CF 101: GI Disease in Cystic Fibrosis

This is a continuation of my “CF 101” series. The purpose of the “CF 101” series is to explain and educate people about Cystic Fibrosis using every day language and personal experience. Please click the links for my previous posts in this series, CF 101: Lung Disease in Cystic Fibrosis and the CF 101: CF Diagnosis.

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Let’s look a bit closer at the second major issue for CF patients – the GI system.

GastroIntestinal (GI) Disease

You know those growth charts that pediatricians use to see how a child is growing and gaining weight against the expected healthy ranges? Most children with CF typically do not grow the way otherwise healthier children do, mostly because their Pancreas malfunctions, making it very difficult for the body to digest food and absorb nutrients. Adding lung disease to the equation further diminishes the fuel available as the body uses much more energy to simply breathe.

As a child, my body never paid any attention to those growth charts. I was not only skinny, I was also very short. I don’t recall my height until I was a teenager – I hit 5 feet tall and 100 pounds the summer before high school, but I was always the shortest in my class. Often, as Catholic school teachers did during the time, our class organized us by height for everything – setting up the rows of desks, taking the brief walk from the school building to the church, and seating every class photo the same way – all shrimps front and center.

Yep, I was always shrimpy!

Yep, I was always shrimpy!

We wore uniforms to school, and for many 2-3 year periods, I didn’t grow enough to warrant going up in size. I remained in the smallest size jumper for the first few years of school. I was lucky in the sense that I had two older sisters (twins), so I had plenty of hand-me-downs once I did sprout a few inches.  One day a year – Picture Day – we got to wear “regular clothes” to school, and I remember wearing the same dress two years in a row – kindergarten and first grade (see first grade photo above).

The Pancreas

Historically, CF was originally called Cystic Fibrosis of the Pancreas. Next to Lung disease, Gastro-Intestinal (GI) disease is the most severe complication of CF, and most of those problems are caused by Pancreatic disease.

GI issues begin in the Pancreas, which is responsible for creating and excreting digestive enzymes and insulin. CF’s effect on chloride channels in all cells – mucous – blocks the pancreatic ducts, preventing both enzymes and insulin from doing their jobs. Essentially, the trapped enzymes digest the pancreas from the inside, turning the organ into fatty tissue and over time, rendering it practically useless.

Pancreatic Enzymes: I take seven (7) with meals, 2-3 with snacks.

Pancreatic Enzymes: I take seven (7) with meals, 2-3 with snacks.

Eighty-five percent of CF patients are Pancreatic Insufficient (PI). What does that mean for patients?

Three (3) month supply of Pancreatic Enzymes. Retail: $9,246.49.

Three (3) month supply of Pancreatic Enzymes. Retail: $9,246.49.

Two major conditions arise due to a diseased pancreas – Diabetes and Malabsorption.

  1. Diabetes  – CF Related Diabetes is more similar to TYPE I, and most patients end up needing insulin. As patients live longer with CF, more and more patients are developing Diabetes. Though some CF patients develop CFRD as young children, its onset more typically occurs in adulthood (18+).
  2. Malabsorption – The absence of digestive enzymes requires Pancreatic Insufficient patients to take supplemental Pancreatic Enzymes with each meal. As we grow and age, our enzyme requirements may change, but don’t go away. In fact, as the pancreas becomes more and more damaged, the stronger strength enzymes we often need. A damaged pancreas leads to a host of other health issues, which I will cover below.

Diabetes

CF-Related Diabetes (CFRD) is not Type I or Type II, but closer to TYPE I in that nearly all CFRD patients need insulin. As our pancreas literally digests itself from the inside, the cells responsible for creating and excreting insulin stop working. As patients live longer with CF, more and more patients are developing Diabetes. According to the CF Foundation,“CFRD is found in 35 percent of adults aged 20 to 29 and 43 percent for those over 30 years old.” Though some CF patients develop CFRD as young children, its onset more typically occurs in adulthood (18+).

Insulin and Blood Glucose Meter.

Insulin and Blood Glucose Meter.

I developed diabetes around the age of 30, and it was a complete shock to me. Managing healthy blood sugar levels in CFRD is difficult due to slow or poor digestion of food, and medications (the oft-prescribed Steroids for breathing difficulty can increase the blood sugar) . It takes a lot of work to prevent blood glucose levels not only from going too high, but preventing “crashes” – episodes when blood sugar is too low.

A low blood sugar episode, a.k.a. Hypoglycemia, is NOT like the scene with Julia Roberts in “Steal Magnolias.”

When I crash, it starts out feeling like an anxiety attack. I start to feel shaky, uncoordinated. The perimeter of everything I see is dimmed, and sounds are muffled. I’ve only completely “passed out” once – coincidentally, I was in the hospital, a nurse was taking blood at my bedside, and I just lost consciousness. I’ve come close probably a dozen more times in my life, and have had countless low blood sugar episodes. Sometimes, no matter what you do, this will happen – I try to carry hard candies for such occasions.

Complications of hyerglycemia (too-high blood sugar) include:

  • Diabetic neuropathy – nerve damage that can occur anywhere. Loss of sensation can lead to unnoticiced injury, especially to the foot, which when unchecked, can lead to infection, gangrene, or sepsis.
  • Peripheral Arterial Disease – decrease of blood flow to foot. May result in amputation of affected foot/feet.
  • Poor circulatation of blood can cause reduce your body’s ability to heal wounds.
  • Poor blood glucose control can contribute to heart disease, and a higher incidence of Heart Attack or Stroke, when combined with other risk factors.
  • Kidney damage from poor glucose control.
  • Eye disease:
    • Glaucoma – increase in fluid pressure causing damage.
    • Cataracts – clouding of the eye’s lens
    • Diabetic Retinopathy  – increased fluid pressure in the eye damages the optic nerve.
    • Blindness.

Malabsorption

When we eat, the pancreas is responsible for releasing enzymes and insulin to digest and utilize the nutrients, calories, carbohydrates, fats, and proteins in our food. For the 85% of CF patients with Pancreatic Insufficiency (PI), even with the aid of supplemental enzymes, we often don’t digest all of what we eat. I have listed many of the conditions that result from PI:

Inability to gain/keep weight – Many CF patients with PI have chronic or occasional problems maintaining a healthy weight, despite increased calorie intake. Doctors have found a strong correlation between BMI (body-mass index) and lung health, so CF patients are encouraged to take in much more food and calories in any way possible. Treatments are based on the causes:

    • Not enough enzymes – even if we’re eating the calories, if our bodies aren’t digesting them, their useless. Doctors may test the level of fat in stool to assess if the patient needs to take more Digestive Enzymes.
    • When the body has to work harder to get enough Oxygen, it uses up more “fuel”. Oxygen supplementation helps decrease the amount of work the body has to do to acquire the necessary oxygen.
    • Lack of appetite, often due to acute lung infections, digestive issues, or medication side effects. Some patients drink high-calorie shakes to supplement their food intake, require IV nutrition in the hospital, or even rely on a feeding tube.
    • Nausea/vomiting due to coughing, medication side effects, reflux, other GI issues. Sometimes changing the timing of medications, meals and Airway Clearance can help prevent nausea episodes. There are also a number of different types of medications – OTC and prescription –  that help relieve nausea once it’s already happened.

Nutritional deficiencies – CF patients don’t digest fat well, and as a result, are often deficient in the fat-soluble vitamins A, D, E and K. It’s vital to replace those vitamins for various reasons:

  • Deficiency in Vitamin A can cause eye disease, skin conditions and impairment of the immune system.
  • Deficiency in Vitamin D can cause bone mass depletion, cancer, increased risk of death from cardiovascular disease, hair loss, and  cognitive problems.
  • Deficiency in Vitamin E can lead to neuromuscular changes, skin and hair problems, anemia.
  • Deficiency in Vitamin K is linked to poor blood clotting, which can become life-threatening.
I take 15,000 IU of Vitamin D3 and 1200 IU of Vitamin E daily.

I take 15,000 IU of Vitamin D3 and 1200 IU of Vitamin E daily.

The Intestines

Once food passes into the stomach where it meets our Pancreatic Enzymes – whether or not we have them, take supplements, or take enough – not all food is digested well. This leads to a variety of “stomach” symptoms and conditions.

Stomach pain and cramping – Often dubbed “CF Tummy,” the stomach pain and cramping is similar to what might happen when someone eats a greasy, fatty meal. The pain is often diffuse – all over – and very little makes it feel better.Over-the-counter medications might help, but sometimes there’s often nothing we can do, but wait for it to subside.

Gas – Gas is a common CF complaint.  Otherwise healthy people typically have some each day, but CF patients experience much more frequent, noxious, room-clearing gas. Again, typical OTC (over the counter) medications like Gas-X or Pepto Bismol usually don’t help, because they don’t address the root cause.

Greasy, bulky stools – Many CF patients’ stools contain a lot of undigested fat. Stools often frequent (5 or more times per day), voluminous, float, and are foul-smelling.

Constipation – the combination of undigested food, excess mucous in the digestive tract, a slow digestive system due to lack of enzymes, dehydration, and inflammation can create thickened stool that is very difficult to pass. Two more serious conditions can arise that go beyond simple constipation:

  • Intestinal Obstruction – partial or complete blockages that, if not treated, can be life-threatening. Treatments for an acute obstruction include high-dose traditional oral laxatives (sometimes delivered via NasoGatric tube), high-volume enemas, medications to bring water into the intestines (see next), and possibly surgery. CF patients sometimes need a “bowel clean out” during which many treatments can be given in a short period.
  • Distal Intestinal Obstruction Syndrome – chronic formation of intestinal obstructions. Many CF patients routinely take medications that bring more fluid into the digestive tract to help move stool. Unlike traditional “laxatives,” these medications – such as Lactulose or Miralax – don’t work like traditional laxatives, which stimulate the bowels to contract and usually result in explosive “relief”. The preferred medications for CF patients provide much gentler relief.
  • Meconium Illeus – Meconium is the first stool of a newborn. In some newborns with CF, that stool is too thick and causes a blockage in the Ileum (a part of the small intestine). Surgery is a common treatment for MI.

Diarrhea -It might sound contradictory, but CF patients often experience Diarrhea as well as Constipation. Most people have occasional bouts of loose, watery stools, but CF patients sometimes experience it at much more frequent and severe levels. Causes include malabsorption, medication side effects (antibiotics can kill off the gut’s “good bacteria”), an occurrence that can quickly turn dangerous, causing dehydration, electrolyte imbalance (which can cause minor problems like muscle cramping or catastrophic events like heart failure), loss of important nutrients.

The Gall Bladder

Gall Bladder disease in Cystic Fibrosis happens due to the thickened bile, leading to scarring, gallstones, and requiring surgical removal of the gall bladder. The hallmark symptom is upper right quadrant abdominal pain after eating fatty meals. I had my gall bladder removed in 2002 after spending six days in the hospital with a severe acute gall bladder attack. The level of relief was so great and overwhelming that post-surgical pain – where they cut – barely registered.

The Liver

Liver failure is the #2 cause of death due to Cystic Fibrosis. There are generally no clinical symptoms of liver disease until the damage is quite severe, but bleeding and jaundice can indicate liver disease at any time.

Scarring – an increase in thickness of bile can clog and damage the bile ducts.

Cirrosis – as the liver begins to scar, it replaces healthy liver tissue, eventually resulting in liver failure.

Portal Hypertension – an increase in the blood pressure within the portal venous system. WebMD describes it well -“Veins coming from the stomach, intestine, spleen, and pancreas merge into the portal vein, which then branches into smaller vessels and travels through the liver.”

The Esophagus

Gastro Esophageal Reflux disease (GERD), is the condition in which stomach contents, including acid, leak into the esophagus. GERD can cause:

  • Heartburn
  • Erosion of esophageal tissue
  • Pneumonia caused by aspiration of acid contents into the lungs
  • Barrett’s Esophagus
  • Esophageal cancer

GERD is much more serious and uncomfortable than “heartburn,” and patients often require a number of treatments:

  • Changing WHAT you eat – Avoid foods which are notorious for triggering reflux, like tomatoes, coffee, and alcohol, as well as those foods that you find triggers your symptoms.
  • Changing WHEN you eat – Doctors suggest eating your last meal well before you go to bed, giving your digestive system the opportunity to digest as much food as possible before you lay horizontally for eight hours.
  • Changing HOW you eat – Try eating smaller, more frequent meals. This gives the stomach time and space to do its job.
  • Medications that slow down the creation of acid and/or reduce it once it’s formed.
  • Surgery – Used as a last resort, funduplication surgery can help resolve GERD, but can lead to additional digestive problems later on.

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A final note: This post is based on my personal experiences and research. Cystic Fibrosis is a complex disease, and it’s important to note that it is different for everyone. Not only do the thousands of potential gene combinations play a role in how CF manifests and progresses – there are almost 2,000 known mutations – but so do hereditary conditions and circumstances, as well as environmental issues, such as access to doctors, medications, specialized treatments, financial resources, lifestyle, etc.

*Please do not make any changes to your treatment regimen without consulting your CF team or physician(s). All of the information presented on this website is from my personal perspective and should not be taken as medical advice, nor as any official statement on Cystic Fibrosis, by the CF Foundation, my doctors, or hospital.

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