DIC
SCA
SCA Acute Symptoms
Nursing Assessments for Cystic Fibrosis
100

Pulmonary Hemorrhage

  • Hemoptysis
    • coughing up blood
  • Tachypnea & dyspnea
    • dt/t impaired oxygenation from blood pooling in the lungs
  • Orthopnea
    • worsening breathing difficulty when lying flat
100

First Symptom of SCA?

 

  • Dactylitis, severe anemia and leukocytosis may appear in the first year of life
    • Dactylitis: bone infarctions in the hands and feet, causing pain and swelling
100

What is Vaso-occlusive crisis? And what are its manifestations? 

  • Most common form and is an acute painful episode
  • Localized extremity pain/all over the body
  • Fever 
  • Swelling/Dactylitis 
  • Tenderness 
  • Tachypnea
  • Low O2 sat. 
  • HTN
  • n/v
  • Priapism
100

What are three airway clearance therapies? 

1. Continuous postural therapy 

2. High frequency chest compressions 

3.Huffing 

4. Aerobic exercises

200

Hypovolemic Shock

  • d/t significant blood loss, leading to shock
    • Hypotension
    • Tachycardia 
    • Signs of poor perfusion
      • Cool clammy skin
      • Weak pulses
    • Potential progression to multi-organ failure
  • Massive hemorrhage 
    • Hematemesis (frank red blood) or coffee ground emesis (partially digested blood)
    • Upper (black/tarry) and lower (bright red) rectal bleeding
    • Coughing up blood
    • HA and vision changes
      • Suggests intracranial hemorrhage 
  • Oozing from IV sites
200

What can cause a sickle cell crisis?

SICKLE

S: significant blood loss

I: illness 

C: climbing high altitudes 

K: keeping continued stress

L: Low fluid intake 

E: Elevated Temperatures

200

What are the manifestations for acute chest syndrome?

  • Manifestations 
    • Chest pain
    • Fever 
    • Cough 
    • Tachypnea
    • Low O2 sat. 
    • Wheezing
    • Hypoxia 
    • Death 
  • Pulmonary infarct manifestations 
    • Pulmonary HTN
    • MI
    • HF
    • Cor pulmonale
      • Enlarged right ventricle → RHF
200

What are the considerations for the transition to adulthood?

  • Delayed puberty is common in both genders, but women can have viable pregnancies
    • Most men are infertile due to absence of the vas deferens, though they produce sperm and can father children with reproductive technology
300

Respiratory System (Thrombosis Sx)

  • Tachypnea, dyspnea and chest pain
    • sx of pulmonary embolism and respiratory distress
  • Risk of ARDS
    • d/t widespread lung tissue damage, leading to hypoxia and respiratory failure
300

How does sickling occur?

  • HgbS reacts to deoxygenation and dehydration by solidifying and stretching the RBC into a sickle shape 
  • Degree of deoxygenation that is need to produce sickling varies with the % of HgbS present


300

What does an Aplastic crisis reduce?

temporary decrease in production of RBCs and their short lives (3-6 point drop in Hgb = REALLY FAST)  → decrease in reticulocyte count (immature RBCs)

300

How do you administer Pancrelipase (Creon)?

  • Taken within 30 minutes of eating snacks and meals
  • Amount of enzyme is adjusted to promote normal growth and reduce stools to 1-2 per day
  • Avoid with dairy/alkaline substances
400

Intergumentary System (Thrombosis Sx)

  • Cyanotic extremities
    • d/t microvascular clotting.
  • Hemorrhagic necrosis
    • where dead tissue appears to be bleeding
  • Risk of limb loss if severe ischemia is not reversed
  • Ischemia
  • Gangrene 


400

What are the Stressors for sickling?

  • Infection - most common
  • Decrease PO2 in the blood 
    • Decreased pH
  • Increased plasma osmolality – dehydration
  • Decreased blood volume 
  • Low temperatures – vasoconstriction 
  • Blood loss
  • Emotional stress
400

By what age does sequestration happen? And what are the steps?

By 4years old (auto infraction of the spleen)

  • Life threatening pooling of blood in spleen and infrequently liver → blood in spleen is no longer circulating b/c of blocks → splenomegaly → severe anemia → decreased blood volume because of pooling → shock → infarction → organ failure → death
400

What can be a side effect of using a Hypertonic saline (6-7%) solution?

It may cause a bronchospasm treat IMMEDIATELY

500

Renal System (AKI) & Failure 

  • Oliguria: decreased urine output (<30 mL/hr) 
    • d/t poor kidney perfusion.
  • Elevated BUN & creatinine
    • indicating kidney damage.
  • Progression to renal failure
    • requiring dialysis if not managed promptly

500

How does a sickle cell regain its normal shape?

  • Sickle cells regain normal shape initially after homeostasis is achieved 
    • Reoxygenation and rehydration is achieved
    • BUT frequent sickling damages the cell membrane, making some cells permanently sickled
500

What is the treatment for a Hypermolytic crisis?

  • Supportive care
  • Blood transfusions if necessary
  • Treating the underlying cause
    • Infection (viral, bacterial)
    • Drug-induced hemolysis
    • Autoimmune reaction
    • Delayed hemolytic transfusion reaction
500

What are the interventions for Malabsorption issues?

  • Need for a high calorie diet
  • Delayed growth
  • Fat soluble vitamins
    • Steatorrhea
      • bulky, frothy stools 
    • Azotorrhea
      • foul-smelling stools
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