When you pray God sized prayers, you’re going to begin to see the greatness and goodness of His Power. The key is having the confidence to “Ask, Believe and Receive.” – Joel Olsteen
Thirty days ago, Evan and I arrived in Boston. I remembered feeling so relieved, grateful and a little intimated to be at the #1 ranked Children’s hospital. I will never forget that day. The emergency room was like a scene out of the TV series ER it was huge and the hospital itself was huge. It felt much bigger then than it does now that I have gotten my bearings straight and learned my way around. This post is about why we made the decision to come to Boston and why our journey does not end once we head back to Pittsburgh. Children’s hospital of Pittsburgh is also a highly ranked hospital in fact we had issues with our insurance company approving us coming to Boston because we could receive good care in our own backyard. So why Boston? Here is why:
Pulmonary vein stenosis is a very rare and serious disease of the pulmonary veins and Boston has a team of cardiologists who have been studying pulmonary vein stenosis for over a decade. Pulmonary vein stenosis is very different from other congenital heart defects because it is not usually corrected by surgery. The sutureless repair is done to widen the narrowed veins but often times the obstruction re-occurs. This lead the cardiologists to think of PVS differently since it was not your typical heart defect and they sought to find ways to stop the veins from re-narrowing. Even though PVS is so rare because BCH has a specialized PVS team they have gained an expertise for treating kids with PVS. They are currently conducting a clinical trial which Evan is a part of that incorporates a chemotherapy drug in order to slow down the grown of the cells within the veins which is causing them to narrow. The cells in the veins that are causing the narrowig are called myofibroblasts; we don’t know what causes these type of cells to grow causing blockage of blood flow but what they have found is that these cells have similarities to cancer cells. The difference is these type of cells do not travel to other parts of the body.
The standard treatment is surgery and catherization but often times these are only temporary solutions as the veins would re-stenosis. The only other option for treatment lis a lung transplant which is a MAJOR deal. Boston is offering a clinical trial using chemotherapy drugs that have been shown to attach to the rapidly growing cells. The chemotherapy drugs are Gleevac and Avastin. The goal is for the chemotherapy drugs to slow down or stop the rapid growth of the cells. The Gleevac and Avastin are very well tolerated by infants and children. Evan received his first does of Gleevac on Thursday, August 14th and is tolerating it very well. He gets a dose of Zofran about a half an hour before the Gleevac is given to make sure it doesn’t upset his stomach. The Gleevac comes in a pill form and is dissolved in water and given through his NG tube but will eventually be given to him by mouth though I heard it is disgusting! The Avastin is only given if the cells don’t respond to the Gleevac.
As I mentioned before this is a trial drug however it has shown some very promising signs in stopping the growth of the cells until the child outgrows the disease. The treatment typically lasts for one year. Once we are back home Evan will be followed by a cardiologist and an oncologist who will work very closely with the team here in Boston. His follow up care will include echos, lung scans, heart catherizations and blood work in order to monitor things to ensure his body is handling the medications.
I met with the oncologist and signed consent forms to start the Gleevac and felt very confident that we are doing the right thing and I believe with every fiber of my being that the Gleevac is going to work and he will eventually grow out of it. The oncologist did explain that the treatment does not necessarily start immediately. He said that we should expect re-occurrence which can be treated through cath interventions but the longer he is on the drug the less and fewer and far between the interventions will need to be done. He compared the Gleevac to a fast moving truck coming to a stop; it takes some time for the truck to actually stop once the brake is applied.
I can’t say enough good things about the team here especially Evan’s surgeon. He came in to talk to me today about the plan when we get home and he made it very clear that if I thought something was wrong then there was and if I wasn’t getting the answers I wanted at home to bring him to Boston as we have to be very aggressive in staying in front of the veins. I explained that I have no problem whatsoever doing that. My mommy instinct hasn’t failed me yet!Evan will need more catherizations and Ryan and I have decided that we would like for them to be done in Boston. The cath lab doctors here have more expertise in PVS and as explained to me by the surgeon tend to be more aggressive than others outside the hospital because of that expertise. I have full confidence in the combined team in Pittsburgh and Boston. I know that we will be in good hands once we are back home.
God gave me a spirit of hope and brought us to Boston and will continue to guide our journey. I have a peace that God has given me and a belief that Evan will be healed. Like Joel said, I have the confidence to ask, believe and receive! I also believe that by Evan being in this trial he will help other children who are diagnosed with this disease and give their parents hope.
I know this is a lot of information in one post so I will stop here for now but will continue to share information in future posts.