General Health

What are the Effects of Cystic Fibrosis on Various Organs?

Lungs and sinuses

Lung disease is caused by mucus build-up, reduced mucociliary clearance (primary innate defence mechanism of the lung), and resultant inflammation in the airways. Changes in lung architecture, such as disease in the main airways (bronchiectasis), increase breathing problems in advanced stages. Other symptoms include pulmonary hypertension (high blood pressure in the lungs), heart failure, a lack of oxygen in the body (hypoxia), and respiratory failure necessitating breathing masks, such as bilevel positive airway pressure devices or ventilators. The three most prevalent organisms that cause lung infections in CF patients are Staphylococcus aureusHaemophilus influenzae, and Pseudomonas aeruginosa. Additionally, the opportunistic infection caused by the Burkholderia cepacia complex can arise, particularly through patient-to-patient transmission.

People with CF are more likely to acquire lung illnesses other than normal bacterial infections. Allergic bronchopulmonary aspergillosis is one of them, in which the body’s complex hypersensitivity reaction to the common fungus Aspergillus fumigatus causes breathing issues to worsen. Another is infection with the Mycobacterium avium complex (a bacterium linked to tuberculosis that includes Mycobacterium intracellulare and Mycobacterium avium) a bacterium that can affect the lungs and is resistant to conventional medicines.

Mucus in the paranasal sinuses is thicker than in the nasal sinuses, and it can clog sinus passageways, resulting in infection. Facial pain, fever, nasal discharge, and headaches are all possible side effects. Inflammation from chronic sinus infections can cause enlargement of nasal tissue (nasal polyps) in people with CF.

Gastrointestinal

The thick mucus observed in the lungs has a mirror in the pancreas’ thickened secretions, which provide digestive juices that aid in the digestion of meals. These fluids obstruct the exocrine passage of digesting enzymes into the duodenum, causing irreparable pancreatic damage, typically accompanied by severe inflammation (pancreatitis). In more advanced cases, the pancreatic ducts are completely blocked, which is commonly seen in older children or teens. The exocrine glands atrophy and fibrosis progress as a result of this.

Vitamins A, D, E, and K, which are fat-soluble, are similarly difficult to absorb in patients with CF.

Individuals with CF have greater heartburn, intestinal blockage due to intussusception, and constipation, in addition to pancreas issues. Distal intestinal obstruction syndrome, occurs when faeces become thick with mucus (inspissated) and causes bloating, pain, and incomplete or complete bowel obstruction in older people with CF, can cause bloating, pain, and incomplete or complete bowel obstruction.

The majority of CF patients (85 percent to 90 percent) have exocrine pancreatic insufficiency. It’s mostly linked to “severe” CFTR mutations, such as F508/F508, in which both alleles are completely nonfunctional. Enzyme supplementation is not necessary in these milder cases because sufficient pancreatic exocrine activity is still present. In pancreas-deficient phenotypes, no other GI issues are common, and such individuals typically have excellent growth and development. Despite this, a subgroup of pancreas-deficient people with CF can develop idiopathic chronic pancreatitis, which is accompanied by recurring stomach pain and life-threatening consequences.

In CF patients, thickened secretions can potentially cause liver issues. The liver’s bile, which aids digestion, can clog the bile ducts, causing liver damage. Steatorrhea is caused by a problem with fat digestion or absorption. This can cause scarring and nodularity over time (cirrhosis). The liver is unable to remove toxins from the blood and does not produce critical proteins such as those involved in blood coagulation. The third most common cause of death in people with CF is liver illness.

Around 5–7% of people have a severe liver impairment that results in symptoms, such as gallstones causing biliary colic.

Endocrine

The islets of Langerhans are located in the pancreas and are responsible for producing insulin, a hormone that aids in blood glucose regulation. Damage to the pancreas can result in the loss of islet cells, resulting in a type of diabetes that is unique to persons who have it. One of the most prevalent non-pulmonary complications of cystic fibrosis is diabetes, which includes symptoms of both type 1 and type 2 diabetes.

Calcium and phosphate control is aided by vitamin D. Because of malabsorption, poor dietary vitamin D absorption can lead to bone disease and osteoporosis, which causes weakening bones to be more susceptible to fractures.

Infertility

Infertility affects men and women equally. At least 97 percent of males with cystic fibrosis are impotent, but not sterile, and can have children with the application of assisted reproductive technology. The most prevalent cause of infertility in boys with CF is a congenital deficiency of the vas deferens (which connects the testes to the ejaculatory ducts of the penis), but alternative mechanisms such as no sperm, poorly shaped sperm, and few sperm with poor motility are also conceivable. During infertility examinations, many men with a mild, previously unknown form of CF are determined to have a congenital absence of the vas deferens. Due to thickening cervical mucus or malnutrition, roughly 20% of women with CF have fertility issues. Malnutrition impairs ovulation and results in a lack of menstruation in extreme cases.

Author

 Yash Batra

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