This post is only for education purposes , always contact a registered physician or professional for any medical advice. CYSTIC FIBROSIS Cystic fibrosis is the most common, life-threatening, recessively inherited disease in the western world. Most common in Caucasians, it affects 1 in every 2500 Caucasians, whilst 1 in 25 carry the gene. Fifty years ago a baby born with cystic fibrosis (CF) may have only lived for a few months, now 75% of babies live on to adulthood .Scullion, J., & Robinson, T. (2009)
Cystic fibrosis (CF) is a hereditary condition that mostly affects the lungs but also affects the pancreas, liver, kidneys, and gut. The CF gene causes an abnormality in production of a specific protein found in the lining of cells especially those in the lungs and bowels. As a result, thick secretions are produced, causing health consequences .Scullion, J., & Robinson, T. (2009)
Long-term consequences include repeated lung infections , difficulty breathing and coughing up mucus . Sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males are further signs and symptoms. Acid reflux may also occur. Pancreatic insufficiency may lead to growth insufficiency leading to malnutrition. By time one may also develop a form of diabetes. Different persons may experience varying degrees of symptoms.
CF is an inherited disease one is born with; the abnormal gene is recessive meaning that 2 defected copies from the parents are needed for a child to be born with CF. If both defective CF copies are present in the parents, there is only a 25% chance the child will have CF, 50% chance of having the gene as a carrier but no CF symptoms and 25% chance of not even having the gene.
Diagnosis
Generally, CF is diagnosed at birth but some cases are diagnosed at an older age. At birth patients may present with a blockage of bowels caused by sticky secretions.
Diagnosis is made from a positive sweat test, this sweat test reads the sodium level in the sweat, an elevated sodium level generally means one has CF. Other forms of diagnosis may come from a surgical biopsy sample. Also a CT or high resolution CT scan may be taken to confirm the diagnosis with imaging.
Nutrition
For both long-term survival and a high quality of life, adequate nourishment is essential. Evidence points to a close connection between lung function and dietary status (Peckham and Conway, 2004; Hodson and Geddes, 2000; Cystic Fibrosis Trust, 2002a). Patients should attempt to consume 20–50% more calories per day than the national recommendations for their age and sex since they need a high-energy diet. Many patients' protein needs are twice what is advised for adults. Nutritional support must become more vigorous as lung function declines; this may involve overnight gastrostomy or nasogastric tube feeding. Eating calorie and protein dense foods will help one to reach one’s goals by eating. less.
Physiotherapy
Physiotherapy treatments should be adapted to each patient's needs, thus referring to a physiotherapist is essential (Peebles, 1998). In CF, a patient's preferred physiotherapy technique promotes compliance (Pryor, 1999). Chest clearance procedures include coughing exercises. Timing of medication with chest physiotherapy is also critical; for example, nebulized bronchodilators before physiotherapy. Cardiovascular fitness is essential to help reduce shortness of breath (Peebles, 1998). Strength workouts maintain muscle strength and function, allowing patients to use oxygen better (O'Neill et al., 1987). A CF physiotherapist must assess all patients routinely.
Kindly refer to your physiotherapist or doctor prior to trying out the exercises in the video. Remember to breathe in and out with each force exertion and to stop if you feel in pain or short of breath.
Thick sputum causes infection and lung damage. Physiotherapy helps clear sputum and reduces infection (Webber & p 1993). Chronic coughing leads to exhaustion and chest pain, thus effectively clearing the lungs is important. Physiotherapy targets progressive lung disease's reduced exercise ability and breathlessness (Bradley and Moran, 2002). CF patients might also have joint discomfort, notably back pain, and females can have stress urine incontinence due to weak pelvic floor muscles from coughing (Orr et al., 2001).
CF physiotherapy aims to help the patient to clear their chest with minimal coughing, to get fit, to ease shortness of breath, to reduce joint pain and to eliminate urine leakage. Overall Physiotherapy is essential to CF patients so that they can maintain independence and quality of life through controlling symptoms.
The video shows multiple airway clearance techniques , it is vital to show this video to your physio and doctor prior to trying them out yourself.
Cystic Fibrosis treatment
Although there is currently no cure for cystic fibrosis(CF), there are several treatments that can help manage symptoms, lessen the likelihood of complications, and make life with the disease more manageable.CF requires daily, lifelong care. Early treatment encourages independence by maximising lung function and nutrition. Repeated respiratory infections burden CF patients. Thus such patients are treated with prolonged oral or at times intravenous antibiotics through a permanent line or port. Home intravenous antibiotics treatments (HIVAT) is a viable and successful alternative to hospital treatment for many CF patients, according to several studies (Pond et al., 1995; Bradley et al., 1999; Esmond et al., 2002; Riethmuller et al., 2002). HIVAT reduces the risk of hospital-acquired infections, improves quality of life, and reduces time off school or work (Littlewood, 2000; Marco et al., 2001). It is best to consult with your physician to find the best solution that suits you /the patient best.
As recently as 2021, the government of Malta ensured that a new drug from the company Vertex ,was to be given to patients. This new drug allowed the faulty gene that causes cystic fibrosis to be tweaked so that it can function properly, decreasing the negative symptoms of cystic fibrosis.
Other medications (including inhalers) help to make mucus thinner and easier to cough up, open the airways and reduce inflammation. Drugs that improve digestion and absorption in conjunction with a healthy diet and nutritional supplements to ward off malnutrition may also be administered. Special procedures and equipment may be done to help clear mucus from the lungs.
Equipment for Chest Physiotherapy
A mask or portable mouthpiece is used to create positive expiratory pressure (PEP). PEP devices do not restrict airflow during inhalation but do so during exhalation. To overcome the air pressure, you need exhale more forcefully. Against this barrier, exhalation takes around four times as long as inhalation. This aids in dislodging mucus from the lining of the lungs and airways by allowing air to pass through it. Similarly, it prevents your airways from closing by stopping them from collapsing through positive pressure in the lungs. Such a technique could also be created through pursed lip breathing, though it would be best to refer to your physician and physiotherapist.
For oscillating positive airway pressure (PEP), the patient repeatedly exhales completely into a device. These oscillating PEP devices, which go by brand names like "Flutter," "Acapella," "AerobikA," and "RC-Cornet," have two modes of operation. First, like non-oscillating PEP devices, they use resistance to make exhaling more challenging. Additionally, exhaling causes vibrations from an oscillating PEP gadget. The vibrations help to dislodge mucus from the airway's surface. The user then huffs to expel the mucus from their lungs after exhaling multiple times through the device. People normally take 10 breaths before stopping to cough or huff cough mucus out of the airways during PEP, though your respiratory therapist may recommend a different method based on your specific circumstances.
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