Li-Fraumeni Syndrome

Every now and then you all know that I like to share some of the things that I encounter. This week I encountered a disorder that I had never even heard of:

Li-Fraumeni Syndrome

If you are scratching your head at the name don’t worry, so was I! This is something I have never come across. You know me, when I don’t know… I am all over the internet until I do know.

I will say I had no idea such a brutal disorder existed.

Let’s get to the gist of what this syndrome is. The disease was first recognized back in 1969. Drs. Frederick  Li and Joseph Fraumeni were studying familial cancers and this study focused around four families that suffered with multiple cases of young adult and childhood cancers. It turned out that a mutation in a gene, TP53 to be exact, made them predisposed to cancers. Oh, while we are talking about the gene, it’s a tumor suppressing gene (figured I would throw that at you so it makes a little more sense). As if that wasn’t enough, it made the carriers more susceptible to rare cancers. We are talking cancers of the “soft-tissue, bone sarcomas, breast cancer, brain tumors, adrenocortical carcinoma and acute leukemia. Other cancers seen in LFS patients include gastrointestinal cancers and cancers of the lung, kidney, thyroid, and skin, as well as in gonadal organs (ovarian, testicular, and prostate.)”(lfsassociation.org)  This article gives a lot of info

Li-Fraumeni Syndrome is an asshole.

It was the statistics surrounding the genetic mutation that shocked me the most. According to the LFS association, individuals with the mutation have nearly a 50% chance of developing cancer by the age of 40. It gets worse. The risk goes up to nearly 90% by age 60. However, hold on for this one, women have a nearly 100% chance of developing some type of cancer within their lifetime (much higher risk for breast cancer)!

Yeah, I was NOT ready for that particular statistic.

Like I said, Li-Fraumeni Syndrome is an asshole.

What should trigger oncologists to test for this mutation is family history. If a patient has a strong family history of the cancers listed above, especially if family members tend to get cancer at a young age (40 or younger), LFS should be looked at.

So, what made me do some research on LFS? A patient. I came across a young patient coming for a radiological study that had a tumor, while one of their parents was battling cancer as well. Yes, parent and child were both going through chemotherapy at the same time.

Once again, Li-Freumani is an asshole.

I happened to be a part of the care and saw some prior scans of the patient and had never really seen such a large tumor. Someone from the patient’s medical team happened to be down here with us and they were able to tell me the name of this syndrome.

I can’t even imagine what it would be like knowing that I carry such a genetic mutation. Would I ever want to have children? If I decided to have children, would I feel guilty if I passed it on to them? I had this discussion with some of my coworkers and we all had differing views. So bloggaverse, I ask you two things:

  1. Have you ever heard of LFS?
  2. Would you ever have children if you knew you carried the mutation?
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Unnecessary

I almost lost my temper.

One of the anesthesiologist does NOT know how to talk to people.

It feels like she is being condescending at all times. I get it, you’re a doctor. I respect that. However, it’s totally unnecessary to speak to people in that manner. I am a nurse. If you talk to me instead of at me you would know I have plenty of nursing experience. I know quite a bit more than you give me credit for. I am fully capable of the tasks that are required of me during this anesthesia case. I can do whatever you need if you just ask. Contrary to popular belief, nurses don’t read minds.

I’ll say this, she has one more time to speak in her condescending tone. One more. While I am going to be professional and respectful, I’m going to put her in her place. I’ve had to do it with plenty of residents and a few attendings. This isn’t new for me, I know how to get my point across. She needs to be knocked off the pedestal she placed herself on… I’m just the one to do it.

Appreciated

A while ago my nurse manager came down to the department I was in to check on me.

My nurse manager came to check on me.

The day was turning into a hot mess and I had to get help from our sister nursing department to help get things back on track and help me put out the flames. After things started to calm down, my nurse manager came down to where I was to check on me and do you know what she said? “Fred, I am so glad I hired you.”

Say. What?

Did… Did she just show appreciation for how hard I was working?

HOLY HELL MANAGERS DO THAT?!?

Turns out, good management does. I cannot even count how many times either my nurse clinician or nurse manager has popped up in the department giving us updates or just checking in.

I have never really had that happen before. In fact, I was so unused to it that the first time my nurse clinician came down to the department checking in I thought I was in trouble! I really thought I had gotten reported for something and was about to be written up! That is how little I was used to seeing management (unless they were asking if we updated our whiteboards).

I was talking to another floor nurse, in fact, the one from the last blog, and telling her about management coming and checking in and she was also flabbergasted. She, too, was only used to seeing management whenever there was an issue.

That’s sad.

I don’t place 100% of the blame on management, though. I know they are encountering the same problem. Their higher-ups only want to discuss what they are doing wrong, give them unrealistic expectations, and unrealistic time-frames to complete the unrealistic expectations. Meeting after meeting they get bombarded with complaints. It’s a miserable existence and I can totally understand why so many nurse managers leave the job.

This is a bigger problem with how hospitals are a business focused more on numbers than patients. It trickles down. Miserable management creates miserable staff, and that leads to the high turnover rates in the nursing field.

No one feels appreciated and that needs to change.

I am lucky enough to work in a department where I actually feel appreciated. Yes, we have our foolishness just like every other area in the hospital. However, I find myself far less stressed in this position. I want more nurses to be able to feel this way. I want it to get to a point where seeing management becomes a positive thing. I wonder how we, as a group, can change this?

That’s… unfortunate

A majority of my patients cannot read and know nothing about their medical care.

That’s… unfortunate.

In fact, it’s scary.

My patients have to fill out a checklist before having their MRI or CT scan. It asks numerous questions about prior procedures and certain health issues.

So many of my patients can’t fill out the questionnaire. In fact, a lot of my patients don’t even know why they are having the scans! They are here because they have an appointment. They don’t know which doctor ordered the scan, what is getting scanned, or what the particular doctor even does for them. It’s sort of the mindset that “if the doctor ordered it then I should do it”, no questions asked.

That is frightening. Those of you that have been following me know I am big on patient education. With how fast paced my department is, I don’t have the time I would like to have to educate patients. And let’s be real, at this point I can’t teach someone to read. I guess what is so disappointing to me is the fact that it’s just glossed over. It’s accepted. The lack of patient education, understanding, and participation has become the new norm. I can’t stand it. I want patients to understand what is going on. I want patients to be a part of their plan of care. I want patients to be set up for success.

Apparently, I want to live in the NCLEX world where everything is perfect and everything runs smoothly.

I want my patients to be happy and healthy. Sometimes I feel like I am being unrealistic.

 

Burn out

I had a nurse shadowing me that was applying for a position in radiology. She seemed very nice and very knowledgeable. She is currently working at the bedside and decided it was time for a change. We began conversing about the job I currently do and how different it was from bedside nursing. Let’s be honest, my job can have chaotic moments but for the most part it is chill. I wanted to hear more about what made her want to transfer into our department.

Surprise, surprise… She was burned out. She started sharing why she was burned out. She felt unappreciated. She felt mentally exhausted. She was frustrated. I knew exactly how she felt. We swapped stories of our nights of hell. She was curious as to what made me leave the ICU and transfer to radiology. I was honest… I was burned the hell out at the bedside! I worked bedside for eight years. Eight years of endlessly cleaning poop, call bells ringing simultaneously, angry family members, unsafe staffing ratios, little to no lunch break, and management asking “did you update you white boards?”. I realized I was just over it. Now I will say this: I loved working in the STICU. It was hell on wheels some nights but I learned so much.

And that’s the thing, I feel like walking through the nursing “flames” made me a better and more rounded nurse. At this point I can handle just about anything you can throw at me. Being a beside nurse is what really made me a good nurse. While it was stressful, I don’t think I would change anything if I could go back in time and do so. However, I realized I was done and exited bedside nursing stage left.

I recognized I was burned out. I felt it. I could see the change in my patient and family interactions. I literally drove to work with anxiety because I just KNEW the night was going to be a sh*t show. I had to take benadryl just to sleep. Things were not okay. So I made a change. It looks like she is ready to make a change. I commend her for recognizing that. In fact, I commend any nurse that recognizes they have reached the burn out stage. More than that I deeply respect nurses that not only recognize they are burned out, they start making the necessary changes to beat burn out. Know when you feel burned out, it is okay. It is just fine to leave the situation you’re in. You are not running. You are not “abandoning” anyone. You are doing what is best for you.

Have any of you (nurse or not) ever had to leave your job because you knew it was making you miserable?

GFR

Now that I am in the radiology department I spend a lot of time focusing on GFR and kidney function. Why? Good question!

In MRI and CT we give contrast to a lot of patients. In CT the contrast is iodine based. In MRI the contrast is gadolinium (metal) based. Both types of contrasts are filtered out through the kidneys and thus the reason kidney function is so important in this department. The way we assess kidney function is by checking a patient’s creatinine level in their blood. Luckily for us we have machine called the i-Stat that can test the blood and give a result in two minutes. The result transfers into Cerner (our EMR) and the computer then uses that result to calculate the GFR. Great… except I didn’t really have an understanding of why we were checking the creatinine, what GFR really was, or why there is a GFR result for African Americans and non-African Americans. I decided to do a little reasearch and I figured, since this is a nursing blog and all, why don’t I share what I have learned?

What is “GFR”?

GFR stands for glomerular filtration rate. Basically, the GFR tells you the flow rate of fluids through the kidney. Your glomeruli are the capillaries in your nephrons inside the kidney. Blood is filtered across the capillary membranes helping to remove waste that can ultimately be excreted through the urine. Taking you back to anatomy and physiology in nursing school aren’t I? *shudders*

A simple google search will bring up lots of GFR calculators. Typically the GFR calculator takes into account serum creatinine, age, gender, and race (African American versus not) and then it will give you the estimated GFR. A GFR >60 indicates a generally healthy kidney. Less than 60 can indicate potential kidney disease. Less than 15 can indicate full on failure. Here is a little infographic that is patient centered.

Why creatinine?

Why does the GFR equation use creatinine? In the most basic terms, creatinine is a waste product of creatine. Creatine is used by the muscle cells for energy. Your kidneys help filter the creatinine out of the blood to be excreted in the urine. Low creatinine typically indicates good kidney function (which makes sense, healthy kidneys will filter out creatinine effectively). High creatinine indicates the opposite, kidney function is probably on the lower end because the kidneys are unable to filter out the waste product. Creatinine is primarily filtered out through the kidneys which is why it is a pretty good indicator of kidney function.

Why is the result different based on race?

Many, many times I have looked at my labs and wondered why the GFR had a result for African Americans and then essentially everyone else. It wasn’t until I started working here and paying attention to the GFR that I decided to look it up. Turns out studies show we have “higher than average” muscle mass so we generate higher levels of creatinine. Higher creatinine levels lead to higher filtration rates. The difference in results account for this.

Now I can actually explain to my patients why I am taking blood after I start an IV. I like to be able to asnwer my patient’s questions so of course I had to do a little learning on my end. Hopefully some of you will also find this information useful! (Also here is a great reference for frequently asked questions from the National Kidney Foundation because, why not!)

A moment of prayer

How does it make you feel when the family of a patient starts to pray with you in the room? I used to feel uncomfortable because for a long time my relationship with religion has been iffy at best.

See, I am a bisexual woman that was raised Baptist. Yeah, “gay people are not of God and are going to burn in Hell” Baptist. My mom was a pretty liberal woman but our religion was not. I only came out two years ago but was well aware of my sexuality as early as high school. Religion and I didn’t sit well since I was pretty much condemned to Hell. This personal struggle affected how I reacted whenever my patient began to speak about religion or whenever anyone wanted to pray in the room. Typically it turned into “let me page the chaplain” as I awkwardly slid out of the room. I was allowing my own issues to affect my patient care. NOT OK! I really had to get it together. It took a lot of introspection and acceptance of what religion  means to me, and understanding that religion is different for everyone, before I became comfortable with religion in the hospital.

Standing and bowing my head while a family member is praying shows respect. I don’t have to pray like they pray or pray to who they are praying to. I can bow my head and pray for my patient in my own way. I can now listen to my patient talk about their faith and have an engaging conversation with them. Instead of religion making me feel like I was condemned and judged, I now look at it differently. I had to realize my patient was speaking from their point of view. They are sharing aspects of what religion means to them. At no point was my patient judging me. I know it sounds strange but when you are in the LGBTQ community, you tend to feel judged a lot simply for being who you are. I had to understand my patient had no idea about my sexuality and honestly, with what they are going through at the moment, they probably could care less! They are looking for hope. They are clinging to faith to get through a difficult time. They are coping with whatever is going on and for a lot of people, religion is the best way for them to cope.

This wasn’t about me. To bring my own insecurities into this was selfish! I was being so egocentric. I am not normally like that so why be like that now? I really had to make some adjustments to how I thought about religion. I had to learn that at that moment my patient needed someone to listen to them, to give them hope, to have empathy instead of just sympathy. At that moment, my patient needed Fred the nurse to be there for them.

I had to learn that it’s not all about me.

Look at me when I’m talking to you!

I am going to vent for a moment so bare with me okay?

I ABSOLUTELY HATE WHEN MY PATIENT DOES NOT LOOK AT ME EVEN ONCE WHEN I AM SPEAKING TO THEM!

This has nothing to do with eye contact. I know for some people, eye contact is uncomfortable or unusual in their culture. I get that. However, when I call someone into my IV chair and they can’t bother to put their  phone down long enough to raise their head and answer my questions it burns me up! I just feel like it is so disrespectful! Is that how they converse with everyone? No, I highly doubt it. I think *that* is what bothers me the most. I am simply trying to provide care within my environment. I didn’t force them to come to this hospital, nor did I force them to make an appointment for whatever reason they are here. I feel like the least someone can do is acknowledge that a human being is standing in front of them providing care.

There have been times when I am trying to go over information with a patient and they are so engrossed in whatever is happening on their phone that they have a hard time answering my questions. Typically this statement will get me the acknowledgment I prefer: “Let me know when you are done on your phone and then I I’ll continue.” After that I take a step back and wait. Patients will typically put the phone down and pay attention.

In all honesty, I don’t need their undivided attention the entire time they are in my care. Since I am the radiology nurse, I am going to be the one to go over the contrast questionnaire with the patient and then I will obtain vascular access. This isn’t dramatic stuff here. I really only need the patient to pay attention when I am asking them questions, after that I actually prefer they occupy themselves because most often it means they’ll focus on their phone and not on the 20g I am about to stab them with.

I don’t know, maybe I’m just getting old or something but a little acknowledgment wouldn’t hurt.

Uniform… Acceptance…

The hospital I work for has a uniform policy. As nurses we wear ceil blue and/or white. I hated the idea of uniforms… At first.

Now, I kind of like the fact that each department in our hospital has a uniform.

Yeah, it surprised the hell out of me too!

It helps me know who I’m talking to or who just walked into my patient’s room. I’ve often had patients say, “the doctor said I can have something to eat!”, however I haven’t seen the docs come onto the unit. Now I’m trying to figure out who my patient was actually talking to so I can find out what was actually said. With everyone being in uniform I can ask my patients “what color uniform were they in?” I cannot tell you how many times I’ve asked that question and then find out it was xray technician that came in to do the morning portable chest xray that the patient talked to! For a lot of our patients, anyone in scrubs is a doctor.

The fact that I can identify a department just by their scrubs is a real help and as much as I hate to admit it, uniforms made things a lot easier. I only have one big complaint, THESE COLORS!!!!

I despise the ceil blue/white combo. I would really prefer a darker color. Something like a hunter green or a navy blue would work for me but it is what it is.

So tell me, what policy did you initially hate that you’ve learned to accept and perhaps even like?

n00b

So you’re the newest person on the unit. You may not be new to nursing but you are new to this place. Don’t let that freak you out.

As the newbie I was always really quiet and reserved. What I didn’t realize was how off-putting that was to others. It never failed, once I got to know my coworkers they would all say the same thing “I thought you were so mean when I first met you!” For the longest time I didn’t understand why that seemed to be the case. You know me though, if I don’t know then I’m going to ask. That is when I found out that I sort of appeared unapproachable because of how I tended to distance myself from my new coworkers. I changed that once I started the new position I am currently in. This time I made a conscious effort to get to know my coworkers.

Here are a few steps to transition into your new work environment:

  • Introduce yourself to your new coworkers. If you know of a good ice breaker then use it (having the name Shaunelle but being called Fred is one hell of an ice breaker, everyone loves the story of how I got my nickname.)
  • If you are an experienced nurse understand this: your experience is much appreciated but you are the new nurse on the unit. Don’t walk around like you already know everything there is to know. You may know cardiac ICU but you don’t know how they run their cardiac ICU. Have a little humility (ran into this with a new coworker, she didn’t last long).
  • Don’t be afraid to ask questions. It is safer to ask than to assume you know what you are doing and then do it wrong. Your new coworkers will remember that screw up, trust me.
  • If you are a super proactive person, join one of the committees. You are bound to meet your coworkers that way.
  • Become familiar with your physicians and introduce yourself to them. Let them know you are new to the unit, I mean you will be working with them too.
  • Know that not everyone is going to warm up to you immediately and that’s okay. They may still be “feeling you out” so to speak. That is not your problem, that is theirs. They’ll either come around or they won’t. It’s not the end of your world. However, DO NOT allow bullying behavior towards you. You may be the newbie but you deserve respect and if they want it from you then they should earn it. Forget that “nurses eat their young crap”!

Those first few weeks, hell even first few months, are a weird time. You’re trying to adjust to a whole new setting. Things may feel off and that is normal. You may feel a little out of place at first, and that’s normal too. Give yourself time to get acclimated to your new surroundings, you are going to do great!