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Audio Transcript Auto-generated
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hi and welcome to a cystic fibrosis.
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101 I'm Laura Pennington on the new nurse practitioner for
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the CF Clinic, and I'm here to give you just
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a quick review of a really complex disease.
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So here we go.
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Cystic fibrosis is caused by several different gene mutations that
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control the flow of sodium chloride and bicarbonate across cell
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membranes, which causes problems everywhere.
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Really remember that water follows salt, which is sodium chloride
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in CF.
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AA lot of chloride stays inside the cells, but sodium
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does not.
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So there's not sodium chloride on the outside of the
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cell, and water doesn't cross the cell membrane very well.
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This croc causes secretions to be really thick in multiple
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organ systems in the loans.
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Thes secretions make the patients prone to infection because they're
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so thick that they even bob down the silliest so
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that Celia can't get the bacteria out of the airways
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and thus the bacteria just sit there and multiply.
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This is a nice diagram of what happens.
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This is a normal CF for CFTR channel, where the
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sodium and chloride just flow nicely.
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Here, look at this.
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Chloride is inside the cell, so there's some sodium out
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here, but not chloride.
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Thus no sodium chloride.
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Thus really, really thick mucus.
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Pardon me.
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This causes problems everywhere in the lungs.
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Those secretions gum up the cilia so that they can't
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get rid of bacteria.
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Bacteria sit there in the airways and multiply and can
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cause scarring in the pancreas.
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Those secretions block pancreatic ducks and cause scar tissue in
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the G I tract water and nutrients have trouble passing
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through the intestinal wall in the bones.
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Pour water soluble vitamin absorption, including vitamin D slows, bone
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formation and speeds.
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Bone loss.
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This should really be called pulmonary treatments.
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My apologies.
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Uh, airway clearance is done by vibrating the chest.
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This could be done with CPT or with a therapy
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vest that the patients might bring in.
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Huffing is a special way of coughing that helps bring
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up secretions.
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Regular deep coughing again mobilizes secretions pretty well, and so
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does exercise, which, as you know, causes nice deep breaths
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and opens the airways and constant relate secretions.
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More therapies done by rt our beta agonists like l
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beauty roll.
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Unbelievable. Beautiful.
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This illustration it says pathology of asthma.
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But I still think it's a nice, uh wayto visualize
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how these medications open the airways.
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Another therapy is using hyper tonic sailing.
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Uh, putting a lot of sodium chloride into the airways
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draws water across those membranes, and that thins secretions.
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It's also a little bit irritating to the airways, which
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stimulates coughing, which mobilizes secretions.
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A lot of CF patients are colonized with pseudomonas, uh,
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and they will use S Tree and M for 28
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days on 28 days off.
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Or they will use a combination of Tober myson and
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probably mixing E 28 days and 20 or 28 days
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on 28 days off.
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Palm design is a medication that thins mucous by breaking
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up its DNA and makes it easier to expel.
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So again, respiratory therapy will do these treatments, usually up
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to four times a day, in somebody who's having a
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nexus survey shin for um, in the pancreas, those thick
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secretions again cause scarring.
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That way, the pancreas can't produced digestive enzymes well enough
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or produce enough insulin.
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Often times these patients are not.
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They're not controlled with a good commander regimen.
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They'll have their own home regimen, and you may have
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to advocate on their behalf to continue that and not
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be put on glue commander.
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So just be aware because they don't produce enough enzymes.
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They take supplemental enzymes before they eat pangkor like paces.
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Given there are different forms with different names and different
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dosages, the patients will know their regimen, and they should
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have their supplements at bedside so that they don't have
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to call you all the time.
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Further digestive issues or constipation or diarrhea, constipation is the
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most dangerous one.
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There's a syndrome known and CF patients called dios, or
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distal intestinal obstruction syndrome.
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Because water isn't transported across the membranes very well, the
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stool becomes hard and dry, blocking the intestine.
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Your patients should have at least one bowel movement a
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day and will have a regimen in place to help
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promote that osmotic laxatives.
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Such a select ILO's or Merrill axe are used.
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Gastro graph in is used with some patients.
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Stimulant laxatives, such a center and mutual itics to help
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thin the secretions that block water transport, such as a
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seagull Sistine are used sometimes with this condition.
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Surgery is obviously a last resort that air to prevent
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problems than treat them.
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Cystic fibrosis patients with certain mutations are lucky enough to
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be able to use CFTR conduct INTs regulators.
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Not all not all mutations could be treated, but for
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those patients that can take regulators there often life changing.
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There are four that were current went using, try CAFTA
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Politico or Combi and some Deco.
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They're each used for a different type of mutation, and
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what they do is just help.
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Help Trent that transport perform a little bit better.
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Here are just some pearls for your nursing care for
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these patients.
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Uh, the way that they're admitted, some tests to expect
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and nursing care that you need to be aware of
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at this time with Kobe going on their first admitted
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to the same team to rule out Cove it.
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Once they're rolled out for Cove it, they're transferred to
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the heavy metals team, either silver, copper or gold that
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the admitting team will consult Coleman Ology.
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They'll consult me the C F A, P P and
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the CF dietitian, um, who consults with the floor nutritionists
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to make sure that they're getting what their what they
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need and I will call the Four West Charge are
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in to give a heads up so that these patients
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could be given the proper room.
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They'll need to be on contact precautions with a mask.
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Some are gonna be on droplet precautions, depending on what
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their colonized with.
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They should all be in a private room, of course,
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and they can leave their room.
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They just need to wash their hands where a mask
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and were clean.
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Hospital gown.
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Some nursing considerations You'll need to follow contact precautions with
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masks. These patients will have a central line s so
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you'll have to do central line care you're gonna be
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getting. Definitely I v antibiotics.
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Sometimes Pio antibiotics don't be need toe.
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You'll need to monitor blood glucose because of their pancreatic
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insufficiency. They'll need daily weights and no need to monitor
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the consistency and color of their stools.
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And again, they should be having at least one stole
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per day.
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Some more things to remember.
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Leave their pancreatic enzymes at bedside, and they'll know their
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pancreas or their enzyme regimen.
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If the enzymes are ordered differently by the provider, you'll
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have to advocate for the patient and ask for the
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patients. Usual dose and all CF patients, because they absorb
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food products so poorly, should have a double portion high
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fat diet, not a diabetic diet, and again see if
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patients know their illness and you may have to advocate
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for them to maintain their usual home medication regimen.
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Ah, a couple psycho social considerations.
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They're usually well versed in their illness, and they know
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their medication regimen really well.
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So if they come across as pushy, it's It's not
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you. It's just that they've had to fight to get
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their usual medications at the hospital before, usually, so you
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just may need to be a go between and home.
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Talk to the provider.
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If he or she is not providing the patient's usual
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medications, who do you call?
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You can call me.
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It's best to use Tiger Connect because I'm not always
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in my office.
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I'm here Monday through Friday.
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Eight. If I and you can call me with any
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questions, I may not know the answer, but I'll try
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to find out for you, the Five West Charge nurse,
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since that's where CF patients used to be houses a
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good resource for you and her number.
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Is there his or her number?
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The respiratory therapist for CF patients is Abby Redway.
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Her number is there, and, um, you'll usually see her
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on the unit giving treatments.
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Tracy Trotter is our clinic office RN.
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She can answer a lot of questions for you.
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Kate. Hi is our social worker.
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That will work with your case manager.
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And our CF nutritionist will work with your floor.
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Nutritionists. Her name is Sonya Pie.
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As she's her number is right there.
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Here are pictures.
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This is me with the goofy grant up top.
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Our nutritionist, social worker, respiratory therapist and clinic coordinator.
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Some tests that your patient might need while they're there.
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They'll definitely need a sputum specimen for culture and sensitivity
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They will need or we'll have gotten a portable chest
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X ray for line placement.
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They might want a p A and lateral chest, X
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ray or chest C t to see what their lungs
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look like.
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Tell me, the CBC and CMP.
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Ah, blood culture.
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If they're if they If septicemia is suspected, they'll need
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a liver function tests.
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They may get a bronchoscopy.
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They will definitely get pulmonary function tests to see how
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well they're exchanging air.
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And if they're on Ivy October or Banca Myson, they'll
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need peaks and troughs.
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I just wanted to expand a little bit on the
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roles of our team members.
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The CF dietitian serves as a consultant for nutritionists on
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the floor, and she also make makes recommendations for pancreatic
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enzymes and insulin for these patients.
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A respiratory therapist will be administering nebulizer medications, and she
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also helps coordinate discharge medications and supplies.
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She performs Pft S and she'll obtain your sputum cultures.
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I hope that this really brief overview has been helpful.
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Please don't hesitate to call her office at any time.
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If you have questions about these patients, thank you for
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your time and attention.