It was in or around 1986, that Mirek’s parents began to notice Mirek was a little unsteady on his feet.
Mirek’s primary school education began in September 1987 in St Malachy’s National School, Dundalk.
On the 17th September 1989, Mirek’s parents had Mirek assessed by their family GP, Dr Bernard Knox. Dr Knox felt Mirek had a Cerebellar Ataxia, perhaps due to undiagnosed Encephalitis as a baby. Ataxia is a condition which affects balance and coordination. Dr Knox referred Mirek to be seen by consultant paediatric neurologist, Dr Mary King in Temple Street Children’s Hospital, Dublin.
Dr Mary King assessed Mirek for the first time on the 11th May 1989. Dr Mary King carried out quite a number of tests, physical exam, neurological exam, balance, coordination, and reflexes. Dr Mary King also requested laboratory tests and a CT scan, which were carried out in November 1989.
On the 13th of December 1989, Mirek and his parents visited Dr Mary King at Temple Street Children’s Hospital for follow-up assessment and the results of the CT scan and laboratory tests. All laboratory tests were normal. The CT scan showed nothing significant, other than an enlarged Cisterna Magna, which refers to a normal variant and is an incidental finding on neuro-imaging, and no imaging follow-up was necessary. Dr Mary King noted Mirek improved in overall motor function, was less unsteady, was better able to walk from heel to toe, he had no evidence of a progressive disorder, that Mirek should continue to improve, but that Mirek would need support from his family with his balance when walking and standing.
During the summer of 1990, Mirek began to take a great interest in football. Most mornings during the summer months, Mirek’s mum and his two sisters, would take Mirek to the Christian Brothers football pitch at Dundalk Fair Green, or to the football pitch in the Marist school grounds to play football. Mirek also played football with his friends, and was a substitute player on one of the local junior football teams. During this time, Mirek began to read books and the football news in the newspapers quite a lot.
In 1992, Mirek with his parents and two sisters spent six weeks of that summer in the former Czechoslovakia, two weeks of which they spent with friends in the village of Nové Veselí in Moravia, central Czechoslovakia. During the family stay in Nové Veselí Mirek became ill, and was hospitalised in the nearest town, Žďár nad Sázavou. The Doctors in Žďár nad Sázavou hospital diagnosed Mirek with salmonella, which he possibly picked up from a swimming pool while we were in Prague.
Several days after Mirek was discharged from hospital in Žďár nad Sázavou, the family returned to Prague. The morning after arriving in Prague, on waking up, Mirek was very weak on his feet, and had very painful cramps in his legs. Mirek’s parents decided to take him to the children’s hospital in Prague, where a doctor carried out x-rays on Mirek’s feet. The x-rays revealed no abnormalities, so Mirek was immediately referred to Dr Alena Zumrová, the paediatric neurologist on call for further investigation.
Dr Zumrová first asked Mirek’s mum, what the Irish doctors told them, the parents, about Mirek’s unsteadiness. Mirek’s mum explained to Dr Zumrová that Dr Mary King’s diagnosis was that Mirek had a Cerebellar ataxia, which was not progressive. Dr Zumrová replied, “Mum, that is not so. Your son has a spinocerebellar ataxia, and in my view it could be Friedreich’s ataxia”. Dr Zumrová found it hard to believe Mirek was diagnosed with Cerebellar ataxia, and not spinocerebellar ataxia.
Cerebellar ataxia is a form of ataxia originating in the cerebellum. Non-progressive congenital ataxia is a classical presentation of cerebral ataxias.
Spinocerebellar ataxia is a term referring to a group of hereditary ataxias that are characterized by degenerative changes in the part of the brain related to the movement control (cerebellum), and sometimes in the spinal cord
Friedreich ataxia is a rare inherited disease that causes progressive damage to the nervous system. Symptoms typically appear between ages 5 and 15 years but can begin in adulthood. Damage to the peripheral nerves and the cerebellum (part of the brain that coordinates balance and movement) results in awkward, unsteady movements and impaired muscle coordination (ataxia) that worsens and eventually spreads to the arms and the trunk of the body. Other symptoms include loss of sensory function, speech problems, and vision and hearing loss. Thinking and reasoning abilities are not affected. Many people with Friedreich ataxia develop scoliosis (a curving of the spine to one side), which, if severe, may impair breathing. Some individuals may develop diabetes.
As the family were going home to Ireland the following day, this would not allow Dr Zumrová enough time to carry out further tests. Dr Zumrová recommended to Mirek’s parents, that when they return to Ireland, they should advise Mirek’s paediatric neurologist, Dr Mary King, of her concerns (Dr Zumrová’s), that Mirek may have Friedreich’s Ataxia. Dr Zumrová recommended that Mirek’s parents request special metabolic tests to confirm or rule out her suspicions of Friedreich Ataxia. Dr Zumrová also recommends an MRI brain scan, and an MRI of Mirek’s spine to make sure there was nothing on Mirek’s spine.
Immediately on getting home from Prague, Mirek’s parents made an appointment to see Mirek’s Irish paediatric Neurologist Dr Mary King. At that appointment on August 18th, 1992, Mirek’s parents informed Dr King that Mirek had been diagnosed with Salmonella and had to be hospitalised for several days while on holiday in the Czech Republic, and that several days after being discharged from hospital Mirek developed a weakness and very painful cramps in his legs for which he was referred to paediatric neurologist Dr Alena Zumrová, who suspected Mirek may have spinocerebellar ataxia, possibly Friedreich’s ataxia, but not Cerebellar ataxia, as Dr Mary King advised Mirek’s parents two and a half years previously.
At the follow-up appointment on November 16th, 1992, Dr King advised Mirek’s parents she had carried out the tests and MRI scans looking at the base of Mirek’s brain and the cervical spine, as suggested by Dr Zumrová. All test results were normal, apart from the enlarged Cisterna Magna as already seen on the CT scan in December 1989.
Dr King then informed Mirek’s parents that the results of further tests carried out confirmed a diagnosis of Friedreich’s ataxia, and that there was no treatment or cure for Friedreich’s ataxia and that Mirek’s life expectancy was guarded. Dr King also explained to Mirek’s parents, that when she confirms a diagnosis of Friedreich’s Ataxia, the patient is then referred to the Central Remedial Clinic in Dublin. Dr King advised Mirek’s parents to become members of the Friedreich’s Ataxia Association of Ireland, and offered them literature from the Friedreich’s Ataxia Association.
In a letter dated October 20th, 1993, from Dr King to Dr Vincent Carroll, Senior Area Medical Officer, Community Care Centre, Dublin Road, Dundalk, Dr King writes:-
“Mirek has classical Friedrich’s ataxia which was suspected during a visit to Czechoslovakia when he saw a neurologist and confirmed on investigation here in Dublin”.
Mirek’s parents say they attended numerous Friedreich’s ataxia Association of Ireland (FASI) meetings and socials, and during those times they met many patients who were diagnosed with Friedreich’s ataxia and unknown ataxia’s with ages from 7 to 65. They also met with ataxia patients who got married, or were getting married. A fellow member of (FASI) who was diagnosed with achalasia underwent an operation without complications.
Mirek’s family were devastated with the diagnosis of Friedreich’s ataxia from what they learnt with regards to the progression of this type of ataxia, and the realisation that Mirek would have more serious problems with his balance and his walking in the future. Therefore, Mirek’s parents requested a meeting with Mirek’s teachers at St Malachy’s NS to try and come to some arrangement which Mirek to be allowed to either leave the class before the other students, or to wait and leave when the other students had left the classroom. Mirek’s parents explained to the principal that Mirek had been diagnosed with Friedreich’s ataxia, which affected his balance, and therefore Mirek was unsteady on his feet, and they were fearful he would be knocked over with the rush of the other students in and out of the classroom. Mirek’s parents also explained to the principal, that children and people with ataxia become physically tired, and more mentally exhausted, quicker than others. Therefore, it was important to create a suitable environment for Mirek’s education, and most importantly his safety. But the then principal of the St Malachy’s NS wasn’t at all helpful. In fact he did not want to know, and made no provisions, or allowances for Mirek’s condition.
In a time when Special Needs Assistants were not available, Mirek’s parents had no option but to request the Health Board place Mirek in a smaller school, where his special needs would be accommodated. But we were informed that no such schools existed in our town, except for St. Bridget’s Special school, which was for children with more severe physical and mental disabilities. This meant Mirek’s parents had to balance what was better for Mirek. To either maintain his academic levels with children of his age, by keeping him in St Malachy’s NS, or to take care of his physical safety, and overall well being. Mirek’s parents decided for the latter, since St. Bridget’s special school had smaller classes and more teachers; therefore Mirek should receive the extra attention and allowances needed for his condition, and that he would be cared for more closely.
Mirek was then assessed by a clinical psychologist, who informed Mirek’s parents that St Bridget’s was a school for children with mild mental handicap, and that test findings did not indicate the necessity for special education for Mirek. While Mirek’s parents understood the psychologist’s findings, there appeared to be no solution for Mirek’s safety and well being at school. It was now late September and the school term had already started. As Mirek’s parents believed that it would be unsafe for Mirek to attend his original school, they decided that if Mirek could not be accommodated in either a mainstream, or a special school, they would educate him at home, where he would be in a safe environment. Mirek had special needs which were determined by his balance problems, but his language, reading and mathematics were all considered to be average.
In a letter dated January 1st, 1994, from Prof Peter S. Harper, Institute of Medical Genetics, University Hospital Wales to Mirek’s consultant pediatric neurologist Dr Mary King, Prof Harper describes Mirek in the following extracts as:-
“…a bright 10 year-old boy with a clear ataxia of gait …”, “…he is still an active boy who enjoys football and is generally active.”, “heart sounds were normal and he had no scoliosis.”, “The family history is entirely negative. The parents are healthy and unrelated”. “I think that the combination of past and present findings in Mirek fits very well with the diagnosis of true Friedreich’s Ataxia.” “..there are not really sufficient features of any other cause of Ataxia to make me seriously concerned about the diagnosis here.”.
When the psychologist realised that Mirek’s parents were not going to send Mirek back to a mainstream primary school that refused to make allowances for Mirek’s medical condition, Mirek was reassessed, and Mirek was granted permission to be educated in St Brigids Special School. Mirek really enjoyed his time in St St Brigids Special School, especially as the atmosphere was so relaxed. Mirek’s favourite teacher was Mr O’Kane, who Mirek often referred to as a “big softy”. Mr O’Kane told Mirek’s parents, that every morning, he would present Mirek with the morning newspaper, and allow Mirek some time to read the newspaper and later in the morning, Mr O’Kane and Mirek would have a discussion about articles in the newspaper. Mr O’Kane told Mirek’s parents, that Mirek had a great interest and understanding of politics, and especially social justice. Mirek’s parents also say he had a great sense of justice, and although he was young, he also had great sense of social justice. Mirek knew that it was unacceptable to live and not care if people were hungry, or suffering from all kinds of injustice.
Mirek loved to read books, about footballers and football managers, Robin Hood, The Three Musketeers, the Scarlet Pimpernel, and especially books about his favourite character Zorro. Mirek also loved films about Star Wars and Zorro. But Zorro was Mirek’s great favourite character, because Zorro always fought against injustice.
During his time in St Bridget’s Mirek went on many trips, including trips to Disneyland Paris, and Old Trafford home ground to his favourite team Manchester United. Mirek played football every Friday on St Bridget’s football team. Mirek had a great love for football, and played football almost every day, right up to the day before his operation on October 4th, 1999. Mirek also loved to commentate on soccer matches. So much so, that being a soccer commentator was on his list of possible jobs, when he left school. Mirek also loved to write and would spend many hours at his computer typing match reports, or stories about his heroes. Sports journalism was another possible career Mirek considered on leaving school.
During the period 1992 to 1999, Mirek also went on several holidays to the Czech Republic, and one trip to London all in the company of his family.
Mirek attended his local hospital, Louth County Hospital for physiotherapy. Mirek’s physiotherapist was very kind and giving of his time with regard to Mirek’s physiotherapy. The physiotherapist developed and demonstrated a program of exercises for Mirek’s dad to carry out twice a day on Mirek’s legs to exercise and help prevent his growing muscles becoming stiff and deformed. Mirek also attended the Central Remedial Clinic in Dublin approximately twice a year between 1992 and 1999.
In 1995, the family moved to a bigger house in Bay Estate, in another part of Dundalk. During this period, Mirek was quite happy, and although his balance deteriorated slightly, he continued to play football.
In a letter dated February 13th, 1995, to Dr Mary King from Dr Owen Hensey of the Central Remedial Clinic; Dr Hensey stated:-
“Since I last saw him he has been physically stable with no major deterioration”.
In 1996, when the genetic tests for Friedreich’s Ataxia became available, Mirek’s genetic tests were normal. Therefore Mirek did not have Friedreich’s Ataxia.
During this period, investigations were also carried out for Spinocellebellar ataxia Types 1, 2 and 3, which Mirek tested negative for. Also Hereditary Motor and Sensory Neuropathy Type 1A were also negative.
In a letter dated February 22nd, 1996, to Dr Mary King from Dr Owen Hensey; Dr Hensey stated:-
“He has been physically stable since I last saw him with no particular problems”.
Temple Street Children’s Hospital evaluation charts dated September 30th, 1998, document, “reviewed by Dr King and assessed”, yet Dr King has no written notes in Mirek’s medical chart of this evaluation.
Letter dated October 23rd, 1998, to Dr Mary King from Dr Owen Hensey; Dr Hensey states:-
“generally he has become a little more stable over the recent past”.
The following is an extract from a letter dated December 21st, 1998, by Andrew Green, Professor of Medical Genetics, National Centre for Medical Genetics, Dublin, to Dr Ray Murphy Consultant Neurologist, Tallaght Hospital Dublin:-
“Dear Ray, I saw Mirek along with his parents in the Genetics Clinic on September 23rd, he is their 3rd child and he has two healthy elder sisters. He had been seen by Dr Mary King in Temple Street.
Undoubtedly he has a slowly progressive ataxia.
I had a chance to go through Mirek’s Temple Street notes.”
February 17th 1999, Mirek and his parents attended consultant Dr Lennon’s Gastrointestinal outpatient’s clinic in the Mater Hospital, Dublin, for assessment of Mirek swallowing problems.
Dr Mary King arranged for Mirek to be seen by Prof John Stevenson, a consultant neurologist in Glasgow, who was visiting Temple Street Children’s Hospital, Dublin on April 2nd, 1999. Temple Street Children’s Hospital has no documentation of Prof John Stevenson’s assessment of Mirek from his visit there on April 2nd, 1999.
In a letter dated April 12th, 1999 from Dr Mary King to Dr Knox (Mirek’s GP), Dr King stated the following:-
“I arranged for Mirek to be seen by Prof John Stevenson, consultant neurologist in Glasgow who was visiting here on the April 2nd 1999. He agrees that Mirek has an unusual spino-cerebellar ataxia, probably accompanied by a neuropathy. He noted the negative genetic tests for Friedreich’s ataxia and metabolic studies including creatine kinase, liver function tests, ammonia, lactase, urine organic acids and peroxisomal studies”.
“It may not be possible to put a specific label on Mirek’s condition apart from spinal cerebellar ataxia with neuropathy but I am going to discuss further genetic investigations with my colleagues and we may consider doing a spinal fluid examination of Mirek when he is in for MRI of the spine in a few months time.”
Mirek had esophageal Manometry tests for Achalasia carried out in the Mater Private hospital on April 13th, 1999.
With achalasia, the lower esophageal sphincter fails to open up during swallowing, leading to a backup of food within your esophagus.
Manometry tests will tell your doctor if your esophagus is not able to move food to your stomach normally.
Dr. Lennon, the consultant Gastroenterologist who suggested these tests, assured Mirek’s parents that he would be present for the tests, but failed to turn up on the day. The manometry tests on April 13th, 1999, were performed by a young lady technician who was a student and alone, so she was not supervised by a qualified doctor or nurse, and was very nervous and caused Mirek pain and distress for over one hour, while trying to insert the probe through Mirek’s nose and into his esophagus.
On failing to insert the probe, the student technician then excused herself and left the room. On returning, she was accompanied by consultant gastroenterologist Dr MacMathuna. Dr MacMathuna appeared unhappy that the technician requested his assistance. Before Doctor MacMathuna re-inserted the probe, Mirek’s mum asked Doctor MacMathuna to please use a local anaesthetic so that Mirek did not have to be in so much discomfort and pain. But he just said indifferently that there was no need for local anaesthetic, and he proceeded to reinsert the probe into Mirek’s nose and then turned to the student technician, telling her “you finish the rest” and left the room. The student technician was again left unsupervised and without any assistance, and was finding it difficult to complete the test. She then told Mirek’s parents that she would try to telephone one of her superiors for advice, but was unable to get any of them to answer her call. The technician then disconnected the probe the Mirek’s nose and told us we would be informed of the findings by Dr Lennon.
The oesophagus is the “food pipe” leading from the mouth to the stomach. During esophageal Manometry, a thin, pressure-sensitive tube is passed through the nose, down the oesophagus, and into the stomach. Before the procedure, numbing/local anaesthetic is given inside the nose.
Prof Tom Gorey, the surgeon who was to perform the Heller Myotomy did not discover that the Manometry tests from the Mater Private on April 13th, 1999, were of no use until Mirek was scheduled to have his Heller’s Myotomy surgery. Prof Gorey reordered the Manometry studies. Prof. Gorey told me he would have no problem performing the surgery, as it would be a straightforward procedure, once he had confirmation of the test results from Prof. P. Byrne. The Manometry tests were then carried out in St. James Hospital by Prof. P. Byrne and without causing Mirek discomfort. The Manometry tests confirmed Achalasia.
Heller’s Myotomy, the surgeon makes incisions in the lower esophagus and stomachs external muscular tissues. The incisions reduce the pressure of the lower esophageal sphincter, enabling food to pass more easily through the lower esophageal sphincter downward to the stomach.
On June 3rd, 1999, Mirek and his parents had an appointment with Dr Mary King, in Temple Street Children’s Hospital. On arriving at the hospital, Mirek’s parents were told that Dr King wished to see only Mirek’s parents, and that Mirek was to remain in the waiting room. Dr King explained that she did not intend to assess Mirek during the appointment, and that she wished to discuss Mirek’s manometry tests and his options for treatment. At one point in the discussion, Mirek’s Dad had to leave Dr King’s consulting room for several minutes. During this time, Dr King explained to Mirek’s mum, that Mirek’s prognosis was guarded, and that he would not live very long. Mirek’s parents believe that what Dr King told Mirek’s mum can only be called a prediction, and not a prognosis, because she had no medical evidence to justify her “prognosis”. Just two months earlier on April 2nd, 1999, Dr Mary King had assessed Mirek with Prof Stevenson from Glasgow, and neither Dr King, nor Prof Stevenson documented any concerns that Mirek’s prognosis was guarded, or that he would not live very long. This prognosis/prediction was made without any further assessment or diagnostic tests in the two months that followed the assessment of Mirek on April 2, 1999, by Prof Stevenson and Dr King.
Dr Mary King was Mirek Bingham’s consultant paediatric neurologist between 1989 and June 1999. Dr Mary King assessed Mirek on five occasions during that period:-
- May 11th 1989
- December 13th 1989
- August 13th 1992
- November 16th 1992
- September 30th 1998.
- June 3rd 1999
So, Dr King’s final review with Mirek’s parents on June 3rd, 1999, prior to Mirek’s admission to the Mater hospital, Dr King did not assess, or meet with Mirek at that review. Mirek remained in the waiting room during the entire consultation.
In July 1999, Mirek, his parents and his two sisters went for what was to be their final family holiday to Tralee, County Kerry.
Mirek’s first admission to the Mater hospital was on August 6th, 1999.