Pfeiffer Syndrome Part IV- Tracheostomy

Pfeiffer Syndrome is a genetic disorder that creates structural differences in one's face, usually resulting in obstructed upper airways for many kids. Caliyah's small nasal airway and small lower jaw are leaving little room for her tongue, causing blockage of her pharynx. All of this makes it difficult for her to breathe through her nose and mouth. Due to that, Caliayh was intubated at birth because she was struggling to sustain her breath. At five weeks old, she had a scope where they inserted a small camera in her trachea while removing her breathing tube. During that procedure, her heart rate went up, and she worked overtime to breathe. The doctors made it clear that Caliyah could not sustain her breath, that the blockage in her upper airway plus her abnormal trachea wouldn't allow her to breathe comfortably longer than a few minutes. We decided to move forward with a tracheostomy at six weeks of age, which has required a lifestyle shift. Having a child with a trach is complicated as the parents, and all caregivers must be fully trained on how to care for her critical airway. My husband and I went through two months of training and hands-on practice. We had to learn how to suction her through a catheter, clean her stoma, handle emergency resuscitation, see signs of infections, and more.

On top of that, we need registered home nurses to care for her; she cannot have your typical nanny or go to a standard daycare. She has to have someone who is certified in her critical airway care at all times. We also can not travel alone with her in case of an emergency. We must move in twos, and one has to be a trained caregiver. We are in the early stages, so I cannot speak to how we are dealing with it, but I know the training has been eyeopening on what parenthood will look like for us in the future. When people learn of her trach, they often asked me various questions, but the most common are the following.

Why does she have a trach?

This is the setup for Caliyah’s trach cares. We must clean around her stoma daily to make sure she does not get an infection or irritation on her skin. This is a two person job. One holds the trach and the other cleans.

Caliyah's obstructed upper airway is the critical reason she needed a tracheostomy. The trach will help her breathe and will bypass her nose and mouth. Her breathing will go from the opening in her trachea called a stoma, to her lungs and repeat.

When someone needs a trach, it is usually due to one of many significant reasons. Still, a few are, having an upper airway obstruction, lung diseases that create weak lungs, neurological where their brain does not tell them to take breaths, and more. You will see kids with trachs due to their specific medical issues, and even adults have them, some due to an injury.

For Caliyah, she does not have any apena so her brain triggers her breath but she can't do it without straining herself and putting unnecessary stress on her body. We want her to breathe comfortably so she can use her energy to grow and do everyday activities other babies do. We don't want her to be exhausted from trying to walk or play. Overexerting herself can lead to a failure to thrive if not addressed early on. We also want her brain to receive the right amount of oxygen to prevent damage and long-term developmental delays. Since Caliyah already has brain pressure concerns due to hydrocephalus and fused sutures, we don't want to add another obstacle to her. Caliyah having a trach was the best decision for long term growth. 

Caliyah also uses a ventilator which is a machine that provides mechanical ventilation by moving breathable air into and out of her lungs. For Caliyah, the ventilator keeps a certain amount of pressure in her lungs so her narrow airways don’t collapse. Caliyah doesn’t use the vent to trigger her breathes but more to help her have bigger breathes. As her lungs get stronger and she gets bigger her airways will also grow. She is currently taking breaks off the vent and our goal is to get her completely off the vent in due time.

So now we know why she has the trach, the big question is for how long?

In my previous post, "Pfeiffer Syndrome Part II- Treatment," I talk about one of Caliyah's major surgeries that will come at the age of two. To learn more in detail- click here. The operation is called "monobloc" were they remove strips of bone and pull her forehead, midface, and upper jaw forward. They put a distractor halo on her head, and it slowly moves her face forward a cm at a time. This operation will widen her nasal airway and mouth by pulling her jaw forward. It will also give her eyes protection and by giving her larger eye orbits. We hope that at this point, she can remove the trach. Caliyah has strong lungs, and so her most significant obstacle is her narrow airways. All kids are different, and we can’t predict the future, but for many kids, this is the surgery that allows them to be decannulated (removal of the trach).  She will need a sleep study to monitor her breathing to see if she is ready for decannulation and then once the trach is removed the skin will heal over itself on its own.