Hematology & Endocrine Disorders

• Sarah Nixon RN MN adapted these slides from work by Leah Tellier RN, MN, CCNE, CHSE, and Kara Sealock EdD MEd BN RN CNCC

Topics Included:

• Hematology and Endocrine issues are focused on in these notes
• Hematology will cover disorders of erythrocytes
• Iron deficiency and anemia will be key in these notes
• Sickle cell crisis, and disorder of coagulation such as - Hemophilia
• Endocrine will cover hyper/hypothyroidism and Hypercortisolism – Cushing's Syndrome
• Adrenocortical hypofunction and Addison's disease
• Chronic complications of diabetes mellitus
• Syndrome of Inappropriate Antidiuretic Hormone (SIADH)

Learning Outcomes

• Critical reflection upon the overall anatomy and physiology of the hematologic and endocrine systems will be achieved
• Explain how chronic illness leads to significant changes in overall systemic functioning of Hematological and Endocrine disorders.
• Begin to prioritize patient conditions related to nursing assessment and clinical manifestations

Red Blood Cell Pathologies

• Iron Deficiency Anemia (IDA) will be highlighted
• Sickle Cell Disorders (SCD) will be addressed

Iron Deficiency Anemia (IDA)

• Lack/loss of iron causes a decrease in production of RBCs
• Blood loss, caused by pregnancy [and lactation] and heavy menstruation
• Chronic causes include GIB, ulcer, colorectal cancer, alcohol abuse/dependence, cause IDA
• The most common nutritional deficiency in children globally
• IDA can cause developmental delays, behavioural problems
• Common amongst children in the phases from birth to age 2 – rapid growth and in adolescence – rapid growth and potential poor eating habits

IDA Pathophysiology

• Inadequate supply of iron
• Needed to produce hemoglobin of RBCs
• Low serum ferritin and transferrin lead to low hemoglobin and hematocrit levels

IDA Clinical Manifestations

• Early signs: Fatigue, Weakness, Shortness of breath, and pallor to earlobes, palms and conjunctiva
• Brittle, spoon-shaped nails or concave (koilonychia)
• Angular stomatitis/Angular cheilitis
• Pica (eating non-food materials i.e. dirt, chalk, paper)
• Irritability
• Decreased activity tolerance
• Weakness
• Smooth, sore tongue
• Glossitis
• Dyspnea
• Dysphagia

IDA Clinical Manifestations in Pediatrics

• Mild Anemia includes lethargy and listlessness [~80-110g/L] and behavioural issues
• General irritability, decreased activity tolerance, weakness, and lack of interest in play
• Severe IDA leads to pallor, tachycardia, and systolic murmurs [below 70/80 g/L]
• Also leads to cognitive impairment (long-lasting and irreversible)
• Results in teens scoring lower on cognitive and motor tests

Causes of IDA in Adults

• Dietary deficiencies: Vegetarian diet and inadequate protein intake
• Impaired absorption: Partial or total gastrectomy, chronic diarrhea, and malabsorption syndrome
• Increased metabolic requirements: Pregnancy and lactation
• Chronic blood loss: Gastrointestinal bleeding (ulcers/PUD, chronic use of aspirin) with heavy menstrual losses

Causes of IDA in Pediatric Population

• Dietary Deficiencies: Inadequate intake
• Impaired Absorption: Celiac disease, Chronic Diarrhea and Malabsorption syndrome
• Increased Requirements: Growth spurts
• Chronic Blood Loss: GI damage, Parasitic infestation and Hemorrhagic disease

IDA Diagnostic Evaluation

• CBC, serum iron, iron-binding capacity, serum ferritin, transferrin saturation, reticulocyte count, red cell distribution width (RDW), and peripheral blood smear
• Bone marrow biopsy is rare

IDA Nursing Considerations

• A nurse is performing an assessment on a client with a diagnosis of anemia that developed as a result of blood loss after a traumatic injury. The nurse would expect to find shortness of breath with activity
• Nurse is caring for a patient admitted with iron deficiency anemia. Upon reviewing the patient's lab values. White blood cells 18 x 109/L would be the most concerning

Sickle Cell Anemia

SCD Information

• Inherited and results in an abnormal version of Hemoglobin S (HbS) in RBCs
• HbS distorts RBCs into a sickle shape

SCD Disorders

• Sickle cell trait is the most common form of sickle cell disease
• Heterozygous condition means the child has one sickle cell hemoglobin gene and one normal hemoglobin gene
• An afflicted child is a carrier of sickle cell anemia and rarely has symptoms of the disease
• Sickle cell anemia is a Homozygous condition
• An afflicted child has two sickle hemoglobin genes and is subject to sickle cell crises

SCD Pathophysiology

• Sickle cell trait: one beta-globin replaced with HbS
• Sickle cell anemia: both beta-globins replaced with HbS
• HbS causes RBCs to distort into sickle or crescent shape during times of deoxygenation
• HbS RBCs in sickle shape are inflexible and get stuck in small blood vessels
• "Sickling and unsickling damages RBC cell membranes leading to hemolysis
• HbS RBCs die prematurely-can lead to anemia
• Sickled cells can resume a normal shape when rehydrated and re-oxygenated
• Bone marrow spaces enlarge in response, to produce more RBCs or erythropoiesis

SCD Symptoms

• Varies from person to person and is a Chronic hemolytic anemia
• Frequent and recurrent infections that create susceptibility in inflicted parties
• Episodes of pain – crises – in different parts of the body such as bones and joints, in the form of acute chest syndrome or abdominal issues.
• Initiated by stress, infections, fever, cold, dehydration, excessive exercising and high altitudes
• Causes acute painful crises and is a result of sickled RBCs blocking small blood vessels in various organs.

SCD Risk Factors

• Sub-Saharan African, Indian, Saudi Arabian, and Mediterranean ancestry
• High prevalence in areas where malaria is endemic
• Worldwide: 7.4 million people have SCD.
• Canada: ~ 6500 people including children have sickle cell disease

SCD Precipitating Factors

• Hypoxia, or low environmental or body temperature
• Excessive exercise, dehydration, infections, fever or pregnancy

HBSS Pain Crisis in Sickle Cell Anemia

• Hemolysis
• Bone marrow Hyperplasia/Infarction
• Stroke as a result of Thrombotic or hemorrhagic, subarachnoid bleeds
• Pain episodes, priapism, or psychosocial problems
• Anemia, or aplastic crisis and avascular necrosis
• Infections: CNS, pulmonary, GU, bone, or joints
• Chest syndrome, cardiomegaly, CHF
• Retinopathy or renal failure
• Infarction: bone, spleen, CNS, muscle, and bowel from obstructed blood vessels
• Sequestration crisis, involving spleen and liver
• a, urinary from hyposthenuria

Types of Sickle Cell Crisis

• Episodes cause increased numbers of HbS RBCs to sickle leading to symptoms of acute pain resulting from ischemia
• Vaso-occlusive thrombotic
• Blood verss all over the body
• Splenic sequestration
• Spleen enlarge and rupture
• Aplastic crisis
• Separation of bone marrow

Coagulation Disorders: Hemophilia

Notes on Clotting Factors

• Fibrinogen (Factor I) functions as an adhesive protein that forms the fibrin clot and leads to congenital deficiency (afibrinogenemia) and dysfunction (dysfibrinogenemia)
• Prothrombin (Factor II) in its activated form is the main enzyme of coagulation. A deficiency or dysfunction leads to Congenital deficiency or dysfunction
• Labile factor, or proaccelerin (Factor V) serves as a cofactor for activation of prothrombin to thrombin and can lead to Congenital deficiency (parahemophilia)
• Stable factor or proconvertin (Factor VII) works with tissue factor and initiates extrinsic pathway. A shortage leads to Congenital deficiency
• Antihemophilic factor (Factor VIII) serves as a cofactor for intrinsic activation of factor X and a Congenital deficiency is hemophilia A (classic hemophilia)
• Christmas factor (Factor IX) in its activated form is an enzyme for intrinsic activation of factor X. A Congenital deficiency contributes to hemophilia B
• Stuart-Prower factor (Factor X) in its activated form is an enzyme for final common pathway activation of prothrombin and Congenital deficiency
• Plasma thromboplastin antecedent (Factor XI) serves in activated form as an intrinsic activator of factor IX. Congenital deficiency is sometimes referred to as hemophilia C
• Hageman factor (Factor XII) nominally starts aPTT-based intrinsic pathway and Congenital deficiency is not associated with clinical symptoms
• Fibrin-stabilizing factor (Factor XIII) works as a transamidase that cross-links fibrin clot and leads to Congenital deficiency

Hemophilias

• Hereditary bleeding disorders caused by mutation of the genes that code for the clotting factors VIII or IX
• This leads to deficiencies of clotting factors VIII, IX, XI
• Two types - both are X-linked recessive and leads to Hemophilia A and B:
• Hemophilia A - Factor VIII deficiency and effects 80% of patients
• Hemophilia B - Factor IX deficiency- Also called Christmas Disease

Hemophilia Prognosis

• Severity depends on amount of the particular clotting factor in the blood
• The lower the amount the greater the severity
• There is no cure - but the disease can be managed with a control of symptoms
• The existing Treatment is to replace the deficient clotting factor but this can be episodic or regular
• Patients with mild-to-moderate hemophilia can live near-normal lives

Hemophilia A Pathophysiology

• Disease presents as an X-linked recessive trait
• Males are mostly affected
• Females may be carriers and occasionally symptomatic
• Shortage of Factor VIII (antihemophilic factor- AHF)

Hemophilia Clinical Manifestations

• Prolonged bleeding and excessive bruising
• Subcutaneous and intramuscular hemorrhages
• Hemarthrosis (bleeding into a joint space)
• Signs of swelling, warmth, redness, pain and loss of movement of limbs
• Bleeding in the neck, mouth, or thorax
• Hematuria is common
• Anemia
• Hemorrhage from any trauma (cuts, injections, circumcision)
• Hematomas in the spinal cord and potential paralysis

Hemophilia Diagnosis

• Based on the basis of history of bleeding, laboratory findings, and family history
• Platelet count, prothrombin time (PT), partial thromboplastin time (PTT) will be evaluated
• Factor VIII and IX assays are needed to determine type and severity of hemophilia
• Pregnancy can be diagnosed through amniocentesis, bearing in mind the increased risks
• Genetic testing of family members to identify carriers with possible genetic counselling as needed

Hemophilia Nursing Considerations

• Would there be expected abnormalities in patients Platelets, hemoglobin, and/or hematocrit with hemophilia? More information needed

Laboratory Tests of Coagulation

• Evaluated during patient interaction of the blood disorder
• Assays include: Thrombin time, Prothrombin time (PT), Activated partial thromboplastin time (PTT or APTT), PT or APPT mixing study, and Specific factor assay
• Thrombin time measures fibrinogen level; usually elevated first because without fibrinogen, blood cannot clot
• Prothrombin time (PT) Decrease indicates a deficiency of factors II, V, VII, or X; also used to monitor warfarin
• Activated partial thromboplastin time (PTT) Assesses for factors XII, XI, IX and VII; also used to monitor heparin therapy
• PT or APPT mixing study Differentiates between factor deficiency and factor antibody activity
• Specific factor assay Measures specific factors; XIII, XII, XI, X, IX, VII, VII, V, II, fibrinogen (factor 1)

Endocrine System Dysfunction Alterations

• Hyper/hypothyroidism
• Hypercortisolism
• Adrenocortical hypofunction
• Chronic complications of diabetes mellitus
• Syndrome of Inappropriate Antidiuretic Hormone (SIADH)
• Diabetes Insipidus (DI)

Hypothyroidism & Hyperthyroidism

• Thyroid hormone (TH) regulates the basal metabolic rate (BMR) as well as the protein, carbohydrate and fat metabolism
• Thyroid secretes two types of hormones: TH -- made up of: Thyroxine (T4) 90% - reservoir for T3 and Triiodothyronine (T3) 10% -- most active form
• Calcitonin -responsive to hypercalcemia
• Hyperthyroidism comes from hypermetabolism
• Hypothyroidism stems from slowed metabolism

Risk Factors for Thyroid Disease

• Men: Age > 60 years old
• Women: Age > 50 years old
• Personal history or strong family history of thyroid disease
• Includes any diagnosis of other autoimmune diseases
• A past history of neck irradiation
• Previous thyroidectomy or radioactive iodine ablation
• Drug therapies such as lithium and amiodarone
• Dietary factors concerning iodine excess and iodine deficiency
• Certain chromosomal or genetic disorders, like Turner syndrome, Down syndrome and mitochondrial disease

Hypothyroidism

• Hashimoto's thyroiditis (autoimmune) can occur
• Is considered to be and described as "Low and Slow" and leads to:
• Low energy
• Low metabolism resulting in weight gain and water gain, leading to edema
• Low digestion and constipation
• Low Hair loss (alopecia) a dry skin turgor.
• Becomes severe enough it is considered a medical Low Respiatory Rate, Respiratory failure Low Blood Pressure and Heart Rate emergency with the diagnosis as Myxedema Coma which features
• Low Temp “cold intolerance"
• Significant Clinical Manifestations including: Fatigue and weakness, Cold intolerance, Dyspnea on exertion, Weight gain and cognitive dysfunction. Also mental retardation (infantile onset), Constipation and Growth failure
• Coarse skin, Hoarseness and Edema
• Puffy facies and loss of eyebrows and Periorbital edema along with enlargement of the tongue
• Diastolic hypertension with pleural and pericardial effusions or ascites also can be apparent
• Decreased hearing
• Myalgia and paresthesia
• Depression or Pubertal delay

Hypothyroidism Assessment

• Observe for early and subtle changes like weight gain, and mental status changes
• Ask patient about fatigue, slowed and slurred speech
• Watch for: cold intolerance and dry skin, constipation and dyspnea, as well muscular aches and pains Also look for bradycardia and "distended abdomen

Potentional Complications

• Myxedema Coma is is medical emergency involving a long history of hypothyroidism with uncontrolled low thyroid production precipitated by illness, infection, trauma, meds that suppress CNS, and exposure to cold
• Presents Clinically as: Decreased mental status/LOC or coma, Hypoventilation, Hypothermia, Hypotension, Seizures, Shock, or Myxedema from thickened, nonpitting edema of skin
• Can stem from a primary concern or cause:
• Globally in iodine-deficient regions because of a lack of Iodine
• Resulting in Autoimmune thyroiditis or Hashimoto's disease
• Primary Hypothyroidism: Hashimoto's thyroiditis (autoimmune), Congenital, Surgical removal of thyroid gland, Ablation with radioactive iodine, Radiotherapy and Thyroid tumor
• Drug toxicity of lithium, interferon, amiodarone
• Secondary Hypothyroidism from Disorders of pituitary or hypothalamus
• A nursing mnemonic for the differing states of Hypothyroidism: INCREASED TSH secretion with decreased T3 and T4, or decreased TSH secretion with decreased T3 and T4

Hypothyroidism Diagnosis

• History includes evaluating the underlying issue
• Serum TSH and free T4 levels
• Serum T3 [not sensitive to hypothyroidism] plus serum T4 levels
• Can involve a Thyrotropin-releasing hormone (TRH) stimulation test
• Thyroid peroxidase (TPO) antibody (TPOAb) test can be used to evaluate
• Thyroid ultrasound or nodules
• Thyroid Used to evaluate
• Lab tests include checking for increased TSH with a decreased T3 and T4 Normal TSH values are >7

Hypothyroidism Nursing Considerations

• Nurse is caring for a patient admitted with hypothyroidism. The nurse should anticipate patient experiencing Weight gain and cold intolerance

Hyperthyroidism

• Known as Graves' and results in significant gains and described as High and Hot
• Emergency Thyroid storm (Thyrotoxic crisis)
• Associated with Agitation, Confusion, and Restlessness
• Extremely High Blood Pressure with a High Heart Rate and Tachycardia
• Heightened temperature
• Nausea,Vomiting,and Diarrhea
• Dehydration
• Grap eye or Exopthalamos (bulging Eyes)
• Goiter
• Characterized by Heat Intolerance and High GI which can result in Diarrhea and excessive diaphoresis

Hyperthyroidism Clinical Manifestations

• Metabolic: Heat intolerance, increased sweating, weight loss
• Neurologic: Headaches, hyper-reflexia, extreme seizures
• Psychiatric: Personality changes-anxiety, irritability and nervousness
• Dermatologic: Thin hair, pretibial edema
• Ocular: Exophthalmos, light sensitivity, dryness/tearing
• Gastrointestinal: Diarrhea
• Gynecologic: Less frequent or lighter menses
• Cardiovascular: Tachycardia
• Other manifestations: hoarse voice, and slow speech

Hyperthyroidism Assessment

• Patient should be asked about: Weight loss and increased appetite Heat intolerance and night sweats Tachycardia Cardiac dysrhythmias from A-fib

Grave's Disease

• A major cause of hyperthyroidism has autoimmune causes from Autoantibodies binding to TSH receptors in thyroid and act like TSH to stimulate thyroid to release thyroid hormone
• A key factor includes an Opthalmopathy, Pretibial myxedema or Goiter

Thyroid Storm (Thyrotoxic Crisis)

• The most acute and life-threatening condition concerning Hyperthyroidism
• Occurs with poorly controlled hyperthyroidism.
• Precipitated by infection, surgery, trauma, seizures, obstetrical complications, emotional distress, or dialysis
• Characteristics: Hyperthermia, tachycardia, heart failure, tremor, extreme agitation, delirium, nausea, vomiting, diarrhea, dehydration, and coma

Causes of Hyperthyroidism

• Most common cause: Grave's Disease
• Thyrotoxicosis occurs in 1 in 100
• Autoimmune • Autoantibody effects against thyroid receptor for TSH stimulating the synthesis and secretion of over-excessive T4 & T3 Other common causes:
• Multinodular goiter or single, autonomous, hyperfunctioning "hot" nodule
• Thyroiditis Other causes include: • Excess iodide ingestion, or other reasons • Pituitary tumours will lead to excess secretion
• Thyroid cancer

Pathology of Hyperthyroidism

• Increased synthesis and secretion of thyroid hormones from primary reasons • Pituitary over-stimulates the thyroid to secrete thyroid hormones - the secondary reason. • Exogenous source of thyroid hormone or exogenous T3 as a way to lose extra weight.

-Increased levels of TH binding to receptors on mitochondria •The increase means an increase of metabolic rate/increase in the nutrient breakdown. •There is a metabolic rate that to lead to increased use of fat reserves.

Diagnosis of Hyperthyroidism

History examination T3 & T4 levels TSH level is in the level of primary Hyperthyroidism

• Increase TH causes negative, but there is an increase of TSH with high T3 and T4 causing secretion of the pituitary gland • Radioactive iodine uptake test - this test will determine a nodule or tumour.

Assessment

Indicate a potential tumor It will result in • The excess from the increased TSH levels

Nursing Considerations for Hypertthyroidism:

_Nurse involved in administering care for pretibial, exophthalmos, and likely goiter:

• Graves’ Disease

Goiter

• Indicates a major condition that causes the thyroid gland for growing more abnormal.
• Results for access stimulation of TSH.
• It will cause the Thyroid gland is cause to increase amount and size with produces more.
• The occurs/results by being having under production of thyroid hormone.

Summary

• Hypothyroidism has low circulating thyroid hormones. There includes a Myexedema Coma and can be Hashimoto’s Thyroidosis.

• Hyperthyroidism has the excess synthesis from the Thyroid storm is a thyrotoxis • Which as Grave's Diese

Clinical Presentation: Hypothyroidism can result by including the following

• Sensitivity cold
• Joint in Muscle Pain
• The excess is in the Heavy Periods
• Trouble Sleeping
• Difficulty having concentration _ The emergent needs with some conditions.

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• Adrenal Gland* The adrenal gland helps balance the body with salt and sugar This is done through a glucocorticoid or a mineralocorticoid. • Aldosterone or hydrocortisone. This balances BP and also sugar.

• Alteration can be related to a disease

It is to be noted that is alludes to Pathologies of the Adrenal Causes the Cushing's Syndrome which is Hypercortisolism Another is the Addison’s with less production like Hypocortisolism

Cortisol Information

• Controls stress/stimuli response, blood glucose levels, inflammatory responses
• When secreted in response to stress/stimuli, facilitates control of blood sugar – acts on liver, muscle, adipose tissue, pancreas
• Increased cortisol increases gluconeogenesis to increase production of glucose - body's need for energy

Cushing Syndrome Vs Addisons

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HYPERCORTISOLISM and Cushing Syndrome

• This is what's called "Cushion of Steroids"
• Caused by Cushing Disease • Forms in • Excess anterior pituitary
• Disorder of high levels of cortisol occurs
• May be the use of steroids or may be a prolonged use This may be the cause of excessive steroids from exogenous use which needs to be discontinued immediately
• It can be because of the conditions is reversible is the condition is discontinued with the steroids steroids is be precipitating withdrawal.

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Hypocortisolisм: ADDISON’s DISEASE

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Addison Nursing Considerations:

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