How many times have you or your doctors assumed that certain symptoms are just part of having CF? When I was younger, it seemed nothing was a simple cold or stomach bug. If I was sick, it was CF-related. While it's true that, to varying degrees, we CF patients share a lot of similarities - distinctive voices, chronic coughs, clubbed fingers, charming personalities - not everything can be attributed to CF. The older I get the more I realize things usually aren't as simple as they seem. The role gastroesophageal reflux disease (GERD) has played in my life is proof.
"Diagnosis and the road to relief may be long…"
Complex in its many manifestations, GERD in a person with CF (PWCF) is especially precarious. Diagnosis and the road to relief may be long because reflux not only causes heartburn, but symptoms similar to sinusitis, asthma, allergies, gallstones and other ailments as well.
When the digestive system works properly, swallowed food passes through the esophagus through a one-way valve called the lower esophageal sphincter (LES), the opening of the stomach. Normally, the LES opens when you swallow and then closes after food has entered the stomach. In the stomach, acids and enzymes break down the food before it moves into the intestines. Reflux occurs when the LES malfunctions and allows partially digested stomach contents and acid to come back up into the esophagus.
Not Just Heartburn
"On rare occasions the damage can be severe…"
A little reflux is normal and usually goes unnoticed. However, if it occurs often or if the acid remains in the esophagus for very long, the mucous membranes of the esophagus become irritated and inflamed. The result is heartburn, or esophagitis (inflammation of the esophagus). On rare occasions the damage can be severe enough to cause bleeding, or a narrowed area of scar tissue, called a stricture, within the esophagus can make breathing very difficult.
GERD may cause pulmonary problems in one of 2 ways: Refluxed material may reach the back of the throat and actually be aspirated into the lungs; or, nerves in the esophagus become so irritated from the stomach acid, they send signals to the bronchial tubes in the lungs to get narrower, causing breathing problems similar to asthma.
GERD and CF
Studies show that by the age of six, 50 % of CF patients have GERD. That's not surprising because the antidotes used to treat CF may, in fact, aggravate reflux. For example, many of us have been on theophylline, a bronchodilator, which can relax the LES. Studies also show that postural drainage techniques - especially positions requiring us to lie upside down - exacerbate GERD. Furthermore, the mechanical influence of a depressed diaphragm caused by hyperinflation (which is common in CF), along with the increased abdominal pressure with chronic coughing, may contribute to GERD.
"…no wonder our internal anatomy shifts in response."
A cough can generate 100 cm/H2O of pressure in the chest, raising intra-abdominal pressure. This puts quite a strain on the stomach and esophagus. It's no wonder our internal anatomy shifts in response.
My Personal Experience
At its worst, GERD has meant more than a year of sleepless nights, an incessant hacking cough, a sore throat and bouts of laryngitis. It also meant many a wasted meal. Inevitably, my husband would take me to my favorite restaurant, I would enjoy my favorite dish, and before it was time for dessert a coughing spasm would overcome me. By the time we got home I would be vomiting. In an hour I'd be hungry again.
"…the sore throats were attributed to the cough…"
I slept - or at least tried to - sitting up on the couch in my living room, hacking away. In the morning I would be exhausted, my chest tight and sore, my voice hoarse, my throat on fire. At first it seemed to be out-of-control asthma, and the sore throats were attributed to the cough, a recurring virus, and/or postnasal drip. My allergist suggested I was only aggravating the situation by sleeping on the couch: Where better for dust mites to thrive than on an old couch?
"…difficult to communicate, especially on the phone."
Professionally it was disruptive: First, the exhaustion caused from not sleeping obviously made it very difficult to get up in the morning. I felt unproductive and had a hard time concentrating. Second, the recurring bouts of laryngitis and severe sore throats posed a challenge: While having a distinctive voice didn't hurt me as a public relations practitioner, laryngitis made it difficult to communicate, especially on the phone. Fortunately, my employer was flexible and let me set my hours - I could work late, early, on weekends, whenever. Unfortunately, my unpredictable hours made supervising people impossible. I now work as an independent contractor from home.
"…my body was rebelling against everything…"
Antibiotics didn't seem to improve the situation because although my throat was raw and red, there were no white patches. My cough was powerful but unproductive. While it was reassuring that it wasn't pneumonia or worse, it was frustrating. I was on high doses of Prednisone in an effort to ease the wheezing. Although it seemed to work at first for a day or two, in time the burning in my stomach would just get worse and the coughing would return. It felt as if my body was rebelling against everything meant to make it well. In time, it became instinctively clear that the acid churning away inside must have been wreaking havoc on all my systems. The more medicine I took, the more my stomach churned and more irritated my throat and esophagus felt. Fortunately, my team of CF doctors and allergist were becoming more aware of asthma-induced reflux. Finally we decided to see how bad my reflux really was, and whether or not taming it might bring some relief.
Although an upper GI revealed reflux and a hiatal hernia in the early 90's, my other symptoms were never attributed specifically to GERD until 1998. In fact, I had been taking Zantac, Pepcid, etc for years. I was going through a bottle of Mylanta every few days.
Today there are a plethora of new remedies designed to treat GERD symptoms. The following categories represent typical medicines that might be recommended for you:
· Antacids - help to neutralize acid quickly, but offer no long-term benefits. To control acid one would take antacids at least 7 times a day. Be sure to ask your pharmacist if and when it can be combined with other medications.
· H2 antagonists - including cimetidines such as Tagamet, Zantac, Pepcid and Axid, suppress acid in the stomach.
· Proton pump inhibitors - significantly block stomach acid production and are used for the short-term. They include Prilosec (omeprazole), Prevacid (lansoprazole), Aciphex (rabeprazole), Protonix (pantoprazole) and Nexium (esoopmprozole).
· Medications designed to tighten the LES - including Propulsid (cisapride) which is no longer on the market, and Motilium (domperodone), which is not available in the U.S. These help strengthen the squeezing action of the esophagus and tighten the LES, presumably to keep acid where it belongs. Reglan (metoclopramide) is sometimes used as well and has the added benefit of making the stomach empty faster.
While these remedies help reduce heartburn, they don't do much to help pulmonary symptoms.
If after a few months your heartburn is better, other culprits such as sinusitis and allergies have been addressed, and you still have pulmonary symptoms, your medical team may recommend diagnostic tests to determine the severity your reflux and whether or not it could be a contributing factor to your symptoms.
To help determine what course of action will provide the best results, one or more of the following tests may be performed:
· Barium upper GI - While drinking thick, chalk-like barium, an x-ray monitors the barium dye as it travels to your stomach, capturing any reflux action on film. The x-ray will also reveal whether you have a hiatal hernia. The test does not hurt. (One technician actually used chocolate syrup to make the barium more palatable!)
· Esophageal endoscopy - A lubricated probe is inserted down your throat to the stomach. You may be given a sedative and your throat may be sprayed with an anesthetic to make it easier to swallow the probe, which is a small, flexible tube. You may have a sore throat afterwards.
· Esophageal manometry - A flexible tube is guided through your nose and throat into your stomach. Water is passed through the tube and measurements are taken as the tube is slowly and gradually withdrawn from your stomach into the esophagus. It measures the muscle tone of your lower esophageal sphincter and helps your doctor tailor your surgery according to your anatomy. It takes 30 minutes and you may have a sore throat afterwards.
"This is the gold standard for demonstrating the act of reflux."
· 24 hour pH monitoring - A thin, acid-measuring probe is placed through your nose down into your esophagus for up to 24 hours to record how much acid washes back from your stomach into your esophagus. You will be sent home with the tube and a small monitor, which can be attached to your hip. This is the gold standard for demonstrating the act of reflux. It is especially helpful if there is any doubt that reflux is present, or when your main complaint isn't heartburn but unexplained coughing, sore throat, etc.
· Ultrasound - People with CF often have gallstones so a painless ultrasound may be performed to rule out gallbladder problems. High frequency sound waves create images on a screen and your abdomen is scanned with a small wand.
I'd already had several upper GIs so we knew I had a hiatal hernia and reflux. An ultrasound ruled out gallstones (at that time - they came a year later!) I then went for the esophageal manometry and the 24-hour pH-monitoring test.
Tests revealed that surgery was a viable option for me. To stop reflux, surgery involves wrapping the upper part (fundus) of the stomach around the bottom of the esophagus to strengthen the LES, essentially creating a new valve in the stomach that prevents acid reflux into the esophagus.
Years ago, the traditional way of performing this surgery was through an 'open' incision. Thankfully technological advances have made a laparoscopic version - called a Nissen fundoplicaton - possible. Studies show that the laparoscopic fundoplication is as effective as the open surgery, which is great news for CF patients. Minimally invasive, it means that instead of one long incision, patients have four or five smaller incisions (ranging in size from 5 mm to 1 cm) through which a laparoscopic camera and surgical instruments are inserted. These smaller holes heal easier, cause less pain and mean you can get back to your regular activities much faster.
Immediately after surgery you will be very sore, as if you have been doing hundreds of sit-ups or were struck about the abdomen. You may be surprised that your shoulders hurt more than your stomach! This is from the carbon dioxide gas used to inflate your stomach to create more space for the surgeon to work and see. It can settle in your shoulders and cause discomfort. The best thing to relieve it is to get up and move around. I was in the hospital 2 nights and out of work for 2 weeks.
Remember that it takes 4 - 6 weeks to heal fully (if not on steroids). It's important not to lift heavy objects while you heal, and to brace yourself with a pillow when you cough. There is a coughing technique called 'huff' coughing that might be worth learning prior to your surgery - it seems a lot of the younger people know how and I'll bet it would be helpful during recovery.
The results come quick: I was amazed when I could sleep through the night - flat! The 'presence' and 'awareness' in my middle was lifted. The wheezing was calmer. My cough was less forceful and the retching waned.
There is a special diet your doctor will prescribe to gradually introduce solid foods back into your diet. It takes some time, and varies patient to patient. Since nutrition is so critical to CF patients, this is obviously a critical component to recovery. Your esophagus will be a little swollen for some time so it's important to go easy - it took me quite a while to be able to swallow bread and meat, never mind sandwiches. You may feel fuller faster, so make sure you eat more often to get your calories.
Due to chronic coughing, CF patients are at slightly greater risk of undoing the wrap sooner than healthier folks. When the doctor tells you not to lift heavy objects or do too much, listen!
Three Years Later
Last May I had my gall bladder removed. At that time my surgeon advised me that my wrap had loosened a little and my hiatal hernia had slipped some. It looks like I'll need surgery again someday. Will I do it? Probably. Since my surgery, stronger materials have been developed which reinforce the wrap even better. New non-surgical endoscopic techniques are also being developed to tighten the LES. I can't wait!
Tips to Relieve Reflux:
· Don't smoke or drink alcoholic beverages. They increase stomach acid production and cause irritation. Alcohol weakens LES.
· Avoid caffeine and chocolate. Caffeine is a muscle relaxant and weakens LES tone.
· Avoid carbonated drinks, citrus, onions, tomatoes, fatty and fried foods, peppermint and spicy foods.
· Doctors recommend raising the head of your bed 4 - 6 inches, either by raising the mattress or legs at the end of your bed. It's not enough to prop up your pillows, or use twice as many - you'll end up bending more at the waist and risk actually increasing your reflux. You must physically lift the head of your bed. I've seen 'bed-lifters' in store catalogs - they're designed to create storage space under one's bed, but I bet they'd do the trick.
· Wait 2 - 4 hours after eating before bending over, lying down or going to sleep.
· Stick to upright postural draining positions.
· Let your doctor know you have reflux and ask if any medications you are taking might be contributing to it (calcium channel blockers, progesterone and some tranquilizing medications).
· Wear loose clothing.
· Eat smaller meals more frequently.
Please speak to your doctor if you are suffering from any symptoms discussed in this article. It is important that a CORRECT diagnosis is secured before making any changes to your lifestyle, should you suspect GERD (i.e. changes in diet or physio/physical therapy).
Our thanks to Dawn for sharing her experiences and giving us an introduction to Gastroesophageal Reflux Disease. This article was written in collaboration with, and proofread by her CF specialist team.