Alexander had been coughing from the back seat on our way back from Columbus. “Are you alright?” my wife asked him. “Yeah” he said. The coughs kept sneaking out of his throat with increasing frequency, nagging at us. He is scheduled for his next Spinraza shot for Valentine’s day, we need to be strong or face a re-schedule.
Surely he isn’t sick, maybe he just got choked on his sippy cup? After all, he did just have 4 separate doctors, two of whom were Pulmonologists, listen to his chest via stethoscope without cause for concern. Yeah, nothing to worry about.
The next morning Alexander woke up with a fever, igniting our adrenaline. We took him to the pediatrician where his fever had skyrocketed to 104.6 degrees! Scary stuff, but we were still shuffled back into the waiting area and queued up for our turn for our consultation. Three hours of waiting and we were sent to Princeton Community Hospital for a chest X-ray and blood tests.
Lungs looked fine, Flu test was (-) negative. Are we in the clear?
We received a call later in the night from his pediatrician. Respiratory Syncytial Virus (RSV). RSV can force even the strongest children into hospitalization, how would Alexander fair?
We consulted with Nationwide Children’s and were provided instructions for breathing treatments to occur every 4 hours. Breathing treatments included his Nebulizer, Percussion Vest, and Vital Cough. They felt it best for us to fight this at home while monitoring the situation with his local doctor. They wanted us to minimize his exposure to other sicknesses swirling around the hospital.
Alex was a champ and made it through RSV without any hospitalization. Most SMA patients end up hospitalized or in the intensive care unit with pneumonia. We count this as a victory for Alex’s strength and for the effectiveness of Spinraza.
A few days prior, before the coughing signals in the car, we were at Nationwide Children’s for Alexander’s scheduled MDA/SMA clinic visit. My wife was anxious to discuss Alexander’s progress and had a few questions regarding his needs at school. I was nervous about what they may say in their assessment. I’ve always tried to be cautious in my expectations, strong for Alex, but realistic and practical in his needs. My feelings weren’t helped by the troubling things that I had interpreted as muscle weakening in the previous few weeks.
From my perspective, Alexander seemed to be slurring his speech a little bit and even occasionally choking on food. These are both pretty bad signs for those with SMA, signs of lost muscle function responsible for swallowing. This can lead to aspiration of food into the lungs when eating. Any foreign objects or contaminants can develop into pneumonia, the biggest baddie to these warriors.
My wife didn’t seem too concerned, in fact, she was excited to talk about how far Alexander was coming along in strength. Momma Bear and I were on different pages, but I wasn’t about to ask her to turn her’s.
Alexander’s physical therapist came in for her assessment, asking him to stick his arms out in front of his chest, squeeze her hand, and even reach above his head. He scored higher on every benchmark since his last visit. My wife looked at me with a familiar gaze, I was once again wrong and this time, I was happy to admit it.
The Neurologist came in for his assessment, he was very positive with a permanent grin frozen to his face. He seemed sincerely excited to hear about how Alexander was doing. We discussed his progress since he started Spinraza almost a year ago. It was amazing to digest all of Alex’s improvements in that moment. He really had improved so much in the past 9 months. IMPROVED. No weakening, or atrophy, but muscle strengthening.
We talked about my perception of his slurred speech and general malaise. The doctor pressed me on those observations, asking when I had noticed him doing those things. It always seemed to be after I had arrived home from work and Alex had been at school. He said that it is most likely that Alex is tired from a long exhausting day and that this isn’t due to some muscle weakening, but rather the increased demands we have put on him to attend school. I wrapped up my worries and tucked them away.
He moved on to tell us that Alexander is one of about 50 Nationwide patients currently receiving Spinraza. All 50 are showing signs of improvements in strength and abilities. This was a complete shift in the world of SMA. Not only were patients no longer getting weaker, but they were getting stronger, each at their own pace. We were gushing with excitement, especially for the upcoming 6th dose 3 weeks later.
It was difficult to watch Alex cough. His cough was always weak and pitiful and you could always hear the mucus lingering around his lower esophagus without escaping his lungs. He really struggled with his RSV, but we were diligent with his breathing treatments and checked in with the local doctor frequently, receiving multiple blood panels and X-rays. His lungs remained fairly clear and healthy throughout his fight, although it would certainly test his stamina. His doctor said overcoming RSV could take 3 weeks for moderate cases.
No matter how bad he felt he would always offer up a smile. He complained a little at night about having difficulty sleeping, but that was more due to his legs getting stiff and him needing to be re-positioned. It was about a week during his RSV battle that he started to regain a lot of energy, even getting into mischief throughout the house in his wheelchair and visiting me (in his disguise) while I was taking a bath.
He was fighting this thing with such strength, we were so excited for him to get healthy for his injection on Valentine’s! We called a week before to confirm that we could still make the trip to Morgantown, WV for his treatment. His recent RSV recovery would eliminate anesthesia as an option for his injection, but his Neurologist decided that it was better for him to have his treatment as scheduled without anesthesia than to push his injection forward up to 6 months. We agreed and planned our trip.
Valentine’s Day had the whole family crowded in the car making the 4 hour drive to Morgantown. We regularly have a pre-injection appointment with the Neurologist to discuss how things have been going for Alex. She is always happy to see him and shows genuine enthusiasm for each of his new developments.
We also talked with Alex’s injection coordinator, who is working with the drug company and insurances for approvals. In fact, they are in the process of getting Alex’s insurance to cover the drug, which is a challenging task and no easy feat considering the tremendous price tag. She also informed us that his insurance recently put out a policy for the coverage of Spinraza, meaning that they now cover it for patients, but we just have to get Alex’s individual approval.
We are still waiting to hear back about their decision, but we were reassured it would be worked out before his next injection in June. All treatments up to this point had been provided to Alex by the Biogen drug program.
Pumped from our encouraging visit, we made our way to the PICU for the procedure. Surprisingly, or maybe not so surprisingly given the prevalence of influenza infections this season, we were informed that the PICU was at capacity and that we would have the lumbar puncture procedure in a different room a few hallways down.
We made our way to a treatment room with a sedation nurse and the child life specialist, who calms Alex with her iPad while he waits. His big brothers played Xbox in the family lounge with the hospital staff during the visit.
The procedure went pretty smoothly, albeit a little more prickly with multiple lumbar sticks from the doctor. Poor Alex was quite upset with the first poke, and understandably so; having needles stuck into your spine isn’t fun for anyone. Weirdly, we kept getting odd interruptions from random staff poking their heads in the room, looking around for something or someone and leaving. Momma Bear wanted to focus on supporting Alex and wasn’t happy with the odd, disruptive distractions making him uneasy while spinal fluid was being swapped from a syringe to his back.
The procedure finally wrapped up and little man was ready to go. He did complain of his back hurting this time, and the sedation nurse said that is quite common when kids get lumbar punctures. We had quite the surprise waiting for us on our way to get big brothers. A pony, dressed up in pink, with little booties and bows, was paying a visit to the children’s wing of the hospital. Alex nervously petted her snout, she was eager for his attention! Owen and Ethan were just as enamored.
Each year we try to host at least one, sometimes two large fundraisers to help cover some of the costs of caring for our little man.
This year, we held two raffles with prizes that were generously donated by local businesses and friends in the community. Our goal was to raise enough from proceeds to fund the building of a wheelchair ramp. The ramp provides Alex with a seamless path to and from our home. Previously, we had to carry Alex inside, either sitting in his chair or we’d carry his wheelchair in separately.
We’ve raised over 70% of the funds necessary from donations and raffles to build the ramp. We budgeted for the remaining amount so that we could get the ramp built in time for our insurance request for a powered wheelchair.
This ramp is an important step in Alex’s independence and was completed this past week, just in time for Alex to take advantage of another first: riding the school bus, watch below: