Hemolytic uremic syndrome is a life-threatening medical condition most commonly associated with complications of E. coli food poisoning, according to the Mayo Clinic. HUS typically affects children under the age of 10, but adolescents and adults can develop the illness as well. Hemolytic uremic syndrome causes damage to the red blood cells, which leads to a low red blood cell count (anemia), blood clots, and damage to blood vessel walls.
Most cases of hemolytic uremic syndrome are preceded by gastrointestinal illnesses, often from a foodborne germ that causes bloody diarrhea. This is referred to as Typical HUS. The most common microorganisms associated with Typical HUS are Escherichia coli 0157:H7 and Shigella, although it seems that nearly any infection that results in bloody diarrhea could lead to hemolytic uremic syndrome. The majority of HUS cases result from E. coli contamination of food like meat, dairy products, and juice. According to some studies, as many as 15% of children who are infected with E. coli develop HUS later in life.
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What Causes Hemolytic Uremic Syndrome?
While the root cause of many cases of hemolytic uremic syndrome cannot be determined for certain, some medical experts believe the triggering events may include:
- E. Coli
- Pregnancy / Postpartum Depression
- Medications (chemotherapy, immunosuppressive drugs, ticlopidine, and quinine)
Signs and Symptoms
In E. coli-related hemolytic uremic syndrome, gastroenteritis occurs with abdominal cramping, vomiting, and bloody diarrhea. This may cause dehydration, weakness and lethargy, as well as electrolyte imbalances because of the loss of sodium and potassium in the vomit and diarrhea. These symptoms tend to resolve before the onset of HUS. Anemia and uremia is usually present with weakness, lethargy, and sleepiness. Purpura or small bleeding areas in the skin may be seen because of low platelet counts.
Hemolytic uremic syndrome treatment in children tends to be self-limiting, and supportive care including IV fluids for rehydration and rebalancing of electrolytes is sometimes all that is needed. Blood transfusions are only used for the most severe cases of anemia. Kidney damage can be managed by observation and supportive care, and dialysis is not often required.
Adults with hemolytic uremic syndrome tend to become more ill and need more aggressive therapy than children with the condition. In addition to supportive care, plasmapheresis or plasma exchange may be required. Since it is thought there is an abnormal chemical in the plasma stimulating the abnormal clot formation, removing the plasma and replacing it with donor plasma can be helpful in treating HUS. Temporary dialysis may be needed while awaiting recovery of the kidneys from the illness.
Signs and symptoms of hemolytic uremic syndrome include:
- Bloody diarrhea
- Decreased urination or blood in the urine
- Abdominal pain, vomiting and occasionally fever
- Small, unexplained bruises or bleeding from the nose and mouth
- Fatigue and irritability
- Confusion or seizures
- High blood pressure
- Swelling of the face, hands, feet or entire body
- Avoid unpasteurized milk, juice and cider.
- Wash hands well before eating and after using the restroom and changing diapers.
- Clean utensils and food surfaces often.
- Cook meat to an internal temperature of at least 160 degrees Fahrenheit.
- Defrost meat in the microwave or refrigerator.
- Keep raw foods separate from ready-to-eat foods. Don’t place cooked meat on plates previously contaminated by raw meat.
- Store meat below produce in the refrigerator to reduce the risk of liquids such as blood dripping on produce.
- Avoid unclean swimming areas. Don’t swim if you have diarrhea.
HUS Kidney Damage
Most patients with hemolytic uremic syndrome experience almost complete recovery and nearly no risk of recurring symptoms. With careful and aggressive treatment, the risk of fatality due to HUS is less than four percent. However, up to 30 percent of children who survive hemolytic uremic syndrome will be left with permanent damage to their kidneys. Children who recover usually do so quickly, while afflicted adults may experience longer recovery times since kidney damage is usually more extensive in adult HUS cases.
According to recent studies, cases of HUS caused by E. coli infection is now the most common cause of renal failure for children living in the United States. It is recommended that people who recover from HUS undergo long-term follow-up and observation to monitor for the potential onset of chronic kidney disease, hypertension, and chronic neurologic disease.
Approximately 7,500 cases of HUS are diagnosed each year in the U.S., but until all 50 states classify it is a reportable disease, an accurate rate of incidence will be impossible to determine.
HUS is characterized by damage and destruction of the red blood cells, which leads to a lower than normal number of red blood cells (a condition called anemia), blood clots, and damage to blood vessel walls. Complications of HUS are severe and can include the following:
- Hemolytic anemia and associated blood complications
- Abnormal kidney function
- HUS kidney failure (renal failure) that may require a kidney transplant
- Gall stones
- Elevated pancreatic enzyme levels that could lead to insulin-dependent diabetes mellitus
- Central Nervous System (CNS) disturbances such as irritability, behavior changes, disorientation, delirium, hallucinations, dizziness, and tremors
- Respiratory disease syndrome
- Heart problems, including heart attack (myocardial infarction), cardio myoptahy, cardiogenic shock, congestive heart failure
- Cortical blindness
- Thrombocytopenia (platelet deficiency in the blood)
Specific preventative measures for treating hemolytic uremic syndrome are the same as protecting yourself from E. coli infection:
- Wash hands, utensils, and food surfaces thoroughly and often.
- Keep raw foods separate from ready-to-eat foods.
- Defrost raw meat in your microwave or refrigerator, and do not leave meat on the counter to thaw.
- Cook ground beef to at least 160 F – hamburgers should always be well-done.
- Wash fruits and vegetable under running water.
- Avoid pasteurized milk, juice, and cider.
- Avoid swimming in water potentially contaminated with feces.
Make sure that everyone in your family washes his or her hands after using the toilet or changing diapers and before eating. In child care facilities, diapers shouldn’t be changed or disposed of in the same room where food is being prepared or eaten.
Most children afflicted with HUS recover fully, but the majority of adults with the syndrome do less well. Without aggressive therapy, up to 40% of those affected may die, and 80% may have permanent kidney impairment. With treatment like plasmapheresis and dialysis, the death rate falls to 10% or less and kidney impairment to 25%.
Patients with HUS not related to diarrheal illness have a worse prognosis than those whose illness is due to an E. coli infection. In individuals with aggravating genetic predispositions, relapsing illness is common as is kidney failure requiring dialysis.
Most cases of E. coli-related HUS can be prevented by thorough cooking of meat products. The vast majority of HUS cases are caused by poor food handling, so proper technique in the kitchen is a main key to prevention.
Although much more rare, atypical HUS, characterized by many of the same symptoms and risks that are common to Typical HUS, will sometimes follow the use of certain drugs, pregnancy, or cancer.
Rather than being caused by an external agent – such as a foodborne pathogen – cases of atypical hemolytic uremic syndrome seem to be caused by an internal factor. Certain experts suggest that some people have a genetic predisposition, meaning their bodies do not make a protein or enzyme that is crucial to resisting atypical HUS.
The latest discoveries in human genetics have allowed medical professionals to classify atypical HUS into three major categories: a) Pneumococcus 2) Genetic (Complement system) and c) Others. It is believed that a majority of the cases are genetic in origin.
There are currently 4 genes associated with this disorder. They are Factor H, Factor I, Factor B, and MCP. The first three genes are responsible for producing proteins that originate in the liver. The fourth, MCP, is not synthesized in the liver, but rather is a protective coating that lines the kidneys.
An important distinction between the typical and atypical varieties of hemolytic uremic syndrome is the severity and likelihood of recurrence of symptoms. While Typical HUS tends to be more severe in the short run, atypical HUS often lingers for much longer.
Preparing for a HUS Doctor’s Appointment
Because the vast majority of individuals with HUS are admitted to the hospital after a trip to the emergency room or following a visit with their doctor, it is not likely that you or your child will have a routine office visit. However, if you or your child is experiencing symptoms of hemolytic uremic syndrome after an extended bout of diarrhea, call your doctor right away and be prepared to answer the following questions:
- Have you noticed blood in you or your child’s diarrhea?
- What other symptoms — such as fever, swelling or decreased urine — have you or your child experienced?
- How long have you or your child been experiencing these symptoms?
- How long has it been since you or your child urinated?
Do I have a Hemolytic Uremic Syndrome Lawsuit?
The Pharmaceutical Litigation Group at our law firm is an experienced team of trial lawyers that focus exclusively on the representation of plaintiffs in hemolytic uremic syndrome lawsuits. We are handling individual litigation nationwide and currently accepting new injury and death cases in all 50 states.