Caring for children with acute rheumatic fever and rheumatic heart disease

By Melanie Irwin, Primary Times chief writer 

Source: APNA Primary Times Winter 2023 (Volume 23 Issue 1)

 

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Erin Ferguson runs the Happy Heart Clinic in far north Queensland, where she cares for children with acute rheumatic fever and rheumatic heart disease, a condition that is 124 times more common in First Nations peoples than in the general population. Erin and her team are working hard to raise awareness about the condition in high-risk communities. She says that primary health care teams have a vital role to play in the prevention and eradication of this insidious illness. 

Erin Ferguson first encountered acute rheumatic fever (ARF) and rheumatic heart disease (RHD) when she was working as a nurse on Thursday Island in the Torres Strait. Kids would be admitted to the general ward with a fever and joint pain and her job was to administer the long-acting Penicillin injections (LA Bicillin). 

Those injections were the treatment used to prevent and manage group A Streptococcus (group A Strep) infections. Group A Strep is the bacteria that leads to ARF, an immune response which can occur following multiple exposures to the germ. That acute response can damage the heart valves, which is RHD. Complications of RHD can include heart failure, arrythmia, and stroke. It can also be fatal. Many patients require open heart surgery.^1,2 

First Nations peoples are 124 times more likely to suffer from RHD than non-Indigenous people. In Australia, the condition takes the lives of two Aboriginal and/or Torres Strait Islander people each week. Appallingly, the rates of diagnosis are increasing in northern parts of Queensland.² 

Erin remembers thinking ‘What is this illness?!’ Working in Brisbane and Dublin she’d never seen it before – because in higher-income, well-resourced urban settings the condition has been eliminated. 

All Aboriginal and Torres Strait Islander people living in rural and remote settings are at high risk of developing acute rheumatic fever. 

These days Erin is a paediatric RHD clinical nurse consultant, based at Cairns Hospital and running the Happy Heart Clinic, a flexible clinical-care model that puts patient first. She works closely with paediatric cardiologist Dr Ben Reeves, coordinating care and education for close to 400 patients and their families in Cairns and across 13 outreach sites. 

Erin’s goal is for all children to receive multidisciplinary coordinated care, regardless of where they live. It’s a huge challenge. The catchment they serve takes in the area around Cairns and runs north to Cape York and the Torres Strait Islands – and it has higher rates of paediatric ARF and RHD than the rest of Queensland combined. 

Every community across that vast area is different. A big part of Erin’s role is raising awareness in a way that empowers people to come up with solutions and respects everyone’s unique circumstances and culture. Erin listens to patients and their families, asking what is and isn’t working for them with injections and their medical care in general. She also collaborates with staff in the clinics she visits to make sure clinics are a place where families want to come for care, so that kids have as much continuity of care as possible. 

That continuity is critical. Children with ARF must receive an intramuscular injection of the antibiotic long-acting Penicillin every 21–28 days for at least 10 years. The medication prevents recurrence of ARF, which prevents RHD from developing. The injection is very painful, though, and – understandably – it can be traumatic for the child and their family.  

‘The injections are awful’, Erin says. The medication is extremely viscous and must be injected slowly through a large needle over 2 minutes, deep into the muscle. Patients can feel the substance expanding in the muscle, and pain can persist for 24 hours after the injection.  

The children referred to the Happy Heart Clinic are those who have had trouble with adherence to treatment. Some have been traumatised by being physically restrained during previous injections in other settings. Erin is devoted to giving patients a good experience because she knows it will make it more likely that kids and their families will come back for the next injection. She explains that one negative injection experience is enough to throw children off track with their treatment. 

The model is focused on giving patients options, which allows them a sense of agency in the process. Children also choose their injection site and what pain-control method and distraction techniques they want to use. For pain control, many children choose Entonox, which is a mixture of 50% nitrous oxide and 50% oxygen. According to Erin, it’s been a real game-changer. ‘It helps with the pain control, and also with anxiety’, she says. ‘We also use Buzzy, a vibrating bee-shaped device with ice-pack wings. It’s placed on the patient’s skin above the injection site and the cold vibration distracts the brain’s attention away from the pain.’ 

‘I spend time asking the children about their experience with the injections. Many explain that they don’t like being forced to have the injection, and that they don’t understand why they must have it. In contrast, children who are on track with their injections will explain that it is because the medical staff have been nice and have spent time with them.’ 

To help keep up the continuity of care, Erin and Ben have developed the ‘Butt Card’, a medication game plan that kids can take with them to appointments. The card shows each child’s preferences for how they receive their injections and increases their confidence when meeting new clinicians. It also reminds clinicians about the importance of asking about the child’s preferences. 

Another way to build trust that Erin initiated is a video narrated by a 10-year-old patient, Jalil. In the video, Jalil chats with Erin, chooses his injection site and the pain-control options, and explains the injection experience to the viewer. He encourages the kids who are watching the video: ‘If you have rheumatic fever or rheumatic heart disease, remember, if you keep getting your needle, it will keep your heart strong and healthy!’³ 

‘I show the video to the new patients, and I can tell that they really relate to it,’ Erin says. ‘They’re reassured to know that they’re not the only one who has to have these needles.’ 

 

Eradicating ARF and RHD 

The eradication of RHD from Indigenous communities requires a whole-of-government response to the social determinants of the disease. Healthy housing and sanitation, reduced household crowding, cultural safety, good education and employment, and access to health services are all known to reduce rates of ARF and RHD in a community.⁴ Unfortunately, some communities have such a high prevalence of group A Strep that it’s difficult to avoid, even if living standards are relatively high. 

Until remote and rural Indigenous communities have social outcomes on a par with other Australians, Indigenous kids are at risk of developing ARF and RHD, and this reduces their potential to live a full and healthy life. Equity is the real issue, of course, but the prevention of this condition would also save the country millions of dollars: open-heart surgeries and other medical care for RHD currently cost our health system $27 million each year.² Cultural sensitivity is also key for health care providers working with First Nations communities. It’s important that communities are empowered to come up with their own solutions. ‘We need to ask, “What would work for your community?”’ Erin says. 

Despite the challenges, Erin is optimistic about the future. ‘Awareness is growing’, she says. ‘Because the illness is so prevalent in some communities, people know about it and they’re trying harder to avoid it. We’re a small team in Cairns looking after this massive cohort of patients, and it can be overwhelming. It’s so frustrating because this is entirely preventable. We need to keep pushing for more government funding and a more sophisticated multidisciplinary approach to eradicate this illness. In the meantime, my goal is to improve the outcomes for these kids in any way that I can, and to keep their hearts happy.’ 

Left to right: Dr Ben Reeves, Happy Heart Clinic patient Jalil Bullio, Jalil’s mum Lynette Bullio and clinical nurse consultant Erin Ferguson. Erin and Jalil created a video that encourages children with ARF to keep up with their injections. 

 

What can primary health care nurses do to help prevent ARF and RHD? 

Primary health care teams have a vital role to play in the fight against ARF and RHD.⁵ Prevention of group A Strep infection is the best place to start. Nurses in general practice, school nurses, maternal and child health nurses and community nurses can all help to raise awareness.  

‘Nurses and health workers can be the leaders of change’, Erin says. ‘We all need to inform communities about the risks, especially in high-risk parts of Australia across the Top End. Parents, guardians, teachers and other community members need to know that sore throats and skin infections should be treated straight away, rather than left to get better on their own. And daily hygiene habits make all the difference: handwashing, bathing, and swimming in the local chlorinated pool can prevent infection. Cuts, scratches and insect bites should be covered with a water-tight dressing. Even pregnant women should be informed about the risks so that they can recognise any sign of infection once they’ve had their baby. We also need to inform our colleagues about the condition, especially if they’ve moved to northern Australia from elsewhere. They may not have come across it before.’ 

‘My goal is to improve the outcomes for these kids in any way that I can, and to keep their hearts happy.’— Paediatric RHD clinical nurse consultant Erin Ferguson 

If group A Strep infection is identified, it needs to be treated immediately before it has a chance to develop into ARF. Symptoms of throat infection include a sore throat, difficulty swallowing, and feeling hot. Symptoms of bacterial skin infection are ‘inflamed blisters that pop, weep and form crusts.^’6 This kind of infection takes hold through breaks in the skin caused by scabies, fungal skin infections and insect bites.⁷ 

People at high risk should be given antibiotics immediately when they present with a sore throat or when a skin swab has been taken. Don’t wait for confirmation of group A Strep infection from a lab. This can take days, which gives ARF time to take hold. 

All Aboriginal and Torres Strait Islander people living in rural and remote settings are at high risk of developing acute rheumatic fever. Always consider ARF as a possible diagnosis, especially in high-risk patients. The symptoms of ARF include: 

• fever 
• painful and tender joints and limited range of movement 
• rash 
• shortness of breath 
• tiredness 
• jerky body movements. 

According to RHDAustralia’s guidelines, the symptoms can be diverse, subtle and evolve over time.⁸ If you suspect that someone has ARF, they need clinical work up, including ECG, blood tests (ESR, CRP, ASOT and antiDNase B), and referral for ECHO. 

As mentioned above, the social determinants of ARF and RHD need a whole-of-government approach. However, it’s important that primary health care nurses know the nine healthy living practices identified by RHDAustralia,^4,8 all of which reduce the risk of infections, ARF and RHD. Sharing this information can be a good way to prevent infection and to create the change needed to eradicate this condition from high-risk communities. 

 

For more information on ARF and RHD, see: 

The ARF guidelines published by RHDAustralia: https://www.rhdaustralia.org.au/arf-rhd-guidelines 

RHDAustralia has also developed clinical support apps for iOS and Android, which you can download at https://www.rhdaustralia.org.au/apps 

They also have a 45-minute program for medical practitioners to help them diagnose ARF: https://www.rhdaustralia.org.au/recognising-arf 

 

References 

1 S Noonan, What is rheumatic heart disease? RHDAustralia website, 2021, accessed 10 October 2022. www.rhdaustralia.org.au 

2 Queensland Health, Ending rheumatic heart disease: Queensland First Nations strategy 2021-2024, Queensland Health, Brisbane, 2021. 

3 RHDAustralia, ‘A patient's guide to getting a Bicillin injection’ [video], RHDGuidelines, YouTube, 11 October 2021, accessed 5 May 2023. https://youtu.be/ZPhoI9qnu-s 

4 RHDAustralia, Primordial prevention and social determinants of health, RHDAustralia website, 15 December 2022, accessed 5 May 2023. www.rhdaustralia.org.au 

5 R Wyber, AC Bowen, AP Ralph and D Peiris, 'Primary prevention of acute rheumatic fever', Aust J Gen Pract, 2021, 50(5):265–269, doi:10.31128/AJGP-02-21-5852. 

6 Department of Health, State Government of Victoria, Impetigo: school sores, Better Health website, accessed 5 May 2023. www.betterhealth.vic.gov.au 

7 Australian Healthy Skin Consortium, National Healthy Skin Guideline for the Prevention, Treatment and Public Health Control of Impetigo, Scabies, Crusted Scabies and Tinea for Indigenous Populations and Communities in Australia, Telethon Kids Institute, Perth, 2018. 

8 RHDAustralia (ARF/RHD writing group), The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3.2 edn), Menzies School of Health Research, Darwin, 2022.