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Receiving a cystic fibrosis diagnosis can be hard, but you’re not alone. The Larivière et Massicotte pharmacy team is here to support you through this difficult time, working with your healthcare specialists to ensure the best possible pharmacological treatment. Do not lose hope: cystic fibrosis treatments are progressing at an incredible pace and research has never been so promising. We’re here to give you peace of mind. You focus on fighting the disease, we’ll take care of the rest.

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What is cystic fibrosis?

Cystic fibrosis, sometimes called mucoviscidosis, is a serious genetic disease affecting around 1,200 people in Quebec. It is caused by a mutation in the CFTR gene, which results in the production of thick, viscous mucus that blocks the airways, pancreatic ducts, and other organs. Sufferers may experience symptoms such as breathing difficulties, frequent lung infections, pancreatic insufficiency, and incomplete digestion of food. Although this is a chronic, incurable disease, treatment and management have improved considerably over recent years. Patients can now live longer, better lives thanks to medical therapy, physiotherapy, and other adapted care.

How is cystic fibrosis diagnosed?

Cystic fibrosis can be diagnosed through an evaluation of symptoms and testing. All newborns are now screened for this disease to ensure early detection and treatment. Screening can be done by analyzing a sample of the baby’s blood for high levels of immunoreactive trypsinogen generated by the pancreas. High levels of IRT in newborns may be due to premature birth or the stress they experience during childbirth, so further tests are often needed to confirm a cystic fibrosis diagnosis.

The disease can also be detected in a newborn with a sweat test, which involves applying a substance to a small area of skin to stimulate sweat production, then collecting it to check for an abnormally high salt content. A physician may recommend genetic testing to detect the presence of specific mutations in the gene responsible for cystic fibrosis. These tests can be used in conjunction with an analysis of IRT levels to confirm the diagnosis.

Adults and older children who were not screened at birth may be advised to undergo diagnostic testing for cystic fibrosis if they suffer from problems such as inflammation of the pancreas, chronic sinusitis, chronic lung infections, nasal polyps, chronic cough, or are underweight.

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What are the possible complications of cystic fibrosis?

Cystic fibrosis may lead to complications in the respiratory, digestive, and reproductive systems, and may also affect many organs. Patients with cystic fibrosis are under medical supervision to ensure proper management of these symptoms and complications. Problems such as damaged airways, nasal polyps, chronic infections, episodes of hemoptysis (coughing up blood), pneumothorax, respiratory failure, and acute exacerbations are common in the respiratory system.

Cystic fibrosis can also lead to complications of the digestive system, such as diabetes, nutritional deficiencies, liver disease, intestinal obstructions, and distal intestinal obstruction syndrome. Complications of the reproductive system include infertility in men and reduced fertility in women. Mental health problems such as depression and anxiety, and bone problems such as osteoporosis, are also a risk.

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What are the treatments for cystic fibrosis?

Unfortunately, there is no cure for cystic fibrosis, but symptoms can be alleviated and quality of life improved. Early intervention is recommended to slow the progression of the disease and prolong life expectancy. Managing the disease is complex, and generally requires care in a specialized centre with a multidisciplinary team of medical specialists.

Cystic fibrosis treatment focuses on preventing and controlling lung infections, clearing mucopurulent secretions from the lungs, treating and preventing intestinal obstructions, and ensuring adequate nutrition. Treatment may include drugs that target the gene mutations involved, antibiotics to treat or prevent lung infections, anti-inflammatory or mucopurulent clearance drugs, bronchodilators inhalers, oral pancreatic enzymes, stool softeners to prevent constipation or intestinal obstructions, and specific drugs for diabetes or liver disease, if necessary.

Cystic fibrosis transmembrane permeability regulator (CFTR) modulators may be recommended for patients with certain genetic mutations. Other treatments include techniques to clear the airways, pulmonary rehabilitation, and surgical procedures.

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Our mission

Our committed team works hand in hand with community pharmacists, healthcare teams, and various healthcare professionals to support patients with cystic fibrosis, ensuring the best possible care for them.

Common questions

How is cystic fibrosis inherited?

Cystic fibrosis is a genetic disease that is transmitted from parents who carry the gene mutation to their children. Both parents must carry a mutated copy of the CFTR gene for the child to develop the disease. People with only one mutated copy of the gene are called healthy carriers, as they show no cystic fibrosis symptoms. A child has a 25% chance of inheriting two mutated copies of the gene if both parents are healthy carriers, which would lead to the onset of the disease. There is a 50% chance that the child will inherit both a mutated and a normal copy of the gene, making them a healthy carrier of the disease. Finally, there is a 25% chance that the child will not develop cystic fibrosis by inheriting two normal copies of the gene.

What are the symptoms of cystic fibrosis?

Cystic fibrosis is a condition in which the production of mucus obstructs the lungs, digestive system, and reproductive system. Its symptoms can vary in severity depending on the individual, and can appear at different points in a person’s life. Since it causes high salt production in sweat, parents may notice a salty taste on the child’s skin. Mucus in the lungs can lead to wheezing, coughing, and breathing difficulties. The disease can generate mucus that obstructs the pancreas and prevents the release of enzymes needed to break down food, which is digested incorrectly and expelled by the body as waste in stools, which are often large and foul-smelling. Affected children are often smaller than their peers.

Does cystic fibrosis affect fertility?

Children with cystic fibrosis may reach puberty later than average. The disease can lead to fertility problems: in men, this is caused by the absence of the vas deferens, which carries sperm out of the testicles, and affects 98% of men affected by the disease. In women, infertility may be the result of mucus on the fallopian tubes, or due to irregular periods. Women with cystic fibrosis can nevertheless become pregnant and give birth to healthy children if their partner does not carry the gene. However, the child will carry the gene.

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Have you or a loved one been diagnosed with diagnosed with cystic fibrosis?

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