1. Carlye Tomczyk, APRN, CNP University of Minnesota
The Shrinking CF Patient – A Multidisciplinary Approach to Vitamin D Deficiency Treatment
Carlye Tomczyk, APRN, CNP
University of Minnesota

2. There are no relationships to disclose related to this presentation.
Presenter Disclosure
There are no relationships to disclose related to this presentation.

3. Case Presentation
19 yo male, F508del homozygous, pancreatic insufficient, CFRD, g-tube dependence, poor compliance. Frequently hospitalized
FEV1 decreased 10% over past month
7 pound weight loss over previous 5 months
8.6 cm decrease in height over past 5 months
Kyphosis on physical exam
Vitamin D level <17 ug/L
Admitted from clinic
Patient presented for close CF follow up visit (March). Had been seen monthly in CF clinic (since January) as he was struggling to care for himself at college. On Lumacaftor/ivacaftor for ~18 months prior to admission.
- New onset hemoptysis. C/O chest pain related to frequent coughing. FEV1 at admission was 65%. (Most recent personal best FEV1 90% - 7 months earlier)
- Glucoses running high, forgets to carb count. Reports doing tube feedings 4-6 nights per week.
- Two months prior to this visit, height was noted to have decreased by 1 inch – talk about measurement issues in clinic etc.
- Dexa scan completed 1 month prior to this visit showed decreased bone density for age, but increase in overall bone density compared to previous study 4 years earlier (which was normal).
- Height down about 1 inch at each of the monthly visits start in January – total of about 3 inch decrease in height over 3 months.
- On admission patient admitted to not taking his vitamin supplements for “a long time.” (vitamin D level above is from most recent annual studies)

4. Hospital Course Treated for CF pulmonary exacerbation
Endocrine consult for CFRD and osteopenia
Neurosurgery consult – lumbar spine film shows L1 compression fracture
CF team dietitian and pharmacist consult
CF pulm exacerbation treatment – increased vest/nebs, IV antibiotics, nutrition management with nightly tube feeds, CFRD management.
Endocrine consult- Last DEXA (2/10/17) with lumbar spine Z-score -2.1 (improved 11% from previous) and total body Z-score -1.4 (down 2% from previous). Concern for height loss of 1.5cm given documented heights on growth chart, although consider erroneous height documentation that was carried forward. No fracture noted on DEXA.
- c/o back pain in hospital which patient attributed to sleeping on a couch rather than from trauma
- recommend ongoing oral vitamin D supplements (8000 IU daily + CF multivitamin) – at discharge initially recommended 3x/week vitamin D2 (ergocalciferol) dosing.
- recommend lateral spine film
Neurosurgery – further imaging revealed chronic T5-T7 compression fractures, schmorl's nodes, and T8-T10, T12 thoracic vertebral wedging with kyphosis. No activity restriction, no bracing, clinic follow up in 6 weeks with repeat x-rays at that time.
Dietitian/Pharmacist – Stoss vitamin D dosing as a way to address non-adherence while treating hypovitaminosis D.

5. Osteopenia
Likely multifactorial related to poor overall nutrition, inflammation, inadequate vitamin d levels and calcium absorption from the gut
Normal testosterone level 1 month prior to admission
Bisphosphanate therapy recommended by endocrine
Stoss Vitamin D dosing recommended by CF dietitian and CF pharmacist
Why is vitamin D so important?
IV pamidronate Q3 months for 1 year. First infusion given during hospitalization.
Importance of vitamin D –
- Vitamin D & calcium helps bone mineralization, also plays a role in muscle function and immune system function.
- Studies have shown vitamin D decreases depression, also decreases inflammation.
- How do you get vitamin D - body can make vitamin D with sun exposure, dietary sources, supplements.
- CFF recommends serum 25-hydroxyvitamin D goal of at least 30 ng/ml
- emphasize importance of monitoring levels and prescribing adequate supplemental dosing

6. Stoss Vitamin D Dosing
High dose (500,000 IU) vitamin D3 – one time oral dose given in the hospital
Baseline labs (calcium, phosphorus, vitamin D, parathyroid hormone) measured
Follow up labs at 1 month, 3 months and 12 months following stoss dose
Shepherd, D et al. Single high-dose oral vitamin D3 (stoss) therapy – A solution to vitamin D deficiency in children with cystic fibrosis? J Cystic Fibrosis. 2013; 12:
Vitamin D level prior to stoss was 31
Stoss – German “to push”
Explain study and findings.
Study showed:
- following stoss and daily maintenance vitamin D supplement – able to maintain optimal vitamin D levels for 12 months.
- higher vitamin D levels are achievable with stoss therapy
- safe, efficient and practical approach – especially with non-adherent patients.

7. Discussion
Case highlights the importance of accurate anthropometric measurements for CF patients
Importance of adherence to fat soluble vitamin supplementation
Multidisciplinary approach - Pharmacist role in CF outpatient clinic.
Importance of vitamin D – re-emphasize this!
Med reconciliation – importance of adding vitamins to the medication list, keeping up to date
- we knew what he should be getting for his Vitamin D dose and as a result of having him fill at the pharmacy vs. buy OTC we knew that he wasn't taking them. If his med list wasn't accurate or he was just buying willy nilly otc we wouldn't have known this!

8. Questions?
1. Did the patient have a history of recent/chronic glucocorticoid therapy?
2. Does this make a case for earlier imaging for those with multiple risk factors for osteopenia?