Daphné has been plugged into a ventilator since birth
Breathing is something we all take for granted. Our lungs just automatically inhale and exhale. But in very rare instances, children are born without this innate ability. Daphné is such a child. She was born with a very rare defect known as Congenital Central Alveolar Hypoventilation Syndrome, or Ondine's Disease.
Last fall, Dr. Hélène Flageole, a pediatric surgeon at the Montreal Children's Hospital of the McGill University Health Centre, implanted two phrenic nerve pacers in the two-year-old. A phrenic nerve pacer is similar to a heart pacemaker. It causes the diaphragm to contract and relax, which then in turn causes the lungs to inflate and deflate. Christopher Reeve, Superman, uses a phrenic nerve pacer. Daphné carries her pacers around in a knapsack.
The Montreal Children's Hospital is the only pediatric hospital in Canada with a Phrenic Nerve Pacer Program. The last time the Children's performed this surgery was four years ago. We currently monitor the condition of several children and teens with phrenic nerve pacers.
Daphné returned to the Children's from her home in Chicoutimi this week so Dr. Michael Davis, the medical director of Pediatric Respiratory Therapy Services at the Children's, could make adjustments to her pacer to ensure she is getting the right amount of oxygen. As she grows older, she will need similar periodic adjustments.
Thanks to the phrenic nerve pacers, Daphné can run around like other children and will be able to lead a normal life.
Daphné's mum is certainly glad the surgery has taken place. Her rambunctious daughter was fed up with being tied to her ventilator. Daphné would yank out her tracheotomy tube and go scurrying off. The ventilator would beep and mum would frantically search the house for her daughter to plug her back into the machine.
Ondine's Disease is named after Ondine (Undine), a female water sprite who loved a knight but was condemned to stay awake in order to breathe; she wasn't allowed to sleep.