Hemolytic-Uremic Syndrome (HUS) Lawyer

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Hemolytic-Uremic Syndrome (HUS) Lawyer

Hemolytic Uremic Syndrome Lawyer: What is hemolytic uremic syndrome?

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.[1] 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:

  • Most commonly, HUS is a result of an infection from Shiga toxin-producing Escherichia coli. Bacterial toxins “cross from the intestines into the bloodstream and damage the very small blood vessels.”[2] Damaged blood cells can lead to clogged glomeruli (tiny filter units in the kidneys) and result in renal failure. These cases are usually due to ingested food contaminated with Shiga-toxin producing Escherichia coli. bacteria, otherwise known as E. coli or STEC.
  • It rare cases, HUS can also be caused by certain medications such as those used in chemotherapy, immunosuppressant treatments, and antiplatelet medications. [3]
  • In other rare cases, conditions like HIV/AIDS or infections that stem from pneumococcal bacteria can be the source of HUS.[4]

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),[5] 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),[6] 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:

  1. An employees’ failure to practice good personal hygiene;
  2. A failure to prevent insects of vermin form coming into contact with food;
  3. The failure to ensure suppliers are following GMPs and providing safe, pathogen free products;
  4. A failure to properly clean and sanitize equipment; and
  5. A failure to hold foods at proper temperatures during all stages of handling and production.

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[7] 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.” [8] 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.[9] 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:

  1. Ongoing bloody diarrhea;
  2. The decrease in the frequency of urination;
  3. Pallor, or a paleness or loss of color in the skin;
  4. Small bruises without a known cause;
  5. Bleeding from the nose or the mouth;
  6. Fatigue and irritability;
  7. Swelling in various parts of the body like the face and the extremities; and
  8. Increased blood pressure[10]

Individuals with “heightened susceptibility” to HUS following a STEC infection include individuals with specific genetic mutations making them more prone to atypical HUS,[11] and according to the CDC, those of heightened susceptibility include “children younger than 5 years and adults aged 65 and older.”[12] 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.[13] 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.[14] 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%).[15]  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.[16]

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.

  1. Dehydration is a serious side effect of both STEC and HUS that affects the sodium/potassium balance and can lead to tachycardia, arrhythmia and myocardial infarction;
  2. Kidney failure is the most direct complication. Kidney failure is the inability of the kidneys to filter blood as they are supposed to. When kidneys lose 10% of their function it is considered kidney failure (medical records will often indicate a stage 1 to stage 5 level of acute kidney failure).[17] The Mayo Clinic identifies acute kidney failure to be sudden onset whereas chronic kidney failure develops over time;[18]
  3. High blood pressure is a common effect and can last for years;[19]
  4. Stroke is another possible complication. A stroke is when a part of the brain is deprived of oxygen because the blood flow has been cut off. This can cause serious damage to brain cells and it can cause them to begin to die;[20]
  5. HUS has also been linked to comas in severe cases. A coma is an unconscious state that lasts for an unknown period of time. This usually happens when a person experiences traumatic injuries or illness;
  6. Intestinal issues can arise, such as inflammatory colitis, due to the destruction and damage of blood vessels throughout the body. This is known as post-infectious IBS.
  7. Heart problems can also arise due to the damage of blood vessels;
  8. Seizures;[21]
  9. Blood-clotting problems;[22]

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:

  1. Due to the difficulty in retaining food and nutrients, health care providers need to intensively monitor fluid and electrolyte balances in patients with HUS. Patients will nearly always require IV fluids and nasogastric tube for nutrients;
  2. Blood transfusions are often required due;
  3. According to the National Kidney Foundation, dialysis, a short-term kidney replacement treatment, is necessary in about 50 percent of cases;[23]
  4. Plasmapheresis, a filtration process where harmful antibodies are removed from the blood, is also required in many of the seriously ill.[24]

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.[25] 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.[26]

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.

[1] Obrig, T. G., & Karpman, D. (2012). Shiga toxin pathogenesis: kidney complications and renal failure. Current topics in microbiology and immunology357, 105–136. doi:10.1007/82_2011_172 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3779650/

[2] https://www.kidney.org/atoz/content/hemolytic

[3] https://www.mayoclinic.org/diseases-conditions/hemolytic-uremic-syndrome/symptoms-causes/syc-20352399

[4] Id.

[5] https://www.govinfo.gov/app/details/CFR-2011-title21-vol2/CFR-2011-title21-vol2-part110

[6] https://www.fda.gov/regulatory-information/federal-food-drug-and-cosmetic-act-fdc-act/fdc-act-chapter-iv-food

[7]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

[8] https://www.fda.gov/food/guidance-regulation-food-and-dietary-supplements/hazard-analysis-critical-control-point-haccp

[9] https://www.cdc.gov/features/ecoliinfection/index.html

[10] https://www.mayoclinic.org/diseases-conditions/hemolytic-uremic-syndrome/symptoms-causes/syc-20352399

[11] 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/

[12] https://www.cdc.gov/features/ecoliinfection/index.html

[13] 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 .

[14] 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.

[15] Id.

[16] 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.

[17] https://www.davita.com/education/kidney-vocabulary/kidney-failure?utm_source=msn&utm_medium=cpc&utm_source=bing&utm_medium=cpc&utm_campaign=Search%20-%20Core_B_Non-Brand_Non-RT_Kidney%20Failure_US&utm_term=failing%20kidneys&utm_content=Signs%20%26%20Symptoms&gclid=CILGl637mOICFQmexQIdfucPuQ&gclsrc=ds

[18] https://www.mayoclinic.org/diseases-conditions/hemolytic-uremic-syndrome/symptoms-causes/syc-20352399

[19] http://www.bloodpressureuk.org/BloodPressureandyou/Yourbody/Kidneydisease

[20] https://www.stroke.org/understand-stroke/what-is-stroke/

[21] https://www.health.ny.gov/diseases/communicable/e_coli/hus.htm

[22] https://www.health.ny.gov/diseases/communicable/e_coli/hus.htm

[23] https://www.kidney.org/atoz/content/hemolytic

[24] https://www.kidney.org/atoz/content/plasmapheresis

[25] https://www.cdc.gov/features/ecoliinfection/index.html

[26] 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

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