Nursing is set to be at the forefront of “revolutionary” changes in haemophilia care, but the profession still carries a burden of “guilt” from the contaminated blood scandal that saw hundreds of NHS patients infected with HIV and hepatitis C, according to a leading specialist nurse.
Dr Kate Khair, chair of the Haemophilia Nurses Association, told Nursing Times haemophilia care was now at a turning point with the advent of new treatments that could transform lives and models of care, with significantly more nursing involvement likely.
“My colleagues in adult care struggled a lot with seeing people their age dying quite traumatic deaths”
However, she said “older” nurses like herself who were working in the 1970s and 1980s when contaminated blood products were given to haemophiliac patients could “never forget” the consequences for those people and their families.
Meanwhile, those who definitely knew they had inadvertently given infected blood plasma products to patients could not help feeling a sense of guilt, said Dr Khair, who works at Great Ormond Street Hospital for Children NHS Foundation Trust.
“Batch recording and traceability has always been very important in haemophilia, so some nurses did go back and see that they were the one who actually gave the infected blood to the patient so there is a sense of ‘It’s my fault, I did it’ and big guilt,” she said.
“They may have been caring for a family of three boys with haemophilia who all got HIV at the same time, trying to support the individuals and family as well as struggling with what was an evolving disease – we didn’t really know what it was in those days,” said Dr Khair.
At least 2,400 people died after they were given the infected blood products – which came from abroad – while others have had to cope with living with both haemophilia and HIV or hepatitis C.
Exclusive: Nurses set to lead ‘revolution’ in haemophilia care
“I know that certainly my colleagues in adult care struggled a lot with seeing people their age dying quite traumatic deaths right at the beginning,” said Dr Khair. “Now patients are not dying of HIV so much but are dying of hepatitis C and liver cancer – that is still happening – these patients are dying 30 years later.”
She told Nursing Times that nurses affected had received psychology and team support, however, they continued to be confronted with the devastation caused. For example, relatives of those affected were now coming to her for treatment for the mainly inherited genetic disorder that impairs the body’s ability to form blood clots.
“The thing that is always striking to me is when the daughter of a man who had haemophilia and HIV and died 20 years ago suddenly she pitches up and is pregnant,” she said.
“It is everything that brings up for the family about the potential of another baby with haemophilia and the treatment,” she said. “It is huge and obviously it is going to be there for many, many more years to come.”
Last summer, prime minister Theresa May announced there would be a public enquiry into the contaminated blood issue, but a chair and panel has yet to be appointed.
Then in the autumn more than 500 victims and relatives of those who died as a result of receiving contaminated blood were given permission to launch a High Court action to seek damages from the Department of Health.
While the impact of this particular tragedy continued to be felt, Dr Khair said the field of haemophilia care was on the brink of a revolution, with nurses set to play a leading role in rolling out new treatments.
“It really is one infusion and your life is revolutionised – you don’t have haemophilia anymore”
As a consultant haemophilia nurse at London’s Great Ormond Street Hospital, she combines direct patient care with research focusing on the experiences of children and families with haemophilia.
Treatment for the condition has changed dramatically in the last 25 years or so, with a much greater focus on preventing painful bleeds that can require hospital treatment and teaching and supporting young people and their families to manage the condition themselves, she noted.
Younger patients, in particular, were more likely to have remained “bleed free” but that often meant adhering to an intensive treatment programme involving IV infusions every day or every other day.
“I am working with one child aged nine, who has never had a bleed, which is amazing so it is possible to be bleed-free, but that still comes with that burden of having to infuse every other day – be it Christmas, your birthday, the day you are flying or when you have got a really bad cold,” she said. “You can see why people miss a dose or say they will do it later and then later doesn’t happen.”
Exclusive: Nurses set to lead ‘revolution’ in haemophilia care
However, recent changes in some factor concentrates mean it is now possible to infuse just once or twice a week, giving patients more freedom. Over the last year, nurses have been involved in helping children and adults understand how their revised treatment works and how to manage it.
Meanwhile, even more radical changes are on the horizon with the advent of sub-cutaneous injections to treat the condition and encouraging trials of gene therapy that could eventually provide a cure.
“There are a whole load of drugs that are coming that can be given sub-cutaneously so that means – like a diabetic – you can inject that way rather than IV,” she said. “Some of those injections only need to be given once a week or once a month, so that is going to be revolutionary for people and their families and mean many more are bleed free.”
One drug, which has just been licensed in the US, could be available in the UK as early as this year for a sub- group of haemophilia sufferers with antibodies to factor VIII, while other drugs are undergoing clinical trials and should hopefully be available in the near future.
In addition, researchers have been investigating the use of gene therapy for haemophilia B for several years and have just started trialling the treatment for haemophilia A.
“They have both got some amazing results,” said Ms Khair. “At the moment this is only in adults, because they want to see what the long-term side effects are, but it really is one infusion and your life is revolutionised – you don’t have haemophilia anymore.”
As well as saving younger patients from a life of treatment, the therapy could help prevent further deterioration in patients aged 30 and up who have already suffered arthritic damage to their joints, as a result of internal bleeding, and are more prone to bleeds.
“It is really great to have that long-term contact with families and individuals”
Meanwhile, numbers of people with haemophilia in the UK are on the increase and this is likely to keep on growing as treatment becomes yet more advanced, making it easier to live with the condition.
“When I started as a haemophilia nurse there were hardly any families that had more than one child with haemophilia, because it was a horrible disease and the treatment was difficult,” she said. “It is still a horrible disease, but the treatment is so much easier that it has become more normal to have more than one child with haemophilia.”
Female siblings who carried the disease were also more likely to have children, having seen their brothers grow up “completely normal apart from the odd injection”, she added, meaning general nurses were more likely to come into contact with people with the condition.
“In another 50 years’ time there will be many more people with haemophilia than we have now and that will mean nurses that don’t do specialist haemophilia care are more likely to come across people with bleeding disorders than they have been in the past,” she said.
Expert nurses would, therefore, have a key role in raising awareness and when it came to peer education and support, she added.
Dr Khair, who initially worked in haematology oncology before moving into haemophilia care, admitted she had not intended to stay in the field, but was drawn in by the sheer variety and the enduring relationships formed with families she supported long-term.
“I thought ‘I will do this for 18 months’ and here I am 25 years later thinking it’s really exciting still,” she said. “It is the variety – so you can go into work one day and think it is going to be really quiet and 20 things happen.
“It is also the longevity of the condition,” she said. “There are young men that I am looking after that I knew before they were born. You’ve known them, their mum, dad, granny and everybody in the family for years and years and that is a really nice relationship that I don’t see many of my other nursing colleagues having. It is really great to have that long-term contact with families and individuals.”
“There is great scope for different ways of offering and delivering care that could be very much nurse-led”
With much haemophilia care in the UK being nurse-led, she said nurses would be at the forefront of introducing new treatments to patients. “For nursing, the move towards subcutaneous and once-a-month injections is really exciting,” she said.
“As a patient, do you need to come to hospital to see doctor if you are not bleeding and everything is fab and lovely? There is great scope for different ways of offering and delivering care that could be very much nurse-led,” she told Nursing Times.
“That could be outreach clinics or community outreach and all sorts of new ways of caring for people with haemophilia that are already out there and mean we are taking yet another step forward,” she added.