Important factors to consider when treating children with chronic fatigue syndrome.... Crawley et al

[Denise]

BMC Pediatr. 2017 Feb 1;17(1):43. doi: 10.1186/s12887-017-0799-7.

Important factors to consider when treating children with chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME): perspectives of health professionals from specialist services.
Parslow RM1, Shaw A2, Haywood KL3, Crawley E4.

Author information:

• 1Centre for Child and Adolescent Health, School of Social & Community Medicine, University of Bristol, Barley House, Oakfield Grove, Bristol, BS8 2BN, UK. roxanne.parslow@bristol.ac.uk.
  

• 2Centre for Primary Care Research, School of Social & Community Medicine, University of Bristol, Canynge Hall, Bristol, BS8 2PS, UK.
  

• 3Royal College of Nursing Research Institute, School of Health and Social Studies, University of Warwick, Coventry, CV4 7AL, UK.
  

• 4Centre for Child and Adolescent Health, School of Social & Community Medicine, University of Bristol, Barley House, Oakfield Grove, Bristol, BS8 2BN, UK.
  

Abstract
BACKGROUND:
Paediatric Chronic Fatigue Syndrome (CFS)/Myalgic Encephalomyelitis (ME) is relatively common and disabling. Improving treatment requires the development of Patient Reported Outcome Measures (PROMs) that enable clinicians and researchers to collect patient-centred evidence on outcomes. Health professionals are well placed to provide clinical insight into the condition, its treatment and possible outcomes. This study aimed to understand the perspectives of specialist paediatric CFS/ME health professionals and identify outcomes that are clinically important.

METHODS:
Focus groups and interviews were held with 15 health professionals involved in the care of children with CFS/ME from the four largest specialist paediatric CFS/ME services in the NHS in England. A range of clinical disciplines were included and experience in paediatric CFS/ME ranged from 2 months to 25 years. Ten participants (67%) were female. Focus groups and interviews were recorded, transcribed verbatim and data were analysed using thematic analysis.

RESULTS:
All health professionals identified the impact of CFS/ME across multiple aspects of health. Health professionals described four areas used to assess the severity of the illness and outcome in children: 1) symptoms; 2) physical function; 3) participation (school, activities and social life); and 4) emotional wellbeing. They also described the complexity of the condition, contextual factors and considerations for treatment to help children to cope with the condition.

CONCLUSIONS:
Clinically important outcomes in paediatric CFS/ME involve a range of aspects of health. Health professionals consider increases in physical function yet maintaining school functioning and participation more widely as important outcomes from treatment. The results are similar to those described by children in a recent study and will be combined to develop a new child-specific PROM that has strong clinical utility and patient relevance.


Open access to full paper;
http://bmcpediatr.biomedcentral.com/articles/10.1186/s12887-017-0799-7

 

[Solstice]

Most important factor would be to not let them over-exert and possibly invest in some proper research.

 

[Tyto alba]

In a condition with no objective measures of outcome, it is important to collect subjective measures of health-related quality of life (HRQoL) directly from patients.

I suppose they've never heard of actigraphy for activity and sleep monitoring.

 

[RogerBlack]

I note the peer review comments are open in this case. It would be interesting to see them in other papers.
It would be especially interesting to look at systematic strengths or weaknesses of reviewers.

For example - if three reviewers happen to be picked that never comment on statistical test appropriateness, it would be a big red flag that the peer review means almost nothing with respect to that.
Similarly if you've got someone who's deeply into stats on the reviewer panel, it may be less important to check the stats in detail.

 

[RogerBlack]

If you actually read the paper, the quotes from clinicians are reasonable in general.
It's just the conclusions which are all fitting them into the prior papers viewpoints.
I would quite like to hear the raw discussion of actual clinicians.
There seemed relatively little comment on 'faulty illness beliefs'.

 

[Hutan]

If you actually read the paper, the quotes from clinicians are reasonable in general.

I agree, I'm no fan of Crawley but I was quite surprised and impressed with many of the comments quoted. I was reading expecting to be outraged, but actually I think a lot of those clinicians are being of some use to their patients.

For example, there was a theme of school attendance not necessarily being crucial - that often reducing school attendance allowed for a more healthy and varied life for the patient.

“…they might attend loads of school but all of their efforts are into attending school and they might see no friends and do nothing outside with the family.” (HP11)

“…if they are getting into school more and more but their wellness score is staying the same or going down, I would be concerned and talk with them about decreasing the amount of school they do.” (HP6)

The lack of flexibility of many schools was highlighted as a problem. Anxiety about school was presented as a result of the lack of understanding in schools rather than a cause of ME/CFS.

A lack of school support can hinder reintegration. Some health professional described how returning to school can be difficult for children due to the ‘hustle and bustle’. Children can develop anxiety about their ability to cope. How supportive schools were affected children’s desire to return to school.

Some clinicians seem to understand that there are many paths to achieving a young person's academic and vocational aims and that pushing for full time attendance at a school is often not going to be the most useful approach.

“…we encourage them to leave mainstream school and go into colleges and further education, they are much better at handling people with needs” (HP15)

These ideas are consistent with our experience.

The year my son was entirely home schooled (doing work lying in bed or on the floor) but continued on with his football team was his most productive academically to date and he was happy. The subsequent year when he tried to return to full time school (at the urging of a psychologist who felt school was 'protective') resulted in a major deterioration in health and well-being.

We have found that the flexibility of distance education, including at a vocational tertiary level while still of secondary school age, is very helpful. And colleges set up for returning adult learners and young people who, for various reasons don't fit in standard schools, are better able to cater for part-time face-to-face study and tend to have less rigid, more supportive staff.

There seemed relatively little comment on 'faulty illness beliefs'.

Indeed. I think the paper is on balance mildly helpful. I think if, for example, a GP with limited experience in managing ME/CFS in young people and some pre-existing vague views about ME/CFS being psychogenic read it, they could come away with ideas that will help them serve young ME/CFS patients better.

 

[kangaSue]

Ideally, identifying a cause is better than just learning to live with it.

Severe Nutcracker Phenomenon was something I came across recently that can cause the symptoms. There's not a lot of research around on the subject but it appears a lot of kids grow out of this.
https://www.ncbi.nlm.nih.gov/pubmed/10749295 Does severe nutcracker phenomenon cause pediatric chronic fatigue?

 

[RogerBlack]

Ideally, identifying a cause is better than just learning to live with it.

Severe Nutcracker Phenomenon was something I came across recently that can cause the symptoms. There's not a lot of research around on the subject but it appears a lot of kids grow out of this.
https://www.ncbi.nlm.nih.gov/pubmed/10749295 Does severe nutcracker phenomenon cause pediatric chronic fatigue?

Well - yes.
But ...
"Their symptoms filled the criteria of chronic fatigue syndrome or idiopathic chronic fatigue (CFS/CF). An association between severe NC and autonomic dysfunction symptoms in children with CFS/CF has been presented."

Idiopathic CF is something you have if you _don't_ have CFS.

I do wonder (I have not read the full article) if they are simply using 'CFS' as they think CFS doesn't really exist and therefore it can be meaningfully conflated with 'chronic fatigue'.

The common nutcracker symptoms are not common in CFS.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2878259/
refers to the various symptoms.
'
Hematuria is the most commonly reported symptom and is attributed to rupture of thin-walled varices, due to elevated venous pressure, into the collecting system.48-50 It varies from microhematuria to macrohematuria, occasionally with resultant anemia that requires blood transfusions.3,50-55 Cystoscopy may identify a left ureteral origin.22,24,50,56,57 In a study by Shin et al,30 the causes of isolated hematuria could not be identified by routine methods in 69% of pediatric cases. Of those, 40% were found to have NCP by renal Doppler ultrasonography (DUS); although microhematuria in these patients was 4 times more common than macrohematuria, there were no differences in peak renal vein systolic velocities.

Pain is the next most common symptom. It is sometimes described as part of the gonadal vein pain syndrome, which is characterized by abdominal or flank pain that occasionally radiates to the posteromedial thigh and buttock. The pain is exacerbated by sitting, standing, walking, or riding in a vehicle that shakes.36,58,59 Left flank pain can also be due to left ureteral colic, from the passing of blood clots down the left ureter."

This certainly seems to cause chronic fatigue in some patients.

However, it's absolutely not the main cause of CFS, simply as serious abdominal pain and blood in the urine is not part of the CFS symptoms.

Blood in the urine so bad you need transfusions in some cases.

 

[kangaSue]

@RogerBlack
I'm merely pointing it out as one of the options that can apply that I came across.

You've listed typical symptoms, guidelines which the typical doctor goes by too but if you dig below the surface, there can be huge variation in severity or occurrence of symptoms so it is not unusual to have neither blood or protein in the urine and you can be completely asymptomatic. Only a CT of the abdomen with contrast will rule it out for sure, providing someone skilled in vascular abnormalities is interpreting the results.

 

[RogerBlack]

@RogerBlack
I'm merely pointing it out as one of the options that can apply that I came across.

You've listed typical symptoms, guidelines which the typical doctor goes by too but if you dig below the surface, there can be huge variation in severity or occurrence of symptoms so it is not unusual to have neither blood or protein in the urine and you can be completely asymptomaticI Only a CT of the abdomen with contrast will rule it out for sure, providing someone skilled in vascular abnormalities is interpreting the results.

I can find no reports of many CFS symptoms in this group.
For example, post-exertional malaise.
On what basis do you believe that a non-trivial fraction of 'CFS' have this issue?
Or indeed, any, looking carefully at the full CFS criteria - PEM, cognitive issues, ...
I note that the various recent surveys doing bloodwork on large CFS cohorts would easily have found kidney dysfunction.

Unfortunately, the article is not available online, even for purchase, so one can't see what CFS symptoms they claim to have found.

 

[Dolphin]

Some clinicians seem to understand that there are many paths to achieving a young person's academic and vocational aims and that pushing for full time attendance at a school is often not going to be the most useful approach.

“…we encourage them to leave mainstream school and go into colleges and further education, they are much better at handling people with needs” (HP15)

Pity there is no mention of home tuition.

 

[Dolphin]

If you actually read the paper, the quotes from clinicians are reasonable in general.
It's just the conclusions which are all fitting them into the prior papers viewpoints.
I would quite like to hear the raw discussion of actual clinicians.
There seemed relatively little comment on 'faulty illness beliefs'.

Yes, agree. It was quite good/not bad given the type of people asked:

The services provided activity management, CBT and GET as treatment approaches.

 

[Dolphin]

I read the peer review comments and replies and don't recall of anything of interest.
There was a technical discussion about different qualitative methods which is not something I know anything about.

 

[Dolphin]

They described one overall health outcome domain of participation where school and leisure activities such as sport and hobbies provide children with the ability to participate in normal social structures, with a cumulative impact for improvement.

“So your social participation with your friends might be when you go and play football.” (HP7)

Personally I think it is better if people give up sports like football. There are too likely to lead to payback for days afterwards as well as possibly major relapses.

 

[Dolphin]

“We tend to get a lot with extra issues like anxiety and stress and worrying and low mood coming in then because they have stress for exams.”(HP8)

At a pivotal developmental stage where many young people are completing important exams, having CFS/ME can demotivate them and make them anxious about their future.

Probably true for the odd person but too much motivation could also be a problem if it leads to people pushing themselves too hard.

 

[Dolphin]

Circularity was also described as a feature of the condition. Children experience a ‘boom and bust’ pattern with increased symptom severity (payback) following activity which can lead to a downward spiral of reduced activity. They additionally described the circularity of low mood as maintenance factor preventing improvement in CFS/ME. Children can have low mood due to symptoms and a lack of participation and can then become more vigilant to symptoms. This can then lower their thresholds for participation, further lowering their mood in a negative cycle.
“Um, trying to stop that boom and bust pattern. A lot of them are still really, really pushing themselves” (HP13)
“Obviously, if they’ve got symptoms and that stops them participating, their emotional wellbeing deteriorates and that makes their symptoms worse, so you actually get a negative feedback loop.” (HP15)

A bit annoying though it is certainly true that if you push yourself too hard for certain activities it can cause people to get worse.

I'd make a distinction between doing less activity and one's CFS/ME getting worse. If you underdo it, you can generally bounce back. Overdoing it however can lead to a lower activity ceiling.

 

[Dolphin]

Several health professionals talked about providing hope for the future, particularly for older children looking at alternative pathways for children to achieve their goals. They often used the success of previous patients to encourage and support those at an earlier stage who don’t see a future or even see themselves getting better.
“…CBT has a really important role to deal with some of the being negative, um, views about the illness, and about symptom management and moving people from ‘I’ve got pain and it’s difficult, therefore you know I can’t do anything about it, therefore my pain is worse’, trying to break those cycles as well.” (HP12)
“I’m much more interested in getting them to focus on what they can do.” (HP15)
“I think one of the most important things about our service is giving those young people hope” (HP13)

In this study, setting baselines to reduce boom and bust, realistic individual goals and giving children hope for the future were key treatment priorities.Mackenzie and Wray [39] advocated the importance reassuring patients and their carers that they will recover and go on to achieve academic qualifications.

The paired interview included two clinical psychologists who talked more in depth about the loss experienced by children and the importance of providing hope.

There is a bit of talk of hope and recovery. I agree that giving some hope is useful. However false hope that people will definitely recover can also cause problems also. Also there is a big release of relapse in the condition.

Some people would probably be better to be forewarned. Some lifestyles/jobs are probably not going to see people with ME/are risky for people with ME.

 

[Dolphin]

Health professionals in this study described the difficulty of reducing a child’s school attendance to improve function yet maintaining participation for the child. School has been described as one of the most important outcomes for children with CFS/ME [20] and is a critical protective factor for many adverse outcomes among children and adolescents [50]. Schools were reported to vary in their understanding and support, acting as a barrier to children with CFS/ME returning to normality [51]. Working with schools was a key facilitator described by health professionals to reintegrate children and reduce social withdrawal.

This is from the discussion. There is no mention of home education as I mentioned. Nor of the possible problems with attending school though the full text does mention there can be problems with attending school.

 

[Dolphin]

Anyway I have just picked out some extracts where I have some quibbles. But there are other pieces I liked.

 

[kangaSue]

I can find no reports of many CFS symptoms in this group.
For example, post-exertional malaise.
On what basis do you believe that a non-trivial fraction of 'CFS' have this issue?
Or indeed, any, looking carefully at the full CFS criteria - PEM, cognitive issues, ...
I note that the various recent surveys doing bloodwork on large CFS cohorts would easily have found kidney dysfunction.

I've got no idea how many with CFS could be affected as I weren't looking into this from a CFS aspect. I'm not making any claims that it is the case other than being a possibility for childhood CFS going on that paper I referenced but there was a couple of other papers I saw dated back in the '90's mentioning the same.

With regards to bloodwork though, they won't get a line on this as being involved unless they are testing and comparing renin content directly from both the left and right kidney as renin output increases in the left kidney with Nutcracker but output decreases in the right kidney as a homeostasis balance to maintain a normal renin content in the circulatory system.

The closest connection I can make to it maybe being implied in CFS is that there is a tie in with having POTS in Nutcracker Phenomenon too. It's from the elevated renin level in the left kidney that can cause POTS symptoms in some people I gather.
http://forums.phoenixrising.me/inde...e-–-linked-with-pots-and-childhood-cfs.48793/