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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3 Lung Cancer Treatments

Staff Writer | March 7, 2022

Pathways to Healing Lung cancer starts in the lungs but can spread to other regions of the body. While there is no single cure, there are different treatment options that can be used. In this article, we will look at treatment options, as well as symptoms of lung cancer Roughly 1 in 16 Americans will be diagnosed with lung cancer. While smoking can increase the likelihood of getting a diagnosis, non-smokers are not exempt from this condition. There are many different causes of lung cancer, and people of all ages can be diagnosed. Unfortunately, there is no specific cure for lung cancer. Beating cancer is a little more complicated than taking a few pills or going to a doctor’s office. The disease is more serious and requires more intense intervention techniques. Different Lung Cancer Treatment Options Depending on the progression of the cancer and the patient's general health, a physician may recommend several treatment options. Know that with all treatment options, there is a risk of harsh side effects and a chance that the treatment does not work. Still, these are the best options when it comes to defeating lung cancer, with increased chances of success in cases of early detection. Treatments can be used in combination with one another, or as a standalone option depending on the particular case. Chemotherapy Chemotherapy has been one of the most common treatment options for a long time. It works by using strong chemicals that target rapidly dividing cells. When introduced into your body, chemotherapy drugs will attack all rapidly dividing cells. Unfortunately, these drugs cannot differentiate between cancer cells and healthy cells that just happen to be dividing. The side effects of such treatments can be unpleasant, but most reverse when the treatment ends or can be reduced during the treatment. Common side effects include: Nausea/vomiting. Hair loss. Mouth sores. Pain. Bowel disturbances (diarrhea/ constipation). Loss of appetite. Bruising easily. [youmaylike] Harsher and potentially permanent side effects include: Organ damage (heart, lung, kidneys). Nerve damage. Infertility. Heightened risk of a second cancer. Side effects will also vary depending on the type of drug that is used in your treatment and the way you “take in” the drug. The most common chemotherapy drugs for lung cancer include the following: Afatinib. Bevacizumab. Ceritinib. Crizotinib. Erlotinib. Chemotherapy for lung cancer can be done in the following ways: Injected: Most often this is administered through infusions (IV) but can be given through shots. Implanted: Thin wafers containing the drug can be inserted directly into the site of a tumor or surgery. Pills: Pills and capsules containing the drug are sometimes available. Radiation Radiation is another treatment method. Radiation therapy uses beams of energy to damage cells in targeted regions of the body. The intense energy beams aim at precise locations in the body and damage the cells in that region. While this, unfortunately, means that healthy cells will also be attacked, they are often able to “bounce back” from the damage where cancer cells will be killed. As it is a targeted treatment, side effects are often limited to the region in which the treatment was applied. This means that patients can expect skin problems in the area of application as well as fatigue; these are mostly short-term side effects. Specific side effects characterized by treatments aimed at the chest include: Breast soreness. Stiff shoulders. Difficulties swallowing. Respiratory problems. Radiation fibrosis (permanent lung scarring). There is a low chance of developing long-term side effects, such as the development of a second cancer, as an effect of radiation exposure. Surgery If the cancer is caught early enough, then it can be treated by removing the cancerous tissue at the initial site. The operation can only be successful if the cancer has not yet spread to other regions of the body. The side effects of this treatment will depend on the depth of the removal, the nature of the removed tissue, and its size. For example, removing a tiny bit of the lung will not be as hard to recover from as removing a large piece. Other Lung Cancer Treatment Options Other treatments are being developed that aim to keep the effectiveness of chemotherapy and radiation treatments without the harsh side effects. While some remain experimental, recently emerging treatments, such as immunotherapy, offer the potential to battle lung cancer. Talk to a licensed care physician to get the most valuable recommendations on which cancer treatment is right for you. Everybody has a different experience and treatments are decided on a case-by-case basis.

Planning on Getting Botox? Here’s What to Know

Staff Writer | March 7, 2022

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Everything You Need to Know About Colic

Staff Writer | March 7, 2022

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