Breaking the Cycle of Pain Part II: The Building Strength Approach to SCD

Here’s the problem (and why you should care):

    SCD is the single most important genetic cause of childhood mortality globally. Without intervention, 50–90% of children will die in childhood.
  1. There are over 2 million SCD sufferers in Nigeria alone. Hematologists, those specialized in the treatment of SCD are fewer than a hundred.
  2. Most healthcare workers do not know how to effectively manage an SCD crisis, thus increasing the likelihood of death, even for those who arrive at the hospital on time. This risk is increased exponentially among those living in remote parts of the country.

Our goal this year is to reduce the frequency and severity of crises in at least 100 children by over 90%.

This is a drop in the bucket, admittedly, but it could conceivably help in a few ways:

  1. Establish the Building Strength Approach as a viable option, even in remote regions, and
  2. Encourage interest in the Nigerian Government and the “Powers that Be” in investing in the training and equipping of healthcare workers in these modalities, and
  3. Maybe, just maybe, give these 100 children a chance of a normal life – to laugh and play, to learn, and fight and grow…just like other kids their age.

So, how we address Sickle Cell Disease???

The Building Strength Approach is a term I coined over a decade ago in an attempt to express what I believed was an under-appreciated fact: that the human body has remarkable, and for the most part, untapped ability to heal, repair and correct itself, if given the right resources. This approach, then focuses on identifying and supplying the resources the body requires (and thus strengthening it) to do what it does best.

The “Root of All Evil”:

Let’s apply this philosophy specifically to SCD. The trouble begins when, under certain conditions (eg, extremes of temperature, dehydration, fatigue, stress, etc), normal red blood cells (rbcs for short) lose their elasticity and shape and become “sickle” shaped.

This results in one or two things:

  1. Blockage of arteries and capillaries, leading to “vaso-occlusive” or “infarctive” crises and
  2. Rupture or disintegration of the defective cell, leading to anemia and the release of harmful toxins (hemolytic crisis).

This may be an over-simplification, and I certainly would not argue with that, but those two events eventually lead to most, if not all the problems we see in SCD.

“The Four Horsemen:”

So then, the obvious focus should be on 1. Stopping that transformation (from normal to “sickled” cell), and 2. Repairing the damage already done.

And there are four main areas we focus on strengthening (or improving), all of which are implicated in SCD:

  1. The Immune System
  2. Anti-oxidant protection/Protection against free radical damage
  3. Inflammation
  4. Vascular health