Christmas, it truly is the most wonderful time of the year.
It was a wonderful year for us and also for the SMA community as a whole.
Christmas can be an emotional time for SMA families. It’s one of the biggest milestones each year. It can be the highest of highs and the lowest of lows, sometimes within the same day.
This year we celebrated Jadon’s 8th Christmas and his 7 ½ year birthday. It’s kind of cool having a half birthday on Christmas day. Both of these far surpass the numbers stated with the SMA prognosis we were given with his diagnosis. In fact they told us not to plan on more than one of each. Therefore, you can imagine how blessed we feel each and every year.
Inevitably, when we are taking down the decorations, we wonder if it was his last. It’s hard to imagine a Christmas without our little Super BUG. We always try and live in “the now” but sometimes fear just has a way of sneaking up on us.
We know far too well how blessed we are to have Jadon celebrate his 8th Christmas. We owe it all to the protocols of his SMA specialist, Dr. Schroth. We started her protocol when Jadon was just 4 months old. This keeps his respiratory strong enough to not just live, but live a life worth living… To “Dance in the Rain of this Life Storm.” We have always wanted Jadon to get out and experience as much as possible. We are forced to constantly weigh out what truly is possible.
Jadon’s entire life has been a balance of risk vs potential reward. Jadon goes to school for only part of the year because in Cold & Flu season the risk far outweighs the potential reward. He does get to facetime with his class twice each day.
We struggle each year with exactly what day to pull him out and what day to send him back. We shoot for Halloween each Fall so he can partake in all the holiday festivities and the reward is tremendous. Dressing up and just being “one of the kids” is one of his best days of the year.
Each and every outing gets weighed out in our minds before we step out of the house. A hot baseball game, a walk on a cool day, going out in public during winter months, these things are fun for him, but how fun? How much risk? Could the trip turn deadly (literally)? We have gotten out this year more than any other year of his life, it was a great year.
As we were preparing to soak up every second of Jadon’s miraculous 8th Christmas, there was an announcement about to be made that will drastically change Christmas for every future SMA diagnosis.
On December 23, 2016 a miracle was announced.
The FDA approved the first ever treatment for Spinal Muscular Atrophy (SMA). The drug is named Spinraza and it is a treatment not a cure. A “cure” would be a ONE TIME application that eliminates the disease. A patient receiving Spinraza will need six lumbar injections the first year and 3 injections every year for the rest of their lives.
BUT IT WORKS!!!!
IT REALLY, REALLY WORKS!!!
The drug had been in trial previous to the FDA approval and some of the Type1 children starting their dosings at a very young age are now walking. Below is a picture of Jadon’s buddies, Mateo & Javier, two amazing brothers who both have SMA Type1 just like Jadon. Javier has been getting Spinraza since he was only 12 days old.
Let the water works flow as a true miracle is witnessed.
This is what we have prayed for ever since we were so rudely introduced to SMA back in 2009. We have been told year in and year out that a viable treatment/cure was on the horizon but now IT’S REALLY HERE!
We are overflowing with joy.
HOWEVER.
We truly wish that joy was the only feelings that came with this announcement.
HESITATION –
As we have expected from any potential treatment or cure, Spinraza halts the progression of the disease. The deeper the progression the less impact any treatment will likely make. The less any improvements may actually be noticed.
So what does that mean for Jadon? It is very uncertain. Jadon had lost most of his movement by the age of two. Only his feet and fingers retained VERY limited mobility at that point, but we haven’t seen movement of any kind, not even the slightest little foot twitch for over a year.
So we are back to weighing risk vs potential reward.
With the impact of this drug likely being minimal what kind of risk are we willing to take to receive it? This is the unfair struggle we are faced with.
All things are relevant and for Jadon we would be ecstatic if he could regain movement in his fingers to enable him to use switches and possibly communicate. A child who can only lay flat would be happy to sit. One who can sit would like to stand, and someone standing would of course want to walk. If we felt this drug would make Jadon walk you would not be able to keep us from attaining it.
This is one of the most heart wrenching decisions we have had to make yet.
DISAPPOINTMENT –
We are not only disappointed that this didn’t come sooner for Jadon, we are disappointment for the kiddos who just missed being helped by this miracle drug. Those kiddos who were stronger than Jadon but passed away within the last two years. Those children who passed away this year who were younger than a year old could have been helped the most.
Why are young kids still passing away? Because even now, many doctors still tell families with an SMA diagnosis to simply take their child home and love them for the little time they have. Hopefully, this thought process will be eliminated with a viable treatment available…we certainly hope so.
POLITICAL –
Always hate to wander onto the topic of politics but one of the hottest topics right now is the Affordable Care Act (“Obamacare” when speaking negatively of it.) The Affordable Care Act (ACA) removed Lifetime Limits and Pre-Existing Conditions. Prior to its enactment, an insurance company could set a lifetime limit and when a policy holder hit that limit, they could drop the insured client. Then with a pre-existing condition other companies could decline coverage. This can SEVERELY impact families and individuals with a severe and/or chronic diagnosis. One hopes to never need insurance but the fact is that NO ONE with a chronic illness can afford to be without it.
Spinraza is very expensive. Rightfully so I guess, as it will not simply save lives of SMA kids it will change their entire future with the disease. If a lifetime limit is allowed to be part of an insurance policy the children receiving Spinraza would meet those limits VERY quickly. We pray that somehow these provisions of the ACA will remain a part of whatever new healthcare plan is implemented upon our country.
So the short of it all is that Jadon will not receive Spinraza in the near future but we are certainly watching for options. We are praying for those friends who are traveling to get this miracle for their children. We pray it will help even the weakest of them to become strong.
We are blessed to have a front row seat to a miracle which is changing the course of young SMA lives, and it is AMAZING.
We also have a front row seat to this young man’s life, which is also very amazing and miraculous.
Jadon's Hope Blog 