What exactly is a mucus plug?
Mucus plugs are the single scariest thing to a parent or a caregiver of a child with SMA.

It is a condensed ball of very thick secretions that blocks your child’s airway. To put it in simple terms, it closely resembles a hairclog occluding or blocking your sink drain. There is enough room for some of the water to pass but not enough to keep the sink clear.  The plug itself may not be reachable by a suction catheter.  Plugs can occur in the bronchial tree deep into the chest.  This is why the Cough Assist is so vital to restoring their airway.

bronchial tree

A mucus plug most often does not completely block the airway but there is not enough space for a sufficient amount of air to pass to sustain life. There is not one absolutely right way to handle this situation and we are more likely to act on emotion when in the face of this crisis. So you should take a moment now and make a plan for what to do when this happens. Hopefully this article will be able to help you plan for such an event

Step 1: Prevention

Your cough assist is your best friend. Yes, your BiPAP helps your child breathe and, yes, your suction extracts the secretions but if you have a plug blocking the airway, neither of those machines will be able to do their jobs. Chest PT (CPT) is of the utmost importance. When your child awakes in the morning, you need to initiate CPT as soon as you can. Secretions are thicker in the morning, so your chances for a plug are at their peak. Another preventative is humidification. Keeping your child’s BiPAP humidified will help in keeping his/her secretions thin. But be careful not to over-humidify as this may cause issues of its own. You will know if you are over-humidifying if you see standing water in the tubing. Taking the precautions to prevent a mucus plug is your child’s best option.

Step 2: Recognizing the Early Signs of a Mucus Plug

We all know the obvious signs, i.e. pulse oximeter going crazy, white/blue lips, that terrifying look on your child’s face. And recognizing the early signs can be particularly difficult depending on the type and limitations of SMA your child has. If your child is a particularly “chatty” child, then you are going to want to listen for the silence. Have a test word/sound you ask your child to make to be able to tell if there is sufficient air movement. Listen to your child’s breathing. You can actually physically hear fluid either in your child’s trachea and/or lungs. This may be resolved with a few coughs or an extra round of CPT. If your child is more silent, then you can watch their energy level. A child with a potential mucus plug will not be as active and will also tire more easily.

Step 3: We have a plug! What do we do!

Suction first then COUGH COUGH COUGH COUGH COUGH!!! There is no magical number of coughs that will do the trick.

The beginning to this solution is simple. Suction first then COUGH COUGH COUGH COUGH COUGH!!! There is no magical number of coughs that will do the trick. Use your cough assist until you see the oxygen saturation begin to rise, then suction. Then keep those coughs coming and suction in between every 1-3 coughs or if you feel or hear a particularly thick cough. The plug WILL move in one direction or the other. There is some controversy as to whether or not calling 911 should be on your mucus plug protocol. The plug WILL move in one direction or the other And there is no right or wrong answer to this question. However, EMS personnel do not know your child and, chances are, will not be familiar with SMA nor will they care at this point. If you call 911, once those paramedics arrive, your wants and needs will go out the window. EMS staff are trained to recognize basic needs and to treat those needs with the quickest solution possible. This may involve intubation since EMS may be unfamiliar with the use of the Cough Assist machine. They also may not take your equipment to the hospital, so if you do go to the hospital, TAKE YOUR COUGH ASSIST WITH YOU! This is imperative! There are a number of great success stories by calling EMS and there are a number of horror stories. This article is designed to help make you aware of what may happen based on stories we've solicited from other families. Overall the basic rule of thumb is, once you no longer feel comfortable treating your child yourself, call for help.

Step 4: We need help and are calling EMS

This step is a good thing to start while someone is performing coughs from step 3. This should not replace step 3.


Being a healthcare provider, if my patient had a mucus plug I could not clear and I had to call the ambulance this is what I would tell them:

I have a (age) (gender) with a chronic pulmonary disease that has a mucus plug unable to clear with cough assist. Oxygen saturation is (%), pulse is (###). (Gender) (does or does not) have a trach. (Gender) plugged off (#) minutes ago.

This keeps it nice and clean, only relaying the information that is absolutely needed. Another way to help EMS get your child out the door as quickly as possible and to make sure your child gets the care that he/she needs is to give the paramedics a copy of your child’s Advanced Directives. Also, when you know EMS is on the property, unplug your child from the pulse oximeter. This will prevent it from being dragged along behind your child. Once you have started the ball rolling on obtaining paramedic help, you cannot undo this. So the best thing you can do now is to move out of the way and let them help. If at all possible, one caregiver should ride in the ambulance with the child and someone should follow behind with the cough assist in their car. Due to insurance reasons, you will not be allowed to take your cough assist in the ambulance.

Items to bring in the ambulance

Pack a small bag and keep in where it can be grabbed at a moments notice. The bag should contain

Trauma bag

  1. A spare Anesthesia mask that fits your child
  2. portable pulseOx (if you have one)
  3. spare feeding tube
  4. batteries for portable pulseOx
  5. one 6 foot spare hose for cough assist
  6. spare filter for cough assist
  7. roll of co-flex tape (aka SMA Duct-tape)
  8. nasal and catheter suction
  9. saline

meet with your ambulance crew before you need them

If possible schedule a meeting with the EMT crew so you can go over your particular needs and introduce them to your equipment. This can help in a future emergency

Step 5: We’ve cleared the plug, what do we do now?

Well if you opted to call for paramedic help then you are now at the hospital and will need to make the appropriate decisions that are in the best interest of your child. If you stayed home and have cleared the plug, then a round of CPT is in order. This brings us back to step 1.

Mucus plugs are inevitable. They are going to happen and it is a very scary situation to be in. Being prepared is the best thing you can do as a caregiver of a child with SMA. No two situations are ever the same. And at the end of the day you have to do what’s best for your child. Just remember that prevention and early recognition are essential to potentially saving your child’s life.

Thank You

Parents/caregivers who helped contribute to this article:

Sarah Turnbull
Nate Lee
Meri Phelps
Nurse Kim Lovin

Thank you VERY much