Herbie’s story

Six-year-old Herbie was diagnosed with NEMO, a mutation of the NEMO gene, in 2011, aged two. At the age of five, he had a 10/10 matched transplant using cells from his brother Rufus. Herbie spent nearly 10 weeks in hospital and is 100 per cent engrafted 9 months down the line.

Emily, Herbie’s mum, shares their experience and her top tips for other parents whose child is going through a stem cell transplant.

Our journey to the transplant

Doctors told us that Herbie might need a bone marrow transplant (BMT) a few years ago, after he suffered his second serious bacterial infection in the short period of 14 months. Using prophylactic antibiotics and immunoglobulin replacement therapy, Herbie stayed well until 2013, but then a serious mycobacterial infection, diagnosed in November of that year, meant a BMT was more pressing and a decision was needed on whether to go ahead. It was a tough, but in many ways inevitable, decision. Without a BMT, it was uncertain whether Herbie would reach double figures. 

Our brave sons

We found out that we had a sibling match early on, and when we were finally informed that it was our then six-year-old son Rufus we had mixed emotions: elation at the perfect 10/10 match which offered Herbie a chance of a normal life, and sadness and worry that our other boy had to do something that was scary.

Rufus was worried about the anaesthetic (‘sleepy medicine’) needed for the donation process. He was scared he wouldn’t wake up. Amazingly Herbie helped reassure Rufus about this as he had had quite a few anaesthetics himself.

Inevitably there were lots of tests prior to the BMT, and in the run up we seemed to be constantly travelling to Great Ormond Street Hospital (GOSH) for lung functions, scans and counselling sessions with social workers and psychologists. I started to feel frustrated that our precious time as a family was being eaten away before the imposed separation needed for Herbie’s BMT.

Herbie’s transplant

On 6 May 2014 we headed to GOSH to start treatment. The doctors warned us that due to Herbie’s ongoing mycobacterial infection the BMT might be rough, with stays in the paediatric intensive care unit. We were also told that there was a 10 per cent chance that the procedure would not be a success. This seemed like a huge percentage when you are dealing with the life of someone so precious to you.

We left our other three children at home with grandparents. I stayed with Herbie in his isolation room and Dan, my husband, had to stay in hotels nearby. Herbie had a double lumen Hickman line inserted to administer the intravenous (IV) meds, and we opted for a gastrostomy (a tube inserted into the stomach) to give oral meds (there were loads) and to feed him should he become unable to eat.

Sailing through the chemotherapy

Herbie sailed through the chemo. Others on the wards suffered sores in the mouth, throat and so on, but Herbie was lucky. Of course, he was on anti-sickness medicines like all the other children going through BMT, and he was never sick. But lots of the other kids were sick despite the anti-sickness meds, and again we felt lucky.

Day zero

On the actual day of the transplant, known as day zero, we found it very hard having Rufus in surgery and the whole worry of that, as well as worrying about Herbie. You just can’t be in two places at once, although you want to be. The actual transplant itself was very much like a blood transfusion, but the nurses made a big thing of it for Rufus, allowing him to come into the room and be with Herbie to start the infusion of the new cells. Rufus then went home that night. Again it was tricky to let him go home post-op with grandparents and not be there to support him.

Day 1 onwards

Eventually Herbie’s hair began to fall out. He’d had long hair, which a stylist had cropped prior to transplant, but eventually it needed shaving off. Herbie was bereft at losing his hair and sobbed the first time he saw himself in the mirror.

At day 12 after the transplant, Herbie got unexplained fevers. Protocol for this meant that he started three new IV antibiotics and IV fluids. The machines ran and beeped all day and all night. He recovered from this first episode but there were other fevers, and then graft-versus-host disease (GVHD) kicked in. The GVHD was of the skin, and it was scary and awfully painful for Herbie. It was eventually brought under control with steroids, but the envisaged 7-week stay in hospital became almost 10 weeks. Every time there was any talk of going home there seemed to be another setback. This was tough, especially when many of the friends we had made were taking their children home before we were. Still, Herbie left with a 100 per cent engraftment.

And getting through

We did a number of things to help us get through Herbie’s BMT. We covered the room in laminated photos of people, places and pets that Herbie chose prior to entering hospital. These kept him connected to his life outside but were a great talking point, too. We encouraged letters from friends, and not a day went by when some post didn’t arrive for Herbie. This really helped break up the day.

The room contained a large whiteboard and, being a teacher, I loved prepping the board daily. We had the date, weather, number of days into the transplant, a list of the things we had to do, such as change the bed linen, and details of meals and oral care. Herbie loved the board and so did the nurses and doctors.

As Herbie was of school age he had hospital school daily in his room, which was invaluable, and there were volunteer playworkers, too.

I think of everything we went through, my biggest shock was how the stories of the other families I met at hospital during the transplant affected me. I not only lived and breathed our journey but also everyone else’s, and that can be exhausting at times. That said, the support and friendship were invaluable and we are all still in touch.

A long and often slow journey

It’s worth remembering that once you get home it’s not over; you are just entering a new phase of transplant. Everyone I’ve met during this process agrees that we all thought once you get 100 per cent engraftment and leave hospital things go back to normal, but they don’t! This is a long and often slow journey. Herbie was re-admitted to our local hospital five times post-transplant with fevers, and I often felt it was harder then than during the transplant process.

Herbie and Rufus now

Herbie is still 100 per cent engrafted 9 months down the line. He attends school for 1.5 hours a day right now but we hope to extend this soon. He’s still on lots of medicines each day (15 different ones, some 4 times a day, plus 3-weekly immunoglobulin infusions and 3-monthly pamidronate) and he has both his lines still in situ. We had daily community nurse visits for IVs from July to December, and the nurses still come weekly for bloods and dressing changes.

Rufus got back to normal within a few days of the transplant. He is proud to have helped his brother but often worries that his bone marrow wasn’t good enough as Herbie is still not ‘back to old Herb’. Thankfully everyone made a big fuss of him. He says: ‘I’m proud of myself for helping him but it was scary. I think I was quite brave!’

Herbie’s likes and dislikes about his BMT

Likes

  • Hospital school
  • The TV 
  • The nurses
  • The food

Dislikes

  • Weekly tests for MRSA, NPA (the soft tube in the nose used to suction the ‘snotty’ stuff to test for viruses)
  • Other tests involving samples taken from his nose
  • Being stuck in his room
  • Not seeing his family
  • Losing his hair

Our top tips

  • 'Face time' family members daily but warn them that sometimes your child might not feel sociable when they call (and you might not either!).
  • Decorate the room to make it feel homely for you and your child.
  • Share the burden of care: take time out of the ward when the offer is there to release you.
  • Prime friends and family to send postcards and letters, but plan it so they don’t all come at once.
  • Keep in close contact with your child’s school so that he/she feels part of the class despite not being there. Herbie’s school Skyped him in assemblies.
  • Take comfortable and cheap clothes to the hospital. I didn’t want to wear any of my ‘BMT’ clothes when I came home.
  • Remember it’s also hard for everyone back at home. They are worried about you and miss you, so make sure they have a good support network, too. Our children were well supported by their school, family and friends, thankfully, but they can still feel isolated.

Posted March 2015