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

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

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.

There are times when a child’s situation is such everyone concerned can sit around a table and talk things over. At other times, however, this is not possible because events are moving too fast. Robert’s situation was of the latter sort, and these situations are often the most terrifying to a child’s family. In rapidly evolving scenarios like Robert’s, I try as much as possible to explain to families what is going on as it is happening.

This sort of discussion on the fly, however, lays bare the inherent uncertainty surrounding much of what we do in the PICU. We proceed with the best information we have at that moment, but we recognize new information may appear or events occur that send us down an entirely different treatment path. That is the reality of critical care practice. Hindsight is said to be twenty-twenty; in contrast, it is often very unclear to us in the PICU what the next minutes, hours, and days will bring as they unfold. Foremost in my mind was the fact that, although I knew Robert’s liver was failing, I did not know why.

Yet in spite of the urgency of the situation, I knew I very much needed to take some time to talk to Gail about what was happening to her son, the boy who had never really been sick before in his life. This is a crucial task. To do so is not merely a favor to the family; it is the doctor’s responsibility. No matter what the circumstances, parents must get the opportunity to ask the doctor tough questions.

I sat next to Robert’s bed while Gail lay in it beside him. She was long past being upset and distraught; she was numb. She had eaten little for twelve hours, subsisting on crackers and coffee from the nurse’s station. She had driven hundreds of miles chasing her son in the aircraft. She was terrified by what was happening. She was also tortured by what she believed to have been her own negligence, her responsibility for Robert’s condition.

Many, many parents blame themselves as Gail did for not recognizing their child’s critical illness in its early stages. Blame, though, is not relevant here. What is most helpful is for parents to recall as best they can a timetable of events, of what happened when. For example, Gail realized now Robert had not sickened quite as suddenly as we first thought. She told me, in retrospect, Robert had not been quite himself for the previous two days. He seemed “pale” to her, which she now knew was actually the early stage of jaundice, of liver failure. He also took a nap during the afternoon for several days previously, something he had not done for years. She berated herself for not taking Robert to the doctor then.

I did not know what to tell her, other than reassure her she had done nothing wrong; the early signs of many serious illnesses are quite subtle. She didn’t hear me–there really is no balm for that sort of pain. After I had listened to Gail for a time, I told her what I needed to do for her son.

The first thing Robert needed was a secure way to breathe, since his ability to do that on his own was rapidly deteriorating. He needed one of the most common articles of high-tech medicine we use in the PICU–a mechanical ventilator, a breathing machine. The machines we use are called positive-pressure ventilators. (This distinguishes them from negative-pressure ventilators, the “iron lungs” used during the polio epidemics of the nineteen-fifties.) The first positive-pressure ventilators were primitive devices, being essentially nothing more than big bellows filled with air and oxygen that blew their contents into the child to expand the lungs. In contrast, our current machines are extremely sophisticated, with onboard electronic systems that not only breathe for the child, but also tell us a great deal about what is going on in the child’s lungs. They can even sense what a child’s own breathing is doing and respond appropriately.

A mechanical ventilator is high-tech, but the process of placing a child on a ventilator is decidedly low-tech, and has changed little in fifty years. The first step is endotracheal intubation, inserting the breathing tube that will connect the child’s lungs to the ventilator. This is not easy. If I were simply to push the endotracheal tube into Robert’s mouth and down into his throat, it would most likely not go where it needs to go–the windpipe, or trachea. Rather, it would pass down into his esophagus, the swallowing tube leading to the stomach. Unrecognized placement of an endotracheal tube in the esophagus, rather than in the trachea, is one of the most dreaded catastrophes that can occur during intubation.

To get the endotracheal tube in the right place, I use a device called a laryngoscope. This is nothing more than a curved metal blade with a light on the end so I can see into the trachea. With the child lying on his back, I first use my left hand to place the smooth-edged blade in the child’s mouth and with it move the tongue out of the way. I next manipulate the tip of the blade to lift up the epiglottis, a flap of tissue that protects the opening to the trachea by preventing food from getting into the airway when the child swallows. When all goes well, I can do this maneuver in nearly one quick motion, thereby exposing the opening to the child’s trachea. Once I can see where to put the breathing tube, I then use my right hand to pass its tip between the child’s vocal cords and on down into the trachea.

I then remove the laryngoscope from the child’s mouth and connect the end of the breathing tube, which now protrudes from the mouth by a few inches, to a hand ventilator, a simple device with a soft bag full of oxygen I squeeze to force air through the tube and down into the child’s lungs. This tells me the tube is in the right place.

Being intubated is extremely uncomfortable. Indeed, it is essentially impossible to manage to do it on a fully conscious child, although some adults can be intubated using only light sedation and some medication to numb the tissues in the back of the throat so the tube will not cause them to gag. In children, particularly very young ones who cannot comprehend what is happening to them, we routinely use medications to put the child completely asleep and relax the muscles. These drugs carry significant risks. In fact, potential disaster lurks at every step of the intubation process. In Robert’s case, however, everything went well.

Once I had placed his breathing tube in the right place, I connected it to a mechanical ventilator and adjusted the settings on the machine to give him a satisfactory breathing pattern. I then attended to some of the other life-support and monitoring equipment he needed. Since he was in a deep coma, he could not urinate normally and needed a plastic tube, a Foley catheter, put into his bladder to drain urine. He also needed yet another plastic tube, a nasogastric tube, passed through one of his nostrils and down into his stomach and connected to a suction device, since without this his stomach and intestines would become bloated with air. Later on in a child’s course we often use these tubes to give liquid feedings into the stomach.

I also needed the nasogastric tube to see if Robert was bleeding into his stomach, since this is a common problem in patients with liver failure. In fact, he was bleeding profusely from the lining of his stomach. I knew this because after I passed the tube through his nose I put a syringe on the end and pulled back on the plunger; nearly a cup of fresh blood came back into the syringe. This was serious and required immediate treatment with fresh plasma, which contains the needed blood clotting proteins his liver was not making. I also gave him a drug that blocked his stomach’s ability to produce acid. This medicine helps decrease the bleeding by reducing the irritation stomach acid causes to the stomach lining.

I next needed to place two kinds of long, thin tubes, or catheters, in Robert’s blood vessels. Robert needed these “lines” to monitor minute-to-minute what was happening with the function of his cardiovascular system and with the amount of oxygen in his blood. The lines also allowed me to give Robert all the many medications, blood transfusions, and plasma transfusions he needed. Additionally, they allowed me to take blood samples from Robert without the need for additional needle sticks. For one of these lines I used the femoral vein in his groin as an entry point to pass the ten inch long catheter into his inferior vena cava, the large vein in the center of his body. For the other I used his radial artery, the artery in his wrist where one normally feels the pulse.

Whether parents should be present when these things are done to their children is a subject of some controversy. My own practice is to try to gauge how the parents might react and I then give them the option of staying or not. Over the past decade, I have noticed more and more parents choose to stay with their children. If they wish to stay and see the procedure, however, it is important for the physician to explain everything that is happening. I have found it is also important to have the parents sit down, since I have had more than a few parents get queasy or even faint to the floor when they see such procedures being done to their child. On the other hand, many parents have told me their imagination of what their child might be going through was worse than seeing the real thing, so they were glad they stayed. Their presence may even help their child tolerate these procedures. If you feel you need or want to be present, make your wishes known, even if your child’s doctor does not ask you about it.

Gail felt she would be too nervous and frightened to offer reassurance to Robert while I did these procedures and so she chose to wait outside. When she got back I met her at the door to prepare her for what she would see. If your child’s problem is similar to Robert’s, you will be confronted by the sight of plastic tubes coming out of the nose, mouth, groin, and wrist, and wads of tape holding all these things in place. The chest will be plastered with electrodes that go to the heart and breathing monitors. The eyes will appear slimy from the artificial tears we use to protect the eyes of patients who are too sedated to blink normally. There will be a lighted probe on the finger or toe to tell us the blood oxygen level. The ventilator will hiss periodically and the room will be filled with strange beeps and alarms.

All this technology is familiar to PICU workers, but no one else is really ready for a sight like that. Yet within a few hours Gail, like most parents in this situation, gathered her courage and found the strength to look past the tubes and lines to see her son lying in the bed. We are truly an adaptable species.

Once I did all the things Robert needed immediately, I turned my attention to the most important issue, which was what had happened to Robert and what was wrong with his liver. To find that out I needed help from my expert colleagues, particularly those who specialize in liver disease. Since the liver is primarily a part of the digestive system, the experts in liver diseases are those physicians with special training in digestive diseases, the pediatric gastroenterologists. One came to see Robert the afternoon the child arrived in the PICU. He went over all the tests we had done thus far, examined Robert, and spoke with Gail. Although he could not be sure, the gastroenterologist suspected Robert had a severe viral infection that had attacked his liver.

The reason he could not know for sure is because diagnosing viral infections is not easy. Viruses grow very slowly in the laboratory, if they grow at all. Instead of growing the virus, we often diagnose viral infections indirectly by testing the blood for evidence the child has been exposed to a virus recently. These tests, however, also take time and they are not universally reliable.

The best way for us to determine what was wrong with Robert’s liver was to get a sample of the liver itself, a biopsy, both to look for viruses and to see under the microscope what was happening to the liver cells. Liver biopsies can be done in several ways. The most common and easiest way is to pass a long, thin needle through the skin and into the liver. The needle has a device inside it to take a small piece of liver and hold it securely while the needle is removed. Although the piece is small, it is usually large enough for our purposes.

Another way to biopsy the liver is for a surgeon to do an operation called a laparotomy–open the child’s abdomen and cut out a wedge of liver tissue for study. A variant of laparotomy is called laparoscopy, in which the surgeon makes a smaller incision near the liver and through it inserts into the abdominal cavity a laparoscope, a device about the size of one’s finger, and with this snips off a piece of the liver. Of these, the needle biopsy is the least complicated and safest to perform.

Robert, however, was too sick to have any of these procedures. He was having too much trouble with bleeding because his liver, which normally makes the proteins that cause our blood to clot, was too diseased to make those needed clotting proteins. The liver is prone to bleed even under normal circumstances when its surface is nicked or poked. Doing a biopsy on a patient who is already bleeding is highly risky.

It is true we could have given Robert large doses of clotting proteins in the form of blood plasma, a procedure which would have transiently improved his clotting situation and briefly lowered his risk of bleeding. We sometimes do that. However, the gastroenterologist told Gail that to care for Robert at that moment we did not need the information the biopsy would give us, so doing the procedure then was not worth the risk. This is a key point for parents: nothing in the PICU is risk-free, so when a doctor wants to do a procedure on your child, always ask if the risk of doing it outweighs the risk of not doing it.

While the gastroenterologist completed his evaluation, Robert was getting worse. I was already giving him plasma to reduce his bleeding problems, high doses of glucose to keep his blood sugar in the acceptable range, and several medications designed to reduce the amount of ammonia in his blood. But despite my efforts, Robert’s coma was getting deeper. I could tell this by testing his brain function using some decidedly low-tech procedures: shining a light in his eyes to test what his pupils did, brushing a wisp of cotton on the surface of his eye to see if he blinked, pinching him to see if he moved, and touching his tongue with a wooden stick to see if he gagged. I also did a much more sophisticated test, a computerized tomographic scan (CT scan) of his brain. This test, which is a kind of x-ray of the brain, showed Robert’s brain was swelling, a condition called cerebral edema. If it continued unabated, it would kill him.

Cerebral edema in children with liver failure is a well known problem. The main reason it is potentially so deadly is because of the brain’s anatomy. The brain is completely enclosed in the hard case of the skull, armor which protects it from blows to the head. But this means if the brain begins to swell, there is no place for the swelling tissue to go; it pushes against the constricting bony cage of the skull in a way that can block blood from flowing into the brain.

The concept is simple: for blood to get into the brain, the pressure inside the skull must be less than the blood pressure outside the skull because blood, like any fluid, will not flow uphill. And if the brain does not get a constant supply of blood, the brain cells will be severely damaged. Even worse, if the pressure inside the skull gets too high, the brain itself may be forced out the bottom of the skull, a catastrophe called brain herniation. It is always fatal, and it is a common cause of death for patients with severe cerebral edema.

Unfortunately, our treatments for severe brain swelling of the sort Robert had are meager. We can give one of several medications that help draw the fluid from the brain, and I gave him one of those. It seemed to help for the moment. Extreme treatments, such as removing part of the skull to relieve pressure on the brain, have been tried by some physicians in these situations. Although doing this makes intuitive sense, my own experience in the past with that sort of intervention has not been good.

I did ask a neurosurgeon to evaluate Robert to see if he had any suggestions to offer; he did not think any brain surgery would help. Moreover, Robert’s bleeding problem from his liver failure was so severe the neurosurgeon also did not even want to take the risk of placing in the brain a monitoring device we often use to guide our therapy, a device which tells us exactly what the pressure is inside the child’s brain. The surgeon feared placing the monitor would cause catastrophic bleeding in Robert’s brain.

So, only twenty-four hours after Robert arrived in the PICU, I had to tell his mother he was on the edge of death. What could be done? The only thing that could save Robert was to find him a new liver–to get him a liver transplant. We needed to take his destroyed liver out and give him a new one. Unfortunately, we had little time to find Robert a new liver because the experts estimated we had only a few days, perhaps a week at most, before Robert would die of liver failure.

Organ transplantation in children is now common. Improved surgical techniques, better ways of preserving organs for transplant, and better regional and national organization of organ transplant networks have made organ transplants more frequent. Probably the single most important advance in organ transplantation technology was the discovery of a family of powerful drugs–the first of which was cyclosporine–to prevent the organ recipient from rejecting the newly transplanted organ.

Even though organ transplants are now common, they change a child’s life forever. As a parent, if your child needs such a transplant, you need to get all the information you can on how, where, when, and why. Quiz the experts called in to consult on your child’s case, but do some investigating on your own as well. Carefully used, the internet is an excellent information source. Look for reliable sites, such as those sponsored by major children’s hospitals. The internet is also an excellent place to look for support groups made up of parents who have gone through the same ordeal as you.

Some organ transplants are done by having a living person donate the tissue. This is now the case for over half of kidney transplants. Liver transplants, however, are only rarely done using living donors, and the very notion is a controversial one because, whereas donating a kidney puts the donor at little risk from the surgery that removes the organ, there are substantial risks to donating part of one’s liver. Some donors of liver tissue have even died. For this reason liver transplants are generally done using all or part of a liver donated by someone who has died.

Even though there are many more patients waiting on transplant lists than there are organs for them, there is a priority system such that patients in dire need move to the top of the list, and Robert certainly qualified for top priority. Early in his second PICU day, our surgeons listed Robert in the national transplant database requesting a liver for him.

Meanwhile, Robert’s condition steadily worsened. His blood ammonia level was difficult to control, his blood sugar levels were dangerously low in spite of giving him the maximum amount of sugar in his veins we could, and his bleeding problems were so bad he required a near constant infusion of blood plasma. To make matters worse, Robert’s kidneys were starting to fail as well. There was nothing intrinsically wrong with his kidneys, but children in liver failure often develop this additional problem.

Hardly an hour passed in which I did not need to intervene in some way to keep Robert alive because he had life-threatening derangements in his blood oxygen, his blood acid, his blood potassium, his blood sugar, or the amount of water in his circulation. His brain continued to swell. He had several convulsions, or seizures. He oozed blood from his nose, his mouth, his lungs, his intestines, and the holes in his skin where needles had been.

Robert’s mother had been at her son’s bedside this entire time. Like many parents in a situation like this, she was terrified to leave her child for fear he would die when she was gone, even if only to take a shower or get a meal. Gail was also dealing with a problem many parents find a difficult one when their child is critically ill in a large PICU–an overabundance of doctors. It is good to have so many experts to help and give advice, but the inevitably conflicting interpretations of what is going on and what to do can be unbearable for parents. Nevertheless, you must steel yourself to hear it, ask questions and get information. (You will read more about this in the next chapter.)

What I and most of my intensivist colleagues strive to do to alleviate this problem is to be there at the bedside when each of the many experts speak with a child’s family. In Robert’s case, this list included the gastroenterologist, the transplant surgeon, the hematologist (an expert in bleeding problems), and the nephrologist (an expert in the kidney). After each of these consultants had weighed in with their opinions, I interpreted for Gail what they meant and how it all fitted together in the big picture of Robert’s situation.

Pediatric critical care is in many ways like an old-fashioned general practice, because, like the old-time general practitioners, I and my colleagues must deal with all aspects of a child’s problem. And minding the high-tech machinery is only one of those aspects. In fact, for some children in the PICU, the technology plays only a minor role in their care. This is the paradox of intensive care: nowhere is humanistic medical practice more important than in medicine’s most technologically sophisticated setting. Robert’s situation shows the truth of this statement.

Gail was progressively falling apart under the strain. She was a twenty-two year old single mother, had no other children, and had no close family members living nearby. Robert’s father was not an active parent, and she had not spoken with him for several years. Gail’s own mother lived far away in another state, although she planned to come to be with Gail as soon as she could.

Gail suffered from a constellation of problems we commonly see in families whose children are in the PICU, and which we lump together under the term “PICUitis.” The cause is a combination of stress, fatigue, fear, and information overload. Chronic sleep deprivation, not eating enough, and worrying take a fearful toll on the body. I usually see families get “PICUitis” beginning on about the second day of their child’s stay in the PICU, particularly if the child’s situation is one of constant crisis such as was Robert’s. Gail had a severe case.

Most PICU staffers do what they can to get families to eat, sleep, and shower, but most parents find it difficult to leave their child’s bedside. Remember, if you are to be the best help to your child you must take care of yourself, eat adequately, and get sufficient rest. In our PICU, like most, there are no set visiting hours, and we encourage parents to come and go as they wish. This policy is a good one, but sometimes it is difficult for parents to accept we do not expect them to be there all of the time.

Gail, like many parents, felt she needed to be there constantly. If your child is in a PICU, know that parents often feel helpless in such high-tech surroundings. Yet even if you are only holding your child’s hand, only you can really give that special sort of comfort to your child. Like Gail, many parents feel some responsibility for their child’s illness, and this makes it even more difficult for them to take a break from watching over their child. It is important, though, to get away, if only for a short time, to revitalize and help you cope in a very difficult time.

If you find yourself in Gail’s situation, you also need to cope with information overload because you will be bombarded with information. Some of this will come from doctors, nurses, and other PICU staff. You may glean some of it from sources as varied as the monitor screens on the equipment in your child’s room and overheard conversations. Understand that, although this abundance of information can be confusing, even frightening, it is overall a good thing. Some parents sit at their child’s bedside and surf the internet on their laptop computers, searching for any information they can find on their child’s condition.

The relationship between physicians and patients has been evolving over the past decades from the paternalistic, “doctor knows best” view of fifty years ago, that parents should be told little about their child’s case, to a viewpoint that stresses parent’s involvement, even control, of all decisions about treatment. Although this can be taken too far–doctors must not use it to shirk their responsibilities–this is overall a healthy trend.

I believe parents of children in the PICU need to be told all aspects of what is going on with their children. Parents, however, still need to make decisions with clear guidance from physicians. That guidance comes from the physician taking the time to sit down with parents at the bedside, not by standing near the door, glancing at the clock. Insist on your physician giving you all relevant information about your child. If necessary, request a scheduled time to meet. No matter how hectic the situation, parents of a critically ill child should feel as if they have my complete attention.

The next time I sat down with Gail in her son’s room we talked about many things: liver transplants, what the future might bring, when her mother might come, whether she should continue to try to locate Robert’s father. I gave Gail the advice of which I earlier spoke: having a critically ill child in the PICU is like running a marathon, and she needed to find a way to pace herself, to hold some emotional capital in reserve to spend during the coming days and weeks. This is advice I strongly pass on to you.

Gail also felt an ambivalence about organ transplants I have encountered before. Parents of children waiting for a transplant realize for their child to live, another person must die. So even though they hope every day for the transplant to happen, they wrestle with the sad feeling that this may amount to wishing for someone else’s death. This situation is especially poignant when a child dies in the PICU whose parents wish to donate their child’s organs, there is another child waiting in the PICU for an organ, and the two are a match. In one case where I saw this happen, although the PICU staff tried very hard to be circumspect about what was going on, the respective families found out. The bond that developed between those families was one of the most profoundly moving things I have ever seen.

As events turned out, Robert was extremely lucky. He spent about a day and a half waiting on the transplant list before an appropriately matched liver became available for him in another city. Our transplant group quickly assembled a team to travel by jet to that city to get the liver while we in the PICU readied Robert for the operation as best we could, although he continued to be quite unstable. But although he was in poor shape to have a major operation, we had no alternative.

To save precious time, the surgeon typically begins the operation on the recipient before the donor liver actually arrives in the operating room. However, the surgeon does not proceed to the point of no return, that of removing the child’s old liver, until the new liver is actually there and verified as clearly an appropriate one for the child.

The time the operation actually takes to complete varies a great deal. Often the most difficult part for the surgeon is removing the old, diseased liver, because it may be scarred tightly into the child’s abdominal cavity. But Robert’s old liver, which was by then shrunken down to a fraction of its healthy size, was easy for the surgeon to remove. He placed Robert’s new liver where the old one had been, connected the donor blood vessels to Robert’s vessels, and sewed Robert’s new bile duct into his intestine. When all this was finished, Robert returned to the PICU, where Gail waited anxiously in his room with Robert’s grandmother, who had just arrived.

Meanwhile, Robert showed something we occasionally see in children who receive a liver transplant; within minutes of having his new liver connected to his system, Robert began to get better. By the time he got back to the PICU from the operating room his blood chemistries and blood sugars were already much improved. By the next day his blood clotting problems had markedly improved. In fact, one of our great concerns just after liver transplant is that a child’s blood clotting problems will have improved so much the child’s system clots off the vessels leading to the new liver. We do various things to lesson the chances of this happening, but it is always a risk.

Robert had no such problems and by two days after his transplant operation he awakened to the extent I was able to take him off the mechanical ventilator machine. Later that day he smiled and spoke to his mother for the first time since that initial morning in the emergency department. His new liver was working just fine.

Over the next few days Robert continued to get steadily better. We were able to take some of his lines out and even to let him start drinking sips of juice. Everything was going great until the morning six days after his transplant, when he had a fever. Fever is particularly concerning in children who, like Robert, are taking medications that suppress the immune system. These medicines are what allow us to do transplants, since they suppress the recipient’s immune system just enough so it will not attack and reject the new organ, but not so much the recipient’s immune system will be totally unable to do its job of fighting off infections. Achieving this balance is tricky in practice, and children like Robert are especially vulnerable to all sorts of infections.

I started some tests to see if the fever might be caused by bacteria in his bloodstream, started Robert on several antibiotics, and waited for the test results to come back. By the next day the tests showed Robert did have a serious infection in his bloodstream. He also was getting sicker. He had a high fever, his blood pressure dropped below normal, his kidneys began to fail, and he became short of breath and needed oxygen to breathe. By the next day, he was again critically ill.

He was so sick I had to put him back on a mechanical ventilator because his lungs had rapidly filled with fluid. I also had to connect him to yet another high-tech machine–an artificial kidney machine–because his own kidneys were not working. He needed infusions of several potent medicines in his veins to keep his heart working adequately. After all Robert and Gail had been through, what had happened to the poor child now?

Unfortunately and unluckily for Robert, he had developed a condition that goes by several names, but which is most commonly called sepsis or septic shock. Sepsis is best described as an out-of-control inflammatory state that affects the entire body. There are many things that can trigger the disorder, but bacteria in the bloodstream, such as Robert had, is the most common of these. Once the condition is fully underway in a child, it can cause derangements of most of the organs of the body, two of the most important of which are the lungs and the kidneys. This is what happened to Robert, and it made him as sick as he had been before his liver transplant. And as was the case with his initial liver failure, sepsis is a frequent killer. So, in a sense, we were back to square one.

This turn of events was particularly cruel for Gail, who had sat next to her son’s bedside for a week as he endured all of this. If your child is in the PICU you, like Gail, will need to walk the emotional tightrope the PICU demands of parents: hoping for the best while preparing for the worst.

Gail pasted pictures on the wall of Robert’s room to show all the doctors and nurses, and to remind herself, what her child looked like before he was sick. She went from almost never sleeping during the time of his transplant, to dozing continuously in the recliner chair in Robert’s room. She did this partly because she was still sleep-deprived from her son’s and her ordeal, but it seemed to me she also did this as a way to defend her psyche from the surrounding chaos; if she was asleep, no one could approach her with yet more bad news about her son.

Parents are never ready for the shock of how their children can look when critically ill in the PICU. In spite of this fact, nearly all parents handle this extremely difficult time with grace and courage. And Robert’s appearance was indeed frightful. He was once again comatose, this time in part from the sedative medications we had to give him to enable us to use the ventilator machine. Once again he had plastic tubes and lines coming out of everywhere in his body. His skin was still yellow from the jaundice and was covered with old bruises. But now his whole body was bloated, his skin shiny and stretched because his kidney failure caused his body to retain too much fluid. His face was particularly affected by this, and his eyelids were swollen shut. Some of the fluid oozed out through the edges of the huge incisions on his abdomen from his liver transplant and from places in his body where lines and needles had previously broken his skin.

Robert was connected to machinery even more complicated and sophisticated than he had required before his liver transplant. He was tethered via large catheters into his blood vessels to a complicated apparatus that helped his kidneys try to deal with all the excess fluid and the waste products in his body. His mechanical ventilator was different from the standard one we used before his transplant. Now his lungs were so stiff with fluid he required a breathing machine we call a high-frequency oscillator. Unlike his previous ventilator, which hissed quietly in the corner as it breathed for him, an oscillator sounds like an unbalanced washing machine on spin cycle. It is so loud what most parents notice when it is finally not needed is the eerie silence that follows, so accustomed have they become to the noise. In Robert’s case, the settings on his oscillator were turned up nearly as high as they could go, and still we were only barely getting sufficient oxygen into his bloodstream to keep him alive.

Robert endured this condition for over a week, living quite literally on the threshold of dying. Hardly a six hour period passed without some new crisis with his blood chemistries, or wild swings in his blood oxygen levels, or some issue with his life support machinery that needed adjustment. His need for constant infusions into his veins of medicines to support his heart was so marked that any interruption in these infusions, even for the few seconds it took to make necessary changes in the pumps, caused his blood pressure to plummet.

In spite of it all, his new liver continued to work. That was the ray of hope for his mother and those who took care of him; we knew if we could get him through these sepsis-related problems, we still had a chance to cure him. I expressed that hope to Gail every day. If you ever find yourself in a situation like Gail’s, the key question to ask the doctor is: “Is there still hope?” Know that there usually is.

Finally Robert began to get better–not all of a sudden, but gradually and very steadily day-by-day. We were not really aware at the moment any corners had been turned or thresholds crossed. It was only by looking back that his steady improvement became clear to all of us. The first small step on Robert’s path to recovery was he quit being the center of attention in the PICU. He no longer had the dubious honor of being the sickest child in the PICU, and I could go and get dinner or coffee without the expectation my beeper would summon me back to his bedside with some new crisis. Gail could leave the hospital for a few hours without fearing catastrophe. After a few more days, she even could spend the night sleeping in a real bed outside the hospital.

Robert’s recovery then accelerated. His kidneys improved to the point we could remove him from the artificial machinery, and his swelling resolved so he looked like himself again. His new liver cleared the yellow jaundice from his skin. The wounds from his transplant surgery healed. His lungs were healing, too, and I was next able to take him off the oscillator ventilator machine and place him on a standard ventilator. He was still deeply sedated with medications, however, so we could not tell if his ordeal had caused him any permanent injury to his brain, as sometimes happens. As Robert’s lungs improved further, we began the steps to take him off the breathing machine, a process aptly termed “liberation from mechanical ventilation.”

Over the next few days we reduced the doses of Robert’s sedative medications and turned down the settings on his ventilator. This had the effect of waking him up and asking him to take over more and more of his own breathing; it weaned him from the machine. With a patient such as Robert, we often do not know how this weaning process will go until we try. Some children are so weak from their illness (particularly if they needed a ventilator for many days) they will not be strong enough to breathe completely on their own. These children continue to need prolonged help from the machine for some or all of the day until they build their strength back. Other children take a very long time for the sedative medications to wash out of their systems and they are too unaware to breathe normally until that happens.

In Robert’s case the weaning process took about five days. At the end of that time I pulled the endotracheal tube from his mouth and he breathed fine on his own. He was weak and groggy, but he clearly recognized his mother, who was at last able to hold him again without a nest of tubes, lines, and wires getting in the way. By the next day Robert could drink some clear fluids like juice, and by the day after he actually ate a more regular diet without problems. He even got out of bed and sat in a chair with Gail for a few hours. By then the only medications he was taking were those he needed to prevent his body from rejecting his transplanted liver. He was safely back from the edge of the cliff.

Robert owed his life to high-tech medical machinery, procedures, and medicines, and his story shows what the PICU and its marvelous wonders can do. Mere hours after he first got sick, an aircraft staffed by a specially trained pediatric transport team flew Robert to the PICU. There complicated life-support machinery and medicines kept him alive while he waited for his liver transplant, a procedure which required the skills of dozens of highly trained individuals. His subsequent episode of septic shock would have killed him were it not for additional sophisticated machines and the skills of still dozens more of highly trained doctors, nurses, and technicians. Twelve years later he was in high school, complaining his mother would not let him drive their car to the big city by himself. She thought it was too dangerous.

Robert’s story encapsulates several of the principal challenges for parents of children in the PICU. Chief among these challenges are understanding just what the PICU is and what can and cannot be done there. Yet it takes more than just this understanding for families to successfully run the physical and emotional marathon that PICU care can be for them and their children; it takes active parents who question, get information, and help make crucial decisions for and about their critically ill child, aided by the guidance of their child’s doctors and other caregivers. It also takes patience and faith. The rest of the stories and information in this book will show you why that is so and give you crucial advice and insights if your child ever needs PICU care.

CRUCIAL ADVICE FOR PARENTS

• Be alert and active in finding and gaining the best possible medical specialists and hospital to care for your critically ill child.
• Ascertain from experts just what the exact problem or condition is, in terms that you understand.
• Research your child’s problem on your own so that you understand the function of the parts of the body and the organs affected.
• Keep a record of who is caring for your child, what is being done, and why.
• Insist that your doctor take the time to discuss with you all important information.
• Take care of yourself so that you can properly care for your child; get adequate rest, nutrition, and exercise.