Sickle Cell Disease and Stem Cell Transplant: My Personal Experience

I had just returned from a holiday in Spain when I suddenly felt very bad. My short-term memory didn’t work as well, reading was difficult for me, I felt a stabbing pain behind my right eye. I was tempted to put on sunglasses against the bright sun. “This must be a migraine,” I thought, even though I had never had one before. But a few days later things went wrong. I can’t remember exactly what happened. I do know that I was spouting gibberish right before I fell over.

A few hours later I woke up because I was cold. I lay in a hospital bed and smelled an odor that I immediately recognized: I was in the AMC (Amsterdam University Medical Center). An ICU nurse came in and told me I had suffered some kind of stroke, “sinus thrombosis” to be exact. In addition, a blood clot blocks the draining arteries or veins in the front part of the brain – hence the headache and gibberish. My parents, my girlfriend and my two brothers took it in turns to see how I was. I stared in surprise for a long time. It didn’t feel like something that’s supposed to happen to a 29-year-old.

My name is Orion. I am now 30 years old, engineer by profession and sickle cell patient. Sickle cell disease is a hereditary and severe form of chronic anemia, which can lead to severe pain attacks (also called sickle cell crises) and permanent damage to organs. In 1992, the year I was born, the disease was not yet on the list of the heel prick* – the prick in the heel of newborn babies where blood is taken to check for any serious illnesses, so that treatment can be started as early as possible. My parents initially did not know that I had it among the members. They didn’t find out until I was three years old and got pneumonia. A doctor at the Flevoziekenhuis in Almere diagnosed me with the disease.

Despite the sickle cell, my life was relatively normal. I did have a number of crises, five of which were so severe that I had to go to the hospital, and I once had a pulmonary embolism. I am also often very tired and my condition is not too good.

Back to my sinus thrombosis – the cause of which turned out to be my sickle cell disease. I then received drastic advice from my attending physician: I was strongly advised to start a treatment for healing.

Cure sickle cell disease is namely possiblevia an allogeneic stem cell transplant. Until recently, this treatment was only an option for children with sickle cell disease, but adults are now also eligible. It is often not done yet: with me there are now 30 people who have undergone a stem cell transplant for sickle cell anemia at the AMC.

Such a cure is only started if the sickle cell complaints are so severe that supportive treatments, including the administration of antibiotics, intensive pain relief and blood transfusions, are no longer sufficient. This is because it is risky: there is a chance that the donor cells will be rejected (with comparable treatments in the US, this happened in about 15 out of 100 patients). It is also not exactly easy – in the first place because a suitable donor had to be found, from whom it fabric type exactly matches mine. One of my brothers and my sister do not have the disease, but are carriers. In theory, that made them suitable for donating a stem cell. The chance that their tissue is identical to mine is about 25 percent – ​​so that had to be tested first.

It was nerve-wracking waiting for the results of that test, but I put up a fight. Then I got a call from my brother. He is rather dry, so he casually informed me that he is an identical match. That came in handy, I must say. I didn’t know how many times I had to thank him.

On March 16, 2023 I reported to the hospital. After an intake I was taken to the dialysis center. In preparation, I received an exchange transfusion, in which my blood was taken and replaced with donor blood. Immediately afterwards I felt very energetic. As I held a huge, warm bag of my own blood, I hoped it was a harbinger of the energy I will always have after my healing.

The next day I was scheduled to insert a PICC line (which stands for peripherally inserted central venous catheter, an infusion that allows medication to be administered for a longer period of time) with which I received the drug Alemtuzumab. This drug is an antibody that should shut down my immune system so that the donor stem cells can settle better. Alemtuzumab is a so-called ‘biological’, which means that it is made with living cells in a cell culture. Depending on the brand, these cells come from a rat or a mouse. Unfortunately, there are also a few side effects: I got a rash, my face swelled up, I was short of breath, had chest pain and terrible itching.

I was administered the Alemtuzumab for four days in a row. Then I started taking Sirolimus, a drug that is extremely important for the period after the transplant: it suppresses my own defense against foreign cells. Without Sirolimus, my brother’s cells wouldn’t survive in my body. My whole body also had to be irradiated, to make it even less likely that my immune system would throw a spanner in the works after the transplant.

Before the radiation I was called by a specialist, who went through the risks with me. “You can stop at any time,” he said. The chance of infertility is one percent, and I could develop long-term cataracts – which are treatable.

The irradiation lasted a total of ten minutes. It’s not that long, but it was intense: it felt like the machine was sucking all the energy out of me. The rest of the day I struggled with the simplest things. Going to the toilet, for example. I had to hold on tight when I stood up, otherwise my legs would collapse. Mentally, this was pretty weird. I felt like doing all sorts of things, walking around in circles and I was really looking forward to a visit. But my body struggled.

The day of the transplant was very special for me, and also for my loved ones. It felt like I had worked hard for it, going through all the earlier stages of the treatment. And not only me, but also my friends, my family, my acquaintances and my girlfriend – they got me through it. I was able to draw tremendous strength from their words and gestures.

My brother’s “harvest” was particularly good: a full bag containing a blood-like substance was waiting for me, with a density of 9 million cells per kilo (the norm is 7 million cells per kilo). When they are administered through the previously placed PICC line, I feel a cold current in my arm, nothing else. It was a happy moment. My girlfriend was there, along with two nurses, a doctor and a doctor in training. They congratulate me.

A day later I was allowed to go home. It felt good to be home again. Little things, such as opening a window or sleeping in a little longer, can be missed in the hospital. It has been more than a month since I reported to the AMC. I had already been told that the recovery would be ups and downs. And that’s right. I have now shaved my head because my hair fell out due to the treatment and radiation. I don’t always eat well, because my mouth is full of painful wounds and canker sores from the Sirolimus. Those wounds don’t heal quickly because I have a massive lack of platelets. Every day I have to look at how I feel and whether I’m going to do anything at all, or whether it’s going to be another binge-watch day. But I’m in good spirits: I knew in advance that I could suffer from these side effects, it’s part of it. I still have a long way to go.

In a few weeks I will hear for the first time to what extent my body has accepted the donor cells, and whether I am actually on my way to healing. Until then I remain hopeful, but also combative.

*In Belgium, sickle cell disease is mentioned not yet on the list for the heel prick. That is very unfortunate, because treatment for the disease works best if it is preventive.

Orion keeps track of his recovery on his blog.