Today is Rare Disease Day and we are sharing our first HUS feature story, the story of Jensen. Some stories are longer and more detailed than others, but we hope by sharing these stories you will see the true and real effect HUS has on a young child and his or her family.
•Parents Names: Amanda and Bryan
•Child’s Name: Jensen
•Age of Child at Diagnosis: 2
•Were any other family members affected by E.coli at the time? No
•Do you know what strand of E.coli? No – we do not have confirmation of which strand because he never had a positive stool culture
•Do you have confirmation of where the E.coli was contracted? Though we do not have lab confirmation, we have reason to believe that he came into contact with E. coli from the General Mills flour recall.
Jensen was a perfectly healthy, joyful, easy-going, and extremely bright two-year-old boy. I know most parents think their own kids are the smartest, but he truly was an impressive toddler. He was very verbal from a young age and had an extensive vocabulary even at the age of two. Jensen rarely got upset or cried; he was most definitely not your typical tantrum-throwing two year old. Even when he disobeyed and told us no, it was always a quiet and polite, “No, thank you,” with the sweetest little smile. He also basically potty-trained himself around 18 months old. The only “challenging” part of parenting Jensen was the fact that he did not like to sleep. He had never slept past sunrise, and always woke us up at the crack of dawn with joyful singing. Jensen was truly a breath of fresh air in our family. I’m not sharing these details to boast about my child, but because I feel they are pertinent to his story and to understanding exactly how he was eventually diagnosed with Hemolytic Uremic Syndrome and the extent to which it affected him.
The last week of March, 2016, Jensen started showing symptoms of a typical stomach bug. At first, we thought his loose stool was due to all of the Easter treats just a few days prior. His stool was never overly concerning, just a few mild bouts of diarrhea, never with any noticeable blood. A few days later, Jensen slept past 8:00am for the first time in his life. I actually went into his room to wake him, then he ended up falling back asleep in my bed, which also had never happened. When he awoke and vomited, I started to become concerned. Jensen had never vomited in his life, but it didn’t seem to phase him; he immediately wanted to go back to sleep. He didn’t have a fever, and eventually woke up and ate some breakfast. He was able to keep food and liquids down, but was certainly acting very different. He was quiet, sleepy, wanting to be held, and the biggest red flag of all – when he would respond to us, he would angrily yell or whine.
This behavior and lethargy was much more concerning to me than his gastrointestinal symptoms, which were very mild and even nonexistent for a few days. Of course by now it was the weekend, so I called the pediatrician and paged the doctor on-call. The on-call doctor happened to be a close friend of ours, so I felt comfortable telling him how very worried I was about our little boy. He assured me that it sounded like a typical stomach bug, and we just needed to keep Jensen hydrated, and let it run its course. Monday morning, Jensen woke up with a dry pull up and refused to go potty. He was even more irritable and seemingly confused. He kept yelling at me saying, “I already DID!” when I asked him to try to urinate. When we finally got him to urinate, it couldn’t have been more than a single drop. I called and made an appointment at his pediatrician’s office right away. I was sure that despite giving Jensen pedialyte and his seemingly mild symptoms, he was somehow dehydrated, and I felt like it was all my fault.
To our surprise, his pediatrician checked for signs of dehydration, collected the smallest amount of urine possible to try to test, and sent us on our way with a diagnosis of the stomach flu. She said he was not dehydrated and that gastrointestinal viruses could last up to seven days. Again, Jensen did not have blood in his stool, which is a typical symptom of E. coli infection, and since it was the beginning of spring and not the typical HUS season, there was really no reason for his pediatrician to suspect something so rare. (Most HUS cases are in the summer or fall due to petting zoos, fairs, cookouts/undercooked hamburgers, lake swimming, etc). Looking back, I do wonder if Jensen was still in diapers during the day, if I would have noticed blood. I left the doctor’s office feeling like a crazy, paranoid mom, but knowing in the back of my head that something was definitely not right. Jensen wanted to be held all day. He just slept and whined and was not speaking more than a few words.
When he woke up the next day, which was now a full week after his few bouts of diarrhea, Jensen again did not want to urinate, but he also looked different. His eyes were swollen and his face puffy. I knew our friend who was a pediatrician had the day off, but I called his wife (who happens to be a nurse) and begged for a house call. Thank God for these dear friends. After seeing Jensen and talking to his wife, he wrote a script for stat labs, and we were on our way to a nearby hospital. It was a two hour horrific ordeal to try to get any blood from Jensen. After poking the end of his finger and filling a vial drop by drop with blood, we were finally on our way home…or so I thought. Our friend called just minutes after I left the lab and told me to drive straight to Riley Children’s Hospital. (Thankfully, Riley has an entire team of pediatric nephrologists, and this hospital was a short 20 minute drive from our home. Some entire states do not have even one kidney doctor for children; I can’t even imagine the outcome if this were the case for us).
He calmly told me that based on Jensen’s lab results he had an accurate diagnosis – Hemolytic Uremic Syndrome. He told me to be prepared because the nephrology team was waiting for us, and Jensen would be taken back for surgery right away so he could begin dialysis as soon as possible. I was totally stunned. Kidney failure? Caused by E. coli? How? I asked our friend if Jensen would be okay, and I’ll never forget his response. He only said, “It’s a very serious illness. But he is in the best hands.” Despite my habit of google-diagnosing my children, I had never heard of HUS. I just wanted reassurance that this was not a big deal and Jensen would be just fine, but he could not give me that. To go from thinking our kid had a run of the mill virus, to learning he actually had a life threatening disease, was devastating to say the least.
Jensen’s blood counts, platelets, potassium levels – so many labs were critical. Doctors have such an amazing gift of telling people exactly and only what they need to know in that moment. If I would’ve known how awful Jensen’s labs were, I’m not sure how I would have handled those first few days in the hospital.
HUS begins in the blood stream and creates a catastrophic chain of events, eventually affecting every major organ. When we were admitted, Jensen had extremely low platelets, extremely high blood pressure, critically low hemoglobin, pancreatitis, critically high potassium (did you know that high potassium can stop your heart? Thank God I didn’t know that at the time!), liver issues, and of course he was in acute kidney failure. His creatinine was 9.2, when normal is 0.2-0.9! Some children have seizures or even fall into a coma. Though Jensen did not experience this, he was having neurological effects. One nurse told us the irritability is caused by the pH level of the blood literally and physically agitating the brain. Jensen was definitely agitated, but apparently he was calm compared to other HUS patients they had treated. I tried time and time again to explain Jensen’s “normal” personality and wanted so badly for these nurses and doctors to get to meet our usual happy and outgoing little boy.
Shortly after Jensen was out of surgery, he was given countless medications through his central line and a life-saving blood transfusion. He also began peritoneal dialysis (PD). PD is not traditional kidney dialysis, but it’s the standard treatment for young children with HUS and other kidney problems. During PD, the abdominal cavity is filled with fluid that absorbs wastes from the body and then drains them into a bag outside the body. During this time, Jensen had to lie still, flat on his back, so we were not able to hold him. I truly believe it was the most heartbreaking and terrifying time of all of our lives. He was hooked up to so many monitors, checked on constantly by a team of nephrologists, residents, fellows, nurses, dieticians, and dialysis nurses.
We were told it was a waiting game to see IF Jensen’s kidneys would start to function again. We spent a lot of time in prayer, praying for things we take for granted every single day like pee! The doctors told us that HUS can be a roller coaster; patients usually take one or two steps forward, and one or two steps back. Miraculously, once Jensen started to get better, he just kept improving little by little each day. He finally urinated, and his little kidneys slowly but surely began to work again. It was the most incredible thing to witness a miracle in front of our eyes. After just a few days of dialysis, Jensen started to show us glimpses of his normal, joyful, talkative self. He surprised and impressed the doctors along the way. Despite the circumstances, I remember so much joy in that hospital room. We truly believe, especially with what we now know about his initial dire condition, it was a work of God that Jensen recovered the way he did. So many other children with HUS experience much worse complications and multi-organ failure. Jensen was truly very fortunate. We have been told that with his kidney labs as elevated as they were, we are extremely lucky that his kidneys recovered at all.
Jensen did not have a bowel movement for over a week at Riley, which was two weeks after his initial symptoms. By this time, we were told the E. coli had most likely left his system. Sure enough, his stool cultures were negative. At the time, we were rejoicing, because we were so ready to be able to take off the hospital gowns and gloves that were required while in “isolation.” To be able to leave the hospital room and take a walk/wagon ride was so freeing!
We were at Riley for just over two weeks. Jensen was on dialysis for 11 days. He had four blood transfusions and countless medications. His pancreatitis, low platelets, high potassium, and jaundice all resolved during that time. Though I may have some emotional scars from HUS, Jensen somehow remembers only the good. I think that speaks volumes for the staff of Riley. Jensen truly loved the attention and care, and of course being spoiled with gifts and being able to explore super cool playrooms once he was out of isolation.
Jensen left the hospital with high blood pressure and acidosis, so we took home several medications. We learned how to take blood pressure at home and had to give him meds like clockwork, even in the middle of the night. Slowly but surely, he was weaned off of all of the medications. We saw his nephrologist and had labs drawn every couple of weeks at first. Just recently, we were released to six month appointments. Jensen also had protein in his urine for quite a while afterwards. Protein in the urine is a sign of kidney damage and sometimes a precursor to later kidney disease or kidney failure. He, like all HUS patients, should see a nephrologist at least annually for the rest of his life to monitor kidney function.
A few months after we got home, a family member of ours received a call from Kroger that she had purchased some General Mills Gold Medal Flour that had been recalled for E. coli O121. She remembered that the only time she had used that flour was when she was baking on Easter weekend. Of course, our family was at her house, sharing a meal together for Easter. To my knowledge, Jensen did not eat any raw dough or flour, but I cannot help but think that he possibly came into contact with some flour on the floor or on a surface in the kitchen. If you’ve ever baked with flour, you know how easily it can spread everywhere! I’m assuming, Jensen, being a two year old, at some point put his fingers in his mouth that day. His diarrhea began just a few days after Easter. We have been in contact with the Indiana State Health Department, but we were told that because Jensen never had a positive E. coli stool culture, there was no way to link his illness to the flour. We were also told that even if he did have a positive culture, there was no way for Jensen’s HUS case to be considered “confirmed” by the CDC because our family member did not save the original bag of flour with UPC. I will forever look at food recalls and confirmed case numbers differently after our experience
Now that we are past the terrifying hospital days, the hardest part of this illness is not knowing if Jensen will have any health issues or kidney problems in the future. Some kids stay healthy, some need kidney transplants or have major health complications. There is not nearly enough research because HUS is so rare, and obviously no doctor can predict the future. It has been a true test of our faith – I can look at my son each day and choose to worry and fear for his future, or, I can choose to trust God, the only One who knows Jensen’s future and surrender my fears to Him. Every day I look at my son, I choose to be grateful that he did survive. I try my best to appreciate every single moment with him. Jensen will proudly declare, “God healed me!” and tells everyone that when he grows up, he wants to be a pediatric nephrologist. I pray Jensen continues to thrive; I know that God has big plans for him. Jensen is such a special boy, and I will tell his story as often and as much as I can.
As parents we do our best to keep our children safe and healthy. It’s important to be informed, but it’s also evident to me that I am not the one in control. We have learned that no matter how hard we try to control our children’s lives, crazy, random, and horrible things can happen. It is so difficult to wrap our mind around some of the stories we have heard of children with this rare disease. We are forever grateful for the Dunhams and the Grayson Dunham Foundation for their tireless efforts to raise awareness about HUS. Only the strongest people can use something so tragic to bring about good in this world. We pray Grayson’s legacy lives on and touches countless lives as deeply as it has touched ours. Thank you for allowing us to share our story.