Hemolytic uremic syndrome (HUS) is a serious condition affecting the blood, and blood vessels, in the body. HUS largely results from the abnormal destruction of red blood cells and platelets by bacterial virulence factors like Shiga toxin. Shiga toxin-producing Escherichia coli (STEC) release their toxins in the intestinal tract where the bloodstream absorbs the Shiga toxin. The toxin circulates throughout the blood system targeting red blood cells, endothelial cells, and platelets. Damaged red blood cells and platelets clog filtering units in the kidneys.
The complications of HUS can include damage to the kidneys leading to proteinuria, dehydration, and renal failure. In many cases, dialysis is required, and in extremely serious cases, one or more kidney transplants are required.
By far the most common cause of HUS is Shiga-toxin producing Escherichia coli food borne illness, though there are some exceptions:
How does STEC, the Cause of Hemolytic Uremic Syndrome, Get into Food?
Contracting an infection or illness from something you eat is always distressing, but when doing so results in the acute onset of HUS, it becomes potentially life-threatening. HUS is a serious illness that in almost every case requires hospitalization and can lead to a host of life-ling complications for those strong enough to recover. What is important to understand, however, is that HUS is as preventable as the underlying forborne illness, specifically ingestion of STEC. The problem for consumers is that the ability to prevent STEC form getting into food takes place well outside the average consumers’ observation. The ultimate cause of STEC contaminated food is a failure by food manufacturers (and others who prepare food for consumers) to follow “routine” food preparation, handling, and storage guidelines – a failure that allows fecal matter (by far the most common source of Shiga toxin-producing E. coli) into the food.
The simple fact is that Shiga toxin-producing E. coli does not accidentally appear in food. It is for this reason that hundreds of years of experience has led to the adoption of “quality controls,” “industry standards,” “best practices,” and related regulations that have been set in place to ensure that the food a consumer buys is safe and free of dangerous pathogens. In fact, all restaurants and food manufactures or distributors have a duty to comply with industry standards as well as all applicable health regulations, both state and federal. These include the U.S. Food and Drug Administration’s Good Manufacturing Practices Regulations, 21 C.F.R. part 110, subparts (A)-(G), and all statutory and regulatory provisions that apply to the manufacture, distribution, storage, and/or sale of the food or its ingredients, including but not limited to, the Federal Food, Drug, and Cosmetics Act, § 402(a), as codified at 21 U.S.C. § 342(a), which bans the manufacture, sale and distribution of any “adulterated” food. In fact, most states also have a food, drug and cosmetic act that prohibit the same thing.
When Ron Simon & Associates takes a case, one of the first things we do is to look for the breakdown that led to the introduction of Shiga toxin-producing E. coli into the food that was served to the victims, our clients. The most common breakdowns include such things as:
These “risk areas” are commonly recognized in the industry and among restaurateurs and are why most responsible food-producing manufacturing companies maintain a Hazard Analysis Critical Control Point (HACCP) plan and why good restaurateurs spend significant time training their employees in food safety. For manufacturers, the FDA explains a HACCP as: “a management system in which food safety is addressed through the analysis and control of biological, chemical, and physical hazards from raw material production, procurement and handling, to manufacturing, distribution and consumption of the finished product.”  A proper HACCP plan is meant to prevent pathogens, such as Shiga toxin-producing E. coli, from getting into the food in the first place. For restaurateurs, local and state regulations mandate that food safety inspectors make routine visits to ensure compliance and very often require one or more of the employees on any given shift to have advanced food safety training.
Unfortunately, food borne illness outbreaks still occur with relative frequency, and when they involve STEC, cases of HUS follow.
What are the symptoms and who has a higher risk of contracting HUS?
When HUS is caused by Shiga toxin-producing E. coli, the HUS symptoms will begin with those associated with E. coli gastroenteritis, including one or more of the following: vomiting, nausea, diarrhea (it has a tendency of being bloody), stomach pain or cramps, body aches, headaches, dehydration, and fatigue. The most telling sign, and the one physicians are on the look-out for, is blood in the stool. And while most victims of Shiga toxin-producing E. coli will recover within two to three weeks, according to the CDC, about 5 to 10 percent of people diagnosed with an infection from E. coli O157:H7 develop HUS. In those victims, about a week after the E. coil infection, HUS can surface and have a whole host of symptoms of its’ own, including:
Individuals with “heightened susceptibility” to HUS following a STEC infection include individuals with specific genetic mutations making them more prone to atypical HUS, and according to the CDC, those of heightened susceptibility include “children younger than 5 years and adults aged 65 and older.” It also includes those with HIV or an immuno-compromised medical condition.
Pediatric HUS: The Unique Dangers of Hemolytic Uremic Syndrome in Children
Many food safety advocates and medical professionals consider the most worrisome cases to be those that present in children. This is due to a number of considerations including age, inability to understand what is happening, and the life expectancy of a child coupled with the ongoing effects post-HUS resolution (the number of years a child may face complications into the future).
Multiple studies have demonstrated that children with HUS who have apparently recovered may well develop hypertension, urinary abnormalities and/or renal insufficiency long after their initial recovery. One of the best predictors is the duration of anuria and/or oliguria. In one study of 61 children who were followed for a mean of 9.6 years following the acute episode of HUS (having had dialysis), 52% had proteinuria, 41 % decreased creatinine clearance, and 56% renal sequelae. And in fact, in all children with anuria of 8 or more day’s duration and those with oliguria exceeding 15 days, suffered ongoing chronic disease (100%). In yet another article in Clinical Nephrology (by Gagnadouz, et al, 1996) when 29 children were evaluated 15-25 years after the acute phase of HUS, only 10 of the 29 children were normal, 12 had hypertension, 3 had chronic renal failure and 4 had end stage renal disease (65.5%). Other studies have demonstrated that histological finding of focal and segmental sclerosis and hyalinosis can still be observed several years following HUS, and that only 25% of the children had normal renal function during long term follow-up.
In short, the effects of HUS on children are serious and can last for years or even life. Many of the victims will eventually require a kidney transplant, and those who get a kidney transplant may require a second or third transplant due to complications or rejection by the body.
What Types of Complications are Commonly Associated with HUS?
As noted above, HUS is itself a complication of E. coli O157:H7 illness, but can easily go beyond acute gastroenteritis to effect other health issues. These complications are serious and could, ultimately, be life-altering and/or life-threatening.
How is Hemolytic Uremic Syndrome Treated? Can I Reduce My Chances of Acquiring HUS?
HUS is a serious condition will require an extended hospital stay. The treatment regimen is quite intense and requires ongoing testing and monitoring, though treatment may vary substantially between victims. Due to the varying symptoms brought about by HUS, treatment will vary depending on the patient’s symptoms and kidney function.
Below are some typical treatments:
As noted above, the initial hospitalization and treatments during the acute stage are not the end of the story for many of the victims. Many victims are likely to have a full recovery, even if they required dialysis, within six months. But many more may have lasting kidney issues, post-infectious IBS, high blood pressure, or a myriad other ongoing effects.
Ultimately, people need to take the right steps to prevent this serious condition from happening – but as noted above, this can be difficult. The CDC recommends the best way to prevent HUS is to prevent a STEC infection is by avoiding contaminated food and practicing good hygiene. These are effective on an individual level, and to a limited degree will help reduce food borne illness by those who prepare food at home. Such things as preventing cross contamination between raw meats and uncooked food items, not using dish rags to clean up counters without proper sanitation, and keeping foods at proper holding temperatures, will all reduce the incidents of food borne illness in teh family. But to prevent outbreaks, action is necessary on an institutional level, where a commitment to cleanliness and proper food storage, cleaning, and preparation is even more vital to preventing a STEC or other food borne outbreak. Unfortunately, most of this is out of the control of the average consumer when purchasing food – consumers rely (rightfully) on the expertise and professionalism of food manufacturers every day. When those food manufacturers or handlers fail to follow industry standards and applicable regulations, consumers can acquire Shiga toxin-producing E. coli, and for some of those, HUS.
Contact an Experienced Hemolytic Uremic Syndrome Lawyer
Ron Simon & Associates has over 50 years in helping victims of food poisoning victims, many who contracted HUS. These cases are complex, and understanding the science and the implications of HUS is vital to prosecuting a case against a food manufacturer or restaurant. Contact our hemolytic uremic syndrome attorneys by calling toll free 1-888.335.4901.
 Obrig, T. G., & Karpman, D. (2012). Shiga toxin pathogenesis: kidney complications and renal failure. Current topics in microbiology and immunology, 357, 105–136. doi:10.1007/82_2011_172 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3779650/
See https://www.fsis.usda.gov/wps/wcm/connect/e113b15a-837c-46af-8303-73f7c11fb666/93-016F.pdf?MOD=AJPERES (rule requiring USDA regulated meat producers to have a HACCP plan); see generally https://auditortrainingonline.com/home/category/FoodSafety?https%3A%2F%2Fauditortrainingonline.com%2Fhome%2Fcategory%2FFoodSafety%3Fref=bing&keyword=haccp%20food%20safety&cmpid=366416719&agid=1165482604901178&adid=72842713216578&msclkid=bd21af88105011ea2978f35183289cc0
 Noris M, Bresin E, Mele C, et al. Genetic Atypical Hemolytic-Uremic Syndrome. 2007 Nov 16 [Updated 2016 Jun 9]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1367/
 Perlstein EM, Grnnfeld BG, Simsolo RB, Gimenez, Gianantonio CA. Renal functional reserve
compared in hemolytic uraemic syndrome and single kidney. Arch Dis Child 65:728-731 , 1991 .
 Siegler RL, Milligan MK, Burningham TH, Christofferson RD, Chang SY, Jorde LB. Long-term
outcome and prognostic indicator in the hemolytic uremic syndrome. J Pediatr 118:195-200, 1991.
 Caletti MG, Gallo G, Gianantonio CA. Development of focal segmental sclerosis and hyalinosis in hemolytic uremic syndrome. Pediatric Nephrology 10:687-692, 1996. See also Moghal, et al, noting htat kidney biopsies in children with a history of HUS showed persistent proteinuria and that there was substantial irreversible scarring with larger glomeruli suggestive of hyperperfusion and hyperfiltration in surviving nephrons.
 Additional resources: Neil KP, Biggerstaff G, Macdonald JK, et al. A Novel Vehicle for Transmission of Escherichia coli O157:H7 to Humans: Multistate Outbreak of E. coli O157:H7 Infections Associated With Consumption of Ready-to-Bake Commercial Prepackaged Cookie Dough–United States, 2009. Clin Infect Dis 2012;54:511-518; Mead PS, Slutsker L, Dietz V, et al. Food-related illness and death in the United States. Emerg Infect Dis 1999;5:607-25; Heiman KE, Mody RK, Johnson SD, Griffin PM, Gould LH. Escherichia coli O157 Outbreaks in the United States, 2003-2012. Emerg Infect Dis 2015;21:1293-301; and Torok, M. Focus on field epidemiology. North Carolina Center for Public Health Preparedness. https://nciph.sph.unc.edu/focus/vol1/issue5/1-5EpiCurves_issue.pdf