1. Christine’s legacy

Christine’s story (www.investinme.org/mestory0041.htm) highlights how the NHS fails patients with hypopituitarism.

She describes how she was given the short synacthen test in 2006, and when she had a normal cortisol result despite her condition worsening, was told that she did not have pituitary disease. Instead, she was misdiagnosed with Chronic Fatigue Syndrome, and it was only much later, thanks to Professor Hooper’s help, that she was correctly diagnosed with hypopituitarism and at long last received the hormone treatment she needed.

My name is Jill and I too was incorrectly diagnosed with CFS on the basis of the short synacthen test administered by the same endocrinologist. I read an article by Christine in a newsletter from the support group Thyroid UK www.thyroiduk.org and got in touch. We became friends and worked together to highlight the shortcomings of the test, and to urge professionals to address the difficulties of testing for cortisol deficiency when it is caused by pituitary dysfunction.

Eventually, we found an endocrine team who were prepared to conduct further investigations. The glucagon stimulation test [1], revealed deficiencies in both growth hormone and cortisol, a steroid hormone essential for life. Our diagnosis was changed from CFS to pituitary dysfunction caused by autoimmune disease.

It is with great sadness that I write of Christine’s untimely death. Since mid-2010, when her bladder stopped functioning, she had been wearing a catheter. She battled bravely with the problem, her stamina and determination were always a wonder to behold, but in March 2011 she received a bitter blow. She had been pinning all her hopes on a leaflet which the Society for Endocrinology (SfE) were producing, which she thought would clarify once and for all which tests should be used for diagnosing low cortisol in adrenal insufficiency from whatever cause. When the leaflet came out it was an utter disappointment. This was when her bowel burst and she lost all hope. It was devastating to see. She discharged herself from hospital and there can only be praise for her husband whose devoted care continued until her death in July 2011.

Her final wish was that the battle she started would save people from being left to suffer because of the inadequacies of the short synacthen test to identify cortisol deficiency in pituitary dysfunction. I think she would also wish to broadcast as widely as possible the events that led up to the publication of the SfE leaflet – events, which deeply shook our faith in the NHS complaints system.

2.  The NHS complaints procedure

In 2007 we each made a complaint about the endocrinologist. The Chief Executive of the hospital did not uphold our complaints. In Christine’s case he wrote, “This is a definitive test used for investigating adrenal failure and these results excluded the possibility of a pituitary abnormality causing your adrenal insufficiency.” In my case he wrote “There was no evidence clinically or biochemically to support pituitary dysfunction.”

We then made separate NHS complaints to the Healthcare Commission (HCC), which again were not upheld.

In Christine’s case they obtained medical advice from a Consultant Endocrinologist. He stated, “That the short synacthen test, which is used for Addison’s disease, is the standard test for adrenal insufficiency widely known by trained endocrinologists in the UK would, to the best of his knowledge, not agree that your test was unequivocally normal.”

The independent endocrinologist for my own complaint wrote, more guardedly, that a Short Synacthen test was often used as a first-line conventional test of adrenocortical function.

In April 2009 we made a joint complaint to the Parliamentary and Health Service Ombudsman (PHSO) that a ‘normal’ result from the short synacthen test did not rule out cortisol deficiency caused by pituitary disease. Our evidence proved that the endocrinologists’ statements to the HCC had been incorrect. We wrote that we were particularly concerned because we thought the first medical witness could have been a clinical expert and patient advocate, selected by the professional/specialist and patient/care groups [2].

We were hopeful that because the PHSO was promoted in their “Principles” document as an independent, customer-focused, open and accountable body, we would receive fair treatment.

However, in September 2009 the PHSO reported that they could not find an endocrinologist who would comment, and in October, after taking six months rather than the normal six weeks, they did not uphold our complaints. Their response did not even mention the word “pituitary”. In contrast to the HCC they did not provide the name or medical qualifications of the Professional Adviser they had used.

The Independent Complaints Advocacy Service (ICAS) wrote that if the PHSO hadn’t missed or misinterpreted any aspect of our complaint we had reached the end of the NHS procedure.

We had followed the complaints procedure for over three years and were disillusioned not to obtain justice. We could have applied for a Judicial Review but rejected the idea because we were uncertain where the responsibility lay and an earlier Judicial Review had been unsuccessful [3]

We wrote to ICAS and the PHSO, including the Ombudsman, Ann Abraham, pointing out that significant information in our complaints had been missed and misinterpreted. We also wrote to Andy Burnham at the Dept of Health, all members of the Houses of Lords and Commons, the Strategic Health Authorities (SHAs) and Patient Safety Groups.

We asked the PHSO in November 2009 under the Freedom of Information Act for full details of their Professional Adviser. They did not respond within the statutory 40 days, and when they finally did in February 2010, it was to provide the adviser’s qualifications – MD, FRCP (Retired Consultant Physician & Endocrinologist) – but not his name. Their excuse was “in considering whether to disclose the information the public interest test was applied, the interest can be met without disclosing the name of the adviser, which would cause an unwarranted invasion of their privacy.”

Our reaction was that the PHSO should have treated as suspect, advice from someone who did not support the customer and wished to remain anonymous. A person paid by a public body to give clinical advice should not be entitled to privacy. However the PHSO wrote they would challenge as premature any application to the court before their internal review procedure was completed.

We were also concerned that a retired health professional would not have up-to-date knowledge and would be likely to have the “fixed attitude about causation” which a recent report had pinpointed among health professionals treating ME [4].

The PHSO asked for a recent acknowledgement by the SfE of the failure of the 250-mcg Synacthen test and of the SHAs’ failure to provide facilities for pituitary disease, which we duly supplied.

In April 2010 the SfE Clinical Committee Chair wrote to me that they would be providing information that would describe reliable ways of measuring a person’s cortisol level (including information on the synacthen test) and that would clarify the relationship between cortisol deficiency and endocrine disorders. We were further encouraged by a letter to Christine in January 2011 from the SfE Public and Media Relations Office, agreeing with her that if a patient received the short synacthen test and the results came back as in the normal range, but the patient still exhibited clinical symptoms characteristic of adrenal insufficiency, then further testing to rule out hypopituitarism should be arranged for the patient. Christine and I were jubilant, believing that our battle had been won. She immediately wrote to the Chief Executive of the hospital - which had failed to carry out further testing.

However, in March 2011, the long awaited leaflet from the SfE was published and all our hopes collapsed. It did nothing to resolve the problem of testing for cortisol in pituitary disease. Despite everything, it implied that the synacthen test was a suitable tool for diagnosing pituitary disease. We were totally frustrated by the NHS complaints procedure. No one would tell us we were wrong, but nothing was being done.

By the time Christine received a reply from the Chief Executive, which confirmed that their practice was now in line with NICE Technology Appraisal 64, she was too ill to query why the PHSO had not advised her of the changes, or why the SfE’s leaflet was inconsistent with their statement in January.

Four months after the publication of the leaflet, she died.

Meanwhile, I made protests about the leaflet to the PHSO, DoH, SHAs and the SfE.

Early in July 2011, the longstanding principle that an expert witness was immune from being sued was overturned [5]. Hopefully, this will ensure in future medical advisers respond truthfully, rather than protecting their colleagues from legal action for negligence and misdiagnosis.

In August the SfE wrote they would be happy to help the PHSO. The SHAs were still concerned about the failure of endocrine testing and NHS service provision for ME/CFS/Pituitary patients and recommended I remain in dialogue with the SfE. Despite advising the PHSO and Christine’s husband writing to his MP, neither he nor I have been told that lessons have been learned from our complaints.

"It is only if health professionals are willing to open their minds to widen their knowledge when faced with patients with problems as complex as ours that they will be able to arrange correct referrals and investigations."

 

3. Education

We have to take responsibility for our health. Christine’s and my experience prove that by educating yourself and others, admittedly not easy when you are not well, you can receive help. Remember there are advances in the medical world all the time; it is not always that our symptoms are ignored for financial reasons.

We are dependent on health professionals to help us solve our problems. No matter how frustrated you get, act in a reasonable manner, remember we are all human and none of us respond well to aggression. Also it is negative to think all health professionals are deliberately obstructive.

The problem is more that medicine is becoming “evidence based”, which can stop health professionals using their clinical expertise of patients’ signs and symptoms when making a referral/diagnosis because the patient has “normal” test results. But it should be remembered that health professionals do have a legal and contractual duty to act in the best interests of the patient.

When you have unresolved health problems there should be meaningful repeat investigations. It may be we have to accept that the answer to our problems cannot be found, but the health professional must explain why. For a patient it is of utmost importance for health professionals to show a caring attitude and give hope.

My case is unique in my hospital’s endocrine department and GP Practice and has taken from 1987 to be diagnosed correctly. My experience leads me to believe that some diseases are only considered rare because the medical profession does not know enough about them. It is only if health professionals are willing to open their minds to widen their knowledge when faced with patients with problems as complex as ours that they will be able to arrange correct referrals and investigations.

4. A message of hope

There is hope, the Department of Health (DoH) wrote in March 2010 that the government recognised CFS/ME as a debilitating and distressing condition. It was a chronic illness and health and social care professionals should manage it as such.

In December 2010, in response to a letter sent to Andrew Lansley, the DoH agreed with the WHO classification of CFS/ME as a neurological condition of unknown cause with many different potential causal factors, including those of a neurological, endocrinal, immunological, genetic, psychiatric, and infectious nature.

In the November 2012 issue of the ME Association (MEA) Essentials magazine, there was a misleading article about the synacthen test. The MEA www.meassociation.org.uk agreed that they would print my response letter sent in the February issue of the magazine. If your symptoms are indicative of pituitary dysfunction and the endocrinologist will only conduct the synacthen test, please show him the explanation why further testing needs to be done to rule out a cortisol deficiency. [6]

With such complex causal factors, each of our journeys will be different. From my experience, it is difficult to imagine there is an easy cure for such chronic illness, but once your problems are acknowledged you will receive help and hopefully it will not be too late for you to lead a good life.

5. Help promote Christine’s Legacy

Information has still not been provided on reliable ways of testing for cortisol deficiency (including information on the synacthen test), which is so vital to clarify the relationship between cortisol deficiency and endocrine disorders. However, in recent weeks the DoH has written that NICE has been asked to develop a clinical guideline and quality standards on adrenal dysfunction http://www.nice.org.uk/guidance/cg/indevelopment/GuidelineReferralsUnderpinStandards.isp.

The Countess of Mar has been regularly updated on Christine’s and my endeavours, and she considers the problem will only be resolved from the “bottom up”. It is up to us to discover which are the most suitable tests and to share the relevant research with endocrinologists. This will ultimately mean that NICE provides a useful guideline on adrenal dysfunction and revises NICE guideline 53 appropriately. 

I hope the good news that I am at last receiving help that I should have received in 1987, which has improved my health considerably, makes you realise that we have to restore our faith in mainstream medicine and the NHS.

Minds must be opened, so if you are having difficulties obtaining help, educate yourself, write to your health professional with full details of your medical problems. This helps both you and them assess the situation and if you are unable to obtain help, the papers will be useful if you need to make an NHS complaint or legal claim.

Christine and I, were a great help to each other, we could not have made this journey on our own. It was sad to see her lose hope because of establishment failure.  Hopefully, publicising her story will ensure her final wish to stop the suffering of patients with pituitary dysfunction will come one step closer.

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