Cystic fibrosis - Case Study Example

Case study information: Information per CF clinic visit on 3/30 NA (female) DOB: 10/07/ 1994 Diagnosis: Cystic Fibrosis, pancreatic insufficiency, CFRD, sinusitis, gastroesophageal reflux Past medical history of pneumonia Genetics: delta F 508/delta F 508 Airway Clearance: In-Courage (vest) System, 30 minutes 2x/day, Acapella, Quake Most recent laboratory data: taken 10/7/2009, due for blood draw today for annual labs and allergy labs, chest x-ray done 11/08/2009 Medications: Albuterol nebulizer 2 puffs 2x/day or as needed every 4 hours, Pulmozyme 1 vial 1x/day, Asmanex 220 mcg one click 2x/day, Colymycine inhaled 2x/day 28 days on, 28 days off and gentamycin alternating months 28 days on and 28 days off, (currently on course of gentamycin x 10 days) Nasal spray; Atrovent spray 1 squirt each nostril 2x/day, Nasonex: 1 squirt each nostril 1x/day Ultrase MT 12,000 units of lipase: 3 caps with meals & 2 with snacks Prilosec: 20 mg 2x/day Levemir insulin: 22 units at dinnertime Novolog: 2 units at bedtime Formula: via PEG: Vital HN 3 packs mixed with 500 mls water at rate of 75cc/hr for 10 hours nocturnal feeds Scandishake nutritional supplement mixed with whole milk (240 cc) 1-2 x/day Multivitamin daily Vitamin C: 500 mg 1x/day Vitamin D 2,000 IU/day Align probiotics: OTC ( over the counter) Periactin 4 mg 1x/day (stopped as of mid 2009) Pulmonary Function test: 3/2/2010: info given out during class 3/30/2010 data: FVC: 1.6 (53) FEV1: 1.1 ( 44%)FEV1/FVC: 68.75 (76) FEF25%-75%: .6 (23) Vital Signs: Weight: 46.

60 kg Height: 157.2 cm Temperature: 36.5 degrees Centigrade Respiratory Rate: 18/min Pulse rate: 94/min B/P: 93/63 mmHg O2 Sat 98% Last visit ( 3/2) WT: 45.64 kg HT: 157.2 cm Other Pertinent Information: Last clinic visit date 3/2/10: PT: N A was determined to have a mild pulmonary exacerbation. She was placed on a 14 day course of oral Clindamycin. Interval History: via completed interval questionnaire form and verbal interview with PT and her mother. PT states adherence with CF care plan. PT had a cold about 10 days ago and experienced an increase in fatigue and depressed appetite.

PT reports that she did continue to take her oral nutritional supplements during that time. Blood glucose levels have been maintained within good control per PT report. PT is working with Pedi Endocrine to continue to titrate insulin needs. She notes am glucose levels at ~150 mg/dL. NA reports 1-2owel movements daily. These are well formed and non- malodorous in nature. She reports no symptoms of constipation or rectal prolapse. She reports no abdominal pain, reflux/heartburn or gas however in the past has cited some intermittent abdominal pain after eating.

PT also reports recent vist to Ped GI (gastroenterology) and will be starting erythromycin therapy. PT update: Phone contact made by parents; NA had episode of hemoptysis of ~2ml in the morning of 3/31. PT continued airway clearance (VEST only) with no further episodes noted on that day. On 4/9 NA experienced another episode of hemoptysis . NA to begin course of Clindamycin 300 mg 2x/day for 2 weeks and 5 days of 20 mg Prednisone. Questions: Plot ht and wt on CDC growth chart 1.What is the current BMI for this PT?

Compare to CF Foundation Guidelines for BMI % ile goal for age and gender – 18.7 Normal body mass index 2. What are the goals for nutrition therapy for NA? During NA’s clinic visit what would be significant to review and address from a nutrition standpoint? Calcium is a factor; as well as vitamin D, vitamin supplements assist with fat absorption; probiotics to offset infection risk. 3. Review the medications for NA and discuss indications for usage of each for NA. Albuterol to increase maximal exercise performance, Pulmozyme to loosen mucus resulting from immune response to bacterial infection, Asmanex to reduce bronchial asthma attacks.

Colymycine for antibiotic infection control; useful if P. aeruginosa is suspected, for which Gentamicin can also be effective. Rotating between antibiotics increases the chance of eliminating the population before resistance becomes entrenched. Atrovent to prevent bronchial spasms, while Nasonex and Periactin can ease the secretions of CF by suppressing upper-respiratory allergic responses, such as the sinusitis. Ultrase to moderate the effects of pancrease insufficiency as mentioned above, (normalize bowel movements) With pancreatic difficulties, likely from thickened secretions interfering with normal function, Prilosec as a PPI, should control stomach acids.

Levemir insulin and Novolog to maintain blood glucose levels. PEG tube assists patients with difficulty swallowing; to be expected with CF. Discuss any side effects and/ or nutritional implications. Albuterol may result in airway instability, Asmanex may help offset this risk. Antibiotic resistance is a factor; rotating Colymycine and Gentamicin should help. 4. Review genetic profile of this patient. Discuss the genetic variability of this genotype ie: what are the chances of this PT inheriting CF.

Is this PT’s genotype homozygous or heterozygous? Discuss Based on genetic profile; what phenotype characteristics would you expect?( for example: would you expect PT to be PS or PI?) Discuss The genetics are what would be expected; the three nucleotide deletion at position 508, interrupting the cystic fibrosis transmembrane conductance regulator. Based on the above information, I would anticipate the patient to be homozygous for the mutant gene. Should the patient have children, there is a virtual certainty that a copy will be inherited. 5. Discuss the genetic possibility of someone being born with CF based on gender and race.

Explain the genetic defect that causes CF. The phenotype in indigenous the northern Europe, as an autosomal recessive disorder. Occurring on the 7th chromosome, (position 508) copies can be transmitted without sex-linkage. It is among the most common genetic disorders of Caucasians. 6. Explain the effects of CF on the entire body and compare and contrast to our case study PT NA. The Chlorine ion-transport secretory defects will complicate the upper respiratory tract, changing mucous viscosity; this provides an opportunity for opportunistic infection of the respiratory tract; which correlated with the antibiotics the patient is taking.

The altered secretions block ducts in the pancrease, creating digestive difficulties including frequent and odoriferous bowel movements, for which Ultrase is needed. Sweat may be more salty; males are likely to be infertile due to lesions on the Vas deferens. Filling of the sinuses is common, and necessitates drugs like Asmanex and Albuterol to avoid bronchial spasms. 7.Define the following terms: Steatorrhea – excess fat in feces Bronchiectasis – destructive widening of the large airways Atelectasis – collapsed lung Pneumonia – Severe lung infection Hemoptosis – bloody sputum DIOS – blockage of intestines with thick stool Meconium Ileus – thickened, obstructive secretions in newborn intestines Gastroparesis – weak stomach, less able to move digested food 8.

Define the following tests: 72 hour fecal fat, fecal elastase Discuss indications of use for each 72 hour fecal fat measures the amount of fat released in stool. Fecal elastase tests for insufficient concentrations of pancreatic enzymes. (pancreatic elastase 1) 9. Calculate the digestive enzyme dosage for NA. Is this a sufficient dosage for NA? Explain your answer. Why are digestive enzymes enteric coated? What is the best PH range for digestive enzyme effectiveness? Discuss the usage of PPI’s and H2 blockers in the CF population Proton Pump Inhibitors suppress gastric acid by shutting down cellular gated channels that release ions.

H2 blockers prevent the signals from reaching histamine receptors in the first place that would activate secretion. Based on her Prilosec dosage, it's reasonable to conclude that NA is hypersecretory; as her dose is double the recommended levels. Enteric coating ensures that digestion and release of the tablets occurs where the drug will be most effective. 10. NA had her annual labs drawn today: From a nutrition standpoint what values would be significant to review and why? What would be the normal ranges for the lab data collected?

NA's blood glucose is dangerously high, 150 mg/dl is not yet a diabetic crisis, but it is cause for serious concern. And likely the reason for the extra insulin. If the insulin dosages cannot be safely increased, then dietary steps should be taken to lower sugar intake. 11. Define CFRD. How is this different than Type 1 or Type 1 Diabetes? How is it treated in the CF population? What type of diet would you want NA to have? What therapies or conditions might influence NA’s blood glucose levels?

Cystic Fibrosis Related Diabetes, a result of pancreatic scarring is insufficient insulin production. Insulin levels or dietary sugar should be monitored, based on the above results changes in diet are warranted. With healthier glucose levels, NA will be able to gain weight. Review NA’s insulin therapy. Discuss and compare types of insulin prescribed for NA ( action, length of action, peak times etc.). Why is NA not prescribed an oral hypoglycemic agent such as Prandin? Prandin is contraindicated in patients already taking corticosteroids, and erythromycin.

It may also cause gastrointestinal side effects, such as constipation and diarrhea, and might exacerbate the patient's already fragile digestive system. Her present insulin therapy leaves her with a heightened blood glucose level; to reach a healthier weight – a goal indicated by the use of scandishake, her insulin needs to be controlled. 12. Calculate the range of calories that you would expect NA to consume in order to meet her calorie needs. If NA was an adult calculate her needs using standard and “Mifflin” equation.

Compare/contrast and discuss. How many calories is NA receiving via PEG tube feeds and oral nutritional supplementation? 1,370 BMR and 1,362 RMR via the Mifflin equation. The standard equation is purported to have 5% less accuracy. Given NA's consumption of scandishake supplements, it is probable that due to her condition, her actual caloric needs are in excess of this calculated amount. Scandishake yields 600 calories per serving, with 2 servings daily, it is probable that her needs are 1200 calories in excess of the calculated 1,370. (2,570) 13.

Why might NA’s gastroenterologist prescribe erythromycin? Discuss Prevent infection, or halt ongoing one. NA had a cold ten days ago, and is already vulnerable to respiratory pathogens. While antibiotics cannot affect the cold virus, the disruption caused by cold/flu symptoms may weaken the lining of the airways, creating an opening for opportunistic bacteria. 14. Explain new born screening test for CF, genetic testing, sweat test and NPD CF sweat is known to be excessively salty, likely a result from abnormal chlorine ion secretions.

Nasal Potential Difference is a newborn screening test to measure ionic potential in newborn secretions to determine abnormal ion secretions indicative of CF. A heel stick is routine, to test for unusual blood conditions.