Every child is unique. Every child is entitled to individuality and to being treated as an individual; in its development, happiness and… medical care.
In 2007 it was found that our oldest son, 6 years old by then, has Duchenne* Muscular Dystrophy (DMD). Shortly after that, he started taking on a daily base Calcort, tablets containing Deflazacort, a corticosteroid.
Our son now is 12 years old, and together with him we recently decided to stop taking these pills.
Although this is an individual decision that only applies to him and his specific situation, one that can’t be generalized toward other children, we think it is worthwhile sharing why we did it.
The end of his primary school is coming up. He prepares to make the transition to college. He will go to a ‘normal’ institute, not one specific for disabled people, because (1) his mobility is really good, (2) Duchenne has no cognitive impact, and (3) he wants to show that children with Duchenne can go to a ‘normal’ school. Fortunately we have found one not too far away who is willing to make this happen with us.
However, despite his good mobility, his self-esteem isn’t too great,
- He is very worried over his stature. Our son is very short and rather thick-set, and his face looks quite swollen.
- He often shows highly kinetic behavior; although often beyond his control, it seems. He doesn’t sleep enough in our view, has problems focusing and often just has plainly too much energy.
- Physically it is clear that his puberty is delayed.
Accidentally our attention was drawn to the side effect that Calcort has on delaying puberty. Intrigued as we were, we started checking other side effects again, and found that all findings that we have over our son can be related to the use of Deflazacort or alike products: the weight, behavioral problems (it looks like ADHD, but it isn’t it), swollen appearance (body fluids being held up), inhibited growth, delayed puberty. We retrieved information of specialists and hospitals all warning for such effects, and the absolute necessity to proactively follow up on them.
Our research revealed other interesting concerns too:
- The advantages of Deflazacort are an increased life expectation, less risk of Scoliosis and improved functioning of heart and lungs; however often with indications like ‘possibly’, ‘probably’ and ‘suspected’.
- Our son takes in 18 mg per day. According to his body mass he should have 40 mg, and there is an upper limit of 36 mg. Anyhow, it is a clear under-dose. In the past 6 years his dosage has never been increased. But, indeed, most studies suspect also with lower dozes a positive effect.
- Our son performs very well motorically, but also his bone density (Deflazacort has a risk of bone brittleness) and heart and lungs are in perfect shape, can’t be distinguished from another child.
So, we decided to stop taking Calcort. We are gradually reducing the daily dose so the adrenal gland starts producing the Cortisol hormone again. That was not an easy decision, and particularly emotional. It’s about our son’s life and future after all. Although we have taken this decision autonomously, we are quite comforted by all the information we have gathered. And, after all, every decision is temporary. Like the decision, 6 years ago, to start with Calcort. O U R D E C I S I O N.
We have decided not to pursue the options, presented in some studies, to change the regime or the time of day of administration (specifically advised for the behavioral problems). And our decision, when communicated to the doctor and team guiding us, was not protested by them anyhow. Neither did we get much information or support. Fortunately we were able to have a good talk with our GP.
This is not the first time that we take up our responsibility as parents independently, driven by a holistic view on the well-being of our son, looking at his individual situation (not comparing to others, nor speaking on the behalf of other children). We did so and decided to stop taking these steroids.
A last thought. Deflazacort is the only medical product that is known to have a possible positive effect on the progression of DMD. It is therefore not an obligation, to administer it from some sort of therapeutic stubborness, not taking other aspects of life and life’s quality into account.
Every child is unique. Every child is entitled to individuality and to be treated as an individual. Children suffering from Duchenne are no exception.
- This type of muscular dystrophy was named after the French neurologist who discovered it, Guillaume-Benjamin Duchenne.
- Duchenne muscular dystrophy (DMD) is a recessive and progressive affliction. Due to the lack of the protein dystrophin in muscle cells the muscles’ strength gradually keeps decreasing.