My son and around 17,000 adults and children in England have a sickle cell disorder. Their bodies produce unusually shaped red blood cells that can block blood vessels and lead to a serious complication known as sickle cell crisis.

Sadly, various reports have stated that people with sickle cell disorder experience delays in access to effective pain relief and failures in care leading to avoidable deaths. As a mother and a nurse, I hope NHS England’s ACT NOW campaign will achieve the improvement my son and patients like him need and deserve.

Codeveloped by experts, clinicians, patients and their advocates and published within the Royal College of Emergency Medicine’s best practice guidance, ACT NOW draws attention to eight areas of care that patients and clinicians agree matter most for children and adults experiencing a sickle cell crisis.

Analgesia (A)

Patients in crisis experience severe pain and should have access to effective pain relief within 30 minutes. It can be given in an ambulance or in A&E, while some trusts now have a sickle cell crisis bypass unit, removing the need to wait in A&E.

Not knowing the person or what pain relief they have taken at home, out of date care or pain plans, and reduced staff capacity can lead to delays in administering effective pain relief.

Specialist nurses can help by checking care plans and talking to patients and families about what to do in a crisis. Having an advocate can be helpful for people in sickle cell crisis to communicate symptoms including chest pain, when they might struggle to express themselves clearly due to pain, fear and potential hypoxia. This can impact on care, including understanding of the pain relief a patient has already taken at home.

Compassion (C)

At a basic, cultural level, it’s about people. We don’t call them ‘sicklers’ – we use their name. People with an unpredictable, debilitating condition that can be fatal. Someone who felt fine yesterday, could be poorly today. They might present at A&E frequently, without the severity of their crisis showing. My son had an experience where he presented to A&E nearly every day and on day five things rapidly escalated and he needed care in the ITU.

Day one or 1001, your compassion is crucial in building trust. My patients understand the importance of being transparent about their pain level and the type and amount of pain relief agreed in their care plan. Transparency requires trust in the NHS to act rapidly on that information to keep them safe.

Tests and triggers (T)

Recommended tests include full blood count, reticulocyte count, group and save, routine renal, liver and bone biochemistry, C-reactive protein (CRP) alongside tests indicated by their medical history. If you are considering using a canula, talk to your patient, investigate their medical and blood transfusion history (including any reactions) and consider if an infection is suspected.

People know their condition better than anyone and talking to them to determine any triggers for the crisis can support discussion about admission and discharge.

Notify (N)

Notifying the patient’s advocate as requested or stated in their care plan helps build trust. While their specialist haematology team can provide additional medical advice and support their recovery by exploring options to help them to stay well for longer, such as concordance with medication, any routine blood tests and reducing triggers.

Oxygen (O)

Oxygen should be given if indicated (saturations 95% or below on room air) or where needed to comfort your patient.

Watch and keep warm (W)

Regular observations of pain, blood pressure, pulse, respiratory rate, blood oxygen (Sp02) and temperature are vital in prompting rapid escalation when needed. While you might need to be creative and persuasive to ensure your patient is situated somewhere suitably warm with access to drinks. Sometimes, a blanket is all we have to hand, but a pile of heavy blankets can hinder visibility of your patient and might fail to exclude a draught from an outside door. Dehydration, cold and draughts are crisis triggers for many people with sickle cell.

Increasing education around sickle cell

As myself, siblings and close family don’t have sickle cell disorder, I didn’t know that I was a carrier of the sickle cell gene – or trait – until I was pregnant. I retrained as a nurse in 2012 and became a Clinical Nurse Specialist in haematology in 2017, so I understand why clinicians don’t always know enough about sickle cell. As a mum and a nurse, I have to change that.

I’m thankful to my colleagues in leadership, communications and A&E at Kings College Hospital for promoting ACT NOW. My friend in A&E reported seeing the posters being used. I have had junior doctors opening conversations about sickle cell. I have discussed ACT NOW with my patients, and my son, and asked them to tell me if their level of care doesn’t meet those expectations.

As one of 18 official evaluation sites, this feedback will be used alongside health outcome and patient experience data to enhance future versions of ACT NOW. And do you know my son’s response … ‘Mum, you are so boring sometimes!’ but I am grateful for the opportunity to bore him.