Kaylee Wallace suffers from a rare brain abnormality that can stop her heart in her sleep.Kaylee Wallace suffers from a rare brain abnormality that can stop her heart in her sleep. (Family photo)

The planned heart transplant from a dying Ontario two-month-old to a Prince Edward Island baby did not go ahead Tuesday night.

Joubert syndrome

Joubert syndrome is a rare, genetic disorder that affects the cerebellar vermis area of the brain, which controls balance and co-ordination.

Common signs and symptoms include:

  • Ataxia or lack of muscle control.
  • Abnormal breathing patterns.
  • Sleep apnea.
  • Decreased muscle tone.
  • Jerky eye movements.
  • Developmental delays in gross motor, fine motor and speech.
  • Malformations such as extra fingers and toes, cleft lip, or palate and tongue abnormalities.
  • Kidney and liver abnormalities.
  • Seizures.

Common treatments include infant stimulation, physical therapy, occupational therapy and speech therapy for developmental delays. Infants with abnormal breathing need to be monitored for apnea.

The Joubert Syndrome Foundation knows of about 40 cases in Canada, said spokesperson Karen Tompkins of Essex, Ont.

Baby Kaylee Wallace was to be taken into an operating room in Toronto's Hospital for Sick Children, removed from a respirator that keeps her alive, fall asleep and die.

Once off her breathing machine, Kaylee would have had to die within approximately 30 minutes to ensure her heart was in good enough condition to donate.

But Kaylee did not fall asleep. The medical team waited for about an hour, but she continued to breathe on her own and doctors called off the transplant surgery. Kaylee will now be reassessed.

Kaylee suffers from Joubert syndrome, an extremely rare brain abnormality that can stop her breathing during sleep.

"It's like walking to your end — you know there's a deadline to when your child is going to pass away and then getting yourself prepared for it," her father, Jason Wallace, said Tuesday evening.

Lily O'Connor is in intensive care after a planned heart transplant was called off.Lily O'Connor is in intensive care after a planned heart transplant was called off. (Family photo)

Kaylee and Lily O'Connor, the planned recipient of the heart, are now back in the intensive care unit.

Born March 9, Lily has truncus arteriosus, a rare form of congenital heart disease that leaves her blood short of oxygen.

Doctors have told Lily's mother and father, Melanie Bernard and Kevin O'Connor, their daughter can only survive for a few more weeks without a transplant.

Earlier, Bernard said the decision to go ahead with what could be a risky transplant was not an easy decision.

In an interview with CBC News on Tuesday afternoon, Bernard struggled to find words to describe her feelings about what Kaylee's parents have gone through to make their daughter's heart available for transplant.

" 'Thank you' isn't even the right word. What do you say to people who donate their organs or their children's organs?" she said.

On Saturday, O'Connor and Bernard, who live in Stratford, P.E.I., just east of Charlottetown, thought they had found the miracle they were looking for when they were approached by Wallace and Crystal Vitelli, Kaylee's mother, about a possible transplant.

The Bradford, Ont., couple knew their daughter was dying and they had exhausted all medical resources, Wallace said. They wanted her heart to go to Lily.

"This will give us the joy of life, if she gives the joy of life to someone else," Wallace said.

On Monday, Kaylee's parents were allowed to sign consent papers for the transplant, but the medical opinion was not optimistic.

Physicians had said that when Kaylee was taken off life support, it would likely take her too long to die to make her heart useful for transplant.