Letter from Society of Endocrinology page 1
Letter from Society of Endocrinology page 2
Jill Mizen’s Tribute to Christine - February 2012
After Christine’s death in July 2011, her friend Jill Mizen wrote the following account of her brave fight for proper diagnosis of hypopituitarism. Anyone who has illusions that the NHS Complaints system is fair and unbiased should read it.
1. Christine’s legacy
Christine’s story (www.investinme.org/mestory0041.htm ) described how she was given the short synacthen test in 2006 by an endocrinologist at York Hospital, and when she had a normal result, was told that she did not have pituitary disease. Instead, she was misdiagnosed with Chronic Fatigue Syndrome. It was only thanks to Professor Hooper’s advice the next year that she was eventually given the more reliable glucagon test  in 2008. This test confirmed that she did indeed have hypopituitarism, and she did, at long last, receive the hormone replacement treatment she needed.
My name is Jill and I too was incorrectly diagnosed on the basis of a normal result to the short synacthen test. I read an article by Christine in the support group Thyroid UK’s newsletter and got in touch. We became friends and worked together to highlight the shortcomings of the test, and to urge professionals to address the testing difficulties when cortisol insufficiency is caused by pituitary disease rather than a problem in the adrenal glands themselves.
Eventually, thanks to the glucagon test, we were both diagnosed with pituitary dysfunction caused by autoimmune disease.
Christine wrote her last personal update to her story in February 2010. It is with great sadness that I am writing to report her demise. 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 cortical deficiency in pituitary disease. 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 York Hospital endocrinologist. The Chief Executive 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 [sic] “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 since the first medical witness was believed to have been nominated by the professional/specialist and patient/care groups as a clinical expert and patient advocate .
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 to comment on our complaints, 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 .
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. .
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.
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.
And nothing has been done since then, despite protests to the SfE and the PHSO. Christine’s only consolation was that when she wrote to the CEO of York Hospital to ask whether changes had been made to prevent the problem recurring for other patients, he responded “The question raised was whether hypopituitarism is considered and tested for in someone who receives a normal short synacthen test but still exhibits clinical symptoms characteristic of adrenal insufficiency. The straightforward answer to this question is yes; in these circumstances pituitary insufficiency is most definitely considered as a possibility. Our practice is certainly in line with national guidance.”
By the time Christine received this letter she was too ill to query why the Ombudsman 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.
Mr Wrightson wrote to his MP, but neither he nor I have been told that lessons have been learned from our complaints.
We have to take responsibility for our health. Christine’s and my experience prove that by educating yourself and others, which is not easy when you are not well, you can receive help. It should be remembered 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 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.
The Countess of Mar has been regularly updated on Christine and my endeavours, and she considers the problem will only be resolved from the “bottom up”. 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. Have faith in yourself and never give up hope.
Early in July 2011, the long-standing principle that an expert witness was immune from being sued was overturned in the Supreme Court decision of Jones v Kaney. The Medical Protection Society (MPS), Director of Policy and Communication, Dr Stephanie Brown said, “Having lost their immunity, experts are now exposed to the risk of evidence given in court”. MPS indemnity extended to expert witness work and they would be monitoring whether the ruling would open the floodgates in terms of court proceedings.
Hopefully, this will ensure in future medical advisers respond truthfully, rather than protecting their colleagues from legal action for negligence and misdiagnosis.
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.
My endocrinologist in June last year used his clinical expertise, not just evidence-based medicine. He wrote about my TSH blood test results, “Although your TSH is slightly lower than the normal range I would not recommend a change in the dose of Levothyroxine unless you exhibit symptoms to suggest overactive thyroid. During the clinic your thyroid status appeared normal but if this changes and you develop symptoms to suggest overactive thyroid then I would suggest that you reduce the dose of Levothyroxine to 75 mcg and 100 mcg on alternate days.”
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.
The Pituitary Foundation www.pituitary.org.uk , are trying to raise awareness of the long and difficult journey patients face trying to obtain a diagnosis of pituitary dysfunction.
In the November 2012 issue of the ME Association (MEA) Essentials magazine, there was a misleading article about the synacthen test. The MEA agreed that they would print my response letter sent in the February issue of the magazine. I have given this letter as an appendix to this account. If your symptoms are indicative of pituitary dysfunction and the endocrinologist confines himself to conducting only the synacthen test, please show him a copy of the letter, which explains why further testing needs to be done to rule out a cortisol deficiency.
Christine and I were a great support to each other; we could not have made this journey on our own. Our passion to fight to make people aware of how and why people with pituitary disease suffer before they get a diagnosis, with your help will continue despite Christine’s untimely death. Good luck.
 The glucagon test is advocated in NICE Technology Appraisal 64 to identify growth hormone deficiency. It also tests for cortisol, which is one of the adrenal steroid hormones essential for life. [Note from J Lane: See http://www.ncbi.nlm.nih.gov/pubmed/19996199 for a discussion of this use of the glucagon test.]]
 Appendix B, para C of NICE Technology Appraisal 64)
 Douglas Fraser 2) Kevin Short and NICE about Clinical Guideline 53 Neutral Citation Number: (2009) EWHC Admin (452). Case No: CO/10408/2007 and CO/10435/2007
 December 2009, Interim Report on the Inquiry into NHS Service Provision for ME/CFS, by the All Party Parliamentary Group (APPG) on ME. Recommendation 8 highlighted the difficulty of finding a suitably qualified clinician to provide unbiased advice. It noted evidence that there were serious concerns about acceptability, efficacy and safety with some treatments and gave one reason as fixed attitudes about causation among some health professionals.
Jill Mizen’s Response to the article in the ME Association Essentials magazine
I would like to respond to Dr Shepherd’s advice in the Autumn 2012 issue, on the use of the short synacthen test (SST). The test is easy to administer and increasingly popular; and is highly reliable for diagnosing primary adrenal insufficiency (Addison’s disease) when the problem lies in the adrenals themselves. However, it is important to remember that it cannot be used to exclude secondary adrenal insufficiency, caused by the pituitary gland’s failure to stimulate the adrenal glands. You may have a normal result to this test but still have pituitary disease.
In 2011, the Society for Endocrinology (SfE) wrote to the late Mrs Christine Wrightson (whose story can be found on www.investinme.org/mestory0041.htm as follows:
“Our Clinical Committee … agree with your analysis of the situation that if a patient receives the short synacthen test and the results come back as in the normal range, but the patient still exhibits clinical symptoms of adrenal insufficiency, then further testing to rule out hypopituitarism should be arranged for the patient.”
A convenor of the SfE’s Special Interest Group for Laboratory Aspects of Clinical Endocrinology states:
“The Synacthen test does not test the whole pituitary-adrenal axis”. 
A 2003 review covering 36 years of research found that the synacthen test has only 57% - 61% sensitivity for adrenal insufficiency caused by pituitary failure i.e. misses two people in every 5. 
My own experience as well as Christine’s supports this. In 2006 and 2010, I had the short synacthen test with normal results. However, my symptoms persisted and in 2012, the glucagon stimulation test  confirmed adrenal insufficiency due to pituitary disease. Cortisol was low and I am now benefiting from the prescription of hydrocortisone.
 Dorin RI et al, Diagnosis of adrenal insufficiency, Annals of Internal Medicine 2003; 139: 194 –204. This review “searched the Medline database for all research on the topic between 1966 and 2002 and found that the sensitivity of the 250 microg (synacthen) test is 57% and the sensitivity of the 1 microg test is 61%.”
 [Joanna Lane wishes to add:] Although the glucagon stimulation test can pick up adrenal insufficiency due to pituitary dysfunction, as it did in Jill’s case, it is not 100% reliable when used for this purpose. See http://www.ncbi.nlm.nih.gov/pubmed/19996199 . The gold standard test for secondary hypoadrenalism remains the insulin tolerance test.
Jill Mizen, February 2013