Skip to main content

Wanted: Treatment options for PBD-ZSD and thousands of other rare diseases. Needed: Millions of dollars for research

It's been a pretty rough two weeks in the PBD-ZSD community. Three families I'm connected with via the Global Foundation for Peroxisomal Disorders (GFPD) have had to say goodbye to their children, who bravely fought PBD-ZSD but whose time here on Earth had come to an end, and other parents in our group are currently trying to prepare themselves for the reality that their child may not be with them much longer.

Although we, the GFPD, will never be able to connect with all the families that this horrific disease affects, we know that an estimated 80 babies are born in the US each year with PBD-ZSD and while we can treat some of the symptoms, we, the parents and our children, are desperately in need of more effective treatment options and ultimately a cure.

In the midst of the sadness that is engulfing the PBD-ZSD community, several other disease communities have a reason to be hopefully. Recent articles about potential breakthroughs in the treatment of Neimann Pick Type C, Batten disease, LCA, and a specific type of cancer are worth celebrating, not only because we rejoice with these families at the prospect of being able to improve the quality of life for their loved ones, but selfishly because we hope that the knowledge gained in these efforts will be able to be applied to PBD-ZSD.

There is research being done on peroxisomal disorders, but as a parent of a 5 year old whose daily life and little body is affected by this disease, science isn't moving fast enough. I want effective treatment options and ultimately a cure. Earlier this week I read a post titled "A Disease Murdered My Daughter, and I Want Justice," and when I read that I felt like you could replace cancer with PBD-ZSD and you would have a post written by many families within the GFPD community. PBD-ZSD has not yet taken Ethan's life, he's still with us, but it has and continues to steal so much from him, from us, and from every family this disease touches. I am thankful that I am able to serve on the Board of Directors of the Global Foundation for Peroxisomal Disorders and that for years before we had ever heard of PBD-ZSD there were parents who were finding each other, networking, and looking for ways to improve the quality of life of their children. It is this groundwork that allowed for the formation of the GFPD in 2010.

Over the last five years there has been progress made. The scientists and researchers are gaining more of an understanding of this complex disease. There has been one clinical trial, although unfortunately the results were not what we had all hoped. There are PBD-ZSD fly models and mice models, there are cell lines that are being used for drug screenings, to see if there are any medications that have already been "invented" that might increase peroxisome function. There have been some potential leads, but honestly there is a lot of work to be done and at this time no one can promise us that a viable treatment option is going to be available within the next year or two years that has the potential to be a game changer for families who are given the news that their child has PBD-ZSD.

Last week a friend shared with me a blog post, "When Celebrities Suddenly Care about Rare Diseases" which spoke of the recent awareness and fundraising efforts for Batten Disease in light of the diagnosis of Hollywood producer Gordon Gray's two daughters. Many of his celebrity friends have rallied behind him in raising money for research in order to hopefully find a treatment that will save his children's lives. In many ways the attention that the Gray family and their friends have brought to Batten Disease is a really great thing. People are talking, people are fundraising, people are looking for a cure. However, before the Gray family ever heard of Batten Disease there were hundreds of parents, just every day people, who were fighting for their children and in memory of their children. Not all diseases have a celebrity backer. PBD-ZSD doesn't. Please know that I don't want any child or family to be impacted by PBD-ZSD, every diagnosis is heartbreaking. Yet, the awareness and funding for treatment options and a cure for diseases/disorders/conditions that have the support of a celebrity is something that frequently is what it takes to move the science forward.

However, grassroots movements can still be mighty. You only have to look at what is going on with American politics today to see that when a person believes in something or someone and there are enough like-minded individuals out there than when they unite they can become a force to be reckoned with. Will you join our family and the GFPD family in pushing for the development of effective treatment options and hopefully one day a cure? Will you share our story and the story of hundreds of other children with PBD-ZSD with your friends and family? Will you consider making a donation to GFPD and/or using your family/community network to put together an awareness and fundraising event/campaign? Ideas for "large" events: golf scramble, bowling tournament, family fun run/walks, dinner dance, charity concert, fishing tournament, art auction, etc.. Ideas for "smaller" events: pancake breakfast, spaghetti dinner, chili supper, fish fry, organize an evening with a local restaurant to give a percentage of sales to GFPD --- most people love to eat, and if they can help a good cause while doing it, is always a bonus!

We haven't held a Pancakes for PBD event in a couple of years and when we moved in July 2014 we no longer had the same community support that we had had. However, more than ever I believe that our family has to do more than what we are doing to help raise funds for research. In 2016, my hope is that we can host a Pancakes for PBD all you can eat pancake breakfast once again in at our church that Jeff's family and many of our friends still attend, perhaps in the late spring or early summer, and a Pasta for PBD all you can eat spaghetti dinner with a silent auction in the fall here in the community we live in now (ultimately I would like to see this be a Friday night event with a Saturday morning Pound the Pavement for PBD 5K Family Fun Run/Walk event the next day -- but that is going to take some time to become a reality). I would love to see these become annual events, with the goal of raising a minimum of $10,000 a year for GFPD that would be earmarked for research. We are going to need a lot of help to do this! If you would be willing to join with our family in these efforts, please let me know.

We have been so blessed by the support of our friends and family over the last five years. Many of you have generously donated to GFPD in honor of Ethan, have volunteered with and/or attended a past Pancakes for PBD event, purchased an Exercising for Ethan or a #PauseforPBD shirt, and/or lifted us up in prayer. We are so thankful for that, and can't imagine what the last five years would be like if we didn't have your love, support, and prayers.

Today, I find myself not wanting to just be a mom who is part of a foundation, but a mom on a mission to save my son's life. I hope you will join me!


Comments

Popular posts from this blog

"God Chooses Mom for Disabled Child" by Erma Bombeck

I don't think this is exactly how it works, but I had to share anyway. I believe with all my heart that God does not make mistakes, and although I will fully admit that it hurts my heart that any child is born (or develops) disabilities and/or life threatening illness, I know that God is sovereign and that He has a divine plan (even when we don't understand it!). I believe that God brought Jeff and I together and that He knew Ethan even as he was growing inside of me. While this is not the journey we would have dreamed of or chosen, and there are going to be times (and have been already) when we struggle, stumble and fall  it is our hope that in the end that we will bring glory and honor to the Lord.  God Chooses Mom for Disabled Child Written by Erma Bombeck Published in the Today Newspaper Sept. 4th, 1993 Most women become mothers by accident, some by choice, a few by social pressures, and a couple by habit. This year, nearly 100,000 women will become mother

Faces of Peroxisomal Biogenesis Disorders

This Saturday we are hosting the first annual Pancakes for PBD benefit.  All the proceeds will go to the Global Foundation for Peroxisomal Disorders to help offset the costs for our family and others like us to attend this summer's 2012 Family Support Conference in Orlando, FL.  We hope that this will become an annual tradition and allow us to help raise awareness and money for peroxisomal biogenesis disorders. Here are just a few of the faces of PBD. 

So much and so little to write about...

When I started this blog I don't think that I could have imagined a time would come in which months would go by without me writing. However, that is what has happened. So much has changed over the last almost eight years since Ethan's birth and subsequent diagnosis with PBD-ZSD, yet at the same time so much hasn't. Unlike parents of typically developing children we find ourselves still parenting a child with complex medical needs who's developmental skills range anywhere from 9 to 18 months. Ethan still has PBD-ZSD, and right now his uncontrollable seizures are a major issue. We still fight PBD-ZSD the best we can each day with a basket full of medications and supplements and other medical interventions (feeding tube, cochlear implant, AFOs, etc), numerous therapies, school (which Ethan loves), and prayer. We advocate for him and all children and families impacted by PBD-ZSD and related peroxisomal disorders through our involvement in the Global Foundation for Perox