PEN-Plus Clinical Tools and Programmatic Standards by NCDIPN

PEN-PLUS CLINICAL TOOLS AND

PROGRAMMATIC STANDARDS

Disclaimer: This booklet is a selection of high value tools from the PEN-Plus Resource Library developed by the NCDI Poverty Network. The Resource Library is an online repository of tools to help to support initial implementation of PEN-Plus. All tools included in this booklet have received input and validation from the PEN-Plus Expert Groups (Sickle Cell Disease, Diabetes, and Cardiac) and are aimed to support frontline PEN-Plus providers. The online Resource Library will continue to incorporate edits. These tools are consistent with vetted PEN-Plus training tools, but where applicable, providers should defer to national treatment guidelines.

See link below for full PEN-Plus Resource Library including an electronic version of this booklet https://drive.google.com/drive/folders/1_x8QHVAD0QVKt1cVGsxdePtMTY8I_tZW

We welcome comments on this toolkit and will incorporate them in the next version. Please send any comments or questions to the following addresses; ewroe@bwh.harvard.edu; kdomingues@bwh. harvard.edu; colinpfaff5@gmail.com

Version 1. April 2024

Section 1: PEN-Plus Essential programmatic standards

1.1 PEN-Plus: Essential Programmatic Standards

1.2 Programmatic Standards Checklist

1.3 Indicators for programmatic standards

Section 2:Tools for cardiac disease

2.1 Protocol For Heart Failure

2.2 Pediatric Protocol For Heart Failure

2.3 Warfarin dose adjustment chart

Section 3: Tools for diabetes

3.1 Pen-Plus Working Definition Of T1D Diagnosis

3.2 Insulin Regimen Options

3.3 How To Adjust Insulin Doses Based On Home Glucometer Readings

3.4 Using correction scales (mmol/L)

3.5 Using correction scales (mg/dL)

3.6 Diabetic Ketoacidosis (DKA) Algorithm - Adults

3.7 Diabetic Ketoacidosis (DKA) Algorithm - Children

3.8 DKA Monitoring Chart

Section 4: Tools for Sickle Cell Disease

4.1 Screening for SCD using rapid tests

4.2 Sickle Cell Disease: Prevention Protocol

4.3 Hydroxyurea (HU) Protocol

4.4 Hydroxyurea dose chart for use in sickle cell disease

4.5 Blood Transfusion (Emergency simple)

4.6 Pain Management Protocol

4.7 Acute Complications Protocol

Section 5: Tools for Respiratory Disease

5.1 Asthma Out-Patient Management Chart

5.2 Asthma Exacerbation Management Chart

5.3 COPD Out-Patient Management Chart

5.4 COPD Exacerbation Management Chart

5.5 How to make a bottle spacer

SECTION 1 PEN-PLUS ESSENTIAL PROGRAMMATIC STANDARDS

1.1 PEN-Plus: Essential Programmatic Standards

Introduction

What is PEN-Plus?

PEN-Plus complements the World Health Organization’s (WHO) Package for Essential NCDs (PEN). Whereas WHO PEN focuses on primary care for common chronic NCDs, PEN-Plus focuses on care at the secondary level for more severe conditions, including type 1 diabetes (T1D), sickle cell disease (SCD), and rheumatic and congenital heart disease. PEN-Plus is a clinical model that provides high-quality longitudinal care for people living with severe and chronic NCDs. It is an integrated strategy that focuses on developing teams of nurses, clinical officers, other mid-level providers, and often general medical officers working at first-level hospitals, such as district hospitals. These expert NCD teams focus on diagnosis and care for chronic NCDs that may require services beyond primary care, focusing on caring for patients at the hospital level as well as supporting primary care systems through mentorship and referrals.

Who is this document for?

This document is for anyone leading, implementing, funding, advising on, or advocating for PEN-Plus clinics. It is meant to aid clinic and program planning, budgeting, and operations by providing a crisp definition of PEN-Plus clinical care, including the diagnostic, treatment, and patient support services available. With a clear definition of these services, stakedholders can extrapolate additional parts of the program, such as lists of essential medications and equipment, training needs, and healthcareprovider competencies.

How was this document created?

The PEN-Plus Essential Programmatic Standards are drawn from years of experience implementing PENPlus across 14 countries. The first version of the document was drafted based on models of care in these countries and informed by multiple sources:

• Clinical experts, ministries of health, healthcare providers, and other partners with PEN-Plus experience;

• Clinical Expert Groups in T1D, SCD, and cardiac care through the PEN-Plus Partnership, composed of clinical specialists and other providers across the NCDI Poverty Network;

• Clinical outcomes data from early PEN-Plus clinics in Rwanda, Malawi, Liberia, and Haiti; and

• PEN-Plus training packages, which were refined with input from the above sources over several years.

How should this document be used?

This document can be used as a reference document both for planning a PEN-Plus program and for assessing and refining existing PEN-Plus clinics. A few pointers:

• In defining different diagnostic and treatment services, this document assumes on-site availability and functionality at the PEN-Plus clinic, unless otherwise indicated.

• “Availability” also assumes that key components—including durables, consumables, and staff skills and competencies—that are required to make services possible are available as well.

• This will be a living document that will change and adapt as PEN-Plus systems strengthen and higher levels of care and standards are achieved.

Diagnosis & Treatment Standards

Condition Area

(conditions that are part of PEN-Plus across the lifespan)

Type 1 diabetes

Cardiac: congenital heart disease, rheumatic heart disease, heart failure

Sickle cell disease

Severe asthma

Chronic liver conditions

Chronic kidney conditions

Other*

Essential Part of Package vs. Adapted to Context

Essential

Essential (unless not present from an epidemiologic perspective)

Adapted to context

*Other conditions that might be part of these categories—such as insulin-dependent type 2 diabetes, other types of heart conditions, other hemoglobinopathies, and epilepsy—can all be part of the package, depending on the context.

Cross-cutting Standards

Diagnosis

• Basic physical exam and vital signs equipment: stethoscopes, pulse oximetry probes (both pediatric and adult), blood pressure cuffs, thermometers, weighing scales, MUAC tape

• Full blood count

• Urinalysis

• Chest x-ray

• Blood electrolytes, liver function, kidney function

Treatment and Support

Outpatient

• Patients with conditions in the PEN-Plus package are diagnosed and cared for across the lifespan, with no age limits to enrollment, treatment, or access to PEN-Plus resources

• Trained PEN-Plus clinical providers are consistently available in the clinic

• Patients are given appointment dates and tracked to ensure longitudinal follow-ups

• Social support mechanisms are in place to avoid catastrophic costs—such as consultation fees, medications, lab testing, and transport fees—for patients who need that support

• Patients are enrolled in medical insurance, if available

• Social support systems are also in place to identify and address specific vulnerabilities, such as food insecurity and school fees

• The longitudinal data system tracks patient appointments and individual medical records with data elements required for reporting on core and clinical indicators

Other

Inpatient

• Treatment of NCD emergencies with diagnosis and management

• Identification of new cases of people admitted with severe NCDs

• Education, discharge planning, and referrals to PEN-Plus

• Case finding is incorporated into inpatient settings, outpatient clinics, other specialized clinics, and—where appropriate—community settings

• Peer-support opportunities are available to people living with PEN-Plus conditions

• Contextualized referral procedures are available for complex cases and those requiring specialist assistance, such as surgical options for cardiac patients; exchange transfusions, bone marrow transplant, and hip replacement for patients with sickle cell disease; and gastroscopy and banding for patients with liver disease

• Linkage with other services at the facility as needed, including mental health and palliative care

Type 1 Diabetes Diagnosis and Treatment Standards

Diagnosis

• Fingerstick blood glucose; hemoglobin A1C

• The facility is equipped to diagnose diabetic ketoacidosis (DKA) with blood glucose and urine dipstick with ketones

• Optimal: blood electrolytes, kidney function

Treatment and Support

Outpatient

• Insulin regimens using basal bolus with long-acting insulin (e.g., NPH or glargine) and rapid mealtime insulin

• Home glucometers, lancets, and test strips available free of charge (quantity: 3 checks per day or 90 per month)

• Check A1C every 3 to 6 months

• Behavioral counseling on diabetes self-management; availability of diabetes educator

• Adolescent- and young-adult-specific counseling and therapy

• Screening and management for complications including diabetic ulcers, retinopathy, neuropathy, and proteinuria

• Co-management with a nutritionist, if available

Cardiovascular Diagnosis and Treatment Standards

Diagnosis

Inpatient

• Treatment of DKA with

· IV fluids (normal saline and/or Ringer’s lactate); Short-acting insulin; Potassium replacement (IV preferred); and Monitoring for fluids, fluid sequestration, and urine output.

• Optimal: IV pump

• Echocardiography*, gel, and a place to perform echocardiography *2D echo minimum, color flow Doppler, image archiving system, cardiac probe (optional capabilities are spectral PW/ CW Doppler)

• ECG (minimum of single lead)

• Electrolytes, kidney function, INR

Treatment and Support

Outpatient

• Echocardiography with a trained clinician for initial diagnosis and routine follow-up echo

• Monitoring for atrial fibrillation with ECG

• Display and use of the PEN-Plus cardiac failure protocol to guide assignment of diagnostic category and treatment

• Treatment of heart conditions with furosemide, ACE inhibitors, betablockers, spironolactone, hydralazine, isosorbide dinitrate, aspirin, calcium channel blockers, and thiazide diuretics

• Pediatric formulations of furosemide, spironolactone, and propranolol

• Treatment with long-term anticoagulation, including warfarin with routine INR monitoring

• Optimal: Anticoagulation options include low-molecular-weight heparin during pregnancy

• Secondary prophylaxis for rheumatic heart disease with oral and IM penicillin (and penicillin alternatives for those allergic)

• Management of anticoagulation for thromboembolism, including initiation with heparin and transition to outpatient therapy

• Management of complex cases of hypertension that cannot be managed at the primary care level

• Capability for remote review of echo images, such as by referral hospital cardiology clinicians, cardiac surgeons, or mentors

• Referral pathway for patients who need cardiologist evaluation, including those with rheumatic or congenital heart disease

• Post-surgical long-term follow-up and management for patients who receive cardiac surgery for rheumatic or congenital heart disease

Inpatient

• Treatment for heart failure exacerbations, hypertensive emergency or urgency, and other cardiac complications with:

· IV diuretics, e.g., furosemide;

· IV hydralazine or IV labetalol Heparin; and Medications on the outpatient list

• Identification of potential children with congenital heart disease and referral for cardiologist evaluation

• Optimal: Access to cardiac surgery for some subset of patients every year

Sickle Cell Disease Diagnosis and Treatment Standards

Diagnosis

• Rapid confirmatory testing (SickleScan, HemotypeSC, or Gazelle Hb variant)

• Routine use of on-site testing for people with signs and symptoms of SCD, per the PEN-Plus diagnostic testing algorithm

• Optional: sickling test, peripheral smear

• Per national guidelines: Send out to the lab for advanced confirmatory testing (e.g., Hb electrophoresis, isoelectric focusing (IEF), or high-performance liquid chromatography (HPLC))

Treatment and Support

Outpatient

• Hydroxyurea treatment (initiated based on on-site testing availability) as per the PEN-Plus protocol

• Optimal: Pediatric formulation of hydroxyurea

• Treatment monitoring with complete blood count, Hb

• Preferable: Liver function tests, kidney function

• Preventive care with folic acid, antibiotic prophylaxis, vaccination, and malaria prophylaxis as per the PENPlus protocol

• Pain control, with narcotics available as needed

• Counseling and education on SCD-related complications, a healthy diet, nutrition, hydration, hygiene, and prevention of acute events

• Specific care for SCD during pregnancy, including close monitoring, medication changes per protocols, and ensuring the availability of facility-based delivery with referrals when indicated

Inpatient

• Treatment of acute events with:

· Emergency blood transfusions;

· IVF (RL, NS, or DNS);

· Pain control, including IV narcotics (e.g., morphine or other opioids), as needed; and IV antibiotics

Respiratory Disease Diagnosis and Treatment Standards

Diagnosis

• Chest x-ray and GeneXpert

• Consider: Peak flow

• Optimal: Use of spirometry to confirm asthma based on reversibility and/or to identify additional possible causes of respiratory symptoms

Treatment and Support

Outpatient

• Determination of a clear diagnosis of asthma, with other causes excluded (e.g., TB, a cardiac cause, cancer, COPD)

• Treatment with inhaled beta-agonists (e.g., salbutamol), inhaled corticosteroids, inhaled ipratropium bromide

• No patients on oral salbutamol

• All asthma patients are, at a minimum, on a rescue or short-acting beta-agonist inhaler (SABA) and inhaled corticosteroids (ICS)

• An inhaler-technique assessment on all patients, with appropriate counseling and teaching on inhaler technique

• Counseling on identifying and avoiding triggers

• Availability of spacer device

• Treatment of acute illness with oral steroids and antibiotics as needed

• Optimal: A written action plan for patients to increase their own steroid dose if their condition worsens

Inpatient

• Treatment of acute exacerbations or illness with: Oxygen; Nebulizers; Steroids, including an IV option; Antibiotics

Liver Disease Diagnosis and Treatment Standards

Diagnosis

• Liver function tests, electrolytes

• Full blood count (platelets)

• Abdominal ultrasound

• Optimal: Hepatitis B surface antigen, hepatitis C antibody

• Optimal: Microscopy of ascitic fluid, serum albumin, and fluid albumin

Treatment and Support

Outpatient

• Diuretic therapy with furosemide, spironolactone

• Antibiotic prophylaxis for a history of spontaneous bacterial peritonitis

• Beta-blocker therapy if the patient is at risk for esophageal varices bleeding

• Scheduled or outpatient paracentesis as indicated

• Optimal: Lactulose for hepatic encephalopathy

• Optimal: Serum electrolytes

• Optimal: Antiviral treatment for chronic hepatitis B or C infection with routine laboratory monitoring

Inpatient

• Paracentesis

• IV antibiotics for spontaneous bacterial peritonitis

• Optimal: Lactulose therapy for hepatic encephalopathy

• Optimal: Proton-pump inhibitor therapy and blood transfusion for esophageal bleeding

Kidney Disease Diagnosis and Treatment Standards

Diagnosis

• Urine dipstick

• Creatinine and electrolytes

• Renal ultrasound

• HIV testing

• Optimal: Urine microscopy

Treatment and Support

Outpatient

• Diuretic therapy as indicated

• Treatment for hypertension

• ACE inhibitors

• Iron supplementation

• Laboratory monitoring for kidney function, electrolytes, and Hb

• Treatment and follow-up for nephrotic syndrome, including steroids

• Treatment for symptoms and co-morbidities, including depression, poor nutrition, pain, delirium, and other palliative-care needs

Inpatient

• IV diuretics

• IV antihypertensives

• Treatment of urinary obstruction

• Palliative care

1.2 Programmatic Standards Checklist

This checklist can be used as a quick guide to determine whether a given PEN-Plus clinic is meeting the programmatic standards and to help identify areas for early action.

Cross Cutting / Health Systems

Staffing

c Nonspecialist providers are consistently available on-site to provide integrated chronic care for severe NCDs (A nonspecialist—or mid-level—provider refers to nurses, clinical officers, or medical officers, depending on the context.)

c Number of providers trained is adequate to meet both the clinic’s volume needs and staffing schedules

c The same health care providers are dedicated to the clinic and are consistently staffing the clinic week to week (as opposed to all hospital staff rotating through the clinic)

c Clinic staff members have received thorough training in the diagnosis and management of the prioritized severe, chronic NCDs to care for those patients across the lifespan

Case finding procedures

c There is a clear linkage from the inpatient setting for people with severe, chronic NCDs to be enrolled directly in the PEN-Plus clinic prior to discharge

c Enrollment into the PEN-Plus program is open to patients of all ages, with conditions in the PEN-Plus package guiding eligibility, noting that the PEN-Plus conditions often occur in children, adolescents, and young adults, necessitating diagnosis and care across the lifespan

c Staff in outpatient clinics (such as adult and pediatric OPD) have a working knowledge of the signs and symptoms of severe NCDs and can refer patients to the PEN-Plus clinic for diagnostic workup and enrollment to care

c Community outreach includes education for severe NCDs and, where appropriate, screening and linkage to PEN-Plus care, such as through school programs and community health workers

Appointment structure

c A dedicated and consistent space enables the clinic to be held on all PEN-Plus clinic days

c There are designated days when the PEN-Plus clinic is held, and all staff and resources are available during those days. (Note: This may be every day of the week.)

c Clinic days are scheduled such that severe or complex conditions have sufficient clinician time for appointments. (This indicates a need to separate clinical time for severe or complex NCDs from primary care high-volume conditions, such as hypertension.)

c Optimal: There is a mechanism to track upcoming appointments and provide appointment reminders at least once weekly

Social work & social support

c All patients are receiving lab testing and treatment either for free or at minimal cost for those who can pay (with or without insurance coverage); all those who cannot pay are receiving services without out-of-pocket fees

Notes

c The clinic has a mechanism to identify missed visits in a timely manner AND to perform outreach to try to bring people back to care

c The clinic has a mechanism for assessing each patient’s vulnerability and social support needs

c The clinic has a social support program for patients who are the most vulnerable and/ or the most at risk of being lost to follow-up. This includes, at minimum, coverage of user and clinical fees for any needed medications or diagnostics and transport fees for patients deemed to need it.

Cross-Cutting Equipment

(all of these items are consistently and reliably available)

c Full blood count

c Basic physical exam and vital signs equipment, including stethoscopes, pulse oximetry probes (both pediatric and adult), blood pressure cuffs, and thermometers

c Urinalysis

c Optimal: Chest X-ray

c Blood electrolyte, liver function, and kidney function tests

Data Systems

c The clinic has a longitudinal data system—such as a patient file or electronic medical record system—that allows clinical and other staff to track patients in place and over time

c Data collected routinely for each patient visit include PEN-Plus standardized data elements (“phase 0 data elements”)

c Data systems allow for quarterly aggregation of “core indicators” with a reasonable degree of effort by staff

c Clinic staff can obtain longitudinal cohort data, clinical outcomes data, and other patient-level metrics through a manual chart review or digital data extraction

c The PEN-Plus team works with staff trained in data collection, quality, and aggregation

c The clinic’s data system can produce any nationally or regionally mandated indicators— such as ministry of health indicators for health management information systems—for severe NCDs

Diabetes

All must be consistently available

c Glucometer, test strips, and lancing devices

c A1C testing

c Urine dipstick for ketones and protein

c Long acting insulin

c Short- or rapid-acting insulin

c Insulin syringes

c Inpatient therapy for DKA with IV fluids, insulin, and potassium replacement

c Glucose solution for hypoglycemic emergencies

Patients with T1D are all receiving

c Long-acting insulin and short-acting insulin (and not mixed insulin)

c Home glucometers with strips for 3 checks per day (2 bottles/month)

Notes

c Syringes for home use, 1 for each day (30 / month)

c Lancing devices (disposable is 1 per test, or 90 per month; pen-type lancet is 1 per week, or 4 per month)

c Print logbook for home glucose monitoring

c Safe, cool storage for insulin at home (e.g., clay pot)

c Needle disposal plan (e.g., safety boxes)

c Home treatment plan for hypoglycemia (e.g., candy, sugar cubes, fruit)

c A1C testing every 3 to 6 months

c Individualized behavioral, diet, and self-management counseling

c Adolescent- and young-adult-specific counseling and therapy, where applicable

c Screening for complications—including diabetic ulcers, retinopathy, neuropathy, and proteinuria—with appropriate management or referral

Cardiovascular

All must be consistently available Notes

c Functional echo machine and gel

c An image storage system that can transmit echo images for review

c Functional ECG machine (single lead is acceptable)

c Ability to measure accurate weight

c Pediatric BP cuff and pediatric 02 sat probes

c INR monitoring

c Heart failure medications, including the choice of an ACE inhibitor or a beta-blocker

c Antihypertensives, including HCTZ and calcium channel blockers

c Diuretics, including furosemide and spironolactone

c Additional cardiac medications for specific uses, including hydralazine, isosorbide, and methyldopa

c Pediatric formulations of diuretics and beta blockers

c Anticoagulation therapy with warfarin

c Penicillin therapy for secondary prophylaxis of rheumatic heart disease (e.g., oral penicillin, benzathine penicillin), and an alternative for people allergic to penicillin)

Patients with cardiac diagnoses are all receiving, as applicable Notes

c Annual echocardiography monitoring

c Treatment and monitoring per the PEN-Plus cardiac failure protocol

c For patients with rheumatic heart disease: antibiotic prophylaxis, rate control as needed, anticoagulation as needed

c Heart failure: home management with diuretics, ACE-inhibitors, beta blockers

c Education and individualized counseling for behavior, disease management, danger signs, etc.

c Referrals for cardiologist evaluation for children with possible congenital heart disease

c Referrals for evaluation for cardiac surgery for patients with rheumatic heart disease and children with congenital heart disease

Sickle Cell Disease

All must be consistently available

c Rapid confirmatory tests (SickleScan, Hemotype SC, or Gazelle)

c Complete blood count, Hb

c Hydroxyurea

c Folic acid

c Penicillin

c Antimalarial prophylaxis per guidelines

c Pain control with oral and IV narcotics

c Blood transfusions for emergencies

c IV fluids for acute complications

c IV antibiotics for acute infectious complications

Patients with SCD are all receiving

c Hydroxyurea (unless there is a contraindication)

c Treatment and monitoring per the PEN-Plus hydroxyurea protocol

c Treatment monitoring with Hb at minimum

c Optimal: Treatment monitoring with complete blood count

c Optimal: Monitoring of kidney function and liver function tests

c Folic acid

c Penicillin prophylaxis for those under 5 years or otherwise eligible

c Analgesics as needed

c Antimalarial prophylaxis per national guidelines

c Health education and counseling

Essential Standards Checklist: Optional Conditions

Respiratory

All must be consistently available

c Short-acting beta-agonist inhalers (SABA), such as salbutamol

c Inhaled corticosteroids (ICS)

c Optimal: Anticholinergic inhalers (ipratropium bromide)

c Spacer devices

c Optimal: Combined long-acting beta agonist (LABA) inhalers with inhaled corticosteroids (ICS)

c TB diagnostics (e.g., GeneXpert)

c Functional chest x-ray machine

c Nebulizer machine with medications

c Oral and IV steroids

c Optimal: Spirometry

Notes

Patients with asthma are all receiving

c A clear diagnosis of asthma, with other causes—such as TB, cancer, COPD, or a heart condition—excluded

c Optimal: Spirometry use to support diagnosis

c Short-acting beta-agonist inhalers (SABA)

c Inhaled corticosteroids (ICS)

c Optimal: Combination of long-acting beta agonists (LABA) with inhaled corticosteroids (ICS)

c TB screening and ruling out as applicable

c Appropriate counseling and teaching on inhaler technique

c Counseling on identifying and avoiding triggers

c Optimal: A written action plan for patients to increase their own steroid dose if their condition worsens

Liver

All must be consistently available

c Liver function tests

c Full blood count (platelets)

c Abdominal ultrasound

c Hepatitis B surface antigen tests

c Optimal: Hepatitis C antibody tests

c Optimal: Microscopy of ascitic fluid, serum albumin, and fluid albumin

c Equipment and supplies for paracentesis

c Diuretic therapy with furosemide, spironolactone

c Antibiotics for secondary prophylaxis of spontaneous bacterial peritonitis

c Beta blockers for patients at risk for esophageal varices bleeding

c Optimal: Lactulose for hepatic encephalopathy, serum electrolytes for diuretic monitoring, and antiviral treatment for chronic hepatitis B or C infection

Patients with liver disease are all receiving, as applicable

c Regular evaluation for symptoms and signs of decompensated liver disease (including ascites, encephalopathy, and esophageal varices)

c Antibiotics for secondary prophylaxis of spontaneous bacterial peritonitis

c Beta blockers for patients at risk for esophageal varices bleeding

c Paracentesis when indicated, including scheduled or outpatient paracentesis in clinic

c Diuretic therapy as indicated, with electrolyte monitoring

c Optimal: Annual measurement and estimation of fibrosis through non-invasive tests (i.e., APRI score)

c Evaluation and secondary prevention of potential etiologies for liver disease (e.g., viral hepatitis, alcoholic hepatitis, steatotic liver disease)

Notes

Kidney Conditions

All must be consistently available

c Creatinine and urea testing

c Urine dipstick

c Prednisone

c Palliative care

c Renal ultrasound

c Oral and IV diuretics

c Oral and IV antihypertensive medications

c Iron supplementation

c Optimal: Urine microscopy

Patients with kidney conditions are all receiving

c Diuretic therapy

c ACE-inhibitors if eligible

c Iron supplementation

c Laboratory monitoring for creatinine, urea, electrolytes, and Hb

Notes

1.3 Indicators for programmatic standards*

*Note: These indicators focus on process and are designed to help identify key gaps in the programmatic standards. The standardized indicators for clinical outcomes and targets are defined elsewhere.

Category Indicator

Social Support

Supply Chain

Type 1 Diabetes

Cardiac Conditions

% of patients assessed for vulnerability or at risk of interruption to care

% of patients receiving social support –

# of days with insulin (of any kind) stockout in last year 0

# of days with furosemide stockout in last year 0

# of days with hydroxyurea stockout in last year 0

# of days with glucometer or test strip stockout in last year 0

# of days with sickle cell testing stockout in last year 0

% people with T1D on basal bolus insulin (not pre-mixed insulin)

% people with T1D with home glucometer

% people with T1D with an A1C checked within the last 6 months

% of patients with an echo in the last year

% of patients with suspected congenital or rheumatic heart disease referred for surgical evaluation

% of patients with rheumatic heart disease on secondary prophylaxis

% of patients with cardiomyopathy on beta blocker and ACE-I

% of patients on warfarin with an INR checked in the last 3 months

Sickle Cell Disease

% of people with SCD on hydroxyurea (among those who do not have a contraindication)

% of under-5 children with SCD on penicillin prophylaxis

% of people with SCD on folic acid

Chronic Respiratory Disease

% of patients with asthma who have a rescue beta-agonist inhaler

% of patients with asthma on oral salbutamol

% of eligible patients on inhaled corticosteroid

Liver % of patients tested for hepatitis B virus

% of patients with cirrhosis and at risk for variceal bleed who are on prophylaxis for esophageal varices

Kidney % of eligible patients on ACE-I

% of patients with chronic kidney disease with kidney function and electrolytes checked in last 6 months

SECTION 2 TOOLS FOR CARDIAC DISEASE

2.1 Protocol For Heart Failure

PROTOCOL FOR HEART FAILURE DIAGNOSIS AND MANAGEMENT

Signs or symptoms of heart failure?

Check medical records for previous echo YES

Ultrasound available?

Reduced left ventricular systolic function

Mitral valve abnormality (2/3)

- Thick / calcified mitral leaflets

- Elbow shaped anterior leaflet

- Fixed posterior leaflet

Blood pressure

≥180/110 mmHg or history of hypertension

Follow protocol according to diagnosis

Treat as cardiomyopathy & refer for echo

Cardiomyopathy

Rheumatic mitral stenosis

Hypertensive heart disease

Other valvular or congenital Loud murmur

Dilated right ventricle

Pericardial effusion

Heart failure unlikely (normal echo)

Beta-blocker: atenolol

Beta-blocker: carvedilol

Beta-blocker: metoprolol CR

Beta-blocker: bisoprolol

ACE inhibitor: captopril

ACE inhibitor: lisinopril

ACE inhibitor: enalapril

ACE inhibitor: ramipril

Isolated right heart failure

Pericardial disease

Consider other diagnoses

Non-cardiomyopathy medications:

ASA 100mg daily

Penicillin V 250mg twice daily or Penicillin IM inj

Furosemide (contraindication: Cr >2.3)

Hydrochlorothiazide (HCTZ) (contraindication: Cr >2.3)

Amlodipine: 2.5-10 mg po od (goal BP <140/90 mmHg)

Nifedipine: 20-80 mg po od (goal BP <140/90 mmHg)

1) Furosemide if hypervolemic

2) ACEi (titrate to goal dose)

3) Beta-blocker (titrate to goal dose)

4) if max ACEi and BP not low: add spironolactone

5) Investigate & treat causes of cardiomyopathy

6) If peripartum: add ASA 81 mg od

1) Furosemide if hypervolemic

2) Beta blocker (goal HR 60-80 bpm)

3) Penicillin (secondary prophylaxis)

4) Cardiologist consultation

1) Furosemide and/or HCTZ if hypervolemic

2) BP control: goal sBP <140 mmHg

1) Cardiologist consultation

2) Furosemide if hypervolemic

3) BP control

4) Consider penicillin for secondary prophylaxis if mitral valve thickening and calcification is present

1) Furosemide

2) Treat lung disease (asthma/COPD, TB) if present

1) Refer for pericardiocentesis if tamponade (low BP, tachycardic, IVC dilated)

2) Treat the cause (TB, HIV, cancer)

*Note that combined diagnoses are possible and can be made in consultation with an expert

*Family planning should be considered in all cases of heart failure affecting women of childbearing age

2.2 Pediatric Protocol For Heart Failure

Pathologic murmur, signs & symptoms of heart failure, or cyanosis?

Previous cardiologist plan?

Perform echo

Reduced left ventricular contraction

Mitral regurgitation present (>2 cm jet)

Dilated right ventricle and atrium

Pericardial effusion

Follow previous plan If any concerns, call cardiologist

1. Suspected cardiomyopathy; treat according to general protocolusing pediatric medication dosages 2. Furosemide if hypervolemic

1. Suspicious for rheumatic heart disease 2. Furosemide if hypervolemic

3. Start oral penicillin if available

1. Suspect congenital heart disease

1. Treat for TB unless obvious other cause 2. Refer for pericardiocentesis if tamponade

Starting dose of furosemide: 1mg/kg IV 12 hourly or 1mg/kg oral 12 hourly. Discuss dose increases with cardiologist or pediatrician

2.3 Warfarin dose adjustment chart

INR Action

Greater than 5.0 or complaints of bleeding.

Above goal

(INR raised by more than 2.0 above goal)

Above goal

(INR raised by less than 2.0 above goal)

At goal

Below goal

Below 1.5

Refer to cardiologist or review via telemedicine

• Hospitalize for warfarin adjustment.

• Stop warfarin for 2 days.

• Decrease warfarin dose by 1 mg daily.

• Evaluate for changes in medications or diet.

• Decrease warfarin dose by 0.5 – 1 mg daily.

• Continue current warfarin dose.

• Evaluate for changes in medications or diet.

• Assess for non-adherence.

• Increase warfarin dose by 0.5 – 1 mg daily.

• Hospitalize for warfarin adjustment and possibly subcutaneous low molecular weight heparin

• Assess for non-adherence and discuss with the patient’s community health worker.

SECTION 3 TOOLS FOR DIABETES

3.1 Pen-Plus Working Definition Of T1D Diagnosis

PEN-PLUS WORKING DEFINITION OF T1D DIAGNOSIS

TYPE 1

DIABETES:

Age 1 – 25* years

Age 25 - 45* years

Ever produced ketones?

New onset in pregnancy

*Age ranges can be adjusted based on local epidemiology

Age > 45* years

Age <1 year

None of above but signs suggest T1D: low BMI, weight loss, polyuria, polydipsia, and/or history of long-term insulin requirement

Consider family history of T1D or T2D as supporting evidence. If this does not help and person does not meet any of the considerations so far, proceed to possible T1D diagnosis.

Does not require insulin therapy or new onset with no ketosis

Clinical evidence suggests T1D (ketosis; weight loss; dehydration; low BMI)

Treat and document as T1D

possible TYPE 1 DIABETES:

Treat as T1D and monitor closely. Continue to evaluate for definitive diagnosis. Supplementary lab testing if available

TYPE 2

DIABETES: Treat and document as T2D

Consider other type of diabetes

3.2 Insulin Regimen Options

Mixed Insulin (70/30): 2 times daily

Glargine: 1 time daily

NPH: 2 times daily

Glargine: 1 time daily only Regular: 2-3 times daily

NPH: 2 times daily Regular: 3 times daily with correction dose based on pre-meal glucose

NPH: 2 times daily Regular: 3 times daily with fixed doses

NPH: 2 times daily Regular: 2 times daily with correction dose (based on pre meal glucose)

NPH: 2 times daily Regular: 2 times daily with fixed dose

If consistently checking BG at lunch* and there is hyperglycemia in the evening

Assumes 3 meals per day

NPH: 15 AM, 10 PM Glargine: 25 units daily

Glargine: 25 units daily Regular : 4 units 3 times daily Use 50/50 rule Glargine 50% total dose once daily Regular –50% of total dose –split into 2 or 3 doses

NPH: 15 AM, 10 PM Regular: 3 times daily before meals Correction dose based on chart Checking glucose before breakfast, lunch and dinner and giving an additional dose of insulin, based on scale on chart

per month)

Assumes 3 meals per day

1. Can understand numbers (what is a high, low, sugar) 2. Knows numbers on syringe 3. 60 strips per month

NPH: 15 AM, 10 PM Regular: 4 units 3 times daily Use 50/50 rule ** NPH –50% of total dose –split into half in morning; half in evening Regular –50% of total dose –split into three

NPH: 15 AM, 10 PM Regular: 2 times daily before meals Correction dose based on chart Checking glucose before breakfast and dinner and giving an additional dose of insulin, based on scale on chart

NPH: 15 AM, 10 PM Regular: 4 units 2 times daily Use rule of thirds 2/3 daily dose in morning (2/3 NPH; 1/3 regular) 1/3 daily dose in evening (2/3 NPH; 1/3 regular)

Reduced injections but maintains meal coverage

High risk of hypoglycemia if reduced meal size or inconsistent meals

Reduced injections

Preferred if glargine is available ***

Reduced risk of hypoglycaemia, can reduce hyperglycemia by accounting for pre-meal glucose

Simple, closer to physiologic insulin requirements

Reduced risk of hypoglycaemia, can reduce hyperglycemia by accounting for pre-meal glucose

Easy to start with

Consider during periods where focusing on diabetes may be a challenge (ie teenager) as long as no food insecurity

Will have persistent hyperglycemia from not giving insulin for carbohydrates consumed -May be more expensive

Assumes consuming THREE consistent meals with consistent carbohydrates May be more expensive Will have persistent hyperglycemia from not giving insulin for carbohydrates consumed

Consider during periods of food insecurity or short-term during periods where focusing on diabetes may be a challenge (ie teenager)

Best regimen if can afford

Requires glucose checking prior to meals Requires a greater number of test strips

Assumes consuming THREE consistent meals with consistent carbohydrates

Requires glucose checking prior to meals

Requires a greater number of test strips

Assumes eating at the same time each day and the same amount of carbohydrates

Challenging with clients who are at work or school during the day

Assumes consuming consistent meals with consistent carbohydrates

Consider if glucose is variable and patient has demonstrated competency with previous regimens Requires checking glucose before all meals AND basic numeracy (understanding/ reading numbers).

Consider if hyperglycemia in evening and is consistently checking glucose at lunch

1. Can understand numbers (what is a high, low, sugar) 2. Knows numbers on syringe 3. 90 strips per month *Using as a proxy to indicate that the patient is able to give an injection at lunch ** If moving from 2 times a day to 3 times a day, add short acting dose that most closely matches the meal they are having *** long-action means once a day injection will cover metabo lic needs for entire day. Preferred Network regimen. Move patients to a better regimen –even if A1C in range. Aim is to prevent hypoglycemia

Example

Mixed Insulin (70/30) 20 AM, 15 PM Minimum # test strips/

Pros

Cons

Patient considerations Reasonable starting regimen for most patients

3.3 How To Adjust Insulin Doses Based On Home Glucometer Readings

CHART OF INSULIN ADJUSTMENT BASED ON HOME GLUCOMETER READINGS

1. Does the client have any concerns or patterns with their blood glucose values they would like to talk about?

2a. Evaluate most recent 2-4 weeks of logbook or glucometer history for patterns of hypoglycemia

Address any hypoglycemia, even if infrequent, prior to addressing hyperglycemia

2b. Ask client about symptoms of hypoglycemia in the past 2-4 weeks and typical time of day they occur, including overnight (i.e convulsions, eating at night, night sweats)

present

2c. If hypoglycemia is infrequent, can client explain why it happened? (ie skipped a meal, had extra activity)

Pattern

AM glucose < 4.4 mmol/L (80 mg/dL)

Decrease PM NPH (Lente) insulin 10-20%

Counsel on hypoglycemia prevention

Assess for and address any food insecurity

Pre-Lunch glucose < 4.4 mmol/L (80 mg/dL) Decrease AM Regular (Actrapid) insulin 10-20%

Pre-Dinner glucose < 4.4 mmol/L (80 mg/dL) Decrease lunchtime Regular (Actrapid) or AM NPH (Lente) 10-20%

Bedtime glucose <?4.4 mmol/L (80 mg/dL) Decrease PM Regular (Actrapid) 10-20%

Overnight glucose < 5.6 mmol/L (100 mg/dL) Decrease PM NPH (Lente) 10-20% Patterns

3a. Evaluate most recent 2-4 weeks of logbook or glucometer history for patterns of hyperglycemia

3b. Ask client about symptoms of hyperglycemia in the past 2-4 weeks and typical times of day they occur, including overnight (i.e. nocturia)

Is the client missing or skipping injections?

How is the client storing insulin? Is there a chance the insulin went bad? (highs mostly at the end of the month, or exposure to heat)

Assess injections sites. Is there lipohypertrophy or infection?

Ask client to demonstrate how they draw up and administer insulin. Is it correct? Can they differentiate between both of their insulins? If they mix insulin, are they mixing correctly? (both vials cloudy means insulin not mixed correctly)

Any changes in diet or activity? Are these changes modifiable?

Has the client been sick recently? Any recent hormonal changes? (ie menstruation)

AM glucose > 10 mmol/L (180 mg/dL) Increase PM NPH (Lente) insulin 10-20% Pre-Lunch glucose > 10 mmol/L (180 mg/dL) Increase AM Regular (Actrapid) insulin 10-20%

Ask why? Work with client on plan to increase frequency

Assess for and address any food insecurity

Give new insulin

Educate about storage of insulin

Educate on rotating injection sites

Educate on proper administration of insulin

Educate on improving diet and physical activity

Address illness, consider temporary increase in insulin

Pre-Dinner glucose > 10 mmol/L (180 mg/dL) Add regular insulin at lunch (start with same dose as PM) or increase AM NPH (Lente) insulin 10-20%

Bedtime glucose > 10 mmol/L (180 mg/dL) Increase PM Regular (Actrapid) 10-20%

Overnight glucose > 10 mmol/L (180 mg/dL) If no symptoms of overnight hypoglycemia, increase PM NPH (Lente) 10-20%

When ever insulin dose is changed, explain to the patient why the dose was changed, so that they understand what the glucose numbers mean

3.4 Using Correction Scales (mmol/L)

Another option for patients to improve management of glucose is to use correction scales

These can be introduced after the patient is already used to taking fixed doses of insulin

It assumes that there are 2 or 3 test strips available each day. It requires the patient to have some knowledge of numbers. If using twice daily regimen – the glucose is checked before each meal (breakfast, dinner). The short acting dose for those meals is increased by adding the amount shown in the scale, based on the glucose result. If using three times daily regimen (basal bolus) – the glucose is checked before each meal (breakfast, lunch, dinner). The short acting dose for those meals is increased by adding the amount shown in the scale, based on the glucose result

One can not use the same correction scale for every patient. This is because every patient responds to insulin differently. You need to choose from 1 of 4 correction scales to give to your patient

You choose the scale by calculating the total daily dose of insulin given in 24 hours

E.g. add up the short acting and long acting insulin given in the day

Scale (mmol/L) based on Total daily dose (TDD) (do not need to adjust further for weight)

If TDD < 10 – no correction scale needed

Use this scale if Total daily dose of insulin is 10-19 Units per day (Correction factor 1:7)

If Pre meal glucose is.. Then add this amount to the usual insulin dose

Use this scale if Total daily dose of insulin is 20-29 Units per day (Correction factor 1:5)

Use this scale if Total daily dose of insulin is 30-49 Units per day (Correction factor 1:2.5) If Pre meal glucose is.. Then add this amount to the usual insulin dose

Use this scale if Total daily dose of insulin is > 50 Units per day (Correction factor 1:2)

3.5 Using Correction Scales (mg/dL)

Another option for patients to improve management of glucose is to use correction scales. These can be introduced after the patient is already used to taking fixed doses of insulin. It assumes that there are 2 or 3 test strips available each day. It requires the patient to have some knowledge of numbers. If using twice daily regimen – the glucose is checked before each meal (breakfast, dinner). The short acting dose for those meals is increased by adding the amount shown in the scale, based on the glucose result. If using three times daily regimen (basal bolus) – the glucose is checked before each meal (breakfast, lunch, dinner). The short acting dose for those meals is increased by adding the amount shown in the scale, based on the glucose result. One can not use the same correction scale for every patient. This is because every patient responds to insulin differently. You need to choose from 1 of 4 correction scales to give to your patient You choose the scale by calculating the total daily dose of insulin given in 24 hours E.g. add up the short acting and long acting insulin given in the day

Scale (mg/dL) based on Total daily dose (TDD) (do not need to adjust further for weight)

If TDD < 10 – no correction scale needed

Use this scale if Total daily dose of insulin is 10 - 19

If Pre meal glucose is.. Then add this amount to the usual insulin dose

No correction

Use this scale if Total daily dose of insulin is 20 - 29 Units per day (Correction factor 1: 75. Target 125-275)

If Pre meal glucose is.. Then add this amount to the usual insulin dose <275 No correction

Use this scale if Total daily dose of insulin is 30-49 Units per day (Correction factor 1:45 Target

Use this scale if Total daily dose of insulin is >50 Units per day (Correction factor 1: 30 Target 120-180)

If Pre meal glucose is.. Then add this amount to the usual insulin dose <180 No correction

3.6 Diabetic Ketoacidosis (DKA) Algorithm - Adults (> 15 years)

1. Emergency Room: Initial Management

1. Assess if in shock

2. Assess Level of consciousness (GCS)

3. Weight

4. Lab essential :Blood glucose; urine ketones; malaria, Hb;

5. Lab preferable : urea and electrolytes, FBC.

2. Correction of shock

1. Give O2

2. Two large IV cannula

3. If systolic BP<90 then give 500ml of Normal Saline (NS) over 10 to 15min. Repeat bolus until systolic BP>90. Most require 500 – 1000ml.

3. Correction of dehydration

1. Give 1l NS stat

2. Then give 1l NS over 1 hr

3. Then give 1l NS over 2 hrs

4. Then give 1l NS over 4hrs

5. Then remaining litres to be given within the first 24 hours to make total of 8 litres in first 24 hours

6. The average fluid deficit in an adult presenting with DKA is 5-10 litres

7. Reassess cardiovascular status at 12 hours; further fluid may be required. Check for fluid overload

8. Be more cautious in fluid replacement in young people aged 18-25 years, elderly, pregnant, heart or renal failure

4. Insulin

1. Start short-acting insulin at 0.1U/kg intravenous every 1 hour. This can be with infusion pump, or by bolus given every 1 hour.**

2. If unable to give insulin every 1 hour, use subcutaneous insulin 0,1U/kg every 2 hours.

3. Once the blood glucose is <14 mmol/l, (252 mg/dL) add dextrose to the saline (add 100 ml 50% dextrose to every litre of saline, or use 5% dextrose saline). If only D5W available. Then run NS and D5W on 2 separate IV lines. Do not only give D5W.

4. Once the blood glucose is <14 mmol/l, (252 mg/dL), consider decreasing insulin to 0.05U/kg.

5. Aim for a glucose reduction of 3 mmol/l per hour (90mg/dL).

6. Once glucose is < 9 mmol/L (162mg/dL), use 10% dextrose saline (add 50ml of 50% dextrose ampule to 500 ml of 5% dextrose saline).

7. If glucose is falling less than 3 mmol/l per hour, check that insulin is being given correctly, on time. Increase insulin by 1U per dose every hour until glucose drops by 3 mmol/l per hour.

8. **If severe DKA and infusion pump available – give shortacting insulin at 0.1U/kg every 1 hour. Mix 50 IU Regular insulin in 500ml NS. Infuse at 1 ml/kg/hr. (50 kg adult should receive 50 ml/ hr)

5. Potassium replacement

Potassium replacement is needed for every patient:

1. Check K level if possible. (If K < 3.5, delay insulin)

2. If no lab – do ECG. (Low K on ECGFlattening of the T wave, widening of the QT interval, and the appearance of U waves; High K - Tall, peaked, symmetrical, T waves and shortening of the QT interval)

3. If no lab – start replacing K after patient passes urine.

4. Add 20 mmol (10 mls of a 15% KCl) to each litre of fluid in first 4 hours (except the first litre/or boluses). Then 40 mmol to each litre after 4 hours.

5. Monitor K 6-hourly.

6. If IV potassium is not available, give oral slow K, one tab 6 hrly or fruit juice / bananas.

7. If no IV potassium, give insulin at a slower rate. (subcut 0,1U/kg 3 hourly or IV 0.05U/ kg per hour)

6. Treatment of infection

If infection is suspected (common), treat with broadspectrum antibiotics. E.g. ceftriaxone

7. Monitoring of management

1. Monitor hourly; heart rate, BP, respiratory rate,GCS, glucose

2. Monitor urine ketones in every sample of urine.

3. Record fluid intake, insulin therapy and urine out put. Catheterise if necessary. Minimum urine output ≥ 0.5 ml/kg/hr

4. Repeat electrolytes every 6 hours

5. If unconscious, consider naso-gastric tube to prevent aspiration

8. Transition to usual insulin regimen

1. Once awake, hungry, eating, drinking sips of water change to normal insulin regimen e.g twice or three times daily subcut insulin. Start at next meal. (If using IV insulin, overlap IV insulin infusion with first subcut dose by 30 min)

2. Do not use urine ketones to decide when to change, as they may stay in urine for 12-24 hours

3.7 Diabetic Ketoacidosis (DKA) Algorithm - Children (<

15 years)

1. Initial Emergency Management

1. Assess if in shock (cap refill, HR) / severity of dehydration (mucous membrane skin turgor) . If uncertain about this, assume 10% dehydration in significant DKA

2. Assess Level of consciousness (GCS)

3. Weight

4. Lab essential: blood glucose; urine ketones; malaria, Hb.

5. Lab preferable: urea and electrolytes, FBC.

2.

Correction of shock

1. Give O2

2. 2 x large IV cannula. (or use intra-osseous ).

3. Give repeat boluses of IV normal saline - 20ml/kguntil perfusion improves

4. Make sure that the child is adequately resuscitated –good perfusion - before proceeding to the following steps. Use cap refill and HR

3. Correction of dehydration

1. Rehydrate the child with normal saline(NS) (or Ringers Lactate RL). Use the table below to calculate fluid replacement to give over 48 hours.

2. Reassess clinical hydration regularly, using capillary refill, heart rate, mucous membranes

5. Potassium replacement

Potassium replacement is needed for every patient:

1. Check K level if possible. (If K < 3.5, delay insulin)

2. If no lab – do ECG. (Low K on ECG - Flattening of the T wave, widening of the QT interval, and the appearance of U waves; High K - Tall, peaked, symmetrical, T waves and shortening of the QT interval)

3. If no lab – start replacing K after patient passes urine.

4. Add 20 mmol (10 mls of a 15% KCl) to each litre of fluid in first 4 hours (except the first litre/or boluses). Then 40 mmol to each litre after 4 hours.

5. Monitor K 6-hourly.

6. If IV potassium is not available, give oral slow K, one tab 6 hrly or fruit juice / bananas.

7. If no IV potassium, give insulin at a slower rate. (subcut 0,1U/kg 3 hourly or IV 0.05U/kg per hour)

6. Treatment of infection

If infection is suspected (common),treat with broad-spectrum antibiotics. E.g. ceftriaxone

7. Monitoring of management

1. Monitor hourly; heart rate, BP, respiratory rate, GCS, glucose

2. Monitor urine ketones in every sample of urine.

3. Record fluid intake, insulin therapy and urine out put. Catheterise if necessary. Minimum urine output ≥ 0.5 ml/kg/hr

4. Repeat electrolytes every 6 hours

5. If unconscious, consider naso-gastric tube to prevent aspiration

8. Transition to usual insulin regimen

1. Start insulin after circulation stable - at least 1 hour after IV fluids

2. Give subcutaneous insulin 0.1U/kg every 2 hours. Eg for 30 kg child give 3U subcut every 2 hours *

3. In children < 5 yrs give lower rate of insulin (0.05 U/kg every 2 hours)

4. Once the blood glucose is <15 mmol/l, (270mg/dL) use 5% dextrose saline (add 100 ml 50% dextrose to 1 litre saline). If only D5W available. Then run NS and D5W on 2 separate IV lines. Do not only give D5W.

5. Aim for a glucose reduction of 5 mmol/l per hour (90mg/ dL). A more rapid decline may cause cerebral injury). If glucose drops rapidly, or once glucose is < 9 mmol/L (162mg/dL), use 10% dextrose saline (add 50ml of 50% dextrose ampule to 500 ml of 5% dextrose saline). If glucose continues to fall rapidly, decrease the rate of insulin delivery by 25% (e.g from 8U to 6U)

6. *If severe DKA and infusion pump available – give shortacting insulin at 0.1U/kg every 1 hour. Mix 50 IU Regular insulin in 500ml NS. Infuse at 1 ml/kg/hr. (14 kg child should receive 14 ml/ hr)

1. Once awake, hungry, eating, drinking sips of water, change to normal insulin regimen e.g twice or three times daily subcut insulin. Start at next meal. (If using IV insulin, overlap IV insulin infusion with first subcut dose by 30 min)

2. Do not use urine ketones to decide when to transition. as they may stay in urine for 12-24 hours

9. Cerebral injury

1. Rapidly rising intra-cranial pressure may cause a change in neurological state (restlessness, irritability, increased drowsiness or seizures),

2. If suspected, exclude hypoglycaemia

3. Reduce rate of fluid by one- third.

4. Give mannitol 0.5-1 g/kg IV over 20 min and repeat if no response in 30 min to 2 hours.

5. Elevate head of the bed.

6. Obtain cranial CT scan if possible

4. Insulin

SECTION 4 TOOLS FOR SICKLE CELL DISEASE

4.1 Screening For SCD Using Rapid Tests

HOW TO USE RAPID TESTS* FOR SCD DIAGNOSIS

*For PEN-Plus clinics this refers to SickleScan, HemotypeSC, or Gazelle

Any hallmark signs or complications?

< 35 years old

Anemia (Hb < 8.5)

If needs urgent transfusion –keep a sample in lab for later sickle cell testing

If transfused and no blood sample pre-transfusion available

If meets criteria for testing and/or high suspicion for SCD, enroll in care and start preventive therapy with folic acid, immunizations.

Proceed with rapid confirmatory test 3 months after transfusion.

Sickle cell screening test previously positive

Perform rapid confirmatory test if not yet done

If no sample pre-transfusion is available

History of multiple/recurrent transfusion

Physical signs: bossing of skull, dactylitis, AVN

History suggesting multiple pain crises

Family history of known SCD or sibling death

Anemia with jaundice (even if Malaria is positive)

Stroke in a child

Septic arthritis, cellulitis, osteomyelitis

Other OBVIOUS or confirmed reason for anemia ruled out*

*Including pregnancy, HIV if > 14 years

RAPID TEST FOR SICKLE CELL (Sickle SCAN, Hemotype SC, Gazelle) If already transfused- go to lab and test cross matching sample

Enroll in care, start hydroxyurea*, test all siblings.

*Pursue advanced confirmatory testing only if required by national guidelines. Note hydroxyurea can be started with the positive rapid test alone.

4.2 Sickle Cell Disease: Prevention Protocol

SICKLE CELL DISEASE: PREVENTION PROTOCOL

Vaccinations

Antibiotic

Prophylaxis

• Confirm all routine childhood vaccinations

• Infants: Pneumococcal Conjugated Vaccine (PCV 13) – (6,10,14 weeks)

• Children: Pneumococcal polysaccharide vaccine (PPSV-23) at 2 years and booster at 5 years apart (if available)

• All: pneumococcus, Hib, Hepatitis B, meningococcus (per country schedule / availability)

Oral penicillin

• Children < 3 years: 125 mg twice a day

• Children 3-5 years: 250 mg twice a day

• Penicillin allergy: erythromycin or clarithromycin

• Children > 5 years: discontinue if received the due pneumococcal vaccine. Do not stop if the child had severe pneumococcal infection, splenectomy, or functional asplenia

IM Benzathine monthly if oral penicillin not available

Malaria

Prophylaxis

Folic Acid

Mebendazole

Growth Monitoring

Counseling

• Insecticide-treated bed nets; add indoor residual spraying where available

• Prompt diagnosis and treatment

• Malaria prophylaxis highly recommended if permitted per country guidelines

• 5 mg once a day

• 1-2 years: 200mg every 3 months

• >2 years: 400mg every 3 months

• Infants: Length in supine position (0-24 month); Head circumference (0-36 month)

• 1-5 years: mid-upper arm circumference (MUAC)

• Children and Adolescents: Weight (every visit), Height (annually)

• Seek immediate medical attention for fever (101F, 38C), difficulty in breathing, chest pain, abdominal swelling, sudden weakness, loss of feeling and movement, seizure, painful erection of penis, sudden loss of vision, uncontrollable pain with medication and other signs of dehydration

• Teach families to own and use a thermometer

• Basic hygiene: Handwashing with soap after use of the toilet, changing of infants and toddlers, and before eating

Screening (consider referral if unavailable at facility)

• Eyes: annual fundoscopic examination starting at age 10 years

• Kidneys: annual creatinine & electrolytes. Add annual proteinuria starting at age 10 years

• Cardiac: >18 years echocardiogram every 3-5 years

• Stroke: please see transcranial doppler specific considerations

PROTOCOL FOR SICKLE CELL DISEASE

HYDROXYUREA (HU) TREATMENT

Indications: All patients ≥ 9 months with confirmed sickle cell disease (SS, S/Beta-thalassemia)

Precautions:

2 Initiation of hydroxyurea:

Discontinue

3 months prior to conception in both males and females

Breast milk should be discarded for first 3 hours of taking hydroxyurea

For age > 14 years: Starting dose 15 / mg / kg / day

Infants & Children: Starting dose 20 mg / kg / day (age < 14 years) Weigh child every visit and adjust dose!

Contraindications to initiation: Absolute Neutrophil Count (ANC) <1,500/ Platelet < 100 000/ Hb<5 g/dL Pregnancy

Essential Investigations 1. Full Blood Count (FBC)* 2. Pregnancy Test Recommended Investig ations: 3. Creatinine** and ALT/SPGT

For 500 mg capsules, calculate weekly dose in number of capsules per week and distribute the required capsules throughout the week

Every 6 months (recommended): Creatinine, Urea, SGPT/ALT

Every visit: Essential: Hemoglobin Recommended: FBC

*If only Hb is available –and not FBC –initiate at 10 mg/kg/day

**Chronic kidney disease: Initiate at 5–10 mg/kg/day

Every visit: Monitor for clinical response and side effects, check weight Assess symptoms and medication adherence

Side Effects:

Low granulocyte/neutrophil count (AGC/ANC), Hb, platelets

Mouth ulceration

Skin toxicity: rashes

Note: Clinical response may take 3–6 months

NOT good clinical response: Consider dose escalation

If side effects persist, Do NOT restart hydroxyurea Consult mentor or refer to specialist

Repeat abnormal lab every 2 weeks

4.4 Hydroxyurea Dose Chart For Use In Sickle Cell Disease

Pediatrics. Pediatric dose (9 months to 14 years) – start with 20 mg/kg/day.

*for weight 6 to 8 kg

• If pharmacy is able to safely compound hydroxyurea into syrup, then dispense as per pharmacist instruction

• If pharmacy is not able to safely compound hydroxyurea into syrup, then mother should take one 500 mg capsule each day and dissolve into 10 ml water (give her 10 ml syringe). She should draw 5 ml to give to the baby on alternate days, and throw 5 ml away. (The solution should not be kept for more than 24 hours).

bands

Adults. Adult dose (>14 years) – start with 15 mg/kg/day.

* For adults above 40 kg, consider starting hydroxyurea at 500 mg daily, checking blood results after 2 weeks and then increasing to the dose in this table.

4.5 Blood Transfusion (Emergency Simple)

INDICATIONS FOR EMERGENCY TOP-UP TRANSFUSION:

Pre-Investigations: baseline blood tests

• Full Blood Count (FBC)

• Cross mat ch*

* Remember to ask the lab to save a sample if the patient is not confirmed SCD and needs testing to confirm the diagnosis

Transfusion:

Dose for transfusion (10-15 mls/kg of Packed Red Blood cells or 20 mls/kg of whole blood)

Monitoring BLOOD TRANSFUSION PROTOCOL FOR SICKLE CELL (EMERGENCY / SIMPLE TOP-UP TRANSFUSION)

Post-transfusion

3.6 Pain Management Protocol

Reassurance, hot packs, reposition, massage, distraction (stories, play)

Child: Paracetamol 15mg/kg PO 6 hourly

Adult: Paracetamol 1g PO 6 hourly. Max dose 4g daily.

Treat as mild pain and ADD

Child: Ibuprofen 10mg/kg PO 8 hrly OR Diclofenac 1mg/kg PO 8 hrly

Adult: Ibuprofen 600 mg PO 6 hrly OR Diclofenac 100 mg stat, then 50 mg 8 hrly

If in moderate pain add IV/PO tramadol 50 – 100 mg 6 hrly

Treat as moderate pain and ADD

Infant: Morphine 0.1mg/kg PO 4 hrly

Child: Morphine 0.2-0.5mg/kg PO 4 hrly

Adult: Morphine 10-20mg PO 4 hrly

If in severe pain consider adding IV/ subcut morphine

Adult: 2.5mg – 5mg IV / subcut prn.

Child: 0.1mg/kg IV / subcut prn.

4.7 Acute Complications Protocol

HISTORY

Recurrent acute pain events

SIGNS & SYMPTOMS: Fatigue, pallor, shortness of breath, hepatomegaly+/- splenomegaly, chest pain, limb pain, stroke presentation, jaundice

INITIAL ASSESSMENT:

MANAGEMENT:

Treat the following 4 Key areas:

• Maintain airway

• Give oxygen if sPO2 <95%

• Gain IV access & send FBC, X-match, U&ES and malaria RDT/blood film

• Measure vital signs (including BP, HR, RR, Temp, glucose and oxygen)

1. Rehydration is vital: Give 1 litre IV fluid Normal Saline/Ringer’s lactate over 1 hour (rate depends on hydration status/if hypotensive give rapidly). Encourage oral fluids.

2. Analgesia within 30 mins: For all patients in pain: Assess the pain

Step 1: Mild pain. Child: Paracetamol 15mg/kg PO 6 hourly. Adult: Paracetamol 1g PO 6 hourly. Max dose 4g daily.

Step 2: Moderate pain. In addition to step 1- Child: Ibuprofen 10mg/kg PO 8 hrly OR Diclofenac 1mg/kg PO 8 hrly. Adult: Ibuprofen 600 mg PO 6 hrly OR Diclofenac 100 mg stat, then 50 mg 8 hrly. If in moderate pain add IV/PO tramadol 50 – 100 mg 6 hrly

Step 3: Severe pain in addition to step 1+2. Infant: Morphine 0.1mg/kg PO 4 hrly. Child: Morphine 0.2-0.5mg/kg PO 4 hrly. Adult: Morphine 10-20mg PO 4 hrly

If in severe pain consider adding IV/ subcut morphine. Adult: 2.5mg – 5mg IV / subcut prn. Child: 0.1mg/kg IV / subcut prn.

3. Treat infection: If fever present you must exclude infection (pneumonia/UTI/malaria etc.) as infection common precipitator to an acute crisis (give broad spectrum antibiotic e.g. ceftriaxone / amoxicillin-clavulanic acid)

4. Transfuse: If patient symptomatic and Hb < 5 or drop ≥ 2g/dl below steady state g TRANSFUSE. If patient stable/no symptoms with Hb > 5 - monitor closely. Dose for transfusion (10-15 mls/kg of Packed Red Blood cells or 20 mls/kg of whole blood)

OBSERVE FOR THE FOLLOWING COMPLICATIONS

Acute chest syndrome

(SPO2<95% + Symptoms of respiratory distress, hypoxia, chest pain, new infiltrates on CXR)

• Maintain SPO2 >95%

• IV hydration –monitor for fluid overload

• Ceftriaxone 1-2g od + erythromycin 500mg 6 hrly FOR 7-10 days

• Transfuse to target Hb of 10g/dL (in order to dilute HbS not to treat anemia)

• If wheezing give salbutamol 2.5mg nebs QDS

• This is life threatening- Admit in a high dependency care unit/ICU

Acute severe haemolysis or Acute Severe Anemia

(Hb< 5g/dl, drop of Hb 2g/ dl below the steady state, acute symptomatic)

• Transfuse until Hb >5g/ dL (target Hb 7-8 g/dL) 10-15 mls/kg of packed red blood cells or 20 mls/kg of whole blood

• Check for fluid overload between transfusions

• Treat infections e.g. malaria, UTI if present

• Repeat Hb the following day

Acute stroke

(sudden hemiparesis/ paralysis, unilateral numbness, ataxia, speech abnormalities, seizure)

• Treatment of seizures

• Transfuse to target Hb of 10g/dL (in order to dilute HbS not to treat anemia)

• IV hydration

• Upon discharge, these patients are high risk for future strokes and can benefit from chronic regular transfusion therapy or hydroxyurea

Priapism: prolonged painful erection unrelated to sexual intercourse

Mgt <4hrs: analgesia, anxiolytic agents if indicates, increase fluid intake, encourage micturition, warm bath and walk

Mgt > 4hrs: Surgical intervention, IV hydration, analgesics and anxiolytics, Catheterize if unable to micturate, consult urologist/surgeon OR refer the patient

Acute Splenic sequestration (sudden enlargement of spleen, life threatening)

• Immediately transfuse 5 -7.5ml/kg (50% of what we commonly transfuse) over 3-4hrs

• Give 20ml/kg normal saline bolus while awaiting blood, repeat bolus if needed

• Monitor vital signs (PR, BP, RR, temp, oxygen sat.) at least every 30 mins

• Monitor spleen size hourly for 12-24 hours, until reducing in size

• Check Hb 2 hours posttransfusion, consider repeat transfusion

• Treat underlying cause, where applicable

• Therapeutic splenectomy if >2 episodes of sequestration

SECTION 5 TOOLS FOR RESPIRATORY DISEASE

5.1 Asthma Out-patient Management Chart

ASTHMA: MEDICATION MANAGEMENT

Start at step 2

Well-controlled: consider stepping down after 3 months of stability

Partly controlled: re-assess non-pharmacological management and inhaler use; consider stepping up

Uncontrolled: step up 1-2 steps

Additional notes on asthma

• All patients with asthma should be on steroid inhalers - never salbutamol (SABA) only.

• Check inhaler technique each visit. Use spacers when needed.

• If available – use a combination inhaler of formoterol and corticosteroids (LABA and ICS). Never use formoterol-only inhaler in asthma due to increase in mortality.

• If atopy (rhinitis, eczema, allergies) add antihistamine tabs.

• Avoid salbutamol and theophylline tablets in asthma.

• If no response – obtain chest x-ray to consider alternate diagnosis.

• Teach patients with higher literacy to adjust their own asthma treatment using a written action plan.

• Self increase dose of inhaled steroids if Control is worse – based on 4 questions

Peak flow meter at home, done daily at home, worsens by 20%

• Come to hospital if no improvement after increasing step of steroid inhaler

5.2 Asthma Exacerbation Management Chart

ASTHMA EXACERBATION MANAGEMENT CHART

Step 1: Identify Exacerbation

RR>24

Wheezing

Difficulty breathing

Persistent coughing

Hypoxic?

Accessory muscle use

SpO2<90%

Difficulty speaking

Chest tightness

Manage as outpatient

Respiratory distress? Altered mental state?

Step 2: Manage exacerbation

Refer to Emergency Department *Consider MgSO4 2g IV over 10 min

Salbutamol inhaler: 4-10 puffs every 20 minutes x3 (use spacer if needed)

Give prednisone PO 40-60mg immediately

If no improvement, refer to Emergency

Start prednisone 40-60mg PO OD x5-7 days if persistent symptoms

Continue Salbutamol inhaler 1-2 puffs every 1-3 hours as needed

Identify trigger

Re-assess

5.3 COPD Out-patient Management Chart

COPD: MEDICATION MANAGEMENT

Severity

Group E Lots of exacerbations

Group B Few exacerbations; severe dyspnoea

Group A Few exacerbations; more dyspnoea

(Ask: Do others stop and wait for you on a level ground?)

(MMRC 2-4)

(MMRC 0-1) ≥

Not troubled by breathlessness except on strenuous exercise

Shortness of breath when hurrying on the level or walking up a slight hill

Walks slower than the people of the same age on the level because of breathlessness or has to stop when walking at own pace on the level

Stops for breath after walking about 100 m or after a few minutes on the level

Too breathless to leave the house or breathless when dressing or undressing

Long acting B agonist e.g. formoterol AND Long acting anti muscarinic AND inhaled corticosteroid if exacerbations persist

Long acting B agonist e.g. formoterol AND Long acting anti muscarinic

Short acting B agonist as needed e.g. salbutamol inhaler or short acting anti-cholinergic as needed e.g. ipratropium inhaler

If uncontrolled on maximum dose inhalers: Reinforce non-pharmacologic management

Ensure proper inhaler technique or use spacer Consider other diagnoses (TB,Cardiac cancer)

*Consider theophylline tab in Group E. Max dose 200 mg/day. Check creat every 12m. Be aware toxicity

5.4 COPD Exacerbation Management Chart

COPD EXACERBATION MANAGEMENT CHART

Step 1: Identify Exacerbation

Manage as outpatient

Acute Hypoxia (new SpO2<88%)? Respiratory distress?

Step 2: Manage exacerbation

Refer to Emergency Department *Consider MgSO4 2g IV over 10 min

Salbutamol inhaler: 2 puffs every hour x3, then every 2-4 hours as needed

Ipratropium inhaler: 2 puffs every 4-6 hours

Prednisone 40mg PO OD x5 days

Amoxicillin 1g PO TDS x 7 days AND Azithromycin 500mg PO OD x3 days IF increased cough AND increased sputum production or fever

Obtain Chest X ray after 2nd exacerbation – to consider alternate diagnosis

5.5 How To Make A Bottle Spacer

To modify a 500ml plastic bottle for use as an effective spacer:

1. Wash the bottle with soap and water and air dry for a minimum of 12 hours to reduce electrostatic charge on the interior plastic.

2. Make a wire mould similar in size and shape to the mouthpiece of the inhaler.

3. Heat the mould and hold in position on the outside of the base of the plastic bottle until the plastic begins to melt (~10 seconds). Rotate the mould 180% and reapply to the bottle until the mould melts through to make a hole.

4. While the bottle is still warm, insert the inhaler into the hole to ensure a tight fit between the inhaler and bottle spacer.

New bottle spacers should be primed initially with 10 puffs of the medicine to reduce electrostatic charge on the walls, which attracts small particles, and thus make more aerosol medicine available for inhalation.

A wire mold is used to melt a hole in the base of the 500 ml bottle, to fit an inhaler

How to use a bottle spacer

Correct use of a modified 500 ml plastic cold-drink bottle as a spacer

Use a modified 500ml plastic bottle in a similar way to a conventional spacer

1. Insert the inhaler into the hole at the base of the bottle spacer.

2. Hold the neck of the bottle spacer in the child’s mouth. For a young child who cannot form a tight seal with the spacer in their mouth, a small commercially available facemask that fits on the open end of the bottle can be applied. Do not use a cup or cut the side of the bottle spacer.

3. Give the child a single puff of the inhaler with the spacer, followed by normal breathing, and repeat until the desired amount of medicine is given, which is frequently 2 puffs, but may be up to 6 puffs for relief of bronchoconstriction.

Reference:

The Global Asthma Report 2022. http://globalasthmareport.org/management/spacers.php. Zar HJ, Motala C, Weinberg E. Incorrect use of a homemade spacer for treatment of recurrentwheezing in children--a cause for concern. S Afr Med J. 2005 Jun;95(6):388, 390.

PEN-Plus Clinical Tools and Programmatic Standards