Cystic Fibrosis  ( Cardiovascular / Pulmonary)
Description:  A 10 years of age carries a diagnosis of cystic fibrosis

INTERVAL HISTORY:,  ABC who is 10 years of age and carries a diagnosis of cystic fibrosis, seen in the clinic today for routine follow-up visit.  He was accompanied by his adopted mother.  He is attending the fourth grade and has not missed significant days for illness.  He has a chronic cough that has been slightly increased for the prior 4 days.  Sputum is not produced.  Sinuses are described as clear.  He is an eager eater, eating a regular diet of increased calorie and protein.  He also was taking Resource just for kid juice boxes as well as chocolate milkmaid with half-and-half.  Belly complaints are denied.  He has 2 to 3 bowel movements per day.  He does need a flu vaccine.,MEDICATIONS: , Albuterol premix via nebulizer as needed, albuterol MDI 2 puffs b.i.d., therapy Vest daily, Creon 20 two with meals and snacks,  A-dec 2 tablets daily, Prevacid 15 mg daily, Advair 100/50 one inhalation twice daily, and MiraLax p.r.n.,PHYSICAL EXAMINATION:,VITAL SIGNS:  Respiratory rate 20 and pulse 91.  Temperature is 100.0 per tympanic membrane.  Oximetry is 98% on room air.  Height is 128 cm, which is an increase of 1.0 cm from prior visit.  Weight is 24.5 kg, which is an increase of 500 grams from prior visit.,GENERAL:  He is a cooperative school-aged boy in no apparent distress.,HEENT:  Tympanic membranes clear, throat with minimal postnasal drip.,CHEST:  Significant for 1+ hyperinflation.  Lungs are auscultated with good air entry and clear breath sounds.,CARDIAC:  Regular sinus rhythm without murmur.,ABDOMEN:  Palpated as soft, without hepatosplenomegaly.,EXTREMITIES:  Not clubbed.,CHART REVIEW: , This chart was thoroughly reviewed prior to this conference by X, RN, BSN.  Review of chart indicates that mother has good adherence to treatment plan indicated by medications being refilled in a timely fashion as well as clinic contact documented with appropriate concerns.,DISCUSSION:  PHYSICIAN: , X did note that mother reported that the patient had discontinued the Pulmozyme due to CCS reasons.  He is not sure what this would be since CCS Pulmozyme is a covered benefit on CCS for children with cystic fibrosis.  This situation will be looked into with the hope of restarting soon.  Other than that the patient seems to be doing well.  A flu shot was given.,NURSE: , X, RN, BSN, did note that the patient was doing quite well.  Reinforcement of current medication regime was supplied.  No other needs identified at this time.,RESPIRATORY CARE: , X, RCP, did review appropriate sequencing of medications with the patient and family.  Once again, she was concerned the lack of Pulmozyme due to mom stating CCS issues.  At this time, they have increased the Vest use to twice daily and are doing 30-minute treatments.,DIETICIAN: , X, RD, CDE, notes that the patient is 89% of his ideal body weight, which is a nutritional failure per cystic fibrosis guidelines.  This is despite the fact that he has an excellent appetite.  Mom reports he is taking his enzymes consistently as well as vitamins.  He does have problems meeting his goal for resource drinks per day.  Since the patient has been struggling to gain weight this past year, we will need to monitor his nutritional status and weight trend very closely.  A variety of additional high calorie items were discussed with mom.,SOCIAL WORK: , X, LCSW, notes that mom has recently gradually from respiratory therapy school and has accepted a position here at Children's Hospital.  The patient is doing well in school.  With the exception of issues with CCS authorization, there appears to be no pressing social needs at this time.,IMPRESSION: , ,1.  Cystic fibrosis.,2.  Poor nutritional status.,PLAN:  ,1.  Give flu vaccine 0.5 mg IM now, this was done.,2.  Continue all other medications and treatment.,3.  Evaluate/investigate rationale for no authorization of Pulmozyme with CCS.,4.  Needs to augment current high-calorie diet to give more nutrition.  To follow advice by a dietician.,5.  Continue all the medication treatments before.,6.  To continue off and ongoing psychosocial nutritional counseling as necessary.