Our experience with SMA is a unique one. As parents of a 9 year-old with SMA, being medical professionals has given us somewhat of an advantage over finding doctors, medications, and treatments for our son. We hope to share a little of our experience and opinion with you.
For most of us, the common cold is a nuisance. Most providers will not prescribe anything for the common cold, assuming the viral cause will take its course and, in a week or so, we bounce back. The question presented early on for us was: with these frequent common colds, should we prescribe antibiotics and steroids for our son?
Before Spinraza, our son was sick every 3 months, like clockwork. One of those times happened to be an episode of respiratory syncytial virus (RSV) when he was 20 months old, which turned into pneumonia resulting in intubation, and a month-long stay in the children’s hospital. That, of course, is a different beast and for which he was subsequently given Synagis — antibodies that are prescribed monthly to help protect high-risk infants and children from severe RSV disease throughout the RSV season. However, my son’s every-3-month “colds” were always the same: fever, coarse cough, sore throat, and then the congestion. Even healthy, he can have a small amount of chest congestion that is never really cleared, due to his weak cough muscle and generalized weakness. So, when he develops a respiratory illness, a worsening and increasing frequency of cough, an increase in sputum production, and fever are all signs that would prompt us to start an antibiotic. We also always give him a short course of oral steroids, both of which cause his pulmonologist to cringe.
We could let the “cold” run its course for a few days, but when he develops the change from baseline, we call in antibiotics. Again, it helps that we are medical professionals. We do not have to call the pediatrician, wait in the office to be squeezed into the schedule, and then try to persuade the pediatrician for a antibiotic prescription. I am certain that such practice of starting antibiotics with the first sign of fever will raise some criticism, given the rise of antibiotic resistance. However, because of our son’s muscle weakness and high risk of developing a severe illness, it’s a chance we are willing to take, and it has prevented many hospitalizations, not to mention thousands of dollars of hospital bills and insurance claims.
Mateo is 9 years old, and he has only been hospitalized once as a result of a respiratory illness.
Another very important factor in our treatment approach is our relatively proactive approach to his pulmonary hygiene. My son uses a bipap almost every night. He is not dependent on it, but it helps and, during a cold, it’s a savior. We also use the cough assist machine frequently, suction if needed, and utilize the vest machine multiple times throughout the day when he is sick. The importance of these treatment modalities with a respiratory illness cannot be overstated. For children with SMA, these machines can seem rather archaic and take some getting used to, but they are a “lifesaver.” For Mateo, we introduced this regimen when he was around 18 months old, and by 2-3 years, he would ask for them and it helped his recovery tremendously. As with any sick child, stressing hydration, small sips of fluids frequently, and eating whatever tastes good for children with SMA is extremely important given their weakened condition and their calorie requirements.
Are we over prescribing? Maybe. Have we prevented him from a worse infection? Probably. My son is on the weakest spectrum of SMA Type 2. The fact that he has barely missed school, even before Spinraza, and he has not been hospitalized since the RSV pneumonia at 20 months old speaks something to me. Even though our son has not had a recent cold (in almost a year — thanks Spinraza), when he eventually gets one this respiratory season, we will for sure be back to our usual prescribing fashion. From a parents prospective, families with SMA go through a lot. It is not easy to get these kids to the pediatrician for days on end. If you are a patient or parent, find a good doctor with the time to treat you individually and not treat a guideline. Good pulmonary hygiene must be stressed: cough assist, suction, vest, and if necessary bipap (again, I’m a big proponent of this — it helps).
Rio is a Family Nurse Practitioner at Duke University who resides with her family in North Carolina. Her husband is a Board Certified Emergency Room Physician and they are the parents of 2 children. Their oldest son, Mateo (9), has SMA Type 2 and their daughter Luci is 1. Rio is passionate about advocating for those with SMA.