Hemolytic Uremic Syndrome (HUS)

Large group of foodsHow can a mother deal with the knowledge that when her nine-year-old son reaches puberty, his kidneys are expected to fail completely?  Kim Stauffer‘s son, Coleman, developed hemolytic uremic syndrome (HUS) at just one year of age, which led to weeks of hospitalization, over a year of dialysis, and a lifestyle of much medication and a biweekly injection to increase his red blood cell levels. So how does Kim cope with her son’s situation? By committing, when the time comes, to give Coleman the ultimate gift: her own kidney.

Hemolytic Uremic Syndrome (HUS) and E. coli

So, what is HUS and what causes it? The disease that has driven this young boy’s kidneys to fail, hemolytic uremic syndrome, most commonly occurs in young children, although the elderly and immunocompromised are also particularly susceptible. HUS is almost always the result of an infection with E. coli bacteria, typically a potentially deadly strain known as E. coli O157:H7. E. coli is a bacterium that is naturally found in the intestines of animals. The E. coli present in an animal’s digestive system and feces can contaminate food in several ways, including:

◦ During the slaughtering process – thus contaminating flesh products. In some cuts of meat, E. coli is limited to the surface; in ground beef, one of the most common sources of E. coli poisoning, the grinding process spreads the E. coli throughout the meat.

◦ Through feces-contaminated water – thus contaminating whatever comes into contact with the water. Vegetable and fruit E. coli contamination often occurs when feces-contaminated water from an animal farm runs into a nearby produce field.

A child may ingest E. coli from contaminated food products, usually undercooked ground beef, raw unpasteurized milk, or contaminated raw produce (unpasteurized apple juice, unwashed vegetables, etc.). Drinking contaminated water or swimming in an infested lake or pond can also cause an E. coli infection.

Symptoms of an E. coli Infection

Most E. coli infections present symptoms after approximately 2 to 5 days. Typical symptoms include:

◦ Nausea

◦ Abdominal cramps

◦ Watery or bloody diarrhea

◦ Fever

◦ Fatigue

From E. coli to Hemolytic Uremic Syndrome

Once ingested, some strains of E. coli will produce a toxic substance known as Shiga toxin inside the digestive system of the human body. Shiga toxin producing E. coli binds to the cells lining the large intestine, and these poisonous substances thus gain access to circulating blood. The toxins spread to various organs where they can inflict much damage. They deform and destroy red blood cells, which then clog the tiny blood vessels of the kidneys, hindering the normal filtering function of the kidneys and possibly eventually leading to renal failure. These misshapen red blood cells – and anemia, or lack of healthy red blood cells – can harm the heart, brain, liver, and other organs as well. Thrombocytes, the blood’s clotting cells, are also destroyed, which can lead to bruises and uninhibited bleeding.

Symptoms of HUS usually occur 5 to 10 days after the start of diarrhea, and include:

◦ Paleness, fatigue, and irritability

◦ Small, unexplained bruises or bleeding from the nose or mouth

◦ Swelling of the face, hands, feet, or entire body

◦ High blood pressure

◦ Decreased urine output or bloody urine

Treatment of Hemolytic Uremic Syndrome

Treatment of HUS aims to ease the immediate symptoms and prevent further problems by seeking to maintain an appropriate salt/water balance in the body. Over time, a patient with HUS may need red blood cell and thrombocyte (platelet) infusions, dialysis, and even – as in the case of young Coleman Stauffer – a kidney transplant.

Prevention of E. coli Infection and Hemolytic Uremic Syndrome

Coleman is fortunate to have a mother whose love – and matching donor organ – may very well save his life. While most children who develop hemolytic uremic syndrome survive the experience, they suffer tremendously from its symptoms and often develop life-long health problems.

Prevention of most cases of HUS is possible through the reduction of the spread of E. coli bacteria. Hand washing, clean and safe preparation of food, and cooking foods – especially ground beef – to a high enough temperature (160F for ground beef) are all ways to prevent the ingestion of dangerous E. coli. Ideally, though, our food supply would be free of E. coli contamination before it ever gets into our kitchens or our plates. Improving our food safety system with high industry standards, attentive regulation, and effective legislation can help protect our children from the misery of hemolytic uremic syndrome.

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