When 4-year-old Chantal Waldron arrived from Trinidad in 2002, she was suffering from a congenital defect that left the right half of her heart severely compromised. Tricuspid atresia is a congenital condition in which the tricuspid valve between the two right chambers of the heart is absent, and the right side of the heart is typically very small. The right side of the heart pumps blood to the lungs for oxygenation, so the implications are obvious. There is no communication between the right atrium and ventricle, and the American Heart Association notes that survival depends on there being communicating openings between the right and left ventricle (ventricle septal defect) and right and left atria (atria septal defect).
The congenital defect impairs the body’s ability to move the blood effectively to the lungs and to the rest of the body, and these children typically have a bluish, cyanotic cast to their skin. The condition occurs in 0.3% to 3.9% of all people with congenital heart disease, and is sometimes lethal at birth. Overall life expectancy without intervention is less than five years.
Jayne Cashman, RN, MSN, nursing coordinator for Pediatric Cardiac Surgery at Schneider Children’s Hospital, New Hyde Park, N.Y., acts as the liaison between the patient’s family and the cardiac surgeon. She prepares the child for surgery, including making sure all the preoperative testing is done and that the results are within acceptable parameters for the surgery. When the patient arrives, he or she is seen preoperatively and usually has a cardiac catheterization as well as an echocardiogram. Cashman helped Chantal’s mother cope when she arrived in 2002, and was a familiar face when they returned for the second surgical procedure. Cashman sees patients like Chantal for preoperative teaching and is involved with the children and their families whether they are from New York or thousands of miles away. There is also a preoperative visit with the surgeon and with an overseeing pediatric cardiologist.
After the surgery, Cashman is responsible for discharge planning, making sure patient and family know what the medication regimen is, how to care for the incision, and what to do about follow-up care.
Lisa Felice, RN, MSN, CPNP, clinical nurse specialist in Pediatric Cardiology, works with international patients, coordinating the paperwork and clinical records that are needed to get the medical visa to come to the United States for treatment. Some patients come once; others, like Chantal, return after a period of time at home.
“Once someone is our patient [he or she] remains our patient,” says Felice. The team reviewed Chantal’s most recent echocardiograms and tests, and her most recent medical records, she says. “They agreed that she was ready for the second stage of the procedure.”
When patients come from other countries for complicated medical procedures not available at home, it is never easy. Felice writes a letter requesting a medical visa that eventually goes to the American Embassy in the country of residence. Once approval is received, she helps with arranging surgical dates and assists the family in connecting with resources for transportation and lodging during the stay.
There are several organizations that help folks from other countries come to the United States for medical treatment, says Felice. “The Gift of Life” is one of the largest and is sponsored by Rotary International. Ronald McDonald House is frequently the host, providing accommodations for the family while the child is in the hospital.
All of this can be overwhelming for a family that has been stressed by worry, travel, changes in weather and time zones, and is struggling to understand through language differences and translators.
“A smile and reassurance go a long way,” says Felice. She notes that Chantal and her mother were shy and reserved, but they soon warmed to the caring of the staff. “Chantal’s face lit up with a big warm smile before long,” says Felice.
The Ultimate Bypass
The treatment for this condition involves rerouting the blood supply from the body directly to the lungs, bypassing the heart completely. This is called the Fontan procedure, and it is usually done in two steps. The first part of the procedure, the bi-directional superior cavopulmonary anastomosis (bi-directional Glenn), was performed when Chantal was 4 years old. In this procedure, blood from the upper body is routed to the lungs via the anastomosis of the superior vena cava to the pulmonary artery. The second stage of the process is done at a later time and involves rerouting the blood from the lower body to the lungs. Vincent Parnell, MD, board certified in cardiothoracic surgery and surgeon-in-chief at Schneider Children’s Hospital, performed the complex procedure.
“With this type of congenital defect we are not able to do a repair that creates a normal structure,” says Parnell. “We have to reroute the blood supply directly to the lungs.”
After the initial surgery in 2002, Chantal returned to her native Trinidad and was followed by a local team of pediatric cardiologists. She returned this year to have the second stage of the procedure done. This procedure, the extracardiac Fontan procedure, connects the blood supply from the lower body to the pulmonary artery. The surgeon utilizes a Gortex graft to connect the inferior vena cava to the pulmonary artery, near where the previous anastomosis was created.
“After this surgery, she will need echocardiograms every six months or so, and she may have some limitations at the extremes of exercise,” says Parnell. “Otherwise, she can expect to have a normal, active life.”
This surgery was first performed in the late 1980s, and those patients are now in their 30s. In general, says Parnell, caregivers should be aware that these patients are prone to clots and to cardiac dysrhythmia that can happen anytime.
When we read the stories about those who have come to our shores from far away for surgery or treatment, it is easy to forget that there are many steps to making that happen. The team at Schneider Children’s Hospital has contacts all over the world who refer children for the treatment. Through their efforts, they are able to give the gift of life.