Case 1: Cystic Fibrosis Patient: Angel Age: 19 Gender: Male Diagnosis: Cystic Fibrosis Med Hx 37-yr G2 P2 AB0 old

ld mother, normal full-term pregnancy and delivery Born with meconium ileus Thickened intestinal mucous secretions Diagnosed CF Corrected via surgery Pancreatic insufficiency Given supplemental pancreative enzymes Chronic sinusitis Chronic ear infections Surgical corrections No lung deficiencies until age 16-17 Cough, shortness of breath, decrease activity tolerance, no wheezing Diagnosed as ABPA (Allergic bronchopulmonary aspergillosis) Given systemic corticosteroids Pseudomonas aeruginosa infection Already on inhaled Tobramycin Later given oral Ciprofloxacin Acute viral respiratory infections Exacerbated lung function Counter with higher dosage bronchodilator and chest compression vest Age 16: Guillain-Barre syndrome (bodies immune system attacks your nerves, starts with tingling in extremities, most people recover, but it can be deadly) Indep. From CF Treated with corticosteroids and IVIG (intravenous immunoglobulin) Resolved 2-3 months afterward, no recurrence Age 17: Crohns disease (inflammatory bowel disease= abdominal pain, severe diarrhea and malnutrition. ) Indep. From CF Treated with corticosteroids and Infliximab (immunosuppressant) Had Pyoderma gangrenosum complication (ulcer) Went away but recurred New course of Infliximab started Age 17: CFRD (cystic fibrosis-related diabetes mellitus= combination of Type 1 and Type 2 Diabetes) Due to corticosteroid use= mimic the action of cortisol and makes insuline resistant Insulin for CFRD Currently Seen every 3 months at CF Clinic

Denies cough, wheeze, phlegm, or congestion Currently active, lost weight, good appetite No abnormal stool patterns

Medications 1. Osmotic aids a. Hypertonic saline (inhaled)- rehydrate mucous b. Pulmozyme- thins mucous c. Xopenex- inhaled bronchodilator d. Saline nasal spray e. Rhinocort- nasal spray to reduce inflammation 2. Digestion enhancers a. Creon 20- supplement pancreatic enzymes 3xmeals, 2xsnacks b. Prevacid-antacid c. Asacol- oral, reduce intestinal inflammation d. Miralax- oral, for constipation e. Ursodiol- oral, promotes bile flow in liver for CF-related liver disease 3. Insulin-CF related diabetes a. Lantus- subcutaneous injection at bedtime b. Novalog- subcutaneous injection per 15gm CHO at each meal 4. Antibotics a. TOBI (300 mg/ml) -inhaled antibiotic 5. Pulmicort- inhaled corticosteroid to decrease lung inflammation 6. Anti-inflammatory a. Azithromycin- oral antibiotic for anti-inflammation in CF pts b. Remicade- immunomodulator for Crohns disease 7. Vitamins a. AquADEK vitamins- vitamins enriched with fat soluble vitamins b. Vitamin E c. Folic Acid d. Ferrous sulfate- Iron supplement Allergies: Voriconazole (medication) Patient Information Exercises regularly Medications and chest physiotherapy apx 2 hours per day Current college student at UCSB, full-time student Has roommates Home in the summer: Los Angeles Family Hx Father 64-yr-old no hx of asthma, respiratory allergies, or lung diseases works at LAX airport Mother 60-yr-old

 no hx of asthma, respiratory allergies, or lung diseases Older sister 31-yr-old, healthy No family hx of CF No smokers One dog, Angel shows no symptoms No hobbies that generate dust or fumes PE well-developed, reasonably well nourished no acute distress no cyanosis, mild digital clubbing psychological manner is awake, alert, interactive, pleasant no assistance for breathing regular respiratory rhythm thoracic configuration has slightly increase anterior-posterior diameter, otherwise normal Weight/Height/BMI all within statistically normal range Temp 36.4 Celsius Heart rate 71 Respirations: 24 BP: 118/63

Microbiology Culture (+) for Pseudomonas aeruginosa sensitive to all antibiotics except Amikacin no methicillin-resistant Staphylococcus aureus Assessment 1. CF 2. Chronic CF lung disease 3. Pseudomonas aeruginosa lung infection 1. Reactive airways disease

SPP Case #2: Diabetes

Pathophysiology Type 1 - Due to partial or complete loss of insulin production by pancreatic beta cells - Islet cell antibodies involved in etiology: patients will present with antibodies to islet cell proteins, meaning autoimmune process has begun Type 2 - Due to gradual resistance to insulin - Asymptomatic at first - Slowly develop insulin resistance, leading to obesity and other metabolic changes - Diagnosed based on finding elevated levels of glucose in blood, or glucose in urine - Abnormal levels: >125 mg/dL fasting or >200 mg/dL any time - Can be treated in earlier stages with oral medication and lifestyle management (diet, exercise) - Need to cut back on carbs more than any other nutrient 90-95% Usually over 40 years old 80% obese Sedentary Increased Occasionally asymptomatic Occasionally Rarely Rarely Rarely Occasionally Never

Treatment

Insulin supplements

Prevalence of all cases Age Weight Lifestyle Infection risk Symptomatic Weakness or fatigue Polydypsia(Inc thirst)/ polyuria(Inc urination) Polyphagia( Inc Hunger) with weight loss Blurry vision Peripheral neuropathy Ketonemia( excessive ketone bodies in blood) or ketonuria (ketone bodies in the urine)

5-10% Any age, but usually young Thin to average No associations found Increased Mostly symptomatic Often Often (3Ps) Often Often (increased ECF glucose causes swelling) Rarely Often

Long-term complications Result from hyperglycemia and metabolic changes Leading cause of polyneuropathy, end-stage renal disease, and blindness Eyes

o Damage of arterioles or retina microaneurysms, hemorrhages o Increased formation of cataracts o Gradual vision loss can lead to blindness Kidneys o Glomerular renal damage diagnosed by elevated protein and glucose in urine o Decreased renal function results in renal failure Vascular and Nervous systems o Increased atherosclerosis o Polyneuropathy (damaged nerves, especially in the periphery), angiopathy (damaged vessels, especially coronary arteries and feet) Combined with an infection, may need to amputate feet or leg GI tract can also develop autonomic dysfunction o Smoking greatly increases risk of atherosclerotic (cardiovascular) disease in diabetics 95% of all amputees are smokers because of decreased oxygen to tissues Increases blood pressure, lipids, insulin resistance