After doing some investigation, it seemed that what initially caused her extreme gastroparesis was a combination of reduced digestive capacity due to removal of her terminal ileum, ascending colon, and gallbladder in combination with use of acid suppressing drugs for 30 years, 15 of those years on PPIs. Now, you've all probably heard me rant about the evils of PPIs before, but there is no case that spells out the dangers of PPIs as clearly as Paula's. The longer the she used the PPIs the worse her gastroparesis became. About 2 years ago she stopped using the PPI and discovered that when she vomited she did not taste any acidity in the chyme, and she would vomit out exactly what she ate - unchanged - 8 hours earlier. It was then that she started taking Betaine HCL (a supplemental source of hydrochloric acid) to help boost her stomach acid levels and improve her digestion. It seemed that after so many years of using antacids and PPIs, her stomach's parietal cells had given up trying to produce acid anymore and she needed to supplement with it. As you know, stomach acid is absolutely critical to digesting proteins, helping us absorb food-borne nutrients, protecting us from pathogens that enter our digestive tracts, and much more (click here to read more about stomach acid).
The problem was that in addition to the PPIs, she had been using high doses of NSAIDs for years to control her lung inflammation. She doesn't have a choice about her use of these drugs - if she goes off them her lungs get so inflamed that her FEV1 drops below 20% and she's unable to effectively inhale antibiotics. But a major side effect of NSAIDs is that they reduce the mucosal lining of the GI tract by reducing the conversion of arachidonic acid to prostaglandins by inhibiting the cyclooxygenase enzymes, COX-1 and COX-2. COX-2 is an inducer of some nasty pro-inflammatory prostaglandins, but COX-1 is needed to preserve a healthy mucosal lining of the GI tract by maintaining a baseline level of beneficial inflammation in the gut (we humans would die very quickly if there was no inflammation in our bodies at all - sometimes it's a good thing, in the right amounts and in the right situations). So because NSAIDs block both COX enzymes, overusing them can cause development of stomach and intestinal ulcers. This is what was happening to Paula. At the point at which she started using Betaine HCL, the mucosal lining of her stomach was so thin and fragile that taking too much HCL would cause her stomach to burn and ulcerate. But the problem was that the amount of HCL she needed to properly digest her foods was less than the amount her stomach could tolerate. Over a year ago, she did switch to using a selective COX-2 inhibitor NSAID, which was significantly kinder to her stomach, however even this seems to affect her stomach negatively but it just takes a longer time period.
Paula was frequently using demulcent herbs like aloe to sooth her stomach when she felt the burning, but this wasn't enough to allow her to increase her dose of HCL. So I suggested she try taking licorice to help improve her stomach lining. Licorice contains saponins (soap-like molecules) that gently irritate the gut just enough to encourage it to produce more of its mucosal lining. Some people take de-glycyrrhizinated licorice (DGL) tablets in this situation to avoid the possible blood pressure raising effects that licorice's glycyrrhizine has on certain people, but in her case because she had low blood pressure and needed the other adaptogenic effects of the glycyrrhizine in whole-plant licorice, so I suggested she take whole-plant licorice. The problem was that due to limited access to supplements, herbs, and medications in South Africa, the only form of licorice she could obtain was in tincture, and the alcohol burned her stomach too badly for her to tolerate. So she ended up having to boil the alcohol off and using the sediment that was left over. This method was not sufficient long term, but she finally found access to capsules of licorice (though mixed with other herbs) and I am hopeful that will help her rebuild her stomach lining.
Another issue we wanted to address is that she has very poor fat absorption due to her lack of gallbladder and parts of her intestines. Her poop was always light colored indicating that not enough bile was being excreted (bile makes poop dark). Bile is produced by the liver, then stored and secreted by the gallbladder. Bile is the first step in fat digestion as it emulsifies fats, breaking them into smaller globules so that lipase enzymes have an easier job breaking them down further into triglycerides for uptake in the small intestine. Without an emulsifying agent like bile, lipases cannot properly do their job, no matter how much lipase we take supplementally (in the case of CF). So my strategy was two-fold: first address the acute lack of an emulsifying agent, and second help stimulate her liver to produce bile in a more on-demand fashion during meal time, even without the presence of a gallbladder.
The idea of trying lecithin came up because she was dependent on the supplemental Ensure shakes to keep weight on and maintaining energy levels, and those shakes are full of unnatural, denatured food-like substances and chemicals that are highly inflammatory. But it perplexed me that it seemed they were the only things that she could digest well. So I looked in the ingredients list and saw that lecithin was always present in any high-calorie "nutrition" shake. This makes sense, since lecithin is a natural emulsifying agent, usually derived from soy, sunflower seeds, or eggs. So I suggested she make her own version of Ensure minus the chemicals and additives, and add lecithin to these shakes. And guess what? She started to be able to digest them! It was a huge breakthrough for her because she had not been able to absorb coconut oil at all for about 20 years (would go right through her into the toilet) and now she was absorbing it fine, and her stools were firmer (she tends towards loose stools) and darker too! Another perk to her switching to a home-made high-calorie shake was that the high sugar content of the Ensure was causing her blood sugar to swing wildly and exacerbate her diabetes. By making a home-made shake she could control how much sugar was in these shakes, and even switch over from white sugar to a healthier source of carbs, like honey. The high sugar content of Ensure also caused major fungal growth that was impossible to control even with antifungal meds (evidenced by a thick white coat on the tongue). She reports that stopping the Ensure resulted in an amazing reduction of fungal growth! Using manuka honey in the mix yielded even more impressive anti-fungal results.
She added lecithin granules to a mixture of liquid coconut oil, whey powder, and water, shook them vigorously in a special protein shaker bottle, then let it stand for an hour to let the lecithin fully emulsify the fats in the shake. This process would probably be quicker if lecithin powder is used. When using lecithin, she can now also absorb the fat in avocados too! She now adds lecithin to other fatty meals as well. As the lecithin helped her emulsify and absorb fats better, she needed to use less supplemental pancreatic enzymes (Creon) as a result. In my opinion, lecithin (in combination with bitters) is a better alternative to supplemental bile salts/acids (like ox bile or ursodiol) because it's sourced from plants, not tortured factory-farmed animals, and it will still allow your liver to do some work for you, versus when you take supplemental bile salts/acids they essentially prevent the liver from secreting its own bile, and a lack of bile flow is a bummer for overall liver metabolism, cholesterol metabolism, and hepatic congestion in general.
I had been trying to figure out how to help Paula increase her stomach acid secretion in a way that wouldn't irritate her fragile stomach lining. As I mentioned, tinctures of bitter herbs (what I usually have people use) wouldn't work for her because the alcohol burned her stomach. So she read more about the beneficial effects of a commonly used bitter herb, artichoke leaf (Cynara scolymus), and got ahold of some capsules. She started opening the capsules (about 75mg worth) and sprinkling the contents in a little water (about an ounce) and drinking that down 5-15 minutes before meals. Her poops started to get significantly darker (signifying bile release) and she started producing her own stomach acid (she no longer needed to take Betaine HCL with meals). More bile production means better fat absorption and also better regulation of GI transit time. Artichoke is also a liver protecting herb, and there is actually a good amount of research on artichoke's hepatoprotective effects (do a pubmed.gov or Google Scholar search and you'll get a lot of hits). Furthermore, increased stomach acid production means that gastric emptying can begin to re-regulate itself over time, and she has noticed that since using artichoke her gastroparesis has improved.
So, to conclude, simple solutions like adding lecithin to foods and using bitter herbs like artichoke before meals can help improve fat absorption and digestive secretions. If you or your loved one is dependent on Ensure or other "nutrition" shakes to maintain weight, consider making healthier homemade alternatives that include lecithin. If alcohol extracts (tinctures) of bitter herbs are not doable for you for any reason, consider using bitter herb powders (like artichoke leaf, gentian, dandelion root, etc.) in a little water, or you can brew a bitter tea to drink before meals. In terms of herbs for CF, one of the most basic remedies should be bitters for pretty much all CFers (and all humans, frankly). If I had the money I would set up clinical trials on the beneficial effects of bitters on CF digestion. Maybe someday some cool hospital will set up this trial. Until then, be a citizen scientist like Paula and do your own experiments!