Mystery Case Study - Sickle Cell Crisis

Sickle cell crisis is an acute episodic condition that occurs in children with sickle cell anemia (Otoole, 2017). According to the NIH (National Heart, Lung, and Blood Institute) sickle cell crisis “can occur without warning when sickle cells block blood flow and decrease oxygen delivery. People describe pain as sharp, intense, stabbing, or throbbing. Severe crises can be even more uncomfortable than post-surgical pain or child birth. ” Due to its extremely painful nature sickle cell crisis is often referred to as acute pain crisis or episodes. Sickle cell crisis is a medical emergency, vasoocculsion can lead to organ infarct. Crisis can be brought on by various conditions such as: dehydration/not enough water consumption, drastic change in temperature, high altitudes, stress, or illness.

Patient History

Kayla is a 7 y. o. African American female with sickle cell disease. Kayla was at a friend’s back to school party. Kayla was playing hard all day in hot summer weather. Kayla did not take breaks to rest and drink water, she didn’t want to miss out. Towards the end of the party Kayla was pushed in the cold swimming pool by one of her classmates. The following day Kayla’s symptoms began.

Chief Complaint

Patient stated “It (the pain) started after Krissy’s party (yesterday). It hurts really bad all over. I just want it to stop. Mom tried to warm me up with blankets, but it didn’t work. ”

History of Present Illness

After waking the following morning Kayla was fatigued and sclera had yellow coloring to them(Mayo Clinic, 2018). Upon pain onset intensity rapidly increased, pain rating of 10 on Wong-Baker FACES pain scale. Pain throbbing and stabbing in bilateral legs, lower back, and chest (NIH, n. d. ). Pain unrelieved by warming via blankets and over the counter pain medication.

Past Medical History

Kayla was hospitalized six months ago due to sickle cell crisis. She has had no previous surgeries. Kayla was diagnosed with sickle cell disease when she was 5 months old. She has received all appropriate immunizations, such as: Dtap, MMR, Varicella, Hep A, Pneumoccal, Hep B. Current medications consist of over the counter pain medications and a daily multivitamin. Kayla has no known allergies. There is no history of mental health issues. History of multiple blood transfusions.

Family History

Mother is living, has sickle cell trait and depression. Father is also living, has sickle cell trait, diabetes mellitus type 2, uncontrolled hypertension, and is obese. Maternal grandmother is living, has hypertension and breast cancer. Maternal grandfather is living, has sickle cell disease, depression, and chronic kidney disease. Paternal grandmother is living, has sickle cell trait, diabetes mellitus type 2, and hypertension. Paternal grandfather is living, has hypertension, asthma, and skin cancer. Maternal and paternal grandparents deceased at unknown age and cause.

Personal and Social History

Born in New Jersey and lived there until she was 4 years old. Moved to Maryland with mother, father, and 3 siblings. The move was due to father’s job relocation. Father is a college professor and mother is a hairstylist. Youngest out of four siblings: brother Marvin 12 years old, twin sister Shamika and T’onna 8 years old. Attends Forest Lakes Elementary School. Due living in close proximity to school Kayla must walk to school. Extracurricular activities consist of: cheerleading, girl scouts, and soccer. Kayla does not smoke, drink alcohol, or use illicit drugs. Unhealthy diet consisting of fast food, processed foods, and high caffeine intake. Kayla is not sexually active.

Skin

Coloring appropriate for ethnicity, uniform coloring, and normal turgor. Skin temperature hot to touch, and patient is febrile. No tattoos or scars present. No edema present, skin dry, and no unusual odor. No lesions present. HEENTHead is symmetrical shape and size with head upright in normal position. Facial features and spacing symmetrical. No presence of edema or abnormal findings. Pupils equal, round, reactive light, and can perform accommodation. Periorbital puffiness related to crying due to pain. Sclerae are bilaterally yellow. No visual deficits noted. Nose is midline and symmetrical. Nares are bilaterally patent. Rhinorrhea with thin clear mucus present related to crying. Able to properly discriminate odors and there are no deficits present. Bilateral upper lateral incisors missing. Lips are dry and oral mucous membranes are pink and intact. The tongue is symmetrical and in midline. Gag reflex is present and uvula midline. The voice quality is normal, and sense of taste is intact. Neck shows, trachea midline, swallow reflex is intact. Thyroid is within normal limits.

Chest and Lungs

Chest expansion symmetrical with no presence of retractions or use of accessory muscles. Chest is tender upon palpation (NIH, n. d. ). Tachypnea present with shallow breath depth. Lung sounds diminished bilaterally posterior and anterior. Symmetrical tactile fremitus present. No cough or deficits found. Breast and Female Genitalia Breast are within normal limits for prepubescent female. Nipples are bilaterally symmetrical in shape and size. There are no lesions or scarring present. There are no abnormal findings or deficits present in breasts. Female genitalia is within normal limits for prepubescent female. There is no presence of discharge or lesions found.

Heart and Blood Vessels

Heart rate normal rate and rhythm. S1 and S2 heard with no presence of palpitations or murmurs. Blood vessels within normal limits. Radial and pedal pulses palpable and bilaterally equal. There is no presence of jugular vein distention. No abnormal finding or deficits present. AbdomenAbdomen within normal limits. Normal shape and contour, bowel sounds active and present in all four quadrants. Abdomen is non-tender during palpation. No friction rub heard over spleen or liver. CVA not present and no abnormal findings or deficits present.

Anus and Rectum

Anus and rectum intact and within normal limits. No excoriation or inflammation. There are no signs of hemorrhoids, fissures, skin tags, or hair tufts present. No abnormal finding or deficits present.

Lymphatic System

Lymphatic System within normal limits. Lymph nodes present with normal shape and size. Lymph nodes are non-tender upon palpation. There is no redness or streaks in localized area noted. No deficits or abnormal findings present.

Musculoskeletal System

Normal posture, symmetrical alignment of extremities. Body parts symmetrical with symmetrical muscle tone. Joints are within normal limits. There is no swelling present in joints. There is limited ROM and weakness bilateral legs.

Neurologic System

Neurologic system is within normal limits. Cranial nerves assessed with benign findings. Normal gait and balance present. Sensory functions assessed with benign findings. Superficial and deep tendon reflexes present. No abnormal findings or deficits present.

Labs and Diagnostic Test

Patients when in sickle cell crisis often times are anemic. To test for anemia a CBC would need to be done. An ABG would be done because when in sickle cell crisis patients frequently become hypoxic due to the vaso occlusion in sickle cell crisis. Patients with sickle cell disease are more susceptible to infection, there for the following labs would need to be done: CBC with WBC differential, blood cultures, urine culture, urinalysis, and sputum sample. Kayla presented with and elevated temperature which is a sign of infection, however it is not abnormal to have an elevated temperature without infection during sickle cell crisis. Pulse oximetry would need to be done related to Kayla’s tachypnic breathing and sickled cells causing decreased perfusion. It is important to make sure that there is adequate oxygenation during sickle cell crisis. Vasoocculsion can lead to decreased tissue perfusion, thus leading to possible organ infarct.

The following labs would be needed to monitor for organ damage: ABG, CBC, LDH, AST/ALT, CPK, and BUN. Serum electrolytes and urine electrolytes are needed due to the increased chance of electrolyte losses during sickle cell crisis. Aggressive hydration is a main part of the treatment for sickle cell crisis. Electrolytes may also be lost during sickle cell crisis because patients frequently experience diarrhea, vomiting, and diaphoresis. Sickle cell crisis can lead to acute chest syndrome. Kayla’s elevated temperature and complaints of chest pain are symptomatic of acute chest syndrome. A chest xray would need to be done to rule out acute chest syndrome.

Discharge Instructions and Patient Teaching

After vigorous intravenous hydration, pain management, and red blood cell transfusions Kayla is out of sickle cell crisis and ready to be discharged to home with her parents. Discharge instructions and patient teaching will begin from the moment Kayla is admitted. I will begin by assessing Kayla’s and her parent’s knowledge level of sickle cell disease and sickle cell crisis. Important to figure out what the baseline knowledge level is. When providing discharge instructions, it will be important to spend one on one time with Kayla helping to educate her and help her have a sense of independence with preventing sickle cell crisis and living with sickle cell disease. It will also be important to spend time educating Kayla’s parents so that they will be able to help Kayla.

Discharge Instructions

The following recommendations are from the Mayo Clinic to avoid complications of sickle cell anemia:

• Take folic acid supplements daily and choose a healthy diet. Bone marrow needs folic acid and other vitamins to make new red blood cells. Your doctor might recommend a folic acid supplement. Choose a diet that focuses on a variety of colorful fruits and vegetables, as well as whole grains.
• Drink plenty of water. Dehydration can increase your risk of a sickle cell crisis. Drink water throughout your day, aiming for about eight glasses a day. Increase the amount of water you drink if you exercise or spend time in a hot, dry climate.
• Avoid temperature extremes. Exposure to extreme heat or cold can increase your risk of a sickle cell crisis.
• Exercise regularly, but don't overdo it. Talk with your doctor about how much exercise is right for you.
• Use over-the-counter (OTC) medications with caution. Use OTC pain medications, such as ibuprofen (Advil, Motrin IB, Children's Motrin, others) or naproxen sodium (Aleve) sparingly, if at all, because of the possible effect on your kidneys. Ask your doctor before taking OTC drugs.
• Find ways to cope with the pain. Work with your doctor to find ways to control your pain. Pain medications can't always take all the pain away. Different techniques work for different people, but it might be worth trying heating pads, hot baths, massages or physical therapy.
• Sickle cell support groups- there a local support groups in the area for people with sickle cell disease giving them someone they can talk to who is going through the same thing. There are also are support groups for families for them to have an outlet and resource to talk to other people facing the same challenges. When to see a doctor or seek emergency medical care

According to the Mayo Clinic: “Unexplained episodes of serve pain, such as pain in the abdomen, chest, bones, or joints. Swelling in the hands or feet. Abdominal swelling, especially if the area is tender to the touch. Fever, people with sickle cell anemia have and increased risk of infection, and fever can be the first sign of infection. Pale skin or nail beds. Yellow tint to the skin or whites of the eyes. ”(2018)

Mystery Case Study Summary

Through researching the etiology, it provided an understanding of what is happening in the body during sickle cell crisis. The following contributing factors led to crisis: exposure to extreme heat, dehydration, and drastic change in temperature. Sickle cell crisis then caused fatigue; pain rating of 10 in lower back, chest, and bilateral legs; tachypnea, bilateral yellow sclera, and anemia. Through lab work and diagnostic tests, it allowed for the development of a targeted plan of care. Initially, Kayla’s parents were resistant to patient teachings. By making Kayla and her parent’s involved in Kayla’s care it helped establish a trusting relationship with the healthcare team. This relationship proved to be vital when educating Kayla’s parents on changes and adjustments that needed to be made. Kayla’s readiness to learn and determination aided in providing discharge instructions and patient teachings. Setting Kayla up with a support group will give Kayla an opportunity to speak with kids her age going through the same things as her. Additionally, setting Kayla’s parent’s up with a support group will help them feel supported.