Cystic Fibrosis by https://padlet.com/cvysoky/gjs5mc7i52cu Made with swagger en-us 2020-04-07 21:03:11 UTC 2025-12-01 21:48:00 UTC hello@padlet.com https://padlet.net/icons/png/1f9d8-2640.png DISEASE PROCESS cvysoky https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497637154
  • Mutation in  a specific gene
  • Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene
    • CFTR controls the flow of water and electrolytes in and out of body cells.
  • Alterations affect the exocrine glands that produce mucus, tears, sweat, saliva, and digestive juices.
    • Secretions become thickened and sticky
    • Clogging tubes, ducts, and passageways 

    • ]]>     2020-04-07 21:07:45 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497637154     Signs/ Symptoms (Respiratory): https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497637613 Early: 
    • Wheezing/Rhonchi
    • Dry, non productive cough
    Increased Involvement:
    • Dyspnea
    • Paroxysmal Cough
    • Obstructive Emphysema
    • Atelectasis on CXR
    Advanced Involvement:
    • Cyanosis
    • Barrel Chest
    • Clubbing
    • Bronchitis/ Bronchopneumonia


    ]]>     2020-04-07 21:08:09 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497637613     Diagnostic Assessments evacosta https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497638756 Newborn Screening
    • Blood test checks for high levels of immunoreactive trypsinogen (IRT). 
    Sweat Test
    • measures increased amount of chloride in sweat
    Genetic or Carrier Test
     
    ]]>     2020-04-07 21:09:13 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497638756     Signs/ Symptoms (GI): https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497642294 Large steatorrhea
    • Voracious Appetite (Early)
    • Loss of Appetite (Late)
    • Weight loss
    • Delayed growth
    • Distended Abd
    • Thin extremities
    • Fat soluble vitamin deficiency
    • Anemia

    ]]>     2020-04-07 21:12:29 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497642294     Signs/Symptoms (Skin): https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497646049 Salty sweat, tears, saliva]]> 2020-04-07 21:15:41 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497646049 Signs/ Symptoms (Endocrine and Repro): https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497646991
  • Vicious cervical mucous
  • decreased/absent sperm
  • Males - infertility
  • Females - may have difficulty conceiving
    • ]]>     2020-04-07 21:16:32 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497646991     Labs: https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497648213 Blood Specimen: 
    • Nutritional Panel 
    Sputum Culture:
    • Detect Infection
    ]]>     2020-04-07 21:17:42 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497648213     Nursing Interventions https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497649949
  • Assess Respiratory Status
  • Obtain Vitals
  • Obtain IV Access
  • Obtain Sputum Culture
  • Provide Support to child/family
    • ]]>     2020-04-07 21:19:22 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497649949     Pulmonary Management https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497652026 1. Airway Clearance Test (ACT) 
    • 2x/Day
    • Avoid immediately before/after meals
    • Several types:
    • Chest Physiotherapy (CPT)
    • Positive Expiratory Therapy
    • Active-Cycle of Breathing Techniques
    • Autogenic Drainage
    • High Frequency Chest Compression
    2. Aerosol Therapy
    • Brochodilator
    • Before ACT
    3. IV/aerosolized Abx
    4. Encourage physical aerobic exercise
    5. O2 PRN


    ]]>     2020-04-07 21:21:26 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497652026     GI Management https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497659842
  • Well balance protein/caloric diet
  • 3 meals/day + snacks
  • Fluid Intake
  • Administer pancreatic enzyme 30 min prior to eating
  • Supplemental Vitamins
  • Laxatives/Stool Softeners
  • Histamine-receptor antagonist
  • Motility meds for GERD
  • Formula supplements in addition to breast feeding
  • Consult Dietitian
    • ]]>     2020-04-07 21:29:16 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497659842     Endocrine Management https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497665598
  • Monitor BG
  • Administer Insulin
  • Oral Glycemic Meds not effective with cystic fibrosis related diabetes

    • ]]>     2020-04-07 21:35:28 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497665598     Lungs cvysoky https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497693367
  • Mucus in the lungs is still able to trap bacteria, but the mucus becomes trapped in the lungs causing infection

    • ]]>     2020-04-07 22:05:18 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497693367     Pancreas cvysoky https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497693731
  • Thickened mucus blocks ducts between the pancreas and intestines
  • Digestive enzymes cannot reach small intestines
    • Problems breaking down food and absorbing nutrients
  • Insulin secretion may also be affected 
    • Cystic fibrosis related diabetes (CFRD)

    • ]]>     2020-04-07 22:05:43 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497693731     Liver cvysoky https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497694124 Bile ducts become blocked and inflamed 
    • cirrhosis and portal hypertension
    ]]>     2020-04-07 22:06:13 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497694124     Sweat glands cvysoky https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497694482
  • increased salt in sweat on skin

    • ]]>     2020-04-07 22:06:39 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497694482     Reproductive organs cvysoky https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497694668
  • Tube connecting testes and prostate gland can be blocked
    • Infertility
  • Fallopian tubes blocked
    • Infertility 

    • ]]>     2020-04-07 22:06:54 UTC https://padlet.com/cvysoky/gjs5mc7i52cu/wish/497694668