Nielyn's Story

New Beginnings Start with a Fresh Heart for Oakland County Girl

When 10-year-old Nielyn , spent time in the doctor’s office over the years to try to discover the source of her occasional breathing problems as well as vomiting and stomach issues, her mother Sandy became frustrated that a definitive diagnosis was not made as to what was causing her daughter’s health issues.

Nielyn had several tests performed when she was younger and doctors could not pinpoint any specific problems other than her esophagus was inflamed. A change in her diet seemed to help.

On New Year’s Day of 2016, Nielyn was at her grandfather’s house and complained that she was having stomach problems.

Sandy decided to take her daughter to the emergency room at the Children’s Hospital of Michigan DMC to be evaluated.

A number of tests were performed leading to startling news.

“The doctor wanted to get echocardiography images of Nielyn’s heart before she was discharged. I didn’t think anything of it since a previous chest X-ray several years before did not show any problems,” Sandy says.

Sandy thought she would be leaving the hospital soon, but the images took a while and it made Sandy nervous that something was going on. In fact, the images showed that Nielyn had an enlarged heart.

It blew my mind. I kept thinking of a friend’s mom who died of an enlarged heart and could not believe what was happening with my daughter. I started crying and then my daughter started crying so I knew that I needed to stay calm to be there for her,” she says.

Daisuke Kobayashi, M.D., pediatric interventional cardiologist on staff at the Children’s Hospital of Michigan, had the difficult task of explaining to Sandy that her daughter had a critical heart condition that would likely require a heart transplant. He drew a picture of a regular heart and an enlarged heart to show Sandy how an enlarged heart can cause the heart to pump harder than usual and damage the heart muscle. He also explained that Nielyn's present symptoms of vomiting and stomach ache were signs of heart failure because the stomach did not receive enough blood flow.

“It was not easy to share the news with Sandy because Nielyn was such a beautiful, healthy girl without any past medical problems,” says Dr. Kobayashi, who immediately admitted her to the hospital for further evaluation and treatment.

Fortunately Nielyn’s condition did not progress quickly and became stable.

Jennifer Blake, M.D., pediatric cardiologist on staff at the Children’s Hospital of Michigan, who managed Nielyn’s care, says tests determined that Nielyn had restrictive cardiomyopathy (RCM), a rare life-threatening disorder where the heart doesn’t relax properly and causes blood to "back up" into the atria (the upper chambers of the heart) over time, which leads to the atria becoming very dilated. It is the rarest type of cardiomyopathy and is seen in less than one in a million children. Medical treatment options are limited and ineffective in improving long-term outcome, especially in patients like Nielyn who have already developed pulmonary hypertension (high pressure in the lung arteries).

“The long-term outcome for a child with restrictive cardiomyopathy depends on what symptoms the child presents with at the time of diagnosis. These children are at risk for blood clots (around 20 percent of children), abnormal heart rhythms, and fluid retention (abnormal swelling of the legs, feet, belly, face). The abnormal heart rhythms are also concerning because they can cause sudden cardiac death. Overall, the average two year survival for children with RCM varies between 45-70 percent,” says Dr. Blake.

“Heart transplant is presently the only known effective treatment for RCM particularly when a child with RCM has already developed pulmonary hypertension. A heart transplant also allows a child with RCM to get back to a normal life including activities like competitive sports,” she adds.

After the diagnosis in January, Nielyn was in and out of the hospital due to her heart. A mass was also found in the area just behind the heart. Fortunately, this mass was not malignant and it gradually became smaller over time.

Nielyn was on the heart transplant list for a few weeks and on March 11, 2016, a family donated a loved one’s heart to make the transplant possible.

Henry L. Walters III, M.D., chief of Cardiovascular Surgery on staff at Children’s Hospital of Michigan, says at the time of the transplant, Nielyn’s diseased heart was very enlarged due to its extreme thickness, but the donor heart was perfect and the procedure went very smoothly, without any technical problems. “Nielyn recovered extraordinarily quickly and, within and very short period of time, she regained her normal appetite and energy level. She was so energetic that we had to gently remind her that she needed to get plenty of sleep rather than stay up too late texting her friends,” he says.

“Words cannot describe all of the emotions I had during this time. I was scared, hopeful and so appreciative that my daughter has a chance to live a full and productive life,” Sandy says.

Sandy and her daughter want to emphasize how thankful they are to the family who donated their loved one’s heart. They plan to write a letter together to the family with the hope that they will find comfort in knowing a child’s life was saved because of their selfless act.

“We are so thankful to the donor’s family and the entire staff at Children’s Hospital of Michigan. The doctors, nurses, social workers and transplant coordinators were amazing throughout this entire process,” Sandy says.

Nielyn, who documented her journey on video, including when she left the hospital on Good Friday, is surprising many with her speedy recovery. She was back at home after just a couple of weeks and is being home-schooled for the next couple of months. She plans to return to school next Fall.

Sandy says Nielyn is happy to being back to her normal self.

“She is walking, dancing and playing with her phone non-stop. She also wears a necklace that says "faith."

It sums up her incredible journey as she moves forward.