A Lil' Boy's Journey to Live….

Archive for October, 2011

Information on the Gleevec Study from Children’s Hospital of Boston. ~ ♥

Information on the Gleevec Study from Children’s Hospital of Boston. ~ ♥

Our innovative approach..

The problem Historically, doctors have had to rely on one of the following treatments to care for children with pulmonary vein stenosis:

open-heart surgery to widen the narrowed veins and improve blood flow from the lungs back to the heart cardiac catheterization with balloon dilation to try to stretch the vessel(s) open lung transplantation to replace the lungs and the pulmonary veins

Response to surgery or catheterization is usually short-lived. Typically, within three to four weeks, the obstruction recurs, and repeating surgery only provides temporary fixes.

In some cases, lung transplantation may be an option, but this comes with major risks, including rejection of the transplanted lung and serious infection. Patients must also take immunosupp ressant drugs for the remainder of his or her life to help reduce the chance of the body rejecting the new lungs.

Using these methods, many children with the most severe form of pulmonary vein stenosis do not survive past early childhood.

Innovative solution The Dana-Farber Cancer Institute and Children’s Hospital Boston are conducting a research study in patients who have multi-vessel (greater than two vessels) pulmonary vein stenosis. The purpose of this study is to determine the effectiveness of using two standard chemotherapy drugs that target myofibroblasts in pulmonary veins and control the abnormal growth of these cells.

The study involves treatment with Avastin and Gleevec, two medications used to safely treat childhood tumors. Multi-vessel intraluminal pulmonary vein stenosis is not thought to be a tumor, but it does share a common feature with tumors: the unwanted growth of cells.

Gleevec is given daily by mouth, while Avastin is given intravenously once every two weeks. They both have lowside-effects and have been well tolerated in infants, children and adults. The goal of treatment is to stabilize the unwanted cell growth to improve patients’ life expectancy and quality of life. The trial involves treatment for one year, and can be coordinated close to a patient’s home area.

Considering the Gleevec Study in Boston……

We have Opened the conversation to seeing if Boston can help Riley through the Gleevec Study. Cardiac Team at CHOP doesnt believe that the study is successful enough, yet, Pulmonary has told us to see about a consultation. We have all the right connections its all going to come down to whether the Gleevec could be beneficial to Riley and if its something We think would be in Riley’s Best Interest ♥

Riley’s DX… Up to this point…

Riley was born 4/4/08 with Heterotaxy Syndrome (Asplenia Type) with CAVC (common AV canal), Pulmonary Atresia, Right Ventricle to Aorta, TAPVR (total anomolous pulmonary venous return)(Infradiaphragmatic-uno bstructed) and RRA s/p LMBTS (right aortic arch status post left modified BT shunt), patch augmentation of Pulmonary Artery bifurcation and TAPVR repair at 4 days old. He subsequently developed stenosis of each of his pulmonary veins and has undergone over a dozen Cardiac Catheterization with dilation/stent placement in all four pulmonary veins. He has also had a stent placed in the BT shunt. As of today Riley only has his RUPV and LLPV remaining. The other two have completely occluded. As of 11/2/09 Riley has been listed for a Bilateral Lung/Heart Transplant and requires continuous O2. He did have a failed “suterless repair” of his PV’s 7/14/08. Riley also had the Ladds procedure 1/6/09 along with placement of a G Tube.

Visit to Granny and Pop-Pop’s… In Pics…. :)

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Riley’s Next Cardiac Cath….

Riley’s next cath has been scheduled for November 7th. Feel like we Just did this!
There is a slight possibility that the cath could be moved up because Riley’s reflux has gotten pretty bad. I doubt this will happen, though, because Dr. Rome already Hates cathing every 8 weeks because of the extreme risk! I don’t see him making it sooner but we’ll see.
I will update if anything changes. Hugs to you All for Caring about Riley and this Journey he is on. Thank You…..

Riley’s visit…10/17/11… to See Dr. Hanna…In Pictures… :)

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Everything went ok Monday at Riley’s visit to Cardiac. November 7th is a tentative date for his Next Cath.
Thank You All for Your Prayers and Constant Support! The Love of Mostly Strangers is What is Carrying Us Through this Journey!

Riley Being Silly as Usual :)

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