How to keep sickle cell patients healthy during pandemic

Less than six months into the pandemic, there are already over eight million confirmed cases and more than 450,000 deaths globally. 

More than 4,000 cases and over 100 deaths have been reported in Kenya and the country is yet to reach the its peak.

The majority of the deaths are among those considered to be high-risk populations. People above 60 years old and those with chronic blood disorders like sickle cell anaemia (SCA), diabetes, heart and lung diseases are classified in this group. It is important to note that carriers of sickle cell gene without clinical disease are not at any higher risk than the normal population.

Regardless of the chronic condition, the general guidelines for reducing risk of exposure as provided by the World Health Organization and Centers for Disease Control and Prevention are universally being applied by countries. Key among these is shielding high-risk populations by having them stay at home whenever possible, social distancing, use of face masks and frequent sanitisation while in public spaces.

Other measures include avoidance of contact with people with a new continuous cough, fever, and/or loss of smell or taste as they may be harbouring Covid-19.

How does Covid-19 affect SCA? Children under the age of 18 years appear to be less susceptible to Covid-19.

The coronavirus infection triggers SCA crisis and super-imposes its sequelae on an already immune compromised child. Of major concern is Acute Chest Syndrome which even under non-Covid-19 circumstances is associated with high risk of death.

This is not surprising given Covid-19 manifests with severe chest infection that interferes with normal oxygen exchange. The virus also causes abnormal blood coagulation that could make painful crisis and blockage of blood vessels in SCA worse.

With countries having marked the World Sickle Cell Day on Friday, it would be good to look at how the Covid-19 pandemic affects ongoing care of children with SCA under regular follow up. This is really where prevention becomes better than cure, meaning stay at home, social distancing, wearing masks and sanitising rules are important for these patients.

The usual measures of avoiding situations that precipitate crises such as dehydration, exposure to cold, excessive physical exertion and infections should be observed.

Covid-19 prevention measures are indeed very good for SCA as they also reduce transmission of other common airborne and hygiene related diseased.

Adherence to preventive medication like folate, penicillin-V and hydroxyurea that reduce the risk of crises is not negotiable during pandemic. Those that may be lagging behind on essential vaccines must also update their status as priority. While administration of the flu vaccine is recommended, its interaction with Covid-19 is still under scrutiny.

Patients should not miss regular clinics. Innovations in health care provision by providing non-contact consultations are now also becoming increasingly available. Aga Khan University Hospital has, for example, introduced tele-consultations, tele-laboratory and tele-pharmacy.

Other measures being promoted are issuance of longer duration prescriptions or multiple refills for stable patients. 

Prof Macharia is a consultant paediatric haemato-oncologist at Aga Khan University Hospital