Mariah is as cute as a button and a ham in front of a camera. She has Spinal Muscular Atrophy (SMA) Type 2; SMA is in a group of inherited diseases that cause progressive muscle degeneration and weakness, eventually leading to death.
Her primary physician, Susanna McColley, MD, Head of Pulmonary Medicine and Associate Professor of Pediatrics at Northwestern University’s Feinberg School of Medicine and Children’s Memorial Hospital in Chicago described Mariah as a Type 2 patient that presents similar to a Type 1 patient. The most severe form is SMA Type 1, also called Werdnig-Hoffman disease. Infants with SMA Type 2 have less severe symptoms during early infancy, but they become progressively weaker with time. SMA Type 3 is the least severe form of the disease.
Mariah’s attending physician, Adrienne L. Prestridge, MD, explained her recent history, “She has been in and out of our hospital for numerous months with recurrent pneumonias, recurrent pulmonary infections of different types, severe mucus plugging.” Because of Mariah’s severe muscle weakness she cannot breathe well enough on her own and her family chose to give her a tracheotomy and place her on a ventilator. Dr Prestridge stated, “So, she is vent dependent now 24 hours a day. And she’s doing quite well.”
The progression of SMA creates complications because patients cannot naturally clear their own secretions and since Mariah is also on the ventilator she is unable to produce a strong enough cough to keep her lungs clear from secretions so her physicians prescribed The Vest® Airway Clearance System. The Vest® System is used to help keep secretions from becoming retained in her lungs. She uses The Vest® System in conjunction with an insufflation- exsufflation device.
Mariah’s mom said, “Mariah is an amazing child, she learns things quickly and likes to keep busy. It is not hard to keep her occupied. I get good vibes about using The Vest® System with her, as I said, and it really helps my daughter.” According to her respiratory therapist Mariah tolerates therapy with The Vest® System well, even enjoying her Sponge Bob musical toy during her treatment.
Dr. Prestridge believes that using The Vest® System with Mariah has been positive; clearing her secretions and allowing her to have less infections. Prior to her therapies and treatment with The Vest® System she was frequently sick.
When asked what Dr. Prestridge would tell her peers about The Vest® System she exclaimed, “It’s simple. It’s easy. It’s not labor intensive for the families, which I think is a really important thing.” This was also echoed by Mariah’s mom, “It is not hard to use, just put in the numbers and settings. I would recommend The Vest® System because it really helps my daughter, I really like it. It brings up her secretions.”