End-Stage Idiopathic Pulmonary Fibrosis

by Andria RN · 2 comments

in ICU,Uncategorized

What I learned and what you might be able to learn from my 4/10 and 4/11 nightshift in the ICU.

Shave your patient before this happens!

Room 1: 82 year old male with pneumonia that had been extubated 3 days prior to my shift and he had transfer orders to move out of the ICU. He waited for a bed all night long and was stable so there were not many good learning opportunities…he was pretty bored being in the ICU so I spent about an hour with him and shaved his beard and mustache. It’s amazing the little things you can do to make a patient feel better.

Room 2: 52 year old male patient, recently diagnosed with Idiopathic Pulmonary Fibrosis in Jan 2011. Idiopathic just means we don’t know why it happens. He had no other PMH besides HTN which was well controlled with daily Cozaar. At home this patient was starting to rely more and more heavily on oxygen until he was on 6L at home with SOBOE. IPF is essentially a scarring of the lungs and it can progress slowly over years or rapidly as in this case study- in fact, this patient is now considered end-stage IPF and his only chance is if he receives a bilateral lung transplant. Again, IPF causes stiff lungs and inflammation. One of our thoracic surgeons described removing an end-stage IPF lung from the chest as comparable to scraping spackle off of a wall.

Normal Lung vs IPF Lung

Idiopathic Pulmonary Fibrosis











If you admit a patient with IPF, you’ll probably see the MD order a CXR, CT of the chest, and possibly a lung biopsy to determine if the disease processes is causing rapid deterioration or is somewhat stable. IPF can cause chronic hypoxemia (secondary to poor gas exchange through the stiff lungs), Pulmonary HTN and right sided heart failure (Cor Pulmonale). My patient had an ABG drawn on admission and was found to be in respiratory acidosis because his body was unable to rid itself of CO2 despite the fact that his respiratory rate was anywhere from mid 20’s-40’s. He was placed on a non-rebreather and came up to the ICU on a high flow nasal cannula 60L 100% FiO2. I haven’t seen the high flow nasal cannulas very often in the ICU. It is my understanding that they are used to deliver continuous flow of oxygen while allowing the patient to eat, drink and speak. They are much less cumbersome to a patient (and the nurse!) than a BiPap or CPAP but they can’t deliver a set volume. Auscultating the lungs revealed course crackles through upper, middle and lower lobes. Once again, a result of all that stickiness and stiffness.

High Flow Nasal Cannula

The patient also had a 2D Echo. If you’ve never seen a 2D Echo, the ultrasound tech comes to the patients bedside and does an ultrasound of the heart. The Cardiology team can then get important information from looking at the ultrasound- they can estimate the patient’s ejection fraction (RN’s like it when the EF is 50-55%! because we know the patient can handle fluid), the patient’s pulmonary pressures, and whether or not there is any stenosis or regurgitation in the valves, etc. Luckily, the patient’s 2D Echo was normal. He is currently in the ICU pending a work up by the Transplant Team to decide whether he is a candidate for a lung transplant. The patient gets a full work up to determine whether there are an underlying issues like cancer, heart issues and even psychosocial issues like family support. A patient undergoing a transplant surgery has a long road ahead. They will be required to take immunosuppressant drugs and are at very high risk of infection which limits the activities they can partake in. The family has to be very willing to support their loved one before and after the surgery.

Quick assessment:

Neuro: Completely intact with air hunger anxiety treated with PRN Ativan IV and scheduled Xanax PO. Activity is as tolerated, patients should be encouraged to maintain a daily schedule (up in the chair during the day) and possibly working with PT/OT and Pulmonary Rehab if they’re able to handle the exertion.



Resp: High flow nasal cannula. Lungs with crackles throughout. RR 18-20 while asleep, and anywhere from low 20’s to 40’s while awake. Patient currently able to speak in full sentences but breathing is labored. O2 sats are low to mid 90’s and patient will occasionally desat into the low 80’s after a period of exertion, anxiety, or coughing fit. *Typically IPF patients recover very slowly from an episode of desaturation. It may take close to 5 minutes for a patient to return to their baseline O2 sats if not longer. Give them a chance to recover before you call the MD. If they continue to desaturate or maintain a low O2 sat, THEN let the MD know.* Patient has a hacking cough that occasionally produces a thick white sputum. Patient receives Mucomyst PO in attempts to lower the viscosity of his mucous secretions (probably mostly cellular debris from the stiff lungs) and to help him cough up sputum instead of letting it sit in his lungs and cause mucous plugs. Patient also has Tessalon ordered PRN which is supposed to suppress the cough reflex by slightly numbing the lungs and airways. It comes in a small pearl form and is supposed to be swallowed whole, not sucked on or chewed.

Tessalon Pearls: Don’t Chew or Suck!

CV: Sinus Rhythm in the 80’s while calm, during desaturation and anxious periods, HR up to 120’s. Pulses palpable, good coloration (we don’t want our lung patients turning blue!), and no audible murmur. BP stable.



GI/GU: Regular high protein diet ordered. Patients need strength and nutrition to keep up their energy before and after the surgery. The work of breathing uses a lot of energy which burns calories AND it hinders the eating process. Imagine yourself trying to eat a full meal after you just held your breath for 60 seconds and you might get the idea of what it feels like. Some of our Pulmonologists even order Pediatric diets for lung patients to encourage higher calories and to get patients to eat more (yes, most patients would rather eat a hamburger than slightly cold pureed carrots). Good bowel sounds, is able to void in urinal and is continent of stool. Patient is also getting AC/HS blood sugar checks because he is on steroid therapy which increases blood glucose. His sugars run in the low 200’s.

Skin: intact and patient is able to reposition himself (when does that ever happen in the ICU??)

Family: Very supportive wife and family came to visit from out of town.

**Families can sometimes add to the patient’s anxiety levels even though they mean well- it is hard for a family to watch their loved one struggle to breath and they feel helpless and they look to the nurse to “fix” the patient but the patient needs to try to relax and breathe steadily to recover from an episode of desaturation. I’ve had to ask a family to step out before and I explained it in a way they understood. After the patient was more calm, the family came back in with instructions to provide minimal stimulus.

**I’ve also seen families cause anxiety in the patient by continuing to tell the patient to relax. It is difficult to empathize with an IPF patient but you have to try to remember that they hardly ever feel comfortable and are continuously short of breath- imagine how scary that would be and save some additional patience for your IPF patients.

It's not just Doctors that can make patients anxious, RN's and family members can too!

And that should explain a little about IPF patients. As they progress they may have to be placed on BiPAP or CPAP and intubation is often avoided as much as possible because this can actually take a patient off of the transplant list! Some IPF patients are placed on Nitric Oxide therapy- inhaling the NO vasodilates the airways that are still able to participate in gas exchange and ideally boosts gas exchange. Unfortunately there is no cure and a lung transplant may give a patient a few more years of life but transplant surgery also comes with its risks and obviously, post transplant patients are severely immunocompromised and highly susceptible to infection.

Nitric Oxide MOA

  • minurse

    my brother was just diagnosed with IPF. I am a nurse and had never heard of this disease. I am devastated and trying to learn as much as possible about this. BLOG was very helpful. Dr. has discussed the need for oxygen at home. is the high flow nasal cannula available for home use?

  • Andria RN

    I’m sorry to hear about your brother. Hopefully he has a less aggressive course. I think the high flow nasal cannula can be used for home use but normally reserved for patient’s who are pretty sick. You’d have to ask his physician if this is an option.

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