Sickle Cell Disease

Rationale for Cognitive behavior Therapy (CBT)

Negative emotions are brought about cognitive processes developed through influences of learning and temperament. Adverse life events elicit automatic processing, which is viewed as the casual factor. Cognitive Triad: Negative automatic thoughts center around our understanding of ourselves, others (the world), and future. Throughout CBT focus on cognitive beliefs and developing rational responses.

If only one parent has SCT, each of their children has a

-1 in 2 (50%) chance of having SCT. -1 in 2 (50%) chance that they will not have SCT.

Pain Management: Cognitive behavior Therapy (CBT)

-Relatively short-term, focused psychotherapy -Thoughts (what we think affects how we feel and act) then Behaviors (what we do affects how we think and feel) then Emotions (what we feel affects how we think and act)

What is the prevalence rate of sickle cell disease and sickle cell trait? In the Black/African American population? In the Hispanic population?

-Sickle cell trait: 1 in 12 African Americans. -Sickle cell disease: affects 90,000 to 100,000 people in the U.S i. 1 out of every 500 Black or African Americans. ii. 1 out of every 36,000 Hispanic Americans

What are some treatment options for someone with sickle cell disease?

-Treatments are different for each person depending on the symptoms. -Treatments can include receiving blood transfusions, maintaining a high fluid intake (drinking 8 to 10 glasses of water each day), receiving IV (intravenous) therapy (fluids given into a vein) and medications to help with pain. -For severe SCD, a medicine call hydroxyurea might be recommended.

Anemia

Anemia is a condition in which a person's blood has a lower than normal number of red blood cells, or the red blood cells don't have enough hemoglobin.

Cognitive Conceptualization

Current Situation - Automatic Thoughts about self, world and others - Physiology, feelings, behavior Childhood and early life events - underling assumptions and core beliefs (connects to Automatic thoughts) - compensation strategies (connects to part 3 of above) slide 34

What causes sickle cell disease/sickle cell trait?

SCD is inherited; a person is born with it.

Sickle Cell Disease

Sickle cell disease is a genetic condition that is present at birth. i. The red blood cells become hard and sticky and look like a C-shaped farm tool. ii. A person with SCD when both of his/her parents have at least one abnormal trait.

How do sickled cells cause episodes of severe pain, organ damage, serious infections, etc?

Sickle cells get stuck in small blood vessels and block flow of blood and oxygen to organs in the body. These blockages cause repeated episodes of severe pain, organ damage, serious infections or even stroke.

The Working Model of Cognitive behavior Therapy (CBT)

Triggering Event - Appraisal (perceive it) - Affective and Biological Arousal (stomach hurts, sweating, etc.) - Behavioral Inclination (don't want to do this) - Maladaptive Behavior (something that won't help) slide 29

The Problem with Sickle Cell Trait:

a. Sickle Cell Trait: Most people do not have any health problems but in rare cases if a person with SCT have pain when traveling to or exercising at high altitudes. i. Should drink plenty of water when exercise and contact and inform doctor is they notice blood in their urine.

Sickle Cell Trait

a. Sickle cell trait is a person carried a single gene for the sickle cell disease and can pass this gene along to their children. i. Can live a normal life.

Can Sickle Cell disease be cured?

a. The only cure available is a bone marrow and stem cell transplant. i. A bone marrow or stem cell transplant is a procedure that takes healthy stem cells from a donor and puts them into someone whose bone marrow is not working properly. These healthy stem cells cause the bone marrow to make new healthy cells. • Bone Marrow/Stem Cell Transplant- Risky • First case ever to have a teenager's sickle cell reversed with world-first therapy- Gene Therapy for 2 years

If one parent has SCT and the other parent has another abnormal hemoglobin gene (like hemoglobin C trait or beta- thalassemia trait)

each of their children has a i. 1 in 2 (50%) chance of having SCT. ii. 1 in 4 (25%) chance of having SCD (not sickle cell anemia). These other types of SCD can be more or less severe depending on the specific abnormal hemoglobin gene. iii. 1 in 4 (25%) chance that they will not have SCD or SCT.

The Problem with Sickle Cell Disease:

i. "Pain Episode" or "Crisis": Sickle cells don't move easily through small blood vessels and can get stuck and clog ii. blood flow. This causes pain that can start suddenly, be mild to severe, and last for any length of time. iii. Infection: People with SCD, especially infants and children, are more likely to experience harmful infections such as flu, meningitis, and hepatitis. iv. Hand-Foot Syndrome: Swelling in the hands and feet, often along with a fever, is caused by the sickle cells getting stuck in the blood vessels and blocking the blood from flowing freely through the hands and feet. v. Eye Disease: SCD can affect the blood vessels in the eye and lead to long term damage. vi. Acute Chest Syndrome (ACS): Blockage of the flow of blood to the lungs can cause acute chest syndrome. ACS is similar to pneumonia; symptoms include chest pain, coughing, difficulty breathing, and fever. It can be life threatening and should be treated in a hospital. vii. Stroke: Sickle cells can clog blood flow to the brain and cause a stroke. A stroke can result in lifelong disabilities and learning problems.

If both parents have SCT, each child that they have together has a

i. 1 in 2 (50%) chance of having SCT. Children with SCT will not have symptoms of SCD, but they can pass SCT on to their children. ii. 1 in 4 (25%) chance of having sickle cell anemia, one of several types of SCD. Sickle cell anemia is a serious medical condition. iii. 1 in 4 (25%) chance that they will not have SCD or SCT.

Prevalence of Symptoms at Age

• 6 months 6% • 12 months 31% • 2 years 61% • 6 years 92% • 8 years 96%

Signs and Symptoms

• Anemia - Fatigue - Pale skin - Nails beds - Jaundice - Shortness of breath • Pain (Sickle Cell Crisis) - Sudden episode of pain throughout the body - Common sites Bones Lungs Abdomen Joints - Lack of blood flow can cause pain and organ damage

Prevalence

• Approximately 100,000 Americans have sickle cell disease • Occurs among approximately 1 out of every 365 Black/African American births • Occurs among 1 out of every 16,300 Hispanic American births • 1 in 13 Black or African-American babies is born with sickle cell trait

Prevention of Pain

• Chronic pain will come after some acute pain • Identify what can trigger the "Crisis" such as stress, avoid extremes of heat and cold weather, don't travel airplane that is not cabin pressurized. • Maintain Healthy Lifestyle - Eating healthy - Exercising regularly - Staying hydrated - Avoiding alcohol - Avoiding smoking - Get enough sleep and rest • Regular medical checkups and treatments are important

Core Beliefs

• Core Beliefs Automatic Thoughts • Underlie and produce automatic thoughts • Assumptions influence information processing and organize understanding about ourselves, others, and the future. • Core beliefs remain dormant until activated by stress or negative life events. • Categories of core beliefs (helpless. worthless, unlovable) • Examples: Helpless (inadequate, loser, vulnerable, weak, inferior, defective, etc.), Unlovable (unlikable, unwanted, rejection, flawed, unattractive), Worthless (unacceptable, dangerous, hurtful, toxic, evil, crazy, broken, doesn't deserve to live) • Picking up on the themes the person is talking about, what their thoughts are revealing.

Treatments for Pain

• Goal is to relieve the pain; prevent infections, eye damage, strokes and control complications if they occur. • Pain Medicine: Typical things like ibuprofen; Acetaminophen, nonsteroidal anti-inflammatory drugs, and narcotics such as morphine. • Heating pads • Hydroxyeura, Folic Acid: Some side effects are severe where person can develop leukemia, dangerous infections, etc.

What brings on a pain crisis?

• Illness • Temperature changes • Stress • Dehydration • Being at high altitudes • Smoking • Consuming Caffeine

Complications

• Most common death with children who have sickle cell disease are pneumonia and acute chest syndrome. • Hand-Foot Syndrome: Sickle cells block the blood vessels and the hands keeping the blood there. • Splenic Crisis: helps filter the blood and fight infections, but spleen can hold too many sickle cells within itself and is at greater risk for infection. Results the spleen in shrinking and cannot clean the blood as well. • Infections • Acute Chest Syndrome: Severe pain in the chest, caused by clumps of sickle cells in the lungs. Difficulty breathing, sharp kind of pneumonia. • Delayed growth and puberty in children: Tend to be slight, slender, not going to hit puberty as early. Relates to amount oxygen and blood flow they have going on. • Stroke • Eye Problem • Priapism: Can have long extended erections that wont go away and can lead to infertility later on. • Gallstone • Ulcers on the legs • Pulmonary Arterial Hypertension • Multiple Organ Failure: Blockages at multiple points it can be that two or more vital organs will shut down at the same time.

Epidemiology of SCD Pain

• Pain drives the majority of interactions with the healthcare system. • 2 types of pain - Vaso-Occlusive Crisis (VOC): Known as sickle-cell crisis; An attack of acute pain due to vaso-occulison that is the hallmark symptoms of the disease. - Chronic Pain: Due to accumulation of organ/tissue

Cognitive Distortions

• Patients tend to make consistent errors in their thinking • Often, there is a systematic negative bias in the cognitive processing of patients suffering from pain. • Help patients identify the cognitive errors/ he is most likely to make

Mortality and Sickle Cell Disease

• Prognosis is good to fair: 95% of children will live beyond the age of 18 • In 1973, average lifespan was 14 years. • Life Expectancy: - 42 years for males - 48 years for females - Common cause of early death are stroke, acute chest syndrome, and acute episode of pain

Treating Acute SCD Pain in the Emergency Department

• Rapid clinical assessment and involve the patients about their medications, what works, last time took medication, comparing the pain episodes, etc. • Aggressive management typically involves opioids to manage cute pain crisis. • Frequent re-assessment and re-administration of pain medicine

Automatic Thoughts

• Stressful Situation Automatic Thoughts Negative Emotions • Happens spontaneously in response to situation • Occur in shorthand: words or images • Do not arise from reasoning • No logical sequence • Hard to turn off • May be hard to articulate

Addiction & Substance Abuse Among Patients with SCD

•Abuse and addiction among patients with SCD to be lower than, or at most the same as, that found in the general population.

Epidemiology of SCD Pain p2

•Iceberg model of SCD pain: Majority of the pain experience by patients with SCD isn't observed by healthcare providers •55% of patients reported pain on at least 51% of their days •29% of patients reported pain on at last 96% of their days

Diagnosing Acute SCD Pain

•Lab Work: None known •Radiographic Findings: None known •Vital Signs: None known •Relies on the patient's self-report by which is VOC is identified