Talk to Pediatrician on BBS 10,ZNFX1 Immunodeficiency 91

My baby received both of these diagnosis at birth through genetic testing baby is 3 month old and has stage 4 kidney disease wirh polycystic kidneys, polydactyl, And with the very rare immunodeficiency disease we are very worried about it and baby is fine now but immunologist is concerned about HLH inflammation but they are not looking at the whole picture and want to do a trail on him with bone marrow transplant. He is completely fine now other than kidney disease. No signs of HLH should we go ahead with bone marrow transplants at 6 month age or we should wait and see as its a very rare and underreported case. So we are looking for second opinion. We are doing ivig for now .