Every time an E. coli O157:H7 outbreak occurs, we get yet another reminder how devastating the bacteria can be, particularly when it causes hemolytic uremic syndrome (HUS). See www.about-hus.com.. And every time we represent a young child with HUS, I am reminded of the story of Regan Erickson, who was sickened in the spinach E. coli outbreak in September 2006 (We represented over 100 victims, including nearly 30 who suffered kidney failure and hemolytic uremic syndrome).
Tiffany and Russ Erickson were just like most Americans until September 2006. Their four-year-old son Regan (pronounced "Ree-gun") was one of many young kids whose future was unalterably and forever changed by spinach. What appears below is Regan’s story. It is a little long, but that must be forgiven. Regan’s illness very nearly cost him his life.
ONSET OF ILLNESS:
Regan’s mother, Tiffany, and his sister, Emma, were both sickened during the Spinach outbreak as well. Tiffany actually fell ill first, on August 28. It felt like cruel timing, given that it was only three days before Emma’s birthday and little more than a week since discovering that she was pregnant with her third child, Maggie, but Tiffany took everything in stride. She had no reason to suspect that she was dealing with anything more than a run-of-the-mill flu, and her primary concern was with the health of her unborn child.
After twenty-four hours or so, however, thoughts began to change about the nature of Tiffany’s illness. Her bouts of diarrhea had grown more frequent and severe, and her abdomen was beset by cramps more severe than labor pains. Then, the evening of August 29, after a particularly painful bout of diarrhea, Tiffany noticed that the toilet bowl was streaked with blood. Up until this point, Tiffany had endured everything with resolute confidence, but this symptom suggested something that she had never before reckoned with.
Tiffany soon underwent a diagnostic procedure called an endoscopy to shed light on what was wrong. Of his wife’s illness, before his thoughts turned to Regan alone, Russ recalls:
We left the urgent care facility and gave the drugs some time to work, but the pain continued to be unbearable. As my concern shifted from the baby to Tiffany I couldn’t stand seeing her in that much pain, tired from lack of sleep, and not able to get comfortable.
Meanwhile, Regan had begun to develop symptoms, and Emma soon would. “We didn’t realize that the illnesses could be related,” Russ recalls, “since Regan couldn’t express his pain as well as Tiffany. He just knew his ‘tummy’ hurt and he began having diarrhea.” Emma’s symptoms began the very next day, September 1.
Everyone in the family was sick, tired, and the children being so young, not knowing how to tell or deal with the symptoms like diarrhea, I was continually cleaning, comforting, and helping where I could, all without Tiffany’s help who is usually the stalwart caregiver. We knew that we had some kind of ‘bug’ but not how severe yet. It presented a lot like flu symptoms, but we began to know it was more serious as the kids, just as Tiffany, began to have blood in their stool, and then blood instead of stool. That is a scary, unnerving experience to see blood when your 3 and 4 year olds are using the bathroom.
Compared to four year-old Regan, the illnesses that Tiffany and Emma Erickson suffered were nothing more than a small current in a raging sea. Nevertheless, to hear Russ describe what his wife and daughter endured is to fully comprehend the aggressive nature of this virulent pathogen. Emma endured many days of an illness more acutely painful than anything her parents had ever seen. But as sick as she was, her older brother was fast-becoming critically ill, and her parents thoughts and attention soon went solely and exclusively to Regan.
During an appointment with his pediatrician on September 4, Tiffany reported that Regan had had twenty bouts of bloody diarrhea that day, prompting endless complaints of a sore stomach. Regan been unable to eat or drink for days, and had developed redness and inflammation in the rectal area due to the frequency and severity of the diarrhea. These seemingly benign signs were immediately concerning to the pediatrician, who immediately sent Regan and his parents to Mckay Dee Hospital’s emergency department. Tiffany and Russ rushed to the ER and carried their son in, having no concept of the road that lay ahead.
Regan remained hospitalized at Mckay Dee for the next two days. Gradually, blood tests showed that he was becoming anemic and losing platelets, and he was also not urinating normally. Soon, stool tests showed exactly why. Regan had been infected by E. coli O157:H7, and he was developing HUS. Doctors at Mckay Dee knew that Regan was critically ill, and they transferred him by Ambulance to Primary Children’s Medical Center (PCMC) in Salt Lake City on September 6.
Meanwhile, Tiffany and Russ had begun the painful process of contacting friends and relatives. Russ’s mother dropped everything and, leaving at 4:00 AM, drove to Salt Lake City from Las Vegas. She recalls:
I arrived at Primary Children’s just after the ambulance arrived. They had just gotten Regan in his room. It was a flurry of activity, doctors and nurses in and out of the room. I was taken aback by his appearance. The last time I had seen him was on the 4th of July. He was playing with all his cousins, excited with all the fireworks, eating bar-b-que, and being a normal kid. Today he was pretty much unresponsive; he didn’t even know I was there. So pale and vulnerable, it broke my heart to see him in that condition. I was also taken aback by the appearance of Russell and Tiffany. It was obvious that Tiffany was still not well. She looked pale and tired. As if she was just barely making sense of the whole bizarre turn of events. Russell looked like he hadn’t slept in days . . . come to find out he hadn’t. He had been holding his little family together for over a week now. The only one of them not affected, the burden of care had been fully placed upon his shoulders. And they sagged at the weight of it all. He was tired; with worry in his eyes, fear in his voice and faith in his heart he told me that they were in the best hospital, with the best doctors and that Regan would be okay.
Regan had a difficult night his first night at PCMC. He had run a fever consistently; he had been nauseated despite Zofran; and he had vomited and suffered from painful abdominal distention all night long. Perhaps more significantly, he had had little to no urinary output all night, his face was swollen, and there were signs that his pancreas had already been affected, in addition to his kidneys, by the shiga-toxins released by the E. coli O157:H7 bacteria in his gastrointestinal tract. Regan’s nephrologist planned to begin dialysis immediately if the Lasix, a diuretic, did not stimulate more urine production.
Tiffany placed a call to her mother, Tonya Peterson, the afternoon of September 7. Tonya remembers:
Tiffany told me that his pancreas and kidneys had shut down. I couldn’t believe it. I sat at work at my desk, crying for several hours, unable to concentrate on my work. I thought that Regan might not make it. I called my dad and asked him to take me to see Regan. I knew I wouldn’t be able to drive to the hospital in my condition. I was too distraught at the thought of losing Regan. He was such a sweet, innocent little boy. He called me “Ma-mah,” his version of “grandma.”
Regan continued his descent toward total kidney failure that night. He had stopped producing urine completely, and his nephrologist ordered that Regan be prepped for peritoneal dialysis. Accordingly, Regan was transported to the operating room on Friday morning, September 8, where doctors placed a spiral peritoneal catheter for dialysis. He also placed a PICC (peripherally inserted central catheter) line in Regan’s right arm to facilitate infusion of blood products, medicine, and IV nutrition. Then dialysis began.
Regan ran a fever all night on September 8 and vomited five times. He produced no urine and continued to suffer bouts of bloody diarrhea, producing 76 ml of mostly blood before 11:00 AM on September 9. Attendants administered morphine to ease the little boy’s immense discomfort. And because he continued to suffer from nausea and vomiting, doctors began Regan on total parenteral nutrition. Dialysis continued with hourly exchanges.
Over the next two days, Regan remained critically ill. He continued to have no renal function, and his hematocrit continued to drop, which indicated progressive anemia. In addition, Regan had consistently elevated blood pressure readings and was overloaded, in fact bloated, from fluid retention because he was unable to urinate.
Russ recently recalled his sense of devastation and total helplessness during this time:
Together with the surgeries, dialysis, and transfusions was a lot of heartache and a lot of pain. As I watched Regan suffering, I felt helpless. He wasn’t comforted by me and there wasn’t anything I could do to fix the situation. I could just watch, wait, and hope that he would pull through this illness.
I also had Emma and Tiffany to worry about. Emma was still having trouble adjusting to what was going on, and she was still regaining strength from her own illness. She couldn’t understand why Regan had to stay at the hospital and have tubes coming out of him all over the place when she had been sick and gotten better without any of that. Tiffany was still recovering, ragged from stress, and she was in a difficult first trimester with all the ailments that come along with that. I was very worried about what I could do to help my family, and it didn’t feel like much.
Regan’s hematocrit continued to drop on September 12, indicating progressing anemia. Doctors ordered that a transfusion of leukocyte filtered packed red blood cells be administered as soon as possible. Regan’s bloody diarrhea continued, but seemed to be improving, and his WBC count dropped as well. Nonetheless, Regan continued to be anuric, leaving his nephrologists little choice but to continue peritoneal dialysis.
By Thursday, September 14, Regan’s medical picture continued to be dire. He continued to be medicated for nausea, hypertension, fevers, to stimulate red blood cell production, and he continued to receive a bronchodilator. He also remained positive, by stool culture, for E. coli O157:H7, and consequently remained under strict quarantine.
Russ’s mother stayed at the hospital, alternating with Tiffany and Russ, at least one of whom was always at Regan’s bedside. She says of Regan’s first week:
The chair made into a bed of sorts and the nurses brought me blankets and pillows. We realized very quickly that the hour drive to PCMC every day was not only costly for the kids but most inconvenient. One of Russell’s cousins lived in the area of the hospital and invited Russell and Tiffany to stay at their house. The Ronald McDonald House had a distance rule that they missed qualification for by just 7 miles. They decided that as long as I could stay with Regan at night, they would go home to sleep in their own bed and then when I had to leave they would go to Russell’s cousin’s house and take turns staying at the hospital at night with their boy.
Throughout the week Regan was so sick that I still wondered if he even knew who I was. He progressed somewhat with the dialysis and his lab tests improved at a snail’s pace. Toward the end of the week they declared him E. coli free and he was allowed to ride in the little red wagon and get out of that tiny room for a minute. Up until that time not only was he confined to the room but his sister, Emma, wasn’t allowed in the play room and his family had strong precautions they were to take to insure that the E. coli would stay contained.
Regan remained very irritable and uncommunicative on Saturday, September 16, prompting an examining physician to describe him as an irritable young lad who had pulled the blanket over his head and his knees up to his abdomen during the examination attempt. Nevertheless, no new symptoms had arisen, and Regan’s hemolytic anemia and thrombocytopenia had begun to improve. Peritoneal dialysis, however, continued just as before.
By September 18, Regan had begun to pass a small amount of urine, and his stools had firmed up. He nevertheless remained extremely irritable and very uncooperative, evidently suffering significant discomfort from his ongoing symptoms. He had begun to moan during the drainage cycle of his ongoing peritoneal dialysis, which had been further reduced to twelve hours per day. But despite the encouraging sign of modest urine production, Regan’s kidney labs had not improved; BUN and creatinine levels remained very high at 62 and 6.1.
Over the next several days, Regan’s kidney lab values continued to fluctuate, so doctors ordered an increase to seventeen hours per day on September 20 due to an unexpected increase in creatinine to 6.7. And on September 22, in fact, Regan again could muster no urine at all. He was anemic and symptomatic with weakness, fatigue, and shortness of breath—all of which convinced his medical team that another transfusion was in order. Dialysis continued as well.
Dr. Sherbotie evaluated Regan the morning of September 23, noting that Regan’s appetite was “clearly” improving with only occasional vomiting. Regan was also able to produce a “substantial amount” of urine that morning. Nevertheless, his lab values indicated ongoing renal dysfunction, and peritoneal dialysis continued with a decrease in the number of hours.
Regan’s nephrologist noted significant improvements during his initial assessment on September 26. Generally speaking, Regan appeared well and “was quiet but interactive.” He had lost weight—significant to the clinical picture because it suggested a reduction in fluid retention—and he continued to produce more urine. Nevertheless, he remained on dialysis.
On Wednesday, Regan’s urinary output continued to increase. He produced 641 ml (3 ml/kg/hr) with 550 ml intake. His appetite also continued to improve and, though still cranky, he was more playful than he had been previously. Additionally, his hematocrit, WBC, platelets, sodium, potassium, and glucose were all within normal range, and his BUN and creatinine were trending down with values of 56 and 5.4. With this positive news, Regan’s nephrology team ordered that peritoneal dialysis be stopped for the first time since September 8.
The order to stop dialysis did not, however, mean that the dialysis catheter could be removed. Labs on September 28 indicated another increase in BUN and creatinine, to 60 and 5.9, which values had remained elevated since dialysis was discontinued the day before. Regan’s blood pressure was stable but high at 118-122 over 70-90. Accordingly, Amlodipine continued for Regan’s hypertension.
On Friday morning, Regan was doing well overall. He continued to be anxious, but seemed to be less cranky, and he was able to eat and drink more and had further increase in urinary output. His facial edema had also improved, but Regan was considered to be generally mildly edematous. He also continued to have elevated but stable blood pressures.
Later in the day, Regan again went to the operating room—this time, however, to have his dialysis catheter removed. Tiffany recalls:
This was a surgery that wasn’t quite as scary to send him in to. He’s laughing because the anesthesiologist put some type of goofy medicine in his PICC line that just made him into a hoot and a half. I was still quite nervous but it felt better to send a laughing child into surgery than a sickly one.
September 30—Discharge from PCMC
On Saturday, September 30, 2006, after four weeks in the hospital and countless dialysis treatments, Regan Erickson was discharged home. His discharge diagnoses included HUS; pancreatitis; acute renal failure requiring peritoneal dialysis from September 8 to 26; placement of PICC on September 8 and removal on September 26; placement of peritoneal dialysis catheter on September 8 and removal on September 29; anemia requiring blood transfusions and ongoing Darbopoetin infusion; emesis requiring Prevacid and Erythromycin Ethylsuccinate (anti-infective); hyperphosphatemia requiring calcium carbonate with meals and phosphate restriction; hypocalcemia status post calcium supplements between meals and IV Calcium chloride x 2; and reactive airway disease.
REGAN’S PRESENT CONDITION AND PROGNOSIS:
Sadly, a child’s HUS illness doesn’t really end when he is discharged from the hospital. Regan is a perfect example of this. He remained on blood pressure medications after discharge, and he continued to suffer physical and emotional problems related to his prolapsed rectum and the trauma of going through an illness as severe as his was.
A couple of months after Regan’s hospitalization, we made a decision, which hindsight tells us was a bad one, to go to Las Vegas with my husband for a conference he had. His parents live there so we thought it would be a nice cheap vacation. We drove down. The morning after we arrived Regan’s prolapse came back out but this time we couldn’t get it back in the entire day. I had been speaking over the phone to a pediatrician throughout the day. By the time the prolapse had been out for 12 hours we were advised to take him to the ER in Las Vegas. He was hospitalized.
When we got home I took Regan to a local surgeon and then to a pediatric GI doctor. We decided to allow Regan to use a pullup and stay off the toilet for several months. Regan had a hard time with the toilet because it really scared him when the prolapse would appear. We bought a box of rubber gloves and made sure we had plenty packets of lubricating gel.
Regan is one of many people, mostly children, sickened in the Spinach outbreak whose lives have permanently changed as a result of their illnesses. He is forecast by several of the country’s leading pediatric nephrologists to require multiple kidney transplants due to the severity of the kidney injury that he suffered in the Spinach outbreak. Lifetime medical costs will run into the millions of dollars.