Cystic Fibrosis: Signs and Diagnosis (Pediatric Nursing)

00:01 What are the signs and symptoms of cystic fibrosis? Well, the most common symptoms are salty-tasting skin, patients with persistent coughing, patients will have shortness of breath.

00:13 They may have wheezing.

00:15 These patients are going to have poor weight gain, despite an excessive appetite.

00:20 These patients will have greasy, bulky stools, and they may have nasal polyps, or small, fleshy growths that grow inside the nose.

00:29 On exam, the provider is going to collect a full health history.

00:33 They're going to observe the patient's vital signs, and because of the situation, this patient may have tachypnea or an increased respiratory rate, and may also have hypoxia or decreased oxygen levels, and this is due to the mucus buildup.

00:45 You're going to do a full HEENT exam, so head, eyes, ears, nose, and throat.

00:52 The patient may have abdominal distension from bloating, and they may have digital clubbing, and this can happen due to the chronic hypoxia.

01:00 In the nose, the clinician may note nasal polyps, and the patient may have purulent nasal discharge, and this is an example of a sinus infection.

01:09 When you percuss the patient's sinuses, they may also be tender.

01:14 When you listen to the lungs, you may hear hyperresonant sounds, wheezing, crackles, a productive cough, and the patient may have purulent sputum.

01:24 You can also examine the patient by having them do lung function testing.

01:29 Monitoring a patient with cystic fibrosis is complex.

01:32 It requires the regular review by a multidisciplinary team at a specialist cystic fibrosis center.

01:39 These specialists include pediatricians or family physicians, nurses, physiotherapists, dietitians, pharmacists, and clinical psychologists.

01:53 The next part of the assessment of your patient with cystic fibrosis includes the clinical assessment.

01:58 This is where you review their clinical history.

02:00 You assess their adherence to their medications, including lung and GI medications.

02:05 You will do a full physical exam, and you will measure the weight and length or the height of your patient to track their growth.

02:13 Next, you'll do the lung function testing and this is where the spirometry comes in.

02:18 You'll check the FEV1 of your patient, and this is the forced expiratory volume in 1 second.

02:23 You'll also assess the FVC, which is the forced vital capacity.

02:27 If the spirometry is normal, you can do a lung clearance index.

02:33 The next part of the exam involves respiratory secretions samples, and this is to check for microbiologic investigations.

02:40 If possible, the patient will expectorate sputum to be sent to the lab.

02:45 You can also obtain a nasopharyngeal swab.

02:49 And you'll also assess the oxygen saturation of your patient to be sure that they're not hypoxic.

02:55 Cystic fibrosis is usually diagnosed through a sweat test.

02:58 Sweat is collected and the amount of chloride, a component of salt in the sweat, is measured.

03:03 A high level of chloride is an indication of cystic fibrosis.

03:08 So the newborn screen is a genetic test or a blood test.

03:11 If the patient scores a positive genetic test, they may have a flawed CFTR gene.

03:16 Then a blood test can be done to check for abnormal pancreatic function.

03:21 If the genetic or the blood tests are positive, the patient will undergo 2 sweat tests to confirm their diagnosis.