Robbie's Law: 25 years in the making

18 May 2015

Robbie's Law: 25 years in the makingRobbie's Law: 25 years in the making

25 years ago on Easter Tuesday, the 17th April 1990, the Powells watched their beautiful ten year old son needlessly die of a suspected disease that was treatable with the administration of daily tablets.

Summary of Events

5th December 1989: Dr Elwyn Hughes [GP] admitted Robbie to Morriston Hospital, as an emergency, by ambulance, at 10 o’clock in the evening. Robbie was seriously ill with vomiting and abdominal pain and was critically dehydrated, with excessive weight loss.

Robbie’s weight 4 years earlier, when he was six, was recorded in the hospital records as being 21 kgs – his weight on admission was only 21.5 kgs. It was later accepted that the child had lost at least 25% of his body weight as a consequence of an Addisonian Crisis. The extent of Robbie’s weight loss is clearly demonstrated by the above photograph in his dressing gown, which was taken 17 days after he was discharged.

Robbie’s electrolytes results were available within an hour or so of admission. He had low sodium 122, high potassium 6.0, low chloride 89, low bicarb 12, high urea 13.8, high creatinine 111 and low blood sugar 3.2, which dropped dangerously low to 1.2 while receiving intravenous dextrose. These results are absolutely characteristic of Addison’s disease and would not be explained by either gastritis or gastroenteritis.

Robbie was an in-patient for four days and received intravenous dextrose and saline to address the critical dehydration. Robbie also received Stemitol for the vomiting and Disprol for pain relief and fever. These drugs were given per rectum ruling out diarrhoea and therefore gastroenteritis.

Addison’s disease was suspected and the ACTH test was ordered to confirm the diagnosis. Although the ACTH test would have confirmed the level of cortisol being produced, by the adrenal glands, both Robbie’s cortisol levels and ACTH levels could have been checked by carrying out blood tests. The results would have shown a deficiency in cortisol and elevated ACTH which is consistent with Addison’s disease. Robbie’s blood could also have been tested for adrenal anti-bodies which, in Robbie’s case, had caused the destruction of his adrenal glands. None of these 3 tests were carried out.

We had also been told that Robbie’s condition on arrival was critical and that the child had been admitted to hospital just in time.

9th December 1989: Robert was discharged.

Although Addison’s disease is treatable, but invariably results in death without treatment, the paediatricians did not inform the Powells of the suspicion or that the ACTH test had been ordered. In fact the Powells were erroneously informed that Robbie had suffered from gastroenteritis, which had been caused by a throat infection, when such a diagnosis was, as we know now, untenable in the absence of diarrhoea.

However, Morriston Hospital informed the Ystradgynlais Health Centre, a practice of 7 GPs, the following:

·  Robbie had a hormonal imbalance;

·  Addison’s had been suspected;

·  The ACTH test had been ordered;

·  The parents had been informed of the ACTH test; and

·  There would be an outpatient appointment arranged for January 1990.

11th December 1989: Robbie still wasn’t eating and drinking properly and was feeling and looking unwell. The health centre was called and Dr Keith Hughes arrived at our home. He was informed of the hospital admission and about the discharge 2 days earlier. We also informed Dr Hughes that Robbie had received intravenous fluids and that gastroenteritis had been diagnosed. He examined Robbie and advised fluids and a light diet.

18th January 1990: Robbie was taken to Morriston Hospital for the out-patient appointment by which time his weight had gone from 21.5 kgs to 28.2 kgs. The paediatrician said he couldn’t believe it was the same child. My wife asked if it had been gastroenteritis the previous December and he confirmed it had been even though Robbie’s symptoms and blood test results could not be explained by this erroneous diagnosis. Again, no mention was made of the suspicion of Addison’s disease or the need for the ACTH test. Robbie’s electrolytes, cortisol, ACTH and blood sugar levels were not checked and neither was his blood checked for adrenal anti-bodies. The paediatrician even failed to take Robbie’s blood pressure. As far as we were concerned all was well and he had fully recovered.

Please note that hyperpigmentation of the skin is a symptom of Addison’s disease that was overlooked by the paediatricians as was Robbie’s two contacts with TB. The TB contacts were actually recorded in Robbie’s hospital records. TB was known to have caused one in five cases of Addison’s disease.  

Following the out-patient appointment the paediatrician wrote to the GPs, keeping them on notice of Addison’s disease, and requesting immediate re-referral back to hospital if Robbie became unwell. 

It is therefore difficult to believe that between the 2nd and 17th April 1990 Robbie was subsequently seen and examined by 5 different GPs on 7 separate occasions with all the characteristic symptoms of Addison’s disease. The child was actually seen on four separate occasions in the last three days of his life, by three different GPs, and still he was not referred back to the hospital until it was too late to save his life.

2nd April: Mr Powell took Robbie to the health centre. Robbie was seen and examined by Dr Elwyn Hughes. He had a sore throat and jaw and was lethargic. This was the GP who had admitted Robbie to hospital, as an emergency, just 4 months earlier. Mr Powell informed Dr Hughes just how seriously ill Robbie had been and that gastroenteritis had been diagnosed. Dr Hughes claimed he could find nothing wrong with Robbie and didn’t read the child’s available and thin medical records. Had he read the discharge documents from Morriston Hospital they would have put him on notice of the suspicion of Addison’s disease and the instruction to immediately re-refer Robbie back to hospital for the ACTH test.

Please note that the GPs accepted post death that any minor illness could precipitate another potentially fatal Addisonian Crisis.

3rd April: Robbie was sent back to school because the all clear had been given by Dr Elwyn Hughes.

5th April: Robbie was sent home from school unwell. In a subsequent letter Robbie’s teacher confirmed the reasons why the child was sent home. She stated:

“On Thursday, April 5th, 1990, after mid-morning break, Robbie complained of being unwell. His colour was rather ‘oriental’ and because of this and the fact that he rarely complained, arrangements were made for him to be collected from school as soon as possible.”

The lethargy and the ‘oriental’ colour were characteristic symptoms of Addison’s disease. The oriental skin colour was due to hyperpigmentation a well known symptom that, as mentioned above, both the hospital and the GPs had negligently missed when Addison’s disease was suspected.

6th April: Mr Powell took Robbie to the heath centre where he was examined by Dr Nicola Flower. The history about the previous hospital admission, the consultation with Dr Elwyn Hughes and the fact that Robbie had been sent home from school unwell, was communicated to Dr Flower. Dr Flower also claimed she could find nothing wrong with Robbie and didn’t read the child’s available medical records containing the crucial information from the hospital regarding the Addison’s disease and the request for immediate re-referral.

Robbie lost his appetite, became weaker by the day and started to visibly lose weight. On the night of the 10th April Robbie was persuaded to eat a meal, which he then vomited back onto his plate.

11th April: Mr and Mrs Powell took Robbie to the health centre as they were both concerned about Robbie becoming critically ill again with vomiting and dehydration. Dr Williams was given the full history about the hospital admission, as known by the Powells, and that Robbie had been seen by two of his partners on the 2nd and 6th April. Dr Williams was also informed of the weakness, vomiting and the weight loss. Dr Williams examined Robbie and read the medical records containing the information about Addison’s disease and the immediate instruction for re-referral back to the hospital. Dr Williams informed the Powells that he would immediately re-refer Robbie back to hospital and prescribed Dioralyte for the vomiting. Dr Williams subsequently informed the police that he had suspected leukaemia.

However, Dr Williams did not inform the Powells of the previous suspicion of Addison’s disease, or that Robbie’s life was at risk. He also failed to provide the Powells with a care plan if Robbie deteriorated further. At this stage Robbie had all the characteristic symptoms of Addison’s disease and was heading for an Addisonian Crises and potential death. In a statement after Robbie’s death Dr Williams confirmed the following:

“An Addisonian crisis is precipitated by an intercurrent illness and the stress it causes.”

We took Robbie home and waited to hear from the hospital regarding the referral. In the following days Robbie became so weak that he couldn’t stand up unaided let alone walk.

On this Easter Bank Holiday weekend my niece and her husband visited us from Swindon. They both recognised that Robbie looked ill. My niece and her husband subsequently described Robbie’s condition in formal statements to the police.

14th April - Easter Saturday [My niece’s husband]: “I was in the room with about eight children, Robert being one. They were all running around about except Robert was lying down with a cover over him. This was unlike him, it was as if he didn’t want to know. He was thin and a pasty colour. He looked very ill and I kept on thinking he was suffering with a serious illness such as leukaemia.”

15th April – Easter Sunday [My niece]: Robbie was “lying motionless on the couch with a cover over him”, “I could see him from the neck up, his whole face was drawn, his eyes were sunken”, “he was a pasty colour, he looked very ill”, “he didn’t say anything to me, this was unusual for Robbie”, “he basically gave me a look, there was no life in him”.

Later that day I telephoned the heath centre and requested a home visit. Dr Boladz refused but instructed us to bring Robbie to the Ystradgynlais Community Hospital, which we did. Robbie was so weak and frail that he had to be carried to the car and then from the car into the examination room of the Community Hospital. Dr Boladz was given the full history, as we knew it, and informed that Dr Williams had re-referred Robbie back to the hospital and had prescribed Dioralyte for the vomiting. Robbie had to be supported as Dr Boladz examined him. Dr Boladz thought that Robbie had Glandular Fever, which is a viral condition. However, Dr Boladz prescribed Amoxicillin, which is penicillin based and, unknown to us at the time, can have an adverse affect on a patient with a viral illness let alone a child with undiagnosed Addison’s disease. Dr Boladz also instructed us to bring Robbie to the health centre on Tuesday the 17th April for blood tests. I carried Robbie back to the car and took him home.

The comments below are from a gentleman who was diagnosed with Addison’s disease when he was 15 years of age.

“I too nearly died from Addison’s disease. I was 15 years old 5.8inches tall and 5 stone when I was finally taken into hospital. I could not stand up without being held up, I could not move under my own steam and I had to be carried everywhere like a bag of bones. I could barely see, my head felt like it was exploding and I was constantly vomiting. I was apparently within 1 to 2 hours of my death and by then I was well ready for it. There is no getting away from the fact that this is a terrible disease to die from.”

Unknown to us, Robbie’s life was fading away before our very eyes.

16th April - Easter Monday:  Robbie spent another day lying on the couch. The child started to vomit froth so we called the health centre and Dr Keith Hughes arrived. The child was by this time very irritable and was hot and cold. As with all the GPs, we gave Dr Keith Hughes the full history, as we knew it, and informed him of Robbie’s current symptoms. Robbie clearly looked thin, ill and gaunt when Dr Hughes examined him and was so weak he couldn’t get up from the couch and had to be raised to sip his drink. Dr Hughes said that he would test Robbie’s blood sugar level and went to his car. When he returned he informed us that his test kit was out of date. Low blood sugar is a symptom of Addison’s disease and the result would have been significant regarding Dr Hughes’s consideration of hospital admission. Please note that Robbie’s blood sugar level was below 1.00 the following day. Dr Hughes refused hospital admission and said that if Robbie continued to vomit or deteriorate we were to call the heath centre and he would be admitted to hospital.

Dr Hughes cancelled the blood tests arranged by Dr Boladz for the following day and rescheduled them for the 18th April, by which time Robbie would be dead. Dr Hughes also permitted us to continue with the inappropriate penicillin based medication prescribed by Dr Boladz the day before. Robbie had, by now, been examined by 5 different GPs on 5 different occasions in the last 14 days and we had been reassured the child wasn’t seriously ill. Not one of the GPs had tested Robbie’s blood pressure or blood sugar levels notwithstanding, we now know, they would have both been dangerously low.

17th April – Easter Tuesday: Robbie didn’t vomit again nor did he appear to deteriorate any further following the examination the day before by Dr Keith Hughes.

As mentioned above Robbie wasn’t eating and had lost his appetite. I remember Robbie wanting a particular soup and going out to the shop to fetch it for him. While out I also bought him a rear tyre for his bike in the hope that it would help cheer him up. When he saw the tyre he said, “Thanks dad” and smiled through his gaunt little face. We had bought Robbie a new Mounting Bike for his Christmas. However, he had ripped the tyre skidding and was very happy to have had a new one ready for when he got better. Little did we know he would never ride his bike again or bless us with his beautiful smiles and love! Robbie was a child that made Diane and I feel very special parents. I would ask him whose boy he was and he would always say “I’m my dad’s boy but I love my mammy”. Our hearts break for Robbie every day.

Because of his condition Robbie hadn’t been going to the toilet but asked if he could go. His mother supported him from his bed in the bedroom to the bathroom but he slipped through her arms unconscious and fell to the floor. Diane screamed for me and I ran to them. Robbie’s eyes were closed and he was lifeless. I immediately ran to the telephone, which was downstairs, and telephoned the health centre as instructed to do by Dr Keith Hughes the previous day – I wish to God I had dialled 999. I told the receptionist “my 10 year old son Robbie Powell has fainted”. She asked, “Is he still unconscious?” I replied, “I don’t know as he is upstairs with his mother - I need a doctor straight away”. The record of this telephone call was altered after Robbie died to support Dr Flower’s untruthful version of events.

I went back upstairs. When Robbie opened his eyes he had said in my absence, “Mammy I can’t see you – I can see you now”. Robbie was carried back to his bed. His pupils were dilated and his lips blue but at least we were happy he was conscious.

Dr Nicola Flower arrived at approximately 3.30 pm and was given a full history regarding the previous hospital admission and that she and 4 of her partners had examined Robbie in the last 15 days due to his deterioration. She was told about the fainting, the dilated pupils, the blue lips and that Dr Keith Hughes; her senior partner, had said the day before that Robbie should be admitted to hospital if he deteriorated. She refused our request for hospital admission and contemptuously dismissed the instructions of her senior partner.

Dr Flower claimed that the throat infection diagnosed by Dr Boladz [Glandular Fever], 2 days earlier, had now moved to his chest. She stopped us giving Robbie Amoxicillin and prescribed Augmentin. This is also a penicillin based medication and therefore contrary and inappropriate regarding a diagnosis of a viral illness. Dr Flower left our home with no further instructions.

By this stage we were really worried about Robbie so decided to call Ward 7 at Morriston Hospital where Robbie had been an inpatient the previous December. Diane and I both spoke to a staff nurse to whom we gave a full history from the December hospital admission to date. We were told that we could not bring Robbie directly to Ward 7, without a GP referral letter, but could bring him to casualty. However, we would then have to wait our turn to be seen by a doctor. We were advised to believe in the GP but to call her out again if we were at all worried.

Robbie developed abdominal pain so we called the health centre again requesting the immediate attendance of a doctor. Dr Flower arrived at approximately 5.30 pm. She again refused hospital admission and only relented following a heated argument. She agreed to admit Robbie but only because of our concerns and not because he was seriously ill. Dr Flower telephoned the hospital and I went upstairs and wrapped Robbie in his quilt expecting an ambulance to be called. I went back downstairs. By this time my sister had arrived and went up to see Robbie – it was approximately 6.00 pm.

In the presence of Diane and my sister I asked Dr Flower if she had ordered an ambulance. She threw the referral letter, which she had just written, across our breakfast bar and said, “No, take him by car”. Dr Flower then stormed out the house without a thought or a care for Robbie or us. Her attitude towards us on that day was absolutely appalling. My sister subsequently provided the following evidence in an official police statement:

“I immediately went upstairs to see Robbie. I spoke to him. Robbie’s lips were purple, his eyes dilated, I mean his pupils were really big.....I remember saying to myself “He’s going to die.”

My brother asked Dr Flower if she had “ordered an ambulance”. She threw the note onto the kitchen unit and it slid across the unit.....And she “replied, “Take him by car”. She turned around and walked out.”

I immediately carried Robbie to the car and lay him on the back seat wrapped up in his quilt. As Diane and I drove him to Morriston Hospital he appeared to be falling asleep but was actually falling in and out of consciousness. We arrived at the hospital about 30 minutes later, which was no later than 6.45 pm.

I carried Robbie into Ward 7 and was told by a nurse to lay him on a bed in a consultation room. We were asked “how long has this child been like this?” We said the GP had claimed there was nothing seriously wrong with him and the Staff Nurse said, “did she now”. She asked if we were the parents that she had spoken to earlier and we said yes. At this stage Diane almost fainted and was taken into another room. I looked at Robbie; his pupils were dilated and eyes fixed in the back of his head with his mouth wide open – he was lifeless. The child had stopped breathing and I had just watched our beautiful son take his last conscious breath. I shouted Robbie but there was no response. I was asked to leave and joined Diane in the other room.

We were told that Robbie would have to be taken to Intensive Care and placed on a life-support machine. Please note that Robbie’s dilated pupils and blue lips [central cyanosis] was recorded on arrival at hospital, which supports our evidence and that of my sister.

On duty was Dr Agarwal who was one of the paediatricians that had suspected Addison’s disease, the previous December, based on the electrolytes. This was the paediatrician that had initially given Diane the erroneous diagnosis of gastroenteritis. Dr Agarwal interviewed us in a private room. They were told Robbie may have encephalitis or a brain tumour and that he needed a CT scan. There was no mention of Addison’s disease notwithstanding Robbie’s electrolytes were almost identical to the December admission, four months earlier. The electrolytes on the night of death were recorded as follows:

Low sodium 117 [December=122], high potassium 6.2 [December=6.00], low chloride 85 [December=89], low bicarb 12 [December=12], high urea 13.1 [December=13.8], high creatinine 135 [December=111] and low glucose <1 [December=1.2].

I asked if there was hope and Dr Agarwal abruptly stated “Of course there’s hope we wouldn’t be trying if there wasn’t any hope”, which reassured me as I thought Robbie had died or was going to die when I last saw him. We asked to see Robbie. He was motionless on a life-support machine, with his eyes still fixed in the back of his head. He was a distinct golden yellow colour. We kissed Robbie, told him how much we loved him, and then left him to be taken for a scan which, we now know, showed no abnormalities and therefore excluded both encephalitis and a brain tumour.

We were told later that Robbie had died – it was 9.45 pm – almost 3 hours after Robbie was admitted and more than 6 hours after Dr Flower had first refused hospital admission.

We were not asked permission to turn off the life support machine and told that the cause of death was unknown. We offered Robbie’s organs for transplant but were told they couldn’t be used because the child’s blood had not been pumped sufficiently around his body.  I was asked to give consent for a post-mortem, which I did without hesitation. We needed to know what had caused Robbie’s death.

The cause of death, confirmed at post-mortem, was auto-immune Addison’s disease. This confirms that adrenal antibodies would have been present in Robbie’s blood during the 4 day in-patient episode, in December 1989, and at the out-patient appointment in January 1990, had the blood been tested.

The GPs continue to deny any responsibility whatsoever for Robbie’s death and say they did nothing wrong:

RIP Robbie xx

Will Powell

NHS Adviser for