{Guest Post} When Cooley’s Anemia Hits Close to Home

In this very special guest post, Bergen County, New Jersey residents — and mom a la mode family members —  Lisa and Joe Vigliotti share their first-hand experience of parenting a young child with a rare genetic blood disorder, Beta Thalassemia (Cooley’s Anemia).  Their 2 year old son, A.J. is an absolute ray of sunshine — you would have no idea what this child endures as he bravely battles his disease.  Please take a minute to learn more about Cooley’s Anemia, and what you can do to support A.J. and help him get closer to finding a cure…

Our son A.J. Vigliotti was born with a genetic blood disorder called Beta Thalassemia, also known as Cooley’s Anemia. This is a rare life long genetic blood disorder where A.J.’s little body produces deficient hemoglobin (which transports oxygen throughout the body) because it lacks enough beta protein.

To treat this disorder, A.J. and others affected with this disease, must undergo monthly blood transfusions and take a rigorous chelation medication, which reduces the level of iron in his liver and heart.  Whenever someone receives a blood transfusion, they also receive a large amount of iron into their body, which the body cannot remove it on its own.  Over time, with monthly transfusions, the level of iron in the liver, heart, kidneys and other major organs will cause them to fail.  For 30 years there was only one medication called Desferal, which is injected daily into the patient, and it is given over an eight-hour period via pump.  For the past ten years (2) oral chelation medications have become available but there are serious risks and side effects of the oral medications that we are learning about every single day.  The choice for a Thalassemia patient who wishes to chelate is to either have the needle and pump which is a relatively safe medication, or take the oral medication which most medical doctors will tell you they believe is safe.  Along with the medication and transfusions, A.J. receives monthly blood and urine monitoring, vision and hearing tests and medical appointments to ensure that the drugs are not damaging his organs and that his vision and hearing are not impaired. Even with a good treatment regimen, Thalassemia patients often suffer life-threatening complications and negative effects on their quality of life and their lifespan.

Thalassemia is an orphan disease as there are only about 1,000-2,000 people living with this disease in the United States but there are an estimated 60 to 80 million carriers of the Beta Thalassemia trait most of whom don’t even know it.  The issue is that there is not enough funding, knowledge or awareness about the disease because it only affects such a small percentage of the population that major drug companies and health care organizations lack the information and the tools to adequately treat the patients and families who deal with this disease every single day.

As we stated above, this is an orphan disease, but no less tragic or deadly as the Thalassemia community has seen in the last couple of months.  The community lost a 3-year-old girl, Zayna Connelly, to complications from RSV (the respiratory virus that is going around and can be deadly to children and the elderly) and her chelation medication.  The need for assistance and awareness is real, the need for funding is real and the timing is critical for the patients who battle this disease every day of their lives.

When we found out that A.J. had Thalassemia we felt as helpless as we have ever felt in our entire lives.  As a parent, we wanted to fix it immediately and we simply couldn’t and it almost broke us down.  The information that is available is antiquated and told us that A.J. would live to his late teens and would die, his bones would be deformed and he would have to endure blood transfusions and a lifetime of painful medical procedures.  Then one of our doctors told us to call the Cooley’s Anemia Foundation (“CAF”) and our whole outlook changed.  We went to the patient family conference in Philadelphia (all costs with the exception of the hotel was covered by CAF) with world-renowned specialists with the most up to date information and met people just like A.J. who had Thalassemia who were thriving and living very normal lives and coping with this disease.  The old information simply was wrong and unfortunately that is what most people think about Thalassemia when they do any research independently of CAF.

CAF is the only foundation in the United States that patients can turn to who have Thalassemia for the most complete up to date information on medical trials, medication, treatment and most importantly, hope.  The foundation holds an annual Care Walk to raise much-needed funds while serving as an opportunity to bring together the Thalassemia community and its supporters around the Country,

Our family, “Team A.J.”, participated last year and our community was so supportive of our efforts we raised an astonishing $25,000 in our first year!  We were compelled to do more and Joseph joined the CAF Board of Directors later in the year so that he could work with the other Board Members to bring the hope that we received from CAF to other patients and families out there who are struggling who may not even know about the Foundation.

We believe that NO ONE should suffer the consequences of this disease. We believe in a future in which NO ONE will have to endure thalassemia of any type. We hope and pray daily that a cure is found for him and for those patients and families who struggle with this disease every single day. For this reason, we are walking again in his honor in the 2015 Cooley’s Anemia Care Walk.  We are hoping to raise awareness and funds to continue the research to help A.J. live a full and active life; one that may no longer involve constant needles, hospitals, medications and the like.

Medical advances over the last 30 years have greatly extended lives and improved the quality of life for those with Thalassemia. Over the last few years, exciting developments have taken place regarding gene therapy that could hold hope for a cure for children born with this disease today and in the future. But there is still a long way to go. Thalassemia is a rare disease and it receives very little funding or support and thus research and progress toward curative therapies is painfully slow.

We have never been in a position like this before and we pray that no other parent has to ever be in this position.  As parents, we would move heaven and earth for our son and we are trying to do what we can do to make this happen but we are only two people and road is long and the work is great.  In the two years of A.J.’s life he has touched so many people and his smile truly lights up people’s lives.  He is a special child and has brought more joy into our lives that we ever thought imaginable.  We’ve been humbled and blessed by how many people have shown us that they care about A.J., about how thalassemia has affected our lives, and about the many others worldwide that are born with this devastating genetic illness and how they deal with it with grace and strength every single day.

John F. Kennedy once said in a speech “If a free society cannot help the many who are poor, it cannot save the few who are rich.”  We would take that a bit further and say that if our society cannot save the few who have this disease, we cannot save the many who are well.  There are people suffering and it matters not if it is 100 or 1,000.  If the only solution to this problem is money and awareness (which it is) we as parents have to do all we can to make sure that when our journey here on earth is over, we did absolutely everything we could to help alleviate the suffering of our son.  What parent would do anything else?

This year’s Care Walk will take place on Sunday, May 3, 2015. This is Team A.J.’s 2^nd annual walk. We decided to also host a Blood Drive at our walk! A.J. is living today because of those that selflessly donate their blood monthly. The need for blood is constant and critical. Every 2.5 seconds someone needs blood, a friend, a family member or maybe you. We have never before witnessed how real this need is until A.J. was born.

By supporting Team A.J….. whether you walk with us, donate toward our efforts, or spread the word about what we’re doing – you are helping us get closer to a cure for this disease. Together, we can do so much more to fight Thalassemia, than we ever could do alone.

If you are interested in joining us in our efforts, here is how you can help. Please visit www.tinyurl.com/carewalkTeamAJ and you can either:

WALK WITH US: Sunday, May 3, 2015 at Van Saun Park in Paramus, NJ. From 10:00 a.m – 2:00 p.m.

DONATE TO TEAM A.J’S EFFORTS:  Every penny we raise goes to The Cooley’s Anemia Foundation.

SPREAD THE WORD: Show us you care and help us raise awareness of this rare disease.

DONATE BLOOD: We will be hosting a Blood Drive on site from 10:00 a.m – 2:00 p.m.

Our sincere thanks to you in supporting this cause we hold so dear.

 

Love and blessings to you and your family,

– Joe, Lisa & A.J. Vigliotti

 

Care Walk is the Cooley’s Anemia Foundation’s annual fundraising event and opportunity to bring together the thalassemia community and its supporters around the country. The Care Walk on May 3, 2015 will raise funds to fight thalassemia.

To sponsor TEAM A.J. by check: Make check out to THE COOLEY’S ANEMIA FOUNDATION, and note: “TEAM A.J. 2015” on the check. Mail check to: The Cooley’s Anemia Foundation / 330 Seventh Avenue, #200 / New York, NY  10001

 

 

{mom a la mode was not compensated in any way for this post.  All opinions expressed here are those of the guest authors.}