Cystic Fibrosis: Management (Pediatric Nursing)

00:01 So, how do we manage cystic fibrosis? Unfortunately, there is no cure, yet.

00:06 Treatment is focused on the management and on preserving your patient's lung function.

00:12 You can manage a lot of things with your patient.

00:14 The first is their respiratory status.

00:16 You're going to practice airway clearance techniques, and this is where the patient will be helped with postural drainage and percussion.

00:23 They'll go under chest physiotherapy and treatments to help shake up those secretions.

00:28 The patient's also going to be taking inhaled medications and this is going to help preserve their lung function.

00:34 Your patient's also going to take antibiotics to prevent infections, such as pneumonia, that can further compromise their respiratory status.

00:40 And there's new medications.

00:42 These are the CFTR modulators.

00:45 Regarding the management of your patient's nutritional status, they're going to need to take pancreatic enzyme supplements and this is because the pancreas gets clogged with this mucus and is not able to release these.

00:57 The patient's going to need to take pancreatic enzyme supplementation with every fat-containing meal and snack, and these are active for 45-60 minutes.

01:06 Your patients with cystic fibrosis are also going to be put on a high-calorie, high-fat diet because they require a lot more energy just to breathe and fight their lung infections.

01:16 The energy needs of people with cystic fibrosis are estimated to be 1 ½ to 2 times the needs of those without cystic fibrosis.

01:23 So, a high-calorie diet, high fat, and about 40% of their total calories from fat is generally recommended.

01:31 The next involves preventing infection.

01:34 This involves hand hygiene, also avoiding secondhand smoke, avoiding sick contacts.

01:41 Patients with cystic fibrosis should get their annual flu shot, and eventually, the patient may need a lung transplant.

01:48 Lung transplantation is the only definitive treatment option for patients with cystic fibrosis with end-stage lung disease, but it's still not a cure, because the defective gene that causes the disease is found in all of the cells of the patient's body, with the exception of their newly transplanted lung.

02:05 Here, on the left, you'll see a diseased lung being removed in a patient from cystic fibrosis.

02:10 On the right, you'll see the new fresh donated lung being transplanted into the recipient.