He has had two liver transplants and is waiting for a bone marrow donor. It would not have happened if the National Health Fund had reimbursed him a life-saving drug, which is free of charge in almost all European Union countries. Therapy with it costs over … two million a year. Does it have to cost so much, or is someone cynically feeding on patients because they know that the family will spend their last money on treatment?
– How much is my 20-year-old son’s life worth? How much is his health worth, which he would have a chance to regain if in Poland he was reimbursed for an excruciatingly expensive medicine. We are the only country in the European Union, next to Romania, which condemns patients like my child to euthanasia – says Anna, mother of Konrad Krzemiński, 20, who suffers from hepatic vein thrombosis and the extremely rare nocturnal paroxysmal hemoglobinuria.
– Yes, I know, it sounds tragic: thrombosis, hemoglobinuria … – adds Anna. – However, please imagine that the son’s situation would not be hopeless if pharmacological treatment was started after the first liver transplant. Thanks to the hospital in Szczecin, which purchased a preparation sufficient for the first weeks of treatment with eculizumab, we could for a moment enjoy the hope that it would only get better from now on. The son was recovering literally overnight. He also recovered mentally. However, all this effort was in vain. The resistance from the National Health Fund is unbreakable. The procedures speak for themselves, and they handed down the death sentence on my child.
Damaged liver
The tragic story of Konrad from Legnica began only a year ago. Right after the holidays, the high school graduate, who was healthy and full of plans for life, began to complain of stomach pains. At first he downplayed them. He thought he overeat or overdoing it in the gym. But the pain persisted. Anna, despite the weekend, took her son to the ultrasound. The doctor noticed water in the abdominal integuments and referred him to the hospital.
– In Legnica, they put their son in the ward – says the mother. Konrad was resentful. He was planning to go to a wedding. So many things to be covered by an ordinary young life. But they wanted to do tests and … First, I heard that the abdominal scan did not show anything dangerous. The liver is fine. A few days later, a 360-degree change!
– They sent their son home with a diagnosis of liver damage of unknown etiology and referral to a hematology clinic. That’s all. Soon after, it turned out to be cirrhosis of the liver! – says the shaken mother.
Cirrhosis of the liver is a chronic disease that causes irreversible damage to this organ. It is caused, among others, by viruses, autoimmune diseases, and toxins, including alcohol. Cirrhosis is the fibrosis of the liver parenchyma destroying its structure.
The diagnosis is amazing
Anna decided that she would not wait for their son to be admitted to the clinic. She got a referral to the hospital, packed her bag and took Konrad to the hospital in Wrocław, which was recommended to them by a doctor from Legnica!
Anna: – We arrive at eight in the morning. They take him on research until late afternoon. When the decision was made that my son was to stay in the gastrointestinal ward, I was XNUMX% sure that it was not good. I was still under the illusion that they would say: – Luckily, it’s nothing dangerous! Please calmly come home. But it didn’t
The boy’s next landing took place at the hospital in Szczecin, where they could complete the diagnostics. Doctors from Wrocław, Legnica and Szczecin were on the hotline. Finally, the diagnosis is made: hepatic vein thrombosis. The qualification process for organ transplantation begins.
– My world collapsed – recalls Konrad. – This cirrhosis completely baffled me. It’s a disease that takes years to develop, and I’m very young. I don’t drink alcohol. I’ve never been seriously ill before. It was difficult for me to mobilize myself to continue the fight, but I managed – I can be disciplined. I did not know that the worst was ahead of me – says Konrad.
– We came a long way before the final decision was made that they could even do this transplant – recalls Anna. – Dozens of tests, consultations with specialists and the fear of whether it will be possible at all or a donor will be found. Will we make it on time.
The whole family, including Konrad, pushes black scenarios out of their heads.
– It cannot be that a boy lives like gold for twenty years, and suddenly sickness and death look into the eyes – says Konrad’s father, Dariusz. – You have a son. You watch him grow up. You are bursting with pride. And love. The boy graduates from high school, trains kickboxing, has ambitions to make a career in the army. And then comes the crash. You want to move heaven and earth to help him.
First liver transplant. Saving a Life
Konrad is exhausted by his illness. The test results deteriorate, he is again in the hospital in Szczecin. The big day comes on February 24, 2016. At six in the morning, Anna picks up the phone. Konrad is calling.
– Mom, they told me to wash myself with a special liquid. Maybe I’ll have surgery. I think they have a liver for me – Ania hears the rest like a fog. Cry.
– It cannot be described in a few words. I knew that someone had just lost his life, that somewhere in Poland a family was now in terrible despair. And that there is consent for the donation of an organ that gives hope to my 20-year-old child. For the first time in weeks, I heard joy in his voice. A few hours later I was with Konrad. The operation was successful. My son woke up, I could only be with him for a moment. I stroked his cheek. I said, “Everything’s gonna be okay.” And he believed it.
Hepatic vein thrombosis, or Budd-Chiari syndrome (BCS), is a disease whose essence is the blockage of the outflow of blood from the hepatic veins to the junction of the inferior vena cava with the right atrium. Depending on the degree of obstruction of the outflow of venous blood from the liver and obstruction of the hepatic veins, it is distinguished by a fulminant, acute and chronic form of the disease.
Konrad’s new liver starts working. The boy goes to the hospital for a check-up every week, takes drugs to prevent transplant rejection. The results are improving.
Konrad can eat anything at Easter. To dream about what he will do in the future.
– I watched it bloom. He “improved” on his face. He gained weight. And he was still under the care of the hematology clinic – it was recommended because of a history of thrombosis.
Nocturnal paroxysmal hemoglobinuria. Breakdown.
Unfortunately, on April 4, Konrad felt bad. He said: – Mom, I feel the same stomachache as I did then, after the holidays …
– I called his doctors from Szczecin immediately. They calmed down: “It may be some kind of bacteria, or catarrh after a meal”. They advised him to be tested on the spot, in Legnica.
No doctor wants to take responsibility for a liver transplant patient. They go to Szczecin.
Tomograph shows return of thrombosis. The veins in the liver are “clogged”. After collection, the blood “curls” like jelly. Doctors from Szczecin consult Konrad’s case with hematologists all over Poland. The blood taken from Konrad is sent to a specialized laboratory in Warsaw.
– The result was devastating for us – says Anna. – It turned out that, apart from thrombosis, my son suffers from nocturnal paroxysmal hemoglobinuria. The combination of thrombosis and hemoglobinuria is an extremely rare case. Konrad is probably the only patient in the world who has these two diseases at the same time.
Nocturnal paroxysmal hemoglobinuria is a condition in which red blood cells are broken down (erythrocyte hemolysis), and the levels of white blood cells (leukocytes) and platelets (thrombocytes) fall. The disease is associated with a mutation within the hematopoietic stem cell, ie the “mother” cell, from which erythrocytes, leukocytes and thrombocytes are formed. The change in the “mother” cell causes these blood components to also have this defect. The essence of the disease is that at low blood pH, hemolysis, neutropenia (decrease in the level of white blood cells) and the formation of blood clots due to the activation of thrombocytes occur.
Blood pH decreases at night, hence the name of the disease, but also under the influence of acidic foods and medications, after drinking strong tea or taking a lot of physical exertion. An attack can also trigger infection and stress. People between the ages of 25 and 45 suffer from nocturnal paroxysmal hemoglobinuria.
Liver transplant # 2. Searching for a donor
Gegenna starts all over again. But there is once again hope that all is not lost yet. It turns out that there is an extremely effective drug containing eculizumab. Unfortunately, the annual therapy costs as much as 365 thousand. dollars a year. Despite this, the patient is reimbursed in the entire European Union, except for Poland and Romania.
Questions like: why other EU countries, not the richest country, do not save on the sick, and Poland does? And does this drug really have to be that expensive? The annual treatment is as much as 675. dollars, or two million 700 thousand. zlotys. Is production so horrendously expensive, or maybe someone has cynically raised the price because he knows that it is a last-resort drug, that the families of patients will spend their last money on it?
Anna: – It was the last resort for Konrad. In order for him to survive, the hospital in Szczecin purchased the dose of the drug needed for two weeks of treatment. Now hematologists from Wrocław were to take care of their son. We expected more batches to come to the local hospital. It turned out that Wrocław must first apply for a refund. In practice, it was an absurdly long process of obtaining all possible opinions, an analysis of the regulations. Effect? The answer from the National Health Fund comes – “NO”.
Konrad: – I don’t even want to mention this horror. Monstrous stress. In addition, I was in a very bad condition. Discontinuation of the treatment resulted in permanent damage to the transplanted liver. My kidneys started to crash. It occurred to me that I would not survive without the drug. What was I supposed to do? To die slowly in the silence of a hospital solitary confinement? I guess that’s how the officials imagined it.
Meanwhile, in Warsaw, the treatment options parallel to the pharmacological treatment were discussed. Konrad needed a liver transplant again. He was included in the accelerated list – those who need the organ “now”. It was also decided that the boy would be saved by a bone marrow transplant.
– Konrad is not in a hopeless situation. He is not sentenced to death, says Anna. – However, another transplant is needed – this time the bone marrow, and the preparations for it weaken the body. We are looking for a donor. At the moment, Konrad should take eculizumab on a regular basis to avoid a repeat of the situation from spring. The treatment has already shown once that my son responds well to her – there was a significant improvement.
As Konrad’s father announced, the family moved heaven and earth to raise money for treatment. Further collections and actions are underway in Legnica. Foundations are looking for funds: Siepomaga.pl (http://www.siepomaga.pl/konrad) and “Help fight for life” (ING Bank Śląski 25 1050 1575 1000 0090 9712 1306 with an annotation Konrad Krzemiński)
– Konrad has a chance to recover, the disease, although extremely rare, does not cancel him out. We will do everything we can to help him. Thanks to everyone who understands this. I know the sum needed is astronomical, but as a mother I will never say it is too big to save my child. His life has no price to me.