3 easy breathing exercises for children
Is it possible three breathing practices to improve your child's health? In addition to 3 very easy breathing exercises that…
How to relieve cystic fibrosis symptoms?
Most importantly:
- What is cystic fibrosis?
- What are the most common symptoms of cystic fibrosis?
- Control and treatment of cystic fibrosis
- Inhaled medications and nebulizers as part of the treatment of cystic fibrosis
Cystic fibrosis (CF) is a genetic autosomal recessive disease with multi-systemic symptomatology (impacts various parts and processes of the body especially the lungs, pancreas, and other organs) due to the impaired function of the exocrine glands (the exocrine glands produce tears, sweat, milk, saliva, and digestive juices). Symptoms are most often observed in the respiratory, digestive and genital systems. CF changes how chloride (salt) moves through a patients’ body, and makes mucus (which is normally a thin and slippery substance) in various organs become thick and sticky. In turn, this thick mucus builds up and makes it hard for patients to breathe, traps germs, and can lead to inflammation and infections. In this article, we’ll take a detailed look into the various symptoms of the disease.
To date, there is unfortunately no cure for cystic fibrosis. However, there are plenty of treatments which improve life expectancy, help clear mucus, provide nutritional support, and prevent infections. Treatments for CF aim to ensure adequate control over lung infections and the constant management and improvement of other symptoms. Apart from administering antibiotics, various nebulizers are used to deliver medication directly into the airways of mucolytic (liquefying) agents to improve bronchial patency (the ability of a patient to breathe). Additionally to these methods, periodic endobronchial toileting is desirable. Endobronchial toileting is the process of examining and cleaning the airways through procedures and exercises that clear the airways of secretions and mucus, which CF patients cannot do on their own due to their disease.
Inhaled medications through the use of a nebulizer play a key role in the prevention and treatment of symptoms on the respiratory side of the disease. The frequent need for inhalations in these patients necessitates the selection of a quality and reliable nebulizer. This is why Zano Inspire is donating a free nebulizer to patients diagnosed with cystic fibrosis in Albania, Bulgaria, North Macedonia, and Romania. The campaign aims to make life for all patients living with cystic fibrosis even a fraction easier and to provide children living with the disease a chance to have a normal childhood and healthy bronchial passages. To find out more information about the campaign, continue reading below.
Cystic fibrosis symptoms
Respiratory system symptoms
The respiratory system is the most significantly impacted part of the body in cystic fibrosis patients. The disease is characterized by the body’s inability to normally process chloride which results in the buildup of thick and sticky mucus in the walls of all airways. Patients cannot easily cough up the built up mucus themselves which can lead to the secretions becoming infected by various microorganisms that are difficult to treat. This can lead to the development of chronic pneumonia. It’s common for patients to have a prolonged moist cough with discharge of purulent sputum.
A large amount of patients develop severe and prolonged respiratory infections, which is unfortunately also the most common cause of death in CF patients. This is why maintaining clear airways and minimizing infections is of the utmost importance. Inhaling medications through the use of nebulizers has a positive effect on bronchial patency (the ability of a patient to breathe) Inhaled administration of medications has a positive effect on bronchial patency and ensures that the medication is targeted directly into the lungs and reducing any other side effects that could have occurred if the medication was circulated in the bloodstream.
Digestive system symptoms
One of the first symptoms which occurs in the first few hours after birth is meconium ileus. Meconim ileus is the inability to pass stool the first few days after birth. It’s often accompanied by vomiting and abdominal distension (significantly swollen abdomen). When or if these symptoms are observed, it’s highly recommended to have a screening examination to confirm or exclude the diagnosis.
As mentioned earlier, cystic fibrosis is a disease that is caused by impaired ion transport in epithelial cells. This is the body’s inability to normally process and move chloride and sodium ions due to mutations in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene. This means that the thick and sticky secretions are found not only in the bronchial tract, but also in the pancreas. Blockages in the pancreatic ducts leads to the deficiency of pancreatic enzymes and malabsorption. Patients will fall behind and struggle with their height and weight. Patients are also known to have frequent bulky and fatty stools. When the exocrine function of the pancreas is affected, patients are at risk of developing type 1 diabetes mellitus (CFRD).
Other digestive tract symptoms include:
- liver damage
- gallstones
- prolapsed rectum
- abdominal pain
Symptoms from other organs and systems
Frequently observed in CF patients (especially children) is stunted growth due to their frequent infections and insufficient pancreatic enzymes needed for nutrient absorption. A high calorie diet must be followed, and enzyme replacement therapy must be taken to compensate for decreased pancreatic function. In most patients it’s also imperative to take fat-soluble vitamins such as vitamin K, A, D and E.
Patients who are assigned male at birth struggle with reproductive system issues. Many males are born with congenital bilateral absence of the vas deferens (CBAVD), meaning the tubes that carry sperm from the testes to the semen are either blocked or missing. As a result, their semen contains no sperm, leading to infertility. The majority of female patients have no direct issues with fertility the way male patients do, but many struggle with conceiving due to irregular menstrual cycles and thicker mucous secretion through which spermatozoa have difficulty passing. Pregnancy is well tolerated, with exceptions if there are severe lung infections.
Impaired sweat gland function leads to increased sodium and chlorine concentrations in sweat. This makes patients have salty skin, especially observed by patients when kissing. The chronic nature of the disease has its psychological consequences for patients and their families. Frequent lung infections necessitate frequent hospitalizations and school absences. It’s not uncommon for a psychologist to be involved as a part of a patients team of doctors. Cystic fibrosis requires constant rehabilitation, diet and medication.
How to relieve symptoms
Exercise and respiratory therapies
Cystic fibrosis is a multi-system disease and therefore requires a team of specialists to monitor a patient’s progress. Physiotherapist plays a key role in this diverse team. It has been scientifically proven that physical activities and sports have a beneficial effect in cystic fibrosis patients.
Thoracic physiotherapy is commonly performed by patients. It includes assuming various positions which natural drain the bronchial passages. Each position is tactically chosen so that most of the lung is facing downwards which helps with drainage. Percussion goes hand in hand with thoracic physiotherapy – percussion includes a relative or rehabilitation practitioner gentle tapping or vibrating onto the chest wall. This helps move secretions to the larger bronchioles and outwards. Chest physiotherapy is recommended to last between 20 and 40 minutes per session.
Physical exercise also has numerous benefits for lung function. Some include improved posture, and musculature is strengthened and oxygen supply to all cells is increased. This leads to an improvement in the overall functioning of the body. Increasing lung resistance and its proper drainage lead to less infections and an overall better quality of life.
Physical activity also improves glucose uptake in the peripheral tissues which helps to better control diabetes mellitus. Strengthening the musculoskeletal system and cardiac function improves patients’ lifestyle and physical development.
It is recommended to start training with a gradual workload dependent on age and fitness appropriate exercises. The duration, intensity and frequency of exercise are strictly individual and will change over time. A easy and simple way to get patients started in their exercise journey can be through creating a simple schedule such as the following:
- walking 10,000 steps a day
- training 3 times a week
- swimming
- practice their favourite dances or try new ones from Just Dance
Progressively increasing the intensity and repetitions of an exercise is a great incentive for the patient to not give up and set new goals. No matter the type of core workout that is chosen it is advisable to complete each session with stretching exercises which help improve muscle strength and tone, prevent injury and normalize respiratory rate.
Nutrition and hydration
A good training and rehabilitation program should be combined with an appropriately balanced diet. Gaining weight is always a priority for patients due to their issues with growth and nutrition. This is achieved through a high calorie diet that includes a sufficient amount of salt to replace losses. Due to inadequate amounts of pancreatic enzymes, enzyme replacement therapy is needed for better lipid absorption. Take fat-soluble vitamin supplements (vitamin K, A, D and E) are almost always required.
It’s also very important that patients are well hydrated. Due to the rapid rate they lose salt through their sweat, they are extremely prone to dehydrate often and quickly. When practicing any type of sport or exercise, a sufficient amount of water should be nearby to replenish their lost fluids. To avoid dehydration in such cases, it’s best to provide adequate fluid intake before, during, and after the exercise itself. It is also important to consume a sufficient amount of salt to replenish water loss and electrolytes. Various ready-made electrolyte solutions can be taken or found in the form of salty foods – chips, nuts, cheese, etc.
The role of Nebulizers
Administering medications through the use of a nebulizer provides CF patients a wide range of benefits. Through inhaling their medications, the drugs are delivered directly into the airways in an optimal concentration. The daily use of targeted nebulizer medication reduces side effects that would typically affect the whole body if taken any other way. In recent years, significant progress has been made towards making more medications accessible for nebulizers – specifically those used for the treatment of chronic infections (antibiotics, bronchodilators, etc.).
Due to the frequency of their inhalations patients need to be able to rely on a quality nebulizer. The Zano Inspire nebulizer generates optimally sized particles to ensure the effectiveness of each therapy session. It’s compact in size, operates at a low noise level (55db), and comes with interesting custom stickers. Zano Inspire is suitable for both children and adults. It is also suitable for daily use to sooth the most common respiratory symptoms.
The complex treatment that comes with cystic fibrosis requires a good team of specialists and carefully chosen therapy plan. Ensuring sufficient hydration, a balanced high-calorie diet and adequate physical activity should be a part of every patient’s daily therapy. All of this when combined with specifically chosen medications that reduce any pulmonary complications and a quality nebulizer such as the Zano Inspire should ensure relief from otherwise heavy symptoms. This is also why Zano is donating a free nebulizer to all patients diagnosed with cystic fibrosis. To find out more about the campaign, click here.
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FAQ:
When can children start using nebulizers?
- Inhalation therapy has been used for the smallest and youngest of patients as early as premature infants. Therefore, there are no age restrictions for the use of inhalation therapies. But there are age restrictions for some pharmacological elements. Consult your personal physician before using any medication.
What is the survival rate of patients with cystic fibrosis?
- With good control over their disease (every CF patient has their own individual issues and needs) and keeping respiratory infections to a minimum, many patients live well into adulthood.
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