Sisters usually swap toys, clothes and secrets.
But Madison and Sidney Rippy have also shared something quite rare – the need for a new heart.
Both Madi, 6, and Sidney, 3, have been diagnosed with dilated cardiomyopathy – a disease that affects the size of the heart and how it pumps. They have also both received heart transplants at the Mayo Hospital.
For parents Linsey and Noel Rippy of Blaine, the last four years have been anything but what you might expect raising a couple of little girls.
Madi first got sick when she was 2 1/2 years old, while Linsey was pregnant with Sidney in October 2008.
“One day she started acting really weird, her eyes were funny and she was slumped to one side so we called an ambulance,” said Linsey.
After being transferred quickly from Mercy Hospital to Children’s Hospital, doctors performed an MRI and discovered the little girl had suffered multiple strokes.
“When they did an X-ray they found she had this enormous heart,” said Linsey.
An enlarged heart that isn’t pumping properly can throw deadly blood clots.
“In essence the strokes saved her life,” said Linsey.
Madi was started on medication and doctors told Noel and Linsey a third of children diagnosed with dilated cardiomyopathy get better, a third get worse and a third stay the same.
In mid to late July 2009 Madi was stable. Noel and Linsey had gone out to a movie when they got a phone call from her dad and stepmother who were baby-sitting.
“They said ‘we called an ambulance – we think Madi is having a stroke’,” Linsey said.
One of the drugs had gone toxic in her system and she required a temporary pacemaker.
“In early July of 2009 we had started a transplant workup at Mayo (Clinic) just in case, because dilated cardiomyopathy is the number one cause for children to need a heart transplant,” Linsey said.
After complications following open heart surgery to install a permanent pacemaker, Madi was transferred to the Mayo Clinic with the hope of getting her off the intravenous heart drugs.
At that point, she was placed on the transplant list.
“Eighteen hours later she got a heart,” said Linsey.
Despite her easily matched blood type, because of the specific requirements to do a transplant in a child, doctors had told the family to expect a six month wait at least.
Linsey says Madi has done well physically with the new heart, but she is delayed as a result of her strokes and restricted blood flow. She has cognitive disabilities and physical limitations. While most kids heading of to kindergarten can tie their shoes or write their name, Madi is not able.
She attends special education classes to help her try to catch up to other kids her age.
“That’s the hope,” said Linsey. “She’s made some really good gains.”
After Madi was diagnosed, the couple underwent genetic testing but did not show positive results for heredity as a cause for the disease.
“So we were never ever suspecting this to happen to Sidney but they continued to do screening for her,” said Linsey. there were regular EKGs until this past October, when the dilated cardiomyopathy showed up.
Sidney had been managing well after the diagnosis until March when she was tired and not feeling well.
They said her ejection fraction – the measure of how efficient your heart is pumping – had gone down a little bit.
Then she got sick for several days with what seemed to be a stomach virus and constipation, which had to be managed carefully so her electrolytes could be kept in check.
After days of throwing up, Noel took Sidney into Children’s Hospital while Linsey stayed home with Madi.
“Noel was walking into the emergency room with Sidney when she seized,” said Linsey.
While being intubated she went into cardiac arrest and required 28 minutes of CPR. Doctors started a hypothermia protocol, Linsey said, because studies in adults have shown if your body is cool post arrest it helps with any brain injuries.
She had been doing better, but when she required a second intubation Sidney arrested again and required another 18 minutes of CPR.
Sidney was put on ECMO, a bedside heart lung bypass machine. Surgeons created a hole in her heart between the two atria to relieve pressure.
In April she was sent down to the Mayo Clinic. While not quite so quickly as her big sister, Sidney had a new heart two days later on April 25.
She is now recovering at Mayo and is required to stay in Rochester three months post transplant. once she is well enough to leave the hospital they will transition to the Ronald McDonald House.
When Madi was first diagnosed, Linsey said she had no idea what cardiomyopathy was.
A Google search brought her to Children’s Cardiomyopathy Foundation (CCF), which quickly became a source of information and support.
For Linsey, the CCF was a place where she could learn more about her children’s diagnosis and connect with other families who were going through the same thing.
Started in 2002 by Lisa Yue, who had lost two young boys to cardiomyopathy, CCF has become a well established national non-profit.
Yue said the foundation has grown beyond her expectations.
“When we first started we had modest goals to raise money for research studies,” said Yue.
To date it has funded $1.7 million in research grants for what Yue said is an often overlooked disease. When kids drop dead on an athletic field from a heart problem, cardiomyopathy is often the cause, she said.
CCF now has 2,400 members worldwide, connecting families through this rare and complicated disease they have in common.
“We are good at treating the heart,” said Yue. “But how do you deal with chronic disease? We saw this big void there.”
So CCF stepped in to fill the gaps and create a network for education, support and advocacy.
Linsey and her family have worked hard to raise awareness and money for congenital heart defects.
“They are the most common heart defects and they don’t get a lot of press,” said Linsey. “One in 100 kids has a heart defect.”
A few years ago she was asked to be a parent ambassador for CCF, reaching out to families coping with a new diagnosis.
“People end up like me where you are literally thrown head first into this world,” said Linsey. “Thank God for some of my friends I have met because of this. I have a great group of heart mom friends. They are seriously the best group of people I never wanted to be friends with. You never wanted to be in this world.”
Reaching out for help
Last year Linsey contacted CCF to see if there were any opportunities for financial support.
“We were drowning,” she said. the Rippys were struggling to pay the mortgage and their bills.
Back when Madi first got sick Linsey quit her job at Curves to care for the girls. They have been relying on Noel’s income from remodeling and repairing manufactured homes, but he often has to work reduced hours as they juggle schedules to parent two girls with chronic illness.
But Linsey recently got some good news from CCF.
They had been chosen as the first to receive a family assistance grant from CCF as part of a new financial aid program.
“I cried,” Linsey said when she found out about the grant. “There’s not a lot of help out there for people like us.”
Fortunately, they are have health insurance through MinnesotaCare, but there are still premiums to pay and supplies not covered. While they have help from nearby family and friends, none are able to contribute financially, said Linsey.
“On paper Noel makes enough money to pay our bills but there’s no ‘check this box here’ if your kid has had a transplant,” said Linsey.
Families with children diagnosed with cardiomyopathy are coping with increased medical costs as well as loss of income and often expensive travel to see specialists or get a second opinion, said Yue.
After hearing stories of families financially struggling to provide medical care to their child, CCF established the family assistance program with donations from other CCF families.
“We are so glad we were able to help the Rippys, especially since Sidney suddenly needed a heart transplant,” said Yue. “We hope to continue to expand the program and help even more families in the future.”
The Rippys, along with their friends and family, had raised $500 themselves by selling CCF’s cure bands.
“It’s a rewarding complement to one of the many areas of support we offer,” said Yue.
While Sidney recovers at the Mayo, the Rippys know they still have an uphill battle ahead.
“The thing that people don’t realize about transplantation is that yes, it’s better than the alternative but she has a very high risk of cancer and a high of infection,” said Linsey.
Both girls will remain on medication for life to make sure their bodies don’t reject the hearts they have received.
The family is participating in a genetic study at the Mayo Clinic. Just because they didn’t test positive for a hereditary gene, they could carry a gene that has not yet been discovered, said Linsey.
They are also committed to raising awareness about the disease, advocating for more money to be put into pediatric research.
And Linsey reminds people of how important it is to be an organ donor.
“I try to make people aware you should sign up to be an organ donor and make sure your wishes are perfectly clear,” she said.
You can go to the website donatelife.net and sign up to be an organ donor.
“There’s a lot of stress,” said Linsey on being a mom in a situation she never could have expected.
“You definitely go through the ‘why me’ stuff and feel guilty and paranoid. but all things considered, I think it teaches you not to worry about the little stuff.”
To learn more about children’s cardiomyopathy or to make a donation and support families like the Rippys you can visit childrenscardiomyopathy.org.
Mandy Moran Froemming is