Monday, May 30, 2016

Talk to Your Doctor! Fluoroquinolones (Cipro, Levaquin, etc), Collagen Damage Warnings

Classic picture of aortic disease:
 bulging, stretching, curving due to weak connective tissue
There is never a good time to be sick, but why is it that it always seems to happen at the worst time - in this case, a long holiday week end? Yes, this Memorial Day week end my husband's painful left ear sent us to urgent care for help. While there, we had a very important conversation about the right antibiotics for him.

Ear Infection

We don't know why, but this is the second year that my husband has developed ear infection for no apparent reason. He did not suffer with this in childhood, as many children do. Now with an impressive number of birthdays to his credit, what is this all about? He was born with a bicuspid aortic valve, but more than that, there are subtle signs of other "special" things through out his body. No doubt this will move us to investigate further, looking for a reason why this is happening.

Urgent Care

After examining my husband, the doctor explained to us what he saw inside the ear. His stethoscope to my husband's chest had already alerted him to the special sound of the artificial tissue valve in my husband's chest, and he understood the special risk of infection because of that valve. In our experience since the first valve was implanted 26 years ago, this is something that has always been well understood by the doctors he has seen.

"This is in the Cipro family"

As before, both an oral antibiotic and antibiotic drops were suggested. Two choices were mentioned regarding the drops. My husband asked a second time about this. And it was then that the doctor said those words, "This is in the Cipro family"!! I immediately mentioned the recent strengthening of the FDA warning announced on May 12, 2016.  I also said that I had recently discussed this with his aortic disease doctor. We did not want anything from this family of drugs! We settled on the same drops he had received previously, from a "mycin" family, which had not been a problem for him.

Speaking Up

Likely at the pharmacy, but certainly at home, we would have read the labels and caught this. It was so much better to have the discussion directly with the doctor and avoid dealing with it after the fact. It is important to realize that this family of drugs has been available for a long time, and the latest, strongest warning was just issued May 12th. So it is important for us, as the consumers, to be informed and speak up!

My husband is very careful about the medication given to him. Ten years ago, following his last surgery, despite being very unwell in many ways, he asked the name of the medication he was being given in the hospital each time, before taking it. Human beings are fallible, prone to accidentally making mistakes.

Be Informed

Read the May 12th FDA warning.

There is a full paper available to us, published by doctors from Toronto last fall. Those with BAV and other forms of TAD already have issues with the collagen damage/weakness in their aorta and other areas of their bodies. What would taking this drug do to them?

Read it yourself! Print it out! Share it with your doctors!

Fluoroquinolones and collagen associated severe adverse events: a longitudinal cohort study

Sharing information is powerful,
Creating a Climate of Hope.

~ Arlys Velebir
                        Bicuspid Aortic Founation

Thursday, May 12, 2016

What Happened to Amy?

This is Amy's story. Only her name has been changed, to protect privacy.

Where is Amy?

"It's just not like her, Amy would have let us know if she couldn't come!" And so a "welfare check" was requested. Her home was silent. There was no response to the doorbell, the knocks, the calls of her name. Something was terribly wrong!

They found Amy inside her home, lifeless. Apparently there had been no opportunity to even call for help.

What Happened to Amy?

Her family members were notified. The police arranged for her body to be taken directly to a local mortuary. Deeply shocked, her adult children needed answers. Their mother had been an active, independent, and apparently healthy woman. She did not even have high blood pressure. They thought she would be with them for many years to come. How could this happen, without warning?

 They needed answers.

In the place where Amy lived, no one offered to find those answers. After all, there was no evidence of a crime, no villain for the police to pursue. The death was clearly due to "natural causes". But, why? Is it really "natural" to die like this, without warning, when someone is so healthy?

Why Did Amy Die?

Amy's daughter told me that not knowing was unbearable to her family, much worse than the thought of an autopsy would be. Despite their tremendous shock at her sudden death, Amy's family found the strength and the financial resources to request a private autopsy. The local medical examiner was not available, and so Amy's family found their way to someone they describe as special, a compassionate, caring physician who carefully examined their mother's body and helped them understand.  What that physician found inside would have been a gruesome sight.

Quite simply, Amy bled to death. Unknown to her, she had an aortic aneurysm in her chest just above a bicuspid aortic valve in her heart. Because there was an autopsy, Amy's death certificate is accurate, stating that death was caused by the rupture of an aortic aneurysm in the presence of a bicuspid aortic valve (BAV).

Courage to Press for Answers

Sometimes families cannot bear the thought of an autopsy on their loved one.  There also may be veiled opposition, if the death occurs in a hospital, from a medical establishment that fears malpractice lawsuits. Sadly, the days of Dr. M.E. Abbott and Sir William Osler, who learned from autopsies and published extensively about aortic disease in the chest, are gone. As with Amy's family, it is the survivors' legal right to have an autopsy, but they may have to pay for it.

Knowledge - a Gift of Life 

Amy's family was left with the sad, painful task of sorting through her things, closing her home. She was an avid reader, and they found many articles about keeping healthy, preventing disease. Among them may have been the usual warnings about preventing stroke and "heart disease". Like many with BAV, Amy's arteries in her heart were perfectly clean. She was not at risk of a heart attack. Sadly, there was nothing about BAV and aortic aneurysm, the condition that suddenly robbed her of life. Amy would not have even heard about what snatched her life away. If her family had not arranged a private autopsy, the reason for her death and the potential threat to their own lives would never have been known by anyone at all.

Knowledge like this comes at an unspeakable price. It is a price too high, too great to pay. It should not happen.  There is a well known medical center not far from where Amy lived that could have performed her aneurysm surgery. They do this surgery frequently, perhaps almost every day.

Amy's blood relatives are educating themselves, being checked for BAV and/or aortic aneurysm. No one needs to tell them why now.

They know.

The Uncounted

The Bicuspid Aortic Foundation remains extremely concerned about the uncounted deaths due to BAV and aneurysm complications. Amy could easily have been one of those uncounted deaths, if her family had not had the courage and ability to seek the truth. Sudden deaths are too often simply recorded as due to natural causes, perhaps assumed to be due to a heart attack, but without any proof. 

The lack of understanding and recognition of aortic dissection/rupture deaths in the presence of BAV, as well as sudden aortic valve related deaths has consequences not just for those who die, but for the living.

Other blood relatives may be at risk. But there are also consequences for the entire BAV population. Because these people are uncounted, they are not found in the statistics, not included in the medical papers published about patients who receive treatment. How can we understand those most at risk, those who may never even reach a hospital, if we don't learn from them? We don't even know why they died! And so, the statistics upon which treatment of the living is based can not include these unknowns, perhaps the most vulnerable, the most fragile. Must they continue to die?

 Who Will Speak for Me?
Amy began this year, 2016, as we all did, with plans and hopes for her future. They were never to be realized, because she had been born with a bicuspid aortic valve, and over time an aneurysm developed. Today her voice is silenced. Who will speak for her, and the others we lose? 

At the Bicuspid Aortic Foundation we can help give them a  voice and bring into focus these most vulnerable. We are enlisting the aid of pathologists to do so.
They are the physicians who speak for the dead, and in doing so, may save the living. We are also encouraging families where sudden death has occurred to take courage and inspiration from Amy's story, and seek the answers they need.

In giving a voice to Amy,
And others we have lost,
May we help the living
And in their memory, 
Create a Climate of Hope.

- Arlys Velebir