Care of School Age Children with Sickle Cell Disease. Lesley Ann Owen, RN, MSN Vanderbilt Children s Hospital Pediatric Sickle Cell Clinic
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1 Care of School Age Children with Sickle Cell Disease Lesley Ann Owen, RN, MSN Vanderbilt Children s Hospital Pediatric Sickle Cell Clinic
2 Sickle cell disease around the world African Americans Latin Americans Latin Americans Middle Easterners Middle Easterners Indians Africans Africans Indians
3 1/12 African Americans carry Sickle cell trait 1/4 West Africans carry Sickle cell trait 1/100 1/10 Central Asians carry Sickle cell trait 1/400 African Americans have Sickle Cell disease 1/1000 Hispanic Americans have Sickle Cell disease ~ 70,000 Americans have Sickle Cell disease
4 Types of Sickle Cell Disease SS SC SB+thal SB0thal SD SE
5 Normal red blood cell Sickle cell Red blood cell with hemoglobin A Red blood cell with hemoglobin S
6 The Sickling problem Hgb A with O2 RBC is round Hgb A without O2 RBC is round Hgb S with O2 RBC is round Hgb S without O2 Hgb S stick together to form a rod, the rods come together to form a cable, which kinks the red blood cell into a rigid sickle shape.
7 What are some of the complications of sickle cell disease? Fatigue Jaundice Breathlessness Back Pain Hand Swelling Arm and Leg Pain Joint pain Foot swelling
8 Types of pain Acute sickle cell pain crisis - minutes to days, caused by blocked blood flow from the sickle RBCs. Usually deep in the bones and muscles of the arms, legs, and back. Chronic pain from sickle cell bone damage (avascular necrosis, osteonecrosis) - lasts longer than a few weeks and may be present constantly, due to bones being damaged by blocked blood flow. Often in the hips. Hydroxyurea prevents pain!
9 Treatment for pain Ibuprofen = Motrin = Advil. Blocks pain in muscles and bones. Does not make you drowsy. Can cause stomach upset and ulcer, and should be taken with food in the stomach. Good for menstrual cramps. Lortab = hydrocodone + tylenol. MS Contin = long-acting morphine. Best for chronic pain. MS IR = immediate release morphine. Good for breakthrough pain, if already on long acting morphine. Methadone - a form of morphine that is not addicting. Good for chronic pain.
10 Fatigue Sickle cells break open (hemolysis) Anemia (not enough RBCs) Low energy More RBCs More energy Hydroxyurea: increases hemoglobin F, prevents sickle cells from breaking down Folic acid : helps make more red blood cells
11 Stroke = blocked blood flow in the brain Area of brain deprived of blood Blood clot Blood vessel Blood unable to pass 10% of sickle cell patients have stroke
12 Signs of Acute Chest Syndrome: Fever Labored breathing Wheezing Chest pain Oh, no! normal Treatment: Oxygen, antibiotics, breathing treatments, transfusion
13 Prevention of Acute Chest Syndrome (ACS) Hydroxyurea! Albuterol & Flovent inhaler!
14 Pain prevention and management F - for Fluids and Fever. Drink lots of water. Carry a water bottle. If you get a fever, see your doctor. A - for Air. Get enough oxygen. No airplanes with unpressurized cabins, commercial airplanes OK. R - for Rest. Do not overdo it. Take plenty of breaks when your body feels tired. M - for preventive Medicines. Hydroxyurea for pain prevention. Folic acid for making new red blood cells. Asthma meds to prevent asthma attack and chest pain. S - for Situations to avoid, like too hot or too cold. Avoid smoking, alcohol, or illegal drugs. Avoid emotional stress by pacing projects and work. Join a support group.
15 What School Nurses need to know about Sickle Cell Disease Assessment Observations Recommendations 1. Pain Event Complaint of arms, legs, back, or abdomen hurting 2. Fever Temp or higher Notify parent Rest, notify parent. Administer medication according to medical provider's approval 3. Hot Weather Temperature > 90 degrees Dress lightly, increase fluids, cool area or air conditioned area, limited outside activity. Increase frequency of water and bathroom breaks. 4. Cold Weather Temperature < 40 degrees Dress warmly, hat, gloves and boots in the snow. No outside activities 5.Fatigue 6.Nose bleeds 7.Decrease school performance 8.Slurred speech, limp or headache, decrease concentration 9.Growth and Development Decreased activity, decreased class participation, sleeping in class Bleeding continues without intervention Gradual or abrupt change in behavior or academics Change in physical or cognitive ability May be smaller than other peers( but not necessarily) Rest, limitation of physical activity including gym Apply pressure for 20 min. do not apply ice Check hearing, vision. Notify parent, may require testing, IEP May signal impending stroke. Notify parent. If no response after 30 min call ambulance. Will mature at a slower rate 10.Other injuries Any injury to extremities, hit in eye DO NOT apply ice to area, ok to use heating pad. Notify parent
16 Famous people with Sickle Cell Disease PAUL WILLIAMS, THE TEMPTATIONS Miles Davis
17 Tionne T-Boz Watkins, singer (TLC) Larenz Tate, actor
18 ANY QUESTIONS?
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For the Patient: Eribulin Other names: HALAVEN
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