Saturday, September 26, 2020

Reflections on aHUS Awareness Day

 aHUS Awareness Day is observed on 24th September every year. The date was picked by members of the aHUS Alliance, an umbrella group of patient organisations from around the world. It started being observed only 5-6 years ago as that was around when the Alliance was formed. Every year, a flurry of activity is seen around this date. People from all over the world participate in various activities like videos of hope by patients, dissemination of information among the public and a lot of education about the disease.

And yet, with every passing year, while some countries move from one successful drug to another, Indian patients continue to die or be condemned to a life on dialysis. Some do recover after the initial flare but these are only a small proportion of the total.

I started The Atypical HUS India Foundation a few years back with the intent of providing information and support to Indian aHUS patients and their family members. When I was diagnosed way back in 1997, there was hardly anything that was known about this disease. We scoured the internet only to find small bits of information here and there. I thought having a website and social media accounts that provided information and support to Indian patients would provide at least some hope.

I was only partially correct.

When people get diagnosed and come across the aHUS India Foundation, their first question is what is the solution? They somehow cannot get their heads around a diagnosis that has a cure but is not available in India. What are the other options, they ask? My heart sinks when I get this question because I hate to have to tell them that there are no other options. Is there a way to get Eculizumab to India? There are some shady companies that claim to be able to get it to India but the quality is doubtful and the price is beyond the reach of anyone but maybe the top 0.0001% of Indians.

The US is now moving on from Eculizumab to Ravulizumab. The infusion frequency is reduced and patients would need to go only once every 1-2 months, roughly half the number of visits that Eculizumab required. Other developed countries will soon evaluate this new drug as well.

Several other drugs are supposed to be in various stages of clinical trials. The concern however is even if these drugs get approved eventually (several years from now), will they ever be brought to India? How would they be priced?

The curse of this disease is many fold. On the one hand is the severity of the disease. On the other hand is the miniscule number of patients that exist. Any drug that pharmaceutical companies develop has only a few patients to profit from. So each patient is charged a bomb.

I hate to sound negative. But the situation is hopeless. Unfortunately, I just do not see things changing in the near future at all. In India, even basic healthcare problems are far for being resolved. Just to take one example, India's infant mortality stands at 32 per 1000 births compared to the US's 5.7 and the UK's 3.9. This is hardly surprising when you see that India spends about 3.6% of its GDP on healthcare (of this, government spending is a shocking 1.3%) while the US spends about 18% and the UK spends about 10%

So, the problems are aplenty and the resources are few. In this dire state of affairs, how can we expect the Indian government to spend massive sums of money on drugs that will save only a few lives? If the money spent on one patient's cost of Eculizumab is spent on improving infant mortality for example, by strengthening the primary healthcare centre infrastructure in a rural village, several hundred lives can be saved. Which investment offers a better return?

And yet, try explaining that to Ananya's father. He has no clue about GDP figures and healthcare budgets. All he knows is that he lost his beautiful little daughter when there was a drug available that could cure her. But it was simply an accident of geography coupled with a cruel accident of genetics that took her away forever.

Wednesday, September 2, 2020

The Secret Recipe to make the Best Idlis ever

 I love Idlis. The best part about this wondrous dish from South India is its simplicity. When I rave about the beauty of an Idli, people who are not as fascinated by it as much as I am wonder what all the fuss is about? But, the craze for Idlis is something that has been passed on to me through my genes. My family is equally crazy about Idlis as I am. Which is why, when my niece, Nidhi says she is not too find of Idlis, it makes me wonder, was there a mistake in the hospital after she was born? I mean, you know, mix-ups, exchanges and so on?

My aunt, Rita makes the finest Idlis. Her Idlis are something else entirely. When she calls us over for Idlis, I am almost tempted to skip a meal or three in anticipation. She probably got the recipe from her mother-in-law, who stayed in Chennai for a better part of her life and was a legendary cook herself. 

When I had quite perfected the art of making a decent sourdough loaf, my attention turned to Idlis. To me, this was the next culinary bastion to be conquered. I started doing my research. I spoke to many people who made good Idlis including of course, my aunt. I scoured the internet for recipes and methods. I even tried reading up about the science of Idlis.

The variation in the proportion of Urad Dal (Split and Skinned Black Gram) to rice was so dramatically different from person to person that I was at a loss as to whom I should follow. I decided to use my aunt, Rita's 1:2.5 proportion. For every cup of Urad Dal, it was 2.5 cups of rice. This was a hugely successful recipe, so why bother experimenting, I thought?

The quality of the ingredients is very important. Only whole Urad Dal is to be used. The rice is also called Idli Rice and is a form of parboiled rice. Both these ingredients are available in most supermarkets. While there seems to be less variation in Urad Dal the Idli Rice is another story altogether. Different shops have different qualities and you will need to try out a few before you finalise which one you will use and stick to it. Unfortunately, experimenting is the only way, here. In Hyderabad, where I stay, Ratnadeep stocks a decent Idli Rice and so does the P Store in my apartment complex.

Wet grinders give the best results for grinding for Idli. While theoretically you could use a regular mixer-grinder, the results are very disappointing.

One more trick which many people use these days is to put 1 tablespoon of soaked methi seeds (fenugreek) in the grinder before grinding the Urad Dal. Yet anther trick is to put a handful of white rice cooked the previous day of grinding into the grinder while grinding the Idli Rice or to grind a handful of poha (puffed, flattened rice), mix it in water quickly and pour the resultant paste into the grinder while grinding the Idli Rice.

The final trick is to NOT mix the fermented batter. This was a big mistake I was doing until I read about it on the internet here. The key is to mix salt after mixing both the ground Urad Dal and Idli Rice and then allowing it to ferment to not necessitate mixing the batter at all after the process. Simply put a spoon and take small portions of the fermented batter from top and put in the Idli Plate that goes into the Idli steamer. Keep taking such portions right down till the end. Never mix. This, honestly, is the game changer. I had tried the entire recipe as explained above for months. I got Idlis that were good but nowhere close to my aunt's. With this step, things went up several notches.

Try to finish off the Idli batter in a couple of days. Every time you keep it in the fridge, the quality of the Idlis will deteriorate a little.

Here's the full recipe:

Ingredients:

1 cup whole urad dal

2.5 cups Idli rice

1 tablespoon methi seeds

1/2 cup poha

Salt to taste

Method:

1. Wash the urad dal couple of times and soak in plenty of water (at least double the quantity). Wash the Idli rice 3-4 times and soak in water. Soak the methi seeds. Soak all this overnight.

2. Start wet grinder and pour the methi along with the water. Grind for 4-5 minutes. Then while the ground methi is in the grinder, start the grinder again. Drain the Urad Dal and put into the grinder slowly. Add small quantities of water (few table spoons every time) to ensure that the batter does not become thin. It should be like a thin paste. Too much water can mess up the Idlis. My experience is about 1 cup of water over the entire process but you should experiment with this to get the quantity right. Grind for about 30 minutes.

3. Remove the ground urad dal and then put the Idli rice into the grinder after starting it. Quickly grind the poha, mix with the water and immediately put it into the grinder. Allowing it to stand will cause the whole thing to become very gelatinous.  Grind for about 6-7 minutes only.

4. Remove the ground Idli rice and mix with the ground urad dal. Add salt to taste.

5. Allow to ferment. 12-24 hours depending on the ambient temperature.

6. Do not mix the fermented batter. Take a wet muslin cloth and place on the idli plate. Scoop out the batter, one by one and put it on the cloth. Steam for 7 minutes.

7. Serve with ghee, various chutneys, karam podi and sambar.