Philadelphia – January 5th, 2016


Here is a picture of Addie in our camper this summer. Her faithful companion Sue close by. Addie is not interested in many pictures these days – and I know most of you would rather see pictures of her than of Ryan and myself.

Addie had her 4th procedure yesterday. It was another long procedure and by the time we were allowed to be with Addie the doctors were gone for the night. This morning the main surgeon came in to talk with Addie and me. He said that they did find a few more holes to patch. However, where the leakage is occurring is from  lymph “masses”, and the “masses” are growing, and he is not convinced they have been able to find them all. The next step will probably be to do a pleurodesis, a procedure where they would artificially obliterate the pleural space where some of the masses are located and leaking the fluid. This would hopefully clear up the chylothorax, but would not rid the body of the other lymph masses that also appear to be growing – one on her back and one in her shoulder. Dr. Dori and Dr. Itkin are working with Dr. Adams, an oncologist that has worked with Gorham’s patients to find out if there is another medication we should add to her sirolimus to stop or reduce the growth of the lymph masses. I have also turned to my Lymphangiomatosis & Gorhams Disease Alliance support group. From the support group I am hearing that others do have these masses, some of them have been able to control the growth of these masses with medication, and some have had their masses removed successfully.

We should here from Dr. Dori this afternoon or tomorrow when they will schedule the pluerodesis.

Ryan and I continue to enjoy being here together with Addie, but Ryan will return home to Montana this Thursday, and is very excited to see the crew. Addie and I will continue to blaze this trail. Although it has been one trial after another – I continue to feel the support, prayers, and love around us, Ryan and I’s spirit and strength remain strong. I continue to be hopeful that a solution will be found, yet aware that this disease is one we will and must be on top of for years to come. Addie remains   quiet about her thoughts – strong, stoic and quiet. I long to know her thoughts and ideas – but she often only gives information about her situation when Ryan and I are wrong – so every day we try to be wrong a few times 🙂

Love to all, Michal