WORCESTER — The cousins played happily, sharing Oreo cookies and toys in the adjacent room of the apartment on the third floor of a Sigourney Street triple decker.
Meanwhile, their parents talked about something they were sharing: the anxious wait for word that their respective children would be saved by an organ transplant.
“She’s a big support to me just like I am to her,” said Rebecca Williams, 28, of her sister Sheena Sierra, 31. “Even though [the children’s] conditions are very different, we know we have to stick together for these two.”
Carlos Gerald Cecilio Rolon, “Baby Carlos,” was born Sept. 20, 2013, with a congenital heart defect consisting of a small left ventricle and underdeveloped valves and other parts of his heart. He’s had multiple heart procedures and four heart surgeries, which have allowed him to leave the hospital and run and play just like any other preschooler. But he is now in heart failure and on the waiting list for a heart transplant.
His cousin, Rebecca Lucia Pagan-Williams, was born June 13, 2015, and within a few weeks was diagnosed with a rare liver disease called biliary atresia. “Baby Rebecca” is also home and just learning how to walk. But she has cirrhosis of the liver and is on the waiting list for a liver transplant.
Neither condition is believed to be caused by genetics.
Meanwhile, Ms. Williams and Ms. Sierra have strengthened their sisterly bond, as their children’s conditions have given them more in common than just blood.
Biliary atresia is a disease where either in utero or soon after birth, a child’s bile ducts are either not present or destroyed in the first few weeks of life. This leads to the baby being jaundiced, as bile cannot drain out of the liver into the intestine.
Biliary atresia occurs in about 1 out of 10,000 live births, and is the leading cause of liver transplantation in kids, according to Dr. Scott Elisofon, Medical Director of the Liver Transplant Program at Boston Children’s Hospital.
There are two ways to treat the disease. The first is through surgery – called the Kasai procedure – which has about a 50 percent success rate, according to Dr. Elisofon.
This surgery was done when Baby Rebecca was 6-weeks old and was unsuccessful. This leaves her with the second option: a liver transplant.
Most biliary atresia patients needing a liver transplant will need the transplant by two years of age. The transplant will cure the biliary atresia and transplant recipients can live many years on a transplanted liver, Dr. Elisofon said.
Baby Rebecca turns two in June. It is unclear how long her current liver will hold out.
“She’s jaundiced, having some issues with weight gain and growth, but her liver is still functioning enough to keep her moving along,” Dr. Elisofon said.
Nevertheless, he noted that – although livers can be donated from live donors – there are not enough donated livers to give to everybody in need.
But the recent bonds between the two sisters strengthened before the biliary atresia diagnosis.
The bonds first strengthened when Ms. Sierra learned that Baby Carlos would be born with a serious heart defect.
“I was real, real lost,” said Ms. Sierra. “But the first thing I thought of was my sister because she had already gone through it.”
Ms. Williams’ first child, Robert, died of a heart condition just eight days after being born in 2004. He didn’t have the same heart condition as Baby Carlos; but many of the emotions and experiences – dealing with doctor’s appointments, weighing surgeries, and more – were similar, Ms. Sierra said.
“Basically, I would say it’s fair to say she gave me a lot of emotional support,” Ms. Sierra said. “And she also helped with the whole part of acceptance.”
But the tables soon turned: an ultrasound revealed that Baby Rebecca also had a heart defect.
“I was just like, ‘not again, I can’t do this again,’” Ms. Williams said. So she called Ms. Sierra. “The same things I told her she was repeating back to me – ‘Don’t give up,’ and all that stuff,” Ms. Williams continued.
Thankfully, Baby Rebecca’s heart condition required monitoring rather than surgical intervention. But a few weeks after birth, Baby Rebecca was diagnosed with biliary atresia.
Ironically, the sisters found themselves in the hospital at the same time as their respective children underwent surgeries – Baby Rebecca with the Kasai procedure and Baby Carlos with his fourth heart surgery.
“It was the luck of the draw,” Ms. Sierra said.
Familiar waiting game
But now both families are waiting for a little luck that organ donors will be found.
Again, they have shared a lot of experiences.
Like Baby Carlos, Baby Rebecca has used a feeding tube, requires frequent hospital visits when her condition worsens – lately a 48-hour stay every four weeks for Baby Rebecca, Ms. Williams said – and receives palliative care.
Both children’s skin is also tinged: Baby Carlos’ skin can be tinged blue due to a lack of circulating oxygen, and Baby Rebecca’s skin is tinged yellow due to jaundice.
“She should be as light as me,” Ms. Williams said.
Both Ms. Williams and Ms. Sierra said they have experienced depression at times during their respective children’s illnesses. And both are trying to manage care of a sick and frequently hospitalized child while also caring for two other healthy children.
But most importantly, Baby Carlos and Baby Rebecca share one thing: they are both fighters, their parents agreed.
“Their sickest days, they still manage to smile,” Ms. Sierra said.
Ms. Sierra also understands the financial difficulty of having a child who is frequently hospitalized. And she understands that many people want to help.
So the two held a community fundraiser on March 18 to help both children, with a goal of raising a total of $25,000 so that Ms. Williams and Ms. Sierra can stay at their children’s bedside when a hospitalization occurs. Ms. Williams is a full-time mom, Ms. Sierra is a single mother and works nights.
But perhaps the most valuable way that Ms. Sierra has been able to support her sister and niece is by connecting them with a potential donor.
Ms. Williams shared a newspaper article about a Holden woman named Marybeth Sbrogna who was planning on donating a portion of her liver to a child with biliary atresia. Ms. Sierra reached out to Ms. Sbrogna and explained Ms. Williams’ situation, hoping that Ms. Sbrogna could give Ms. Williams hope that a donor would be found for Baby Rebecca.
Then four days before Ms. Sbrogna was scheduled to go into surgery for the transplant, the intended recipient received another donation.
“As soon as I found that out, I knew I was going to donate to Baby Rebecca,” Ms. Sbrogna said. “If I can prevent [Ms. Williams] from having to lose another child, I’ll do anything in my power to do that.”
Ms. Sbrogna said she has been approved as a donor. Baby Rebecca still has to go for further testing, according to Ms. Williams. Dr. Elisofon said the hospital is “in the process of determining whether Ms. Sbrogna’s liver will be a good fit for [Baby] Rebecca.”
Ms. Williams said she is trying not to get too excited, as one never knows what can happen. Another of her sisters was ready to be a donor, but it didn’t work out. Another time baby Rebecca had to refuse a transplant because she was sick with a cold, Ms. Williams said.
But Ms. Williams said she also had some good inspiration to stay positive: her daughter.
“She just keeps on pushing, she’s not giving up on me one bit,” Ms. Williams said.