By, Krista Brown
Breathing was one of those automatic functions that my family easily took for granted years ago. Not anymore! Our son Joshua has Ullrich congenital muscular dystrophy, and restrictive respiratory disease. He is now a fifteen-year old high school sophomore that has a challenging schedule and social life, which he tries to keep up with. Like others on this journey through life with a muscle disease, there have been ups, downs, and plateaus of all kinds.
As parents though, our biggest daily focus is to keep him healthy, safe, and breathing comfortably. Luckly for us, technological advances in medicine have enabled Joshua to do just that. It has been an exhausting journey, but for now, Josh’s life has a fairly normal routine, and his energy level has been bolstered enough, so that he can make it through a full day of activities.
Josh uses a variety of modern marvels to do this. Making it easy to breathe, and keeping his capacity high has prevented him from the fatigue and illnesses he once experienced more frequently. When Joshua was eleven, he was admitted for a scheduled sleep study. The result of that study set the wheels in motion for all that was to come. He was diagnosed with apnea, and experienced a build up of CO2 in his lungs. Shortly after that, he was fitted for his first Bi-PAP. He began using it at night during sleep, and when in a lying position in bed. He had very little resistance to using the device. It took some time, but once he found a comfortable headgear and mask, he was able to appreciate the effect the machine had on his sleep, and adapted well. He rested more soundly, rolled-over fewer times during the night, and felt more energized in the morning.
About a year later, Joshua’s orthopedic surgeon confirmed that unfortunately, surgery was required to stop the progression of his scoliosis, and straighten his spine as much as possible. This, in turn, would also to help him breathe more easily, by creating room for his lungs to expand. Initially, the sergery was very hard on his body. He suffered a partially collapsed lung that had to be re-inflated while recovering in the hospital. He lost a significant amount of weight, and spent many weeks using his Bi-PAP to assist breathing during the day as well as night. He couldn’t seem to inhale enough air in each breath without the assistance. Eventually, he was able to reduce the use of his Bi-PAP, but he remained very uncomfortable without it. Over the next eight to nine months it became apparent that his orthopedics had improved, but he was still tired, winded, and had markedly diminished energy during the day. It was also difficult to hear his words when he spoke. School had started again, and he was asking to use the Bi-PAP at school any time he could stop and hook it up. Something was not quite right, but what followed was an introduction to another amazing piece of equipment.
Josh’s pulmonologist suggested that he should try a “sipper” ventilation system to assist his breathing during the day. He was prescribed an LTV 950 ventilator. The portable system attached to his chair, and allowed him to take an assisted breath whenever his desired. We found he wanted the assistance often! This has definitely been the most amazing piece of equipment yet. We had all been a bit apprehensive about the direction his respiratory system had taken at the time, but this ventilator was the answer to our prayers. Learning to use it was like second nature for Josh. It is now in use all waking hours with the exception of showering or bathing. It gives him the freedom to maintain his daily routine and boost his energy level. He can take deep breaths, stack his breathes when needed, and can even get enough air to help blow his nose and clear mucus. Exciting …especially when you have seen what life was like before the aid of this system! One other bonus to having the portable ventilator is that it can also run on its own battery power long enough to get through emergencies, like a power outage.
Finally, there is one last piece of technology that has positively impacted Josh’s breathing. This is the cough-assist machine. He exercises his lungs with it daily by completing at least 25 repetitions of inspiration with the insufflator. We also assist him in using the exsufflator when it is necessary to clear his throat or nose of secretions. The combination of ventilator, Bi-PAP, cough-assist machine, and preventing illness whenever we can, has allowed Josh to lead a very stable daily life. He is definitely dependent on the technology, but it has given him a brighter future. He is a happy, infectiously witty young man who has talent to share with the world. Now, he has more time and energy to do it. We are extremely grateful to the proactive approach of his pulmonologist, neurologist, team of caring doctors, and of course, technology.