How Cystic Fibrosis Affects Adults

How Cystic Fibrosis Affects Adults

Pamela Bandelaria |Mar 7, 2022

Cystic Fibrosis in Adults

One of the most common autosomal recessive diseases is cystic fibrosis (CF). It occurs in 1 in 2,500 to 3,500 newborns. Does this illness sound familiar? If it does not, do not worry. In this article, you will learn about cystic fibrosis in adults, including symptoms and how it affects different organs in the body.

What is Cystic Fibrosis?

Cystic fibrosis is an inheritable disease. In order for the disease to manifest, two copies of the abnormal gene must be present. Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene (a gene that encodes a chloride channel) causes problems in the CFTR protein, which leads to imbalances in the salt and water inside the cell. This imbalance leads to the body producing thick and sticky mucus. Mucus is ideally fluid and runny so that it can act as a lubricant in the body, but for people with CF it tends to clog tubes, putting you at risk of other health concerns.

Cystic fibrosis is also a multi-organ disease, as its effects are seen in different organs, such as the lungs, pancreas and the other gastrointestinal organs, the sinuses and sweat glands.

1. Symptoms in Lungs

Mucus in the lungs normally helps in the lubrication and trapping of foreign substances and microorganisms. The increased viscosity of mucus brought about by cystic fibrosis leads to mucus plugging of the bronchial passages and small airway ducts. This leads to obstructive lung disease and inflammation. It also provides a good habitat for the growth of bacteria, leading to pulmonary infection. Because of its effect on the lungs, people with this condition have significant respiratory problems leading to extreme difficulty in breathing over time.

2. Symptoms in the Gastrointestinal System

Normally, the pancreas secretes enzymes that empty into the small intestine to help with the digestion of food. For patients with cystic fibrosis, this is impaired because the thick mucus secretion blocks and clogs the pancreatic ducts, leading to pancreatic insufficiency. The release of pancreatic enzymes for digestion is compromised too. The impairment due to cystic fibrosis causes greasy stools and difficulty of absorption of nutrients, especially the fat-soluble ones (vitamins A, D, E and K).

Not only that, but because the pancreatic enzymes are not released, it results in inflammation within the pancreas which can lead to destruction of pancreatic tissue and eventually pancreatic failure. Further damage of the pancreas can lead to complications that are similar to type 1 diabetes mellitus.
Duct obstruction also affects the gallbladder and liver, leading to liver cirrhosis and gallbladder disease, with an increased risk of gallstone formation.

3. Symptoms in the Sinuses and Sweat Glands

Obstruction in the sinus passages leads to increased inflammation. Cilia, which are hair-like structures that line the sinus passageways, are impaired. Cilia are part of the respiratory system’s defense and trap potentially-harmful substances, including bacteria that can lead to increased bacterial growth and sinusitis. Dysfunction also occurs in the sweat glands, and it causes the sweat in skin to have a higher salt concentration. In severe cases, this can lead to dehydration.

How is It Treated?

Cystic fibrosis can be diagnosed at birth through newborn screening tests. Because it can be detected early, treatment can also be started early and optimized. Treatment is also multi-factorial and patients are usually managed by a team of specialists.

The main goal in the treatment of cystic fibrosis is to ensure that all the organs affected by the disease are not damaged and function optimally for as long as possible.

Because cystic fibrosis has a significant impact on the lungs, one of the goals of treatment is to maintain lung function and avoid further impairment by controlling infection and inflammation.

Antibiotics are given to control infection and adequate oxygenation is provided by bronchodilators. Anti-inflammatory medications are given to control inflammation. If there are signs of respiratory distress, oxygenation and breathing are supported by using devices such as nasal cannulas or a bilevel positive airway pressure. Nutritional support is also important as malabsorption of important nutrients can lead to poor weight gain and weaker immunity. By addressing all these concerns, treatment is not only multi-factorial, but also holistic.

In Conclusion

While many people believe CF is limited to the lungs, this is not true. Cystic fibrosis in adults can occur in other areas of the body, affecting the sinus cavities and digestive tract. If you believe you are at risk, talk to your doctor as soon as possible.

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10 Most Common Symptoms of Poor Circulation

John David Abundo | March 7, 2022

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Sinead Carey | March 7, 2022

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Follow These 8 Tips to Prevent Balding

Elizabeth Dickson | March 7, 2022

How to Prevent Balding If balding or thinning hair is one of your worries, we have got the solutions for you. In this article, we will chat about the symptoms of balding, the causes, how to prevent balding and how to cope with hair loss. Losing a few strands of hair every day is completely normal, but what do you do when you begin losing more hair than you should? According to the American Academy of Dermatology (AAD), 80 million people in America have hereditary hair loss (alopecia). In addition, according to the American Hair Loss Association (AHLA), approximately 25% of men who have hereditary male pattern baldness start losing their hair before the age of 21. By the age of 50, about 85% of men are bald or have significantly thinner hair. 8 Ways to Prevent Balding If genetics is the reason for your hair loss, there might not be a lot that can be done to prevent it. However, these tips can help slow down or prevent loss if other reasons are causing it: Treat your hair gently and avoid pulling; use caution when washing, brushing and styling your hair. Avoid harsh treatments, such as coloring and perms. Protect your hair from the sun; wear a hat and avoid tanning beds. Quit smoking. Some studies suggest there is a link between balding and regular smoking. Eat a balanced diet rich in nutrients and antioxidants. Avoid hot showers and shampoo that causes scalp irritation. Talk to your doctor or dietician about supplements that may help slow down your hair loss. If you are getting treatments for cancer or taking a medication that causes hair loss as a side effect, speak to your doctor about getting a cold cap or other measures to prevent hair loss. If you try the above tactics and still feel that hair loss is negatively impacting your life, it is important to seek out medical advice. First, talk to your doctor about prescription or over-the-counter treatments for hair loss. Your doctor may refer you to a dermatologist who specializes in hair loss. Hair Loss Symptoms Hair loss can look different from person to person, depending on the severity and cause. However, here are some common symptoms to watch for. Slow and Consistent Loss of Hair Starting at the Top of the Head This is the most common form of hair loss. As you age, you can experience thinning of the hair on the top of your head, especially men. In men, this typically looks like a receding hairline or thinning hair or a thinning patch on top of the head. In women, this typically looks like the widening of the part of the hair, along with loss of hair around the forehead. Sudden Loss of Hair It's as if it has become loose from your scalp. Our bodies are extremely sensitive to changes and will work hard to cope with those changes. Therefore, environmental and emotional stress can cause you to lose handfuls of hair at a time. Thankfully, this type of hair loss is typically temporary and will stop once the stressors have been addressed. [youmaylike] Patches of Hair Loss All Over the Scalp With this type of hair loss, hair consistently falls out from the same spot on your scalp. This leaves your scalp with patches or circular spots of missing hair. Not only can this affect the hair on your head, but it can also leave patches of missing hair on your face, such as in your beard or eyebrows. Loss of Both Scalp and Body Hair This type of hair loss is less common than the others. It typically only affects individuals who are taking a strong medication or treatment, such as chemotherapy. The lost hair generally regrows with ease once the treatment has stopped. Causes of Hair Loss There are several reasons why you may experience hair loss, including: Hereditary and genetic factors. Hormonal and systemic body changes. Certain medications and medical conditions. Mechanical stress, such as consistent pulling on the hair. Emotional and environmental factors. Radiation exposure. Some degree of hair loss is entirely normal. Typically, you lose between 50 to 100 strands of hair per day. New hair strands grow at about the same rate, meaning that hair loss isn't generally noticeable until you have exceeded this number.