Family hopes for Chaeden Manuel laughs and chases his sisters around the house with the vigor of a normal 2-year-old. But the heart that beats inside this active little boy is far from normal.
healing for 2-year
-old son’s heart
Rare condition forces
numerous surgeriesBy Leila Fujimori
lfujimori@starbulletin.comThe Kaneohe youngster was born with a hole in his heart, a condition associated with an uncommon genetic condition known as Di-George Syndrome. Chaeden had three operations, including two open-heart surgeries, and is scheduled for another operation next month.
"It hurts me to see him," said his father, Freddie Manuel. "He doesn't even know what he's going to go through. And there's nothing we can do about it. Hopefully, everything is going to go OK."
The July 11 surgery is to correct a narrowing of the aortic valve to allow blood to flow.
Almost two years since his last heart procedure, Chaeden's parents were caught by surprise when his pediatric cardiologist discovered the latest problem about two weeks ago. Dr. Lance Shirai immediately scheduled surgery for June 12 with specialists at Children's Hospital in San Diego.
That date was too soon for the Manuels to scrape up air fare and hotel money, and the surgery was pushed back to July 11, as far back as safely possible.
Doctors say no surgeon in Hawaii has the expertise to perform the complicated, risky procedure. Surgeons may need to cut out membrane that may be obstructing blood flow.
"The risk of that is, they try to cut out as much of the tissue as possible and, in the process, may injure the electrical system of the heart," Shirai said. "He may have to have a pacemaker put in."
The operation has all the usual risks of heart bypass surgery as well, he said.
Freddie Manuel, a milk truck driver, said the cost of the surgery should be covered by health insurance through his employer. His wife, Napua, works nights full time for an answering service, which allows her to care for their daughters and Chaeden and take him to his frequent doctor visits during the day. But their paychecks cover rent and necessities and leave little for the trip.
Though they have been through this before, the Manuels have been wrought with worry over their youngest child.
The Manuels first learned something was wrong in November 1998 when Chaeden, just 2 1/2 weeks old, began wheezing. Doctors discovered a hole in his heart, and both large vessels of the heart, the pulmonary artery and aorta, were coming out of the left ventricle (pumping chamber), when normally there is one on each side, Shirai said.
The couple flew to San Diego with their son, where he underwent surgery to patch the hole and correct the other problem. After the four-hour surgery, the Manuels saw their infant son with wires and tubes attached to his tiny body.
"It was hard seeing my son like that," Freddie Manuel said. "Seven days later, he had to go back."
Surgeons cut extra muscle tissue blocking the pulmonary artery, which had thickened.
During the surgery, doctors noticed an unusually small thymus gland, a sign of Di-George Syndrome. Chaeden later was diagnosed with the condition.
In August 1999, Chaeden was flown back to San Diego to have a balloon angioplasty to stretch open the pulmonary valve of the heart. Afterward, the Manuels did not suspect any major problems.
"If you feel him now, you can feel the vibration of the blood flowing," said Napua Manuel. "When he plays too hard, his heart beats fast. And he takes long naps."
Di-George Syndrome, also known as Vela-Cardio-Facial Syndrome, can cause problems including a lowered ability to fight infection, hearing loss and hindered speech development. Psychiatric problems, behavioral and learning difficulties also are associated with the condition, Shirai said.
Children with Di-George often have eyes that look like they are squinting, dark rings under their eyes, small and low-set ears, cleft palate and other defects down the center of the body.
Chaeden suffers from chronic ear infections and has undergone numerous surgeries to insert tubes in both ears, causing some hearing loss. At 2 years old, he is shorter than average and still cannot speak. Shirai said the speech problem may be due to his hearing loss but may also be a learning problem associated with the syndrome.
Those wishing to assist the Manuels may make donations at any branch of First Hawaiian Bank in the name of Chaeden O. Manuel