Conference: 2011 PH Professional Network Symposium
Release Date: 09.22.2011
Presentation Type: Abstracts
M Bagby, RN, MSN, CNP, R Hirsch, MD
The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
PURPOSE: To discuss the methodology and experience of initiating de-novo sub-cutaneous treprostinil in a young pediatric patient where no evidence based practice data exists.
BACKGROUND: De-novo commencement of sub-cutaneous prostanoid therapy for pulmonary hypertension (PH) in young pediatric patients is based on various medical and practical considerations. While treprostinil is approved for continuous sub-cutaneous infusion therapy, the best practice when initiating such therapy in this age population is not established. This report summarizes the planning and logistic considerations involved with the de-novo initiation of this therapy in a complex 5 year old patient at our center.
METHODOLOGY: The patient's medical and social situations were carefully reviewed. Special attention was given to the progression of her pulmonary vascular disease, response to conventional oral vaso-dilators, medical co-morbidities, need for chronic central venous access and developmental level. A detailed plan for the safe initiation of the drug in an intensive care setting was established with input from pulmonary hypertension experts, the manufacturer, and support from her family and Child Life Therapists.
FINDINGS: The patient was a 5 year old female who underwent repair of congenital diaphragmatic hernia and tetralogy of Fallot in infancy. Due to severe tracheal-bronchomalacia, she had been ventilator dependent for the first 14 months (and still retains a tracheostomy). A cardiac pacemaker had been placed for complete heart block. Increasing right ventricular (RV) pressures were first noted at 12 months. With steady deterioration in her PH, an incremental approach to therapy was assumed, initially with sildenafil, and later with the addition of bosentan. During her course, repeated cardiac catheterizations ensured an unobstructed RV outflow tract without pulmonary artery stenoses. At 5 years and 5 months of age, despite maximal dosage oral pulmonary vasodilators, her RV pressure was systemic. She was also increasingly fatigued. A sleep study and repeat airway evaluation demonstrated no obstruction, carbon dioxide retention or hypoxia. As next line of therapy, sub-cutaneous treprostinil was chosen with the following considerations in mind: i) Risk of central line infection relative to the proximity of the tracheostomy; ii) Need for life-long central venous access in light of pace-maker dependent congenital heart disease; iii) The child's activity level and impact of sudden discontinuation of therapy in event of pump or central line failure; and iv) The adverse impact on quality of life with a tunneled central catheter. Successful drug initiation was without incident during an admission of less than 72 hours. Her subsequent clinical course has been marked by improvement in symptoms, mild site pain, and improved hemodynamics on follow-up catheterization.
IMPLICATIONS: We have shown that the selective use of de-novo subcutaneous prostanoid therapy can be successfully instituted and maintained in a young pediatric patient with complex medical requirements. With careful planning and family support, the medical and quality-of-life advantages of sub-cutaneous therapy could be offered to other pediatric patients with similarly complex medical situations.