PART I – The Technical Perspective

My son, Jack, is thirteen years old and he has an unknown subtype of dystroglycanopathy congenital muscular dystrophy.  Jack suffers from severe muscle weakness and respiratory insufficiency and has been ventilator dependent since he was four months old.

Jack’s History

Jack was born via emergency c-section at thirty-three weeks.  Despite being premature, Jack did not require any ventilatory support after he was born.  Jack spent six weeks in the Special Care Nursery and was discharged, requiring only a small amount of supplemental oxygen.  Within a month of his discharge, Jack was re-admitted to the pediatric intensive care unit after suffering an apneic event.  It was discovered that he had a vascular ring that was compressing his airway and esophagus.  He had surgery to divide the vascular ring and was discharged after two weeks, still requiring only supplemental oxygen.  Shortly after his discharge, Jack was again admitted to the pediatric intensive care unit in respiratory distress.  After several months in the hospital and multiple unsuccessful attempts at extubation, Jack received a tracheostomy and was discharged from the hospital on a home ventilator.  During the first few years of his life, attempts were made to wean Jack from the ventilator for at least part of the day. Jack’s CO2 levels became dangerously high when he was off the vent, however, and he was never able to successful wean from the ventilator.  Today, Jack requires full ventilatory support 24/7.  Jack also has a weak and uncoordinated swallow and, therefore, receives all his nutrition via a g-tube.

The Equipment

Jack’s first home ventilator was the T-Bird Legacy.  This “portable” home vent was about the size of a small air conditioning unit.  Needless to say, it made getting out and about with Jack very difficult.  When Jack was 3 years old, he was switched to the first truly portable home ventilator – the Pulmonetics LTV.  The LTV is slightly larger than a laptop computer and is probably the most widely used home ventilator today.  Our insurance company allows us to have two ventilators so that we always have a backup in the event that there is a malfunction with one of the ventilators.  We use one ventilator as our “home vent” and have it attached to a stand and hooked up to a humidifier. This is Jack’s primary ventilator.  The other ventilator is the “traveling vent” and is carried on Jack’s wheelchair in a special case that was designed by the wheelchair company.  The traveling ventilator isn’t attached to a humidifier.  Jack has been on the LTV for the past ten years and it has worked well for him, with only a few instances where it has malfunctioned.  In the very near future, however, Jack will be switching to the newest home ventilator on the market – the Trilogy by Philips Respironics.  The Trilogy incorporates newer technology and is said to be quieter and more sensitive to the patient’s own breathing.  The Trilogy is small and compact, and has a much longer battery life than the LTV.

After the ventilator, the second most important piece of equipment that Jack requires is a suction machine.  Jack has very weak respiratory muscles and has virtually no ability to cough up his own secretions.    As a result, Jack’s airway must be suctioned frequently.  Jack also uses the Vest system to loosen up the secretions in his lungs and a CoughAssist mechanical insufflator-exsufflator machine to “cough up” his secretions so that they can be suctioned.  Included in our repertoire of equipment is also a nebulizer machine for Jack’s daily breathing treatments.  Because he has an artificial airway and is dependent on a machine to keep him alive, Jack is monitored almost continuously using a pulse-oximeter, which measures his oxygen saturations and heart rate.

Associated with all of Jack’s durable medical equipment is a plethora of ancillary supplies, most of which are disposable and must be replaced on a daily, weekly or monthly basis – such as ventilator circuits, filters, extension tubing, adapters, sterile water, water chambers, suction catheters, pulse-ox probes, and nebulizer kits – to name a few.

Respiratory and Airway Management

Jack follows a strict daily routine to maintain his respiratory health.  Every morning, he uses the Vest for two 10-minutes sessions.  During the Vest sessions, Jack is given a Pulmicort neb to manage lung and airway inflammation.  If necessary, he also receives a Xopenex and/or Atrovent neb.  The Vest treatment is followed up with the CoughAssist to bring up all the secretions. Throughout the day, Jack requires continuous suctioning to keep his airway clear. Jack is also unable to swallow and requires a fair amount of oral suctioning throughout the day as well.  When Jack is sick, the biggest challenge is to keep his lungs open and moving air, which can require multiple Vest, CoughAssist and breathing treatments a day and, seemingly, non-stop suctioning. The Vest and CoughAssist have been instrumental in keeping Jack healthy and out of the hospital.

Jack is fairly stable from a respiratory standpoint.  As he gets older and bigger, the tidal volume, rate and pressure support numbers on his ventilator need to be adjusted to accommodate his size.  The best way to measure what changes should be made is through an overnight sleep study.  Jack’s last sleep study was done four years ago and it was determined at that time that his vent settings were appropriate and that he was being well ventilated.

Jack’s trach is changed out approximately every three weeks and replaced with a new trach tube. Because the tracheostomy tube is a foreign object than can cause damage to Jack’s airway, Jack’s otolaryngologist performs a routine bronchoscopy under general anesthesia every two years to make sure there are no areas of granulation tissue or trauma to Jack’s trachea caused by the trach tube or from the continuous suctioning that Jack requires.  Monitoring the health of Jack’s airway is particularly important for him because Jack was born with a right aortic arch and his aorta pushes up against and touches his trachea.  During a routine bronch several years ago, an area of erosion in the trachea just below the tip of the trach tube was discovered.  This was considered life threatening because if the trach tube erodes through the trachea, it can puncture the aorta.  Jack’s otolaryngologist called in a cardio-thoracic surgeon to help deal with Jack’s unique situation. They ended up putting a pericardial patch between Jack’s trachea and aorta to serve as a barrier in the event Jack’s trach tube should ever erode through his trachea – which isn’t unheard of in people who are long time trach users.

Transporting and Traveling with a ventilator

Leaving the house with a ventilator dependent child requires planning and organization.  You can’t simply load your child and his wheelchair in the car and be on your way.  You must have the following things with you at all times: the suction machine, ambu bag, extra suction catheters and an extra trach tube. There have been times when I’ve had to perform emergency trach changes because the trach is plugged with mucous or use the ambu bag to “blow open” Jack’s lungs because he’s in distress. The ambu bag is a must when traveling with a ventilator dependent child because if the ventilator malfunctions or you can’t find a power source, you can always bag your child until you get home or get to a hospital.  We also carry a Delee Suction Catheter that we can use to manually suction Jack if there is ever a problem with the suction machine.  It’s imperative that you are never without a way to manually ventilate your child or suction his airway because, as we all know, machines break and a power source isn’t always available. 

Managing a child with an artificial airway and dependent on mechanical ventilation is challenging, exhausting and overwhelming at times.  However, with the appropriate equipment, a consistent respiratory management routine, competent home health nurses, and a supportive team of physicians, it is possible for your child to stay healthy, thrive, and enjoy many of the same life experiences as everyone else.