Zachary Ramsay | Addressing challenges in sickle cell disease pain management

WE WRITE in response to the article ‘Pain and despair’ published on September 27. The Sickle Cell Unit deeply empathises with the concerns voiced by the patients suffering from sickle cell disease. We recognise their struggles regarding stigmatisation, as well as access to medical care and medications.

Their experiences are valid, and we strive to maintain ongoing communication with patients through various advocacy efforts, continually learning from their perspectives to enhance care. It is worth emphasising that while we share similar values with most healthcare workers at The University Hospital of the West Indies, our staff and policies are separate, and we can only speak to the experiences within our clinic. There are a few points that we believe need to be addressed.

As discussed in the news story, sickle cell disease pain is often very severe and requires powerful pain medications known as opioids. These drugs, which include Pethidine and Morphine, may have significant side effects such as constipation, nausea, vomiting, drowsiness, dizziness, respiratory depression, itching, dry mouth, confusion, tolerance, and dependence.

TOXIC BY-PRODUCT

It is very important to note that in several published studies, when Pethidine is compared to other opioid medications, it results in more side effects including more withdrawal symptoms and achieves less sustained pain relief as it is one-tenth as potent as Morphine. Pethidine also metabolises into a toxic by-product known as normeperidine, which can lead to seizures. In contrast, morphine offers the advantage of being more safely increased in dosage to provide better pain relief with a lower risk of harmful effects. As a result, several professional societies such as the Royal College of Emergency Medicine and the UK National Institute for Health and Care Excellence recommend that Pethidine should no longer be used for managing acute sickle cell disease pain.

Unsurprisingly, physician prescriptions for Pethidine have fallen over the last two decades, and manufacturers have reduced supplies globally. While we recognise that some patients have preferences for medication, this should not supersede the safety concerns surrounding Pethidine use. There are many safe alternatives to opioids that can be considered, particularly for patients with allergies, including non-steroidal anti-inflammatory drugs such as Cataflam and Voltaren and antispasmodics such as Metamizole. Additionally, many of the opioid-related symptoms reported by these patients, such as itching and vomiting, are not really allergies but are temporary side effects that can be prevented by co-administering medications such as anti-emetics (dimenhydrinate) and antihistamines (diphenhydramine).

Recent work of the Ministry of Health and Wellness (Sickle Cell Disease Technical Working Group) and the Sickle Cell Unit have made many of these medications more accessible and subsidised through the National Health Fund. Looking ahead, continued education of healthcare workers will be important to ensure these practices are routinely offered to patients and to avoid stigmatisation associated with the use of opioids.

PAIN EPISODES

The news story also stated that “the patients say when they visit the UWI facility (the Sickle Cell Unit), they are most times rerouted to the University Hospital next door”. It is important to note that while the Sickle Cell Unit has provided excellent pain management for over 30 years as part of the services we offer, our capacity is limited due to being a small clinic with daytime operations and constrained funding. Our goal is to prevent and alleviate pain for all patients. However, some will require hospital visits for further management, including possible admission. According to a 2021 study published in the International Journal of Clinical Practice by Asnani et al, approximately 70 per cent of all pain episodes treated at the Sickle Cell Unit between August 1, 2016 and July 31, 2017 were successfully managed without the need for hospital referral.

The Sickle Cell Unit attends to more than 3,000 patients annually. This includes preventative routine care to provide screening and early detection of disease complications, vaccines to prevent potentially life-threatening infections, and hydroxyurea, a disease modifying medication which can decrease painful episodes.

In addition to clinical care, our mandate includes research, newborn screening, education and training. The Sickle Cell Unit has made significant efforts to advance sickle cell disease care in Jamaica, through partnerships with the Ministry of Health and Wellness and other stakeholders. In 2012, sickle cell disease was included in the National Strategic and Action Plan for the Prevention and Control of Non-communicable Diseases. Medications to treat sickle cell disease became subsidised by the National Health Fund in 2015.

Since 2021, free medication at any public pharmacy became available with the use of a prescription from the Sickle Cell Unit. Notably, early detection and enrolment into a programme of care via newborn screening has been vital in reducing the under-five mortality rates to levels comparable to that of the general Jamaican population. Universal newborn screening was established in 2015 for all public hospitals and extended to private hospitals in 2020. Moreover, regional workshops have been held throughout the island since 2019 to educate healthcare workers on the use of hydroxyurea and our practice guidelines. Additional educational efforts include ongoing clinical training of medical students and resident physicians at the Sickle Cell Unit clinic.

We would like to reiterate that the experiences of these patients are valid, and we encourage patients to access routine preventative care. Efforts to improve healthcare access and to educate healthcare workers are ongoing, with the long-term goal of improving care and patient satisfaction. We remain committed to advancing our services and addressing any gaps in care to better serve the needs of our patients, ensuring that they receive timely and effective treatment.

Dr Zachary Ramsay is research fellow at the Sickle Cell Unit, Caribbean Institute for Health Research, The University of the West Indies. Professor Monika Asnani, Professor Jennifer Knight-Madden and Dr Lesley King contributed to this article. Send feedback to zachary.ramsay@uwimona.edu.jm