Sunday, February 19, 2017

Recipes I'd like to try - sweet stuff, soup and bread

Raspberry Hungarian Pastry

Makes 25 servings of 2 1/2-inches square
3 large eggs, separated 
3/4 cup unsalted butter, softened 
3/4 cup sugar
1 1/2 teaspoons vanilla
2 1/4 cups flour
1/4 teaspoon baking soda
3/4 teaspoon baking powder
1/2 teaspoon salt
1 cup raspberry jam
1 cup finely chopped walnuts, divided
Preheat the oven to 300ºF. Prepare a 10-x-15-inch jelly roll pan by greasing lightly with cooking spray or butter.
Whip the egg whites with an electric mixer until they form stiff peaks. Gently transfer to a separate bowl and set aside.
Use the mixer to cream the butter and sugar. Add vanilla and egg yolks, and beat for a full three or four minutes, until their volume has increased and the mixture is yellow and smooth.
Add the flour, baking soda, baking powder, and salt. Stir into the butter mixture with a wooden spoon or your hands. Pat it out in the prepared pan, crimping it into the corners; if the dough is a little crumbly just press it in firmly.
Spread the dough with jam; sprinkle 1/2 cup ground walnuts over the jelly and spread beaten egg whites evenly over the nuts. Sprinkle the remaining nuts over egg whites.
Bake for 30 minutes. Watch carefully and avoid over-baking the egg white topping. It should be crisp but not really brown at all. The crust should be just barely golden brown.
Cut into squares when cool. Store in a closed container at room temperature for up to 5 days. These are best on the day they are made, as the egg white topping softens after a day.

Raspberry-Coconut French Macarons

Makes about 48 macarons
For the shells:
120 grams ground almond flour
228 grams powdered sugar, sifted
2 teaspoons freeze-dried raspberries, finely ground
140 grams egg whites
70 grams granulated sugar
Pinch cream of tartar
Pink gel food coloring (optional)
For the filling:280 grams good white chocolate, chopped
1/2 cup whole-fat coconut milk
1 teaspoon coconut extract or emulsion (optional)
For the shells:Cut a piece of parchment paper to fit inside a baking sheet and draw 1 1/2- to 2-inch circles on the parchment. Flip the paper over so that the ink or pencil markings are not on the cooking surface, but are still visible through the paper. Alternatively, cover the parchment with a Silpat, but make sure you can still see the circles through the Silpat.
Sift together the ground almond flour, powdered sugar, and powdered freeze-dried raspberries in a large mixing bowl.
Place the egg whites in the bowl of a stand mixer fit with a whisk attachment. Begin whipping egg whites on medium speed until they start to foam. Add the granulated sugar and turn mixer up to medium-high speed for about 5 minutes. Once egg whites are fairly fluffy, add the cream of tartar and turn up to high speed. Continue for another few minutes until the egg whites hold stiff, but not dry, peaks. (See this post for more details on whipping egg whites.) Be sure not to overmix or the meringue will start to break down. Add the food coloring during the last minute of mixing.
Add half of the meringue to the dry mixture. Using a rubber spatula, give the mixture about 5 big folds (see this post for more details on folding). Add the remaining meringue and continue to fold. Begin smoothing out the batter by pressing the mixture around the sides of your mixing bowl while turning the bowl and knocking a bit of the air out of the meringue. Continue to fold the batter up and over from the bottom of the bowl, making sure there are no dry clumps left behind. Mix until the batter makes thick, lava-like, ribbons. Do not over-mix.
Fit a pastry bag with a plain, round piping tip (about 1/2-inch wide). Fill the pastry bag with batter and carefully pipe out dollops of batter onto the parchment, using the circles as a size guide. Once one tray is full, set the pastry bag aside. Rap the baking sheet against the work surface 2 to 3 times to eliminate any trapped air bubbles.
Let macarons rest for about 20 minutes, or until the tops of the rounds are dry and not sticky to the touch. Meanwhile, preheat oven to 325°F. Bake the macarons for 9 to 10 minutes. Check to see if they are done by gently wiggling the top shell. If the shells feel attached, they are done. If not, bake for 1 to 2 minutes longer. Let cool slightly before removing from the parchment or Silpat. Match together similar-sized shells and set aside.
For the filling:
Place chopped white chocolate in a heat-safe bowl. Heat the coconut milk in a small saucepan over medium-low heat. Bring the coconut milk to a slight simmer and then pour over the chocolate. Let sit for 15 to 30 seconds before whisking to combine; the warm coconut milk will melt the chocolate. Add the coconut extract, and mix until smooth.
Let filling thicken at room temperature or chill in the refrigerator. Once filling is cool, transfer it to a small pastry bag fitted with a small, round tip. Pipe filling on the half of the shells. Top filling with the second shell.

Recipe Notes

  • The most accurate way to make macarons is to measure by weight. The raspberry powder is very light, and might not be picked up well by all kitchen scales, so it is measured in teaspoons.
  • Be sure that your oven is calibrated or you are using an oven thermometer. Oven temperature is vital to baking times.
  • When mixing the batter, it is always best to test it early rather than after it is already overmixed. When batter is smooth yet still thick, take your spatula and create little peaks in the batter. Wiggle the bowl around and see if they fall back into the batter. If they are still standing, then the batter is under-mixed. If they fall back slowly, the batter is just right. If they disappear immediately, then your batter is overmixed.
  • If shells are slightly overbaked, do not worry. Fill shells and refrigerate in an airtight container overnight. The flavors will mature and the filling with help soften up the shells. In fact, some recommend this maturing process for all macarons. Bring back to room temperature before serving.

Pumpkin Snickerdoodles

Makes 18 cookies
6 tablespoons unsalted butter, softened
1/4 cup pumpkin puree
1/2 cup sugar
1/2 cup brown sugar
2 large eggs
2 cups all-purpose flour
2 teaspoons cream of tartar
1 teaspoon baking soda
Pinch of salt
1/4 cup sugar
1 tablespoon cinnamon
1 teaspoon ground nutmeg
Preheat your oven to 325°F. Line a baking sheet with parchment or a Silpat.
In the bowl of a stand mixer (or in a large bowl with a hand mixer), cream together butter, the sugars, and the pumpkin puree until it's smooth. Mix in the eggs one at a time.
In a separate bowl, stir together flour, tartar, baking soda, and salt. Slowly stir in the dry ingredients into the creamed mixture. Mix it just enough to combine the ingredients, but try not to over mix the cookie dough.
In a small bowl, stir together sugar, cinnamon, and ground nutmeg.
Measure out heaping tablespoons of dough. Roll the cookie dough into a ball and then roll it in the sugar mixture. Place the rolled cookie on the baking sheet. Repeat until the dough is gone. You should get about 18 medium-sized cookies out of the batch.
Bake the cookies for 11 to 12 minutes until they are slightly puffed and golden brown around the edges. Let the cookies cool before serving. Keep extra cookies in an airtight container for up to 3 days.

Recipe Notes

Gluten-Free Pear Tart with Walnut Crust & Frangipane

Serves 8
For the crust: 1 cup (120 grams) all-purpose gluten-free flour blend (See Recipe Note)
3/4 cup (90 grams) walnut pieces
1/2 teaspoon kosher salt
1 stick (4 ounces) unsalted butter, frozen and coarsely grated
3 tablespoons ice water
For the poached pears: 2 cups water
2/3 cup granulated sugar
1 teaspoon ground cinnamon
1/2 teaspoon ground ginger
1/2 teaspoon ground vanilla bean or vanilla bean paste
2 whole D’Anjou or Bartlett pears, slightly underripe
For the frangipane:1/2 cup (100 grams) granulated cane sugar
1/2 cup (60 grams) walnut pieces
4 tablespoons (2 ounces) unsalted butter, softened
2 large eggs, warmed to room temperature
1 1/2 tablespoons all-purpose gluten-free flour blend
Special Equipment: 14- x 4.5-inch rectangular tart pan with a removable bottom (See Recipe Note)
To make the crust: In a food processor, combine the flour, walnuts, and salt. Process until the walnuts are thoroughly ground into the flour, about 10 seconds. Transfer the mixture to a bowl and chill in the freezer for 30 minutes.
Return the flour mixture to the food processor and add the grated butter. Process until butter is incorporated but little flecks remain throughout, about 5 seconds. Add the ice water and pulse briefly, just until the water is distributed evenly and the dough begins to come together into large pieces.
Turn the dough out into a 14- x 4.5-inch rectangular tart pan with a removable bottom. Use your fingers press it into the pan evenly, making sure to get it firmly into the corners. Do this quickly to avoid warming the dough.
Place a piece of parchment paper on top of the crust, then pour in a single layer of pie weights or uncooked beans. Refrigerate for 20 minutes.
While the crust chills, preheat the oven to 375°F.
Place the tart pan on a baking sheet lined with parchment paper or aluminum foil. Bake until the butter in the crust has begun to steam and bubble a bit around the edges, about 12 minutes. Remove from the oven, remove pie weights and parchment, and allow the crust to cool to room temperature.
To make the poached pears: Peel the pears, then cut them into quarters and remove the cores. In a medium (2-quart) saucepan, heat the water, sugar, spices and vanilla over medium-low heat, stirring until the sugar has dissolved completely.
When the sugar has dissolved, add the quartered pears. Bring up to a rapid simmer, then turn down to low and simmer, covered, until the pears are cooked through but still firm, about 10 minutes.
Use a slotted spoon to transfer the pears to a wire rack. Let them cool until you can handle them comfortably. Slice each pear quarter into 1/4-inch thick wedges.
To make the frangipane: In a food processor, combine all ingredients and process into a thick, smooth batter.
To assemble the tart: When ready to bake, preheat the oven to 350°F.
Place the crust in its tart pan on a baking sheet lined with parchment or aluminum foil. Pour the frangipane batter into the cooled crust. If you made the frangipane in advance and kept it chilled, it will have thickened slightly — use an offset spatula to spread it into an even layer. Gently layer overlapping slices of pear down the middle of the tart. Don’t worry if the pears sink into the batter a bit — the batter will rise and puff up around them as it bakes.
Bake the tart until the frangipane is golden brown and puffy, 45 to 55 minutes. Remove it from the oven and allow it to cool completely.
To unmold the tart, push the base of the pan up from the bottom and put aside the frame. Leave the base of the pan underneath the tart and carefully slide it onto a serving plate. Cut into slices and serve with whipped cream or vanilla ice cream and a sprinkle of cinnamon, if desired.

Key Lime Pie

Makes 1 pie. 
Adapted from Martha Stewart.
1 1/2 cups graham cracker crumbs
1/2 cup unsalted butter, melted
2 tablespoons sugar
Pinch salt
1 14-ounce can sweetened condensed milk
4 large egg yolks
1/2 cup Key lime juice, from about 12 limes
2 teaspoons Key lime zest
Whipped cream for garnish (optional)
Heat oven to 375°F. Grease an 8 or 9-inch pie or tart pan. Place graham cracker crumbs, butter, sugar and pinch of salt in a large mixing bowl, stirring until combined. Dump this sand-like mixture into the tart pan and press evenly to create a thin crust. Bake for about 10 minutes. Remove from oven and cool on a wire rack.
Lower oven to 325°F. In a large bowl, mix together the lime juice, zest, egg yolks and condensed milk. Pour into crust and bake for 15 minutes. Remove from oven, cool on a wire rack and refrigerate until serving.

Turkish Lentil Soup
  • 1 small onion
  • 1 small carrot
  • 1 small potato
  • 3/4 cup red lentils
  • 1 teaspoon cumin powder
  • Salt and pepper to taste
  • 6 cups (1 1/2 liters) water
  • 2 tablespoons butter or margarine
  • 1 tablespoon all-purpose flour
  1. Peel and coarsely chop onion, carrot, and potato and place into a large saucepan. Add the red lentils, cumin, salt and pepper to taste, and water. Bring to a boil then reduce the heat to low and cover.
  2. Let the mixture simmer slowly until the vegetables are very soft and the lentils fall apart. Remove the pan from the heat.
  3. To make a smooth mixture, press the contents through a fine wire mesh strainer using a wooden spoon, or process in your food processor or blender. Hand blenders (immersion blenders) are great for puréeing the soup right in the pot.
  4. In a small skillet, melt the butter or margarine and then add the flour. Stir the mixture for a few seconds then add it to the soup (don't let the flour burn).
  5. Stir well, then let the soup simmer on low for about 15 minutes. Adjust the seasonings to your taste.
  6. If the soup seems too thick add a little more water. Serve hot with chunks of crusty bread and a wedge of lemon.

Turkish Pide

Ingredients:
  • 4 cups flour
  • 1 packet instant dry yeast
  • 1 1/3 cup warm water
  • ½ cup warm milk
  • 1 tbsp. soft butter or margarine
  • 2 tbsp. olive oil
  • 1 tbsp. sugar
  • 1 tsp. salt
  • 1 egg yolk
  • ½ cup plain yogurt
  • 1 tbsp. sesame seeds
  • 1 tbsp. nigella seeds
Directions:
  1. Put the flour in a large mixing bowl. Empty the packet of dry yeast on top and combine with your fingers.
  1. Add the warm water and combine lightly with a fork. Next add the milk and do the same.
  2. Add the softened butter or margarine and olive oil and combine. Lastly, add the sugar and salt.
  3. Now it’s time to knead the dough. If you are using a mixer, process the dough for about 10 minutes using the dough kneading hooks. If you choose to knead by hand, knead the dough in the bowl for 15 minutes.
  4. Turn the dough out on to a floured surface and knead for about five minutes more. The dough should be very soft. If it’s too firm, knead in another tablespoon of olive oil until smooth.
  5. Transfer the dough to a warm place. Cover it with a damp cloth and let it rise for about two hours.
  6. After two hours, the dough should be about double in size. Remove the cloth and turn it on to a floured surface. Divide the dough into two pieces with a dough cutter or sharp carving knife.
  7. Shape your ‘pide’ loaves using your fingers . Make each loaf flat and either oval or round, about ½ inch thick.
  8. Place each loaf on a metal baking tray sprinkled with flour.
  9. Using a sharp knife dipped in flour, first cut about 1 inch in from the edge to make a circle all the way around the loaf. Then, inside the circle, make diagonal cuts in opposite directions to make a diamond pattern. Do the same with the other loaf.
  10. In a bowl, whisk together the yogurt and egg yolk. Using a pastry brush, coat the top of the loaves generously with the yogurt-egg mixture.
  11. Sprinkle the sesame and nigella seeds evenly over the tops.
  12. Bake in a preheated oven at 430° F/ 225° C oven with a shallow tray of water placed at the bottom for about 30 minutes, or until the top becomes deep, golden brown. Serve the ‘pide’ hot out of the oven.
  13. https://www.thespruce.com/turkish-ramadan-flat-bread-3274362
apple pancake
 
Print
Serves: serves 2-3
Ingredients
  • 2 tablespoons butter (or vegan earth balance)
  • 3 apples, peeled, cored and sliced
  • ¼ cup brown sugar
  • ½ teaspoon cinnamon
  • 3 large eggs
  • ½ cup almond milk (or regular milk)
  • 2 tablespoons flour (any kind: white, wheat, spelt*, or a gluten-free blend)
  • ¼ teaspoon baking powder
  • powdered sugar, for dusting (optional)
Instructions
  1. Preheat oven to 375.
  2. Combine brown sugar and cinnamon.
  3. Whisk together eggs and milk.
  4. Mix together flour and baking powder and stir it into the egg/milk mixture.
  5. In a skillet, heat butter, add the apple slices and 1 tablespoon of the brown sugar mixture and cook, stirring, until soft. About 5 minutes.
  6. Pour the batter over the apples and sprinkle the remaining brown sugar on top.
  7. Bake until it puffs up, about 18-20 minutes.
  8. Dust with a little powdered sugar.

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Monday, February 06, 2017

A doctor searches

https://www.nytimes.com/2017/02/04/business/his-doctors-were-stumped-then-he-took-over.html?smid=tw-share&_r=0

His Doctors Were Stumped. Then He Took Over.

By KATIE THOMAS



Dr. David Fajgenbaum, who has Castleman disease, with his wife, Caitlin, at home in Philadelphia.Nicole Bengiveno/The New York Times

They called him the Beast.
David Fajgenbaum was the fittest of his friends at the University of Pennsylvania’s medical school, a 6-foot-3 gym addict and former quarterback at Georgetown. His mammoth hands seemed more suited to spiraling footballs than the fine fingerwork a doctor-in-training might need. He had endurance to match, taking multiple hits and returning to the field to play on.
“This guy was a physical specimen,” said his former roommate, Grant Mitchell, who used to walk to work with him. When they would arrive at the hospital for his obstetrics rotation, his friend recalled, “he would basically coerce me into doing pull-ups on the tree outside.”
In July 2010, that all changed. The 25-year-old woke up at night drenched in sweat. His lymph nodes were swollen. He felt stabs of abdominal pain, and odd red bumps began sprouting across his chest. Most bizarre of all, he felt very tired — so tired that he began slipping into empty exam rooms between patients, stealing five-minute naps to get through the day.
“Guys, I think I’m dying,” he recalled telling his friends.
A visit to the emergency room confirmed his fears. A doctor told him that his liver, kidneys and bone marrow were not working properly. Even more troubling, the doctor had no idea why his body was failing. “What do you think is going on?” he remembers the doctor asking him.
Pursuing the answer to that question, it turned out, would become his life’s work. It would transform Dr. Fajgenbaum from a patient on the brink of death five different times, whose illness stumped specialist after specialist, to one of the leading researchers in his field. He has even used himself as his own test subject, and may have discovered a treatment for his rare disease.
His story, which has been circulating inside medicine, is more than one person’s remarkable journey, however. It offers a look into the world of rare diseases, a corner of medicine that continues to frustrate — and flummox — those who seek cures for obscure conditions. About 95 percent of all rare diseases have no approved drug treatments.
Fewer than 8,000 people in the United States are found to have Dr. Fajgenbaum’s condition in any given year. But taken as a whole, rare diseases are not unusual: An estimated 30 million people in the country — or about 10 percent of the population — are living with one of the nearly 7,000 rare diseases that have been identified.
Prompted by financial incentives passed by Congress, the drug industry is hotly pursuing treatments for a throng of rare diseases. That has led to breakthroughs in several conditions, including cystic fibrosis and spinal muscular atrophy. Nevertheless, with a small number of subjects to study and relatively few people to sell new drugs to, many rare diseases are overlooked by doctors and scientists, hampered by a lack of resources and public awareness.
Dr. Fajgenbaum’s condition is one of the many that have been given relatively little attention. Initially, doctors thought he had a common form of cancer. A CT scan — a series of images that give doctors a clearer picture of what is going on inside the body — revealed a body riddled with enlarged lymph nodes, a hallmark of lymphoma. The news struck a particularly cruel blow: Only a few years earlier, while Dr. Fajgenbaum was in college, his mother died of brain cancer.
He declined rapidly as his immune system went haywire. A retinal hemorrhage, a type of ministroke, temporarily blinded his left eye. Fluids leaked out of his blood vessels, a sign that his liver was failing. Over the next two weeks, he gained about 70 pounds of extra fluid and his brain fogged over.
For those who knew him as the Beast, the transformation was jarring.
“Even when he got sicker and sicker and eventually went into the hospital, it was kind of like this, oh, weird, Dave must have some really awful virus — that’s one hell of a flu,” his friend Dr. Mitchell said.


Gary Gravina, who also has Castleman disease and suffered a relapse last August, being helped into bed by his wife, Stacey, as Dr. Fajgenbaum watched. Jessica kourkounis for The New York Times
When Dr. Fajgenbaum’s brain started slowing down, Dr. Mitchell said, the severity of the illness sunk in. “I would ask him a simple question, and he would answer in a couple words, like 30 seconds to a minute later,” he said.
Dr. Mitchell and the other medical students scoured textbooks and the internet, searching for clues. The top medical experts at Penn, meanwhile, were not having much more luck. Lymphoma was just one theory. Others thought it might be a severe case of lupus or even mononucleosis. But test after test was inconclusive.
Finally, the doctors tried a huge dose of steroids. Slowly, his body began to fight its way back. His kidneys and liver began functioning again, and the extra fluid receded. Seven weeks after he was admitted, Dr. Fajgenbaum walked out of the hospital. It was September 2010.
“I remember asking the doctor, saying, ‘What was this?’” he said. “And I remember the doctor saying, ‘We don’t know what it was, but let’s just hope it doesn’t come back.’”
It took only one month for symptoms to come rushing back, while he was convalescing at his childhood home in Raleigh, N.C. Doctors there shipped a piece of his lymph node to the Mayo Clinic in Minnesota, where pathologists finally pinpointed his disease. The condition, called Castleman disease, was so rare that doctors at the hospital in Raleigh had no experience with it.
Castleman disease had been known since the 1950s but has remained largely a mystery. A hallmark of the condition is enlarged lymph nodes, and most people with the disease — about two-thirds — have a form that affects just one part of the body and can usually be cured through surgery.
The form ravaging Dr. Fajgenbaum’s body — multicentric Castleman disease — is even more rare and deadly. Only about 1,200 to 1,500 people are discovered to have it every year in the United States. It defied classification, occupying a no man’s land between cancer and immune disease. Doctors weren’t sure of the cause in patients like him: Some believed it was a type of cancer, while others thought it was an inherited genetic disorder, or was triggered by a virus.
One thing was clear: The disease was deadly. Only about 65 percent of people with the condition live for five years after it is diagnosed, studies have shown.
Over the next few years, Dr. Fajgenbaum alternated between extended periods of relative health and frightening relapses. His condition stumped even leading experts in the disease, people like Dr. Frits van Rhee of Little Rock, Ark., who has treated the largest number of patients — about 100 — with Castleman.
The tools that Dr. van Rhee and others had were blunt and harmful in their own right. More than once, Dr. Fajgenbaum underwent a devastating, 21-day course of chemotherapy that annihilated his immune system in an effort to knock his disease into remission.
Dr. Fajgenbaum was also granted emergency access to siltuximab, a drug that Johnson & Johnson was developing for people with his form of multicentric Castleman disease, which would be approved in 2014. That year, in 2011, Dr. van Rhee secured a rare exception to try it on Dr. Fajgenbaum.


Far left, David Fajgenbaum in 2004, in a football drill at Georgetown University, and how he looked in February 2011, two weeks after his third flare-up of the disease.

But the drug, like other previous treatments, did not work.
Dr. Fajgenbaum was undaunted. With the zeal he once devoted to bench-pressing a personal best — 375 pounds, reached six months before he first fell ill — he dove into the scientific research on Castleman disease and began to familiarize himself with the world’s top experts.
On Sundays, his roommate tried to coax Dr. Fajgenbaum out of his bedroom, with its white board covered in notes, organizational plans tacked to the wall. He rarely succeeded. “I’m on the couch watching TV, and Dave is just cranking away on Castleman,” Dr. Mitchell recalled.
The more Dr. Fajgenbaum learned, he said, the more he realized how much the field that studied Castleman was in disarray. Researchers focusing on the disease used different terminology to describe the condition, making it difficult to compare published work. Leading experts weren’t in regular communication, and studies were being done over again, even though previous ones had failed.
“It became just abundantly clear that just because you have smart people thinking about a problem doesn’t mean that it’s coordinated at all,” he said.
One of the people he called was Dr. Thomas Uldrick, a clinical researcher at the National Cancer Institute who had studied multicentric Castleman disease. The two struck up a correspondence. “Clearly he was a very bright medical student, and he was scared of dying from this disease,” Dr. Uldrick recalled.
He also began collaborating with Dr. van Rhee, who knew that Dr. Fajgenbaum was a different kind of patient after he arrived armed with charts, graphs, timelines and slide presentations. “There are patients who keep meticulous records,” Dr. van Rhee said with a chuckle, “but he was definitely in the top 1 percent.”
In spring 2013 Dr. Fajgenbaum earned his medical degree, and a few months later he entered the Wharton School at Penn, reasoning that, to solve the tangled mystery of Castleman disease, business smarts would serve him well.
But in December of that year he got sick again, with his blood platelets dropping so low that he barely avoided a fatal brain bleed.
This time, though, he was able to use his relapse to further his search for a cure.
For months, Dr. Fajgenbaum had been collecting weekly blood samples that served as snapshots of his immune system, tabulating the results in a spreadsheet and adding them to a detailed slide presentation that he had been preparing since he received the diagnosis. And when it was clear the disease had returned, he persuaded his doctors to remove a piece of a lymph node, test it and save it for future research.
After a round of chemotherapy, he improved enough to be discharged and started looking into what secrets the tests might reveal. It turned out that five months before he started noticing symptoms in December, his T cells — one of the key weapons in the body’s immune arsenal — had starting activating, preparing for a fight even though there was no apparent threat. Then, about three months before his relapse, he noticed that he had started producing more VEGF, a protein that instructs the body to make more blood vessels, and is another sign of an immune system gearing up.
These two hints gave him an idea: Maybe the problem was with one of the body’s communication lines, the one that triggered production of VEGF and also told the T cells to begin activating. If Dr. Fajgenbaum could get his body to shut down that communication line — known as the mTOR pathway — he might be able to stop his immune system from overreacting and prevent a relapse.


A nurse at the Hospital of the University of Pennsylvania checks the port on Dr. Fajgenbaum’s chest through which he receives treatment. Jessica kourkounis for The New York Times

The discovery was exhilarating. “I felt like I was part of steering the ship,” Dr. Fajgenbaum said. “This time I was part of this team.”
With this major clue in hand, he and his doctors turned to potential treatments, existing drugs that were known to shut down the mTOR pathway. The one that seemed the best option was practically hiding in plain sight. Sirolimus, also known as Rapamune, was commonly given to kidney transplant patients to prevent their bodies from rejecting the organ. The drug had been on the market for years and was known to have few serious side effects.
“I wouldn’t think I ever could have prescribed this to another patient, or told a patient to try it, because we just didn’t have very much data,” Dr. Fajgenbaum said. “But at this stage, I’d had four episodes, and I’d failed everything the doctors had ever given me.”
In choosing to become his own test subject, Dr. Fajgenbaum was following a long line of medical researchers who have experimented on themselves. At the turn of the 20th century, government researchers studying yellow fever in Cuba allowed themselves to be bitten by mosquitoes who harbored the disease; one researcher caught the fever and died. In 1929, Dr. Werner Forssman inserted a catheter into the vein of his arm and guided it to his heart to prove that the procedure worked (he shared in a Nobel Prize for his work in 1956). As recently as 2005, Dr. Barry Marshall won the Nobel after drinking a broth infected with bacteria to prove that it caused ulcers.
This activist approach, Dr. Uldrick said, did not sit well with everyone. “There was some tension between the various doctors about who was driving the boat,” he said.
But the idea that Dr. Fajgenbaum needed to try something new was unanimous: Chemotherapy had worked three times, but the powerful drugs take a heavy toll and can themselves cause cancer if given too often.
Dr. Fajgenbaum eventually prevailed. In January 2014, he stopped taking his old cancer drugs and started on sirolimus. Six months passed, then a year. The weekly blood tests showed that his immune system was returning to normal.
Dr. Fajgenbaum, his health improving, returned to the other challenges that were hampering progress in the field of Castleman disease. He started the Castleman Disease Collaborative Network, a nonprofit whose mission was to prioritize and coordinate research into the disease, which operates out of the Perelman School of Medicine at Penn. Dr. Fajgenbaum, an assistant professor of medicine at the school, and his collaborators assembled an advisory panel of the world’s experts in the disease and set an agenda for answering the most pressing questions.
First on the list is figuring out the cause: Is the disease genetic, a form of cancer or caused by a virus?
As always, the work is intensely personal. Last summer, he drove to Allentown, Pa., to collect a blood sample from a patient with Castleman disease. He placed the vials in his vest pocket, explaining that he needed to keep the blood warm so the tests he had planned would work properly.
“You remind me of a mother hen,” the patient said with a laugh.
That gave him an idea: Dr. Fajgenbaum sat on the vials as he drove two hours back to Philadelphia, the car heater cranked up to ensure they were warm. It was a sweaty ride back, but worth it. “These samples are precious,” he said.


Dr. Fajgenbaum, in blue shirt at left, and his patient Gary Gravina, right, who both have Castleman disease, having blood drawn last July at the Abramson Cancer Center of the University of Pennsylvania.Nicole Bengiveno/The New York Times

In medical research, discoveries come slowly and take twists and turns that no one saw coming. Seasoned researchers have learned to rein in their optimism and to know that true breakthroughs can take years, if not decades, to realize. Not Dr. Fajgenbaum.
“I almost wish that every disease had a David to be a part of the charge,” said Dr. Mary Jo Lechowicz, a professor at the Emory University School of Medicine, who has studied Castleman disease and serves on the network’s advisory board.
Dr. Fajgenbaum’s single-minded mission to take on his own disease is also typical of the rare-disease world, said Max Bronstein, the chief advocacy and science policy officer at the EveryLife Foundation for Rare Diseases in Novato, Calif.
“A lot of mom-and-pop patient organizations emerge to take on these huge challenges in rare diseases,” he said. “I don’t think there’s one correct model for each disease; there’s been so many different approaches.”
Last month, Dr. Fajgenbaum marked his three-year anniversary since starting on sirolimus, a period more than twice as long as any of his other remissions. “I feel 100 percent,” he said.
These days, Dr. Fajgenbaum, now 31, walks through the hallways of Penn’s medical center, his frame again projecting the easy confidence of the athlete he once was. But he jokes that he would have to pull out photos of his days as a quarterback to explain to people why his friends still call him the Beast.
Now, he said, every time he tries to exercise, his mind wanders back to an email he needs to write or a call he needs to make. “It’s not because I don’t have the energy to do it; it’s because all of my energy is going toward this disease,” he said.
Not everyone is convinced that sirolimus is what has been keeping him healthy. Dr. van Rhee noted that while the results in Dr. Fajgenbaum are promising, his is just one case and treatments need to be proven in many more people. “I think the finding is very interesting,” he said, “but we need to see whether a similar mechanism is active in other patients.”
But Dr. Fajgenbaum said he grew more confident every day that it is the drug that is helping. He has begun sharing his experience with more doctors and researchers, is conducting laboratory tests to see if the drug is likely to work in other patients and has started writing an article about his experience for a medical journal. Soon, he hopes, doctors will begin prescribing the drug to other patients.
Dr. Fajgenbaum is optimistic about the drug’s chances but is aware medical science is unpredictable. “Who knows; maybe it will work for only a small percentage,” he said. “So we’ve still got a lot of work ahead of us.”
He now oversees the network’s research from his office in the Hospital of the University of Pennsylvania, five floors above the emergency room where he first learned his body was falling apart. It is also the same building where he spent weeks in the I.C.U., so sick that he said his final goodbyes to friends and family.
At first, when he learned his office would be in the same building, he felt anxious, unsure if he wanted the constant reminder of the ordeal he had endured. A few months ago, Dr. Fajgenbaum was called to the I.C.U. to meet a patient who had just found out he had Castleman disease. He spoke to the patient about enrolling him in a new study, and as he looked out the window, realized the view looked familiar.
On his way out, he bumped into a nurse who remembered him. She noted that he had stayed in the same room as a patient.
Ultimately, he said, he is glad for the proximity. “I didn’t think that I would ever get to leave the hospital, and now here I am, fighting back,” he said. “It’s the ultimate motivator.”

A version of this article appears in print on February 5, 2017, on Page BU1 of the New York edition with the headline: Doctor, Cure Thyself. Order Reprints| Today's Paper|Subscribe
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