‘These patients literally die because of the lack of proper services’

Gut failure could happen to anyone. It might be the result of cancer, a clot, accidental injury, inflammatory bowel disease or surgical complications. Or maybe, like 18-year-old Dylan Donohoe Sherlock, you were born with bowel issues.

Whatever the cause, if your intestines are incapable of processing and absorbing the fluids, electrolytes and nutrients from your food, you must be fed intravenously. Patients on what is known as total parenteral nutrition, administered through a central line into the chest, are complex cases. The lack of a specialised, inpatient unit in the State to treat and rehabilitate them is, clinicians say, resulting in more than 12 avoidable deaths a year and serious complications for others.

“These patients literally die because of the lack of proper services,” says Niamh Rice, director of the Irish Society for Clinical Nutrition and Metabolism. The society has been campaigning for 10 years for a national unit of expertise to support the estimated 100 people a year who develop severe intestinal failure. Some will go on total parenteral nutrition temporarily; for others it is long term, which they can do at home with the right backup. Across the Border, there is a unit in Belfast with 12 beds serving such patients among a population of 1.9 million; in the State, with a population of 5.1 million, there is none.

Donohoe Sherlock can’t remember life without being on total parenteral nutrition. Initially, a problem with his bladder was spotted in a pregnancy scan and, within two days of his delivery by Caesarean section in Dublin’s Rotunda Hospital in September 2004, he was transferred to Crumlin hospital and fitted with a bladder bag. His mother, Mandy Sherlock, recalls how as a baby he regularly had a distended stomach, bouts of projectile vomiting and was in and out of Crumlin. At age two, he was admitted to the hospital for bowel failure and remained there for the next 18 months.

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“He was really, really unwell, losing loads of weight,” she says. He had a peg feed tube inserted before it became clear his bowel was not absorbing nutrients, so he needed to go on total parenteral nutrition. As his primary carer, Donohoe underwent training over six weeks at Crumlin in the strict protocols for connecting and disconnecting her son for feeding at home. The risk of infection in the central line, down which the total parenteral nutrition flows, is high and sepsis accounts for the majority of life-threatening, and sometimes fatal, complications in patients with intestinal failure.

“He got a good run for about nine years,” says Donohoe. “He had a fairly normal life; okay he had a bladder bag, a colostomy bag and was wearing nappies and you had to very mindful of his central line”, which rules out contact sports and swimming. But he did well at school, sat his Leaving Certificate at age 16 “and is in a relationship with a lovely girl the last 2½ years”.

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However, recent years have been more difficult and since October 2021, he has had nine admissions to St James’s Hospital, lasting three or four weeks at a time, for administration of IV antibiotics to fight infection. He is waiting to be approved for a bowel transplant, which would be carried out in the UK. After Dylan turned 17, he and his mother had to deal with the transition of his care from the specialised paediatric team at Crumlin into adult services. Back in 2017, the deaths of two of the three adolescents who made this transition to care in their local catchment area had highlighted with new urgency the need for a specialised adult unit.

At a meeting that year with then minister for health Simon Harris representatives of the Irish Society for Clinical Nutrition and Metabolism and St James’s Hospital believed that verbal agreement on the requirement and benefits of such a unit was the go-ahead for its establishment. However, only funding for a consultant to manage the transition of adolescents from Crumlin’s specialised team into general adult services eventually materialised.

Gastroenterologist Dr Cara Dunne, who took up that job in January 2020 and now works both in CHI Crumlin and St James’s, explains what a difference a dedicated unit would mean to adult patients, for setting them up to use total parenteral nutrition at home, dealing with any complications and potentially weaning them off it. Currently, she has just one fortnightly outpatient clinic to see St James’s patients who are managing on total parenteral nutrition at home, yet also gets referrals from elsewhere.

As there are no dedicated beds, anyone who need inpatient care has to come through the emergency department, “which is quite terrifying for them”, she says. The importance of protecting their line is drilled into them, yet in an emergency department it is very easy for a health professional to approach the line and not realise they must be completely sterile. Infections in the line can cause blockages and sepsis.

“A lot of my patients end up going on the surgical ward because the surgical nurses are used to total parenteral nutrition – people may go on it for or five days after major surgery,” she says. Patients on home total parenteral nutrition outside Dublin who become ill go to the emergency department in their local hospital, where there is unlikely to be sufficient expertise to assess and treat them.

As Rice points out, the lack of a national unit is a problem not just for the patients but for doctors as well. “Doctors are in a situation where they don’t have the experience, facilities or resources to deal with these complex patients.” For instance, removal of a seemingly infected central line might be the immediate answer but it could have long-term consequences. There is a finite number of access points to the body’s large veins for the administration of total parenteral nutrition. Every time a line is pulled out there is scarring and it can’t go back into the vein. If a patient is on home total parenteral nutrition six nights a week and ends up losing IV access, they will die, says Dunne, who will try to salvage a line first but that, again, is specialised work.

“There are patients walking around who only have one point of access left – their lines are precious,” she stresses. The only option then might be to try to send them to the UK for a transplant. While the five-year survival rate for recipients of small bowel transplants is about 80 per cent, sometimes these patients need both a liver and small bowel transplant, for which the five-year survival drops to 45 per cent.

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A 2016 audit showed that of 67 patients on prolonged total parenteral nutrition, 77 per cent had experienced at least one major complication. Some 54 per cent had required an average of 2.9 emergency admissions in the previous year, lasting an average of 38 total days.

“These complication rates are far in excess of those reported for patients treated in dedicated IF [intestinal failure] units in the UK,” says the Irish Society for Clinical Nutrition and Metabolism. Dunne, who has established a registry at St James’s to track complications among patients with intestinal failure countrywide, says reducing the time people need to be on total parenteral nutrition is another aim.

If you, say, take out somebody’s colon, they lose their brakes, she explains. But artificially slowing down their gut, with medication and diet, will help allow their small bowel to absorb nutrients. The gut will eventually adapt and the ileum, the last part of the small intestine, will make itself look like colon, she explains. Why you need an IF unit, she continues, is so that there is expertise to accelerate adaptation. She believes it would “get patients under control faster, get them home faster and have less total parenteral nutrition if we can”. Also, as there is so much for patients and their carers to deal with, she is very conscious of the current lack of psychology/psychiatry support.

‘They have a right to proper care and they do not receive it’

In October, a detailed business case for a national 12-bed unit to be established at St James’s was submitted to Government. The annual cost for year one of running the centre at 90 per cent capacity is put at €6.04 million, including both staff costs of €2.03 million and non-pay costs.

The estimated cost covers not only recruitment and training of staff but also the bed days, which the patients would be spending in another hospital anyway, Rice points out. “All we are talking about is getting patients in the right unit; they are in hospital somewhere, therefore it is not an added €6 million.” It is, she argues, a “very modest sum for saving people’s lives”.

At the outset of seeking such a unit, Rice never envisaged that they would still be fighting for it a decade later. She has not encountered any politician or clinician who disputes the need for it, yet nothing is done. Her frustration and outrage on behalf of a disparate group of patients who have no voice is clear.

“Just because they are a small number, therefore they can be ignored, this seems immoral to me,” she adds. “They have a right to proper care and they do not receive it.”

Donohoe Sherlock gave his mother permission to talk about his condition because he wants people to know how “brutal” life can be for those who experience intestinal failure. She worries about his mental health as his characteristic positivity is being sorely tested by frequent readmissions to hospital. After starting a pre-nursing course in October of last year, he was only able to attend for a total of seven weeks in six months.

“Dr Cara Dunne and the other staff in St James’s are brilliant but they need more help and support,” adds Mandy, who has four other younger children to care for at home in north Dublin. “Dylan’s a great young fella and to see him going through this is horrible.”

‘It’s a medical procedure and we’re not trained nurses’

Eileen Ryan had a neuroendocrine tumour in her bowel taken out in May 2020 and then developed sepsis a week later, requiring removal of a further section of bowel.

Discharged from St James’s hospital in Dublin after five weeks, she was only home for three weeks before being admitted for nine more weeks. She was rapidly losing weight as it became evident her shortened bowel could no longer adequately absorb fluids and nutrients from her food and she was put on to total parenteral nutrition. Before she could go home, her daughters, Nicola and Fiona, had to be trained to look after the connecting and disconnecting of their mother’s central line for the nightly 12-hour infusion that was keeping her alive.

“We were petrified at the beginning,” says Nicola Ryan, being all too aware of the risk of infection and embolism. “It’s a medical procedure and we’re not trained nurses.” But they wanted their mother out of hospital and back home in Rathdowney, Co Laois, as “it was the middle of Covid and she had been in there for 14 weeks”.

After Eileen had been on total parenteral nutrition for about a year, Nicola went in one morning as usual to disconnect her mother’s line and found her very ill. “She wasn’t able to hold a conversation, not coherent at all.” Nicola rang for an ambulance to take her to Portlaoise hospital but she was discharged that night having been told she had a kidney infection.

They are not trained in Portlaoise to look after such patients, says Nicola. “I knew she wasn’t herself when she got home ... She couldn’t hold a coherent conversation and her temperature was spiking in the night.” The following morning Nicola rang Dr Cara Dunne’s team and was advised to come to St James’s with her mother. Eileen had to go through the emergency department to be admitted, where she was put on IV antibiotics for two weeks and needed to have her line changed.

Eileen, who is 78, has since been able to come off total parenteral nutrition but still requires infusions of fluids, which are done over six hours at a time, four days a week. Nicola, whose brother James was trained to assist their mother after Fiona moved away to Monaghan, stresses the importance of Dr Dunne and her team, for the whole family, as well as in caring for their mother.

“We would be lost without them. I couldn’t see us going through the last two years without them. They have always been at the end of the phone.”

Crohn’s disease

When going on to total parenteral nutrition was first suggested to Kevin Henry, the 67-year-old was a bit reluctant, not knowing what it would involve and fearing he might never come off it. A long-time sufferer of Crohn’s disease, an inflammatory bowel disorder, he had then had a perforated bowel and underwent emergency surgery. Biopsies indicated cancer and the subsequent removal of a section of intestine affected its functioning and he was given a colostomy bag.

At the end of his chemotherapy treatment, he ended up in ICU for several days, with his weight down to 50kg. Sometime later, after being told he was “in starvation mode”, he was admitted to St James’s, where TNP was proposed.

Kevin recalls how Dunne and her team explained that, without it, he would be in and out of hospital and would never put on weight. He felt he had no choice and was glad that at least, once he was stabilised and his wife was trained in the connection protocols, he could have it at home in Leixlip, Co Kildare. A private company that supplies the packs of total parenteral nutrition and all the paraphernalia required for its administration employs nurses to train the carers of adults with intestinal failure.

“I was on that for five nights a week from July of last year to May of this year,” says Henry, who soon started feeling better and gaining weight. A week after he came out of hospital, he was up a ladder in his son and daughter-in-law’s new house helping with the painting.

“Before that I couldn’t button my shirt, I was so weak. I couldn’t butter the bread; I could hardly lift a cup of tea it was so heavy; I couldn’t open a bottle of water.” He put on 20kg in weight over almost a year.

Kevin has been able to come off total parenteral nutrition and just needs to take supplements and magnesium “as the food goes through a bit quicker when you have a stoma”. The colostomy bag is there to stay.

“I don’t like it, who would, but I am lucky to be alive,” he adds. “I’m thankful.”