On June 16th, 2010, Everett and Cooper Hornung were fraternal twin boys born at 5lbs & 5oz and 6lbs 3oz, respectively, to a set of very excited and determined parents in Austin, Texas who had overcome both medical and economic challenges to bring these boys into the world.
After undergoing in vitro fertilization, mother, Theresa Hornung got laid off from her job during her seventh month of pregnancy and father, Wyatt Hornung’s independent contract expired just weeks before her giving birth. While they were forced to live off of unemployment and health care coverage by COBRA, these economic obstacles and less then desirable circumstances did not even come close to dampening the heightened emotions of joy as this couple was blessed with the miracle of not just one life, but two.
As every new parent can attest, Theresa and Wyatt Hornung were overwhelmed with the amount of love they felt for their newborn children. The doctors and nurses cooed over their baby boys, but there was some additional murmuring and fundamental strain when they worked with Cooper that triggered the mother’s warning circuits. It became apparent, that their son, Cooper, was going to face a few challenges of his own as he made his mark into this world.
A doctor believed that Cooper had a rare chromosomal syndrome called: Beckwith-Wiedemann Syndrome, (“BWS”) due to his large tongue, pitting in his ears, low blood sugar and overly developed muscle tone in his legs. The tongue, however, was the dead giveaway.
BWS is an overgrowth disorder often characterized by overgrowth of internal or external organs and sometimes a combination of both. The cases for children with Beckwith-Wiedemann Syndrome are varied, in fact, no two cases are alike. There is a long list of symptoms, but generally there are five major characteristics to look for:
1) Macroglossia (large tongue)
2) Hypoglycemia in the first four months (low blood sugar)
3) Ear Creases or Pits
4) Abdominal wall defect
5) Birth weight 90-95% over the norm
Source: Beckwith Wiedemann Children's Assn of NZ (Inc)
Dr. Ankur Rana, Pediatric Surgeon at Dell Children’s Medical Center (“DCMC") says, “Children with BWS have a higher risk of certain disorders which require certain operations. BWS is associated with a type of congenital abdominal wall defect called omphalocele which may need an operation as a newborn. These children are also associated with a higher incidence of abdominal tumors, so they undergo an extensive screening program involving blood work and ultrasounds which may last until 10 years of age.”
The Hornungs were fortunate to have family pediatrician, Dr. Dick Worrel at the Austin Diagnostic Clinic, who really guided the family with delicate care and understood the risks associated with BWS, obtained the appropriate screening tests and recommended them to a pediatric geneticist.
Tests performed by pediatric geneticist, Dr. LaDonna Immken at Dell Children's Medical Center of Texas, indeed, confirmed that Cooper had BWS. Immken, however, knew it was BWS before any blood tests came back, helping the Hornungs understand their situation much earlier. Theresa stated, “When you hear a word like ‘syndrome’ in reference to your baby, it makes your heart stop and your stomach clench, but I only loved him even more fiercely. I knew that it didn’t make a difference to me.”
The Hornungs absorbed all of the information given to them about this potentially life-threatening syndrome, such as the increased risk of childhood cancer, “about 7% chance of developing an abdominal tumor with kidney tumors being more common. (Wilms' tumor)”, says Rana.
Studies show that “with screenings, most BWS cases are ‘treatable, manageable, or even curable’ and there is a likelihood that a child born with BWS will grow to be a normally healthy adult.”
Source: Beckwith Wiedemann Children's Assn of NZ (Inc)
BWS is a genetic syndrome, but there are many cases, as in the Hornungs, where the parents have no family history of it.
There are studies that say that BWS is correlated with in vitro fertilization. “The research indicates that the chance of a child being born with BWS increases markedly in children that are conceived using IVF. Current estimated occurrences of BWS for natural fertilization are around 1:15000, whereas the occurrence for IVF conceived children appears to be somewhere around ten times that (1:1500).” Source: Beckwith Wiedemann Children's Assn of NZ (Inc)
Theresa said, “I would not have changed a thing. I would still do it with the risks. I would not do anything to take it away. I wanted kids. I loved them.”
As Cooper grew, Theresa and Wyatt noticed that Cooper couldn’t close his mouth. At six months old, upon the advice of a medical team, they opted to have surgery on Cooper’s tongue to reduce his size in order to avoid painful jaw surgeries at a later stage in life.
Pediatric plastic surgeon, Dr. Jeffrey L. Marsh at Saint Johns Mercy Medical Center in St. Louis, world renowned for his work with facial birth defects, including children with BWS Syndrome, operated on Cooper.
Dr. Marsh has evaluated 412 children and done 318 tongue reduction surgeries to date. The Hornungs had great confidence in Dr. Marsh and felt very fortunate to have a physician with impeccable surgical skills combined with authentic humanity. When asked why he became a pediatric plastic surgeon, Dr. Marsh humbly replied, “To use my surgical skills to improve the physical and psychosocial functions of children with birth defects involving the face and skull.”
Marsh said that Cooper’s tongue reduction surgery involved the “removal of three wedges of tissue from the front and sides of his tongue to reduce its size so that the teeth and jaws could assume a normal relationship and, hopefully, avoid the need for jaw surgery as a teenager.”
Cooper heroically endured the pain, stitches and trouble with eating, with a burst of positive energy that could convert a cynic and a smile that could brighten anyone’s day. His tongue healed beautifully and his brother Everett had his companion back in full form again. Early childhood intervention specialists suspect that Everett was aware of Cooper’s trauma and decided to hold off with his social development. Cleverly, Everett turned up his baby charm to get what he wanted without having to speak. The unspoken brotherhood bond had formed and nothing could break it. Both twins were now equally behind on speaking.
However, there was another obstacle looming on the forefront that was detected on Cooper’s first birthday that would require Cooper to put on a new set of boxing gloves. During an ultrasound check-up, a spot was detected on his liver. An MRI confirmed the Hornung’s fears. Cooper had a tumor.
Another well-respected physician joined Cooper’s journey, who became the captain of the ship trying to steer Cooper back to a healthy port. Dr. Don Wells, Pediatric Oncologist and Hematologist at Specially for Children Medical Group, affiliated with Dell Children’s Medical Center of Central Texas, determined that Cooper had a malignant hepatoblastoma. At that point, Wells had to navigate all the possible road maps with the Hornungs in his attempt to eradicate the tumor. His gentle hand, tender heart and clear information spoken with a charming southern accent, reassuringly guided the Hornungs through the nightmare.
Wells oversaw the treatment plan, first collaborating with the highly esteemed and soft hearted, Dr. Ankur Rana, who removed a grape sized tumor during a 90 minute surgery. Both Rana and Wells find it very gratifying to work in a specialty like pediatric care, where there is a higher cure rate and the continuity of care and relationship developed with the patient and family.
Within a week, Cooper was giving family and friends his heartwarming smiles and crawling happily with his brother, Everett. Cooper deserves a special accolade for his extraordinary human spirit which refused to let these invaders stop his journey with his loving family. He has shed such fierce spiritual determination; one can only imagine just how much more greatness and inspiration this remarkable boy will give to the world with his angelic soul.
Cooper had not crossed the finish line yet, however. Wells had to implement four rounds of chemotherapy in case there were any residual cancer cells. He says, “We are a bit more aggressive with dose intensity with children because they are more resilient. Their organs are stronger. We push the doses, but decrease the frequency.” While the Hornungs were concerned of the effects of the toxicity on Cooper’s development, Wells says, “the toxicities are reversible.”
Theresa said, “My greatest hope for Cooper is that he doesn’t remember it. He can show his scars and say, ‘I beat cancer before I was two years old. What did you do?’”
While Cooper was staying strong during his chemo-treatments, there were great financial after-effects for the Hornungs that at times weakened their spirit. Prior to Cooper’s liver surgery, unbeknownst to them, their COBRA had run out. After a large amount of appeals, they obtained coverage through the beginning of chemotherapy at an exorbitant price, $1,500 a month plus 20% of all the costs. Once that ended, they had two full months without any health care coverage until CHIP kicked in, a children’s Medicaid Program in Texas.
Family and friends all over helped the Hornungs, participating in a triathalon to raise funds for Cooper and general donations, but even with family and community support; this young couple’s lifelong savings were depleted, emphasizing the frightening realism of our country’s healthcare costs. Theresa says, “our system is clearly broken.”
She says, “It is amazing how quickly your savings can be gone. It’s hard enough dealing with your emotions during a medical crisis, but then to find out financially that everything we’ve worked for all of our lives is gone? My husband and I have always had a job. We did not know what to do when we had no health insurance.”
While Cooper has, thus far, won his health battle, the Hornungs continue to wade through piles of medical bills, sadly losing their financial battle. Even though they are still in the throes of this monetary disaster, having Cooper returned to his journey of life gives them the much needed fuel to continue fighting for their right to the American dream.
On the home front, Cooper is back goofing around with his brother and showing the world his incredible smile. Until he is about ten years old, however, the Hornungs will have to do routine check-ups with Cooper to make sure no cancer has invaded his system, a typical screening procedure for BWS children. In most BWS cases, the risk factors decrease as the child grows.
Certainly both Cooper and his family have had a myriad of obstacles thrown their way, but they’ve championed them with grace, persistence, love and tremendous human spirit. They are quick to recognize their great fortune of having the best medical care for Cooper.
Cooper continues to have family, friends and a professional medical army standing by his side. With medical dignitaries and humanitarians like Worrel, Immken, Marsh, Wells and Rana, Cooper also got an extended family that looks forward to hearing about his next journey.