Platform: OUR PLATFORM IS TO Raise awareness About spondylocostal dysostosis, klippel — feil syndrome, and thoracic insufficiency syndrome. FURTHERMORE, TENLEIGH’S TROOPS PROMOTES advocacy and support!
Disclaimer: The content is NOT intended to be a substitute for professional medical advice, diagnosis, or treatment. ALWAYS seek the advice of your physician or other qualified health providers with any questions you may have regarding a medical condition.
Supporting Parents of Medically Complex Children:
It is not about who came first, but who came and never left.
Mama & Tenleigh before Surgery #20.
In general, SupportingParents of Medically Complex Children can be a difficult obstacle. Once inducted into “the parents of special needs club”, their priorities and responsibilities reach a whole new altitude. Suddenly, the parenthood they once expected turns into a battle of survival.
Post-Traumatic Stress Disorder in Parents of Medically Complex Children:
Additionally, the Journal of Health Psychology conducted an analysis correlating the prevalence of post-traumatic stress disorder (PTSD) in parents of children with a chronic illness. Overall, the data showed that 23% of parents met the criteria for PTSD following an extended Pediatric Intensive Care Unit (PICU) hospitalization. Furthermore, parents of medically complex children live in a state of hyper-vigilance, meaning they are in a state of increased alertness. In summary, the fight or flight response becomes their active defense system.
Symptoms of Post-Traumatic Stress Disorder:
When most think about post-traumatic stress disorder (PTSD), combat soldiers usually come first to mind. However, these last two challenging years of my life have brought me greater insight into the disorder. According to the Anxiety and Depression Association of America (ADAA), PTSD can include some of the following symptoms;
Usually, PTSD is related to recurring flashbacks or nightmares, causing them to relive the trauma repeatedly.
Severe distress is evident, reminded of the trauma. Triggers are included but are not limited to words, objects, situations, and more.
Hyper-vigilance and being easily frightened are some of the many symptoms that cause disruptions in daily routines.
Likewise, PTSD causes difficulty concentrating and general memory problems. PTSD often results in amnesia when recalling events related to trauma.
Without treatment, PTSD results in feeling hopeless about the future, emotional numbing, detaching self from others, and engaging in self-destructive actions.
Additionally, PTSD may also experience irritability, anger, overwhelming guilt, or feeling on edge following their trauma.
Lastly, PTSD can cause hallucinations or hearing noises that are not real.
As expected, parents of medically complex children are more than likely checking off a few symptoms as well.
Becoming the “Expert” in a Rare Condition:
Raising a child with a rare medical condition is both busy and unpredictable. After discovering our child’s diagnosis, we devote our entire soul to researching, consultations, appointments, therapies, and so on. As a result, becoming an expert in your child’s condition is a life-altering process and is not easily relatable to other parents. Sooner or later, we turn to the internet in desperation to find others who “get it.”
The “Invisible” Patients:
It’s not easy, but it is worth it.
Supporting Parents of Medically Complex Children.
We carry endless bags of medical supplies, specifically for emergencies.
The inside of our house looks similar to a hospital supply room – you never know when there could be a pandemic!
Not to mention we always have at least one back-up plan. In other words, crisis preparation is our hobby.
Our doctors are on speed dial because We do not have the time or patience to beat around the bush.
Overall, the majority of our conversations are with doctors, nurses, therapists, DME, surgical coordinators, etc.
In fact, we can recite our child’s medical history without skipping a beat.
Without a doubt, this all came with countless sleepless nights spent researching.
Packing Tenleigh’s medical supplies for Surgery #21!
Finding the Right Support Group is Key!
Most importantly, parents need to consider joining a support group, but the trick is finding the right one. Although there is no manual on raising a child with spondylocostal dysostosis, there is a Facebook group consisting of 906 members. These members have also earned the title expert in Thoracic Insufficiency Syndrome. Above all, these are the experts we go to when the doctor cannot be reached.
Parents and VEPTR children relay at the Thoracic Insufficiency Syndrome Reunion! Yes, I thought the clown bow went on my head…
VEPTR Moms and Dr. Campbell.
Haylee, Tenleigh, Dr Campbell, and Lee at the Thoracic Insufficiency Syndrome reunion in 2018.
The first time Isla, Tenleigh, and Haylee met! The Homewood Suites in University City.
Isla and Tenleigh! Isla has spondylothoracic dysostosis and Tenleigh’s condition is spondylocostal dysostosis. Both conditions fall under the umbrella term Jarcho-Levin Syndrome.
SupportingParents of Medically Complex Children.
Raelyn, Tenleigh, and Trilby
Wendy, Raelyn, Tenleigh, Trilby, and Haylee at the 2018 Thoracic Insufficiency Syndrome reunion.
The 2018 Thoracic Insufficiency Syndrome Family Reunion.
Halo Gravity Traction Extended Family at Boston Children’s Hospital:
During the thirty-nine days admitted at Boston’s Children’s Hospital, the halo blessed us with multiple families who understood the extent of our struggles. Usually, we are not inpatient with families going through the same experience, and certainly not for as long as we were. For this reason, departing from Boston was the epitome of bittersweet.
You were given this life because you are strong enough to live it.
Supporting Parents of Medically Complex Children:
Overall, parents of medically complex children yearn for human interaction, just like everyone else. Additionally, we want to relax with family and friends despite always discussing the medical world. Although the experts cannot tell us what the future will bring, positivefeedback and normalcy is the golden ticket to ease our nerves.
What you can do to support a family member or friend who has a medically complex child:
First, wewantyou to read this!
Second, offersupport because we only share a fraction of our trials and tribulations.
Invite them to have a mom/dad night because god knows we need it!
Ask questions because we secretly love to educate others!
We crave human contact just like everyone else.
Do not compare parenting styles because they are incomparable.
Without a doubt, our spouses and parents have heard it all. Therefore, take the time for a venting session, especially without judgment.
For the most part, our responsibilities and priorities revolve around keeping our children alive.
Offer to watch their child, especially for a quick errand run.
By all means, initiate a call or text to see how they are doing.
Research the condition of the child.
To demonstrate an example of inclusion, accommodate their child if possible.
Above all, we ultimately do not want to become a burden.
Please give us feedback and share to promote awareness to supporting parents of medically complex children!
The creator and blogger of Tenleigh’s Troops and a Nurse Technician since 2009. The previous place of employment was at HSHS St. John’s Children’s Hospital in Springfield, IL.
The platform is to bring awareness to spondylocostal dysostosis, klippel – feil syndrome, and thoracic insufficiency syndrome. In addition, Tenleigh’s Troops also promotes advocacy and support.
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.
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