to digest many fats and absorb certain essential vitamins.

As the afternoon and early evening of Robert’s first day in the PICU unfolded, he showed all the signs of fast developing acute liver failure. He fell deeper into a coma because his liver could no longer clear body wastes and toxins from his bloodstream, particularly ammonia. As his blood concentration of ammonia rose, he became ever more deeply comatose because that is what too much ammonia does to the brain. He began to have difficulty keeping his blood sugar level in the normal range, and he needed a constant infusion in his vein of a very high concentration of glucose to make sure his brain and heart received enough. In addition, his liver’s ability to make the all-important blood clotting proteins became steadily more and more impaired. This meant he would require frequent infusions into his veins of fresh plasma, which contains those proteins, to keep him from bleeding to death.

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