Acute Management of Sickle Cell Disease
Objectives - Overview of Sickle Cell disease - Introduction to common conditions seen in the SCD population - Overview of the Therapy Plan outlined for Sickle Cell Pain Crisis - Review of current literature in reference to this plan - Takeaways to put into practice with this population
What is Sickle Cell Disease - A chronic blood disease resulting from a recessive gene mutation - Affects the binding of B-globin in RBCs causing them to destabilize in hypoxic conditions - This destabilization results in a change in shape of the blood cell - This results in a lot of problems
Sickle cell disease - How it presents - Mechanical and chemical damage to vasculature - Increase adhesions between Erythrocytes and vessels - hypoxic conditions can cause a positive feedback loop with increased sickling - Vaso-Occlusive Crisis
What you’ll see Patients with SCD are often admitted with: - Vaso-occlusive crisis - Severe pain often affecting the back, chest, and extremities - Aplastic Crisis - sudden pallor and weakness confirmed by rapidly dropping hemoglobin levels that are accompanied by reticulocytopenia - Acute Chest Syndrome (ACS) - hypoxia leads to adhesion of sickled erythrocytes to pulmonary microvasculature, setting up local hypoxia in the lungs and causing sickling of more RBCs; this sets up a vicious cycle - Accounts for 25% of deaths and can follow vaso-occlusive crises
Other common problems in SCD - Splenic Sequestration Crisis - acute, painful enlargement of the spleen due to intrasplenic trapping of red cells. Patients with splenic sequestration crisis may have a sudden drop in hemoglobin levels, and one should be vigilant about hypovolemic shock - Femoral Head avascular necrosis - Osteomyelitis - Septic Arthritis - Skeletal immaturity - Osteoporosis & Vertebral Collapse
So what can be done?
Sickle Cell Pain Crisis Therapy Plan 1. Tiered Oral Therapy 2. Mobility Plan
Tiered Oral Therapy Plan (TOTP) Phase 1: Day of rest and recover - For the first 24-36 hours after PCA is initiated, rapidly titrate to get pain under control (up-titrating dose 25%-50% every 2 hours as needed) - Continue home basal PO pain regimen - 2x home regime if pain is severe - Adjunctive pain managements are utilized - Lidocaine Goal: Adequately control pain
Tiered Oral Therapy Plan (TOTP) Phase 2: Hold Steady - 24 hour period begins when the patient has reached an appropriate level of analgesia - No escalation or weaning of PCA for 24 hours - PO regime remains the same based on severity of pain
Tiered Oral Therapy Plan (TOTP) Phase 3: Initiate weaning of PCA - PCA weaned by 25% after first dose (usually every 12 hours) - Oral pain meds are tiered down and scheduled farther apart - Home pain regime is continued as Per Nursing: this is when discharge planning start - when PT/OT is often consulted
Tiered Oral Therapy Plan (TOTP) Phase 4: Continued weaning of PCA - 24 hour period of continued weaning Phase 5: Discharge by noon
Mobility Plan Mobility starts following a day of rest (24 hours after admission) Stage 1: Patient up out of bed with each meal (30 mins, 3x a day) Stage 2: Patient up with each meal + ambulate unit twice per day Stage 3: Patient up with each meal + ambulate unit 3-4 times a day
1. Opioids and SCD 2. Exercise and SCD
Opioids and SCD
PO home basal regime PCA - up-titrated then weaned PRN oral dose based on pain severity Three opioid regimes
Relationship between Opioids and SCD Ruta et al. - While SCD population is exposed to opioids often, they make up a very small portions of those affected by opioid overdose - Equally, when compared to non-cancer individuals with chronic pain, SCD makes up the second fewest deaths due to opioids
Opioids and SCD Continued
Relationship between opioids and SCD Akinboro et al - SCD hospitalizations are increasing but the death rate is remaining stable - Equally there was a 350% increase in opioid related deaths in the general population
Exercise and SCD
Mobility, Exercise,and Sickle Cell Disease Prescribing exercise to patients with sickle cell disease is difficult. - Due to the mechanics of HgB mutation, any event that increases the acidic concentration in the blood is more likely to induce sickling of the red blood cells. The main events that may precipitate such changes include: - Low oxygen level in the blood (hypoxia) - Dehydration or loss of body fluids (concentrates acids in the blood) - Infections
SCD population and exercise - In a study conducted with SCD children and healthy children it was found that - SCD children showed significantly worse performance with aerobic tasks - Higher HR and blood lactate levels in SCD group - Lower performance in anaerobic tasks (jumping ) - Greater energy costs at the same, low intensity - Propagating factors for Vaso-Occlusive Crisis associated with exercise - increased dehydration - increased lactic acid production in active muscles - presence of anemia propagates the switch from aerobic to anaerobic metabolism
Benefits of Exercises in SCD population - Alcorn et al. demonstrated that exercise therapy may reduce length of hospitalization stay in SCA children with VOCs - Exercises were of moderate intensity and lasted 10-30 mins - Tinti et al. demonstrated that patients who underwent 2 times a week of 5 weeks of an exercise program had decreased pain - Improved quality of life scores - Barbeau et al demonstrated that moderate exercise could decrease risk of inflammatory reaction to exercise (increase exercise tolerance) and increase vasodilatory reserve - Theoretically may decrease vaso-occlusive crises
So what we should do? - Balayssac-Syransy et al demonstrated that moderate exercise (50% of aerobic max) of 20 mins did not cause hematological alterations - it is safe! - Careful attention should be given to gradually introduce exercise - Mobility plan accounting for hematological changes after vaso-occlusive crisis - Avoid intense exercise and stop at the first sign of fatigue Suggestions - Prolonged exercise longer that 20 mins should be avoided - Rest break every 20 mins - Self-Monitoring education is key to limit over-exertion - Hydrate
Recap: Take home points - Trust the process - While the opioid crisis is real, SCD population has been seen to have legitimate benefit from them - SCD patients should be seen through a different lens than other patients - Self-Monitoring and hydration education is key - RPE scale is your friends - 35%-60% VO2 max is suggested (between 11-13 on the RPE scale) - This can go a long way in introducing person to exercise if they were at first unfamiliar or scared to exercise
Opioid Use Is NOT Associated with in-Hospital Mortality Among Patients with Sickle CELL Disease in the United States: Findings from the National Inpatient Sample Oladimeji Akinola Akinboro, Stanley Nwabudike, Camille Edwards, Diana Cirstea, Nana Oduraa Addo-Tabiri, Michael Voisine, Hassan Yameen and Adetola A. Kassim The Opioid Drug Epidemic and Sickle Cell Disease: Guilt by Association Nadia S. Ruta, JD Samir K. Ballas, MD, FACP Sickle Cell Anemia (Sickled Red Blood Cells) https://www.healthhype.com/sickle-cell-anemia-sickled-red-blood-cells.html Borhade MB, Kondamudi NP. Sickle Cell Crisis. [Updated 2019 Jun 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK526064/ Habibi, E., Dehghan, H., Moghiseh, M., & Hasanzadeh, A. (2014). Study of the relationship between the aerobic capacity (VO2 max) and the rating of perceived exertion based on the measurement of heart beat in the metal industries Esfahan. Journal of education and health promotion, 3, 55. doi:10.4103/2277-9531.134751 Musculoskeletal Manifestations of Sickle Cell Disease: A Review Raju Vaishya , Amit Kumar Agarwal , Edwin O. Edomwonyi , Vipul Vijay Balancing exercise risk and benefits: lessons learned from sickle cell trait and sickle cell anemia Hematology 2018 2018:418-425; Exercise limitation, exercise testing and exercise recommendations in sickle cell anemia Philippe Connesa,b, ∗ , Roberto Machadoc, Olivier Huea and Harvey Reidd E.A. Balayssac-Siransy, P. Connes, N. Tuo, C. Danho, M. Diaw, I. Sanogo, M.D. Hardy-Dessources, A. Samb, S.K. Ballas and P. Bogui, Mild hemorheological changes induced by a moderate endurance exercise in patients with sickle cell anemia, Br J Haematol 154 (2011), 398–407. R. Alcorn, B. Bowser, E.J. Henley and V. Holloway, Fluidotherapy and exercise in the management of sickle cell anemia. A clinical report, Phys Ther 64 (1984), 1520–1522. G. Tinti, R. Somera Jr, F.M. Valente and C.R. Domingos, Benefits of kinesiotherapy and aquatic rehabilitation on sickle cell anemia. A case report , Genet Mol Res 9 (2010), 360–364 P. Barbeau, K.F. Woods, L.T. Ramsey, M.S. Litaker, D.M. Pollock, J.S. Pollock, L.A. Callahan, A. Kutlar, G.A. Mensah and B. Gutin, Exercise in sickle cell anemia: Effect on inflammatory and vasoactive mediators, Endothelium 8 (2001), 147–155.
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