Healthy Living

How Cystic Fibrosis Progresses in Patients

CF is an inherited disease that progresses throughout a patient’s life. Unfortunately, many effects of CF may be silent at first, which is why damage often occurs before it has the chance to be detected through tests.

How Cystic Fibrosis Progresses in Patients

CF is an inherited disease that progresses throughout a patient’s life. Progression can occur when the thick and sticky mucus that accumulates within the body triggers inflammation and scarring in multiple different organs, thus leading to permanent damage. Unfortunately, many CF symptoms and effects start out in silence, which is why damage often occurs before it has the chance to be detected through tests.

Starting early on, the accumulation in the lungs of thick and sticky mucus becomes a vicious cycle of infection, inflammation, and additional buildup of mucus. An outcome of this cycle is pulmonary exacerbations or acute worsening of symptoms. They are not to be taken lightly because they mean more than just extended hospitalizations or additional rounds of antibiotics. Too many pulmonary exacerbations can cause permanent lung damage and may speed up the progression of CF.

Treating pulmonary exacerbations promptly is also crucial to preventing and slowing down the progression of bronchiectasis. Bronchiectasis is a disease that causes permanent reshaping of the airways. As a result, the airways get scarred and loose, making is harder for the lungs to eliminate mucus.

Assessing and understanding the dynamics of progression in CF

It’s true that CF presents differently in every individual, but every case of CF progresses as time passes. This is why it is important to understand the progression of CF and its potential long-term impact on the body.

The pancreas –

  • Cystic fibrosis-related diabetes (CFRD) - Damage caused by thick digestive juices can lead to scarring, making it harder for the pancreas to function properly. As scarring builds up, many individuals with CF will develop cystic fibrosis-related diabetes (CFRD). CFRD is a condition in which the pancreas does not make enough insulin and the body is unable to properly use the insulin that is produced. If left untreated, symptoms of CF can worsen, resulting in reduced ability to fight infection, nerve problems, muscle loss, kidney complications, and a decline in vision.
  • Pancreatic insufficiency (PI) – 85-90% of individuals with CF have pancreatic insufficiency (PI), a condition in which the body is unable to properly digest food due to lack of enzymes made by the pancreas. PI can lead to a higher risk for pancreatitis, which is a condition that occurs when enzymes are unable to pass through blocked pancreatic ducts. This causes the pancreas to become inflamed, triggering severe symptoms such as abdominal pain, back pain, vomiting, fever, weight loss, and rapid pulse.

The liver –

  • Cirrhosis – Over 9% of individuals with CF develop liver disease, which is considered to be one of the most severe health risks associated with CF. Cirrhosis is a complication of liver disease that makes it difficult for blood to flow through the liver. This, in turn, can result in scarring and damage to the liver, difficulty breathing, low blood counts, and fluid buildup in the abdomen.

The bones –

  • Low bone density – More than 30% of adults with CF have low bone density and there are several different factors that can contribute to this condition. For instance, in some individuals, the inflammation caused by persistent lung infections may casue the breakdown of bone tissue. In others, the inability to absorb sufficient amounts of vitamin D can lead to unhealthy bone growth. As a result, the bones become weak and they can fracture more easily.
  • Osteoporosis – If CF leads to low bone density, there is the possibility that it can contribute to osteoporosis. Osteorpososis is bone disease that occurs when the body loses too much bone, resulting in weak and frail bones.   

The kidneys –

  • Chronic kidney disease (CKD) – 2% of individuals with CF between the ages of 25-35 have chronic kidney disease (CKD). Also known as chronic kidney failure, some of the most common symptoms of CKD are muscle cramps, itching, nausea, vomiting, swelling of the feet and ankles, and loss of appetite. When CKD reaches an advanced stage, hazardous levels of electrolytes, fluid, and wastes accumulate within the body, leading to a gradual loss of kidney function.

The intestines –

  • Distal intestinal obstruction syndrome (DIOS) – In some individuals with CF, the accumulation of mucus along the gastrointestinal tract can hinder the emptying of food. This, in turn, results in distal intestinal obstruction syndrome (DIOS). DIOS involves complete or incomplete blockage of the intestines due to thickened intestinal content.  

The ears –

  • Hearing loss – More than 20% of individuals with CF experience some hearing loss. This is an unwanted side effect from taking certain medications, such as aminoglycosides, for an extended period of time.

The reproductive system –

  • Fertility problems – Individuals with CF are born with normal reproductive organs. However, as CF progresses, it may lead to fertility problems. In fact, around 95% of men and 20% of women with CF are infertile. In men, the duct that carries the sperm from the testicles to the urethra, known as the vas deferens, may be blocked or faulty. In women, the accumulation of thick mucus may block the cervix, which is the opening to the uterus, and prevent sperm from reaching the egg for fertilization.

Mental health –

  • Depression – In dedicating so much time and energy to managing and treating CF, some individuals may lose sight of their mental health. This is why around 30% of individuals living with CF or caring for someone with CF experience depression. Left untreated, depression can affect both physical and mental health. Individuals with untreated depression are less likely to stay on track with their treatment plans and more likely to experience frequent hospitalizations and a lower quality of life.

Beginning at a young age, individuals with CF may experience decline in lung function by 1-3% each year. Part of what makes this decline so severe is the fact that the change is not always felt. If you are living with CF, it is never too early to have a conversation with your healthcare provider about potential lung damage. Doing so can help you to live well into adulthood and to experience a better quality of life.

 

References:

  1. https://www.cfsource.com/uncovering-progression
  2. https://www.uwhealthkids.org/cf-center/parts-of-the-body-affected-by-cystic-fibrosis-cf/34317