Cystic Fibrosis: Complications (Pediatric Nursing)

00:01 There are a lot of complications in patients with cystic fibrosis.

00:04 In the respiratory realm, this involves infective exacerbations of bronchiectasis, and this is a widening of the airways in the lungs caused by chronic inflammation and obstruction of the airways.

00:15 The patients may also get a spontaneous pneumothorax, or a collapsed lung.

00:20 The patients may have hemoptysis and may cough up blood because, over time, cystic fibrosis can cause thinning of the airway walls.

00:27 As a result, teenagers and adults with cystic fibrosis may cough up blood.

00:32 And this can also be caused by an infection in the lungs that's going to irritate the small blood vessels causing them to bleed.

00:39 The blood is going to mix in with the sputum and then this will be expectorated or coughed out.

00:43 This can be quite a frightening symptom for patients, but usually, the blood loss is minimal.

00:49 Patients may also have polyps and this is going to further obstruct their nasal passages and increase their risk for sinus infection.

00:56 The patient may go into respiratory failure.

01:00 They may develop cor pulmonale, and this is right-sided heart failure that develops due to complications from the high blood pressure in the pulmonary arteries, also known as pulmonary hypertension.

01:10 Their right ventricle will slowly enlarge and become less effective, and then ultimately, it will fail.

01:16 The patient may also have lobar lung collapse due to secretions.

01:20 IV antibiotics, physiotherapy, and the use of mucokinetic agents to get that mucus moving can allow that lobe to re-expand.

01:31 The patient will also have complications in their GI tract.

01:34 This includes malabsorption and steatorrhea due to the mucus blocking the effective use of their digestive enzymes.

01:41 The body is going to have a really hard time absorbing protein, fats, and fat soluble vitamins.

01:47 Steatorrhea refers to the fatty stools and it's the result of the impaired digestion and the absorption of the dietary fats.

01:55 The patient can also develop a distal intestinal obstruction syndrome, and this involves blockage of the intestines by thickened stool where the small intestine meets the large intestine.

02:05 Now this differs from constipation, and that the backup of stool is higher in the intestines and it can be the result of not taking enough of their digestive enzymes.

02:15 The patient can also develop biliary cirrhosis and portal hypertension and gallstones because the duct that carries the bile from the liver in the gallbladder to your small intestine will also become blocked and inflamed, leading to liver problems and gallstones.

02:30 There are other complications in patients with cystic fibrosis.

02:33 Patients can develop diabetes and this happens in about 25% of adult patients with cystic fibrosis.

02:39 It's called cystic fibrosis-related diabetes or CFRD, and these share some clinical features with both types 1 and types 2.

02:47 The thick mucus is going to cause scarring in the pancreas and the pancreas can still make some insulin, but not enough to be totally effective.

02:55 Patients will also experience delayed puberty.

02:59 Male patients with cystic fibrosis may experience infertility because the vas deferens, which is the tube that connects the testicles to the prostate, can become blocked with mucus or congenitally absent.

03:10 With the use of certain fertility treatments and procedures, it's possible for some men with cystic fibrosis to become biological fathers.

03:18 The patients may experience stress incontinence due to their forced repeated coughing.

03:23 Patients with cystic fibrosis can also develop psychological problems.

03:27 Remember, this is a chronic illness and there's an emotional burden associated with this disease.

03:32 Cystic fibrosis involves a stressful routine of treatments, and anxiety and depression are not uncommon.

03:40 The patient may also develop osteoporosis and this is decreased bone density, and this happens for a few reasons in patients with cystic fibrosis.

03:48 The first is malabsorption of the nutrients necessary to keep your bones strong.

03:52 The second is recurrent lung infections.

03:54 This is going to cause inflammation and that can interfere with the body's natural process of forming new bones to replace old, damaged bones.

04:02 The third is the decreased weight-bearing exercises due to exercise intolerance.

04:07 And the fourth, certain medications taken to manage the condition and its complications can affect the patient's bone health.