Introduction: Allogeneic hematopoietic stem cell transplantation (Allo-HCT) is potentially curative in eligible patients with sickle cell disease (SCD). Long term survival remains a challenge following allo-HCT and factors that predict for longer term complications and late mortality include increased hospitalization within the first 100 days, low socioeconomic status and poor access to healthcare.
A new cottage industry of medical tourism associated with allo-HCT has emerged where children and adults with SCD living in Africa are traveling to countries that provide allo-HCT. Recent advances in improved disease-free survival and overall survival has resulted in families seeking curative options outside of their low resource-setting. The decision for allo-HCT is heavily weighed to family preference without regards to post-transplant care in their local environment. Unfortunately, the long-term care required for many children following an allo-HCT transplant is usually not available in their home country. Thus, the family desiring an allo-HCT is put in an awkward situation where they may need to have follow-up care in their primary country at a medical facility where neither the expertise nor the resources are available to manage long-term complications of allo-HCT. To explore the relationship between transplant medical tourism and patients with SCD, we report a case series of children who received their allo-HCT in another country only to return back to Nigeria.
Methods: We recently established a post-transplant care clinic at the Lagos University Teaching Hospital, Idi-Araba, Nigeria. This includes a multidisciplinary team of providers, physicians and nurses with expertise in transplant care, and collaboration from Vanderbilt University Medical Center, USA. All cases described received allo-HCT outside Nigeria and returned within 60-100 days post-transplant. Parental preference for curative option was main indication for seeking allo-HCT. Records were obtained through electronic and paper medical records.
Results: All the four cases reported had sickle cell anemia (Hb SS). Cases 1-3 received reduced intensity haploidentical HCT with post-transplant cyclophosphamide (Haplo-HCT), using G-mobilized peripheral blood stem cell grafts from parental donors with sickle cell trait. They all received preconditioning with hydroxycarbamide, azacytidine, and hypertransfusion (Table). Case-1 was 2years old, initially evaluated at day +135 post-transplant, complications included grade-II acute GI graft-versus-host disease (GVHD) at day+37 post-transplant, febrile illness and pseudo-membranous colitis requiring repeat endoscopies and prolonged steroid therapy. Case 2 was 5-years-old, seen day+395 post-transplant self-discontinued immunosuppression and antimicrobial prophylaxis with no guidance (despite the original haplo-HCT required a minimum of 12 months of immunosuppression therapy). Similarly, Case 3 was 7-years-old, off immunosuppression prematurely, and yet to commence routine post-transplant immunizations. Case 4 was 5-year-old who received matched related myeloablative bone marrow transplant. He was seen at day +138 post-transplant. He had poor graft function, EBV reactivation requiring Rituximab chemotherapy. Other management challenges encountered by patient's post-transplant include timely monitoring of immunosuppression and graft function, provision of irradiated blood component transfusion support, provision of anti-malaria and anti-helminthic prophylaxis which are endemic locally. Further, no physician to physician contact was made to transfer the patient back to a hematologist or oncologist with knowledge about post-transplant medical care.
Discussion: The argument for performing the allo-HCT in children moving back to a low-income country has to be weighed against the availability of a strategy for management of late complications of allo-HCT, such as chronic GVHD, infectious complications related to immune reconstitution, as well as endocrine and chronic metabolic syndromes. Ultimately, the decision to perform an allo-HCT in such situations must be done on a case-by-case basis with a clear contingency plan to manage transplant-related complications for at least 2 years after the procedure at a hospital with adequate transplant expertise and support measures.
No relevant conflicts of interest to declare.
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