Cellular Regulation:

Cellular regulation is the coordinated function of the physiological controls within the body to maintain homeostasis from changes to internal and external environment.

Anemias

Inadequate oxygenation delivery to body cells due to lack of RBCs or a dysfunction of RBCs within the blood

Acquired Aplastic

  • Bone marrow fails to produce blood cells (RBCs, WBCs, and platelets)
  • Destruction of red bone marrow due to significant tx (radiation, chemotherapy, and infection)
  • Amount of stem cells in bone marrow are significantly reduced
  • Onset is sudden

Hemolytic

  • Destruction of RBCs faster then they are created

Intrinsice factors

  • Cell membrane defects
  • Defects in hemoglobin structure and function

Extrinsic factors

  • Exposure to drugs, chemicals, venom, bacteria, or trauma

Nutritional

  • Absorption complications
    • Gastrectomy
    • Chronic diarrhea
    • Malsabsorption syndromes

  • Occur from conditions or dietary insufficiencies


    • Vegetarian diets
    • inadequate protien

  • Increased Metabolic requirements


    • Pregnancy

Blood loss

  • Acute or chronic loss of RBCs through active bleeding

  • Triggers compensatory mechanisms


    • increased heart rate
    • peripheral vessel constriction

  • Continued blood loss depletes iron stores


  • Normocytic anemia


    • can become microcytic in chronic cases

Iron Deficiency (Hypochromic)

  • Low hemoglobin caused by iron deficiency

  • Iron binds with protoporphyrin IX


  • Causes can be from low iron diets (vegetarians) and heavy menstruation

  • Most common type of anemia
  • Results from
    • Poor intake (vegetarian diet)
    • Increased demand (pregnancy)
  • Absorption issue
    • Celiac disease
    • Gastric bypass

Pernicious (B12)

  • Failure of stomach to absorb B12 due to lack of intrinsic factor or irreversible malabsorption syndrome


    • Intrinsic factor affects how the GI system absorbs B12
    • B12 is required to produce healthy RBC's

  • Autoimmune response that destroys parietal cells

  • Megaloblastic anemia

  • Onset is gradual


  • Difficulty maintaining balance


  • Impairs cell division and advancement of a cell nucleus

Folic Acid

  • Lacks the folic acid in the blood, due to the inability of the liver to store & metabolize folates

  • Malabsorption Disorders


  • Chronically undernourished patients

  • Alcoholics
  • Patients on TPN
  • Pregnancy
    -Certain medications (methotrexate)
  • Chemotherapeutic medications
  • Onset is gradual

Medical

  • Occurs due to underlying medical condition creating acute or chronic bleeding within the body.
  • GI hemorrhage
  • heavy or prolonged menstrual bleeding

Trauma

  • Due to significant trauma (blunt or penetrating) sustained by the patient
  • Significant trauma: loss of limb, internal hemorrhage due to blunt/penetrating trauma, intercranial hemorrhage

Sickle Cell

  • Genetic disorder causing distortion of the RBC shape
  • Abnormal hemoglobin S
  • Autosomal recessive disease
  • RBCs become curved (sickle shaped), stiff, and sticky
    • Shape results in weak structure resulting in a deminished lifespan

Glucose-6-phosphate dehydrogenase (G6PD)

  • Genetic/hereditary disorder that is incapable of RBC metabolism

  • G6PD is an enzyme that helps protect RBCs from damage and premature destruction


    • Hemoglobin is directly oxygenated resulting in damage

  • Impairs compensatory increases of glucose metabolism resulting in damage

  • Triggered by stressors

Thalassemia

Genetic/inherited disorder of hemoglobin synthesis

  • Missing Alpha or Beta hemoglobin molecular chains
  • Deficient hemoglobin production
    • unable to carry sufficient oxygen
  • RBCs produced are microcytic, hypochromic, and fragile

Acquired Hemolytic

Hemolysis from factors outside the RBCs

  • Trauma to RBCs from:


    • Prosthetic heart valves
    • Severe burns
    • Hemodialysis
    • Radiation

  • Autoimmune disorders

  • Infections
  • Transfusion reactions
  • Exposure to drugs, chemicals, venom, bacteria, or trauma

Care

  • Ensure periods of rest
  • Encourage ADLs
  • Prioritizing of activities'
  • Encourage to sit while performing activities to conserve energy
  • Providing a safe environment

Management


1. Fatigue

  • Pt will verbalize the reduction of fatigue with improvement in energy and activity performance within 1 month of treatment/therapy.
  • Pt will identify 2 activities that aggravate activity intolerance within 1 month of treatment/therapy.
  • Pt will implement 2 necessary behaviors or lifestyle changes that aid in fatigue improvement within 1 month of treatment/therapy.

2. Deficient knowledge

  • Pt has ability to verbalize the disease process, procedures, treatment methods, medications and treatment plan within 1 month of treatment/therapy start.
  • Pt has understanding of dietary therapy by giving examples of foods to intake and food to avoid within the first week of treatment and therapy.
  • Pt has ability to verbalize and identify 3 causation factors affecting their diagnosis within 2 weeks of treatment/therapy.

3. Risk of Infection

  • Pt will show proper use of hand hygiene, oral care, and perineal care by performing tasks with minimal assistance by the first week of treatment and therapy.
  • Pt have decreased signs of infection within 1 month of treatment/therapy.
  • Pt has ability to verbalize understanding of the importance of avoiding eating raw fruits & veggies and uncooked meat by the first week of treatment and therapy.

Medication

  • Iron supplements (ferrous fumarate. ferrous gluconate, & ferrous sulfate)
  • Erythropoiesis (Darbepoetin alfa, epoetin alfa, methoxypolyethylene glycolepoetin beta)
  • Vitamin C

Treatments

  • Iron supplements
  • Blood transfusions
  • Medications
  • Iron-rich food increased in diet

Aplastic Tx

  • Bone marrow transplant
  • Blood Transfusions
  • Separation from or withdrawal of the causative agent

Pernicious (B12)

  • Life long Vitamin B12 IM injections
  • Dietary increase of foods with vitamin B12 content (eggs, meat, and dairy products)
  • Parenteral B12 for cases with lacking intrinsic factor and malabsorption

Iron Deficiency

  • Supplemental iron
  • Dietary increase of iron-rich foods
  • Treat underlying causes

Folic

  • Dietary increase of foods rich in folic acid (folate)
  • Oral Supplemental Folic acid (Supplements are recommended for planned or active pregnency)
  • Green leafy vegetables, fruits, cereals, and meats

Thalassemia

  • Regular blood transfusion
    • will be needed for life
    • May additionally require iron chelation therapy
  • Folic acid supplements
  • Splenectomy is possible
  • Cholecystectomy is possible
  • Stem cell transplant
  • Gene therapy
  • Bone marrow transplant

  • Genetic counseling


  • Mild cases may only require small lifestyle and dietary changes

Blood Loss

Trauma:

  • Controlling hemorrhage

  • Surgical repair of injuries



    Medical:

  • Medication management
  • Surgical repairs if necessary on effected area

Folic

CBC

  • Peripheral blood smear
    • Megaloblastic
  • MCV >100
  • Serum folate <2 ng/mL

Diagnostic Tests

  • Complete blood count (CBC): <3.6 women & <4.2 men
  • Hemoglobin: <12.0 women & <14.0 men
  • Hematocrit: <0.36 women & <0.42 men
  • Serum iron: normal women 50-150 & men 70-175
  • Serum ferritin: normal 18-160 women & 18-270 men
  • Iron-binding capacity (TIBC): normal for all genders 250-450
  • Peripheral blood smear: stained red for cell examination
  • Microscopic analysis: stool, blood & urine

Pernicious (B12)

  • Increased MCH and MCHC levels
    • Vitamin B12 levels - Low levels - <200 ng/L
    • Schilling test - less than 7% excretion
    • Peripheral Blood Smear
  • Macrocytic anemia - Oval-shaped cells
  • Hyper-segmented neutrophils
    • Serum antibodies to intrinsic factor
  • Presence of Type I and/or Type II IF antibodies
    • Indirect bilirubin - Elevated levels
    • Serum lactic dehydrogenase - Elevated levels
    • Serum folic acid - Levels within range 2.5-20 ng/mL
    • Bone marrow biopsy
  • Megaloblastic maturation of erythroid precursors
    • Evaluation of gastric secretions
  • 10% reduction
  • Reduced or absent hydrochloric acid levels in the stomach
    • Iron increased

Blood Loss

  • Serum Ferritin decreased in chronic bleeds

G6PD

  • Ensuring stress free environment
  • Genetic counseling
  • Caregiver support
  • Self esteem facilitation
  • Removal & avoidance of further triggers (Triggers include- some foods such as fava beans, tonic water, and blueberries, certain infections, and medicine such as antibiotics and aspirin

G6PD

Treatment depends upon the patient's overall presentation

  • Supportive care
  • Avoiding aggravating factors (food, medications, enviroment)
  • Treatment of associated infections
  • Blood transfusions may be required

Sickle Cell

  • Hemoglobin electrophoresis: HbA1 96.5% to 98.5% HbA2 1.5% to 3.5% Hb F 0% to 1%
    • Presence of abnormal hemoglobin S (HgbSS)
  • High-performance liquid chromatography (HPLC)
  • Deoxyribonucleic acid (DNA) testing
  • Dithionile

Aplastic

  • Differential for WBC:
  • Bone marrow test
  • Reticulocyte count: shows if bone marrow is making RBCs at correct rate
  • Coagulation test
  • Hemoglobin electrophoresis: HbA1 96.5% to 98.5% HbA2 1.5% to 3.5% Hb F 0% to 1%
  • Flourescence-activated cell sorter
  • Transaminase
  • Bilirubin normal adults 0.3 to 1.0
  • Lactic dehydrogenase
  • Blood Urea Nitrogen (BUN)
  • Creatinine
  • Hepatitis testing
  • Serum Ferritin decreased

G6PD

  • Bilirubin level: normal adults 0.3 to 1.0
  • LDH test: adults <40
  • Reticulocyte count: absolute reticulocyte count = % reticulocytes x erythrocyte count
  • Methemoglobin reduction test: normal 0.4% to 1.5%, >40% is critical value
  • Hemoglobin - urine

Sickle Cell

  • Hydroxyurea (Hydrea)
  • Analgesics
  • Prophylactic antibiotics (to prevent infections)
  • Oxygen
  • Folic Acid
    • Avoid Iron, can result in toxicity
  • Stem cell transplant

Thalassemia

  • Luspatercept (Reblozyl)
  • Iron chelation therapy
    • deferasirox
  • Folic acid

Blood Loss

  • Tranexamic acid (TXA)
  • IV iron supplementation

Aplastic

  • Immunosuppression therapy
  • Antibiotics for any underlying infection causation
  • Androgens controversial tx
  • Sargramostim
  • Antithymocyte globulin
  • Corticosteroids
  • Cyclosporine

Pernicious (B12)

  • Vitamin B12- cyanocobalamin


  • Multivitamin


  • Antidysrhythmic


  • Cardiotonic


  • Diuretics


  • Vasodilators


  • Possible antibiotics

  • Antianemic agents

G6PD

  • Folic acid
  • Pseudoephedrine (Sudafed)
  • Guaifensin
  • Acetaminophen (Tylenol)
  • Ibuprofen (Advil)

Sickle Cell

  • Primary supportive therapy
  • Hydroxyurea
  • Genetic counseling

Sickle Cell Crisis:

  • Precipitating factors treatment
  • Rest
  • Oxygen therapy
  • Vigorous hydration
  • Narcotic analgesia

Acute Chest Syndrome:

  • Careful hydration
  • Hemodynamic monitoring
  • Oxygen therapy
  • Folic acid supplements
  • Transfusion

Iron Deficiency

  • Nutritional management
  • Medication management
  • Energy managment
  • Manage activity intolerance
  • Exercise promotion
  • Exercise therapy
  • Ambulation
  • Vital signs monitoring
  • Infection prevention (hand hygiene, vaccines, etc.)
  • Promoting comfort
  • Promoting good oral care/hygiene

Folic

  • If pregnant supportive care for both mom and baby
  • Nutritional management
  • Nutritional counseling & monitoring
  • Promoting good oral care/hygiene

Pernicious (B12)

  • Nutritional management
  • Nutritional therapy
  • Nutritional counseling & monitoring
  • Fluid/electrolyte management
  • Medication management
  • Coping stratigies for dealing with lifelong illness
  • Education on disease and treatments
  • Promoting good oral care/hygiene

Thalassemia

  • Genetic counseling
  • Caregiver support
  • Self-esteem facilitation
  • Reproductive grief counseling
  • Family, nurse, and social support
  • Prenatal screening

Sickle Cell

  • Warm/hot compress at site
  • Nutritional monitoring
  • Nutritional therapy
  • Self-responsibility facilitation
  • Counseling
  • Caregiver support
  • Encourage adequate fluid intake
  • Implement plain reducing therapies (massage, mediation, aroma therapy, etc.)

Aplastic

  • Environment management
  • Infection control
  • Infection prevention
  • Nutritional management
  • Surveillance
  • Medication management
  • Isolation precaution implementation

Medical Conditions

Diagnostics

Medications

Treatments

Management

Assessment Findings

  • Tachycardia
  • Pallor
  • Hypoxia (insufficient oxygen to tissues)
  • Dyspnea upon exertion
  • Fatigue
  • Fainting
  • Insomnia
  • Dizziness
  • Difficulty concentrating
  • Brittle nails
  • Muscle pain
  • Stool color changes

Care

Aplastic

  • Pancytopenia
    • Anemia
    • Leukopenia
    • Thrombocytopenia
  • Pallor
  • H/A
  • Palpitations
  • SOB
  • Fatigue
  • Tachycardia
  • Heart failure

  • Coagulation problems


    • bleeding gums
    • excessive bruising
    • difficulty clotting

  • Increased chace of infection


    • Fever
    • Sore throat

Folic

  • Progressive weakness
  • Fatigue
  • Pallor
  • Short of Breath
  • Palpitations
  • Glossitis
  • Cheilosis
  • Diarrhea
  • Enlarged nucleated RBCs
    -No neurological symptoms

Iron Deficiency

  • Leg cramps
  • Fatigue
  • Exercise intolerance
  • Craving of ice, clay, dirt, and cold foods
  • Epistasis
  • Glossitis
  • Intolerance to cold
  • Tachycardia
  • Spoon-shaped nails
  • Difficulty concentrating
  • Irritability
  • Hidden blood in stool
  • Children may experience
    • Cognitive impairment
    • Developmental delays

Pernicious (B12)

  • Pallor
  • Fatigue
  • Glossitis
  • Cheilosis
  • Tingling extremities
  • Enlarged spleen/liver
  • Tachycardia
  • Dyspnea with activity
  • Dyspepsia
  • Diarrhea
  • Depression
  • Psychosis
  • Large misshapen RBCs with thin membranes
  • Neurological Signs:
    • Paresthesia
    • Clumsiness
    • Weakness
    • Memory issues

Sickle Cell

  • Sudden pain in the abdomen, soft tissues, bones, and joints
  • Splenomegaly
  • Swelling of hands and feet
  • Elevated heart rate
  • Bilirubin
  • Jaundice or pale skin
  • Malaise
  • Fatigue
  • Pallor
  • Irritability
  • Dyspnea
  • Vascular occlusion (ischemia)
  • Possible vision issues
  • Leg ulcers

Blood loss

Medical:

  • Increased pain
  • Hypotension
  • Contusions/hematomas

Trauma:

  • Visible hemorrhage
  • Hypovolemic shock and circulatory failure if bleeding continues

G6PD

  • Exposure to stressor


    • Infection
    • Drugs
    • NSAIDs
    • Sulfonamides
    • Vitamin K derivatives
    • Hemolytic crisis

  • Hemoglobinuria

  • Elevated reticulocyte count

Thalassemia

  • Patients with Mediterranean, African, and South Asian descent
  • Enlarged spleen
  • Bone marrow hyperplasia
  • Bronze skin coloring
  • jaundice
  • Fatigue
  • Ulcers on extremities
  • Deformed facial and skeletal bones
    • Chipmunk face
  • Cardiac arrhythmias
  • abdominal pain (gallstones)
  • Liver failure and cirrhosis
  • delayed growth rates
  • Hemochromatosis
    • Diabetes Mellitus, hypothyroidism and hypoparathyroidism, chronic arthropathies, early-onset Parkinson's disease

Assessment

The Specific Medical condition and their pathophysiology associated with Cellular Regulation

The diagnostic test associated with each specific condition

The Medications used to treat each specific condition

The treatments methods utilized for each specific condition

The assessment findings specific to each specific condition

How the nurse manages each specific condition

The care required for each specific condition

Aquired Hemolytic

  • Enlarged spleen
  • Bilirubin
  • Jaundice
  • Bone marrow hyperplasia
  • Pathologic fractures

Aquired Hemolytic

  • Serum Ferritin increased
  • Blood & specialized tests (Coombs test)
  • Lactate dehydrogenase (LDH)
  • Aspartate aminotransferase (AST)
  • Hemoglobinemia
  • Hemoglobinuria
  • Bone marrow

Aquired Hemolytic

  • Prednisone
  • Cyclosporine
  • Rituximab
  • Mycophenolate mofetil

Aquired Hemolytic

  • Blood transfusions
  • Stem cell transplants
  • Bone marrow transplants
  • Plasmapheresis
  • Splenectomy

Iron Deficiency

  • Oral iron supplements
    • IV in certain cases
  • Ferrous sulfate

Blood Loss

  • Surgical repair (medical or trauma)
  • Blood transfusion
    • pRBCs
  • Fluid replacement

Folic

  • Folic acid supplements
  • Antianemic agents
  • If pregnant, hydroxyurea

Thalassemia

  • CBC
    • Hemoglobin low
    • MCV low
  • Hemoglobin electrophoresis: larger percentages of HbF and HbA2, absent or very low HbA, mild elevation of HbA2, and mild decrease of HbA
  • Mentzer Index <13
    • Basophilic stippling: applied stain
  • Genetic testing
  • Iron > 150 women & >175 men
  • Serum Ferritin increased
    • Microcytic: <6
    • Hypochromic
    • Target cells
    • Reticulocyte count: Increased

Iron Deficiency

  • Peripheral blood smear
    • Hypochromic hemoglobin <30
    • Microcytic hemoglobin <6
  • Serum iron: decreased
  • Serum Ferritin: decreased
  • Stool guaiac test
    • Positive for occult blood
  • Mentzer index >13
  • Iron staining on bone marrow aspirate smears
    • Grade <2

Outcome
The patient will demonstrate improvement in fatigue and quality of life by noting a 20+ point increase in their Functional Assessment of Chronic Illness Therapy – Fatigue (FACT-An) score within seven days of treatment.
Actions

  1. Patients level of fatigue and quality of life will be evaluated utilizing the FACT-An scoring method


    • Patient will identify periods of fatigue throughout their day.
    • Assess the patient's physical limitations and requirements for assistive devices

  2. The Patient will be provided supervised physical activity sessions such as walking around the hospital floor


    • Patient will be encouraged to established escalating exercise goals upon each session such as increasing distance or time

  3. The Patient will be educated on proper sleep hygiene methods such as establishing a routine before bedtime, going to sleep at the same time, and turning off all electronics.


    • Pt will be assisted in establishing activity priority to coincide with periods of energy.
    • Patient improvement will be evaluated with follow up FACT-An score

Outcome
The patient will demonstrate improved knowledge and management of their condition through teach-back, and nutritional choices by end of shift.
Actions

  1. Assess the patient's learning style and barriers
    • Assess the patient's cognitive ability
    • Assess the patient's willingness to learn
  2. Educate the patient on the importance of proper nutrition
    • Educate the patient's family/caregiver on the importance of iron-rich foods (meats, leafy green vegetables, beans)
    • Educate the patient of the importance of Vitamin C
    • Educate patient on taking their iron supplement before food and not with antacids or milk
  3. Client will demonstrate selection of proper nutritional food choices
    • Patient and family will verbalize understanding of the disease and disease process

Outcome
The patient will display improvement in Iron and B-12 levels through lab testing within one week.
Actions

  1. Assess pt B12, RBC, Hbg, and HCT lab values
    • Assess the patient's oxygen saturation and respiratory effort
    • Assess medical history for celiac disease, IBS, or gastric surgery
  2. Educate the patient on the necessity of B12 injections
    • Educate the patient on why oral B12 is ineffective due to loss of intrinsic factor
    • Educated patient and family concerning pernicious anemia with emphasis on it not being iron deficiency
  3. Lab testing will be completed to evaluate current levels
    • Evaluate the patient's ability to self-inject B12