|Talli S. (Born on October 4th, 2001 Passed on February 17th, 2019)
Talli gracefully and fiercely endured the terrible nastiness of Propionic Acidemia; she departed this life with a sepsis scorecard of Talli 5 and sepsis 1.
She only encountered two bowls of nacho cheese sauce that she did not love; frequently devoured Scooby-Doo fruit snacks, except the orange ones; and was determined to be independent and do things in her way and order, unless she wanted you to be her servant. She could build a Lego tower of questionable stability far larger than her 4’8″ self. Male teens athletes from her Church, were in awe of her endurance and strength in keeping up with them while pushing a handcart for miles in a Mormon Trail reenactment. She hated to miss Church services at the Naperville 3rd ward of The Church of Jesus Christ of Latter-day Saints, even if she was hospitalized or vomiting and would tell anyone who would listen to “watch Veggie Tales to learn a lesson”.
She was highly anticipating her senior year next year at Naperville Central High and she felt she was one of the star athletes of Special Olympics, Team Fire 203 in basketball, bowling, and track. She loved meeting others who have PA; whether it was at a PAF meeting or traveling the country and stopping by to see them at their homes. She had numerous complications from PA including loosing most of her vision and had a compromised immune system; however, she kept a positive attitude and dealt with her challenges with resolve.
Talli is seemingly always on the go, when she isn’t she wants to know what the plans are for after school and the weekend. She loves to go to school and hates missing class if she is sick or has an appointment. She can’t stop talking about an upcoming trip to see her grandparents in Oregon and a sleep-away camp with the teenage girls from our Church over the summer. She loves technology, music, and live performances. We have had to put on parental controls on her tablet just like her typical peers have. She got braces on just after her birthday in the fall.
She is in the 7th grade at our local Junior High School. She is in an Instructional class for her core classes and is mainstreamed for PE, science, choir, and a period that rotates between art, technology/engineering, sewing, and cooking. This year she was on the school’s 7th grade cheerleading squad and she just excelled! She attended practice every day and then came home and practiced some more. She is very excited for next season.
Overtime, new health challenges have some up and she now has some complicated treatments. She was fortunately newborn screened; we found out a week after she was born. She was already in crisis at the time and took several weeks to stabilize her in the NICU. The biggest challenge at that time in keeping her stable was severe food allergies and reflux. She vomited every feeding! Thankfully, she has outgrown some of her food allergies, however, tree nuts and latex can affect her by just being around them.
She had frequent infections and was hospitalized every couple weeks when she was an infant and toddler. After starting IV immunoglobulins (IVIG), she had a significant reduction in the number of illnesses and hospitalizations. She has had sepsis several times, one of those times infecting her hip which required surgery. She has a significant Long QTc wave and had an internal defibrillator placed when she was in the 2nd grade. She also has hypothyroidism and was recently diagnosed with growth hormone deficiency, so takes daily growth hormone injections.
After many years of going to the hospital for IVIG infusions, she will be starting weekly subcutaneous at home. This will keep her better protected from infections, but also free up her (and our) schedule and cause fewer side effects. She had received IVIG at our local hospital which has a Pediatric Critical Care Unit because of poor tolerance to it.
Eating always seems to be a concern for newly diagnosed families. Talli eats inconsistently. We think that a lot of it has to do with her severe food allergies. She had a g-tube placed when she was 6 months old as she was failure to thrive. Her growth only improved once all of her allergens were removed from her diet and her esophagus was allowed to heal (she had erosive esophagitis). She eats a lot more when she can have whatever she is craving at the time (right now it is Taco Bell) and when she is around her friends. She consistently eats about a third of her calories by mouth. She is learning to weigh her food and typically does her own feedings at school, if they are needed, with her nurse supervising her. She is also learning to read labels for her allergens and protein content. Update May 2015
Talli is a thrilled-seeking social butterfly.
She will turn 8 years old in October and she is just finishing up the 1st grade. She is mainstreamed in our local elementary school with resource support, speech and OT. She participates in jazz and ballet and Daisy Scouts. She LOVES to build with Legos, take things apart, play with her friends, and visit Disney World. Her favorite rides are the Tower of Terror, Splash Mountain, Space Mountain and Journey to Atlantis at Sea World.
She was diagnosed with Propionic Acidemia from newborn screening, although she was well into her first crisis situation when we received the results of her testing. She spent nearly a month at CHoP to stabilize her and get her diet just right. She has had over 20 hospitalizations because of illness. For the past 3 years, she has had received IV immuglobulins every four weeks.
She is unable to have the infusion run quickly, so she always has an overnight stay in the hospital. We attempted to have her use a port-a-cath, but after a year of no problems she had two episodes of sepsis, the last one being fungal and bacterial. She has other health challenges that have made it difficult to keep her stable all of the time and also just adds to her complexity. She has Long QT Syndrome, hypothyroidism, allergies, asthma, esophagitis, GERD, secondary immunodeficiency, and occasionally, she has neutropenia, low platelets, and anemia. She has a history of sleep apnea, is on her 4th set of ear tubes, has had her tonsils removed once and her adenoids twice.