A Report and Recap of Research Performed at HUCSCD

A Report and Recap of
Research Performed at the
Center for Sickle Cell Disease
Muftau Shinaba
School of Public Health
University of Maryland, College Park

Internship At-a-Glance
• Over the course of this Fall 2015 internship period,
I have embarked on the following:
• 2 IRB-approved research projects
• 1 Welcome packet
• Assisted on an upcoming patient registry
• Visit from Congo delegation
• Stomp Out Sickle Cell Walk
• International Conference on Stigma (HIV)

Understanding the Efficacy of
Hydroxyurea among Sickle Cell
Patients: A Questionnaire
Study
Patricia Oneal MD, Howard University Center for
Sickle Cell Disease,
Muftau Shinaba, University of Maryland, College Park

Key Terms
• Efficacy – the desired ability to produce a result
• Hydroxyurea – the lone drug approved by the Food
and Drug Administration for the management of
Sickle Cell Disease

Introduction
• Since 1998, Hydroxyurea has been recognized by
the Food and Drug Administration as the only drug
approved for effective treatment against Sickle Cell
Disease. It has resulted in a decrease in frequency
of sickle cell pain crises. However, the benefits of
hydroxyurea has been scrutinized and a poor
endorsement of this medication is presented
among providers. As a result, patients may have
limited knowledge when considering hydroxyurea
as an option.

Pros & Cons of Hydroxyurea
• Benefits
• Primary outcome is synthesis of fetal hemoglobin
• Increased oxygen affinity; decreased risk for pain crises
• Lower mortality in the long-term
• Potential Toxicity
• Decrease in leukocyte count in the bone marrow, increasing
susceptibility to infection
• Decrease in neutrophils
• Thrombocytopenia (All in relation to increased doses of
hydroxyurea)

What to look for
• The purpose is to study the effectiveness of
Hydroxyurea among sickle cell disease patients
enrolled at Howard University Hospital.
• How does sickle cell disease compare with
Hydroxyurea users vs. non-users?

Demographics (N = 23)
• Gender:
• Male – 47.8% (N = 12);
• Female – 52.2% (N = 12)
• Age: 18-63, Mean = 38.65, StDev = 13.73
• Race/Ethnicity:
• Black/African – 91.3% (N = 21)
• Native American – 4.3% (N = 1)
• Prefer not to answer – 4.3% (N = 1)
• Sickle Cell Status:
• SS – 69.6% (N = 16)
• SC – 17.4% (N = 4)
• SB+thal – 8.7% (N = 2)
• SB0thal – 4.3% (N = 1)

Critical Findings
0
5
10
15
20
25
Lifetime Current
Participants(N)
Hydroxyurea Usage
Yes No
87% (N = 20) have used Hydroxyurea; 73.9% (N = 17 currently takes Hydroxyurea)

Critical Findings
Mild Moderate Severe
Yes 12 7 8
No 4 9 8
0
2
4
6
8
10
12
14
Participants(N)
Occurrence and Degree of Pain Crises among Hydroxyurea users

Critical Findings
Yes 8
No 8
0
1
2
3
4
5
6
7
8
9
Participants(N)
In the last month, did you ever
forget to take Hydroxyurea?
0
0.5
1
1.5
2
2.5
3
3.5
4
4.5
2 days 3 days 6 days 7+ days
Missed Days
Reported Missed Days

Critical Findings
Yes No N/A
Do you believe Hydroxyurea helps
manage your sickle cell disease?
11 2 8
Do you believe Hydroxyurea helped in
reducing pain crises?
10 3 8
0
2
4
6
8
10
12
Efficacy of Hydroxyurea against Sickle Cell Disease (N = 21)

Critical Findings
Yes No N/A
Do you believe Hydroxyurea helps
manage your sickle cell disease? (N = 15)
10 0 5
Do you believe Hydroxyurea helped in
reducing pain crises? (N = 16)
9 2 5
0
2
4
6
8
10
12
Efficacy of Hydroxyurea against Sickle Cell Disease among Users

Critical Findings
• Women Only (N = 12)
• 25.0% (N = 3) believe
Hydroxyurea affects
ability to become
pregnant
• 16.7% (N = 2) became
pregnant while on
Hydroxyurea
• Men Only (N = 10)
• 40% (N = 4) believe
Hydroxyurea affects
ability to father a child
• 40% (N = 4) continued
Hydroxyurea once
knowing the status of
fatherhood
• 60% (N = 6) continued
Hydroxyurea while
trying to conceive a
child

Critical Findings
Reasons for starting
Hydroxyurea
• Anemia (N = 8, 36.4%)
• Frequent pain crises (N = 15, 68.2%)
• Stroke (N = 0, 0.0%)
• Acute Chest Syndrome (N = 4, 18.2%)
• Other (please specify) (N = 0, 0.0%)
• Unknown/Not Applicable (N = 4,
18.2%) 0
2
4
6
8
10
12
14
16
Frequency(N)

Critical Findings
0
5
10
15
20
25
Medication History Types of Medications
• Folic Acid (21, 91.3%)
• Morphine (8, 34.8%)
• Oxycontin (7, 30.4%)
• Oxycodone (7, 30.4%)
• Dilaudid (9, 39.1%)
• Fentanyl (3, 13.0%)
• Methadone (0, 0.0%)
• Exjade/Jadenu (0, 0.0%)
• Percocet (3, 13.0%)
• Tramadol (1, 4.3%)
• Other (please specify) (2,
8.7%)

Discussion
• Should providers discuss Hydroxyurea for ALL sickle
cell patients, regardless of circumstance?
• What do you think are the reasons for a lack of
communication among patients and providers
when discussing said treatment options?

References
• Lanzkron S, Strouse JJ, Wilson R, Beach MC, Haywood C, Park H, ... Segal JB.
(2008). Systematic review: Hydroxyurea for the treatment of adults with sickle
cell disease. Annals Of Internal Medicine, 148(12), 939-55.
• Segal, J. B., Johns Hopkins University, & United States. (2008). Hydroxyurea for
the treatment of sickle cell disease (Evidence report/technology assessment, no.
165; AHRQ publication, no. 08-E 007; Evidence report/technology assessment,
no. 165; AHRQ publication, no. 08-E 007). Rockville, Md.: Agency for Healthcare
Research and Quality. http://purl.access.gpo.gov/GPO/LPS91282
• Steinberg, M., McCarthy, W., Castro, O., Ballas, S., Armstrong, F., Smith, W., …
Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia and
MSH Patients’ Follow-up. (2010). The Risks and Benefits of Long-term Use of
Hydroxyurea in Sickle Cell Anemia: A 17.5 Year Follow-Up. American Journal of
Hematology, 85(6), 403–408. http://doi.org/10.1002/ajh.21699
• Strouse, J. J. and Heeney, M. M. (2012), Hydroxyurea for the treatment of sickle
cell disease: Efficacy, barriers, toxicity, and management in children. Pediatr.
Blood Cancer, 59: 365–371. doi: 10.1002/pbc.24178

Understanding Attitudes
and Beliefs of Cannabis Use
among Sickle Cell Patients
Patricia Oneal MD, Howard University Center for
Sickle Cell Disease,
Muftau Shinaba, University of Maryland, College Park

Key Terms
• Cannabis (marijuana) – a plant-based drug
• Schedule I drug – the most restrictive classification
set by the Drug Enforcement Agency
• “get high” – a state of euphoria induced by use of
select substances

Introduction
• Cannabis, or marijuana, is one drug that has roots
to ancient times in terms of its medicinal purposes.
In recent studies, the use of cannabis may present
benefits to those suffering from myriad diseases,
including sickle cell disease; however, cannabis
remains an illicit drug in the United States and
other countries. With many states passing laws
decriminalizing the Schedule 1 drug or allowing it
for certain medicinal purposes, it may prove to be
an effective option with incoming research,
especially in the sickle cell patient population.

Legal Background
DC Maryland
Patients and caregivers "may only obtain medical
marijuana from the dispensary designated on your
registration identification card and may not: (a)grow
or cultivate medical marijuana); b)purchase medical
marijuana through street vendors; or (c) obtain
medical marijuana from other patients and
caregivers." (DOH 2013)
Approved Conditions: HIV, AIDS, cancer, glaucoma,
conditions characterized by severe and persistent
muscle spasms, such as multiple sclerosis; patients
undergoing chemotherapy or radiotherapy, or using
azidothymidine or protease inhibitors. (Amendment
Act B18-622)
"A qualifying patient or caregiver may obtain
medical marijuana from a grower’s facility or from a
satellite facility of the grower.
Approved diagnosis: cachexia, anorexia, or wasting
syndrome, severe or chronic pain, severe nausea,
seizures, severe or persistent muscle spasms, or
other conditions approved by the Commission.
(Senate Bill 923)
Virginia Federal
“It is unlawful for any person knowingly or
intentionally to possess marijuana unless the
substance was obtained directly from, or pursuant
to, a valid prescription or order of a practitioner
while acting in the course of his professional
practice, or except as otherwise authorized by the
Drug Control Act” (VA HB1445; 18.2-250.1).
Approved Conditions: “Certification for use of
cannabidiol oil or THC-A oil to treat intractable
epilepsy”. (VA HB1445; 54.1-3403.3)
Marijuana is still classified as a Schedule 1 Drug by
the Drug Enforcement Administration, and
according to the Office of Diversion Control,
“Substances in this schedule have no currently
accepted medical use in the United States, a lack of
accepted safety for use under medical supervision,
and a high potential for abuse.”

Discussion
• The purpose is to understand the social attitudes
and beliefs of marijuana among sickle cell patients.
• How do people see cannabis use? What are the
social perceptions, even with new laws
decriminalizing or allowing the Schedule I drug for
use around the country?
• Is there any correlation with use and treatment for
sickle cell disease symptoms?

Demographics (N = 24)
• Gender:
• Male – 66.7% (N = 16)
• Female – 33.3% (N = 8)
• Age:
• Range: 18-60,
• Mean = 39.17
• StDev = 12.67
• Race/Ethnicity:
• Black/African – 91.7% (N = 22)
• Hispanic/Latino – 4.3% (N = 1)
• Prefer not to answer – 4.3% (N
= 1)
• Sickle Cell Status:
• SS – 58.3% (N = 14)
• SC – 20.8% (N = 5)
• SB+thal – 12.5% (N = 3)
• SB0thal – 4.2% (N = 1)
• Unknown – 4.2% (N = 1)
• State of Residence:
• DC – 54.2% (N = 13)
• MD – 25.0% (N = 6)
• VA = 12.5% (N = 3)
• CT = 4.2% (N = 1)
• N/A = 4.2% (N = 1)

Critical Findings
Types of Medications
• Folic Acid (20, 87.0%)
• Hydroxyurea (12, 52.2%)
• Morphine (5, 21.7%)
• Oxycontin (6, 26.1%)
• Oxycodone (7, 30.4%)
• Dilaudid (7, 39.1%)
• Fentanyl (2, 8.7%)
• Methadone (2, 8.7%)
• Exjade/Jadenu (2, 8.7%)
• Percocet (6, 26.1%)
• Tramadol (0, 0.0%)
• Other (please specify) (2,
8.7%)
0
5
10
15
20
25
FolicAcid
Hydroxyurea
Morphine
Oxycontin
Oxycodone
Dilaudid
Fentanyl
Methadone
Exjade/Jadenu
Percocet
Tramadol
Other
Frequency(N)
Medication History

Critical Findings
• 16.7% (N = 4) comprise of those who smoke
cigarettes at least once per day
• 16.7% (N = 4) comprise of those who drink alcohol
at least once per day
• 75.0% (N = 18) have or currently use cannabis
during their lifetime

Critical Findings
0
1
2
3
4
5
6
7
8
9
10
Less than one
month
2-12 months More than one
year
Last known use (N = 17)
Years
Duration in years among Participants who
reported using cannabis (N = 14)
Range: 0 -40 years
Mean: 8 years
StDev : 11.02
Modes: 0, 1, 6

Critical Findings
Strongly Disagree Disagree Neutral Agree Strongly Agree
Users 1 1 6 2 8
Non-users 2 2 1 1 0
0
1
2
3
4
5
6
7
8
9
Frequency(N)
If marijuana is legal, I would be more likely to use it

Critical Findings
Strongly
Disagree
Disagree Neutral Agree Strongly Agree N/A
Users 5 3 1 7 1 1
Non-users 1 2 1 1 1 0
0
1
2
3
4
5
6
7
8
Frequency(N)
There should be restrictions on the use of marijuana

Critical Findings
Users 1 1 4 4 8
Non-users 1 1 2 1 1
0
1
2
3
4
5
6
7
8
9
Frequency(N)
I would use marijuana to relieve my pain crises

Critical Findings
Users 1 1 4 5 7
Non-users 1 0 3 2 0
0
1
2
3
4
5
6
7
8
Frequency(N)
I would use marijuana to relieve chronic pain

Critical Findings
Strongly
Disagree
Users 0 1 3 7 7 0
Non-users 2 2 1 0 0 1
0
1
2
3
4
5
6
7
8
Frequency(N)
I would use marijuana to induce relaxation

Critical Findings
Strongly
Disagree
Users 6 4 5 0 1 2
Non-users 2 2 1 0 0 1
0
1
2
3
4
5
6
7
Frequency(N)
I would use marijuana to induce euphoria or “get high”

Critical Findings
Strongly
Disagree
Users 6 0 4 4 2 2
Non-users 2 2 2 0 0 0
0
1
2
3
4
5
6
7
Frequency(N)
There are adverse side effects associated with the use of
marijuana

Critical Findings
Strongly
Disagree
Users 1 0 4 6 6 1
Non-users 0 2 2 1 1 0
0
1
2
3
4
5
6
7
Frequency(N)
Marijuana has the potential to treat sickle cell disease along with
other medications

Critical Findings
Strongly
Disagree
Users 1 2 3 5 6 1
Non-users 0 2 1 1 1 0
0
1
2
3
4
5
6
7
Frequency(N)
I believe that marijuana can help manage my sickle cell
symptoms overall

Critical Findings
Strongly
Disagree
Users 1 2 5 1 6 3
Non-users 1 2 1 0 2 0
0
1
2
3
4
5
6
7
Frequency(N)
I believe that marijuana can help prevent future pain crises
associated with sickle cell disease

Critical Findings
Strongly
Disagree
Users 1 4 7 2 3 1
Non-users 1 3 2 0 0 0
0
1
2
3
4
5
6
7
8
Frequency(N)
I believe that marijuana can help prevent future pain crises
associated with sickle cell disease

Critical Findings
• 45.8% (N = 11) prefer
smoking (i.e. cigarette,
water pipe)
• 54.2% (N = 13) prefer
vaporizing (i.e. humidifier)
• 70.8% (N = 17) prefer edible
means (i.e. food, oral
ingestion)
• 62.5% (N = 15) prefer
topical application (i.e. oils,
creams, balms)
0
2
4
6
8
10
12
14
16
18
Smoking Vaporizing Edible Means Topical
Overall Opinions of Preferred
Method of Cannabis Use
Yes No

Critical Findings
• How would cannabis use help in managing sickle-
related pain crises?
• Increase appetite (16, 72.7%)
• Improves sleep (19, 86.4%)
• Relieves pain (18, 81.8%)
• Relieves stress/anxiety (19, 86.4%)
• Improve mood (15, 68.2%)
• Improve sexual desire (6, 27.3%)
• Other (3, 13.6%)

Research Barriers
• Timing: Study was approved Nov. 2015
• Eligibility: Despite non-sensitive information
required, vulnerable populations (i.e. sick,
educationally disadvantaged, incarcerated) were
omitted from consent and participation
• Perceived Risks: reputation, fears of intervention,
opinions about said modes of treatment, sensitivity
• Limited Knowledge of subject
• Rare: potential for missing responses

For Future Discussion
• What about the risk or priapism among
hydroxyurea and/or cannabis users?
• Are there any correlations with those who have
sickle cell trait?
• Do you believe that there will be a change in
federal drug laws pertaining to substances with
possible therapeutic benefits?

Exigency
• To manage patients’ sickle cell disease symptoms,
there must be a high level of communication
between the patient and provider. No one case is
the same; an approach to an evidence-based
health plan can overall improve outcomes across
the entire population. If a treatment exists, the
option should be there for all patients sans
essential medical reasons.

References
• Carlini, B.H., Garrett, S.B., & Carter, G.T. (2015). Medical
Cannabis: A Survey Among Health Care Providers in
Washington State. Am J Hosp Palliat Care, 1-7. doi:
10.1177/1049909115604669
• Howard, J., Anie, K. A., Holdcroft, A., Korn, S., & Davies,
S. C. (2005). Cannabis use in sickle cell disease: a
questionnaire study. British Journal Of
Haematology, 131(1), 123-128. doi:10.1111/j.1365-
2141.2005.05723.x
• Knight-Madden J, Lewis N., & Hambleton I.R. (2006).
The prevalence of marijuana smoking in young adults
with sickle cell disease: a longitudinal study. The West
Indian Medical Journal, 55(4), 224-7.

A Report and Recap of Research Performed at HUCSCD

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