a toddler or child with chronic cough…

a child with frequent belly pain and poor appetite…

a teenager or adult with heartburn
         or excessive appetite...

It may seem strange, but all these things and more can be caused by the same problem: Gastroesophageal Reflux Disease (GER or GERD). GER is also sometimes referred to as Acid Reflux, Reflux Esophagitis, or Hiatal Hernia (although the last term is inaccurate for reasons which will be explained below). GER is fairly easy to treat/control, but it is sometimes very difficult to diagnose. In this article, I will explain what GER is, when and how it occurs, how it can be treated, and many of the difficulties involved in it’s diagnosis.

GER is a very common disorder in all age groups.  It results from a loose or incompetent lower esophageal sphincter allowing acid contents of the stomach to splash upwards into the esophagus.  This causes pain, inflammation, spitting up, or even aspiration into the lungs.  These effects can, in turn, lead to further complications such as esophagitis, growth failure, or chronic lung disease.  GER occurs in two general forms - an infant form which is usually benign and outgrown, and an acquired form which can start at any time later in life that is usually not so benign.  There are many approaches to treatment, and these must be individualized for each patient.  Diagnostic tests are not always necessary and no one test is "best". They all have pros and cons.  Thus, diagnostic work-up must also be individualized.  Symptoms of GER should not be ignored except in young babies who are growing well, developing normally, and seem otherwise healthy. Symptoms of GER should always be brought to the attention of your physician.

A sphincter is a ring of muscle. There are many sphincters in your body, most of which you are unaware of because they are deep inside and not under voluntary control. The sphincter you are most likely to be familiar with is the anal sphincter, which controls defecation, because it is under (partial) voluntary control.

Sphincters are special, not just because they are rings, but also because, unlike virtually all other muscles, they remain contracted all the time until relaxed for a purpose (in contrast, your biceps or hamstrings remain relaxed until contracted for a purpose). This makes sphincters function like "doors" in the passageways of your body. They stay closed until they open to let something pass, then close again.

One such sphincter "lives" at the lower end of the esophagus, the tube leading from the mouth to the stomach. It controls the passage of food & drink from the esophagus into the stomach. It's opening is the involuntary final step in the swallowing reflex which began under conscious control a second or two earlier in the mouth as a movement of the tongue and palate. It continues as an involuntary wave of contraction through the esophagus pushing the food forward, and finishes as a relaxing of the LES to allow the food to pass into the stomach. Once the food has passed, the LES is supposed to snap shut again.

The LES can be thought of as either the exit from the esophagus or the entrance to the stomach. It's both. Normally, a closed LES prevents stomach contents from flowing backwards into the esophagus. This is important because the contents of the stomach are usually quite acid. The lining of the stomach is designed to withstand such acidity, but the lining of the esophagus is not. If acid gets into the esophagus, it burns. This is immediately painful, can cause tissue breakdown and inflammation over time, and eventually lead to scarring, narrowing, and even cancer of the esophagus (although the latter is something that virtually never happens in childhood because it takes so long to develop).

In persons with GER, the LES doesn't work right. Either it is too weak (or loose) to hold the stomach contents in (remaining partially open all the time), or it intermittently spasms and relaxes when it is not supposed to. This allows the acid contents of the stomach to move upwards into the esophagus. Sometimes the reflux goes only a little way up, and sometimes it comes all the way up and out the mouth. The latter is more likely in a small baby. Beyond spitting up, the symptoms of GER vary by age, degree of acid production, height of the reflux, frequency of the reflux events, and what other medical predisposition’s or illnesses the individual may have.

• Regurgitation - this is the obvious one, "spitting up" or a sour taste in the back of the throat. Unfortunately, not everyone with GER has it because it only happens when the refluxed material is coming "all the way up".

• Pain - the "classic" pain of GER is "heartburn" but this is by no means the only kind of pain people experience, and children younger than about 8-9 years are not able to describe their pain well enough to be sure what they are experiencing is heartburn or not.

• Cough - people cough with GER for two reasons - one is a "reflex" which exists between the nerve endings of the lower esophagus and the lungs, and the other can be if they are directly aspirating (refluxed material getting into the windpipe itself). See below for a discussion of the relationship between asthma and GER.

• Poor Appetite/Poor growth - it's easy to understand how having abdominal discomfort all the time might suppress your appetite, but that's not the only reason people with GER don't eat. They also may "learn" that keeping their stomach less full results in less pain. Unfortunately in a child, this can effect growth. In a baby poor growth may also come from direct loss of nutrients if the spitting is severe.

• Excessive Appetite - this may seem paradoxical, but it happens. The reason seems to be that food itself often has an acid neutralizing effect, and eating can temporarily relieve the discomfort.

• Bleeding - if GER becomes severe enough to cause esophagitis, blood can appear in the stool. Most often it is microscopic and cannot be seen with the naked eye, but sometimes the bleeding is severe enough that visible blood is present. In a baby it may be bright red, as you expect. In an older child or adult it will more likely show up as "melena", a black colored stool which comes from "digested" blood.

Congenital GER is something a baby is born with and, in fact, it is probably normal. We say this because only a minority of babies have a fully "competent" LES at birth. The LES tends to be not fully developed by birth, and allows some degree of reflux in more than 80% of newborns.

The amount of initial GER seen in any particular infant can vary from mild to severe, but because the LES grows/matures steadily throughout the first year of life, congenital GER is usually outgrown by 12 months of age. Unfortunately, this is not always the case. About 2% of children with congenital GER never outgrow the problem. Those who have not outgrown it by one year of age are unlikely to ever do so. When congenital GER isn't outgrown by one year it becomes very similar to acquired GER in it's symptoms, course, and treatment.

Because of the above, it would be ludicrous to say that all spitty babies have a "disease" called GER. In infancy, therefore, the challenge is defining the "border" between what constitutes "normal" vs. "abnormal" GER. Defining that boundary is difficult and controversial. There are no universally agreed upon standards. Generally, however, there are two practical ways of approaching the issue:

One approach is to call "abnormal" (and therefore treat) whatever degree of GER causes distress for the family and results in visits to the doctor. Two babies with the exact same amount of spitting might be treated differently depending on the perceptions of their parents. This is not very sensible or satisfying from a scientific standpoint, and has the downside of labeling as "abnormal" a child who may in fact be normal, but it is an easy & practical way to practice medicine because it usually satisfies families and does no harm.

A second approach, more scientifically rigorous & intellectually correct, is to define objective criteria for when GER becomes a problem for a baby.  Such criteria might include poor growth, bloody stools, more forceful vomiting, pneumonia, wheezing, or other evidence of aspiration (stomach contents spilling into the windpipe, thus effecting the lungs). It should also include failure to outgrow the problem by 12 months of age. The problem with this approach is that it leaves out pain. Pain is very hard to assess in the child under one year of age. In order to do so, we usually depend on parental perceptions. When pain is added to the list, however, the two approaches start to look functionally equivalent. Hence, the controversy.

Acquired GER is simply an LES which becomes loose (incompetent) after having previously been tight (competent). This can start at any age from childhood on into adulthood, and indeed is most common among adults middle aged and beyond. When a child appears to "acquire" GER at an early age (toddlerhood or early elementary school) one always suspects they really had congenital GER that was never "fully" outgrown but is only now being recognized. This may be particularly likely if the congenital GER was mild and therefore did not result in a lot of spitting up at the time. In any case, because they are treated similarly, the distinction does not really matter much.

The reasons why a previously competent LES becomes incompetent at an older age are not fully understood, although there are many contributing "risk factors" we know about. These include:

• Obesity
• Asthma (especially if poorly controlled)
• Smoking or Drinking
• Neurologic problems (especially those that decrease muscle tone or activity level)
• Stress/Emotional upset.

When several of these risk factors combine, it can "tip you over the edge" into GER. Any of them alone can make GER worse in someone who already has it.

Tests may be necessary when symptoms are subtle, atypical, or when other diagnoses are being considered as possibilities along with GER. The available tests include the following:

• pH probe - This is the best test, but it is rather invasive and unpleasant. A tube is placed down the nose into the esophagus. It has a sensor on the end that measures acidity. An x-ray is taken to make sure the end of the probe is in the right place (lower esophagus), and then the pH is recorded continuously for 12 or 24 hours. This is usually done in the hospital, especially in pediatrics, and so often requires elective hospital admission. We can thus see how often, to what degree, and for how long the lower esophagus becomes more acidic than it should be. A negative pH probe "rules out" GER more reliably than any other test, except in very young babies. Under 3 months of age an infant's stomach contents may not be acid enough to trigger the probe (or cause pain). A pH probe also allows us to do a better job of rating the severity of the GER than any other test.

• Endoscopy - This is the most invasive and unpleasant of the available procedures, and it is not fool-proof since someone with mild reflux, or reflux of recent onset, might still have an entirely normal endoscopy. Nonetheless, endoscopy has numerous advantages, especially in long-standing reflux. During an endoscopy, a doctor looks through a sort of "telescope" into the esophagus and stomach. This allows us to see the degree of inflammation in the lower esophagus as well as directly look at the LES to see if it is open or closed at rest. It also allows us to take biopsies (small specimens) of the lining of the stomach and esophagus looking for inflammation on a microscopic level and other abnormal cells. This can be important in judging the long term consequences of GER.

• Upper GI Series ("Barium swallow") - This is the oldest, simplest, least invasive, but also least accurate test. Unlike pH probe & endoscopy, which both require a GI specialist, the UGI can be ordered by any primary care physician. During this test, a child swallows a liquid which can be seen on x-ray. X-rays are then taken over several hours to see where the material goes. In particular, it can be seen whether, once in the stomach, it refluxes back up into the esophagus or not. The reason an UGI is not very accurate is that "false negative" and "false positive" results are both common. In other words, someone with a negative UGI might still have GER, and reflux seen during an UGI is not necessarily abnormal (especially in a baby) or the cause of the symptoms which led to the study. The major advantage of an UGI series is that, unlike a pH probe, it is able to diagnose other problems beyond GER. In particular, it is quite good at identifying anatomic problems like strictures, abnormal passageways, or an unusual intestinal "roadmap". While these things are rare, their symptoms can mimic GER and it is good to rule them out.

The bottom line is that there is no one diagnostic work-up or protocol that is necessary in all patients for the diagnosis of GER. The approach needs to be individualized for each patient by a physician who understands the disorder. In some cases, the diagnosis can be made clinically with no tests whatsoever. In other cases, all three of the above tests will be needed, or perhaps any subset thereof.

• Trigger Reduction: The most straightforward way to deal with GER is to remove the things which exacerbate it. This is particularly true in the older child or adult with the disorder. Bringing asthma under better control is a good idea. Attempts at reducing the stress in one’s life or dealing with psychological problems are always useful. Attempts to lose weight may be in order. Giving up alcohol, caffeine, or nicotine can also be helpful if these things exacerbate the disorder. Getting more sleep and exercise can also help.


• Thickened Feeds: In small babies, thickening the feeds is often a useful strategy to lessen GER. This is usually done by mixing rice cereal with formula - the typical amounts are 1-2 tsp. per four ounces of formula. Sometimes this requires enlarging the hole in the nipple to allow the formula to flow afterwards, but the thickened formula seems to stay in the stomach better with a slightly loose LES, especially when the GER is mild. Thickening feeds is more difficult to accomplish in a breast-fed baby. Sometimes an early introduction of spoon-fed cereal given just after a nursing can have the same effect.


• Anti-Reflux positioning: Pay attention to the position of the body, especially in the first 30-60 minutes after a meal. Pressure is more important than gravity. One wants to avoid positions that place undue pressure on the stomach. The worst position possible is an upright, sitting, hunched forward one (e.g. car seat, infant swing). Even though gravity would seem to be in the right orientation in that position, the flexed spine involved puts a great deal of pressure on the abdomen, squeezing it and making reflux quite likely. The best position is prone (face down) on a slight incline. Prone is better than supine (face up) because the back is more extended and, therefore, the abdomen is stretched and has less pressure. The incline provides a slight assistance with gravity. Sometime this position can be achieved lying on a parent's chest, but it is important to make sure the babies knees are not flexed up underneath them, which defeats the goal of extending the spine. Indeed, the "ultimate" anti-reflux position is the so-called "reflux hammock" (used more commonly in the days before medications were available). In this, the child was literally placed in a hammock in a prone position, producing hyperextension of the spine and taking virtually all pressure off the abdomen.


• Don't Rock-the-Boat: It may seem obvious, but is worth stating, that bouncing and jostling a baby with GER just after a feed is not a good idea.


• Burping: Many parents of babies with GER find that adhering to a particular burping routine seems to minimize the degree of spitting up. There is no science behind this and every baby is different. Indeed, some babies seem to spit up more if they are burped, rather than less. Trial and error will help a parent discover what sort of burping routine works best for their child, however.



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Antacids:

• Acid Neutralizers: The oldest kind of antacids are acid neutralizers. These drugs react chemically with the acid in the stomach to remove it's acidity. Most of these are available over-the-counter and include things like Tums, Rolaids, Maalox, Mylanta, AlkaSeltzer, Riopan, and many others. The active ingredients in these medicines usually include some combination of calcium carbonate, magnesium hydroxide, and aluminum hydroxide. Although they are good for short-term use, and are very effective with a rapid onset of action (usually within minutes), they are not ideal for long-term chronic treatment for several reasons. First, their effects tend to wear off rapidly so frequent dosing is necessary. Second, the calcium containing versions can lead to calcium over dosage and predispose to kidney stones. In contrast, the aluminum and magnesium hydroxide containing versions of these medications can actually leach calcium out of the bones, causing Osteoporosis, when used over the long term. Also, these medications can often cause constipation. Therefore, we generally recommend using acid neutralizers when only occasional treatment is needed. We avoid them for long-term chronic use. The other situation in which acid neutralizers can be helpful is as a "test". Because the onset of action is so rapid, it is quite useful to see if they are effective in treating abdominal pain when it is not clear what the cause of the abdominal pain is. A rapid response to an acid neutralizer is our tip-off that the pain has something to do with acid.

• Acid Blockers: In contrast to an acid neutralizer, acid blockers work by preventing acid from being produced in the first place. Most acid blockers work by blocking the histamine-2 "H2" receptor in the stomach. These are "antihistamines", but different than anti-allergy medicines (which block the H1 receptor).  A few acid blockers work by a different mechanism - blocking the uptake of hydrogen into stomach lining cells (a necessary first step in acid formation).  H2 blockers include such things as Zantac, Tagamet, and Pepcid.  Many of these are now available in over-the counter forms, although liquid forms in pediatric doses are still prescription only.  The only hydrogen uptake blocker commonly used in children is Prilosec, a prescription medicine (adult medicines in this class include also Prevacid, Protonix, and Nexium - which may sometimes be used in teenagers).  All of the acid blockers tend to be well-tolerated, with very few side-effects. None of them have any particular medical advantage over another, although Zantac is the one given to children the most commonly over the years. These are generally considered to be the acid-treatment of choice if long-term chronic therapy is needed.

Pro-motility Agents: Pro-motility agents act by helping things move through the stomach more quickly. They stimulate the stomach to do it’s work faster.  Some also affect the LES, increasing it's tone, thus helping reflux directly. There is are really only one of these available - Reglan (Metaclopromide).  Not long ago another pro-motility drug, Propulsid (Cisapride) was removed from the market because of it's tendency to interact with many other commonly prescribed medicines.   Reglan is a much older medicine than Propulsid, and does not share it's drug interactions.  Unfortunately Reglan can have many side effects and does not work as well as Propulsid did.  The worst side effect of Reglan can be a tendency to produce severe muscle spasms that can come on quite suddenly. This can be quite frightening and really necessitates stopping the medication. Fortunately, however, this side effect can be acutely treated using Benadryl.  Because of these problems we tend to use pro-motility agents as a second line intervention, only after acid controlling drugs alone have failed.  This may change in the future, however, if a pro-motility agent as effective as Cisapride was but without it's drug-interactions or the side effects of Reglan can be found.

SURGICAL TREATMENT:

When all else fails, there is an operation to correct GER. The decision to take this step is never taken lightly. It is major surgery with significant risks, and it is not always 100% effective. It is usually helpful to some degree, however, in cases unresponsive to medications. The surgical procedure is called a fundoplication or sometimes a "Nissen" - after the surgeon who originally designed the procedure. It involves making an incision in the abdomen and taking the upper part of the stomach and essentially wrapping it around itself to tighten the area.

One of the biggest problems with a Nissen is that the LES can end up so tight that food cannot pass through from above in normal swallowing. The child may therefore be unable to eat by mouth and require a tube through the abdomen straight into the stomach. Sometimes this result is actually desired (e.g. a neurologically impaired child who does not swallow properly to begin with, and therefore chokes and gets recurrent pneumonias while eating). A surgeon experienced in this procedure can try to control exactly how tight he makes it during the operation - allowing for oral feeding when that is desired and not allowing for it when it is not desired. This is an inexact science, however, and things do not always turn out as planned.

Another unfortunate consequence of fundoplication is retching. The child cannot vomit. Thus, if they get a stomach flu that would normally cause vomiting and diarrhea, after a fundoplication you may have retching and diarrhea instead.

It is possible to reverse a fundoplication, although this is another difficult operation. Overall, fundoplication should be considered a last resort. It should be done only when all other treatments have failed, and only when the GER is severe enough that it cannot be tolerated - for instance if a child is not gaining weight, or is having recurrent aspiration pneumonias. Children whose GER is associated with neurologic damage meet the above criteria much more often than normal children do.

Asthma: The relationship between Asthma & GER can be maddening - to parents, patients, and doctors alike. That's because it's a two-way street - both diseases exacerbate each other. It can be very hard to know what came first, the chicken or the egg (the GER or the Asthma). Here's how it works: the lower esophagus has nerve endings which, when irritated, send a signal to the brain which in turn reflexively signals the lungs. The lungs respond by constricting airways and coughing. This reflex probably evolved as protection against choking & aspiration, but coughing and constricted airways are the hallmarks of asthma. An asthmatic lung (one with constricted airways) expands. Air gets trapped inside and it hyper-inflates to a bigger size. This pushes down on the diaphragm, increases pressure in the abdomen, and makes any tendency towards GER worse. Thus, one question which "haunts" the care of any asthmatic is whether "silent" GER could be a contributing factor, and whenever cough accompanies other symptoms of GER the question of whether it is "just" the GER or whether there is a separate, independent component of asthma must also be considered. The only sure thing is that when both are known to coexist in the same patient, it is important to treat both simultaneously in order to control either one.

Hiatal Hernia: It used to be thought that GER and Hiatal Hernia were the same. We now know this is false. Hiatal Hernia is the term we use when a part of the stomach is in the chest cavity, rather than the abdomen, and what actually passes through the diaphragm is the upper stomach rather than the lower esophagus. This can be seen on x-ray. It was once thought that everyone with a Hiatal Hernia must have GER and vice-versa. We now know that the two findings are totally separate and neither one causes the other. It is possible to have both, but lots of people with Hiatal Hernias at all ages do not have symptoms of GER, and lots of people with GER do not have Hiatal Hernias. Hiatal Hernia in the absence of GER, however, is probably a variant of normal and does not need treatment.

Peptic Ulcer Disease (PUD): Ulcers are discrete "sores" on the stomach or intestinal lining. They are painful and can become progressively deeper. When deep enough, they can bleed severely. Ulcers usually happen in the stomach or in the first segment of the small intestine, just beyond the stomach. They are thought to occur as a result of excess stomach acid. In that way, they are related to GER, but in GER you have an incompetent LES allowing the acid to into the esophagus, which isn't built to handle it. In contrast, in PUD the LES works fine, but the stomach & intestinal lining (which should be able to) can't handle the amount or strength of acid being produced. Fortunately, PUD rarely happens in childhood. When it does, it tends to be in late adolescence and in families with a very strong history of the same. Therefore, most of the time, this is a disease we do not have to worry about as pediatricians.

Esophagitis is inflammation in the esophagus. GER is the most common cause, but not the only one. Esophagitis happens in children and adults of all ages, and can come from various infections, food allergies, chemical irritants, foreign bodies, and sometimes just appears for no reason at all. A very inflamed esophagus, regardless of the cause, can bleed, develop strictures (narrowing), or eventually lead to cancer. Blood in the stool is a sign of esophagitis, but in an adult or older child this needs to be differentiated from ulcers & other causes of intestinal bleeding. If esophagitis is due to GER, this should make treatment more urgent, and at times itself might be a reason to consider surgical options if medications are not working.

Angina: Finally, much later in life GER must also be distinguished from heart disease. Sometimes the chest pain of cardiac angina (inadequate blood flow of the heart or blocked coronary arteries which can lead to heart attack) can be misinterpreted as heartburn as well. Luckily, this is not a concern where children are concerned because children don’t get angina or heart attacks.