Secondary prevention of acute rheumatic fever and rheumatic heart disease Dr Anna Ralph Clinical Researcher, Menzies School of Health Research Infectious Diseases Specialist, Royal Darwin Hospital

Learning objectives What is secondary prevention of ARF/RHD? Understand the tools of secondary prevention: their use and limitations Recognise the role of a register-based program in secondary prevention Understand the role of primary care facilities Understand the practical challenges of secondary prevention Know where to find relevant information

What is secondary prophylaxis?

Tertiary Secondary Primary Primordial Prevention of further ARF episodes in people who have already had ARF &/or have already developed RHD using an antibiotic to prevent streptococcal infection for minimum of 10 years Tertiary Management of existing RHD Secondary Prevent ARF recurrences after 1st episode Primary Treat GAS infection Primordial Target the socio-ecomonic determinants

What is secondary prophylaxis? Standard regimen: benzathine penicillin G (BPG; Bicillin) injections every 28 days, for a minimum of 10 years For people with penicillin allergy Clarify whether allergy truly present: only 10%-20% of patients reporting a history of penicillin allergy are truly allergic when assessed by skin testing For true allergy, use oral erythromycin 250mg bd Successful secondary prevention requires the support of a register-based program working within a well-functioning primary health system

Antibiotic regimens Agent Dose Route Frequency First line Benzathine penicillin G (BPG / LAB / ‘Bicillin’) 900 mg (>20kgs) =1,200,000 units 450 mg (<20kgs) = 600,000 units Deep IM injection 4 weekly (or 3 weekly for selected individuals) Second line – only in exceptional circumstances; much less effective than 1st line Phenoxymethylpenicillin (Penicillin V) 250 mg oral Twice daily Following documented penicillin allergy Erythromycin Oral Australian Guideline 2012

How does secondary prophylaxis with penicillin work? Reduces the risk of infection with Group A Strep Reduces the change of an ARF recurrence Reduces chance of progression to RHD, or worsening of existing RHD Helps prevent hospitalisation and death from RHD

Benefits Challenges Short term: Long term: Very onerous Reduces risk of getting ARF, which can cause very painful, temporarily disabling arthritis, or chorea, which can be awkward and embarrassing Long term: Reduces the risk of progression to serious cardiac disease, potential need for valve surgery and warfarin, and further risks (stroke, endocarditis, premature death) Challenges Very onerous monthly clinic contact for a painful needle- hard to commit to being in the right place, or having access to health care, every 28 days (what about school excursions, family holidays, going out bush, unexpected funerals to attend?) Complications from IMI especially if needle administered poorly e.g. haematoma, abscess, myositis Potential for adverse penicillin reaction Potential for other organisms to become penicillin resistant Absolutely no doubt that pros outweigh cons, but need to acknowledge the cons, and make sure patients know you appreciate how challenging it is for them to adhere long term

Breakthrough ARF cases can occur despite regular bicillin Uncommon, but rare breakthroughs do occur despite regular injections, with examples seen in NT children in 2014. May be because needle was several days late May be because their penicillin levels were not high enough and they had heavy exposure to group A Strep These individuals are then prescribed 3-weekly injections, and need extra emphasis on primordial / primary measures Currently in NT, 19 of 1371 people (1.4%) are prescribed 3-weekly injections

penicillin concentration in blood Potential risk of acquiring Concentration of penicillin in bloodstream over time, after a single injection 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 days penicillin concentration in blood Potential risk of acquiring new strep infection when penicillin levels are low Definite risk of acquiring new strep infection if next dose not given on time

Dosing schedule Start recalling patient for next dose to be given on or before day 28 Next dose can be given on any of these days

Who needs it and for how long?

Secondary prophylaxis is usually required for many years to protect against streptococcal infections. ARF only For 10 years after last ARF or until age 21 years (whichever is longer) Moderate RHD For 10 years after last ARF or until age 35 years (whichever is longer) Severe RHD Until age 40 or longer After heart valve surgery

How is adherence measured?

Master chart 1. Proportion of scheduled needles received 1 2 3 4 5 6 7 8 9 10 11 12 13 Samuel Tracey Susan Brooklyn Daniel Martha P

2. Days at risk days Peak in ASOT Peak in antiDNAseB GAS exposure GAS Joint/ carditis ARF Chorea ARF 2-3 weeks 6-9 weeks penicillin concentration in blood days at risk days

How successful is secondary prophylaxis delivery? Every needle given is an important opportunity to prevent ARF Most patients struggle to achieve full adherence On average, people get about 60% of the required injections yearly Only 20% of people receive over 80% of their injections Each year 30% of ARF episodes are recurrences ANY late or missed injection leaves the window open for a recurrence Health staff, people on the program, their families and the community need to understand the message: “Don’t be late. Never miss a jab! ” NT RHD Prevention Program 2011

Barriers to adherence: clinic factors Lack of streamlined triaging i.e. long waiting time Failure to provide opportunistic bicillin Right nurse not present Poor relationship with clinic; a need to feel nurtured by the clinic (shared responsibility) Clinic staff may not have explained to patient that when travelling to different communities, needle can be administered by any clinic Mixed messages from different health staff, especially new staff not familiar with ARF

Barriers to adherence: patient factors Chronic diseases in childhood through adolescence to adulthood often pose major adherence challenges. Specific to ARF in Australia: Patient expectations of health care may differ from what is offered by traditional western medical model The treatment is a painful needle Patient populations may be mobile Health literacy in affected groups may be low Healthcare staff turnover rates are exceptionally high in remote settings, and their knowledge of ARF is often poor Parents may be unwilling to subject their child to the treatment, especially if benefits not made clear Patients may not wish to acknowledge the presence of a chronic disease, especially in adolescence (stigma, shame)

Reducing the pain of IM BPG Use a 21 gauge needle Warm the syringe – at least to room temperature Use the upper outer quadrant of the buttock Prior to inserting the needle, apply firm pressure with thumb at the injection site for 10 seconds If using an alcohol swab, ensure the skin has dried Give the injection slowly (over 2-3 minutes) Provide distraction

What are some creative solutions primary health care staff can use to help adherence? Your needle is due this week  0402 433 588

Helpful strategies Health systems improvements in high burden areas, make sure the clinic has someone who takes responsibility for RHD coordination, and that turnover at least in that position is minimised Identify and fix the barriers Reduce problems related to getting needles – pain, waiting time, communicating with clinics at communities where the patient travels to Educate and support staff Communicate with the control program and RHD Australia Use the educational / training resources which are available Support patients Self-management support Improve health knowledge in a culturally competent way

What is the role of the register?

Objectives of register-based prevention program 1. Ensure success of 2ry prophylaxis by Providing lists of people for secondary prophylaxis Identifying when secondary prophylaxis is not being delivered and feeding information back to clinic 2. Facilitate coordination of ongoing care by Generating regular reports to enable recall and review Ensuring that patients are not lost to follow-up Facilitating health education 3. Provide epidemiological data: To monitor ARF/RHD incidence / prevalence For program evaluation

Where can you seek information?

Resources RHD Australia Training modules http://rhdatest.docebosaas.com National Guideline and RHD Australia patient and staff educational materials http://www.rhdaustralia.org.au/resources

Take home message Secondary prevention of ARF/RHD is complicated and expensive, but it is the most cost-effective approach until successes in primordial prevention are achieved. Secondary prevention comprises regular painful injections over many years, mostly to asymptomatic adolescents and young adults. Many challenges. Ongoing education of health staff and people (children, adolescents and adults) in prevention programs, their families and the wider community is critical to the success of secondary prevention. Prevent AT RISK days. “Don’t be late. Never miss a jab!”

Learning objectives What is secondary prevention of ARF/RHD? Define and understand the principles Understand the tools of secondary prevention: their use and limitations Recognise the role of a register-based program in secondary prevention Understand the role of primary care facilities Understand the practical challenges of secondary prevention Know where to find relevant information