Long-term central IV access in patients with mitochondrial disease

Mitochondrial disease results from alteration in genes that control mitochondrial function. Patients with this disease present with multisystem organ involvement that may include gastrointestinal (GI) tract dysfunction, including obstruction, pseudo obstruction, bowel infarction and malabsorption syndromes. For this reason, care of this population may require long-term central intravenous (IV) access for administration of hyperalimentation, fluid, medications and blood products. Additionally, these patients may be immunosuppressed and at risk of sepsis. With vulnerability across many organ systems, symptom exacerbation can result from any physiological, psychological, or environmental stressor. There is no cure for mitochondrial disease, and quality-of-life goals are paramount. If GI tract dysfunction develops, treatment may require high-acuity level home care that includes the use of a central IV access device. This article presents an overview of this challenging population, and a plan for safe and effective central IV access care.