Objectives:
Upon completion of this course, you should be able to:
– Understand the difficulties of Sickle Cell disease patients both from the disease and because of the complexity and nuance of treatment
– Identify behaviors, thoughts, or beliefs that you can change in your treatment of Sickle Cell disease patients
– Commit to disseminating the patient perspectives explained in this course
– Understand the historic role of racism in healthcare and how that has impacted Sickle Cell Disease patients
In order to complete this course and receive your certificate, please be sure to:
– Finish each lesson in its entirety (watch all videos, click on and read through every slide)
– Complete the survey
– Answer all quiz questions and complete the final exam with a grade of 90% or better
This platform allows you to listen to course content as well as read at your own pace. There are also certain items you can download for future reference; look for the download arrow on the top righthand corner of all PDF documents.
Please email us for any issues or concerns while taking the course.
Good luck and happy learning!
Introduction:
Sickle Cell Disease (SCD) is a genetic disease and life-long illness. Because SCD does not present itself in a uniform way amongst all patients, doctors must form treatment plans on a case-by-case basis. Ideally, treatment plans will vary based on the effects and severity each patient experiences. Things like early diagnosis, a personalized treatment plan, and awareness of pain triggers help to improve SCD patients’ quality of life.
When healthcare professionals truly understand SCD and SCD patients’ experience of the disease, they are better able to provide them with the right treatment that will help improve their quality of life. For this reason, this course on SCD not only explains the disease itself but corrects misinformation about it and focuses on the patient experience of the disease. With the correct information about SCD circulating amongst the community of healthcare professionals, everyone involved in the process of care will be better off.
Module 1: Understanding Sickle Cell Disease
A. What is Sickle Cell Disease?
Sickle Cell Disease (SCD) comprises several genetically inherited red blood cell disorders, all of which are present at birth. Specifically, SCD affects the hemoglobin, or the protein within red blood cells that transports oxygen. There are two alpha-globin chains and two beta-globin chains in this protein. SCD is caused by a mutated beta-globin gene.
When red blood cells are healthy, they are round and can easily move through blood vessels to bring oxygen everywhere in the body. SCD transforms a person’s red blood cells into farming equipment called a “sickle,” which is in the shape of a “C” or a crescent moon. Also, the red blood cells of a person with SCD are hard and sticky. Between their shape and texture, these mutated red blood cells have trouble moving through blood vessels. They often get stuck and end up clogging blood flow.
They also don’t live as long as normal red blood cells, leaving SCD patients with not enough red blood cells in their system. Normal red blood cells can live anywhere from 90 to 120 days. Sickle cells usually only live between 10 and 20 days. While the body is constantly making new red blood cells to replace the old ones, the body can’t keep up with how fast sickle cells die. Patients with SCD usually have lower than normal counts of red blood cells, which is why SCD is known as a form of anemia.
All of these issues in the body caused by SCD can lead to varying levels of pain and energy deficiency as well as increased risk for infection, stroke, and acute chest syndrome. A simple blood test can reveal if a person has SCD. Usually, hospitals diagnose it when a newborn goes through standard tests and screenings. Medical professionals can diagnose SCD before birth as well. People who have SCD have an enhanced risk for infections and health problems, which is why catching it early is helpful.
B. Common types of Sickle Cell Disease
The different types of SCD depend on which part of the beta-globin chains are impacted. The most common types of SCD are as follows:
HbSS
The form of SCD called HbSS happens when a child inherits a sickle cell gene (known as “S”) from each parent, leaving them with two sickle cell genes. It is the most severe kind of SCD. HbSS is also called sickle cell anemia.
HbSC
HbSC is a milder form of SCD than HbSS. It happens when a child has inherited one “S” (sickle cell gene) from one parent. From their other parent, they have inherited an abnormal hemoglobin known as “C.”
HbS beta-thalassemia
SCD patients with HbS beta-thalassemia have inherited one “S” from one parent and one beta-thalassemia gene from the other. Beta-thalassemia is a different type of anemia. Two kinds of beta-thalassemia exist. HbS beta 0-thalassemia tends to lead to more severe cases of SCD. HbS beta +thalassemia tends to lead to less severe SCD cases.
Rarer forms of SCD
There are three rarer forms of SCD: HbSD, HbSE, and HbSO. In each of these types of SCD, the child inherits one “S” and one gene from a different abnormal hemoglobin, either “D,” E,” or “O.” These types of SCD vary in severity.
Sickle Cell Trait (SCT)
Sickle Cell Trait (SCT) is called HbAS. Anyone with SCT has only inherited one sickle cell gene, or “S,” from one parent along with one normal gene, “A,” from the other. Most people with SCT do not have the symptoms and issues associated with SCD. However, they can potentially pass that gene to their children.
C. Signs, Symptoms, and Complications of SCD
If you have SCD, you were born with it, but that does not mean SCD complications begin immediately at birth. Most newborns do not experience any issues from the disease until they are around five to six months old.
SCD can cause a wide range of symptoms, and often they are not static, meaning they can change as time passes. Early signs usually include your skin or the whites of your eyes turning a yellowish color. When the skin turns a yellowish color, this is called jaundice. When the whites of the eyes turn yellowish, this is called icterus. Persistent fatigue is also an early sign of SCD, as is painful levels of swelling in the hands, feet, or both. This is called dactylitis.
Later symptoms of SCD can lead to complications, many of which can be serious. These include:
Acute pain crisis – When sickle cells block blood flow, it can cause intense pain that feels like an intense, throbbing, sharp, or even stabbing sensation. It can happen in an isolated region of the body or multiple regions at once. Most commonly, this pain occurs in the chest, lower back, abdomen, arms, and legs. Triggers for acute pain crisis can include dehydration, stress, other illness, high altitude, or a change in temperature. Most people don’t realize when one of these triggers occurs until it has already happened. Acute pain crisis is also known as sickle cell crisis or vaso-occlusive crisis.
Acute chest syndrome – Sickle cells traveling in blood vessels can cause oxygen-deprivation, leading to damaged lung tissue. This in turn can cause trouble breathing, fever, and chest pain and is a medical emergency.
Chronic pain – Chronic pain is reoccurring pain that can be hard for the person experiencing it to fully describe or place its origin. Typically it is the result of some kind of damage to an organ or crisis pain.
Eye issues – SCD can cause damaged blood vessels in the eyes, usually in the retina. Common problems are the blood vessels in the retina bleed, overgrow, or are blocked. All of these issues can then end up with the retina detaching from its normal positioning. Vision loss can result.
Gallstones – The breakdown of red blood cells, also known as hemolysis, causes a release of hemoglobin. After its release, hemoglobin breaks down into what is called bilirubin, which can form into gallstones and get caught in the gallbladder. If gallstones are not painful, they don’t usually require treatment. However, when they are, surgical removal usually becomes necessary.
Delayed growth and puberty – The anemia associated with SCD can cause children to develop later than usual. Their growth is not generally stunted, just delayed.
Infections – The spleen helps protect the body against infections and SCD can cause damage to the spleen. As such, SCD is associated with a higher risk of different kinds of infections, such as salmonella, staphylococcus, chlamydia, and haemophilus influenzae type B.
Issues with joints – SCD can cause a decrease in oxygen flow to certain joints, most commonly the hip joints. However, it can also occur in the knees, shoulder joints, and ankles. The resulting condition is called avascular necrosis, where serious damage to the joints occurs. The pain can be so severe that it can lead to trouble walking and with movement in general. Heavy pain medications, joint replacement, or surgery can become necessary.
Heart issues – SCD can lead to pulmonary hypertension and coronary heart disease. Additionally, the need for frequent blood transfusions can cause iron overload, then leading to heart damage.
Liver issues – While it is uncommon, SCD can cause a severe type of liver damage known as sickle cell intrahepatic cholestasis. This occurs as a result of sickle cells blocking blood vessels inside of the liver and does not let enough oxygen into the liver tissues. Re-occurrence of this problem can eventually cause chronic pain and even liver failure. What’s more, regular blood transfusions can also cause liver damage with the presence of too much iron.
Kidney issues – SCD can make it hard for the kidneys to produce urine as concentrated as it should be. Frequent and/or uncontrolled urination and bedwetting can result. These issues usually begin during childhood.
Ulcers in the legs – Ulcers caused by sickle cells tend to start small but gradually grow larger and larger over time. Some ulcers heal quickly while others do not. Others can be re-occurring. These ulcers don’t typically begin until age 10 or later.
Pregnancy issues – High blood pressure and blood clots are more common in pregnant women with SCD. SCD can also increase a woman’s chances of premature birth, miscarriage, and low birth weight.
Priapism – Uncontrolled, prolonged, often unwanted erections can result from SCD because of blocked blood flow from sickle cells. With re-occurrence, permanent penis damage, including impotence, is possible. If priapism lasts for four hours or more, it is a medical emergency.
Silent stroke – A silent stroke, or silent brain injury, happens when there are no outward signs of a stroke while the damage is occurring. It is a common occurrence in SCD patients, detected on MRI scans. The results can be trouble with decision making, keeping a job, and learning in general.
Dental problems – Patients with SCD can be more susceptible to dental cavities, hypodontia, malocclusion, and dental infections. SCD patients often tend to ignore what they consider “minor dental matters” relative to the overall health issues they experience. This can cause increased chances of deteriorating oral health in SCD patients.
Severe anemia – Mild to moderate anemia is common in SCD patients. However, sometimes, severe anemia occurs, which is a life-threatening illness.
Module 2 – The Role of SCD Patients in Determining Their Treatment Plans
A. All SCD Patients are Different
The pathology of SCD is unlike most diseases in that it does not have predictable, distinguishable stages. For instance, treating cancer can be easier for healthcare professionals because they know what to expect depending on the stage of cancer present in a patient. SCD varies widely in how it manifests in each individual patient. Some patients may have unbearable chronic pain that requires constant or near-constant treatment in a clinical or hospital setting, including hydration through an IV and narcotic (usually opiate) analgesics. Others have lesser levels of pain that are more easily managed at home most of the time.
Because the pathology of SCD varies so much between patients, treatment becomes all the more difficult. There are no dependable guidelines for treating SCD as a whole. Varying degrees of pain in a wide range of complications that can arise from SCD has led to less than adequate treatment of SCD, causing patients to have a lower quality of life as a result.
The only dependable part of SCD pathology appears to be that patients experience their first SCD crisis by the age of six. By the time, the body starts to its fetal red blood cells with adult red blood cells. Fetal red blood cells don’t sickle, so SCD complications don’t tend to start until the adult red blood cells, which do sickle, enter the picture.
The first basic crisis an SCD patient experiences usually establishes a pattern in the body that is likely to repeat in a similar manner over the course of their lifetime. That is to say that if an SCD patient experiences their first crisis as hip joint pain, it is probable that will continue over throughout their lifetime. While other crises can develop and occur with age and circumstances, the major crises are most likely associated with the initial one.
As stated, SCD patients can vary substantially in how they experience the disease. That is why the development of treatment plans should be on a case-by-case basis depending on an individual patient’s experience. A treatment plan that is successful for one SCD patient will not necessarily be successful for another. When a successful treatment plan is found, often including a specific regiment of pain medication, that tends to remain over the course of their lifetime.
Some of the generalized treatment options for SCD include pain medication, blood transfusions, stem cell transplants, vaccines and antibiotics, and the medication hydroxyurea. In addition, preventative measures for pain and other related crises include drinking lots of water, regular eye exams, and taking folic acid. However, this is not enough. Listening to SCD patients is necessary to further treatment plans to make them more effective and raise patients’ quality of life.
Adjustments can and should be made to replace an old regiment if and when new medications arrive or new symptoms come into play. However, understanding that the primary physical patterns of symptoms in individual SCD patients tend to remain the same. That is why a collaborative approach to treating SCD between healthcare professionals and patients is essential. Healthcare professionals should continuously listen to their patients’ experience of their illness tomake adjustments to treatment when necessary.
B. The Importance of Listening to SCD Patients
Because SCD patients will never go into remission, they will need to manage their symptoms throughout their entire lives. SCD patients become experts of their individual experience of their disease. This is important because of the unpredictable nature of SCD pathology. Often, because a successful generalized treatment plan has not been available, they have needed, for their own sake, to be able to identify the pattern of their crises from onset to resolution. SCD patients have learned to quickly understand what is happening to their bodies and how to best deal with it at any given moment to get their pain under control.
Traditionally, healthcare professionals do not accept patients as being more informed about their disease. Healthcare professionals have become accustomed to assuming the educated role, where patients simply fall in line with what their doctors prescribe and recommend. This can lower an SCD patient’s quality of life by keeping them tethered to a single doctor’s office or hospital for the majority of their lives. It keeps them from the luxury of moving to a new city or even traveling, limiting the scope of life for them. When a doctor is unfamiliar with an SCD patient, they are less likely to trust the patient’s interpretation and personal recommendations for the best treatment, even if they know what they’re talking about from years of experience. Because of this, with SCD, getting treated in a new location by new doctors can have serious consequences, perhaps even meaning the difference between coming home in a week versus not coming home at all.
Placing more trust in SCD patients’ expertise in their own illness is necessary in finding a better way to treat the disease as a whole. Having become aware of their body and its behaviors in relation to the disease over time, it makes sense that they would have quality ideas for how to treat the problem. The collaboration of ideas between healthcare professionals and SCD patients is likely the best way to find the most effective treatment plan. Healthcare professionals must be willing to work together with SCD patients and trust and respect their breadth of knowledge about their individual experience with their disease. Only then will the open communication necessary for successful treatment become more commonplace.
Module 3: The History Behind the Miscommunication Between SCD Patients and Doctors
A. Racism in Healthcare
The stigmas that SCD patients are drug-seeking liars and faking the severity of their condition is rooted in racism. SCD is historically an African-American disease. Because of socio-political-economic structures, it has concentrated in this specific minority community. SCD affects around 1 in 500 African-American children (and an additional 1 in 36,000 Hispanic-American children). It has been passed down from generation to generation and is now part of a shared memory of African-Americans.
The history of painful race relation in the US, with African-Americans graduating over time from slaves, to heavily discriminated against, to slowly receiving a semblance of equality in US society, means it should come as no surprise that a disease that primarily plagues this community has not been high on the list of priorities in terms of finding a proper treatment or a cure. To this day, not enough progress has been made in treating SCD.
Part of the problem lies in the distrust between medical professionals and African-American and other minority SCD patients. Having been largely groomed by society to assume minorities are dramatizing their pain in search of opiate prescriptions, SCD patients and their very real and painful experiences of the disease have often been dismissed. Properly and successfully studying and treating SCD requires the elimination of misconceptions and beliefs about SCD patients. African-American SCD patients deserve the respect and empathy of their doctors. They deserve and need inclusion in the development of their treatment plans, not have their pain continue to be ignored.
Evidence of discrimination can be found by way of example. SCD was first described over 100 years ago, though funding for research into effective therapies remains a necessity. On the contrary, cystic fibrosis, a disease that affects mostly white Americans and far fewer overall Americans than SCD, receives between 7 and 11 times the research funding per patient. This is not an accident but a product of structural racism.
More evidence exists within SCD patient testimonials. Countless African-American SCD patients have described being disrespected, dismissed, and outright ignored by doctors and nurses, even as children. The initial rude or dismissive bedside manner of healthcare professionals toward SCD patients has too often been a clear indication that the patient can not expect quality treatment. It shows they will not use their medical expertise and training to provide the care they could and would be giving to a different patient. This has forced SCD patients, beginning in childhood, to fight for the treatment they need and deserve to improve their quality of life. Many times, the process of finding a doctor who will provide quality care is not easy or quick. It can take going through several doctors before finding one that does not discriminate against African-American SCD patients.
Another part of the problem comes with the capitalistic nature of the Pharmaceutical Industrial Complex, developing medications that will bring profit over the needs of a hurting population. The pharmaceutical industry has largely determined that SCD patients are not worth the time, money, and hassle to develop adequate medications that will ultimately help them manage a lifetime of symptoms associated with the disease. Racist political policies have kept the majority of the African-American population unable to afford the high costs of a potential drug, so the medications are never developed.
B. Moving Forward and Building Trust
To develop successful treatment plans for SCD patients, both SCD patients and healthcare professionals must start looking at their interactions as a partnership. Using their combined knowledge of biology, medicine, and individual experience of the disease is the best path forward. Doctors and nurses will need to trust an SCD patient’s opinions about what they need in terms of medication and dosage. This will have to come with an understanding that SCD patients have been historically ignored, which has forced this community to learn what their bodies need better than those entrusted to care for them and failed to meet their needs.
SCD patients should no longer have to fend for themselves medically. The disease is not only a source of immense, chronic pain and regular crisis, but it is also a life-threatening illness in many cases. Medical professionals need to treat it as such, getting rid of discriminatory prejudices and policies that keep African-American SCD patients suffering. Many SCD patients know how to understand, identify, and clearly communicate what they are feeling, and using that information, doctors will be able to more successfully help them manage their symptoms.
Module 4: UNIQUE
The acronym UNIQUE can be used by both medical professionals and the SCD community to help bridge the gap between a troubled past and a united future.
1. Doctors and nurses must put in the effort to UNDERSTAND their SCD patients, trusting that they are knowledgeable about their illness and how to treat it. They must remember that years of being ignored has forced them to learn about their illness as the medical community has been unavailable and unwilling to learn about it in the past. SCD patients are a resource, and their life experience, in this case, is often as or more valuable than medical degrees. After spending much of their lives fending for themselves on top of going in and out of hospitals their whole lives, SCD patients have a lot of valuable information to share.
2. Doctors and nurses should properly assess patients’ NEEDS before doing a physical exam and giving out orders. Doctors and nurses should not dismiss their complaints as faking or lying and thus undeserving of proper treatment. This will help create trust after such a long history of having no trust at all. The medical community must overcome the stigmas associated with SCD so that the seven million people affected worldwide affected by the disease can finally start to see some positive changes their care. Medical professionals legitimately seeing value patients’opinions and using them for guidance will go a long way. Always keep in mind that SCD patients go through a lot of physical pain, so even the slightest pressure can exasperate the pain and lower trust.
3. Doctors and nurses should be INFORMED before coming up with a treatment plan. This means reading the charts thoroughly, investigating their medical history, and listening to the patient openly. SCD patient crises are usually related to ones that have occurred in the past, so having a complete understanding of what is on their charts can prove invaluable in coming up with an effective treatment plan. If the charts are not helpful in evaluating a crisis, asking the patient directly can help to find the right answer as well.
4. QUESTIONS are the key to better SCD treatment plans. Asking SCD patients questions is another way to build a relationship of open communication and trust. Some medical professionals might worry that asking questions will make them seem unprofessional, but in the end, medicine is a science that often requires a great deal of inquisitiveness. That’s how cures are found.
5. One of the most important goals for the future of unveiling Sickle Cell Disease is UNIFICATION. The SCD community will have a greater impact and ability to advocate for themselves if they are united. Unification helps to build a network of resources, which ultimately leads to garnering more respect. Respect from the medical community will lead to SCD being taken more seriously so that it reaches professional conversations in hospitals, research facilities, and society at large.
6. Both the SCD community and medical professionals must continue to EDUCATE themselves and others about the disease. More education on the disease will provide more opportunities to share new information. New information will lead to increased visibility, and visibility will lead to further research so that hopefully, scientists will develop more effective treatments and maybe even a cure for SCD.
Final Thoughts
Sickle Cell Disease does not currently have a cure. It is an illness that lasts a person’s entire lifetime, starting at birth. About seven million people globally and up to three million Americans are affected by SCD. Between 8 and 10 percent of SCD patients are African Americans. Stigmas against the American Black population have led SCD patients to have to fend for themselves as their attempts to explain their experience of the disease have largely fallen on deaf ears, even within the medical community. Hopefully, with an awareness of these stigmas and advancing knowledge and research about the disease, the appropriate changes will be made so that SCD patients can finally start getting the attention and care they need.
References:
National Heart, Lung, and Blood Institute. (2020). Sickle Cell Disease. National Institute of Health. Retrieved from: https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease
National Heart, Lung, and Blood Institute. (2020). Anemia. National Institute of Health. Retrieved from: https://www.nhlbi.nih.gov/health-topics/anemia
AlDallal, S. M. (2017). Dental health in sickle cell disease. J Med Therap 1. Retrieved from: https://oatext.com/Dental-health-in-sickle-cell-disease.php#Article_Info
Borhade, M. B. & Kondamudi, N. P. (2020). Sickle Cell Crisis. StatPearls. Retrieved from: https://www.ncbi.nlm.nih.gov/books/NBK526064/
Standford Children’s Health. (n.d.). Sickle Cell Disease in Children. Lucile Packard Children’s Hospital Stanford. Retrieved from: https://www.stanfordchildrens.org/en/topic/default?id=sickle-cell-disease-in-children-90-P02327
Renedo, A., Miles, S., Chakravorty, S. et al. (2019). Not being heard: barriers to high quality unplanned hospital care during young people’s transition to adult services – evidence from ‘this sickle cell life’ research. BMC Health Serv Res. Retrieved from: https://link.springer.com/article/10.1186/s12913-019-4726-5#citeas
Power-Hays, A. & McGann, P.T. (2020). When Actions Speak Louder Than Words — Racism and Sickle Cell Disease. The New England Journal of Medicine. Retrieved from: https://www.nejm.org/doi/full/10.1056/NEJMp2022125
Bulgin, D., Tanabe, P., & Jenerette, C. (2018). Stigma of Sickle Cell Disease: A Systematic Review. Issues in mental health nursing. Retrieved from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6186193/
American Society of Hematology. (2020). Sickle Cell Trait. Retreived from: https://www.hematology.org/education/patients/anemia/sickle-cell-trait
Multiple Choice Quiz
1. How does a person get Sickle Cell Disease?
a. It is transmitted through airborne germs.
b. It is genetic and can only be passed to children by their parents.
c. It is transmitted through sexual activity.
d. It is transmitted through bodily fluids like saliva and blood.
2. Where does Sickle Cell Disease get its name?
a. The disease gets its name from Andrew Sickle, the first person to discover the disease.
b. The first known person with Sickle Cell Disease came from the Sickle River region in Africa.
c. Sickle cells are shaped like a piece of farming equipment known as a “sickle.”
d. The word “sickle” comes from a Proto-Indo-European root word meaning “to cut,” and sickle cells look like they have been cut in half.
3. What is Sickle Cell Trait?
a. when a person has only inherited one sickle cell gene from one parent and one normal gene from the other
b. a mild form a Sickle Cell Disease that rarely causes symptoms
c. a severe form of Sickle Cell Disease where the patient requires stem cell transplants in order to live
d. when a person experiences Sickle Cell Disease symptoms without having the disease
4. Which of the following is an early sign of Sickle Cell Disease?
a. blurry vision
b. migraine headaches
c. frequent and unexplained nausea
d. jaundice
5. Why do some Sickle Cell Disease patients experience pain in their joints?
a. SCD can cause decreased oxygen flow to the joints.
b. Sickle cells attach to the muscles around the joint.
c. SCD can lead to bone loss and weaken joints.
d. Pain medications for SCD can cause joint pain as a side effect.
6. What is the normal pathology for Sickle Cell Disease?
a. The body attacks its red blood cells and causes a slow but continuous deterioration of the immune system.
b. There is no normal pathology for Sickle Cell Disease.
c. There are four stages of Sickle Cell Disease, each stage progressively worse and requiring more aggressive treatment.
d. Serotonin and dopamine neurons deteriorate in the brain causing progressively worsening pain throughout the body.
7. Why is Sickle Cell Disease difficult to treat?
a. because SCD patients often lie about their symptoms
b. because SCD shows no pattern of behavior in individual patients
c. because SCD patients experience a wide range of symptoms and complications resulting from their disease
d. because it is difficult to tell if a patient has SCD, which makes it hard to know how to treat their symptoms
8. By what age do most Sickle Cell Disease patients experience their first crisis?
a. within the first six months after being born
b. age 18
c. age 30
d. age 6
9. What is the best treatment plan for Sickle Cell Disease patients?
a. Treatment plans should be determined on a case-by-case basis after thorough communication between the patient and the doctor.
b. The dosage of pain medications should increase as symptoms worsen.
c. Treatment plans should always include weekly blood transfusions to prevent crisis episodes.
d. Diet and exercise should be utilized for treatment before the use of pain medication.
10. What is one preventative measure for avoiding SCD-related crises?
a. increasing vitamin C intake
b. avoiding high-sugar foods
c. regular cardio exercise
d. drinking lots of water
11. Why do many SCD patients lack trust in medical professionals?
a. Some doctors are known to fib to patients are their conditions to avoid confrontation.
b. The high amount of African-American SCD patients and the racism in the healthcare industry has led doctors and nurses to be dismissive of patient comments about their pain.
c. SCD patients sometimes lose their mental faculties, which leads them to become suspicious of doctors’ advice.
d. The idea that SCD patients lack trust in medical professionals is a myth.
12. Which of the following is a serious complication from Sickle Cell Disease that can be life-threatening?
a. severe anemia
b. severe migraines
c. sudden vision loss
d. prolonged leg ulcers
13. How many African-American children have Sickle Cell Disease?
a. 1 in 5
b. 1 in 500
c. 1 in 5000
d. 1 in 100,000
14. What is the most likely origin of the stigma that SCD patients are faking their symptoms in order to get opiate pain medications?
a. sexism
b. homophobia
c. religious prejudice
d. racism
15. What is one of the main causes of symptoms and complications related to Sickle Cell Disease?
a. Sickle cells break in half and one or both pieces will get lodged into organs.
b. Sickle cells move in the wrong direction through the blood vessels.
c. Sickle cells get caught inside of blood vessels and clog blood flow.
d. Sickle cells attack healthy cells and prematurely kill them.
16. Why do adult Sickle Cell Disease patients often have expertise over their own illness?
a. SCD patients do not have expertise over their own illness and would be best off listening to their doctor’s advice without questioning it.
b. Most information about SCD can be found on the internet, and adults can easily find quality advice there about how to care for themselves.
c. SCD is a lifelong illness that adult patients have been dealing with since young childhood, so they have learned about how the disease manifests in their body and how to manage it over time.
d. SCD patients often become doctors or nurses and learn how to care for themselves in the process of their study.
17. Why should Sickle Cell Disease patients and healthcare professionals adopt a collaborative approach in treating the disease?
a. Productive adjustments to a treatment plan can be made more quickly and easily with a collaborative approach to care.
b. Doctors are busy do not have time to figure out all the answers to issues in treatment on their own.
c. SCD patients have more medical knowledge than doctors about SCD treatment.
d. Patients have never studied medicine while doctors have, so they should not be collaborating with doctors in their own treatment plans.
18. When are most cases of Sickle Cell Disease diagnosed?
a. between the ages of one and two
b. early adulthood
c. most cases go undiagnosed
d. at birth when the newborn goes through standard screenings and tests
19. How is Sickle Cell Disease diagnosed?
a. through a doctor’s diagnosis of symptoms
b. through a simple blood test
c. through an MRI scan
d. through a psychological evaluation
20. What is a common trigger of an acute pain crisis caused by SCD?
a. stress
b. prolonged physical exertion
c. prolonged exposure to the sun
d. overeating
Jess Lakritz 