Sickle Cell Disease

Acute Complications
-Sickle cell pain: Diagnosis of exclusion!
-Fever: Patients are functionally asplenic, prone to sepsis. Give antibiotics.
-Chest pain/dyspnea: Acute chest syndrome (leading cause of death), PE
-Headache: CVA ischemic (children and older) or hemorrhagic
-Abdominal pain: 70-75% of adults with SS have gallstones, intrahepatic cholestasis
-Bone pain: avascular necrosis, osteomyelitis

-Gold standard for pain assessment: patient report (no objective fining HR/RR can be used)
-Significant psychosocial needs
-Progressive neurologic disease: overt CVA or silent infarcts leading to cognitive decline (may not understand treatment)

NIH Guidelines for SCD
1.Triage as high priority (ESI 2)
2.Check for signs of other complications (aplastic crisis, neurologic event, sepsis, osteomyelitis)
3.Treat pain aggressively (helps to prevent splinting which can lead to ACS)
-Begin analgesic management <30 min of triage
-Give IV opioids every 15-30 minutes until pain relief is obtained
-Use non-pharmacologic approaches such as heat
4.Begin PCA in ED when possible

-Educate all staff, particularly triage nurses on SCD complications
-SCD pain protocols to streamline care, triage as ESI 2
-Start PCAs for those being admitted
-Pediatrics: Intranasal fentanyl 1st, oral opioid trial if considering discharge
-Adults: Individualized care plans, Sickle cell disease order set for IV pain medication dosing

-Benefits of Hydroxyurea in Adult SCD: Pain and ACS reduced by 40-50%
-HbF induction and anti-sickling medications
-Many trials currently in the pipeline


Pearls and Pitfalls of Writing Discharge Instructions in the ED

Critical part of patient care (~80% patients discharged)

-Language should be plain, easily understood: “Return to the ED immediately for any new of concerning symptoms or worsening of your current symptoms.”
-A picture is worse a 1,000 words
-Give written and verbal instructions
-Teach back, have the patient confirm understandings
-Time and action specific follow up plan
-Future: video discharge instructions

Essential Elements:
2.ED workup and care
3.Post-ED care
4.Follow up
5.Return Instructions

-Pre fabricated discharge instructions
-Leaving out essential elements
-Using complicated language, abbreviations, or medical jargon

Additional Resources: Discharge Instructions – EMRAP 2016


Where are you FROM from? The Discriminatory Patient
-35% of 377 Canadian FM residents surveyed experienced intimidation – race, gender, culture – during training
-35% of these incidents were patient interactions alone, 25% related to race or culture
-59% physicians received discriminatory comments , majority felt a lack of institutional guidance or support on how to handle these incidents (online survey data)

1964 Civil Rights Act, Title VII
-Employees have a right to a workplace free of discrimination
-Independent contractors not covered under Title VII

NEJM: Dealing with Racist Patients

The Discriminatory Patient and Family: Strategies to Address Discriminatory Patients
1. Ignore comments in an emergency
2.Focus on the shared goals of treatment
3.Depersonalize the event
4.Foster a community of support within the hospital

Penn State Hershey Medical Center – Penn State Health
-Will not allow switching of providers based on patient preferences (race, ethnicity, religion, sexual orientation, gender identity)

Mount Sinai Hospital: Are you an ALLY?


Ethics Discussion
-Think about patients as moral agents, capable of making decisions, including bad decisions.
-Among patients with substance use disorders, many have serious self-worth issues.
-Consequentialism/Utilitarianism: Do the most good for the largest number of people.
-Virtue Ethics: Good people do benevolent things
-Policies can be narrow and limited, be aware of them, but use case by case judgement in decision making
-Avoid punitive responses


Pediatric Oral Findings
Hand Foot Mouth (and butt) Disease = Erythematous macules, progressing to vesicles
-Supportive care, analgesia

-Coxsackie A6: very contagious strain, affects adults as well, wide lesion distribution, consider admitting eczema toxicum for IV antivirals

Measles, Koplik spots = Millimeters, erythematous blue=-white or grey speaks on the on the buccal mucosa
Call department of public health!
Herd immunity = 92-95% Vaccination rate needed
In MA, High poverty = low vaccination rate

Herpangina = <10 yellow/greyish white papulo-vesicular lesions on soft palate
Many viruses possible, serious cases caused by Enterovirus A71

Three good things
-Write down 3 things in the day that were good, and WHY!
-Duke study: 15% reduction in burnout among residents, lasting effect 1 year later


Resident Author: Jeffrey Rixe, MD