Aavah Mangus update: Nothing short of a miracle

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Aavah Mangus was born with a very rare medical disorder called heterotaxy that sometimes causes certain organs in the body to form on the opposite side of where they would normally form and often causes heart problems. Patti Carpenter photo

Aavah Mangus was born with a very rare medical disorder called heterotaxy that sometimes causes certain organs in the body to form on the opposite side of where they would normally form and often causes heart problems.
Patti Carpenter photo

mber Mangus said she really never expected to witness a miracle in her lifetime, but since the recent birth of her daughter Aavah, she has seen one after another. Little Aavah was seven months old on June 8, which is a miracle in itself in light of the medical challenges she faced at birth.

Aavah was born with a medical disorder called heterotaxy, a condition so rare that it is only seen in one out of 1 million babies. The disorder causes certain organs in the infant’s body to form on the opposite side of the body to where they would normally form. For example, the heart forms on the right side instead of on the left side. According to the Heterotaxy Foundation, it is also known to affect the development of the heart and other major organs like the liver, lungs, intestines and spleen.

Much to everyone’s surprise little Aavah’s birth went very well and the disorder did not cause quite as many health problems as expected.

“She actually surprised everyone and was pretty much holding her own at birth,” explained Amber. “They had an operating room booked for her right away to install a pacemaker, but she acted so good they didn’t have to do that right away.”

After two days in the intensive care unit, the baby underwent surgery to install a pacemaker. She was only five days old at the time.

“When her heart rate dropped into the 40s they decided to take her in for the pacemaker,” said Amber.

Aavah’s doctor’s predicted that she would be an undersized baby but she was actually “chunky-sized,” said Amber.

She was already defying the odds with a birth weight of 8 pounds, 5 ounces and a heart rate of 50 beats per minute.

“She came out crying like a normal baby and they actually let me hold her because she was doing so well,” said Amber. “This is not something we were expecting.”

She was born at the University of Utah hospital but was moved to Children’s Primary Hospital because of the special care she would require due to her condition.

“This is probably the hardest thing we’ve ever gone through as a family,” said Amber. “We were told so many things (by doctors) before her birth that turned out not to be true. We have reports that show us that her stomach was on the wrong side and that her liver was out of alignment, indicating that she would have a lot of problems, but none of that turned out to be true.”

Amber said they asked the doctor how she could explain the discrepancy between the test results and the actual position of the organs and the doctor could not.  All of the tests showed the organs in the wrong place but what appeared to some, as somewhat of a miracle, none of this was evident at birth.

“We were pretty excited about all this,” said Amber.

The doctors found later that though she was holding her own, she would require a heart transplant. They anticipated this to be somewhat of a complicated surgery because her valves appeared to be in the wrong position.

At a certain point, they sent the family home to wait for the heart to fail and then it was determined that the option of switching the valves was no longer viable.

While the family was home during the Christmas holidays, the baby had to be taken to the Billings emergency room, where a heart specialist recommended they take  her back to Utah due to the complexities of her disorder. Amber, her husband Brandon and little Aavah hit the road but had to take the baby into another emergency room just two hours short of reaching the specialists at Children’s Primary Hospital.

The doctors in the emergency room determined that her anatomy and condition was so complicated that it would be better for the family to continue on to Children’s Primary for treatment, which they did.

“When we walked in they asked ‘are you the heart baby’ and we said ‘yes’ and they took us right in,” she said.

Though Brandon and Amber are both trained nurses, Amber said the trip to Primary Hospital was “super scary” requiring constant medical attention the entire way.

“They told us four times they didn’t know what to do,” said Amber. “They kept preparing us to say goodbye to her.”

The previous surgeon who implanted her pacemaker said he observed that her valve was in the wrong place and that she would not be a good candidate for a heart transplant. Further tests indicated this no longer to be true, which is still puzzling to both the doctors and the family.

As a result of the new information, little Aavah was back on the heart transplant list, where in 29 days a heart became available to her and she received her transplant.

Once again little Aavah proved them wrong and the surgery was much less complicated than expected, since the valve turned out to be in the correct position, in spite of what the previous surgeon observed.

“You know my husband and myself have tried to prepare for all of the emotions involved in this,” she said. “They told us because of her anatomy that things would be complicated and might turn out differently, that we might lose her.

“The week she got her heart, we were literally told to say goodbye to her. They told us she probably wasn’t going to make it. The heart became available literally within hours of that news.”

Amber said she suspects the heart came from a baby who died nearby, maybe even in the same hospital because it arrived in a very short period of time.

“On that day we were so relieved that a heart became available but at the same time realized that another family had a loss on that day and we felt very sad for them,” said Amber.

Though the family does not yet know who the donor was, they know it must have been a child similar in size. They have written a letter to the family and hope to meet them someday to thank them in person for their generous donation during what must have been a difficult period of loss for them.

Amber and the baby have been in Utah since January but were able to come home for a visit on Memorial Day weekend. They have returned to Utah, where they will live in an apartment while the baby continues to undergo medical treatments.

Prior to this visit, she and the baby have only been home for a short visit during the Christmas holiday season. She said due to the immune-suppressing qualities of the anti-rejection drugs required due to the heart transplant, the baby is only able to have limited exposure to the public.

A special fund has been set up for the family at Big Horn Federal Bank in Lovell and a Facebook page has been setup by Matty Moody. Several fundraisers have been set up to help the family with their on-going expenses.

For more information about how to help the family contact Moody at 899-0598 or Tina Toner at 272-4834 or Sara Walker at 272-3751.

By Patti Carpenter

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