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Fracturing Points: Bone Disease in CF Camille Washowich, MSN, ACNP

Fracturing Points: Bone Disease in CF Camille Washowich, MSN, ACNP. Camille Washowich, MSN, ACNP. Bone Physiology Pathophysiology Prevelance in US Diagnosis & Treatmen t. Understanding Bone Disease. Bone Formation: Osteoblasts Bone Remodeling: Osteoclasts Bone Types:

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Fracturing Points: Bone Disease in CF Camille Washowich, MSN, ACNP

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  1. Fracturing Points: Bone Disease in CFCamille Washowich, MSN, ACNP Camille Washowich, MSN, ACNP

  2. Bone Physiology Pathophysiology Prevelance in US Diagnosis & Treatment Understanding Bone Disease

  3. Bone Formation: Osteoblasts Bone Remodeling: Osteoclasts Bone Types: Cortical: 80% bone mass; mineralized collagen; major component of tubular (long bones) Travecular: sponge, providing strength/elasticity to axial skeleton Bone Physiology

  4. Normal Bone Development

  5. Normal vs Osteoporotic Bone

  6. Bone disease first seen in CF 1979 Noted low BMD (bone mineral density) on DXA Abnormal bone turnover markers CFTR protein dysfunction 2004 Decreased production of osteoportegerin and prostaglandin E2, regulators of bone formation/resorbtion process Pathophysiology of Bone Disease

  7. Chronic infection/inflammation cycles: Increased release of cytokines (IL 1/6) and TNF increases bone reabsorption and decrease bone formation ABPA: following IgE levels Colonized Organisms: Bacterial: PA,MSSA/MRSA, B Cepecia, Achromobacter, Steno Mycobacterium: MAC, M Abscessus Pathophysiology of Bone Disease in CF

  8. Malnutrition with low BMI • Malabsorption: low vitamin D/K levels • Pancreatic Insufficiency • Delayed puberty/hypgonadism • Decreased peak bone mass • Steroid Therapy (pulse/chronic) • Decreased/Inactivity • Chronic Pain Pathophysiology of Bone Disease in CF

  9. Prevalence of Fractures Non-CF

  10. Secondary Osteoporsis Development

  11. Diagnostics: DXA CXR: fracture CT Chest/MRI Labs: Calcium Vitamin D3 Albumin Diagnosing Bone Disease

  12. Measures BMD in femor/lumbar/wrist Painless Outpatient T/Z Score are similar in adults Results: Normal > -1 Osteopenia -1 to -2.5 Osteoporosis < -2.5 DXA: Dual Energy X-ray Absorptiometry

  13. Normal CXR

  14. CXR: Left Thoracic Fracture

  15. MRI Findings: Lumbar Fracture

  16. Life Style Modifications Medications Nutritional Needs Exercise Surgery Treatment Options for Osteopenia/porosis

  17. Medications

  18. Nutritional Needs

  19. Weight Bearing: Running, Dancing, Sports Non-Weight Bearing: Swimming Safe, Fun, Affordable Exercise Options

  20. Surgery Options

  21. Take Calcium with Vitamin D twice daily Regular bone density testing Normal: every 2yrs Osteoporosis/Osteopenia: every year Regular weight bearing exercise daily Minimum of 30min daily Avoid smoking/excessive alcohol (>1 beer/day) Decreasing Fracture Risk

  22. Thank You!

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