Thursday, September 24, 2015

Today is aHUS Awareness Day!

Today is aHUS Awareness Day. What does it mean for us in India? Statistics estimate that 1-2 in a million have atypical HUS. In India, that comes to about 1000-2000 people. Possibly, less than 10% (about 100-200) of those actually have been diagnosed. Less than 1% are probably receiving any treatment. This means that about 10-20 people are possibly receiving any treatment for this. How successful the treatment is unknown.

In a country like India where the Government is bogged down with many issues, something that affects about a thousand people would be very low on the priority list; even if all those thousand people eventually died. Many times that number die of other causes every day. Many of those causes are things that the Government can do something about quite easily.

What then should those thousand aHUS patients do? 

It is very frustrating that we live in a country where there is no access to the drug eculizumab (that is available elsewhere) that would give us an excellent chance at living a normal life without the sword of kidney failure hanging over our heads. If you are a newly diagnosed aHUS patient, you can avoid any damage to your kidneys and the rest of your body by using eculizumab. If you’ve been diagnosed long back and are on dialysis currently, you can undergo a successful kidney transplant by using this drug. Without this drug, there could be rapid progression to ESRD for newly diagnosed patients and recurrence of HUS and loss of the transplanted kidney if you underwent a kidney transplant.

The question that is probably popping in your mind right now is why don’t we have the drug in India and why can’t we get it to India from where it is available? The answer to both these questions is the cost of the drug. Eculizumab has been declared by Fortune magazine as the costliest drug in the world today. In Indian rupees the cost of one year of treatment currently could be a couple of crores. And you need to take the drug lifelong as per current protocols. The Indian government does not currently have a Universal Healthcare Coverage policy. Insurance companies have a yearly cap that would never touch that figure and maybe only a minuscule number of patients can afford that themselves.  

The good thing is that there are more options coming. As of today, there are five new complement inhibitors (the class of drugs to which eculizumab belongs and which can potentially have the same effect as eculizumab). More options is always a good thing. This would bring down costs and improve availability as well. How soon these drugs would be available? How much would the alternatives cost? How would they be made available in India? Well, the answers to these questions are not known. Your guess is as good as mine!

Future generations of aHUS patients would definitely have better options. For the current generation, the options could be limited for some time to come. 

It is extremely important for us to create awareness about this horrible disease. Without more awareness, people who matter will not even have this on their radar. We need to make some noise so that people at least start talking about this. The ice-bucket challenge did a lot for ALS. Making a noise does help. If the Government gets to know about this problem and has it on their minds while drafting any healthcare policy, there could be a small chance that we get some kind of help in terms of laws or policies that might help us. If not us, at least future generations of aHUS patients will have treatment options. We owe them that much.

To all those who are affected by this disease, here is a quote (modified slightly) from the film "Independence Day":

"We will not go quietly into the night! 
We will not perish without a fight! 
We're going to live on! 
We're going to survive! 
Today we celebrate our aHUS Day!"

Friday, September 4, 2015

A rough couple of months

Things have been a little rough on my health front for the last couple of months. It all started when I got back from London. I had some breathlessness a few days after I got back. I underwent a Coronary Angiogram (done by my classmate and now famous cardiologist, Dr. Anuj Kapadiya) which showed Ectatic Coronaries with Slow Flow. Thankfully there were no blocks. I was put on some blood thinning medication.

However even a month and half after the medication, the breathlessness would return for a few hours or sometimes even a day. I went back to my nephrologist and cardiologist. They did another 2D Echocardiogram. That showed something called "Regional Wall Motion Abnormalities in the Right Coronary Artery Region" along with "Akinesia in the Basal Inferior Left Ventricle". Well, that meant some small problem in the heart. I have had Grade 1 Diastolic Dysfunction for some time now and they wanted to do a Stress Echo to check if that was worsening when I exercised. The test came out ok.

I also consulted the amazing Dr. John Agar of Australia at the Home Dialysis Central Forums. Dr. Agar believed it could be fluid hidden in my body. He suggested that I remove an extra 250 ml every other day and see how I felt.

I have started doing this. I am down about 1.3 kgs from my earlier dry weight without any symptoms. So, it could be the answer. But in kidney disease, you never know until you actually know! So, I am hoping this settles soon.

This has hampered my QoL a lot. I have not been able to swim or travel, both of which I enjoy greatly.

Life with kidney disease is never smooth sailing for too long. You keep having issues on and off. It is therefore always important to try to be in control and do what's advised so that you can at least try and prevent the things that are preventable.