Background_x000D_
Recent advances in the treatment of Duchenne Muscular Dystrophy (DMD) have led to the approval of multiple new therapies. Exon skipping medications aim to restore the disrupted reading frame and thereby lead to production of an internally truncated dystrophin protein. The currently approved therapies are administered weekly via intravenous infusion. Due to difficulties with frequent infusions, many patients utilize implantable venous access devices (IVADs), commonly known as ports. While these devices have increased the ease with which these medications can be administered, they require management due to risk for complications such as migration, malfunction, infection, pneumothorax, and catheter-related thrombosis. These issues can lead to additional healthcare-related burdens for the patients and their families. In addition, standard operating procedures for the care of these devices must be developed for neuromuscular programs, who previously may not have experience in this area. _x000D_
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Objectives_x000D_
To review the use of ports in patients with DMD cared for in the Neuromuscular Program at Children’s Health in Dallas, TX. _x000D_
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Results_x000D_
Thirty DMD patients with ports were identified. There were numerous complications experienced, including access difficulties leading to multiple attempts and pain, fibrin sheath requiring tissue plasminogen activator (tPA), local cellulitis at port site, hospital admission for evaluation of fever, pneumothorax, and malfunction or migration requiring replacement. There were two central line-associated bloodstream infections, both occurring in one patient a year apart. Thirteen patients required port replacements during this time frame, most commonly due to fibrin sheath that could not be stripped or catheter malpositioning due to growth. One patient had 3 total ports, another had 4. The longest time period that a port was in place was 7 years; the shortest was 2 weeks. _x000D_
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Conclusions _x000D_
The advancement of therapies for DMD has led to improvement in life expectancy. However, several of these therapies are administered intravenously, which has increased the use of ports in this population. The need for lifelong access also presents a challenge, requiring replacement periodically. Although ports have increased ease of administration of disease modifying therapies, they will require additional monitoring and development of protocols to expeditiously manage any complications.