Overcoming Stigma in Sickle Cell Disease


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Presentation on the main myths of sickle cell patients and data against these claims. Also covers the basics of SCD

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  • Hello everyone, My name is Tosin Ola-Weissmann, and I’m a registered nurse practicing at Oregon Health Sciences University. A little of my background in sickle cell: For starters, I have sickle cell. In 1999 I moved to the US to attend nursing school, and have been practicing as a nurse very since graduation from Oakwood University in 2001. In 2005, I had a really bad sickle cell crises, which left me in the hospital for over 2 weeks. When I got home, I was so overwhelmed with having sickle cell, and I searched online for someone else who…blah blah blah So I started my own blog. This eventually developed into the Sickle Cell Warriors website and Facebook community, which serves as an online support group for over 2300 members. I was featured in an issue of Minority Nursing (Where Darling found me). Having interaction with over 2300 members on a daily basis has given me more insight into sickle cell than I could ever have possibly imagined. I have learned so much from the group, but the one recurring theme that seems to be a sore spot for everyone is the way sickle cell warriors are treated in the hospital setting. That will be the focus of our discussion today.
  • Lifespan: People with severe SCD are now living into adulthood but can experience a life expectancy that is 30 years shorter than those without disease. (CDC) I know most of you probably know the basics of what sickle cell disease is, so I’m going to talk about some stuff that you might not know. First discovered blood disorder Nwiiwii (Faute), Nuidudui (Ewe), Ogbanjes (Ibo) Dr Herrick (attending), Dr Irons (did the work). Walter C. Noel from Grenada, Dental student Trait, S, C, E, S-beta thallasemia According to U.S. News & World Report, some 120,000 infants are born with SCD every year worldwide and 90% of the people with sickle cell worldwide are born outside of the USA. Estimates purport that in the United States, there are over 80,000 people with the disease, and 2 million people are carriers of sickle cell trait. Nigeria is the most populous country in Africa and also has the highest incidence of sickle cell. There are over 4 million people with sickle cell disease in Nigeria and due to the fact that 3 out of every 10 children are born with sickle cell trait, this number is growing astronomically. In India, it is estimated that 20% of the population have sickle cell trait and over 20 million people have the disease (it is just being discovered over there). These numbers mean that it is possible that the number of people with sickle cell disease will grow instead of decline in the next few decades unless proper education is disseminated to the general public to stop the proliferation of sickle cell. Throw in globalization, and the fact that University of Oregon is such an awesome school, this means that in your clinical or educational career, you will come across several sickle cell patients.
  • By now you’ve heard of several athletes with sickle cell trait or disease going into respiratory or cardiac arrests after excessive sports. It is such a huge problem that the National College Athletic Association (NCAA) has started screening on all athletes to test for the trait. The reason behind this is that with rigorous physical activity, the body requires a higher metabolic and oxygen demand. Since sickle cells do not cooperate with that scenario in any way, the rest of the body has to overcompensate to make up for those queens. As a result, the lungs pump out more oxygen, the blood pressure and heart rate increases, and your body is in overdrive trying to keep up with the demand. When you run in overdrive for a long time, eventually, the heart and lungs can’t keep up and basically crap out on you. This is what has led to all those deaths. To avoid this trigger, sickle cell patients need to really pace themselves when exercising. Pain is your body reminding you to slow down and take a break. Listen to the warning, take a break, catch your breath and drink some water.
  • A person with chronic pain might not have the same posturing that you expect with a 10/10 pain. Remember, we have been having this pain since childhood…for me, that is 3 decades of chronic pain. With my 10/10 pain, I can’t do anything, I don’t even want to talk, I just want to cry, curl into a fetal position and pray for a quick, merciful death. However, a friend of mine at a 10, watches TV to distract herself and keep her mind off the pain. At a 9 she can even talk on the phone. And when my parents or family call me on the phone, at a 9/10, I talk and act as ‘normal’ as possible because I don’t want them to be worried. How one deals with pain is should not be the yardstick for the pain scale or your decision to administer or with-hold meds. Under-treatment of pain can lead to pseudo-addiction and seriously undermine a patient's quality of life. Patients report that in the ED, low doses of pain meds are often given leading to lack of pain relief. A recent study showed that there was a 70-120 minute delay in the ED from admission to administration of analgesic to patients with SC. This could be due to high workload, difficulty establishing IV, or low prioritization of SCD patients. Pseudo-addictive behaviors include: Fear of being in severe pain Clock watching Calling ahead of time Ranking pain higher
  • A common thread among the members in my SC community is that they are afraid of getting addicted to narcotics. Many use their pain meds sparingly, to the point that they remain ‘in pain’ even when they have enough medication to completely knock out their pain. In addition, there are so many side effects that come with using narcotics, and many sickle cell patients want to enjoy life, and not let it pass them by in a groggy haze
  • I detest going to the emergency room or getting admitted to the hospital for my disease. I will manage it at home as much as I can, but if I need to go to the hospital, it is because the pain is uncontrollable, and nothing in my arsenal works. I do go to my doctor’s visits, and have a healthy grasp of my disease process, but I still have to go to the emergency room when in an acute pain crises. Most of the time, even if I go to my doctor’s office, it is just a waste of time, because he will tell me to go to the ER. So coming to the ER because one has a chronic condition is not a misuse of the medical facility. If a diabetic came in with a blood sugar of 45 or 545, would he be misusing the ER? After all, he understands his disease process and diabetes is a chronic condition, is it not? In my esteem, SCD patient coming to the ER with an acute flare-up of a chronic condition should get the same compassionate care as a diabetic coming to the ER with an out of wack blood sugar.
  • Anemia: Sickle cells only live for about 10 to 20 days, while normal hemoglobin can live up to 120 days (Strickland et al., 2001, p. 37). Due to the decreased number of hemoglobin cells circulating in the body, a person with sickle cell disease is chronically anemic (Strickland et al., p. 37). Anemia can be evidenced by coldness in hands/feet, fatigue, pallor, headache, chest pain, dizziness, syncope & SOB (NHLBI) Infections: Because of its narrow vessels and function in clearing defective red blood cells, the spleen of SCD patients is damaged early on leading to a decreased ability in filtering out infections (OHSU). SCD patients are susceptible to multiple bacterial infections including salmonella and pneumococcal sepsis which can easily become life-threatening (Tanabe & Myers, 2006). Assess the patient for cold and flu symptoms, diarrhea, elevated temperature or sore throat (American Academy of Pediatrics [AAP], 2002, p. 429). Pain: This is the most common complaint of sickle cell patients (OHSU). A vaso-occlusive crisis is caused by sickle-shaped red blood cells that obstruct capillaries and restrict blood flow to an organ, resulting in ischemia, pain, and organ damage (NHLBI, 2007). However, a thorough physical assessment should be done to check for other problems (Tanabe & Myers, 2006). Jaundice: Sickle cells do not live as long as normal red blood cells and, therefore they are dying more rapidly than the liver can filter them out (OHSU). Bilirubin (which causes the yellow color) from these broken down cells builds up in the system causing jaundice (OHSU). Respiratory distress: Due to their shape, sickled cells are unable to carry oxygen (OHSU). A high incidence of sickled cells leads to decreased oxygenation throughout the body (Tanabe & Myers, 2006) which triggers shortness of breath. In addition, pain in the body can cause the patient to start taking shallow breaths (AAP, p. 430).
  • Any and all major organs are affected by sickle cell disease (OHSU, 2008). SCD patients usually die from the complications, and not the disease itself (NHLBI, 2007). Splenic sequestration is a result of sickle cells pooling in the spleen (OHSU). The spleen can become enlarged and painful from the increase in blood volume and this can be life-threatening. Stroke can result from a progressive vascular narrowing of blood vessels, preventing oxygen from reaching the brain (OHSU). Cerebral infarction occurs in children, and cerebral hemorrhage in adults (NHLBI). Gallstones may result from excessive bilirubin production and precipitation due to prolonged hemolysis (Strickland et al., 2001, p. 40). Avascular necrosis of the hip may occur as a result of ischemia leading to severe pain when walking (OHSU). This ischemia can also lead to leg ulcers and improper wound healing. Priapism is caused by blood trapped in the penis due to infarction of the vessels (NHLBI). Untreated, recurrent priapism may lead to impotence (Strickland, et al). Renal failure is due to acute papillary necrosis in the kidneys, manifests itself with hypertension, proteinuria and worsened anemia (NHLBI). If it progresses to end-stage renal failure it carries a poor prognosis (OHSU). Acute chest syndrome is a life-threatening condition similar to pneumonia caused by an infection or sickle cells trapped in the lungs (NHLBI). Symptoms can include fever, pain, and a violent cough (NHLBI). Multiple episodes of acute chest syndrome can cause permanent lung damage (OHSU). Pulmonary hypertension: Damage to the small blood vessels in the lungs makes it hard for the heart to pump blood through the lungs which leads to increased pulmonary arterial pressure (NHLBI). Shortness of breath and problems with breathing are the main symptoms of PAH and can be fatal. Retinal damage: Sickle cells also can clog the small blood vessels in the eyes leading to damage of the retina which can cause blindness if untreated (NHLBI). Opioid tolerance or opiate-induced hyperalgesia can occur as a normal, physiologic response to the therapeutic use of opiates (Weissmann & Haddox, 1999). Pseudo-addiction is defined as an iatrogenic syndrome caused by the wrong treatment of pain with the inadequate prescription of analgesics to meet the primary pain stimulus (Lusher, Elander, Bevan, Telfer, & Burton, 2006, p. 316). Research suggests that drug addiction is no greater among people with sickle cell disease than it is in the general population (AAP, 2002, p. 530).
  • The medical management of sickle cell is an aspect that many sickle cell warriors are familiar with. Hospital routines and management become as natural to us as breathing, and by the time we hit our 20s, we already know the system. Unfortunately, with this knowledge comes a layer of cynicism and lack of trust with the hospital system. Some medical professionals, have no clue of the complexities with sickle cell disease. Many think that it’s just about pain management, but there is more to managing sickle cell than pain. The pain is a symptom of the disease, but unfortunately, this symptom is so agonizingly painful that it often gets the front seat.
  • In conclusion, Sickle cell is one of the least understood blood diseases in the world. It affects many people, and only through education and increased awareness can this disease be eradicated. Collaboration with sickle cell warriors and the healthcare team is important to ensure quality care. Sickle cell patients are by far the strongest people I have ever met and once you get to know them, I’m sure you will see that too. Thank you. Any questions?
  • Overcoming Stigma in Sickle Cell Disease

    1. 1. Overcoming Stigma in Sickle Cell Disease Presented by: Tosin Ola-Weissmann, BSN, RN
    2. 2. What is Sickle Cell? <ul><li>What is sickle cell? </li></ul><ul><ul><li>Hereditary </li></ul></ul><ul><ul><li>Evolutionary response to malaria </li></ul></ul><ul><li>Population sickle cell affects </li></ul><ul><ul><li>Millions worldwide </li></ul></ul><ul><ul><li>Globalization </li></ul></ul><ul><li>Lifespan </li></ul><ul><li>Nature of SC </li></ul>
    3. 3. What Does Having SC Feel Like? <ul><li>Anemia: Fatigue, exhaustion, low energy </li></ul><ul><li>Excruciating Pain </li></ul><ul><ul><li>Side effects </li></ul></ul><ul><li>Insomnia </li></ul><ul><li>Depression </li></ul><ul><li>Jaundice </li></ul><ul><li>On top of this, we deal with negative perceptions and ignorance of sickle cell in our homes, hospitals and communities </li></ul><ul><li>People with SC trait can exhibit these symptoms but to a milder intensity </li></ul>
    4. 4. Triggers of a SC Pain/Crisis <ul><li>Dehydration </li></ul><ul><li>Stress </li></ul><ul><li>Infection </li></ul><ul><li>Temperature changes </li></ul><ul><ul><li>Swimming </li></ul></ul><ul><ul><li>Weather change </li></ul></ul><ul><li>Pressure changes </li></ul><ul><ul><li>Snow sports </li></ul></ul><ul><ul><li>Flying </li></ul></ul><ul><li>Periods </li></ul><ul><li>Sex </li></ul><ul><li>Rigorous physical activity </li></ul>
    5. 5. Sports & Sickle Cell <ul><li>Overexertion requires higher metabolic and oxygen demand </li></ul><ul><li>Overcompensation by heart and lungs </li></ul><ul><li>Heart attack or respiratory failure </li></ul><ul><li>Avoid this trigger </li></ul><ul><ul><li>Pace yourself </li></ul></ul><ul><ul><li>Pain is a warning signal </li></ul></ul><ul><ul><li>Hydrate </li></ul></ul><ul><ul><li>Take breaks as needed </li></ul></ul>
    6. 6. Basic Treatment Principles <ul><li>Hydration </li></ul><ul><li>Oxygenation </li></ul><ul><li>Pain Management </li></ul><ul><li>Blood Transfusions </li></ul><ul><li>Bone Marrow Transplant </li></ul><ul><li>Hydroxyurea </li></ul><ul><li>Herbal remedies </li></ul><ul><li>Nutraceuticals </li></ul><ul><li>Dietary </li></ul><ul><li>Lifestyle </li></ul>
    7. 7. SC Voices <ul><li>Sickle cell pain is indescribable. I remember when I was in labor, I didn’t make a sound. My mother had to ask, &quot;Why are you so quiet, doesn't it hurt?&quot; </li></ul><ul><li>I responded, &quot;Mom, I have sickle cell, this is nothing...&quot; ~Belinda Sylvestre </li></ul>
    8. 8. Why do you think SC patients encounter so much ignorance, stereotyping and negative attitudes in our schools, communities and the healthcare setting?
    9. 9. Myth #1: SC Patients are Drug Addicts, Drug-Seeking, and Not Really in Pain <ul><li>Chronic pain </li></ul><ul><li>Under-dosing/Tolerance </li></ul><ul><li>Delay in administration of analgesics </li></ul><ul><li>Pseudo-addiction </li></ul><ul><li>‘ 3 shot rule’ and Quick discharges lead to patients returning to the ED within 3 days from discharge STILL IN PAIN! </li></ul><ul><li>Reference: Solomon, LR. Treatment and prevention of pain due to vaso-occlusive crises in adults with sickle cell disease: an educational void. Blood. 2008; 111:997-1003 </li></ul>
    10. 10. <ul><li>In a US survey, emergency department physicians significantly overestimated the prevalence of substance abuse, although research supports that less than 10% of patients with sickle cell disease meet the clinical criteria for dependence on or abuse of opioid analgesics </li></ul><ul><li>Reference: Elander J, Lusher J, Bevan D, Telfer P. Pain management and symptoms of substance dependence among patients with sickle cell disease. Soc Sci Med, 2003;57:1683-1696 </li></ul>
    11. 11. Myth #2: SC Patients Over-use the ED <ul><li>Do CHF/COPD patients over-use the ED? What about diabetics? </li></ul><ul><li>Patients with SCD with high utilization rates may actually have more severe anemia, more painful episodes, greater pain and distress ratings, and worse quality of life compared to those with lower utilization rates. </li></ul><ul><li>Reference: Aisiku, I. P., Smith, W. R., & McKlish, D. K. (2009). Comparisons of high versus low emergency department utilizers in sickle cell disease. Annals of Emergency Medicine , 53 , 587-593. </li></ul>
    12. 12. SC Voices <ul><li>I detest going to the emergency room or getting admitted to the hospital for my disease. I will manage it at home as much as I can, but if I need to go to the hospital, it is because the pain is uncontrollable, and nothing in my arsenal works. I do go to my doctor’s visits, and have a healthy grasp of my disease process, but I still have to go to the emergency room when in an acute pain crisis. Most of the time, even if I go to my doctor’s office, it is just a waste of time, because he will tell me to go to the ED. </li></ul>
    13. 13. Myth #3: SC Patients are Difficult <ul><li>Traumatized, embittered </li></ul><ul><li>Tremendous pain </li></ul><ul><li>Much more sensitive to delay and negative attitudes by personnel </li></ul><ul><li>The proactive ‘drug seeking’ behavior is as a result of previous negative experiences in the hospital setting. If sickle cell patients tend to be seen as cranky, aggressive, demanding, or with a chip on their shoulder; consider that it could well be a reaction to the treatment that they have received in the past, and understand the connection between your attitudes and the response of the patients. </li></ul>
    14. 14. SC Voices <ul><li>I've been in the ER 8 times last year and admitted 4 times. All times there have been at least one nurse that comes in and treats me like an addict or like I completely don't know how much medicine my body can sustain. I usually end up not getting enough medicine to take the edge off the crisis pain, and in the long run, my crisis lasts longer because of (not to be rude) ignorant nurses who don't know the first thing about a sickle cell pain crisis. ~Simone </li></ul>
    15. 15. SC Voices <ul><li>I abhor the term &quot;frequent flyer.&quot; The ER staff at the hospitals in the DC area use that term to identify someone who has had to return to the ER often. Also, it's always assumed that you are there to seek drugs instead of medical care. I'm ...often told I'm not like the average SC patient because when I come in I'm &quot;genuinely&quot; sick. That really bothers me that we are judged so harshly because our treatment includes narcotics. They really don't understand and make me feel as if they don't care either. ~ Kena </li></ul>
    16. 16. Things You Shouldn’t Say to your Sickle Cell Patient… <ul><li>So how long have you had sickle cell? </li></ul><ul><li>You don’t look/act/talk like most sicklers </li></ul><ul><li>Your lab work says you are ready to be discharged… </li></ul><ul><li>Pain </li></ul><ul><ul><li>How can you still be in pain? </li></ul></ul><ul><ul><li>You can’t be in pain and ____ (talking, eating, sleeping, watching TV, on the computer) </li></ul></ul><ul><ul><li>You should be used to this pain by now </li></ul></ul>
    17. 17. Assess Patient For <ul><li>Anemia </li></ul><ul><li>Pain </li></ul><ul><li>Infections </li></ul><ul><li>Constipation </li></ul><ul><li>Insomnia </li></ul><ul><li>Jaundice </li></ul><ul><li>Respiratory distress </li></ul><ul><ul><li>Acute chest syndrome </li></ul></ul><ul><li>Renal and liver function </li></ul><ul><li>Depression </li></ul>
    18. 18. Complications of SCD <ul><li>Strokes </li></ul><ul><li>Gallstones </li></ul><ul><li>Splenic Sequestration </li></ul><ul><li>Avascular Necrosis </li></ul><ul><li>Iron Overload </li></ul><ul><li>Migraines </li></ul><ul><li>Leg ulcers </li></ul><ul><li>Priapism </li></ul><ul><li>Renal failure </li></ul><ul><li>Acute chest syndrome </li></ul><ul><li>Pulmonary hypertension </li></ul><ul><li>Retinal damage/blindness </li></ul><ul><li>Deafness </li></ul>
    19. 19. SC Patient as a Teacher <ul><li>Your patient knows more about their body and their sickle cell than you think. </li></ul><ul><li>Listen and learn </li></ul><ul><li>Collaborate with your patient on their plan of care </li></ul>Reference: Campbell, A. D., Ross, P. T., Kumagai, A. K., Christner, J. G., & Lypson, M. L. (2010). Coming of age with sickle cell disease and the role of patient as teacher. Journal of the National Medical Association , 102 , 1073-1078.
    20. 20. Conclusion <ul><li>For more information about the Sickle Cell Warriors, visit http://sicklecellwarriors.com </li></ul><ul><li>Email me at </li></ul><ul><li>[email_address] </li></ul><ul><li>Connect with SC patients on Facebook/SickleCellWarriors </li></ul>