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Cystic Fibrosis Trust

Additional complications

The lungs and digestive system are not the only parts of the body affected by cystic fibrosis (CF). With people living longer than ever, additional complications are more frequent.

There are a range of additional complications that people with cystic fibrosis may have to face, somethings as a result of the effect of CF on the lungs or digestive system or because of the treatments required to manage symptoms.

Cystic fibrosis and fertility

Cystic fibrosis can cause fertility problems, although it does not cause sexual impotency. In most men the tubes that carry the sperm are blocked, causing infertility. The nutritional issues associated with CF may affect female fertility as underweight women are more likely to have irregular menstrual cycles and thicker vaginal mucus, making it harder to conceive. Women with cystic fibrosis do produce healthy, fertile eggs, and so effective contraception is necessary.

People with cystic fibrosis are increasingly thinking about or actively planning to have their own families, which demonstrates how far we have come in terms of care and medication. There are special considerations and challenges, and your specialist CF team should be your first port of call if this is something you want to do. If you would like to find out more about the chance of your child being born with the condition, take a look at our information on carrier testing.

For more information, take a look at our starting a family booklet, which contains lots of case studies and expert information to help support you to make the right decision for your health and your family.

Cystic fibrosis and meconium ileus (bowel obstruction)

One in every ten babies born with CF develops a bowel obstruction called meconium ileus at birth or within the first few days of life. In these cases the meconium (a thick black material present in bowels of all newborn babies) is so thick that it blocks the bowel instead of passing through. Babies with meconium ileus often need an urgent operation to relieve and bypass the blockage.

Cystic fibrosis and bone disease, osteoporosis and low bone mineral density (BMD)

People with CF are prone to developing bone disease (thin, brittle bones) due to a lack of nutrition (which is lost through stools because of the damaged pancreas), and other complaints related to the condition. Adults with cystic fibrosis also have an increased risk of bone disease, which is a side effect of the steroids taken to control lung disease.

Thinning bones are a well-documented consequence of CF, with reduced bone mineral content found in people of all ages. Low BMD is common in adults with CF, and is often found with increasing severity of the condition. As a result of this all adults will require regular scans to monitor BMD. Left untreated low BMD can lead to osteoporosis.

Osteoporosis is a disease of the skeleton that causes a deficiency of bone mass and a reduced bone density. Osteoporotic bones are vulnerable to compression fractures, and the backbone can become abnormally curved. Bone strengthening drugs are now available. Find out more by downloading our factsheet on bone health (PDF).

Cystic fibrosis-related liver disease

Cystic fibrosis can cause the blockage of small ducts in the liver, leading to liver disease. Although this only happens in about eight per cent of people with cystic fibrosis and can sometimes be managed by drugs, it is a serious health risk and could require a liver transplant. Find out more about the cause, diagnosis and treatment of liver disease by downloading our factsheet (PDF).

Cystic fibrosis-related diabetes

CF-related diabetes (CFRD) affects more than 30% of adults with cystic fibrosis. Symptoms of this unique form of diabetes include an increase in thirst and urination caused by high blood sugar levels, excessive tiredness, weight loss and a decline in lung function.

Joint pain and arthritis

The likelihood of joint pain and arthritis increase as people with cystic fibrosis get older. Five to ten per cent of people with the condition will develop these complications. The most common form of joint pain in CF is an arthritis that affects large joints, such as the knee, ankle, wrist, elbow and shoulder. Arthritis in CF tends to come and go, with no symptoms in between attacks. Joint pain can flare up during infections and periods of ill health.

Kidney and hearing complications

Risk of nephrotoxicity and ototoxicity to kidneys and hearing – people with CF require a substantial amount of aminoglycoside antibiotics during their lifetime, which can have a toxic impact on the kidneys and hearing. Aminoglycosides, which are broad-spectrum antibacterial antibiotics such as tobramycin, are used extensively for the treatment of many bacterial infections.

Sinusitis

Acute and chronic sinusitis is common in people with cystic fibrosis. Symptoms can include blocked nasal passages, headaches, and a cough that is made worse by lying down. Sinusitis can usually be controlled with medications.

Nasal polyps

People with CF have an increased risk of developing nasal polyps; tissue growths that develop inside the nasal passages and sinuses. Large polyps can cause a blocked or runny nose, and a loss of smell or taste. Like sinusitis, nasal polyps can usually be controlled with medications.

Distal intestinal obstruction syndrome (DIOS)

Previously known as meconium ileus equivalent (MIE), DIOS is a condition that is unique to cystic fibrosis. DIOS causes blockages in the stomach, causing symptoms such as colicky stomach pain, bloating, nausea and weight loss. In most cases DIOS can be controlled with medication.

Acid reflux

A common occurrence in people with CF, stomach acid can be harmful to the oesophagus (gullet) and can trigger coughing. This can be treated with antacids in most cases. Surgery is an option in more severe cases.

Gastro-oesophageal reflux disease (GORD)

A common condition where stomach acid leaks from the stomach into the oesophagus (gullet). Symptoms include heartburn, a nasty sour taste in the mouth, and pain or difficulty swallowing. This can be treated with antacids in most cases. If you have any questions please call our helpline on 0300 373 1000, where you can speak to a friendly expert.


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