CPM Spring 2019 - 14

daup h i n cm s .org
.org

The most common symptom requiring
medical attention is an acute painful episode,
commonly called a "SCD crisis" but now
preferably referred to as an acute vaso-occlusive pain event. Many patients with SCD
experience frequent pain events that they
manage at home with oral medications,
ranging from Tylenol to a non-steroidal
such as ibuprofen to oral opioids (morphine,
hydromorphone, e.g.). Most patients that
present to the Emergency Department for
treatment of an acute pain event require
parenteral opioids - home treatment has
either failed or the event escalated too
rapidly for a home treatment regimen.
One of the major challenges facing
patients with SCD is the difficulty receiving rapid and effective analgesia when
experiencing an acute vaso-occlusive event.
Patients with SCD were perceived by many
in the medical community as "drug seekers"
prior to the opioid crisis. This challenge
has escalated in the face of the opioid
crisis. It has also negatively affected the
large percentage of patients with SCD who
experience chronic pain, many of whom
require chronic opioids. Unfortunately,
these patient encounters with the health
care system are also impacted by implicit
and explicit racial and gender biases. An
atmosphere of mistrust between patients
and health care providers ensues which
does not portend for optimal emergent or
continuity of care.

Other complications relate to the psy- Evidence-Based Management of SCD -
chosocial impact of any lifelong chronic Expert Panel Report 2014 (www.nhlbi.nih.
illness compounded by inequities in health gov/guidelines). Professional societies have
care and society at large.
excellent resource materials: the American
Society of Hematology, the American
College of Physicians and the American
Academy of Family Physicians. UpToDate
also has several excellent articles on the
management of SCD and its wide range
of complications.

This brief article
highlighting World
Sickle Cell Awareness
Day and some
of the challenges
encountered in the US
healthcare system for
those living with SCD,
hopefully will be
the first in a series
on SCD.

I write from the perspective of a patient
living with SCD and a physician who has
treated many living with the disease. Even
as recently as February I personally had to
fight to receive adequate emergent care in
my medical community. This is 2019 in
America - why does this remain an issue?
If as a physician I am unable to receive the
In addition to acute and chronic pain, standard of care, how much less my patients
there is a myriad of complications associated who may not have a voice?
with SCD. They include: stroke, acute chest
syndrome, pulmonary hypertension, sickle
I encourage readers to begin their journey
lung disease, cardiomegaly, acute coronary of increased SCD awareness and sensitivity
syndrome, retinal bleed (more common in to those affected by joining the South
HB SC), renal failure, hepatic sequestration, Central PA Sickle Cell Council, the Sickle
splenomegaly/splenic sequestration, func- Cell Disease Association of America, and,
tional asplenia, iron overload secondary to on World Sickle Cell Awareness Day, Junechronic transfusions, avascular necrosis teenth (June 19th), to "Shine The Light
(usually femoral or humeral head), leg ulcers, on Sickle Cell"! Information on "Shine
osteopenia/osteoporosis, osteomyelitis, gout, The Light on Sickle Cell" can be found at
vitamin D deficiency, priapism in men, sicklecelldisease.org.
severe dysmenorrhea in women, high risk
pregnancies. Some of these complications
Clinicians - you can begin by periodcan prove fatal.
ically reading some clinical information
on SCD. A readily accessible reference:
14 Spring 2019 Central PA Medicine

References:
1 Journal of Community Medicine And
Primary Health Care Vol. 29, No 1,
March 2017.
2 Regional Committee for Africa, 56.
(2006). Sickle-Cell Disease in the
African Region: Current Situation
and the Way Forward Report of the
Regional Director. WHO. Regional
Office for Africa. http://www.who.int/
iris/handle/10665/92663
3 Bull World Health Organ. 2008
Jun; 86(6): 480-487. Published
online 2008 Mar 4. doi: 10.2471/
BLT.06.036673
4 https://www.nhlbi.nih.gov/
health-topics/sickle-cell-disease

http://www.dauphincms.org http://www.nhlbi.nih http://www.who.int/ https://www.nhlbi.nih.gov/ http://www.sicklecelldisease.org

CPM Spring 2019

Table of Contents for the Digital Edition of CPM Spring 2019