Parenting a Medically Complex Child During a Pandemic

Tenleigh Joy Stokes is the eight-year-old daughter of Lee Stokes and Haylee Shrake of Pana, Illinois. At thirty-two weeks’ gestation, Haylee unexpectedly started showing severe signs of pre-eclampsia, resulting into HELLP Syndrome (hemolysis, elevated liver enzymes, and low platelet counts). Weighing 2 pounds 6.6 ounces and 14.6 inches long, Tenleigh was induced into this world on March 23, 2012. Due to her prematurity, Tenleigh remained at St. John’s Hospital in Springfield, Illinois for thirty days in the Neonatal Intensive Care Unit (NICU). Aside from being small, she appeared to be your average healthy newborn. However, at four months old she shocked doctors in central IL with a never before seen condition. Tenleigh’s physician ordered an X-ray in concerns of a respiratory infection after noticing that she was “tugging” to breathe. The X-ray showed multiple skeletal abnormalities including scoliosis, torticollis, hemi-vertebrae’s, a small chest wall, along with fused and missing ribs. Doctors across Illinois were left speechless due the complexity of Tenleigh’s unknown condition. The doctors unanimously confirmed that without surgery, Tenleigh would not survive infancy. Eventually her spine would crush her lungs causing her own ribs to puncture her lung and suffocate. As the months passed, Tenleigh’s condition worsened. She was admitted repeatedly for respiratory infections, including two weeks in the Pediatric Intensive Care Unit (PICU) for respiratory syncytial virus (RSV). Despite the poor prognosis, Tenleigh’s parents did not give up hope. After taking research into their own hands, the twenty and twenty-one-year-old parents found hope thirteen hours away from home at the Children’s Hospital of Philadelphia (CHOP). Dr. Robert Campbell Jr., known for inventing the life saving device called the Vertical Expandable Prosthetic Titanium Rib (VEPTR). The device was created to treat children with rare syndromes and disorders that involved malformed rib cages or missing ribs. The first implantation took place in 1989 but has been modified since to accommodate more critically ill children: “On Aug. 23, 2004, the U.S. Food and Drug Administration approved VEPTR (H030009) as a medical device under the Humanitarian Device Exemption Program. A Humanitarian Use Device is intended to treat or diagnose a disease or condition — such as thoracic insufficiency syndrome — that affects fewer than 4,000 people in the U.S. per year” (www.chop.edu). In May 2013, Tenleigh, her parents, and grandparents drove out to Philadelphia, PA for a consultation with Dr. Campbell. During the consultation, the family was finally given a name for Tenleigh’s unknown condition, spondylocostal dysplasia. According to the National Organization for Rare Diseases, the exact prevalence of spondylocostal dysplasia is unknown. In severe cases like Tenleigh, deformities of the chest, spine and ribs prevent normal breathing, lung growth and lung development. The inability to expand the chest sufficiently causes a decrease in lung capacity, known as Thoracic Insufficiency Syndrome. These malformations increase the risk for repeated respiratory infections, difficulty breathing, pulmonary hypertension, heart failure, chronic back pain, etc. Due to the complexity and severity of Tenleigh’s disorder, Dr. Campbell recommended coming back in August to run tests and ultimately implant the VEPTRs. Tenleigh was admitted to the Children’s Hospital of Philadelphia eight days prior to surgery for monitoring and testing. On August 20, 2013, Tenleigh had her fused ribs separated and two VEPTR rods implanted on both sides of her spine. The top of the VEPTR was clipped around an upper rib and anchored to her pelvis. The surgery was a success and twenty-four hours later, Tenleigh came off the ventilator. She remained hospitalized for a week, before beginning her six-week recovery at home. For the first year and a half, Tenleigh battled repeated staphylococcus aureus infections. Tenleigh was transferred twice via jet from St. John’s Children’s Hospital to the Children’s Hospital of Philadelphia. The repeated infections resulted in a Peripherally Inserted Central Catheter (PICC Line) for antibiotics, negative pressure wound therapy (NPWT or Wound VAC) to clean her open wound, multiple debridement surgeries, removal of her left VEPTR rod and placement of a gastronomy tube. After nine surgeries within a year and a half, Tenleigh finally caught a break. Although Tenleigh remained healthy, the family had more unexpected battles. Since birth, Tenleigh has relied on Medicaid to fund the majority of her expenses. The family was initially informed that if they were to pay in full, the VEPTR implantation would be approximately $100,000. Each VEPTR expansion performed every four to six months would be approximately $20,000 and a VEPTR revision or exchange would be in the middle. In the Spring of 2015, CHOP informed the parents that Illinois Medicaid had not paid any of the bills and was refusing to fund future surgeries. The family hit a breakthrough after many letters from Dr. Campbell, Illinois doctors, Illinois State Representatives, along with the power of the news and social media. When the family arrived for Tenleigh’s VEPTR expansion, they were informed that Dr. Campbell had an unexpected death and would have to reschedule. Tenleigh had gone almost seven months without surgery due to insurance and was experiencing significant pain. Dr. Campbell suggested having his new partner, Dr. Cahill perform the surgery. The parents agreed and the surgery appeared to be a success. The following month after surgery, Tenleigh’s left VEPTR started to prominently appear. Although she was not complaining of pain, it looked as if the rod could come through her skin at any time. However, Dr. Campbell’s health became compromised and the family had to transfer care to Dr. Cahill in order to have immediate surgery. Tenleigh’s VEPTR had completely eroded through her ribs and needed to be reattached. Unfortunately, Tenleigh often needed revision surgeries in between her VEPTR expansions over the course of the next year and a half. On April 25, 2018, Tenleigh was scheduled for a VEPTR revision and expansion. However, due to an uninformed decision made by the medical personnel, MAGnetic Expansion Control (MAGEC) growing rods were implanted in place of Tenleigh’s VEPTR’s. Tenleigh spent twenty days hospitalized from complications related to the MAGEC rods. Tenleigh’s body rejected the rods, requiring two transfers from St. John’s Hospital to the Children’s Hospital of Philadelphia. The complications resulted in removing both MAGEC rods until Tenleigh could recover. Tenleigh spent over a year recovering from infections, skin breakdown, and trying to gain weight. She was also required to wear a Thoracic Lumbar Sacral Orthosis (TLSO) brace to prevent her spine from progressing. The plan was to postpone another implantation surgery until Tenleigh gained sufficient weight or if she began to experience respiratory complications. However, during the summer of 2019, Tenleigh started to become short of breath and severe back pain. A local x-ray showed that Tenleigh’s degree of spinal curvature had increased from 97 degrees to 120 degrees within three months. Due to the severity of Tenleigh’s condition progressing, a halo gravity traction was recommended by Dr. Emans at Boston Children’s Hospital. On January 01, 2020, the family began their undetermined extended stay at Boston Children’s Hospital. Tenleigh had the halo application applied to her skull with four pins on January 07, 2020, spending the next month attached to a weighted pulley system to naturally lengthen her spine. On February 06, 2020, Dr. Emans removed the halo and implanted two customized growing rods. The results were phenomenal! Tenleigh’s Cobb Angle (spinal degree of curvature) improved from approximately 120-130 degrees to 60-70 degrees! Tenleigh immediately felt relief and could preform her activities of daily living (ADL’s) better than before! She continued to impress the doctors and recovered at lightning speed! After 59 days away from home, the family finally returned home on February 28, 2020. View all posts by Hayls