Gas exchange

Sickle cell anemia: Inherited disorder that causes abnormal hemo
RF: Genetic, African american, northern mediterranean, hispanic, middle eastern

s/s: pain, fatigue, SOB, pallor, jaundice

Tx: Opiod analgesics on a schedule, antibiotics for infection

NC Administer IV fluids, blood products, o2 as ordered.

PT: Adequate fluid intake and rest. Prevent infection (hand hygiene, avoid crowds)

Diagnosits: RBC smear - look at the RBcs to see the shape. Gentic screening, newborn screening, gene mapping, hemoglobin electrophoreis test: Blood test that is a diagnostic for the presence of sickle cell trait or disease. CBC- H&H, RBC are decreased. Sixkle Dex Test- chemical solution add to pt blood sample, it changes the solution to cloudy if they have sickle cells

Vaso occlusive criss: RBC stick together in vessels and cause hypoxia - causing acute sever pain. Risks: Dehydration, infection, temperature changes, high altitude, smoking (vasoconstriction), stress, sugery, over physical exertion, pregancy.

Acute Chest syndrome: Red sickle cells block blood vessels in teh lungs, lung injury, tachypnea, SOB, lower 02 level, cough, chest pain

Medication-induced anemia

Cystic fibrosis: Mutated inherited CFTR gene which control movement of chloride and sodium in and out of the body cells. Too much salt not enough water in the cells.

S/S: Wheezing, coughing, dyspnea, msucus plugs, cyanosis, barrel-shaped chest, clubbing, chronic respiratory infections. Steatorrhea (fatty, malodorous stools), delayed growth, fat-soluble vitamin deficiency (A, D, E, K). High NaCl content in sweat, saliva, and tears

Labs: Sweat chloride test, DNA testing, PFTs, stool analysis

Tx: Medications: Bronchodilators, anticholinergis, dornase alfa antibiotics (pulomnary infections), pancreatic enzymes (take with meals and snacks). Procedures: chest physiotherapy uses percussion, virbration, postural drainge, and breathing exercises to loosen respiratory secretions. Schedule treatments before meals or 1-2 hrs after meals to avoid vomiting. Use bronchodilator 30-60 minutes before treatment.

NC: Administer oxygen. Encourage increase fluids, increase protein/calorie diet. Provide supplements (fat-soluble vitamins A, D, E, K)

RF: Genetically inherited - if both parents carry the gene 25% chance with every pregnancy. Genetic testing via saliva. Testing: Sweat testing - measures the amount of chloride in the sweat. Higher in CF clients due to salt wasting. Pilocarpine applied to the forearm or leg to induce wearing and chloride levels are measured externally.

Virtual support groups

Diet: high protein moderate carbs, fats, and ensure adequate salt

ph Low (7.35-7.45)-

pCO2 High(35-45)-

HCO3 High (22-26)-

PaO2 (80-100) cellular oxygen

Chest phsiotherapy

Postural drainge

Blood transfusions

STEM cell transplant

Medications

Pancreatic anzymes medicatitriple combination therapy of CFTRon is given b/c the mucus blocks bile ducts cauinsg pancreatitis and ultimately diabetes. Give WITH meals

Fat soluble vitamins: A,D,E,K

Bronchodilators mucolytics

antibiotic

CFTR modulators

Hydroxyuera: Do not give to pregnat people, can pass through breast milk. N/V. lowers the WBC so if they have an infectiona dn

Crizanlizumab: Decreases the chance of a sickle cell crisis. Produces an antibody to preocue smoother blood flow. Pt have to be 16 and years and older. This an infusion. Joint pain, STOP if they have anaphalyxisis SOB, chest pain, sweating, chills, rash up to 24 hrs after getting the infustion. Do NOT give the pregant ppl.