PHA Pathlight Spring 2014 - (Page 34)

Persistent Voices editor's corner Hello again, PH Community! I hope this finds everyone emerging from this very tough winter and stowing away the hats, gloves and scarves. Spring is almost here, giving us a chance to refresh, regenerate and see life anew. We're sharing two stories with you today, both with overarching themes of NOT allowing illness to prevent us from living and enjoying life. - Joanne Sperando-Schmidt, Persistent Voices Editor Chloë Rand's mother Marie shares her daughter's PH journey with us. It began just after her birth in 1992 before Flolan® was even an approved therapy. Marie says she "learned to be the mom of a sick child" by educating herself and advocating for Chloe. She was fortunate to find the late Dr. Robyn Barst, whose care extended Chloe's life and gave the family additional precious years together. You can read more about Marie's connection to PHA's Greater New York and Philadelphia Chapter on page 31. - Joanne Marie Mascia-Rand, Princeton, N.J. i n 1992, I gave birth to a beautiful baby girl, Chloë Rose Mascia-Rand. On day four of life, Chloë stopped nursing and began sighing each time she took a breath. I took Chloë to the pediatrician who called me a "first time worrying mother." We returned to the doctor several times over the next few days. Finally, a new doctor stuck his pinky in Chloë's mouth, and when she turned blue, we were rushed to Cornell Medical Center where Chloë was diagnosed with multiple cardiac anomalies including a ventricular septal defect (VSD) and a coarctation of her aorta. On her eighth day, Chloë underwent a six-hour heart surgery to replace her aorta. By day 10, Chloë was off life support and doing well. I took the opportunity to have dinner with my nephew. When I returned to the hospital 45 minutes later, Chloë was in full cardiac arrest. The doctors told us Chloë also had diabetes insipidus and a rare neurologic condition. The doctors felt she would not survive, but I disagreed. I learned to draw and administer medications and to place a gastrostomy tube. I learned to be the mom of a "sick child." After seven weeks, we went home. During the months that followed, Chloë required hourly maintenance with either medication or water and was tube fed 24 hours a day. We were granted 12 hours of nursing care a day. Each day that I woke and she woke, too, was a good one. With all that it took to keep her alive becoming routine, I focused on teaching Chloë to enjoy life. It wasn't long before the nurses and I began to recognize things like Chloë waving bye-bye as Grandma and Grandpa were leaving from a visit. At 8 months old, Chloë had her second heart surgery. We left the hospital after just 10 days with new hope. The third and final surgery, the VSD closure, was the one the doctors said would be "a piece of cake." We returned for pre-op testing, and Chloë was added to the operating room (OR) schedule. A week before surgery, as I scurried through the halls of the hospital, I bumped into a nurse with whom we had become friendly. She said, "Marie, I'm so sorry to hear about Chloë." Apparently, Chloë's name had been removed from the OR schedule. I marched into the office of cardiology. PRIMARY PULMONARY HYPERTENSION - That's what was found during the pre-operative testing - a fatal disease causing abnormally high pulmonary pressures. The cardiologist said Chloë would not survive the VSD closure with pressures elevated to twice that of the average adult. 34 PATHLIGHT SPRING 2014 Marie Mascia-Rand

Table of Contents for the Digital Edition of PHA Pathlight Spring 2014

PHenomenal Lives
Coping with PH
Health Matters
Ask a PH Specialist
Helpful Hints
Research Corner
Advancing the Cause
Support Groups
Special Events
Chapter Happenings
Persistent Voices
Community Classroom
Conference Preview
PHenomenal Youth
Family PHocus

PHA Pathlight Spring 2014