Guidelines aim to ensure proper care of patients with sickle cell disease amid COVID-19 pandemic
Acute and chronic complications of sickle cell disease necessitate frequent interaction with the medical system.
These complications — which include pain, fever and acute chest syndrome — make managing the estimated 100,000 patients with sickle cell disease in the U.S. a challenge under the best of circumstances.
During the COVID-19 pandemic, however, sickle cell disease complications may confound diagnosis, and health care providers could face new challenges in meeting the needs of this patient population.
“We are very concerned that COVID-19 infection could make patients with sickle cell disease much sicker than the average person,” Biree Andemariam, MD, chief medical officer of the Sickle Cell Disease Association of America (SCDAA) and director of the New England Sickle Cell Institute at UConn Health, told Healio. “Individuals with sickle cell disease are at much higher risk for severe COVID-19 infection and complications associated with infection. So, we are recommending that patients with sickle cell disease stay at home as much as possible.”
To ensure that patients with sickle cell disease complications receive the best possible care, the association issued an updated provider advisory for the acute and chronic management of these patients during the pandemic.
Andemariam spoke with Healio about the advisory and how physicians can continue to treat patients with sickle cell disease while helping them avoid exposure to the novel coronavirus.
Question: How should clinicians continue to provide routine care for patients with sickle cell disease during the COVID-19 pandemic?
Answer: We’re recommending that providers of care for patients with sickle cell disease switch from primarily face-to-face encounters to telephonic or other electronic means of communication. Routine care, as much as possible, should be provided over the telephone or through electronic consults. This can limit patient exposures to the virus while traveling to medical appointments.
Q: Has telemedicine for sickle cell disease run smoothly during this pandemic so far?
A: Yes. I work with the 33 members of our SCDAA medical research advisory committee who have been instrumental in developing the guidelines and sharing information. These sickle cell specialists — from places such as Italy, the U.K., Lebanon, Ghana and Tanzania, as well as the U.S. — are finding ways to implement telemedicine very effectively. Patients have been very receptive to the idea that they can sit in the safety of their homes and have a doctor’s visit over the telephone or other electronic sources. We prescribe medications electronically and recommend that patients use pharmacies that will deliver medications to their homes.
We can generally pick out who needs to be seen — ideally in a doctor’s office — and who can be managed over the telephone. We’re trying to get the word out that if they don’t have an emergent situation, it’s better not to go to the ED, because there is a greater risk for exposure to people with COVID-19. Patients have been adhering to this very well, and those who need to be seen get seen.
Q: The guidelines mention “overlap of lung disease” between sickle cell disease and COVID-19. Does this overlap worsen the effects of sickle cell disease, COVID-19 or both?
A: I would say both. Patients with sickle cell disease are prone to acute chest syndrome, and the symptoms of acute chest syndrome very much mirror those of COVID-19. We also are concerned that these two conditions can be superimposed, which would not only worsen the severity of the COVID-19 infection, but also the severity of acute chest syndrome. If such a patient presents with fever, a cough and an abnormal X-ray and then tests positive for COVID-19, a physician in an ED who is not familiar with sickle cell disease might treat the patient only for COVID-19. Often, someone with acute chest syndrome related to sickle cell disease needs a large blood exchange transfusion. We would never be keen to do something like that for a patient who has COVID-19 only.
At the same time, we’re concerned that patients with sickle cell disease might present with fever, chest pain and an abnormal X-ray, and the clinician may not even consider COVID-19. We are trying to raise awareness that patients can be at high risk for both, that each can make the other worse, and that the treatments are different but can be deployed simultaneously.
Q: Can you offer any recommendations for making a differential diagnosis between sickle cell disease and COVID-19?
A: It’s important in the differential diagnosis to consider complications people with sickle cell disease commonly have. If they come in with a fever, we generally think about other viral infections. We would rule out other viral infections, and we’d look for a bacteremia or a urinary tract infection. Now, though, any patient with sickle cell disease who comes in with a fever or other signs and symptoms associated with COVID-19 should be tested.
It’s also important not to draw any conclusions based on a patient’s age. Many patients with sickle cell disease are very young, and we’re concerned that these patients might not be perceived as being high risk and may be sent home. We want to make sure that the average physician understands that sickle cell disease is a significant risk factor for severe COVID-19 infection.
Q: How should physicians manage acute sickle cell pain symptoms?
A: The most important thing is to get the pain under control, and that doesn’t change in these circumstances. It is important to get clarity from the patient as to whether it feels like their usual sickle cell pain or if it feels different, particularly if it involves their chest. Usually, you just need to ask them, “Does the shortness of breath feel different? Is this cough your usual cough, or is it something new?” We don’t want doctors to assume it’s just their typical pain crisis. In the setting of this pandemic, we need to dig a little bit deeper.
Q: In the context of potential blood shortages, how should clinicians prioritize the use of blood transfusions for their patients with sickle cell disease?
A: We are completely aware of current or impending blood shortages. At the same time, we’re not advocating for any changes to usual transfusion practices, especially in situations where patients are on regular transfusions to prevent strokes. These decisions need to be tailored to the individual patient, and the patient should consult their physician. We don’t want patients to decide not to have their transfusions out of fear of COVID-19. They need to speak with their physician about the potential risks and benefits.
Q: How should patients with sickle cell disease and COVID-19 be treated?
A: COVID-19 is such a moving target right now, I will leave that question to the critical care physicians. If a patient with sickle cell disease is presenting with pain, we need to treat their pain and consider the possibility of COVID-19. We can’t assume they are in the low-risk category. We need to be vigilant for signs and symptoms of coexisting acute chest syndrome and COVID-19, because this is a recipe for a very poor prognosis. If patients meet the criteria for acute chest syndrome, they should be treated accordingly with empiric antibiotics, oxygen support and incentive spirometry. Physicians should also strongly consider an early blood exchange transfusion in this situation.
Q: Are there any contraindications in treating both sickle cell disease and COVID-19?
A: No. We looked at this very carefully, and we recommend that patients with sickle cell disease stay on all their chronic medications. The goal is to maintain the healthiest possible status, to reduce the likelihood that they will have a flare-up of their sickle cell disease. Keeping a patient with sickle cell disease healthy also may result in a better outcome if they do become infected with COVID-19.
There has been some debate on the use of [angiotensin-converting enzyme (ACE)] inhibitors and NSAIDs. We don’t have a position on that, because the data are not robust enough. A lot of patients with sickle cell disease are on NSAIDs for pain and take ACE inhibitors for blood pressure or kidney issues. Based on where the data stand now, we are not prepared to tell people to come off those medications, but to stay up to date and talk to their physicians about them. This could change as more data emerges. That is why we are updating the recommendations regularly.
Q: What else can physicians do to keep patients with sickle cell disease healthy and safe from COVID-19?
A: One thing we can do is to help replenish the blood donor pool. As we talk to our patients during telephone visits, we could remind them to encourage their family members and community members to donate blood. Even though people with sickle cell disease are not eligible to donate blood, people with sickle cell trait — many of whom are likely in the patients’ families — are eligible. Patients with sickle cell disease are more likely to find blood that matches theirs if it comes from donors who are of African ancestry. Yet, if you look at the blood donor pool across the United States, a very small percentage of donors are of African ancestry. Individuals with sickle cell disease are primarily of African ancestry, so it makes sense to ask them to encourage their family members and extended community members to donate. That’s an important role hematologists and physicians in general can play, but one we don’t often think about: We can help increase the donor blood supply. – by Jennifer Byrne
For more information:
Biree Andemariam, MD, can be reached at 263 Farmington Ave., Farmington, CT 06030; email: firstname.lastname@example.org.
Sickle Cell Disease Association of America. Sickle cell disease and COVID-19: An outline to decrease burden and minimize morbidity. Available at: www.sicklecelldisease.org/files/sites/181/2020/03/SCDAA-PROVIDER-ADVISORY4-3-25-20-v2.pdf. Accessed on April 2, 2020.
Disclosure: Andemariam reports serving on clinical advisory boards for Global Blood Therapeutics and Novartis.