Caring for boys with severe haemophilia A: a family journey

In the first of two blogs about having boys with severe haemophilia A, parents Lauren and Ben discuss early struggles with haemophilia treatment, and overcoming fear, stress and anxiety to master giving their sons intravenous prophylaxis.

Lauren (pseudonym*) has a family history of haemophilia. When she and her husband, Ben* decided they wanted to have children, they knew there was a possibility that they would be affected as she carries the haemophilia gene. They now have two sons, Jacob* and Theo*. Both boys have severe haemophilia A.

“We had genetic counselling,” says Lauren, “Then we had the CVS test (chorionic villus sampling to check for genetic conditions) when I was 12 weeks pregnant and found out that Jacob had the bleeding disorder. With Theo, we just went on the assumption that he would have haemophilia. They took umbilical blood when he was born, and by the time we’d got up to the ward we’d found out that he had it too.”

Knowing from the start

With Lauren’s family knowledge and experience, it would be easy to assume that she might be prepared for having her own children with haemophilia. Lauren views things rather differently: “I think just because I’ve had those experiences, I sometimes think ‘This could turn into something more.’”

Ben also felt anxious, particularly in the early days. “We were in a better position than many because we half expected it,” he says. “But just like parenthood, you can read all the books under the sun, but nothing really prepares you until you’re put in that position. We had firsthand knowledge, people like Lauren’s dad treating and caring for a child with haemophilia their entire life – we had all that experience. But nothing prepares you for it until you’re chucked in at the deep end.”

When Jacob started learning to walk, Ben describes treating him “a bit like a bubble boy”, constantly waiting to catch him in case he fell and had a bleed. However, it’s the journey towards being able to administer treatment in a way that works for the whole family that has perhaps impacted them most.

In at the deep end

Lauren says the first year with Jacob was a blur. He had his first bleed at seven days old, and had intravenous treatment with factor concentrate. He needed further treatment soon after, and at 14 weeks had a port (an implantable venous access device) fitted to help overcome the problems inherent in accessing such tiny veins. Lauren and Ben started to learn how to use the port so they could treat Jacob themselves, but an infection caused problems and the port had to be removed. Then, more treatment issues arose.

“That first one only lasted four weeks, and then Jacob got an infection in the port,” says Lauren. “So, that came out and he had a PICC line (a peripherally inserted central catheter) put in for his treatment. At this point, we were travelling up to London to the treatment centre every day (for treatment) because he also developed an inhibitor.”

Having an inhibitor meant Jacob’s immune system was creating antibodies that stopped his factor concentrate working properly. Daily trips to the treatment centre were necessary as Lauren and Ben were still learning how to treat Jacob. He needed immune tolerance induction, a therapy designed to help his body accept the treatment he needed. It was a stressful and exhausting time for the family – but the therapy worked. Jacob’s inhibitor cleared and he was able to have a second port put in.

Choosing a different way

Once Jacob had his second port, Lauren and Ben really got to grips with how to give Jacob his treatment. Lauren was nervous, though. “We got some kind of life back because we were doing treatment at home from then on,” she says. “But after the infection it really gave me anxieties around accessing the port, thinking whenever I accessed it something was going to happen.”

Later that year, with Covid still an issue and with his younger brother Theo also now being treated via a port, Jacob again ended up in hospital due to a port infection. This time, he was there for weeks due to its seriousness and reactions to some of the medications used to treat it.

Even for experienced healthcare professionals, accessing the veins of babies and young children can be problematic. Lauren and Ben stress just how important ports are in helping to deliver medications that can make all the difference to children’s lives. However, at this point, they decided they wanted to manage Jacob’s treatment in a way that would hopefully mean less risk of infection. So, they learned how to give his factor concentrate intravenously.

The following Christmas, when Theo also ended up in hospital with a suspected port infection, they decided to change to intravenous treatment for him too. For Ben, it felt like a way of regaining control.

“We’d booked to see Father Christmas on Christmas Eve, and just me and Jacob went – Lauren and Theo were in hospital,” says Ben. “I just thought, ‘I can’t do this. I can’t live with this cloud just waiting to turn our life on its head at any moment.’”

Overcoming fear

Lauren and Ben both find that being able to treat intravenously ‘normalises’ the process of giving their sons’ prophylaxis. “I feel like when we can access the vein it becomes a bit more normal,” says Lauren. “It’s less clinical, less medical. Even the set-up is just very much more relaxed.”

However, Jacob’s experiences of being treated for infections in hospital, along with an allergy to the numbing cream normally used to ease injection site pain, mean his parents had to help him overcome his fear. Lauren explains that Jacob was initially so frightened that sometimes several people had to be involved in giving his treatment – it was a difficult time for her and Ben too. “It was quite traumatic,” she says. “It might seem a bit extreme to say that, but it felt like that at the time. He was just terrified because of what we went through in hospital.”

Distraction techniques and finding a numbing cream that Jacob can tolerate helped. Lauren also thinks continuing to have conversations about his treatment and why it’s important has given Jacob a sense of empowerment. “We’re almost giving him the choice,” she says. “We’re still obviously going to give him the treatment, but we’re letting him think he’s got some choice over it, saying, ‘This is why you can do the things you can do, because you’ve had your ‘sharpies’, you’ve got your medicine.’”

Around two years on, Jacob is happy and comfortable for either of his parents to treat him.

“We call it ‘sharpies’, and he actually asks, ‘Have I got a sharpie today?’” says Ben. “If he doesn’t, he’s a little bit gutted because while he’s having treatment he has his iPad or he watches a bit of YouTube or something. That’s how much it bothers him now – he’d prefer to have his treatment because he also gets something he wants. It was super-stressful, but it’s amazing how quickly things have changed.”

Developing a routine

These days, Ben takes pride in being able to treat Jacob and Theo confidently, efficiently and effectively and has a well-practiced routine. But he still carries memories of the stress and anxiety that he and Lauren went through to reach this point.

“Two years ago, I wouldn’t even have imagined it. The level of anxiety… When I was preparing the table, the tray, the needle, I couldn’t even talk to anyone,” he says. “I was just trying to calm down, trying to make sure I’d got everything right. Now, I barely even stress about it.

“I have occasions now where they’re both due treatment on the same day. It’s breakfast time, I keep badgering them, ‘Drink as much water as you can.’ I wrap them up, give them hoodies, get them hot, cream’s on, then they’re eating breakfast and 30 minutes later it takes five minutes and I’ve done both needles, plasters are on.”

Compassion and understanding

Lauren and Ben’s journey through treatment difficulties and the stress and anxiety around it has left them with compassion and understanding for other parents caring for children affected by haemophilia.

Ben’s sureness of his ability to treat his sons has led him to think about the possibility of an alternative career as a phlebotomist. This is partly due to confidence in his skills, but also because his experiences have given him a deep sense of how traumatic venous access in a child is for parents too.

“I know how stressful it is,” Ben explains. “I know it’s the worst day of your life, seeing your kid scream. For someone to walk in and make it quick and simple and easy… just to make people’s lives as easy as possible… I’d love that.”

Reflecting on her experience, Lauren recognises the importance of not being self-critical when learning to treat a child with haemophilia.

“It is tough for everyone,” she says. “Parents, trying to learn ports or whatever, you’re like, ‘Is it me?’ It’s tough for everyone – and everyone goes through the same thing. It’s not you, you’re doing a great job. You just need to not put so much pressure on yourself, relax, it will come.”

“The shakes will stop,” Lauren adds. “It will become your normal – it will become like brushing your teeth. It’s just our daily routine now.”

With our thanks to Lauren and Ben for sharing their story with us

Further information

A number of patient organisations and charities provide support and advocacy for people with bleeding disorders and their families. In the UK these include:

• The Haemophilia Society
• Haemophilia Scotland
• Haemophilia Wales
• Haemophilia Northern Ireland
• Local families with bleeding disorders

About the author

Kathryn Jenner is Communications and Community Manager at Haemnet Ltd.