Sunday Funday POTD: Priapism - a hard problem to solve?

Background:

Named after the Greek god of fertility, Priapus, priapism is defined as an erection that lasts for longer than 4 hours that is not associated with sexual stimulation. Why do we care? Because 90% of men with persistent erections > 24 hours will have permanent erectile dysfunction. There are three types: non-ischemic, ischemic, and recurrent. 

Non-Ischemic ("high flow"):
The less common type, this is due to an excess of blood via a fistula between the cavernosal artery and the corpus cavernosum, usually in the setting of trauma. 

Ischemic ("low flow"):
The more common type, this is due to obstruction of venous outflow as a result of impaired relaxation of cavernosal smooth muscle. This is a urological emergency. 

Recurrent ("stuttering"):
A form of ischemic priapism that occurs in men with sickle cell disease. It's characterized by initially short erections that progressively worsen. 

Workup:
History should include: duration of current erection, prior episodes, any medications used (legal or otherwise), history of hematological disorders (specifically sickle cell), history of trauma, how severe the pain is. 

Your entire workup is used to distinguish between ischemic and non-ischemic priapism. The table below summarizes the characteristics. 

Treatment:

Call urology!! (if available)
If unavailable, believe it or not, high flow or non-ischemic priapism, can resolve spontaneously, actually does not require treatment, and can be managed as an outpatient by urology. 

First of all, analgesia should be provided. An easy way to do this is to perform a dorsal penile nerve block.

Steps:

1. Obtain consent, grab your materials (lidocaine), syringe, needle, alcohol/iodine, cleanse the area

2. Insert a small needle at the 2 o'clock and 10 o'clock position at the base of the penis

3. Feel for the pop of scarpa's (superficial) fascia

4. Aspirate and inject 2mL anesthesia in each position

This can also be done under ultrasound guidance!
Second, the American Urological Association recommends primary aspiration of cavernosal blood. To do this, insert an 18 gauge needle to the lateral aspect of the penis one in each corporal body. 30-60ccs of blood should be removed. 

If this is sufficient, stop here. If tumescence persists, phenylephrine injection may be attempted. This should be given in 1mL aliquots of a 100-500mcg/mL concentration. To mix this, take a vial of phenylephrine (10mg/mL) and take out 1mL, mix this in a 100mL bag of NS. To make it easier, don't take the needle out of the penis and simply attach a syringe with the medication to the needle. This may be repeated every 3-5 minutes for an hour until resolution. 

Fun fact: epinephrine can be used as well based on several case series. The dosing: 2mL of a 1:100,000 (10mcg/mL) concentration. This is essentially your push-dose dose (ie. 1mL of code cart epi in 9mL NS)

If this fails, the patient will need urological surgery... won't bore you with the details here!

What about sickle cell disease?

Priapism in sickle cell disease should be managed in the same way as ischemic priapism. It is rare that standard therapies do not work, but if they do not, exchange transfusion or simple transfusion can be considered. Don't forget to get a retic level in these patients!

TL;DR

• Ischemic priapism is painful and is a urological emergency

• Call urology consult!

• Provide analgesia/perform a dorsal nerve block

• Aspirate with two needles laterally

• Phenylephrine if this fails

• Further management with urology if this fails

• Consider exchange transfusion in patients with sickle cell disease with priapism that doesn't resolve with these measures

Sources:

https://www.uptodate.com/contents/priapism
http://www.emdocs.net/priapism-ed-pearls-pitfalls/
http://www.nuemblog.com/blog/priapism
https://www.uptodate.com/contents/priapism-and-erectile-dysfunction-in-sickle-cell-disease
https://www.asra.com/asra-news/article/151/peripheral-nerve-blocks-for-urologic-pro
https://www.ncbi.nlm.nih.gov/books/NBK535389/
https://www.ncbi.nlm.nih.gov/pubmed/11416834

Wellness Wednesday POTD: Imposter Syndrome

As the academic year is coming to and we all adjust to our new roles within our residency programs or as new attendings or fellows, I thought this would be a good time to talk about this.

Imposter Syndrome

Originally described by psychologists Suzanne Imes, PhD and Pauline Rose Clance, PhD in the 1970s, it is defined as an "internal experience of intellectual phoniness". Essentially, this means that people with imposter syndrome feel that their achievements are undeserved and worry about being "found out"that they are less than adequate despite evidence indicating success and/or competence. 

Dr. Clance described six potential characteristics:

1. The imposter cycle
   The cycle starts with a task, which is then met with anxiety, lead to either over-preparation for the task or procrastination (which is over-compensated with frenzied preparation). When the task is completed, there is relief, but this is short-lived despite positive feedback. Instead, the person believes that their success is either due to their hard work or luck, but not due to their ability. 

   The result is a feeling of self-doubt, depression, and anxiety and a tendency to overwork. 

2. The need to be special or the very best
   Those with imposter syndrome are secretly comparing themselves to others, which leads to a feeling of inadequacy.  

3. Super(wo)man aspects
   Related to the need to be the best, people with imposter syndrome set unrealistic goals for themselves. 

4. Fear of failure
   This can also be identified as the main motivational factor for most people with imposter syndrome. 

5. Denial of competence and discounting praise
   Adding onto feelings of inadequacy, those with imposter syndrome have difficulty internalizing success and will even make excuses about why praise is not deserved. 

6. Fear and guilt about success
   Although people with imposter syndrome crave success, they also fear it because it makes them feel isolated in their success. They also fear taking on more responsibilities as they're more likely to be "found out" with higher expectations. 

This was further elucidated by Dr. Valerie Young who broke down the syndrome into five different personality types in her book The Secret Thoughts of Successful Women:

1. Perfectionists: people who set unrealistic goals and feel like failures despite the level of completion of these goals

2. Experts: people who need to know every piece of information and will overeducate themselves. They are also afraid of looking stupid and will hesitate to assert themselves

3. Natural geniuses: people that are used to achieve success effortlessly, which leads to feelings of inadequacy when any effort is needed

4. Soloists: people who feel that asking for help is a sign of failure

5. Super(wo)men: people who need to work harder than everyone else around them in order to succeed in all aspects of life
   

How to deal with imposter syndrome

As with any problem, the first step is identifying and recognizing the problem. From there, overcoming imposter syndrome requires a lot of self-reflection and much of this comes from reframing your mindset on what qualifies as success. Some potential methods:

• Seek help: this can be found in a mentor, a friend, or a therapist. Vocalizing feelings and concerns can help in several ways. First, it can help identify characteristics that are typical of imposter syndrome, which can lead to increased self-awareness. Talking can also help with the realization that imposter syndrome is not an uncommon occurrence, which helps to normalize the condition. 

• Lean to internalize validation: people with imposter syndrome tend to dismiss positive feedback. Learning to reframe your mindset by resisting this response to positive feedback can help put things into perspective.  

• Be realistic about expectations: the expectations that those with imposter syndrome set for themselves are unrealistic. It is important to realize that nobody is perfect and to properly reflect on one's own successes. Likewise, it's important to recognize that everyone has strengths and weakness and to reflect on one's strengths and not to see weaknesses as failures. 

• Figure out your true goals: it's possible the goals that you've set for yourself would not actually ones that would make you happy. Take stock in what really matters and that may also help to redirect your ambitions. 

Sources:
Abrams A. Yes, Imposter Syndrome is Real. Here’s How to Deal With It. Time Website. http://time.com/5312483/how-to-deal-with-impostor-syndrome/
Roche J. 10 Ways to Overcome Imposter Syndrome. The Shriver Report Website. http://shriverreport.org/10-ways-to-overcome-impostor-syndrome-joyce-roche/
Sakulku J, Alexander J. The imposter phenomenon. International Journal of Behavioral Science. 2011;6(1):75-97.
Weir K. Feel Like a Fraud?. American Psychological Association Website. https://www.apa.org/gradpsych/2013/11/fraud
Wilding M. 5 Different Types of Imposter Syndrome (and 5 Ways to Battle Each One). The Muse Website. https://www.themuse.com/advice/5-different-types-of-imposter-syndrome-and-5-ways-to-battle-each-one

POTD: Measles (Part 2)

Part two in our two part series about measles! 

Again, TL;DR at the bottom and here's another plug for Dr. Anna Pickens' EM in 5: http://www.emdocs.net/em-in-5-measles/

This section goes over the diagnosis, management, and complications of measles. 

Transmission
Measles virus is a single-stranded, enveloped, RNA virus of the genus Morbillivirus within the family Paramyxoviridae. It is spread via respiratory droplets that may remain in the air for up to two hours. 

Clinical Presentation
Incubation period: 6-21 days (median 13 days)

Prodrome (days 2-4): fever, malaise, and anorexia followed by “the 3C’s” (conjunctivitis, coryza, and cough). This phase of infection can last up to 8 days. 

Koplik spots typically present 48 hours prior to the onset of the exanthem. They are white/gray/bluish elevations, described as “grains of salt” on an erythematous base. These are typically seen on the buccal mucosa, but may spread to the soft and hard palates. These generally last for 12-72 hours. 

Exanthem: starts 2-4 days after onset of fever. Classically is a blanching, maculopapular rash that starts on the hairline and progresses downward and outwards to the extremities. It tends to coalesce and become non-blanching with time. 

Patients will become clinically better within 48 hours of the appearance of rash, the rash will darken in color, and eventually desquamate. 

Measles may vary in severity and there are several clinical variants including: modified measles (milder symptoms) in those with pre-existing measles immunity, those who have received IVIG, and in babies with passive immunity from placental migration of immunoglobulins; atypical measles in those who have received the killed virus vaccine (not seen frequently now), which is characterized by higher and more prolonged fevers, pneumonitis, and transaminitis. Patients that are immunocompromised will also not present classically. 

Complications
Immunocompromised patients and pregnant patients are more likely to develop complications. 

Superimposed infection is common because T-cells and dendritic cells are directly infected, which leads to immune suppression that can persist for up to three years. Infections include:

• Otitis media

• Gastrointestinal (most common) - diarrhea, gingivostomatitis, appendicitis

• Pulmonary (most common cause of death) - bronchopneumonia, croup, bronchiolitis

• Neurologic

  • Encephalitis: occurs several days after rash. Patients have neurodevelopmental sequelae in 25% of cases, fatal in 15% of cases

  • Acute Disseminated Encephalomyelitis (ADEM): occurs several weeks after rash. Demyelinating disease likely due to immune response to the virus. Fatal in 10-20% of cases and survivors commonly have residual neurologic abnormalities. 

  • Subacute Sclerosing Panencephalitis (SSPE): occurs 7-10 years after infection. More likely the younger the time of infection

    • Stage I (weeks-years): insidious neurological symptoms (trouble concentrating, lethargy, personality changes, strange behavior)

    • Stage II (3-12 months): dementia, myoclonus

    • Stage III (variable): myoclonus resolves, neurologic function deteriorates leading to flaccidity/decorticate rigidity, autonomic dysfunction

    • Stage IV: death

Diagnosis:
First off, isolate your patient if you suspect measles!! Place the patient in a negative pressure room. Despite the high efficacy of MMR, there is still a 1% chance that you are not immune. As such, everybody entering the room should wear an N95 mask and the patient should wear a mask during transport. 

Test used depends on the prevalence of disease and the local governing body for infection control. In general, IgM and IgG are tested in the serum and a nasopharyngeal swab should be obtained for serological testing. False positive PCR does not rule out infection. 

Treatment:
Mainly supportive and treating any superimposed bacterial infections. In children, they tend to have low vitamin A levels, which can contribute to delayed recovery and more complications. Low vitamin A levels also causes blindness in children in the developing world. As such, children benefit from vitamin A supplementation. Ribavirin can also be considered especially for higher risk individuals (< 12 months, requiring ventilatory support, and severe immunosuppression). 

TL;DR:

• Measles is transmitted airborne, stays in the air for 2 hours

• Characterized by a prodrome of fever, malaise, coryza, conjunctivitis, and cough for 2-4 days followed by a maculopapular rash that progresses downward

• Measles can be complicated by bacterial infections. Most common cause of death is from pneumonia

• Long-term effects include severe neurological sequelae: encephalitis, acute disseminated encephalomeningitis, and subacute sclerosing panencephalitis

• Diagnose via IgM, IgG, nasopharyngal swab

• Treatment primarily supportive

• Consider vitamin A and ribavirin

Sources:
https://www.uptodate.com/contents/measles-clinical-manifestations-diagnosis-treatment-and-prevention
http://www.emdocs.net/em-in-5-measles/
http://epmonthly.com/article/ready-for-the-measles-comeback/