Treatment

There is no cure for MVID, and the current treatment options are risky and can limit the patient’s quality of life. Children with MVID should be under the management of specialized pediatric gastrointestinal centers and professionals.

Intravenous nutrition

The most common method for managing MVID is parenteral nutrition or PN, in which fluids and nutrients are delivered directly to the bloodstream of the patient, bypassing the intestine. If patients only get nutrition from PN, and nothing is taken by mouth, the procedure is known as total parenteral nutrition or TPN. Because of the persistent nature of the diarrhea, patients often need continuous TPN, up to 24 hours a day.

Dependence on TPN creates a challenging way of life and can result in infection, damage to the veins and – over time – liver failure. The majority of children who have unmanageable complications due to TPN must resort to a last option, a small bowel and/or liver transplant.

Organ transplants

Children with MVID have successfully had small bowel and liver transplants, but the process comes with serious challenges. The average wait time to find the proper organ donor ranges from four months to one year. The surgery is complex and lasts from four to seven hours. After a successful transplant, the patient must undergo immunosuppression therapy each day for the rest of his or her life. This therapy essentially lowers the patient’s immune system, preventing rejection of the new organ. Because of the risks of this option, a conclusive genetic test is recommended before a patient has transplant surgery.

Diagnosis and PrevalenceGenetics