It's definitely associated with CF and it seems only CF. Being a person who distrusts almost everything, especially when it comes to absolutes, I expected it was in other disorders. It's not. Not to sound cliche but you asked a great question. What is it about CF to have a unique its own brand of obstructive constipation?
I'm just in the middle of a 2014 scientific article about acute pancreatitis (AP) and the mucus barrier of the intestines. Written in the journal "Shock", it's focus is on what causes complications of acute pancreatitis. In essence, the trauma of the AP causes the protective layer of mucus in the small intestine to shed in a short period, exposing the enormous surface area of the duodenum and the rest of the intestines to microbes, their toxins and all the nasty gunk from the pancreas. The result is sepsis, multiple organ failure and death. Hence why I'm finding a great article in a journal called "Shock". The plethora of drug commercials warning of acute pancreatitis and death has me curious about dying from AP. I've had chronic pancreatitis and more AP attacks than I can count and there have been times when I might have wished to die, but I never considered it a possibility.
I've been thinking of DIOS as caused by exocrine pancreatic failure. Maldigestion seemed to be the only cause of the light, airy, sticky, stinky and voluminous stool that makes for DIOS conditions. When our feces are unable to be sensed, the intestines don't have any signals to move it along. If it was nothing more than maldigestion, everyone with pancreatic disease would be vulnerable to DIOS.
The lion's share of the mucus in a CFer ends up in the gut. A liter of mucus from the nasal passages and everything from the lungs that doesn't get coughed out, gets swallowed daily. Mucus in the digestive system gets added too. One of those sounds when we are hungry, especially that five second growl, is in fact the small intestine squeezing itself out, cleaning up the old mucus and whatever food particles, depleted enzymes and such, in preparation for a new meal.
CF impacts the mucus cells and the GI tract is florid with them. Hyperviscous mucus mixed up with half digested food is a more complete recipe for DIOS. Mucus is produced in considerable amounts in the digestive organs as a protection against digesting our own gut. That thick mucus is the best we can produce. Maldigestion can be fixed by adequate amounts of enzymes.
Absorption of nutrients is made much more difficult with the hyperviscous mucus. Malabsorption is the other major factor in DIOS. In the article they have microscopic images of the intestinal villi with protective mucus coating the sides and tips of a carpet of nearly microscopic villi, looking upright and healthy, to the after effects of villi stripped of its mucus, limp, flattened and dead or dying. Mucus is critical for keeping our digestion going on inside a body made of the same stuff we hope to consume and we need it despite the fact that it makes absorption more challenging. Ideally we can tune the gut to digest and absorb food. Without quality absorption we end up with voluminous stools, and it's virtually toxic when it's not absorbed.
Bottom line is the unique combination of maldigestion, malabsorption and mucus that rivals contact cement are required to have the condition of DIOS. Of course there's meconium ileus at birth.
LL