Question

WM is a 22-year-old female with cystic fibrosis (CF) diagnosed at 8 months of age who presents with a 3 day history of increasing cough, purulent sputum production and shortness of breath. Additionally, she has been having trouble with hyperglycemia, with glucose levels ranging from 250-400 mg/dl. She was evaluated and diagnosed with a CF exacerbation and admitted. During her 5-day hospital admission, she has received intravenous antibiotics, respiratory treatments and aggressive chest percussion to improve her airway clearance. Her appetite has improved, but her weight remains stable since admission at 104 pounds.

Past Medical History: WM was diagnosed with CF by a positive sweat chloride test at 8 months of age. She has had numerous hospitalizations for CF exacerbations, recently averaging 2 to 3 times per year. She was diagnosed with CF-related diabetes at age 14 and has required insulin therapy intermittently for management of hyperglycemia. Additionally, she has a history of gastroesophageal reflux and peptic ulcer disease.

Medications: Current medications include Pancrealipase (Creon 20, 6 caps per meal, 3 caps per snack), 15 units of NPH insulin before breakfast, 3 to 5 units of Lispro insulin with each meal depending on her carbohydrate intake; Prevacid, Itraconazole, Flovent, and oral antibiotics.

Physical Examination

Vital Signs

Temperature: 101.4 (F)

Heart rate: 110 BPM (tachycardia)

Respiration: 24 (tachypnea)

Blood pressure: 136/77 mm Hg

Height: 5'3" (160 cm)

Current weight: 104 lb (47 kg)

Usual weight (UBW): 112 lb (51 kg)

BMI: 18 kg/m2

The patient's physical examination is normal except for the following observations:

General: thin, ill-appearing female

Skin: warm to the touch

HEENT: right nasal polyp

Chest: rhonchi in right upper lung zone with scant expiratory wheezing. There was no dullness or egophony present.

Cardiac: tachycardic, normal rhythm, no murmurs

Laboratory Data Patient's Values Albumin: 2.8 g/L Prealbumin: 11.5 mg/dl Hemoglobin: 12.9 mg/dL Prothrombin time: 13 seconds FEV: 50%

Normal Values 3.5-5.8 g/L 16.6 - 43.1 mg/dL 12-14 mg/dL 10-12 seconds

What's wrong with the patient?

Answer 1

People with cystic fibrosis heve thick mucous that cause scaring pancreas it prevent pancreas from producing normal insulin amount. It caue insulin deficnet and it need insulin therapy to manage hyperglycemia effect. this disease cause chronic infection and affect the organ like liver and sweat glands. due to respiratory insufficienty heart rate and respiratory rate will be high and it block the normal absorption for the key nutrients and cause poor digestion and trouble weight gain. due to obstruction in the lung and airway and high secreation in the airway there is rhochi in the right upper lung zone and low pitched wheezing . there is hypoalbuminemia due to liver dusfunction, poor absorption of nutrients and cause malnutriion.