Cystic Fibrosis is an inherited life-threatening disorder that damages the lungs and digestive system. Cystic Fibrosis is a disease where mucus fills the lungs and doesn’t allow for a person to be able to absorb nutrients property. This is chronic disease that causes multiple health issues, and has no cure to date. Patients with C.
F. go though painful treatments, and take daily medications, to be able to maintain a moderately healthy life, which is also a constant struggle. Any persons born with this terminal disease must gain a copy of the Cystic Fibrosis gene from each parent (“Cystic,” umm.edu 1).
If only one parent has the disease, the trait or is a carrier; there is a fifty-fifty chance that the child will be born with the same trait. In the United States Cystic Fibrosis is the most fatal and most common inherited disease among Caucasian Americans with millions of Americans unaware they even carry the defective gene (“Cystic,” umm.edu 1; “Cystic,” Hereditary 45). Cystic Fibrosis affects one in twenty-nine Americans with over thirty-thousand known carriers among children and adults (“Cystic,” umm.edu 1;”About” 1).
SymptomsC.F signs and symptoms to vary from person to person. An individual’s symptoms may not be the same as others or even as they had previously experience in the past. Symptoms can either worsen or improve based on the person’s severity of the disease. Some people may not experience any symptoms until adolescence or even adulthood whereas some symptoms could begin in shortly after birth. This cause has now made screening for cystic fibrosis to be done in newborns as routine in the United States. The most obvious and known symptoms and signs of Cystic Fibrosis will be the salty tasting skin, poor development as seen by evidence of slow growth and low weight even if the diet is normal, thick, sticky mucus accumulation, frequently develop lung infections, a persistent cough that produces thick secretions known as sputum, inflamed nasal passages or a stuffy nose, breathlessness or wheezing and a decreased ability to exercise.
During infancy or childhood, some of the first symptoms that may appear are a bowel obstruction that was caused by the meconium ileus in newborn babies. A mutated protein in the ciliated epithelial cells leads to an abnormal production of viscous mucus (National Institute of Health, 2012). It is important for children to exercise in order that the mucous from the alveoli is released. A child with poor growth will such as not being able to gain weight/height at the same rate as other children may go unnoticed for having cystic fibrosis until they are seen by a doctor who is concerned in finding out the reason for the poor growth. Multifactorial and chronic lung infection, increased metabolic demands from the body because of severe illness, and poor absorption through the gastrointestinal tract of nutrients are some of the causes of growth failure in children. The viscous, thick mucus could also block ducts that carry digestive enzymes to your pancreas which releases to your small intestine. The lack of these digestive enzymes, proper digestion and absorption cannot happen which also will lead to foul-smelling, greasy stools, intestinal blockage (meconium ileus), and severe constipation.
A rectal prolapse may be a sign for cystic fibrosis because of the frequent straining while passing stool(National Institute of Health, 2012).DiagnosisDiagnosing Cystic Fibrosis is done by a sweat chloride test. Chloride is an electrolyte that our body’s produce that releases salt through sweating). The is done to access a patient who is suspected of having Cystic Fibrosis based on signs and symptoms the patients presents with. It is a common and noninvasive test that measures levels of Chloride (salt), which should be between 0 – 29 mmol/L for infants from birth to six months and between 0 – 39 mmol/L for infants 6 months to adults (2018 Johns Hopkins Cystic Fibrosis Center). Any levels above 60 mmol/L is indicative of a C.F.
Diagnosis. In present day, patients are usually diagnosed by the age of two but, a few rare causes are not diagnosed until the age of eighteen (“Cystic,” umm.edu 1). Since symptoms usually appear early in life patients that are not diagnosed until later in life often have more mild forms of Cystic Fibrosis (Silverstein, Silverstein, and Silverstein 1; “Cystic,” umm.edu 1). Luckily today children with Cystic Fibrosis are diagnosed early, in the nineteen-forties children often died from Cystic Fibrosis before they were old enough to attend school (“About” 1). Even though testing for Cystic Fibrosis is exceedingly simple the list of complications and illnesses associated with Cystic Fibrosis is extensive. TreatmentThere is only treatment for Cystic Fibrosis has no cure.
But over the years, the treatments have improved for someone living with the condition. Some of the goals however are: preventing and controlling lung infections, loosening and removing thick, sticky mucous from the lungs, preventing or treating blockages in the intestines, making sure to receive adequate nutrition and most importantly, staying hydrated. Some common lung problem treatments could include CPT (chest physical therapy) which involves pounding on your chest and back in order for the mucus to loosen from your lungs so that it could be coughed up. An inflatable therapy vest that uses high-frequency airwaves that push the mucus from the depths of your lungs toward your upper airway to cough up. There are many more similar tools that help dislodge mucus to be coughed up.
In some instances, lung transplants may be required after an evaluation is done by a Cystic Fibrosis care team. Chest Physical Therapy (CPT) consists of three components postural drainage, percussion and vibration. These type of clearance is started in infancy and childhood. It is a passive form of treatment for the patient and an active one for the caregiver. A good analogy for CPT is getting ketchup out of a ketchup bottle.
You turn the bottle over (postural drainage), hit it on the side of the bottle to loosen the ketchup (percussion), and then shake the bottle to allow the ketchup to flow out of the bottle (vibration). Postural drainage technique uses a series of gravity dependent positions to drain mucus from the lungs. Positions are limited for infants, keeping them upright or side-lying because they tend to spit up. Older children can tolerate all of the positions that an adult performs. Patients with reflux disease should avoid any head-down positions, as it may increase reflux.Percussion is when ssing cupped hands or a manual device, caregivers create a hollow clapping sound to loosen lung secretions.
For infants, the percussion is made with 2-3 fingers, a bottle nipple, or a percussor cup, and is performed for approximately 2-5 minutes in each position. For older children and adults, CPT is performed using the whole hand, and even doing both sides simultaneously, for a session lasting 25-30 minutes.Vibration is by using their hands or manual devices on the chest, caregivers can vibrate or shake secretions from lung walls, allowing the patient to clear them by coughing. In infants, this force can be created by using “Tickle Me” toys, a bouncy seat with vibration, or bouncing the baby on your knee. Using a trampoline, tumbling, performing wheel barrow races, and tickling are good ways to achieve this with older children. Creating a vibration force in adults is done using one or two hands to vibrate over the specific lung segment.PEP Therapy known as Positive Expiratory Pressure (PEP) therapy is done by breathing through a device that provides resistance while exhaling.
This pressure keeps the small airways of the lungs open to improve the mobility of the secretions. There are many different forms of PEP therapy: Acapella, Flutter, TheraPEP, and Bubble PEP, are some. Each treatment should be done twice a day for 15-20 minutes.Acapella is when oscillating device provides pressure to shake secretions from the lung walls. It comes in two different levels of PEP (blue for younger children and green for older children and adults), each adjustable.
The Acapella can be used in any position and can be combined with postural drainage positions. The Acapella is great for kids starting PEP therapy.Flutter is unlike the Acapella, this oscillating PEP device does not come in different levels of PEP and is not adjustable.
Therefore, it’s more difficult for younger children to use.TheraPEP this PEP device comes with a visual coach to assist the patient with the exercise. There is no oscillation, but the device has six different settings, which make it easier for younger children to use.
Bubble PEP this is an easy, cheap and fun way for children to do their airway clearance every day. To make a bubble PEP, fill an empty milk jug half way with water; then insert a rubber tube for the child to blow through. Picture blowing bubbles in a glass of milk. Food coloring can be added to make it even more fun for the kids, friends and family.
ConculsionIt is most important for infants to be tested when symptoms are noticed at an early age for