Treatment
What to Expect During Hospitalization
The hospital course can range from mild to very severe. Children are generally in the hospital for about two weeks (range 3 days to 3 months), and adults longer, as their course tends to be more severe. Since there is no way to abort D+HUS, supportive therapy, including meticulous attention to fluid and electrolyte balance, is the cornerstone of survival.
The inflamed colon is usually non-functional for a week or two, so total parenteral nutrition (TPN) needs to be administered through a peripherally inserted central catheter (PICC). This provides access to a large vein in the upper chest that allows infusion of highly concentrated glucose. Even after intestinal function recovers, most patients continue to have a poor appetite for a week or so longer. During this interim, nutrients may need to be given through a nasogastric (NG) tube.
Reduced or absent urine output (oligoanuria) occurs in most cases and usually lasts about a week, but can be as brief as two to three days, or as long as a month or greater. Dialysis is required during this time to cleanse the body of uremic toxins and to maintain fluid and electrolyte balance. Peritoneal dialysis (PD) is usually used for young children unless the colitis is severe. Fortunately, the colitis is often resolving by the time PD becomes necessary. Treatment requires placement of a catheter (tube) through the abdominal wall into the peritoneal cavity. Older children and adults are treated with hemodialysis that circulates blood through a hemodialysis machine to remove uremic toxins, normalize blood chemistries and correct any edema (swelling). This requires that venous access be established by inserting a temporary catheter into a major vein that returns blood from the upper body to the heart.
The majority of HUS victims require one or more blood transfusions to treat severe anemia; platelet transfusions are sometimes needed to diminish the risk of bleeding in those with severe thrombocytopenia (i.e., platelet counts less than 10,000), to control bleeding, or in preparation for an invasive vascular procedure that can cause hemorrhage (e.g., insertion of a hemodialysis catheter).
More than half of patients experience high blood pressure (BP) that is usually mild and labile, but may be severe enough to require treatment with anti-hypertensive drugs. This condition usually resolves prior to, or soon after discharge from the hospital.
One has to remain vigilant for signs of extra renal involvement. Intestinal necrosis and perforation can occur at any time during the acute phase of the disease and can be fatal if not promptly diagnosed and surgically treated. Pancreatic damage can cause sugar diabetes that is almost always temporary, but may require insulin. Heart involvement is rare, but can be fatal. Brain involvement can cause stroke and/or cerebral edema (swelling of the brain).
More frequently, however, the encephalopathy (brain dysfunction) is the result of acute metabolic imbalance (metabolic encephalopathy) and is due to abnormalities in the blood concentrations of sodium, glucose, calcium, or to very high levels of metabolic waste products. Since these abnormalities are the result of the acute kidney failure, they can be corrected by dialysis, and the outcome is favorable. The prevalence of metabolic encephalopathy 25 or more years ago was about 50%. With earlier diagnosis and more timely treatment, the prevalence is now down to about 25%. Convulsions are the most dramatic manifestation, and are more likely to occur in toddlers (30%) than older children (15%). Unfortunately, structural damage to the brain (i.e., stroke, swelling) has not decreased over time. When swelling is severe, the pressure strangulates the brain stem that is responsible for maintaining blood pressure, heart rate, and breathing. This usually results in rapid death. Fortunately, children who survive display an amazing ability to recover, though neurological impairment can be permanent.