Baby Diagnosed With Rare Vein Disorder
Currently tipping the scales at 15 pounds, 14 ounces, Chas Howells is a bundle of happiness. Even though noticeably small in size than other children his age, the son of Shawna Seville and Wes Howells is mentally as sharp as a tack. Pulling himself up and cruising around furniture, Chas appears by all aspects to be a normal, healthy boy to those around him except for a scar on his chest and a tube inserted in his nose.
However, Chas has endured more suffering than what most adults will likely incur over the span of a lifetime. The ongoing doctor’s appointments, testing and multiple surgeries dating back to only eight days following his birth at Chambersburg Hospital have resulted in a diagnosis of a rare and incurable disease for the soon-to-be 14-month-old.
Born July 15, 2010, Chas was the healthy and happy baby Seville and Howells, both of Mc- Connellsburg, had been hoping for. Unfortunately at the time of his discharge several days later, unexplained bleeding and a blue hue to the baby’s lips led to a check-up by the on-call pediatrician.
Placed on oxygen in the hospital’s nursery, Seville and Howells not only learned they would not be permitted to take Chas home, but the newest addition to the family was being flown to Hershey’s neonatal unit for further testing and observation.
According to Howells, at the age of 8 days old, Chas underwent open-heart surgery in Hershey to repair a condition known as total anomalous pulmonary venous return (TAPVR). The rare heart defect prohibits the child’s pulmonary veins from connecting to the heart’s left atrium. Instead, the veins are redirected to the right atrium by way of an abnormal or anomalous connection.
Having been successfully repaired, Howells said it was about five months later his first-born was exhibiting weight loss as well as projectile vomiting. Finding themselves back at Hershey over Christmas and New Year’s, Seville and Howells received shocking news and an unexpected diagnosis that their son suffered from pulmonary vein stenosis (PVS), a rare and serious condition where there is a narrowing of the blood vessels carrying blood from the lungs to the heart.
The disorder can affect only a single vein, but more often occurs in multiple veins concurrent. In Chas’s case, all four veins are affected where they attach to the heart.
Howells stated physicians were able to intervene and repair the situation, but, as PVS is a “progressive disease,” doctors were very forthright with information regarding the likelihood of recurring obstructions in the near future.
In addition, doctors also informed Seville and Howells, Chas suffered from congenital lobar emphysema. Having come down with pneumonia in June 2011, Chas was admitted for surgery to deal with the affects of the emphysema and the right middle lobe of his right lung was removed. If need be, Chas is totally capable of surviving with only one lung as well as with what his father describes as a very healthy heart.
The surgery also confirmed the family’s biggest fear that the PVS returned and would need further remedy. A recommendation was then made to Children’s Hospital Boston that Chas’s case be taken under review and consideration. Following a case review, Chas was taken to Boston on July 30 where officials, including pediatric cardiologist Dr. Kathy Jenkins, approved his participation in an experimental study with 18 other infant patients.
“His biggest problem is he needs a healthy set of veins. The lungs and his heart aren’t the real issue,” Howells told the “News.” “We don’t have the luxury of time on our side. We have three options: do nothing, participate in the Gleevec study or wait for a double lung transplant.”
Passionate about his son, Howells said doing nothing to aid in Chas’s survival was not an option nor was awaiting a transplant, which could take anywhere from one week to one year under the first-come, first-served guidelines. Furthermore, in the event the transplant was successful, the likelihood of survival past five years is only 50 percent.
During the recent visit with the top-of-the-line staff at Children’s Hospital Boston, Chas’s veins were bled out through a heart catheterization. Due to the PVS, the chance his veins will have to be “ballooned” again while the Gleevec drugs take affect is extremely likely.
The family plans to return to Boston in January or February with Chas to help determine if Gleevec, a targeted chemotherapy drug, is working. Meanwhile, the two Gleevec pills administered daily through a feeding tube with water or apple juice are in addition to an array of other recommended medications. The costly pills must be taken for a year but are fortunately covered through the study.
Other costs, however, continue to mount for the family from housing, food and gasoline expenses for their travels to Hershey and Boston to bills associated with pediatrics, pulmonologists and cardiologists. To help with these expenses, several benefits have been scheduled for September and October.
Only home two days from the Boston trip, Howells received an unexpected telephone call from longtime friend Greg “Bucky” Zeger, outlining plans devised by himself and friend Josh Hall for a benefit.
“I wasn’t surprised that they would do something like that, but what surprised me was that it would be done for Chas and the family ... . You always hear about benefits in the community, but you just never see yourself as the person behind one,” said Howells.
“Now that word has gotten out about the benefit, it’s amazing how the community has rallied together,” he added.
The event set for Saturday, September 17, at the McConnellsburg American Legion picnic grounds will include a hog roast with alcoholic and nonalcoholic beverages, music, jars and raffles between 4 p.m. and 11 p.m. Tickets are $15 each and are available in advance by contacting Zeger at 717-491-9730 or Hall at 717-658- 3528.
Those wishing to make a monetary donation but unable to attend the hog roast are urged to send donations to Wes Howells at Palmer Construction, 188 Success Drive, McConnellsburg, PA 17233.
Plans for a follow-up benefit are under way, according to event organizer Autumn Seville, of the McConnellsburg-based hair salon Curl Up & Dye. The benefit is currently scheduled for Saturday, October 22, between 6 p.m. and 11 p. m. at the McConnellsburg American Legion. Dinner, music and several auctions are among the evening’s tentative itinerary. More information will be released as it becomes available.