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Harnessing the power of patient expertise: lessons from hereditary angioedema

Angela Metcalfe, CEO of HAE UK

To coincide with HAE Awareness Day, we hear from Angela Metcalfe, CEO of HAE UK, about the challenges faced by people living with the rare inherited condition, hereditary angioedema, and the importance of shared decision-making between patients and those treating them.

What is hereditary angioedema?

Imagine the scenario: you’re seven years old and excited about appearing in the school nativity play, but instead you have to stay home in bed with excruciating stomach ache, vomiting and diarrhoea. As a hormonal teenager, you face bullying because of the swellings in your face and hands, and are hit with an attack the night before an important exam. In adulthood, you struggle with decisions around family planning, knowing the hormonal changes of a pregnancy or simply the stress of being a new parent could trigger an attack. Then there’s work stresses, relationships, travel: all of life’s important events are overshadowed by the threat of a debilitating attack.

This is the daily reality for many people living with hereditary angioedema (HAE), a rare but potentially life-threatening inherited condition. It is one of a number of illnesses that is usually treated by a clinical immunologist. Symptoms of HAE include episodes of oedema (swelling) in various body parts including the hands, feet, face and airway. People with the condition may also experience abdominal pain, nausea and vomiting which is caused by swelling in the intestinal wall. They face unpredictable and painful attacks, which can prove fatal if swelling reaches the larynx. It’s an illness that impacts physical health, emotional wellbeing and overall quality of life, interfering with activities such as work, study and travel.

Patient-centred care

While the cause of an HAE attack is not always known, stress, anxiety, infection, trauma and hormonal changes are all known triggers. In addition, the frequency and severity of symptoms is liable to change according to physiological and external factors. Flexible management of the condition, as well as tailored treatment and care, are therefore vital. 

Historically, treatment options for HAE have been limited, leaving many patients on a sub-optimal plan for years. This has all changed in the last decade, however, with several new treatments becoming available. Perhaps most importantly, there has been a shift towards prioritising patient choice. The National Institute for Health and Care Excellence (NICE) recommends that shared decision-making, where healthcare professionals work with patients to reach a joint decision about care, should be an integral part of any consultation. This is particularly important in rare diseases like HAE, as the patient often knows their own condition and its impact better than those treating them.

Shared decision-making in practice

As CEO of HAE UK, it’s my job to ensure that people living with HAE and their families have access to the best treatment and support. We often hear from people living with HAE that they do not feel sufficiently involved in decisions around their care. Many remain for long periods on sub-optimal treatments, or feeling that their preferences are not important. In some cases, they are told that new treatments are too expensive, or that they aren’t ‘sick enough’ to warrant them.

Following the publication of updated guidelines in 2021, we partnered with BioCryst UK Ltd to carry out research into shared decision-making in HAE. Through qualitative interviews with patients and healthcare professionals, a patient survey and workshops, we set out to find out what was really happening on the ground.

We found that that 36% of people with HAE felt the shared decision-making process was not properly followed when determining treatment options; 26% were unfamiliar with the concept at all. In addition, 32% listed unpredictability of attacks as the biggest challenge of living with HAE, while 26% felt coping with everyday life was a challenge. 

There are many reasons why shared decision-making may not be optimally implemented. Someone with HAE might be anxious about starting a new treatment with potential side effects, or they may not be as engaged with their care if they are not currently experiencing challenges. They may feel their disease isn’t well understood by the people treating them, and that they have to start from scratch each time they see a new member of the healthcare team. 

Our research showed that healthcare professionals feel there is a lack of guidance and training in shared decision-making, and that workload pressures can make it difficult to develop the trusting relationship that is so crucial for open dialogue. Communication with non-specialist clinicians can be particularly challenging, and can mean people with HAE are left unduly distressed in an emergency situation.

We did, however, unearth some promising examples of best practice, and both patients and healthcare professionals spoke about the transformative role shared decision-making can have on care and quality of life. The importance of the relationship between patient and healthcare professional emerged as a key theme, as did the the role of clinicians in helping people to be confident in expressing their needs and preferences. 

Healthcare professionals must be willing to listen, to consider a person’s unique and often changing circumstances, and to integrate their preferences into the treatment plan. Ultimately, it is their responsibility to engage patients in shared decision-making, to provide advice and recommendations, and to build a strong and trusting relationship. In short, the success of shared decision-making depends on a shift in the mindset of both patients and healthcare professionals. 

Together with patients and healthcare professionals, HAE UK and BioCryst have developed the following recommendations to improve shared decision-making in HAE:


  • Patients should be supported to advocate for themselves and to share with clinicians the impact of their condition and their aspirations objectively. This would provide their clinician with all the information to inform shared decision-making conversations and make treatment decisions together.
  • Keeping a log or diary of when attacks occur can help add detail and paint an accurate picture of what has happened since the last appointment, ensuring nothing is missed.
  • Some patients may feel more comfortable speaking about what is on their mind by preparing what they want to talk about before their appointment, or by bringing a loved one to support them. 
  • If patients do not feel comfortable or feel that they don't have the right kind of relationship with their consultant for any reason, they should be able to request to see another member of the team without this being seen as a criticism.

Healthcare professionals

  • Clinicians should take thorough notes in every consultation so that other clinicians or junior staff can pick up a patient’s file and know what is going on.
  • Clinicians should consider asking more in depth, open ended, questions such as ‘what are you worried about?’, ‘how is it impacting you?’ or specifics about their life in order to build a fuller picture of their condition, and better relationships based on deeper understanding. Specific questioning around recognised treatment side effects (e.g. anxiety or facial hirsutism) can help uncover side effects patients may not have thought to mention.
  • Healthcare professionals should encourage patients to record all evidence of disease activity to provide the full clinical picture, which may indicate the need for a change in treatment.
  • Healthcare professionals should keep patients informed about new treatments available in a language that they will understand. Everyone is different and some patients like detailed information at appointments, while others find it too much to take in all at once. The healthcare professional should learn the patient’s preference and tailor their approach accordingly. 

Angela Metcalfe


The research and recommendations, published as a new report, The Power of Partnership, can be found on the HAE UK website.