I am working this weekend instead of watching the Seahawks – 49’s game because I will be sitting down with a couple of families this week to talk with them about the long-term complications that their children actually face because of something they ate.
It is not something that their children’s physicians have not told them, but I have to talk with them about it in the context of a lawsuit and trying to financially prepare for a unknown, but risk-filled future.
E. coli O157:H7 caused Hemolytic Uremic Syndrome (HUS), even in the best hospitals, can be a life-altering event. Even children, who have apparently recovered normal renal function following the acute episode of HUS, have a high risk for the development of late complications from their acute episode of HUS. The risk is substantially lower in children who did not require dialysis and in children who were not oliguria or anuric while the risk is the highest in children who had oligo/anuria for more than 7 days. However, the opposite is also true.
Among the serious complications for children with extensive renal damage caused by E. coli O157:H7 HUS are: (1) alterations in calcium and phosphate balance, known as renal osteodystrophy, that cause the bones to become weak and soft, resulting in bone pain and susceptibility to fracture; (2) anemia—which is characterized by a low red blood cell count and consequent lack of energy; (3) growth failure caused by the damaged kidney’s inability to properly regulate the calcium necessary for bone growth; and (4) high blood pressure, which, among other things, stresses the heart and can lead to coronary artery disease, heart attack, and stroke.
Development of ESRD means the patient 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. Once the ESRD patient has only about 15% of normal filtration capacity, they will start to need dialysis.
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. Fortunately, 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.
Cost of hemodialysis can be in the range of $100,000 annually. Cost of a transplant can be as high at $150,000 with yearly cost for anti-rejection medication being at least $20,000.
Following transplantation, a patient will require immunosuppressive medications for the rest of her life 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.
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.
It is going to be a long week.