Cystic fibrosis (CF) can affect many parts of the body beyond the lungs. As people with CF live longer and healthier lives, it is increasingly important to recognize and manage complications that can impact digestion, bone health, blood sugar levels, liver function, and sinus health. Some complications are common and may develop gradually over time, while others require prompt medical attention. The information below provides an overview of several more commonly seen complications associated with cystic fibrosis.
Distal Intestinal Obstruction Syndrome (DIOS)
Distal intestinal obstruction syndrome (DIOS) is commonly seen in people with cystic fibrosis. Food and mucus may partly block the intestine and cause pain. About 1 out of every 10 to 20 people with cystic fibrosis get DIOS. Symptoms of DIOS can include cramps, stomach aches, pain around the belly button, or lower down. Patients with DIOS may not feel as hungry as they usually do. If the DIOS is severe, the patient may experience vomiting and have a lot of pain. Bowel movements may be very watery. If this happens, go to the emergency department of the hospital closest to you for treatment.
Osteoporosis
Osteoporosis (thinning of the bones to the point of brittleness) and osteopenia (decreased bone mass and a precursor to osteoporosis) is a disease of the skeleton, resulting in lower bone mass and a reduced bone density. All patients with CF are at risk of osteoporosis, especially those with more severe cases and those with low body weight.
The consequences of this disease are that bones become fragile and are more likely to break. If not prevented, or if left untreated, osteoporosis can progress painlessly until a bone breaks. Calcium is the key mineral in maintaining bone density. However, intake of calcium alone is not enough, since vitamin D levels in the body directly affect calcium absorption. Because vitamin D is a fat-soluble vitamin, people with CF often have trouble absorbing this vitamin and thus don’t absorb enough calcium.
Cystic Fibrosis-Related Diabetes (CFRD)
Cystic Fibrosis-Related Diabetes (CFRD) is a unique type of diabetes. It is not the same as diabetes in people without CF. The diagnosis and treatment are not exactly the same. CFRD is extremely common in people with CF especially as they get older. CFRD is found in 35 percent of adults aged 20 to 29 and 43 percent of those over 30 years old.
People with CF do not make enough insulin. This is a result of scarring in the pancreas.
Common symptoms, such as increased thirst and increased urination, are caused by high blood sugar levels (hyperglycemia). Other symptoms of CFRD are excessive fatigue, weight loss, and unexplained decline in lung function.
Many people with CFRD do not know they have it until they are tested for diabetes by having a two-hour Oral Glucose Tolerance Test (OGTT). Since many people with CF have no symptoms of CFRD, this test is the best way to find out if someone has CF-related diabetes. People with CFRD who receive treatment for diabetes often start to feel better, gain weight, and improve their lung function.
Insulin is the medication used to treat CFRD. It allows sugars and proteins to move from the blood into the body’s cells. It is used for energy and to build muscle.
View the Cystic Fibrosis Related Diabetes (CFRD): A First Canadian Clinical Practice Guidelines. If it is determined that you have CFRD an appointment will be set up at a diabetes education center.
Liver Disease
CF-related liver disease (CFLD) occurs in up to 30% of people with CF. However, it is only a serious clinical problem in a minority of individuals. Often there is an abnormality of liver function tests and in some of these, some changes in the liver. In a small percentage of patients, cirrhosis (scarring of the liver) develops. If the scarring becomes severe, liver transplant may be necessary. Liver function is monitored through blood tests and abdominal ultrasound.
Nasal Polyps
Nasal polyps are a very common occurrence in people with CF. Studies have estimated that anywhere from 10% to nearly 50% of people with CF have nasal polyps. Nasal polyps are small, sac-like growths consisting of inflamed (swollen) nasal mucosa, which results in masses in the nose. Most individuals with nasal polyps complain of nasal blockage affecting both sides of the nose but not necessarily to the same extent. Other symptoms include a runny nose, loss of sense of small, facial pain or congestion and less commonly, nose bleeds.
If the doctor diagnoses nasal polyps, simple medical treatment with nasal steroids is often effective. In some patients a surgery to remove the polyps (polypectomy) will be necessary.
