About Me

Wife, Physical Therapist turned stay-at-home SMA mom, and Independent Wellness Advocate for essential oils. I'm Meredith and this is the journey of our beautiful life! I am Mom to our sweet Leo, who was diagnosed with Spinal Muscular Atrophy (SMA) at 3 wks of age. SMA is a terminal, neuromuscular genetic disease. Our life is not what we "planned", and it can certainly bring many tears at times, but Leo shines a light brighter than we could have ever imagined. His love and joy has changed our lives, and we are forever grateful for our greatest blessing. He is medically fragile and requires 24/7 skilled care, but to us he is perfection. He is part of a greater plan we are just beginning to get a glimpse of. We live life embracing every moment, and doing everything we can to give Leo every experience possible all while praising God for our MANY blessings.

Wednesday, August 19, 2015

SMA Awareness: Respiratory

One common question we often get is if Leo uses oxygen, or if his BiPAP is giving him oxygen. Although we do carry an oxygen tank in case of an emergency, Leo does not get oxygen. SMA does not cause a problem with gas exchange in the lungs. Therefore, the blood is getting oxygenated as needed. What happens with SMA is the actual muscles that are used to take a breath are weakened to the point where breathing becomes very difficult. The muscles in between the ribs, the intercostal muscles, are weakened to the point that Leo uses his diaphragm to breathe.
A drop in oxygen levels can occur but it is typically due to 1) airway blockage by mucous or a plug which must be cleared using cough assist or suction 2) Leo becomes so fatigued to the point he can not physically take a breath (this can happen when he gets very upset) or 3) improper BiPAP settings or mask/interface. Unfortunately
we see all too often when these babies are being treated under physicians care that are not familiar with SMA, they are quick to put them on oxygen which only masks the underlying problem and can actually be very dangerous.
Most babies with type 1 SMA would benefit from breathing support at night starting at a very young age. When sleeping, the muscles used to breathe are more relaxed. When these muscles are already weakened, the breaths become very shallow. This can result in a drop in oxygen levels and an increase in carbon dioxide (hypoventilation). Non-invasive support using a BiPAP machine (or ventilator) can be extremely effective in helping provide support. This machine uses a mask that covers the nose, and provides additional respiratory support by forcing air into the lungs. This allows the baby/child to receive a higher volume of air, and also helps keep the lungs more expanded during exhalation. The vent has a set rate of breaths it gives in a minute and can also sense when Leo is taking a breath. So it gives breaths when Leo is having trouble taking them (or asleep), and helps give the breaths he does take on his own more support. A BiPAP can also be lifesaving during times of illness. We call it a BiPAP often but technically this is a ventilator (vent) used non-invasively as a BiPAP not a true BiPAP machine. We also sometimes call this “his mask” when referring to the vent.
When Leo is on his vent, he gets to rest. For the most part he only needs this while sleeping at night and during naps however, there are often times when he had to be put on his vent when awake for different reasons. He is usually on his vent when I am driving any further than our local park or on the interstate. If it is past naptime or if Leo is staying up late one night, we will also put him on it so he can rest. Leo is pretty laid back, but like any toddler he can get upset and have a tantrum. For Leo, this can spiral into serious situations if not handled quickly. He will clamp down and his heart rate and oxygen can drop rapidly, and he will need his vent to give him those breaths to recover. It is also helps him while sitting up or standing. Leo has always been a little fighter, and he will still fight being put on the vent when he does not want it or think he needs. For the most part though, it has become very comforting to him, and he enjoys being able to rest and breathe easy.

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