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