On January 9th, Jacob Wiley was preparing to see his three-year-old daughter Adelaide in the hospital. It was a day that Jacob and his wife Ashley will never forget. Suddenly, there was a possibility that they would lose one or even both of their children.
Adelaide had been diagnosed with Kawasaki disease. Jacob was taking their baby Aislynn with him to switch places with his wife, Ashley so that she could come home and rest.
As the couple reported to Yahoo.com, when Jacob went to wake their baby, Aislynn, she was motionless. Jacob immediately rushed the baby to the Children’s Hospital in Cincinnati where Ashley anxiously awaited their arrival. Ashley scooped the baby up into her arms but completely lost her composure when she held her baby’s limp body. As they rushed to the ER they were both crying and screaming. Yelling for help. Their baby would not wake up.
Doctors began tests immediately. The parents were not sure their baby would survive. Adelaide’s condition was serious, but Aislynn’s illness seemed to be even more critical.
Diagnostic Testing for Aislynn
Ashley reported that there were two dozen medical staff surrounding Aislynn including cardiologists, neurologists, geneticists, and endocrinologists.
One of the doctors drilled a hole in the baby’s leg bone to allow IV infusions. The parents were told that the baby’s blood sugar dropped significantly which explains why they had been unable to wake her. But what caused the drastic drop in blood sugar?
A Very Rare Disease
Aislynn received a series of tests such as a spinal tap, brain imaging, and bloodwork. The physicians were trying to find out if she had an infection and make a diagnosis.
Ashley, her mother, followed the tests on the hospital’s popular My Chart app. Ashley said that by the third day of testing, My Chart registered 364 tests. That included many tests that were repeated “over and over” according to Ashley.
After consultations and analyses, the attending physicians agreed that Aislynn had a disease known as Tango 2. It is so rare that Aislynn is only the thirty-first person worldwide to receive this diagnosis. Tango 2 affects various organs including the heart and the brain. It is so severe that only half the number of children diagnosed with Tango 2 live past the age of nine.
Adelaide and Kawasaki
While much of the attention was centered on Aislynn, Adelaide was receiving treatment for Kawasaki disease. Then her parents received the good news that Adelaide was responding well to the treatment.
Both parents maintained a positive attitude for Adelaide’s benefit while they continued to comfort Aislynn. Ashley said that it was difficult to soothe Aislynn, as she was intubated and the electrodes that monitored her brain activity covered her head. However, a mother’s love finds a way, and Ashley managed to touch her, kiss her, and read to her.
Tango 2 had been discovered six years earlier. A hospital in Houston Texas had guidelines to manage the disorder. The doctors at the Cincinnati Hospital used these guidelines for Aislynn’s illness.
About Tango 2
The Tango 2 gene is critical to mitochondrial function. Mitochondria give power and energy to a cell. Both of Aislynn’s Tango 2 genes have a partial deletion because her father and mother were carriers of a gene that had a small deletion.
The doctors attribute the drastic drop in blood sugar to the mal-functioning mitochondria. In these instances, blood sugar levels are maintained with glucose. Children diagnosed with Tango 2 receive treatment for heart arrhythmias and possible hypothyroidism. Seizures are controlled with medication.
Aislynn left the hospital in March. Electroencephalogram electrodes and the ventilator were removed. She was able to breathe independently. However, she cannot crawl or talk and is fed through a feeding tube.
The doctors gave Jacob and Ashley hopeful news. They explained that even with damage to a child’s brain, some children have recovered. Ashley works with Aislynn at home giving her physical therapy and the child receives additional therapy at the hospital.
Currently, there are seventy known cases of Tango 2. The Wiley family is raising money for much-needed research through the Tango 2 Research Foundation.