Salvador started as a volunteer on the Oncology wing but after a time, moved to the Hospital de Día. This year will mark his 12th as a volunteer. That’s twelve years during which he has dedicated his time to this generous and altruistic work.

We have known him since Bernat was almost two months old and he has proven himself to be a person with a great heart. He knows how to deal with children, parents and/or families during delicate times. His presence infects everyone around him with positive energy. It’s undeniable that volunteer work is his vocation. Thank you, Salvador!

The team of volunteers at the Sant Joan de Déu Hospital is indispensable and throughout our years as patient and family, we have met many amazing and kind people. To them we are forever grateful. They have been beside us during each and every moment and day. Empathizing and navigating the situation, they have been able to distract us and to make us all feel better. Thank you from the bottom of our hearts.

Ruben, who has been with us since Bernat was a baby, just published a book that had nothing to do with him being a doctor:“Entre turrones“, a fun and original book based on a town in Spain called Jijona. What else could we have done besides buy it immediately at the hospital kiosk? Whether it’s for his great personality, or his literary talent, the point is that the book is selling well, and getting a copy isn’t exactly easy.

Taking advantage of our visit, we asked him to sign our copy of the book, and above all, to dedicate it to Bernat. Although still small, Bernat’s hematologist is rapidly gaining fame. Frankly, if the book is even half as good as he is at being hematologist, it’s a guaranteed masterpiece.

As for Bernat and his status before the transfusion, today his red blood cell levels are luckily not as low as earlier this year. Even still, a blood transfusion is necessary; perfect in order to feel strong before the Day of the Three Kings (A Spanish Christmas tradition).

This past Saturday, on the radio program Friends and Acquaintances, Bernat and a Pyruvate Kinase Deficit were the protagonists of a fantastic report carried out by Sergi Bassolas. During the ten minute program, the listeners learned about Bernat’s history, his ailment, and the importance of donating blood for those in need.

We want to thank Dr. Albert Català​​​​​​, Bernat’s doctor at San Joan de Déu, and Enrique Contreras, coordinator of the Blood and Tissue Bank, for their contribution to the report. Of course, we would also like to thank Sergi Bassolas for his heartfelt report, and the esteemed Silvia Tarragona, presenter and director of the program.

You can listen to the whole program at the website of RNE.

This is all because of his own sickness that disrupts the metabolism and the chronic transfusions that make it worse. The lack of oxygen in his blood causes chronic anemia, as well as muscular hypotonia (a muscular weakness). At the same time, it also causes the heart to beat faster than normal in order to distribute oxygen. Those are two examples of how things that appear to be different, are actually linked.

Returning to the main idea that magnetic resonance controls the levels of iron in the organism. Through these resonances we are able to identify if an overload of iron exists and how severe it is.

Pyruvate kinase deficiency causes many problems, one of which is splenomegaly (an enlargement of the spleen). In addition to the constant transfusions Bernat receives, there is an overload of ferritin (iron) that is released in the body. This may seem odd for many people, because most people think that anemia is the lack of iron in the metabolism, but that is not always the case. Anemia by definition is the lack of clean red blood cells in the blood that could be derived from many circumstances….0

In Bernat’s case, the bone marrow produces defective red blood cells that quickly self destruct. To counteract the subsequent deficit of red blood cells, the body absorbs more iron. These levels of ferritin, an iron-binding enzyme, are controlled by continuous transfusions. Dangerous levels of ferritin in some organs, like the liver, must be eliminated. This process is called chelation, and Bernat needs to take daily medication specific to iron chelation therapy.

From the results of the latest MRI, we were hopeful because the level of iron in Bernat’s blood had gone down a lot since the most recent test. Previously, his iron levels were 14, when the average iron level of an adult fluctuates between 3 to 5. The latest MRI showed that it had gone down to 5, a level which is still high, but shows that his chelation therapy was effective.

A level below 7 should be taken very seriously even with kids that are used to having chronic anemia. One problem that exist with chronic anemia is that the heart has to pump harder just to supply enough oxygen to the metabolism of your body. Bernat had never reached a level below 7 before in his life and this resulted in a difficult morning for his family. These low levels were unexpected, and each day more and more surprises arose. However, Bernat stayed strong and was able to ignore the struggles he faced but his family had to get used to the fact that he was very sick.

Thanks to the volunteers at the hospital, there was always a reason to smile and forget about the unfortunate situation at hand. We had a much appreciated visit from Frederic Ferralta as seen in the picture above. Ferralta was great company to Bernat with his kindness and generosity. Above all we were surprised by Frederic’s ability to make all kinds of balloon figures. The balloons looked very professional and he even made a puppy and a sword out of balloons and gave them to Bernat. Thank You!

Just like in the past, it will be a fun day for the whole family where the participants will enjoy the workshops run by the program Hospital Amic (Hospital Friend), designed to minimize the impact that being hospitalized has on a child. You will find therapy dogs, music therapy, the Pallapupas (a clown group) and many other things to spend a day with family and to bring a little bit of solidarity to the hospital.

We can assure you that Bernat won’t miss it, and we will be there to enjoy the day. Last year we went and it was great! There are pictures at the top of the post

You can find all of the information of the Amic Hospital by clicking here.

We, as parents, were astonished. Many times, we don’t know how much our little boy is going through, but we are able to imagine the degree of anemia on transfusion day. Bernat is so accustomed to the anemic state that he is able to trick us into thinking that he is okay.

Last weekend, we saw him running and full of energy. The Sunday prior to the transfusion, he didn’t even take his nap, which is something he always needs. Because of this, we were surprised to learn that he had a dangerously low level of red blood cells. When the level of red blood cells gets below seven, the body begins to notice pretty severe alterations: the transport of oxygen in the blood is sufficiently lower, so the heart needs to beat faster, which leads to noticeable acceleration in the heart.

Given the results, the doctor decided to push our visit up to within the next 3 weeks.

Luckily, once we got to the Day Hospital, Bernat received his transfusion. Montse was the nurse who was by our side throughout the process. And not just on that day! Montse is a veteran in this place and has known Bernat since he was very little and just beginning to speak. Therefore, we want to take the time to thank her, because she always greets us with a smile and has been at little Bernat’s side since the beginning. Thanks, Montse!

Today, we would like to explain what these visits consist of, and what to expect.

Today it started at home, when at first light just before leaving to go to the hospital we have to get bernats port a cath ready with antiseptic cream and until he gets his injection in the hospital its covered up the cream takes 30 mins to go into effect and last for 2 hrs

It always starts at home, as soon as the sun rises. Before we go to the Saint Joan of the Arc Hospital, we have to prepare Bernat’s Port-a-Cath with antiseptic cream. We wait to perform the needle prick until we are at the hospital. The needle is covered with film. The cream takes 30 minutes to go into effect and lasts for two hours.

Once we get to the hospital, around 8am, we go directly to the Day Hospital. There, they introduce the needle into Bernat’s Port-a-Cath, and extract blood in order to analyze it. The needle cannot be taken out until the treatment is finalized, so that after, when they need to transfer the blood, they don’t have to return to pinch the area.

The level of red blood cells was less than the level of red blood cells that Bernat had in his blood on the day of the initial test. If in the time that has passed since the latest transfusion, if during this period there has been any alteration (fever, gastroenteritis, etc.), the level can vary, since blood cells can destroy themselves quickly each month. This March, for example Bernat was at 7’1. Before the transfusion his level was up to 9-11 approximately. This means that as a rule, his level went down 3-4 in just three weeks.

So that we have an idea, the normal level of erythrocytes in a healthy person is around 13-15. It is for this reason that Bernat has chronic anemia, which his body is already accustomed to. This does not mean that he does not have to be monitored, since it is dangerous for metabolism for his level to drop below 7, and it produces changes in the heart, general tiredness, etc.

After the visit, and following the results of the analysis, it is necessary to do the transfusion. In Bernat’s case, it’s always necessary. So we went to the Day Hospital, where they receive the bag of blood and prepare everything necessary to do the transfusion (check the blood type, the blood dosage, etc.) The transfusion, due to the amount of blood and body weight, has to be carried out in an hour and a half. One must add the transfusion to previous and future treatments. Once the transfusion has been carried out, they extract the needle and clean the Port-a-Cath zone. In total, it has been 7-8 hours, and little Bernat has behaved like a true hero.

The great difficulty in the transport of oxygen and a massive destruction of Red Blood Cells, both derived from neuromuscular problems, specifically in hypotania (loss of muscle tone), are presented in the most severe cases of the illness. These symptoms cause him to get tired quickly. They are two occurrences that result in his need for medical control by a cardiologist.

Because when Bernat was nine months old, the doctors detected a psychomotor delay, the neurologists referred him to a CDIAP (Center for Infantile and Early Development) so he could be treated by a physical therapist. With time, patience, and a foundation of specific exercises, they have made a lot of progress. Additionally, the treatments that he has received since nursery school have been key in his advancements.

May 9th marked one year since Bernat took his first steps. He was almost two years old and it was the day before our wedding – a great surprise and a beautiful gift for his parents!

Today, Bernat still requires various types of assistance. He needs permanent support of a physical therapist and neurological monitoring. Genuinely, we feel very fortunate that Bernat has the support of various professionals.

We want to take this time to thank the hematologists of San Joan de Déu Hospital: Dr. Ruben Berrueco and Dr. Albert Catalan. They are excellent professionals and good people. They are very supportive, and we are very fortunate to be in their hands.

In addition, we want to thank the great work that the day staff of the hospital has carried out, from the nurses to the administrative department.

Also, we want to stress the admiration that we feel for the work that the Pallapupas have done. They continue to make us laugh and always include us in their madness. It would not be the same without them.

We can’t forget some of the most essential people in San Joan de Deu Hospital, the volunteers. They are a great source of support for the families and the kids.

And finally, we want to give lots of thanks to all of the anonymous people that have been donating blood. Without them, Bernat would not have a bag of blood ready each time he needed it. They are the real heroes!