Meeting with Immunologist

We had a good day! The Immunologist (whose name is David too) had some interesting information to share. I thought we had exhausted all our resources, but the Dr had a new and compelling paper showing very positive outcomes for boys like David. Just to let you know, Wiskott-Aldrich is rare, so there is not a lot of statistics, making the decision process even more difficult. He was very pleased to see David produces some of the Wiskott-Aldrich Protein. The amount is reduced, but the amount of protein present is very important. David does not have any major immune deficiencies (no record of recurrent upper respiratory infections or eczema). David does not have a sibling donor for the bone marrow transplant (BMT). The doctor said if it was his child and he did not have a sibling donor, he would not do the BMT. So, since it appears David does not have classic WAS or a sibling donor, there really isn't a compelling reason to subject him to a bone marrow transplant. That's what we are currently thinking.

As I was walking out the door to come home, the hematologist literally walked in and wanted to talk for 30 seconds. He said if we do not do the BMT, they would like to follow up with David every three months. He said they do not do platelet transfusions even if the platelets go really low. That was good news too. The less blood products he receives, the better. He said they would treat David for bleeding if there was an accident, but other than that, they would just monitor him. We were pressed for time so my time with the hematologist was limited.

The bad news is that without a BMT, many boys develop auto immune disease (anemia, swollen joints, kidney inflammation, liver problems, arthritis, vasculitis, malignancies). However, the research says boys who do produce the Wiskott-Aldrich protein (like David does), have much better outcomes. The report we read, those with the WAS protein, zero had malignancies, but there is no way the doctors can predict if David will develop other auto immune disease. The genes David inherited, and his environment, contribute to his susceptibility for developing an auto immune disease (same goes for everyone else in the world). My current thinking is to curtail auto immune disease through diet, supplements, and prayer. His platelets will always be low, so David will always be at risk for hemorrhaging if he is in an accident, unless we do a splenectomy (which carries risks too). The research shows an increase in mortality from hemorrhaging especially around the teenage years - maybe we will rethink splenectomy in 13 years. The overall life expectancy for children with the WAS protein and with mild symptoms is really good compared to those without the protein (life expectancy takes a dive at age 15).

We still have time on our hands, but I think we are leaning towards not doing the BMT. David's condition could change for the worse, in which case we might need to change our minds, but not likely. The doctors said there is no right or wrong decision in this case. If we conclude the BMT is too risky, at least the doctors will not think we are medically neglecting David. We have completed all the necessary tests. The next step is on January 3, we have a second opinion coming from a Dr. Wara at UCSF. We will make another update after that appointment.

If we could ask you to pray about one test that is pending. It's not an urgent test, but it is another piece to this complicated puzzle. The National Institute of Health has a few million of David's B-cells that they are trying to test. The cells are not growing properly so they had to repeat the test. They are working with a small sample (a few million cells sound large to me, but it's small according to them). Please pray for the cells to grow so they can complete the test and we will get more information about how David's B-cells are functioning. Grow, B-cells, grow!

Thank you again. Love,

The McNally Family
(Michael, Aimee, Adira, Ariella, and David)