What is Cystic Fibrosis?
Cystic fibrosis (CF) is a disease, passed down from parents or other family members, affecting cells that produce mucus in the lungs or the digestive system. CF causes mucus or fluids to become thicker than normal inside the lungs and gut. This causes body passageways to become plugged up, causing damage to these organs. In addition, CF can have an effect on gastrointestinal (GI) motility, causing the stomach, small bowel and colon to slow down. This is called GI dysmotility.
Overlap of Cystic Fibrosis (CF) and Gastroparesis (GP)
Research has shown that about 38% of people with CF also have GP. According to The National Institutes Diabetes and Digestive and Kidney Diseases (NIDDK), about 10 men and 38 women of every 100,000 people have GP. The impact that GP has on a person depends on multiple factors which include gender, body mass, symptoms, and the degree of stomach delay.
Treatment Considerations for Gastroparesis with Cystic Fibrosis
People with CF and GP will need a carefully chosen treatment plan. This makes it more important for patients to talk about symptoms and concerns with their healthcare provider. Treatment options and symptom management should be unique to each person. A diagnosis of GP can have a significant impact on the treatment and care of CF patients. Symptoms related to GP and CF can lessen quality of life and nutrient intake. Severe GP symptoms can prevent some people from taking drugs by mouth and therefore a feeding tube may be needed in some patients. People with even milder GP symptoms may not properly absorb drugs because of the slowed movement.
Drugs used to treat the pain or allergy symptoms of CF may negatively affect the GI tract and delay stomach emptying. This is especially harmful for people diagnosed with GP. Patients with CF may be prescribed two different types of drugs that can slow down the rate the stomach empties. These are:
- Opiates – This is a class of drugs that are used to treat pain. They are heavily controlled prescription drugs including oxycodone, hydrocodone, and fentanyl.
- Anticholinergics – These are drugs used to treat allergies. One example of this type of medication is diphenhydramine.
Treatment of Gastroparesis
Treatment of GP in CF is not any different from treatment of GP due to other conditions. Treatment of GP and CF will vary from person to person. The treatment options listed below may be used by all people with GP, including those who also have CF. Careful discussion between patients and their healthcare providers will help find the treatment options that are best for each patient.
- Prokinetics – These drugs may speed up the movement of food through the GI tract. This occurs by increasing the strength and number of muscle contractions. This type of drug may also be referred to as a pro-motility agent.
- Metoclopramide – The only Food and Drug Administration (FDA) approved treatment for GP is metoclopramide. Metoclopramide is now available in three forms, a pill to swallow, nasal spray, and a shot (given in the muscle). Drugs approved by the FDA have been deemed safe, with benefits outweighing possible risks. This is done after reviewing studies and tests that have been done on a drug. However, this drug also has a black box warning which highlights risk of tremors and what is called tardive dyskinesia- uncontrolled muscle movements of the face and upper limbs. This is similar to that seen in a patient with Parkinson’s disease. For this reason, patients over age of 65 cannot be prescribed this medication for longer than 3 months.
- Ghrelin Agonists – This drug mimics ghrelin, a natural compound found in the stomach that improves the movement of contents through the stomach. Ghrelin agonists are currently being researched and are not yet available on the market. Current research has shown that these drugs can cause headaches and an increase in appetite.
- Erythromycin and Azithromycin – Both of these can speed up the movement of food through the GI tract. These drugs are not approved by the FDA for use with GP but are used in adults and pediatric patients with these conditions. As they are both antibiotics, caution should be used in long term use to prevent antibiotic resistance. Both drugs also can cause some cardiac arrythmias. An arrythmia occurs when the heart beats too fast, too slow, or out of rhythm. However, many patients with CF are already on azithromycin for prevention of pneumonias.
- Domperidone – A drug that may improve stomach emptying in some patients. This drug is not available in the U.S., unless approved for use through the FDA’s expanded access program and application process. There are possible serious side effects of this drug.
- Antiemetics – These are drugs that block neurotransmitters in the body. This works to ease nausea and vomiting. Neurotransmitters are chemicals that travel through the nervous system and impact body functions. The brain, spinal cord, and nerves make up the nervous system which is a target for many neurotransmitters. These drugs are currently not approved by the FDA for GP treatment, unless short-term use after surgery. A list of these medications is in the table below.
- Proton Pump Inhibitors (PPI’s) – These drugs may be recommended for those who have heartburn or who regurgitate their food or acid. This is called gastroesophageal reflux disease (GERD). PPI’s help by affecting the acid making glands within the stomach to reduce the amount of acid they produce. By lowering the amount of acid, food is digested slower too, causing the stomach to empty slower. However, for patients with GERD these are necessary medications.
- Bethanechol – This drug works on the lower esophageal sphincter (LES), helping it to contract. The LES is located at the bottom of the esophagus and closes to prevent stomach contents from coming back up into the esophagus. Possible side effects of this drug include diarrhea, abdominal cramping, and flushing (redness of the face and neck).
- Botulinum Toxin – Also commonly known as botox, this treatment is injected into the pylorus of the stomach. The pylorus is the last part of the stomach that connects to the beginning of the small intestine. Although this substance is naturally a harmful toxin, it has many medical uses that are scientifically proven as safe. This toxin blocks the release of the chemical acetylcholine from nerves. This chemical causes smooth muscles, which make up the stomach, to contact. Preventing the stomach from contracting closed can allow food to pass into the stomach easier for those with GP. The only common side effect of this treatment is swelling as the injection site.
- Dietary management – Healthcare providers may recommend eating small meals as well as a low fiber or a low residue diet which are more easily digested in patients with GP. Low residue diets leave less contents behind in the GI tract and result in less or smaller bowel movements.
Additionally, although most CF patients need to be on a high fat diet given their pancreas issues and inability to absorb fat and many related vitamins, a dietician can help patients with suggestions for healthy fats that are more readily absorbed and less likely to slow down the stomach. A registered dietitian who specializes in GI illnesses can help create an optimal diet for each person.
- Lifestyle modifications – If physically possible, it may be helpful to modify some habits after meals. Gentle physical activity such as walking after a meal may help with symptoms. Your healthcare provider may also recommend you avoid lying down for 2 hours after finishing a meal.
- Feeding tube – In severe cases of GP a tube may be inserted in the stomach or the small bowel either by endoscopy, surgery or by an interventional radiologist. A 6 to 12-inch tube comes out of the stomach or small intestine to allow foods via a liquid shake and pump as well as your medications to be fed through the tube. This is managed with the support of a doctor and dietitian.
- Parenteral Nutrition – In severe GP cases, when feeding by mouth and tube feeding is not possible, an intravenous (IV) catheter may be placed to provide nutrition while avoiding the GI tract. This is usually placed in the arm and is called a PICC line. An IV catheter is a small flexible tube that goes into a vein. Formula placed into the catheter is specially made to provide nutrients that the body does not need to digest. This allows nutrients to go directly into the blood and be absorbed by the body.
- Other treatment options for GP are in development and may become available in the near future.
Gastroparesis and Cystic Fibrosis occur together can lead to malnutrition and impaired quality of life. All GI symptoms and concerns must be talked about with a healthcare provider so that the nutrition concerns are addressed and so that patients’ daily living can improve. Where a delay in stomach emptying is found, the help of a dietitian should be sought quickly to help improve patient’s nutrition. Especially in CF, the risk of pneumonia and worsening lung disease escalates with poor nutrition therefore this is of main concern to pulmonologists who are the lung doctors usually taking care of these patients.
A strong multidisciplinary team approach is vital to taking care of a CF patient. As research into GP and CF continues, we will gain better understanding of these conditions and improve our patients’ outcomes. The ultimate goal is to develop a cure for GP and CF. All research toward that cure should also improve patients’ symptoms and enhance their quality of life.
Adapted from IFFGD publication #410 Cystic Fibrosis and Gastric Motility Effects by By: Baharak Moshiree MD MSc, Atrium Health, Carolinas HealthCare System, Digestive Health-Morehead Medical Plaza; Marissa Lombardi, Program Coordinator, IFFGD