Here are a couple things to think about in preventing pneumonia in individuals with MECP2 duplication syndrome. They may seem strange, but they are relevant. Tight pants, wheelchair seatbelts, or anything else that fits snugly around the abdomen clearly present a risk. Medications used to treat acid reflux can help solve part of the problem but also increase the risk in an other way. So using these medications requires careful consideration of risks and benefits. Let’s consider them one at a time.
Tight clothing and seatbelts that are too snug. Tight clothes that restrict the waist or abdomen have been shown to increase problems with gastric reflux. They appear to do this in two ways. They have direct effect by increasing abdominal pressure and this can push contents of the stomach back up the esophagus. If some of this reflux enters the lungs it can cause pneumonia. This aspiration pneumonia may be due solely to the irritation of the foreign substance in the lung and we often think of aspiration resulting in this kind pneumonia, but aspiration can also cause bacterial pneumonia but moving bacteria that normally live in the digestive system into the lungs.
The second way this can contribute to reflux is that abdominal restriction can contribute to constipation problems. As the contents of the colon back up, reflux is likely to increase, even when the restriction is removed.
While this phenomenon of tight clothing causing or aggravating reflux problems is well known in the general population, but how does it play our for our family members with MECP2 Duplication Syndrome. Honestly, it is difficult to say. We don’t really have formal research, but common sense would suggest that this is likely to be even more relevant to them than to the general population for several reasons. (1) they generally appear to be more vulnerable to gastric reflux. (2) Many of them appear to be more vulnerable to aspiration because of swallowing dysfunction or depressed protective reflexes. (3) They have hypotonic abdominal musculature that allows external restriction to have much more effect on internal organs. (4) While many children and adults would complain about discomfort if they were wearing clothes of belts that are too tight, many of our loved ones will not complain or have difficulty making their complaints understood.
So simply avoiding abdominal restriction, is likely to reduce reflux and may be beneficial in reducing risk of pneumonia.
Acid Reflux Medications. Because gastric reflux is a risk for many individuals with MECP2 Duplication, many of them use medications that “control” gastric reflux. We need start by defining gastric reflux and what we mean by controlling it. The esophageal sphincter is a kind of muscular valve that allows foods and liquids to enter the stomach but works to stop stomach contents from going back up the esophagus. Reflux occurs when stomach contents move past this sphincter and back into the esophagus.
When people talk about drugs that “control” gastric reflux, there are actually several different categories of drugs that do different things. Surprisingly perhaps, most of them actually do little or nothing to eliminate reflux. Most of them actually are intended to reduce the harm and symptoms of reflux but they do little or nothing to actually keep the contents of the stomach from backing up into the esophagus. So what do they really do?
Antacids. Drugs like Tums, Rolaids, Mylanta, or sodium bicarbonate neutralize some the acidity of the gastric contents. By reducing the acidity, they reduce the severity of symptoms and harm caused by acid in the esophagus, but they don’t actually stop gastric contents from backing up into the esophagus.
Suspensions. Medicines like Peptobismol and Gaviscon coat the esophagus and provide some temporary protection from the acid that enters, but they don’t stop acid from entering. Some suspensions claim to create a barrier that helps keep acid in the stomach, but this effect apapears to be minimal for individuals with severe reflux problems.
H2 Receptor Blockers Medications like Tagamet, Zantac Pepcid, imetidine famotidine actually reduce the amount of acid produced in the stomach. This also can reduce the harmful effects of acid but does not actually stop gastric contents from entering the esophagus or teh airway.
Proton Pump Inhibitors. Drugs like like Prilosec, Prevacid, omeprazole, also reduce the production of acid and promote healing of the esophagus. They are generally considered teh moste effective drugs for people with severe reflux problems and appear to be very effective in reducing acidity for most people, but they don’t actually stop reflux from occurring.
Anti-gas medications. Medications like Oval, Beano, Gas-X, and Phazyme reduce gas production in the digestive tract. Gas can increase reflux by building up pressure that pushes the gastric contents up into the esophagus. For people whose reflux is caused or aggravated by gas, these medications may actually reduce reflux. However, for many people for whom gas plays only a minor role in reflux, these medications will make little difference.
The Bottom Line The point is that these medications are useful in controlling the acidity but generally do little to actually reduce reflux. For individuals with aspiration and reflux problems, this is a mixed blessing. No aspiration is good, but if aspiration occurs strong acid from the stomach is likely to increase irritation in the respiratory tract. That is the good part. Here is the bad part: Stomach acid helps digest food, but it also helps control bacterial growth in the stomach. Medications that reduce acidity also result in higher bacterial counts in the stomach. For individuals with weakened immune system, this means that they may contract pneumonia from these gastric bacteria if they aspirate them.
In fact, among the general population, a large scale study published in the Journal of The American Medical Association found that the use of these medications significantly increased the risk of acquiring pneumonia, even after controlling for other known risk factors. It reports:
In this large cohort, current use of acid-suppressive drugs was associated with an increased risk of community-acquired pneumonia. The increase in risk was most pronounced for PPIs and showed a clear dose-response relationship, which supports a real biological effect. The results are in agreement with a previous small study that was conducted ad hoc in a clinical setting. This study showed that subjects using acid-suppressive drugs more often reported clinical manifestations of respiratory tract infections and complications compared with those who did not use acid-suppressive drugs.
Gastric acid is an important barrier against pathogen invasion through the gastrointestinal tract. Although it is well known that a raised pH increases bacterial and virus colonization, the clinical consequences of gastrointestinal pathogen overgrowth have not been convincingly demonstrated.
This does not mean that these reflux drugs should not be used. They may be needed and justified to prevent pain and acid damage. The main point here is that they cannot be depended on to reduce the risk aspiration pneumonia. If reflux aspiration is a risk, some other measures may be required to control this risk.