Beverley and Jensen

I have two sons; my oldest is Ethan and Jensen is three and a half years younger.

In 2018, when Jensen was just three, I noticed a change in him. If we went to the park, he didn’t want to do anything, and at play gyms he would sit watching the others. He was not getting involved in things and much of the time he would just fall asleep.  

Jensen was in pain when he went to the toilet, and it was this that made me take him to the GP. The doctor said it was constipation, despite going back and forth to the doctors for three or four months, there was no improvement,

Jensen was then referred to a specialist and an X-ray was arranged. This indicated that he was constipated, so he was put on Movicol, which seemed to help for a while. But he was losing loads of weight and as his mum I just knew something was seriously wrong. 

A follow-up appointment was organised where they did an ultrasound of Jensen’s belly which showed thickening of the bowel. The next day his belly became rock hard. I went to the GP and while I was there the hospital rang asking me to go back straightaway. 

Jensen was seriously ill. His bowel had started leaking, causing him to have sepsis and severe hypoalbuminaemia (where the level of albumin in the blood is low, which can indicate a disorder of the liver or kidneys). 

A biopsy was carried out and we had to wait seven days for the results. In the meantime, they put Jensen on a steroid to try and help as he was now really ill. 

In September 2018, Jensen was diagnosed with diffuse large B-cell lymphoma (DLBCL) with the lymphoma blocking his bowel. 

Jensen was moved to a children’s cancer hospital where treatment with 4 rounds of COPADM (cyclophosphamide, vincristine, prednisolone, doxorubicin and high-dose methotrexate) was started. The medical team explained that his lymphoma was very fast-growing but that the treatment could usually tackle it. 

The next four months were mostly spent in hospital and the chemotherapy was pretty tough. Jensen struggled with horrendous sore mouth (oral mucositis). However, it was noticeable that his stomach was reducing down and he was in less pain. After the third round, it was decided to do a biopsy to check the lymphoma had gone.

The results were discussed at a multidisciplinary team meeting (MDT) and it was agreed that they would take out 10cm of his bowel. A scan following the surgery showed no evidence of the lymphoma. 

In time, Jensen was back at school and back to himself. After two years we felt we were out of the woods. But in 2021 his poo was not right again. An ultrasound scan was arranged to look at his bowel. At the time they did not think it would be a relapse of the lymphoma.

In November 2021 some of Jensen’s bowel was removed which revealed he had a whole new B-cell lymphoma. From there, more tests were done which led to them finding a rare genetic immune disorder called X-linked lymphoproliferative disorder (XLP1), which caused both of his lymphomas. 

XLP1 is a rare immune condition where an affected person cannot properly regulate the development of natural killer cells (a type of lymphocyte) in response to a viral infection. Children with XLP1 have a greatly increased risk of developing lymphoma and other conditions related to the immune system. Because of this, an allogeneic (donor) stem cell transplant was needed to correct the error in Jensen’s immune system.  

Donors for allogeneic transplants are often found in siblings, so his brother was tested to see if he was a match. Ethan wasn’t a match, so the team had to search further. We were fortunate that there were a few good matches, so work was carried out to identify the most suitable for the donor stem cell transplant.

During this time, Jensen needed to have chemotherapy to keep the lymphoma at bay. He had R-ICE (rituximab, ifosfamide, carboplatin and etoposide) from November 2021 onwards which caused him to have a severe electrolyte imbalance. He then had two more rounds of chemotherapy to bridge him to transplant in June.  

This time the treatment experience seemed much tougher for Jensen. Perhaps it was because he was that much older and understood more about what was going on. 

Jensen is normally a pretty happy and very chatty boy, but this was making him feel rubbish, and he was fairly down and moody. We could tell how he was feeling by whether he was talking a lot or not.

The transplant was carried out in June 2022 in a specialist transplant hospital two and a half hours from home. He was in isolation for six weeks, so we tried to make it feel homely by putting pictures up on the wall, having lots of sticker books and playing games like Uno, Dobble, Top Trumps and Brainbox.

I was very keen for him to keep to a routine, so made sure he got up, had a shower and did a variety of things during the day. The routine really helped, and he managed the treatment pretty well with just a few issues. He developed some viruses, but the team managed to keep on top of things. He also developed a bit of GvHD (graft versus host disease), which fortunately was sorted out. 

My husband Jason and I stayed with Jensen during the six weeks isolation. After that, for the next 10 weeks, we stayed close to the hospital as they wanted to see Jensen a couple of times a week.

During that time he did have to go back in a few times as an inpatient with temperature. Jason was back and forth trying to spend time with both our sons.  

I worried about how this would affect Ethan. I knew he was being well looked after by my mum and dad, but I could see that he found it much harder this time. He was now 11 and was missing his family being at home. When my parents brought him over to see us he put his hands out to hold mine and his dads hand. It seemed clear he had really missed us. 

Jensen was off school for 14 months as the hospital wanted to wait until the cells were above a certain level. I am still anxious a lot of the time, and I think it’s going to take a while before I can feel more confident about Jensen’s health; I suspect I will always be anxious about him getting hurt or becoming ill again.   

It’s been tough for both boys. For Jensen, he has been ill much of the time since he was three.  For Ethan he has missed out on time with me, Jason and his brother.  But both have done really well.