My Grandson Landen Smith was a very sick baby . Was finally diagnosed in 2014 with a newly discovered immunodeficiency called xiap by Dr Michael Jordan at Cincinnati Children’s Hospital. He was  1 of 200 diagnosed in the WHOLE world. There was no known treatment for it because it was so new. Landen had fevers as high as 106.6, swollen lymph nodes, diarrhea, stomach ache, very lethargic and sepsis many times. After many experimental treatments with every immunosuppressant drug that Dr Jordan could come up with, nothing worked. Those drugs actually suppressed his “working part” of his immune system leading to further bouts of sepsis, seizures, osteoporosis, extreme weight gain due to steroids to control symptoms, necrosis of his knee joints, GI bleed resulting in 10 units of blood, HLH, etc. Our last hope was going to be a bone marrow transplant, however after several bone marrow drives, we did not have a match. Dr Jordan said a bone marrow transplant would be very risky without a 10/10 match because his xiap makes it very tricky. 
    We were going to prepare for a bone marrow transplant with a 8/10 match because we had no choice . We could lose him�. Dr Jordan checked into a drug he heard about that he thought would work for Landen’s condition. It was called Tadekinig made by AB2bio in Switzerland. They were beginning a drug trial with this drug and Landen was accepted into the trial. This was our miracle drug! He has never been sick since he was on the Tadekinig and did receive it compassionately for a few years. Landen’s last injection is Sept 30, 2022. Tadekinig is an injection that Landen takes every other day. There is no “cure” for Landen’s condition because it is genetic. AB2bio says they can’t provide the drug anymore and it hasn’t been approved by the FDA yet. We are in a bad spot, we need this drug!

   Dr Jordan heard there was a drug being made by Novartis, called MAS 825. He believes this drug would work as well, except it is a infusion given every 2 weeks. When Dr Jordan first approached Novartis about using this drug for his xiap patients because they are losing the Tadekinig (which we prefer), they were very helpful and we’re going to rewrite the current study to include xiap kids, and if the study wasn’t ready by the time Landen ran out of Tadekinig, they would let us have it compassionately and it could be given by Dr Dvorak , our local pediatric hematologist/oncologist in Syracuse at Upstate Medical Center. Now we are down to the wire! Dr Jordan can’t get a straight answer out of Novartis and we have a 16 year old with no backup plan and the problem of getting very sick. Life saving drugs should not be withheld by the drug companies, especially large drug companies.