Egyptian child with SMA receives most expensive medicine in world

At $2.1 million per one-time use, Novartis' Zolgensma is the world's most expensive medicine. (Novartis)
At $2.1 million per one-time use, Novartis' Zolgensma is the world's most expensive medicine. (Novartis)
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Updated 05 March 2021

Egyptian child with SMA receives most expensive medicine in world

Egyptian child with SMA receives most expensive medicine in world

CAIRO: Egyptian doctors have succeeded in treating a child with Spinal Muscular Atrophy (SMA) using the most expensive drug in the world, Zolgensma.

Nagia Ali Fahmy, professor of neurology and director of the Muscular and Neurology Unit at Ain Shams Medicine in Egypt, explained that Zolgensma, which has a value of $2.125 million per dose, is the first gene therapy of its kind in the world given to a patient intravenously in a single dose.

The drug was approved in May 2019 by the US Food and Drug Administration.

She added that the manufacturer, Novartis, offers 100 opportunities to obtain the drug free of charge in countries where it has not yet been registered, setting conditions for choosing the children who receive it, including that they should not be over two years old, and their mutation should be in the first gene. Accordingly, Ain Shams Medicine made eight applications for cases under their care, and Novartis selected one, a boy named Rayan from Alexandria, who is turning two in a few days.

SMA of the first and second types leads to the death of the child during the first two years of life as a result of the failure of respiratory functions.

Zolgensma was first clinically tested several years ago, and the first child to receive it is now five years old.

The drug treats breathing functions and motor impairment, and puts the child on a path to normal growth.

But the improvement happens gradually, during which physiotherapy and pulmonary rehabilitation therapy are performed.

Hani Aref, head of the neurology department at the Faculty of Medicine at Ain Shams University, said that SMA happens due to a genetic defect, as there is a defective gene in the body that does not allow the secretion of proteins responsible for feeding the cells connected to the muscles.

“This disease results in gradual, severe muscle weakness and it is divided into three stages depending on the severity in the gene,” he said.

“The first stage affects children immediately after birth in which the child’s condition is very difficult and the atrophy affects the breathing muscles gradually, which leads to death.

“The second stage affects children six months after their birth, and the third stage affects the child at an advanced age and results in severe muscle weakness,” he added.

“Symptoms begin with great difficulty moving, and the child cannot acquire motor skills; if he gains some of them, he will gradually lose them. Most of the children suffering from the disease are put on ventilators, but they eventually die.”


Where We Are Going Today: Khosh Hal

Where We Are Going Today: Khosh Hal
Updated 15 May 2021

Where We Are Going Today: Khosh Hal

Where We Are Going Today: Khosh Hal

Khosh Hal restaurant in Jeddah’s Aziziah district offers authentic Pakistani flavors with a modern twist.

The menu features traditional dishes such as mixed grill platter, butter chicken, mutton kunna and Khosh Hal’s special chicken biryani.

Freshly made bread includes naan, roti, tandoori paratha and the hot and delicately crispy purri — a fried bread eaten with different stews.

Pakora, one of the most popular Pakistani appetizers, is a deep-fried combination of chickpea flour, onion, green chilli and dry coriander.

After a meal of rich flavors and spices, diners can enjoy a refreshing kulfi, a delicious drink or dessert flavored with orange, mango, strawberry or coconut.

For more information visit Instagram account: @Khoshhalrstaurant.


What We Are Eating Today: The Chimny

What We Are Eating Today: The Chimny
Updated 14 May 2021

What We Are Eating Today: The Chimny

What We Are Eating Today: The Chimny
  • The Chimny is perfect as a special Eid treat or as a birthday indulgence

The Chimny is a Saudi ice cream brand that offers a new concept in ice cream cones, inspired by the traditional Hungarian pastry, the “kürtőskalács,” or “chimney cake,” popular across Eastern Europe.

The ice creamery offers ice cream scoops in the pastries as a substitute for the traditional wafer cone. Made by hand, consisting of dough strips rolled around a wooden dowel, moistened with melted butter, coated in granulated sugar, it is baked until it has a golden-brown color.

The cones are filled with soft ice cream in a variety of flavors with different sauces and toppings such as marshmallows and sprinkles.

You can enjoy eating the freshly baked cones with or without ice cream scoops in many flavors, including date, cheesecake, apple pie, banana, and more.

The Chimny is perfect as a special Eid treat or as a birthday indulgence, as it offers catering service for parties.

For more information visit the Instagram account: @thechimny.


Eid treat: Moroccan chef Nargisse Benkabbou’s chocolate chip krachel

Eid treat: Moroccan chef Nargisse Benkabbou’s chocolate chip krachel
Chocolate chip krachel. Supplied
Updated 12 May 2021

Eid treat: Moroccan chef Nargisse Benkabbou’s chocolate chip krachel

Eid treat: Moroccan chef Nargisse Benkabbou’s chocolate chip krachel

DUBAI: Moroccan chef Nargisse Benkabbou is on a mission to demystify her country’s cuisine for international foodies, especially those in the UK where she lives.

Here, she shares her recipe for chocolate chip krachel to sweeten up your Eid Al-Fitr celebrations.

Ingredients:

60g unsalted butter

2 ½ tbsp sesame seeds

2 tsp dried active yeast

50g caster sugar

1 tbsp warm water

300g plain flour, plus extra if needed and for dusting

1 tsp aniseed

½ tsp salt

1 egg

80–120ml warm full-fat milk

1 ½ tbsp orange blossom water

100g dark chocolate chips or chunks

Vegetable oil

1 egg yolk, beaten

 

Instructions:

1.      Melt the butter, then leave it to cool. Toast the sesame seeds in a dry pan over a medium-high heat for about 6 minutes.

2.      In a small bowl, mix the dried yeast with 1⁄4 teaspoon of the sugar and the measured warm water using a fork. Leave the yeast to activate for about 5 minutes.

3.      Mix 2 tablespoons of the toasted sesame seeds, the remaining sugar, the flour, aniseed and salt together in a large bowl. In a separate bowl, whisk the melted butter, yeast mixture, egg, 80ml warm milk and the orange blossom water together until smooth. Combine both bowls and mix to form a soft dough.

4.      Lightly dust a work surface with flour and knead the dough for about 10 minutes until smooth and elastic and add the chocolate chips. Form the dough into a ball and place in a lightly oiled bowl. Cover with cling film and leave to rise for about 45 minutes or until it almost doubles in size.

5.      Divide the dough into 8 equal-sized pieces and shape each into a ball. Place them on a baking sheet, leaving about 5cm between each ball. Cover with cling film and leave the buns to rise for about 30 minutes.

6.      Preheat the oven to 190°C. Brush the buns with the beaten egg yolk and sprinkle with the remaining toasted sesame seeds. Bake for about 17–20 minutes.


Burj Al-Arab’s SAL: Come for the views, stay for the food at this Dubai hotspot

Burj Al-Arab’s SAL: Come for the views, stay for the food at this Dubai hotspot
The restaurant overlooks the main star of the venue, the 100-meter infinity pool. Supplied
Updated 10 May 2021

Burj Al-Arab’s SAL: Come for the views, stay for the food at this Dubai hotspot

Burj Al-Arab’s SAL: Come for the views, stay for the food at this Dubai hotspot

DUBAI: First things first: If you’re familiar with the Instagram account @influencersinthewild, then chances are you’ll spot similar examples of the content it posts at Burj Al-Arab’s SAL. Because for every few individuals who are at the venue for the resplendent views and excellent hospitality, there’s that one “influencer” at work, taking part in an impromptu photo shoot. It would be extreme to claim that they’re ruining the experience for everyone else, but you’ll probably pause and have a chuckle or two at the confidence of it all.

That being said, to reduce SAL to a social media “hotspot of the moment” would be doing it a disservice because judging by our own experience, there’s so much more to it than a backdrop for Insta-models. Jumeirah Group describes SAL, which opened in the second half of last year, as a “chic lifestyle experience at Burj Al Arab where barefoot luxury at an iconic pool and beach destination meets culinary excellence in a striking new dining venue.” With hues of blue forming the picture-perfect landscape, the restaurant overlooks the main star of the venue, the 100-meter infinity pool.

Jumeirah Group opened SAL in the second half of last year. Supplied

However, we’re here to dine, not dip (into the pool), and our entire meal was a feast for the eyes and the stomach. Headed by Culinary Director Marco Garfagnini, SAL’s menu “pays homage to the sea,” with the majority of the dishes made for sharing.

Greeted by a very welcoming and friendly team of hosts, we were guided to our seats before being offered a detailed run-through of the menu.

The heirloom tomato carpaccio, feta cheese and black olives was laid out on a platter in a pretty pattern. Supplied

To start, my dining partner and I opted for the heirloom tomato carpaccio, feta cheese and black olives, and the tuna tartare with caviar. As we waited, we were served water from freshly cut coconuts.

Once our appetizers arrived, it became clear that our phone photography would be reserved for the dishes and not only for the view. The carpaccio was delicately laid out on a platter in a pretty pattern, while the tartare was served in a perfect circle with a dash of gold dusting on top.

Tartare is served in a perfect circle with a dash of gold dusting on top. Supplied

The tartare was divine. Its citrus-based Ponzu sauce marinade offered the perfect balance, “cooking” the fish to eliminate any offending aftertaste sometimes experienced with tartare. In fact, it was one of the best tartare dishes I have ever sampled.

The main course certainly had a lot to live up to, bringing us to the first — the recommended Portuguese dourada, a gilt-head sea bream fish common in the Mediterranean. At SAL, it’s served baked with tomatoes and potatoes. Again, absolutely flawless. Its cod-like meatiness paired with a spinach-infused sauce made for a moreish main that was delightful on the palette.

The Portuguese dourada made for a moreish main that was delightful on the palette. Supplied

Another fish dish we were recommended was the sea-salt-crusted seabass for two, but since my guest isn’t the biggest fan of seafood, we opted to try the third recommendation — the stone-oven-baked lemon chicken empanada, with French fries and baby spinach salad. Now, SAL likes to entertain guests, so similarly to the seabass, this one is carved and served right in front of you. Given the show, we were expecting something big, but if there was one dish that was more “meh” than “marvelous,” it was this one. It was underwhelming after it was cut open and presented, looking more like some lemon chicken in pita bread rather than a baked turnover. It still tasted delightful, but we both agreed that if we could, we would advise the restaurant to serve it as is, as it looked much more appealing at the start.

The stone-oven-baked lemon chicken empanada is carved and served right in front of you. Supplied

It was nearly time to wrap up, and as much as we wanted to go for something sweet, we were simply running on full. One lovely staff member did try to tempt us with the dessert trolley offering a selection from the restaurant’s master pastry chefs, but we stayed strong. In the end, we were presented with a cute little meringue-based dessert topped with fresh berries — just the right amount of sugar to conclude our visit with.

Needless to say, this Burj Al-Arab beach restaurant is definitely one to add to your must-visits. As someone who has been based in this region for many years, this was easily one of the best meals I have had in a long time. 

I don’t make that claim lightly.

A little meringue-based dessert topped with fresh berries. Supplied

Info:

SAL @ Burj Al Arab

jumeirah.com/SAL

 


Maamoul: Why the storied sweet is so important during Eid in Palestine

Maamoul: Why the storied sweet is so important during Eid in Palestine
Maamoul and cakes are one of the most prominent pieces of celebration associated with Eid Al-Fitr, despite the harsh conditions faced by Palestinians. (Shutterstock)
Updated 10 May 2021

Maamoul: Why the storied sweet is so important during Eid in Palestine

Maamoul: Why the storied sweet is so important during Eid in Palestine
  • Prepared in circles, the Eid cakes are stuffed with dates, while maamoul are stuffed with dates or walnuts, pistachios and nuts, and the outer layer is sprinkled with crushed white sugar

GAZA CITY: In the last week of Ramadan, the smell of maamoul and cakes wafts from Palestinian homes in the Gaza Strip. Maamoul, also popular in Lebanon, Syria, Jordan, Egypt and Iraq, is a traditional shortbread cookie popular in the region, and one of the main sweet items prepared for Eid Al-Fitr celebrations.
Samira Al-Burai, 54, is enjoys preparing maamoul with her sons and daughters.
“We bring basic ingredients a few days before making maamoul. All the family members, including my sons, will participate in making it.
“I learned (how to) make cakes and maamoul from my mother, then I taught it to my daughters so that this tradition may continue during the last days of Ramadan. My children are accustomed to the smell of cakes at this time of every year.”
Maamoul and cakes are one of the most prominent pieces of celebration associated with Eid Al-Fitr, despite the harsh conditions faced by Palestinians.
Prepared in circles, the Eid cakes are stuffed with dates, while maamoul are stuffed with dates or walnuts, pistachios and nuts, and the outer layer is sprinkled with crushed white sugar.
Some women earn money during Ramadan by making and selling maamoul to others.
Salwa Kabariti, 57, used to make them for her family. With the passage of time and after they fell on hard times, she began to produce larger quantities and started selling to neighbors, friends and even to some shops.

SPEEDREAD

Maamoul and cakes are one of the most prominent pieces of celebration associated with Eid Al-Fitr, despite the harsh conditions faced by Palestinians. Some women earn money during Ramadan by making and selling maamoul to others.

“Due to our poor economic condition, I began searching for a source of income. This work offered a good source. It helped me and my family to overcome our economic crises,” Kabariti said.
“There is no Eid without maamoul. I love (it) and will continue making it every Ramadan as long as I have the ability to do so,” she added.
Despite the large number of bakeries that sell maamoul in the Gaza Strip, many women prefer making theirs at home to preserve the festive atmosphere in their households.
Lubna Al-Sumairi, 40, said: “I like preparing it in my house with my husband and other family members. Making maamoul is one of the most important customs that we enjoy during the
last days of Ramadan; its preparation, delicious taste, and the pleasant atmosphere gives us a happy feeling.”