Childhood Nephrotic Syndrome:The Clinical Pathway

Cherry Mammen, MD, FRCPC, MHScDouglas G. Matsell, MDCM, FRCPCDivision of Nephrology, BC Children’s HospitalGrand RoundsNov 13th, 2015

Cherry Mammen, MD, FRCPC, MHScDouglas G. Matsell, MDCM, FRCPCDivision of Nephrology, BC Children’s HospitalGrand RoundsNov 13th, 2015

Learning Objectives

To review the clinicalpresentation and treatment ofchildhood nephrotic syndrome

To share our recentlydeveloped childhood nephroticsyndrome clinical pathway

To discuss potential barriersfor pathway implementationfrom BC pediatricians’perspective

To review the clinicalpresentation and treatment ofchildhood nephrotic syndrome

To share our recentlydeveloped childhood nephroticsyndrome clinical pathway

To discuss potential barriersfor pathway implementationfrom BC pediatricians’perspective

Nephrotic Syndrome - Definition

Proteinuria

EdemaHypoalbuminemia

Nephrotic Syndrome - Diagnosis

The commonest pediatricglomerular disease

200

250

300

350

400

BCCH 2008-2013

0

50

100

150

Nephrotic Syndrome - Incidence

Childhood Type 1 Diabetes Mellitus

Daneman, Pediatric Diabetes 2009

Nephrotic Syndrome - Incidence

Childhood Crohn’s Disease

Griffiths, Inflamm Bowel Dis, 2011

Nephrotic Syndrome - Incidence

Ault, Clinical Nephrology 2012

Where do our NS patients live?

Mean 2688.5mg/m2STD 915.3mg/m2N=75

Nephrotic SyndromeLocal Practice Variation

Mean 2688.5mg/m2STD 915.3mg/m2N=75

What is a clinical pathway?

structured multidisciplinary plan of care used to channel the translation of evidence

into local structures details the steps in a course of treatment or

care has time-frame or criteria-based

progression standardized care for a specific clinical

problem, procedure or episode of care in aspecific population

structured multidisciplinary plan of care used to channel the translation of evidence

into local structures details the steps in a course of treatment or

care has time-frame or criteria-based

progression standardized care for a specific clinical

problem, procedure or episode of care in aspecific population

Kinsman L et al, BMC Medicine, 2010

Reasons to develop a pathway

“Too much practice variation in our division” “Hospital administrators are pressuring us to be

more involved in quality improvement” “Patients need to be more involved in their care” “Our documentation is scattered and needs

standardization” “They will improve efficiency and patient care”

“Too much practice variation in our division” “Hospital administrators are pressuring us to be

more involved in quality improvement” “Patients need to be more involved in their care” “Our documentation is scattered and needs

standardization” “They will improve efficiency and patient care”

Nephrotic Syndrome Clinical Pathway

NS Clinical Pathway Resources

NS Case History

4 y.o. male presents to community ERwith “edema”

Noticed swelling 3 wks ago & worsening Started around time of a viral URTI Face, abdomen, & lower legs No major discomfort & able to ambulate

Went to walk-in clinic 2 wks ago Diagnosed with “allergies”

4 y.o. male presents to community ERwith “edema”

Noticed swelling 3 wks ago & worsening Started around time of a viral URTI Face, abdomen, & lower legs No major discomfort & able to ambulate

Went to walk-in clinic 2 wks ago Diagnosed with “allergies”

Nephrotic Syndrome Case History

No gross hematuria Slightly reduced urine output (2x/day)

Drinks 1.5L/day with high salt diet No vomiting or diarrhea No headaches, SOB, or abdominal pain No fever, rash, or joint complaints Past medical history unremarkable No medications or allergies No family history of renal disease Immunizations “up to date”

No gross hematuria Slightly reduced urine output (2x/day)

Drinks 1.5L/day with high salt diet No vomiting or diarrhea No headaches, SOB, or abdominal pain No fever, rash, or joint complaints Past medical history unremarkable No medications or allergies No family history of renal disease Immunizations “up to date”

Nephrotic Syndrome Evaluation

Nephrotic Syndrome Case: Physical Exam

BP: 105/68 (

Nephrotic Syndrome Evaluation

NS Case Investigations (Urine)

Urinalysis: 5 g/L protein, 10-20 RBC’s/HPF Urine PCR: 1250 mg/mmol

NS Case Investigations: Labs

CBC: WBC 5, Hb 145, Platelets 350 BUN 5 mmol/L, Cr 40 umol/L Estimated GFR 114 ml/min/1.73m2 (normal)

Na+ 135, K+ 4.2, Cl- 102, HCO3- 24 Albumin 18 g/L Cholesterol panel not needed Additional nephritic work-up not needed

eg: C3, C4, ANA, anti-ds DNA

CBC: WBC 5, Hb 145, Platelets 350 BUN 5 mmol/L, Cr 40 umol/L Estimated GFR 114 ml/min/1.73m2 (normal)

Na+ 135, K+ 4.2, Cl- 102, HCO3- 24 Albumin 18 g/L Cholesterol panel not needed Additional nephritic work-up not needed

eg: C3, C4, ANA, anti-ds DNA

Where are we in the pathway?

Nephrotic Syndrome “Red Flags”

Where are we in the pathway?Induction Treatment

NS Treatment: Preventing Complications

• Types of nephrotic syndrome• Complications of nephrotic syndrome• Prednisone course & side effects• How to dip urine for protein• Dietary recommendations• Immunizations and infections• Ongoing care

• Types of nephrotic syndrome• Complications of nephrotic syndrome• Prednisone course & side effects• How to dip urine for protein• Dietary recommendations• Immunizations and infections• Ongoing care

Patient Home Monitoring

Home Monitoring Example

Dietary Management

Education resources: sodium content of

foods sample menus label reading food intake records BC Healthline *811

Education resources: sodium content of

foods sample menus label reading food intake records BC Healthline *811

Optimizing Bone Health

Immunizations

Detailed immunization history Obtain immunization records for chart Avoid live vaccines on “high dose” prednisone

Annual flu shot recommended Need to know if VZV series complete

If not, may need VZIG for exposure Recommend Pneumovax (PPV23)

Detailed immunization history Obtain immunization records for chart Avoid live vaccines on “high dose” prednisone

Annual flu shot recommended Need to know if VZV series complete

If not, may need VZIG for exposure Recommend Pneumovax (PPV23)

Where are we in the pathway?Surveillance Checklist

Back to Case

Started on therapy with no complications Patient goes into remission in week 2

Trace or negative protein on dipstick x 3 days Worksheets being completed adequately Growth has been normal Ophthalmology check-up & spinal x-rays normal Off Prednisone in 24 wks, but………

Call from parents: patient has developed URTI &urine dips are 3+ for 3 days with mild edema

Started on therapy with no complications Patient goes into remission in week 2

Trace or negative protein on dipstick x 3 days Worksheets being completed adequately Growth has been normal Ophthalmology check-up & spinal x-rays normal Off Prednisone in 24 wks, but………

Call from parents: patient has developed URTI &urine dips are 3+ for 3 days with mild edema

Where are we in the pathway?Relapse Treatment

Important NS Definitions to know

Our Clinical Pathway Team

Barriers to PathwayImplementation

Questions & DiscussionQuestions & Discussion