The story of Alex

by Luca Catalano Gonzaga
Part of ‘One of a kind’ (on going)
View project

Photography can break silence. The incontrovertible proof lies in the story of a nine-year-old boy named Alex Granini  and lives in Valbrona, in the province of Como. I always knew Alex had something different, explains Mom Patrizia. These are the “strange sensations”, those “strange voices” that only mothers feel and that are impossible to explain. They are just there, and that’s it. To get to a clear diagnosis it took three years; we were rebounded from one center to another, every doctor had his truth, often forgetting it was a baby just a few months old and a frightened, lost family. I have gone from the greatest joy that can be the birth of a child, to the most intense pain, its loss, something to which I have been very close to and that I have experienced twice. The illness my child is suffering from is called X-linked Reticulate Pigment Disorder with Systematic Manifestations. This disease in addition to photophobia, thermoregulation deficiency, hypo-swelling, also causes growth retardation, kidney disease, calculosis, urinary infections, respiratory infections and gastrointestinal disorders. Alex presents most of these symptoms. When we started this journey, the cases in the world were only 8, now after many years, thanks to the disclosure, the cases have become 29, Alex is still the only Italian case. I hope, telling our story, to be able to help other children like mine, other mothers and even doctors. I hope I can help those like me, who live a very rare situation, to feel less alone. (text by Patrizia Gentile).

  • Valbrona, province of Como, Italy. Alex in a playground near Valbrona.
  • Trieste, Italy. Alex in his hotel room in Trieste, where he undergoes checkups at the city hospital every 6 months.
  • Valbrona, province of Como, Italy Patrizia, Alex’s mother, is always ready to protect her son from direct sunlight.
  • Valbrona, province of Como, Italy. Giulia takes a stroll in the surroundings of the lake Como.
  • View of Valbrona, in the province of Como, Italy.
  • Pusiano Lake, Province of Como, Italy. Alex walking along lake Pusiano.
  • Trieste, Italy. After the medical tests are completed, Alex and his family are ready to go to a restaurant outside Trieste.
  • Valbrona, province of Como, Italy. Alex plays with his father in a playground.
  • Valbrona, province of Como, Italy. Alex and his sister Giulia in a playground.
  • Valbrona, province of Como, Italy. Alex together with his mother Patrizia and his sister Giulia leaving a church near the lake Como.
  • Valbrona, province of Como, Italy. It’s 6 a.m., Alex has just woken up and as his routine he curls up on the couch for about 10 minutes, to let the eyes gradually adjust to the light.
  • Valbrona, province of Como, Italy. Patrizia takes Alex’s temperature.
  • Valbrona, province of Como, Italy. To minimize the discomfort to the eyes, Alex has to put special eye drops at least 10 times a day.
  • Valbrona, province of Como, Italy. Portrait of Enea and Giotto the dog.
  • Valbrona, province of Como, Italy. A moment of tenderness between Alex and his mom Patrizia.
  • Valbrona, Province of Como, Italy. Patrizia, the mother, trains Alex’s eyes back to center. This exercise is repeated several times a day.
  • Valbrona, province of Como, Italy. Cuddle moments before bedtime.
  • Valbrona, province of Como, Italy. Alex and his mother just before going to bed.
  • Valbrona, province of Como, Italy. “Giulia, make the dog get off the sofa!”
  • Valbrona, Province of Como, Italy. Alex’s room.
  • Valbrona, province of Como, Italy. Alex among his toys.
  • Valbrona, province of Como, Italy. Saying ‘Goodnight!’ to Mum and Dad.
  • Valbrona, Province of Como, Italy. Patrizia, heavy with fatigue at the end of the day.
  • Valbrona, Province of Como, Italy. Alex’s special stuffed toys : Spiderman, Cookie Monster, Captain Nickers, Mega Man, Stormtrooper, Minecraft Skeleton.
  • Valbrona, province of Como, Italy. 07.00 a.m.: Alex, still half-asleep, has his breakfast.
  • Valbrona, province of Como, Italy. Alex protects his eyes from the sun while going to the supermarket with his father and his sister.
  • Valbrona, province of Como, Italy. Alex needs frequent breaks to restore body temperature after excessive sun exposure or undue physical efforts. As a consequence, the temperature decreases quickly, Alex turns pale and has tremors all over the body: at this point he necessarily must eat something to recover. Here he is recovering in a bar on the Lake Como.
  • Valbrona, province of Como, Italy Though wearing special sunglasses, Alex always travels by car with tinted windows.
  • Trieste, Italy. On the city’s waterfront, not far from Piazza Unità, Alex’s family spends the afternoon, waiting to go to the Children’s Hospital Burlo Garofolo.
  • Trieste, Italy. Alex walks along the city seafront next to his mother Patrizia, holding his stuffed animal.
  • Valbrona, province of Como, Italy. Portrait of Giulia, Alex’s sister.
  • Valbrona, province of Como, Italy. A portrait of Alex. The spots on the skin are called “Reticulate Pigmentary Disorder”. At the moment, the spots are stable but they are changing in color; for this reason Alex is going to see an oncologist in January 2016.
  • Burlo Garofolo hospital, Trieste. Alex at the end of the medical examination. Due to the multisystemic syndrome which affects various body organs, every six months Alex needs to be examined by several specialists and undergo many checkups: general, dermatological, ophthalmic, stomatological, oncological, gastroenterological.
  • Milan, Italy. Dr. Andrew Zinn, a geneticist from Dallas, Texas, illustrates other recognised cases in the world – to date only 9 – during a convention held in Milan in 2014, organized by XLPDR International Association ONLUS founded by Patrizia, Alex’s mother.
  • Burlo Garofolo Hospital, Trieste. Alex is taken to the operating room.
  • Burlo Garofolo Hospital, Trieste. Alex with his mother while he waits to be taken to the operating room.
  • Trieste, Italy. Alex, now at the Burlo Garofolo Hospital, is arranged on the stretcher by nurses and is about to enter the operating room. His face is consumed by the many operations, but his father is there to give him comfort.
  • Trieste, Italy. Alex is ready for surgery along with his faithful friend: Spider Man.
  • Burlo Garofolo Hospital, Trieste. Alex in the operating room during the preparation before general anesthesia with cardiac monitoring. Alex shortly before being taken to the operating room where he will undergo skin biopsy, surgery for corneal ulcer in left eye and oral rehabilitation: lack of salivation causes dental caries, damages the enamel, and makes the mouth more vulnerable to infections, apart from difficulties in swallowing.
  • Burlo Garofolo Hospital, Trieste. Patrizia protects Alex’s eyes from the operating room lights.
  • Burlo Garofolo Hospital, Trieste. Alex’s mum Patrizia embraces Lorena, the nurse who has known Alex since he was 3 years old. Alex has been followed-up regularly for more than 5 years for the monitoring of the XLPDR syndrome. Since his birth he has been hospitalized 58 times.
  • Trieste, Italy. Patrizia, the mother, holds Alex’s hands as he has just awakened from the general anaesthesia.
  • Trieste, Italy. Dad Enea, following the recommendation of Patrizia, the mom, places a blanket on the window to prevent light from entering in Alex’s room who hides, to cover.
  • Burlo Garofolo Hospital, Trieste. Alex shortly before being taken to the operating room where he will undergo skin biopsy, surgery for corneal ulcer in left eye and oral rehabilitation: lack of salivation causes dental caries, damages the enamel, and makes the mouth more vulnerable to infections, apart from difficulties in swallowing.
  • Burlo Garofolo Hospital, Trieste. Alex is waiting for being hospitalized.
  • Burlo Garofolo hospital, Trieste.Alex during the Schirmer test, used to measure the lacrimation of the eye. It is not an invasive examination, but in Alex’s case, given his pathology, it is very painful for him.The outcome of this test has confirmed the absence of tearing. 
  • Burlo Garofolo Hospital, Trieste.Within the elevator with mum and dad, carrying Alex’s medical record.
  • Trieste, Italy. The whole family at Burlo Garofolo Hospital in Trieste. Every 6 months Alex undergoes several medical examinations.
  • Valbrona, province of Como, Italy. Giulia takes the dog for a walk.
  • Valbrona, Province of Como, Italy. Alex going back home with his dog Giotto. Taking the dog down to the yard, walking up and down the stairs, are exercises for the development of his autonomy. 
  • Valbrona, province of Como, Italy. In the mountains nearby Valbrona.
  • Valbrona, province of Como, Italy. Giulia strolls in the snow. Alex would love to be out there with his sister, but the glare from the snow would be too harmful for his eyes.
  • Valbrona, province of Como, Italy. Giulia under the snow.
  • Valbrona, province of Como, Italy. The cold weather, the snow are a special moment for Alex, as he doesn’t risk as he does in the heat. The contact with snow acts immediately with his body, bringing its temperature to the correct standard.
  • Valbrona, province of Como, Italy. Alex in full freedom of movement, still maintaining some fundamental protections : hat, gloves and glasses with a special protection inside them, preventing air and light to reach his eyes.
  • Valbrona, Province of Como, Italy Alex takes karate classes with his father, as suggested by his doctors, but before commencing he must prepare his body so that he doesn’t overheat.
  • Valbrona, province of Como, Italy. Alex simulates a fight with a girl of the same age. On doctors’ recommendation, his parents considered important to enrol Alex for judo lessons so that he could socialize with other children of the same age and did not feel isolated and different.
  • Valbrona, province of Como, Italy. The critical moment has passed and Alex takes care to slowly re-hydrate his body.
  • Valbrona, province of Como, Italy. Alex is refreshed with thermal water, to prevent and control the body overheating, which then determines hyperthermia, a temperature increase that can even reach 39 degrees Celsius within a few minutes, which can cause a confusional state and a cardiac arrest, such are some of the consequences. 
  • Valbrona, province of Como, Italy. Helped by his father Enea, Alex wears his kimono before going to judo lessons. His parents take him to the gym in order to support his integration with other children and to prevent any form of isolation.
  • Valbrona, province of Como, Italy. A moment of rest for Alex after playing at the playground.,Alex can’t run or play for more than 10-15 minutes. He often has to stop in order to keep the body temperature constant.,An increase in body temperature causes strong headaches, skin reddening and a complete confused state.
  • Trieste (Italy). After surgery and 2 days of hospitalization at Burlo Garofolo Hospital, Alex and his parents go to the cinema to see “Kung Fu Panda 3”.
  • Valbrona, province of Como, Italy. Alex plays hide-and-seek with his sister Giulia in a supermarket.
  • Rimini, Italy. Protected by sunglasses and cap Alex plays smiling in the water late in the afternoon when the rays are weak unlike the morning where the environment is much more hostile.
  • Rimini, Italy. Alex’s entire family dives into the water with the little one.
  • Rimini, Italy. Alex looks upward in late afternoon hours as the sun is setting and emitting weaker light rays that allow the little one not to be blinded.
  • Rimini, Italy. The afternoon hours of the beach, less chaotic and loud, allow Alex to concentrate on Patrizia’s voice that accompanies him in his amusements.
  • Rimini, Italy. Alex’s spots on the skin are one of the main features of the syndrome and are related to the cross-linked disorder.
  • Rimini, Italy. Alex immerses himself in fresh water, helped by hisMother Patrizia, to remove the sea salt from his body.
  • Rimini, Italy. Patrizia takes care of her little Alex spraying on hair a refreshing jet of water on his hair. It takes just a few minutes to overheat his small body.
  • Rimini, Italy. In the evening, when the sun is down, the environment is less hostile to Alex who can play outdoors.
  • Rimini, Italy. At the outbreak of fireworks, for unconditional reflection, Alex shakes his eyes in front of the light beam that lights up the night.
  • Rimini, Italy. The beach lights up with the fireworks where Alex’s family decided to spend the holiday.
  • Trieste, Italy. Alex in a moment of play in Trieste.The night is the best time to go out and have fun: the sun has set and there are no jumps in temperature.
  • Valbrona, Province of Como, Italy. Alex wearing his favourite mask, Jason Voorhees’s, an imaginary character protagonist of the twelve episodes from the saga Friday 13th.
  • Valbrona, Province of Como, Italy. Alex going for a walk in the woods with his best friend Simone. They are looking for chestnuts.

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