Virtual Home Visit for a Child With Medical Complexity: Non-invasive ventilation in the
home by Dr. Kathleen Huth, Dr. Laura Amar-Dolan and the Virtual Home Visit Working Group.
Non-invasive ventilation includes CPAP and BiPAP.
CPAP is Continuous Positive Airway Pressure.
Indications for CPAP may include hypoxemia, upper airway obstruction, and obstructive
sleep apnea.
BiPAP is Biphasic Positive Airway Pressure.
BiPAP is sometimes used for hypoventilation, neuromuscular weakness, or to support in the
setting of increased work of breathing.
BiPAP delivers two levels of pressure for inhalation and exhalation and may have a backup
rate.
If you are seeing a child in clinic, or are paged about a child who uses non-invasive
ventilation at home, it is important to understand the indication for its use.
Knowing the indication for CPAP or BiPAP use can guide management, particularly if there
are equipment problems.
Consider-- how much support does the child need?
Review the settings with the caregivers.
And if you are doing a home visit, check the settings on the machine itself.
Does the child need support all of the time, including in the car or at school?
Can they sleep without it if there is a power outage or equipment issue?
Some children cannot tolerate being off of their CPAP or BiPAP for a short while due
to significant neuromuscular weakness, for example.
The device should have a power failure alarm and a charged backup battery in these cases.
And any equipment malfunction must be addressed emergently.
If you are on a home visit look at the settings on the device itself.
Patients may have a secondary or "sick" setting on their home non-invasive ventilation device
that can be enabled if there are difficulties with oxygenation or work of breathing.
Ask families about these settings at a home visit or if you are called about changes in
their child's respiratory status.
Jack has restrictive lung disease and hypoventilation.
He uses BiPAP at night.
So one of the biggest problems is Jack has a super small head.
With a profound brain damage, he's in this category called microcephaly, so he has a
really small head.
So masks don't fit his head.
Then we don't get a good seal.
It's terrible.
And nobody seems to have the right solution.
So he's on BiPAP.
And to try to get it in-- and he's also a mouth breather, so trying to close his chin
in there, and he just bubbles, and bubbles, and bubbles.
So to come out of it, it requires a lot of suctioning.
And then when he gets in the groove, he'll settle in.
But we struggle a lot.
And we've talked to his pulmonologist.
We've had specialists call us and we still haven't found a-- we've tried multiple mask
solutions and it's still not 100% where we're at.
And then, this is something we learned, so if you're dealing with your traditional supplier,
they don't carry mask samples.
They don't have them in inventory.
So they're just guessing.
Oh, I think this will work based on it.
So they don't get it tested on his head or see if it's going to work right.
We get closer, but nothing's been an ideal scenario.
Finally, after multiple years, we had an appointment this spring, we got a special therapist from
Children's and she had other tricks and led us in some better directions.
I still don't think we've hit it out of the park yet.
So it's a super challenge.
It definitely has its benefits because he was getting a little bit of sleep apnea and
he was developing a snore.
And so he was not getting a good night's sleep.
And that was making him tired on top of losing body heat overnight.
So it helps.
But you know what we also use it for?
We learned this trick.
When he's got allergies, or he needs to be cleaned out, he has extra congestion, boy,
does that clean him out and gets rid of it.
And you got to think outside-- we thought outside the box, too, because that's what
we use it-- like, we'll use it during the day because he doesn't need it during the
day.
It was more for the sleep apnea, and the snore, and getting a better night's sleep.
We do definitely struggle with it and him, in general, because he's just so secretional.
And it's like he's blowing bubbles.
But he's also a mouth breather.
So it has its benefits, but it has a lot of complications for us.
There are many potential challenges that families might face in using non-invasive ventilation
in the home.
Poor adherence is a common cause of treatment failure.
This may occur, particularly if there is an unpleasant initial experience with CPAP or
BiPAP, including if it is poor-fitting equipment.
A child may develop conditioned anxiety.
The child may benefit from desensitization measures to help them adjust to the sounds
and sensations to gradually integrate BiPAP into the child's routine.
Some measures for habituation may include trying to put it on after the child has fallen
asleep.
It is important to ask about whether the child is using their CPAP or BiPAP machine and for
how long.
If it is not used consistently, families may face issues with insurance coverage.
Another potential issue with BiPAP and CPAP in the home is poor mask fit or air leakage.
You should feel air coming out of the vents on the side, which is exhaled carbon dioxide.
But you shouldn't feel air escaping from where the mask is touching the skin.
This can be particularly challenging with children who have craniofacial anomalies.
This is also one of the issues that Jack's family has faced.
Another potential issue is skin breakdown.
Alternating between two different types of masks can be helpful to prevent irritation
with different touch points on the skin.
Families can also use a silicone foam dressing as a barrier.
Children may also get nasopharyngeal dryness, or dry mouth, especially if the air is too
cold or dry.
Some children may benefit from humidification.
Another concern is midface hypoplasia.
Pressure of the mask on the nasal bridge can potentially impact facial and sinus development.
Sleep disturbance may occur from airflow-related arousal.
Some children may benefit from sleep aids in this case.
Another challenge, particularly if feeds are being given via feeding tube overnight, may
be the concern for gastric distension and risk of aspiration.
This can occur with a full face mask as air can enter the stomach causing distension.
Caregivers may need to vent the tube or consider using a Farrell bag, which can allow the tube
to vent continuously.
It is strongly preferable to use nasal interfaces when possible to reduce air entering the stomach.
The child may benefit from a chin strap to keep the mouth closed.
Consider this especially if feeds are running overnight.
It's important to consider that if a child is having intercurrent illness, there may
be an increased risk of aspiration in these cases and difficulty with positive pressure
delivery.
If a child is unable to remove their own mask, he or she should wear an oximeter.
Hypoxemia can be an early indicator of obstruction or equipment malfunction when the child is
not directly observed.
The alarm on the BiPAP machine for low oxygen saturation may have a default setting of 88%,
which is too low for most children.
So it is important to have another means to identify desaturation.
Oxygen concentrators can be used with CPAP or BiPAP.
Standard maximum oxygen delivery is 5 liters per minute.
Note that this might translate into a lower FiO2 than with the same oxygen delivery via
nasal cannula due to dilution of oxygen by the device's air flow.
If a child is having desaturation when they're on BiPAP, try repositioning the child.
The head of the bed should be at 30 degrees, ensuring that the airway is open.
Suctioning and recruitment maneuvers like cough assist may be helpful.
Have a bag-valve mask available, if needed.