Cystic fibrosis (CF) is a genetic disease which a baby inherits from both their parents. This occurs when one has inherited two copies of the defective CF gene, one coming from the mother and the other from the father. Cistic fibrosis affects the cells that produce bodily fluids such as sweat and mucus and causes these fluids to become sticky. This usually causes problems such as breathing problems and can cause obstructions in various ducts in the body like in the pancreas. It is known to be a progressive disease that requires daily care.
Cystic Fibrosis Complications
Cystic fibrosis is a complex and progressive disease that leads to a range of complications affecting various body systems.
Damaged Airways
A chronic condition known as bronchiectasis occurs when there is abnormal widening and scarring of the bronchial tubes. This makes it extremely difficult for patients to move air in and out of the lungs while the thick mucus also struggles to clear from the bronchial tubes.
Chronic Lung Infections
The thick mucus in the lungs creates an ideal environment for bacteria and fungi growth. People with cystic fibrosis encounter lung infections on a frequent basis and it becomes tricky to manage and treat when the infection is resistant to antibiotics.
Diabetes and Liver Disease
People living with cystic fibrosis have a higher risk of developing diabetes as well as liver problems. The duct that transports bile from the liver and gallbladder into the small intestine often becomes blocked and inflamed. It can cause an infection, jaundice and even liver cirrhosis.
Electrolyte Imbalance
Due to the defect that causes mucus and bodily fluids to become thicker in people with cystic fibrosis, it also causes sweat to be more salty, resulting in an electrolyte imbalance. This can increase the risk of the person becoming dehydrated, especially in hot environments.
Not only does this disease have complications with the above-mentioned body parts, but it can also have detrimental consequences on a male’s fertility.
Effects on Male Fertility
The average life expectancy of people living with cystic fibrosis is increasing. This puts them at risk of experiencing fertility-related problems later in their life.
Genetic Risk
In most cases, males choose not to have any children due to the fact that the offspring can inherit the gene and also suffer from cystic fibrosis. In the case where men want to have children, they usually consider alternative options such as adoption or using assisted reproductive techniques, such as IVF, by using donor sperm. Some studies have found that men who are diagnosed with cystic fibrosis are able to father their own children. An assisted reproductive technique known as microscopic epidydimal sperm aspiration (MESA) is a procedure where sperm is extracted from the epidydimis. The sperm can be analysed to test for the defective gene and can then be used via IVF or intra-uterine insemination (IUI) in order to ensure biological paternity.
Delayed Sexual Maturation
Usually, men who are diagnosed with cystic fibrosis experience a delay in puberty. This can have severe implications for a male’s fertility. This can cause smaller testicular size that can affect sperm production and sperm quality. Most men are also deemed to be infertile due to the abnormal development of other reproductive organs. When the organs do not function properly, sperm can not be properly produced and the organs can not help with sperm maturation, thus causing a man to be infertile. Most of the time, men are infertile due to the absence of the vas deferens that never developed. This tube is responsible for transporting mature sperm into the urethra before ejaculation.
Azoospermia
Azoospermia is a term used to describe the absence of sperm in semen. In the case of men with cystic fibrosis, the azoospermia is caused by the thick secretions in the reproductive tract. This leads to an alteration in the environment for sperm to function optimally and can lead to abnormal morphology of sperm.
What To Consider
It is important that patients diagnosed with cystic fibrosis be educated on their risk of being infertile. Studies have shown that various patients are not aware that they won’t be able to father their biological child in the future. It is also considerable to see a counsellor or psychologist regarding their condition or regarding the fact that they are infertile. It is a big shock to some men and it is important to prepare them for what the future might look like.
How To Prevent Cystic Fibrosis
There is no active way to prevent the inheritance of this disease by making lifestyle changes or anything like that. The recommendation is to go for a genetic test before becoming pregnant. This test is done via a blood sample and analysed in a laboratory. This can confirm the risk that the child will have of being diagnosed with cystic fibrosis. In the cases where parents choose not to have their own biological children in order to prevent the inheritance of this condition, parents can also consider adoption.