The Outbreak

On May 12, 2008 the Lawrence County Health Department (LCHD) was notified of a case of HUS in a child with a history of bloody diarrhea. The health care provider reported that the child had consumed unpasteurized goat’s milk obtained from a local store, the Herb Depot, in Barry County, Missouri. The milk had been purchased on April 29, 2008. LCHD began an investigation of the illness. It was quickly learned that an additional Barry County child that had cultured positive for E. coli O157:H7 had also consumed unpasteurized goat’s milk from the same store. As a result, LCHD began a full epidemiological and environmental investigation of the illnesses. The investigation revealed that the milk consumed by both ill children had been produced at Autumn Olive Farms.

At the conclusion of its investigation, LCHD ultimately announced that there were four cases of E. coli O157:H7 associated with the outbreak. Of these, three were laboratory confirmed, and one was identified as a probable case (not stool culture positive but Epidemiologically linked to the outbreak). Each of these individuals resided in different counties in Southwest Missouri, and did not have any relation to each other. Nonetheless, each shared a common exposure to milk from Autumn Olive Farms. In addition, the three culture-confirmed cases shared a common, indistinguishable genetic strain of E. coli O157:H7. The strain was identified as a unique subtype of E. coli O157:H7, never before reported in Missouri. Each of the four cases had consumed milk from Autumn Olive Farms within 3-4 days of onset of illness. LCHD reported, “no other plausible sources of exposure common to all four cases were identified [other than the milk.]” LCHD ultimately concluded “the epidemiological findings strongly suggest the unpasteurized goat’s milk from Farm A [Autumn Olive] was the likely source of infection for each of the cases associated with this outbreak.”

The Children

We represent two of the HUS cases. Nicole Riggs is 9 years old. She lives in Willard, Missouri with her mother, Julie; father, Dustin; and her younger sister, Christina. Larry Pedersen is a 2-year-old toddler. He lives in Monett, Missouri with his parents, Brian and Angela, and his two older sisters, Hailey and Kelsey.

Their Acute Illness

Both had a severe episode of HUS as demonstrated by over a week of anuria [no urine output], oliguria [low urine output] for an additional week. Both needed dialysis to survive. Both were hospitalized for over a month. Medical bills were over $100,000 for each.

Their Future

It is likely that both children will develop renal complications in the future, including hypertension and renal insufficiency. Hypertension and renal insufficiency eventually lead to end stage renal disease (ESRD). The development of ESRD means they will require dialysis or transplantation for survival. Most Americans who suffer ESRD opt for a kidney transplant, but the wait for a donor kidney is often a year or more. The preferable course in a transplant situation is for a deceased or living relative (e.g. a parent or sibling over age 18 and compatible) to donate a kidney. While awaiting a donor, an ESRD patient must undergo dialysis treatment while on the waiting list for a deceased donor transplant. Children have the shortest waiting time on the deceased donor transplant list. The average waiting time for children age 0-17 years is approximately 275-300 days; the average waiting time for a transplant candidate who is 18-44 years old is approximately 700 days.

The Effects

Following transplantation the children will require immunosuppressive medications for the remainder of their lives to prevent rejection of the transplanted kidney. Medications used to prevent rejection have considerable side effects. Corticosteroids are commonly used following transplantation. The side effects of corticosteroids are Cushingnoid features (fat deposition around the cheeks and abdomen and back), weight gain, emotional instability, cataracts, decreased growth, osteomalacia and osteonecrosis (softening of the bones and bone pain), hypertension, acne, and difficulty in controlling glucose levels. The steroid side effects, particularly the effects on appearance, are difficult for children, particularly teenagers, and non-compliance with the treatment regimen is a problem with teenagers due to unsightly side effects. Cyclosporine and tacrolimus are also commonly used immunosuppressants. Side effects of these drugs include hirsutism (increased hair growth), gum hypertrophy, interstitial fibrosis in the kidney (damage to the kidney), as well as other complications. Meclophenalate and imuran are also commonly used, each of which can cause a low white blood cell count and increased susceptibility to infection. Many other immunosuppressive medications and other medications (anti-hypertensive agents, anti-acids, etc) are prescribed in the post-operative period. Immunosuppressants like those described above function to reduce the body’s immune response, thereby preserving the transplanted kidney, which the body would otherwise recognize as foreign and dangerous, thereby setting off a chain of events that would culminate in kidney rejection. But because a healthy and timely immune response is a critical host defense against illness, life-long immunosuppression necessarily dictates a life-long, heightened susceptibility to infection, accelerated atherosclerosis (hardening of the arteries), cancer, and chronic kidney rejection.

Bone disease is nearly universal in patients with chronic renal failure. As a result, the children will be prone to develop bone pain, skeletal deformities and slipped epiphyses (abnormal shaped bones and abnormal hip bones) and have a propensity for fractures with minor trauma. Treatment of the bone disease associated with chronic renal failure includes control of serum phosphorous and calcium levels with restriction of phosphorus in the diet, supplementation of calcium, the need to take phosphorus binders and the need to take medications for bone disease.

Another common complication of chronic renal failure is anemia. Patients with chronic renal failure gradually become anemic. The anemia can be treated with human recombinant erythropoietin (a shot given under the skin one to three times a week or once every few weeks with a longer acting human recombinant erythropoietin).

Another complication of ESRD is growth failure. Growth failure ultimately leading to short height as an adult is a very common complication of chronic renal failure in children. Growth hormone therapy with human recombinant growth hormone has been approved for use in children with chronic renal failure and such therapy has been shown to accelerate growth, induce persistent catch up growth and lead to normal adult height in children with chronic renal failure. Growth hormone therapy requires giving a shot under the skin once a day.

As the children develop ESRD, they will not immediately receive a kidney transplant. Instead they will require dialysis. There are two modes of dialysis he might undergo. They can be on peritoneal dialysis or on hemodialysis. Peritoneal dialysis has been a major modality of therapy for chronic renal failure for several years. Continuous Ambulatory Peritoneal Dialysis (CAPD) and automated peritoneal dialysis also called Continuous Cycling Peritoneal Dialysis (CCPD) are the most common form of dialysis therapy used in children with chronic renal failure. CAPD/CCPC. In this form of dialysis, a catheter is placed in the peritoneal cavity (area around the stomach); dialysate (fluid to clean the blood) is placed into the abdomen and changed 4 to 6 times a day. Parents and adolescents are able to perform CAPD/CCPD at home. Peritonitis (infection of the fluid) is a major complication of peritoneal dialysis. Hemodialysis has also been used for several years for the treatment of chronic renal failure during childhood. During hemodialysis, blood in taken out of the body by a catheter or fistula and circulated in an artificial kidney to clean the blood. Hemodialysis is usually performed three times a week for 3-4 hours each time in a dialysis unit.

Finally, no kidney transplant lasts forever. United States Renal Data Systems states that the half-life—i.e. the time at which 50% of transplanted kidneys are still functional and 50% have stopped functioning—is 10.5 years for children 0-17 whose transplanted kidney came from a deceased, unrelated donor, and 15.5 years where the kidney comes from a living, related donor. Similar data for a transplant at age 18 to 44 years is 10.1 years and 16.0 years for a deceased donor and a living related donor, respectively. Each transplant will be preceded by ESRD, dialysis, an increase in kidney-related medical problems and then the recovery from transplantation.

The Lesson?

Was and is the consumption of raw goats milk worth the risk?

  • Milk the world over has been consumed raw since animals have been domesticated.
    Only in the last 50 years or so have we become obsessed with germs and bacteria.
    Chances are, these kids will not develop renal failure. Loose stools for a few days, maybe fever, then back to normal.
    Of course, in the above scenario, you would have no cause to sue a small family business, which will very likely put them out of business.
    What a way to make a living!

  • Mark, you are kidding – a few days of loose stools? Both of these kids nearly died because they drank E. coli O157:H7-tainted goats milk. I hope that they do not loose their kidneys, but it is likely they will.

  • Mary

    Mark, did you even read the post? Both kids were in the hospital for over a month. They both suffered complete renal failure…that means the kidneys shut down and quit working. E.coli 0157:H7 is a is nasty bacteria and it is finding it’s way into raw milk.

  • Mary

    Mark, if you want to champion consumption of raw milk, that’s your business. Before commenting on what might happen to the children, it would be a good idea to actually read the article. It was very clear in Mr. Marler’s post above that the children involved did develop hemelytic uremic syndrome and had to go on dialysis or else die.
    I don’t understand, in light of the foodborne illnesses associated with consumption of unpasteurized milk, how there can continue to be a halo effect regarding the product.

  • Marymary

    For clarification: Mary on August 3rd is Mary. Mary on August 4th is Marymary.

  • Do you know if the farm had any safety measures in place like routine milk testing, etc? I don’t drink raw milk but I know people who do. One friend explained to me the testing the farm does each month… but I wasn’t sure if that was enough to keep problems from entering the milk in between test batches.
    Mainly I’m asking if raw milk could be safe if a proper cleanliness / testing protocol was followed or are there outside factors that could taint it at any time. (I probably would still be too nervous to drink it or feed it to my children but am very curious.)

  • Marymary

    In my less-than-perfectly-informed-opinion, monthly testing would not be enough to ensure safety of the product. Cows shed bacteria at differing rates. At least one very serious problem, Listeria monocytogenes, actually grows under refrigeration temperatures. If there is any listeria in the milk, it can grow, even with proper temperature control. E coli O157:H7 can make a person deathly ill with the consumption of a relatively small number of organisms. Furthermore, and this is something that is difficult for many people to understand, surfaces and premises and animals that appear to be clean to the naked eye could in fact be harboring all kinds of dangerous bacteria and viruses. So if you hear someone say, “My raw milk supplier keeps a clean barn, and clean equipment, and the cows are clean. I’ve seen his farm,” it doesn’t mean that the milk is safe to consume.

  • Raw milk is probably safe if the proper tests were made. Unfortunately this puts a damper on small business because you would hope bigger practices have all the proper safety measures in place.