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.

Related Search Topics (Ads)

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.

Article Resources

Our Most Valuable advice

Top COPD Inhalers for Better Breathing

Admin | March 7, 2022

Best COPD Inhalers Inhalers are designed to deliver medication directly to the lungs, helping to open airways, reduce inflammation and make it easier to breathe. Zemaira is an injectable medication that helps people with alpha-1 antitrypsin deficiency (a genetic condition that can worsen COPD). 1. Zemaira (α1-proteinase inhibitor) By increasing levels of the α1-proteinase inhibitor in the body, Zemaira helps protect the lungs from further damage. It's not a traditional inhaler, but it is an important treatment for people with specific COPD-related conditions. 2. Spiriva (tiotropium) Spiriva is one of the most commonly prescribed inhalers for people with COPD. It helps relax and open up the airways, making it easier to breathe. It is a long-acting bronchodilator, meaning it works for up to 24 hours, providing day-long relief. This inhaler is typically used once a day. [youmaylike] 3. Advair (fluticasone/salmeterol) Advair is a combination inhaler that contains both a steroid and a long-acting bronchodilator. The steroid works to reduce swelling and inflammation in the airways, while the bronchodilator helps relax the muscles around the airways. This combination helps improve breathing and prevent flare-ups of COPD symptoms. It’s typically used twice a day. 4. Symbicort (budesonide/formoterol) Like Advair, Symbicort is another combination inhaler with a steroid and a bronchodilator. It helps reduce inflammation in the lungs and opens the airways. This inhaler is also used to treat asthma, but it’s a great option for managing COPD as well. It is taken twice a day for the best results. 5. Breo Ellipta (fluticasone/vilanterol) Breo Ellipta is a once-a-day inhaler that combines a steroid and a long-acting bronchodilator. It helps open the airways and reduces inflammation in the lungs. This inhaler is typically used for people with chronic COPD symptoms to help improve lung function. 6. Albuterol (ProAir, Ventolin, Proventil) Albuterol is a short-acting bronchodilator that provides fast relief for sudden breathing problems, like shortness of breath or wheezing. It works quickly to open the airways, helping you breathe easier. It is often used as a rescue inhaler during flare-ups and should be kept on hand for emergencies. 7. Atrovent (ipratropium) Atrovent is another short-acting bronchodilator. It helps relax the muscles around the airways and can be used in combination with other medications to treat COPD. Atrovent is often used multiple times a day and can help reduce coughing and wheezing. COPD Control and Relief There are many inhalers available to help manage COPD symptoms, but the best one for you depends on your specific condition and treatment needs. Zemaira is an important option for people with alpha-1 antitrypsin deficiency, while other inhalers like Spiriva and Symbicort help open the airways and reduce inflammation. Speak with your doctor to find the right inhaler for you, and remember that proper use of your inhaler is key to managing your COPD and improving your quality of life. Read on to learn about exercise induced asthma, and how you can cope with it.

7 Treatment Options for Schizophrenia

Carlos Mendoza | March 7, 2022

Talk to your Doctor About Treatment for Schizophrenia Effective treatment for schizophrenia requires a combination of approaches tailored to each patient’s specific needs. Medication like Uzedy, therapy and lifestyle changes all play a role in managing symptoms, and help patients lead more stable, fulfilling lives. In this article we’ll look at common treatment options for schizophrenia 1. Antipsychotic Medications The cornerstone of treatment for schizophrenia is antipsychotic medication, like Uzedy. It's designed to reduce symptoms such as hallucinations, delusions and paranoia. Antipsychotics work by targeting neurotransmitters, particularly dopamine, which is often imbalanced in individuals with schizophrenia. They help stabilize thoughts and emotions, allowing people to better engage in daily activities and therapy. Uzedy (Risperidone Extended-Release Injection): Uzedy is a newer, long-acting injectable form of risperidone, which is particularly helpful for patients who struggle with adhering to a daily medication regimen. Given as a monthly or bi-monthly injection, Uzedy provides consistent medication levels, reducing the risk of symptom relapse. This can be particularly valuable for individuals with schizophrenia, as regular medication adherence is essential to symptom stability. Uzedy’s extended-release formulation allows the medication to work over time, reducing fluctuations and helping maintain a stable mood and thought pattern. Typical (first-generation) antipsychotics: These medications, such as haloperidol and chlorpromazine, were the first types of antipsychotics developed and are effective in controlling positive symptoms like hallucinations. However, they often come with side effects, including stiffness, tremors and tardive dyskinesia (involuntary muscle movements). These side effects can be difficult to manage, which has led to the development of newer, second-generation antipsychotics. Atypical (second-generation) antipsychotics: Newer antipsychotics, such as risperidone, olanzapine and aripiprazole, tend to have fewer side effects related to motor functions. They are often preferred because they also address both positive and negative symptoms of schizophrenia, such as emotional flatness or withdrawal. Although these medications also come with side effects, such as weight gain and metabolic changes, they are generally better tolerated than first-generation antipsychotics. [youmaylike] 2. Psychotherapy and Cognitive Behavioral Therapy (CBT) Medication is often combined with psychotherapy to improve outcomes. CBT, for example, helps patients recognize and change thought patterns that may contribute to symptoms. Through CBT, patients learn strategies to handle paranoia, manage delusions and cope with hallucinations. Therapy provides tools to differentiate between reality and perceptions shaped by schizophrenia, helping patients achieve a better quality of life. Social skills training and family therapy are also valuable, as they equip patients and their loved ones with coping mechanisms and communication skills. 3. Psychoeducation Education plays a critical role in managing schizophrenia. Psychoeducation programs inform patients and families about the condition, helping them understand symptoms, treatment options and coping strategies. Psychoeducation can include information on identifying early warning signs of relapse, understanding the side effects of medications, and managing daily stressors. Patients who are educated about their condition are often better able to manage symptoms and adhere to their treatment plan. 4. Supportive Housing and Vocational Rehabilitation For many individuals with schizophrenia, maintaining employment and independent living can be challenging. Supportive housing programs provide a structured, safe environment where residents receive assistance with daily activities, medication and social skills. Vocational rehabilitation programs, on the other hand, focus on helping individuals develop skills to find and maintain employment. These programs may offer job training, resume building, and interview preparation. Stable housing and vocational support contribute significantly to improved outcomes, providing a sense of purpose and routine. 5. Lifestyle Modifications and Self-Care While schizophrenia requires medical intervention, lifestyle choices also play a crucial role in managing symptoms. Regular physical activity, a balanced diet and adequate sleep are essential. Exercise can help improve mood, reduce anxiety and manage weight gain, a common side effect of antipsychotic medications. Diet is equally important; reducing sugar and unhealthy fats may help avoid metabolic complications associated with antipsychotic drugs. Additionally, sleep is vital for mental health, and establishing a routine can help improve both mood and cognitive function. 6. Long-Acting Injectable Antipsychotics (LAIs) Long-acting injectables like Uzedy provide an alternative for patients who struggle to take medication consistently. LAIs are administered every few weeks to months, ensuring stable levels of medication and reducing the likelihood of missed doses. LAIs can reduce the frequency of relapses and hospitalization, as they eliminate the need for daily pills. Some other examples include Abilify Maintena, Invega Sustenna and Aristada. For individuals prone to forgetting doses or struggling with self-management, LAIs can provide a more consistent and manageable form of treatment. 7. Electroconvulsive Therapy (ECT) For patients with severe symptoms that don’t respond to medication or therapy, electroconvulsive therapy (ECT) may be considered. ECT involves a brief electrical stimulation of the brain while the patient is under anesthesia, and it’s typically used when other treatments have failed. ECT has been shown to reduce severe symptoms, particularly catatonic symptoms and severe depressive states in patients with schizophrenia. It is usually a last resort and performed under careful medical supervision, but it can provide relief when other methods are ineffective.

5 Ways to Improve Your Quality of Sleep

Cassidy Anthony | March 7, 2022

How to Get Better Sleep Many people struggle with sleep disorders like hypersomnia and narcolepsy. Thankfully, treatment options like Xywav exist to help manage consistent, healthy sleep patterns. In this article we're looking at five different ways on how to get better sleep, and breaking each one down. 1. Prescription Medications for Sleep Disorders Certain medications are specifically designed to help regulate sleep patterns and address various sleep disorders. These options can be helpful for those who have severe insomnia or issues like narcolepsy: Xywav: Known for its role in managing excessive daytime sleepiness in narcolepsy, Xywav has proven effective in promoting restful sleep. It works by helping to regulate the brain's sleep-wake cycle and is especially beneficial for those with disorders causing fragmented sleep. Ambien: Commonly prescribed for short-term insomnia, Ambien assists with falling asleep faster. Lunesta: Lunesta is meant for short-term treatment of insomnia, helping those who struggle to fall and stay asleep. Belsomra: Belsomra works by blocking orexin, a neurotransmitter involved in wakefulness. It’s effective for people who have difficulty staying asleep and often provides a gentle, non-habit-forming solution. 2. Cognitive Behavioral Therapy for Insomnia (CBT-I) Cognitive behavioral therapy for insomnia, also known as CBT-I, is a non-drug approach to addressing chronic insomnia. The therapy involves working with a therapist to address thoughts and behaviors that interfere with sleep. Techniques used in CBT-I include: Sleep restriction therapy: Sleep restriction therapy limits the time spent in bed to create a stronger association between bed and sleep. Stimulus control therapy: Stimulus control therapy involves finding activities that promote a restful mindset in the bedroom, such as avoiding screens before bed and not working in the bedroom. Cognitive restructuring: Cognitive restructuring helps individuals shift thoughts from sleep anxiety to positive associations with bedtime, ultimately leading to a better night of sleep. [youmaylike] 3. Lifestyle Adjustments to Improve Sleep Quality While this might seem simple, adopting healthier sleep habits can play a substantial role in enhancing sleep quality. Some essential tips include: Maintaining a consistent sleep schedule: Going to bed and waking up at the same time daily helps regulate the body’s internal clock, making it easier to fall asleep and wake up naturally. Creating a relaxing bedtime routine: Engaging in calming activities, such as reading or taking a warm bath before bed, can cue the body for sleep. Exercising regularly: Physical activity, especially in the morning or early afternoon, can improve sleep quality. Avoiding stimulants: Steering clear of caffeine, nicotine and big meals in the hours leading up to bedtime can help promote restful sleep. 4. Natural Supplements for Sleep Support For those looking for natural remedies, several supplements have shown promise in promoting better sleep. Some common supplements for sleep support include: Melatonin: Melatonin is a hormone that helps regulate the sleep-wake cycle. Melatonin supplements can be particularly useful for those dealing with jet lag or unpredictable and late work schedules. Valerian root: Known for its calming properties, valerian root is believed to play a hand in reducing anxiety and improving sleep quality. Magnesium: This mineral plays a role in muscle relaxation and nervous system regulation, helping to alleviate sleep disturbances caused by tension and stress. Lavender: Either as an essential oil or supplement, lavender has been shown to reduce anxiety and improve sleep quality, especially when diffused in the bedroom or taken in small doses before bed. 5. Improving Sleep Hygiene Good sleep hygiene can make a world of difference for those struggling with sleep quality. Sleep hygiene practices include: Limiting screen exposure: The blue light emitted from screens can suppress melatonin production, making it harder to fall asleep. Consider limiting screen time at least an hour before bed. Using blackout curtains: Blocking out light from outside sources creates a darker environment that can help you fall asleep faster and stay asleep through the night. Keeping the bedroom cool: A slightly cool room temperature is optimal for sleep, as it encourages the body’s natural drop in temperature needed for sleep onset.