Help Harriet

In late November 2012 we went to see the respiratory professor Harish Vyas, at the Queen`s Medical Centre in Nottingham, with Harriet.  We were concerned that Harriet was going to struggle to make it through another winter and that we needed some sort of emergency respiratory plan, should Harriet start to experience problems.

A few weeks later, we were sent a comprehensive plan that was split into three levels of levels of respiratory distress care and what to do should all of these fail.

On Wednesday this week, we went back to see Professor Vyas at the QMC hospital in Nottingham, with Harriet for a review on the new medicine prescribed to help Harriet through the winter months.

We told professor Vyas that Harriet had coped very well over the winter months and had not been admitted to any hospitals since we last saw him in November.

Neither of us wanted to tempt fate by saying Harriet was doing well, but at the same time had to acknowledge that the new medication was helping to control Harriet`s respiratory system and in turn keeping her out of hospital.

Professor Vyas was very pleased that Harriet, was coping better and had adapted well to the Seretide Inhaler and Montelukast tablet, he had prescribed for Harriet on a daily basis.

The Montelukast medication is not normally given to children under four years of age and has not been tested on children. This was something professor Vyas highlighted to us in our last meeting, but we were happy to try anything that would keep Harriet safe.

Professor Vyas then went through Harriet`s current medication including the new Montelukast and Seretide, he decided that they were all fine and that Harriet should remain on all of her current levels of medication. He was also happy to continue Harriet on the Montelukast as she was nearly four and would now come under normal guidelines for this medication.

Harriet had her weight and height taken, before having an examination of her chest and respiratory system.

Professor Vyas was very happy with the way Harriet was maintaining her oxygen saturations and main airways to her chest. He highlighted that Harriet`s chest was filling out well and that we definitely made the right decision, when we put Harriet forward for the scoliosis and kyphosis operation with Mr Michael Grevitt in late 2010.

It was a refreshing change to here a consultant give us some good news about Harriet for once, a bit of hope goes along way.

Professor Vyas asked us if we had heard anything from the geneticist Dr. Suri, that initially looked at Harriet when she was only a few months old.

We told him that we had not seen or heard from Dr Suri or any of his genetic team since we initially took Harriet to see him, when she was only a few months old.

Professor Vyas told us that there was alot going on in the genetic world and that if we were happy, he would write to Dr.Suri and try and get some further tests done, in order to try and find out whether there were any further clues as to what is wrong with Harriet.

The memories came flooding back of when we went to see Dr. Suri and the genetic team, we both initially thought it was for counselling due to us having a disabled child, but quickly found out it was alot more in depth. It was Dr. Suri that referred Harriet to see spinal surgeon Michael Grevitt, who later saved Harriet`s life, so we both owe him alot for his quick intervention.

We both said that we were happy for Professor Vyas to contact Dr.Suri and hoped this would give us some new answers into why Harriet was born this way and whether there was anything we could do to stop this disease or even reverse some of the effects before it was too late.

As we left the office, we had mixed emotions, we glad that Harriet had made some good progress with her respiratory system, but then wondered what would happen with the genetic team.

Jason and Lesley

Harriet had been referred to the Occupational therapy team at the Derby Royal Hospital for hand splints, due the severe problems she was experiencing with both her hands over a number of years.

It seemed that Harriet`s care had not been as smooth as it could have been and without the intervention of a surgeon from the Queen`s Medical Hospital in Nottingham, she would have most likely been left to get alot worse.

The referral letter arrived a few months later and we took Harriet to see the team, at the Derby Royal Hospital. Harriet was measured up for her splints and these were then made while we waited. The team made a great job and we were pleased that Harriet now had something to keep her hands growing in a controlled manor. The splints would also stretch her muscles and keep them from getting too tight.

The Surgeon had also highlighted that Harriet`s ankles were flexing and in fact made one of them click as though it was coming out of its joint. Again we were referred to the Derby Royal Hospital, where we went to see the orthotics team, who looked after ankles joints.

This time Harriet had both her feet cast which took about 30 minutes, she was then cleaned up and we were told the foot support splints would be ready in about 3 weeks. We were given a choice of patterns to select for the splints and that took Harriet home.

After a few weeks had passed, we received a phone call from the orthotics team, to say the foot splints were ready and that Harriet would need to come back into the orthotics department for a fitting.

Harriet had never had foot or ankle supports and we were both pleased when we saw how well they supported her ankles. The orthotics lady told us that Harriet would need to get some shoes, which would help the splints to blend in and at the same time would be beneficial to Harriet when she went in her standing frame.

Today we went back to see Occupational therapy team about the hand splints, they had a look at Harriet and decided the splints needed to be adjusted as they were too wide. The splints were made smaller and refitted to Harriet`s hands.

We were told that Harriet needed to wear the hand splints for at least 12 hours a day, this being for a few hours in the morning, a break and then throughout the night. The team highlighted that Harriet would not need Botulinium injections into her hands, if we kept to a strict regime.

When most people think of Botulinium injections, they think of cosmetic surgery, or injections into the face to stop wrinkles and maintain a person’s looks. But Botulinium injections are also given to children, who have problems with tone; the injections relax the muscles and enable the child to have better movement and also less pain in their muscles. There are risks associated with the Botulinium injection, if too much is given, this can lead to respiratory problems and even death, so the medical professionals are extremely cautions and tend to administer low levels of Botulinium in high risk patients.

Harriet currently has Botulinium injections into her arms and legs, as her tone is so tight that it causes her extreme pain, even though she is taking medication to help control the tone and pain associated with her condition. The problem with Botulinium injections is that they are extremely painful, last time Harriet had a Botulinium injection, and she passed out, as the pain was simply too much for her to take. Alot of children are actually put to sleep, before they are given Botulinium injections, but in Harriet`s case this would simply be too dangerous and medical professionals are not prepared to take the risk.

Now that Harriet has her splints, we hope that we can avoid Botulinium injections into her thumbs and just keep the injections to her arms and legs.

Jason and Lesley

In the search to find a cure for Harriet`s debilitating white matter condition, we have come across many stumbling blocks and have had to do a lot of research into what can only be described as a Stem Cell mine field.

Over the course of the last 3 years, we have constantly been told by senior medical professionals, that Harriet has a debilitating life limiting condition and to try and make the most of the short time we have with her. The doctors in the United Kingdom currently do not have a name for Harriet`s condition, which in turn makes it very hard for us to match a stem cell trial or pay for experimental stem cell treatment.

The United States of America, seem to be the main port of call on lots of new stem cell treatment and stem cell trials, which we are constantly checking on a daily basis.

On contacting some of the main stem cell companies, we were pointed in the direction of the International Society for Stem Cell Research, the world`s leading stem cell research organization, which provides some educational information for patients. The “what to ask” section identifies questions any patient should ask a clinic before considering stem cell treatment. The link to the organization can be found at www.closerlookatstemcells.org .

We were also told by one stem cell Company, that all clinical trials in the United States are registered with the National Institutes of Health (NIH), which maintains a searchable database of those trials at www.clinicaltrials.gov . Currently, the database contains 84,000 + trials with locations in more than 170 countries worldwide.

One of the first stem cell companies we found were based in Texas USA, they would have been happy to take Harriet on for a stem cell trial, but had to refuse Harriet, as we did not live in the state of Texas. There was also a Stem Cell company in Germany, that we were in contact with, but they were closed down before we were able to get Harriet seen by one of their medical team.

We then found that a lot of the stem cell companies across the USA and Worldwide, wanted a firm diagnosis from one of Harriet`s medical professionals in the UK, but were unable to give any form of diagnosis or prognosis and were quickly turned down.

One of the main stumbling blocks, is that the senior medical professionals that surround Harriet, have very little understanding as to what is causing Harriet`s medical problems and simply want to manage the problems, rather than find out what the condition is.

There is some hope, Dr. William Whitehouse, Harriet`s neurologist at the Queen`s Medical Centre in Nottingham, has said that he will continue to help us find our answers, where ever they may be. But has also told us that we must be cautious of Stem Cell companies that promise treatments, they may not be able to deliver.

The Blood core stem cells, from Harriet`s brother Michael are stored at the Future Health Bio Bank in Nottingham, which is only 30 minutes from where we live. These Stem Cells are key to any Stem cell treatment we may find in the next few months or even years. Michael`s Stem cells can be stored for a maximum of 25 years, but Harriet simply does not have time on her side and we must therefore find answers before time runs out.

The endless hospital appointments are necessary to monitor whether Harriet is getting any worse and help prevent any further problems, but there is no light at the end of this road.

Caring for Harriet is 24 hour round the clock management, suctioning is required to keep her airways clear, with medicines throughout the day and night to keep her alive.

Making time to find a stem cell consultant or company is something we try and do every day, writing e-mails to companies and then waiting for a reply they could take weeks or even months and can be very frustrating.

There is also the fund raising associated with getting a stem cell transplant, a rough guide would be about £100,000 , but this would depend on where the stem cell company was situated in the world, but this figure could be significantly higher . To date most fundraising campaigns have stopped, as we simply have not had the time to try and raise the necessary funds, as Harriet`s well being has been our main priority.

In an ideal world we could do with help from a sponsorship company and a medical research company, but we do not live in an ideal world and therefore anything we find will be down to us and us alone.

Jason and Lesley

It is now just over four weeks, since Harriet suffered a serious respiratory attack and was admitted to the Royal Derby Hospital`s, Children Intensive care Unit. Harriet is still not one hundred percent, but is slowly getting back to a stable condition for Harriet.

After Harriet had been discharged from hospital, the real work began, having to maintain her oxygen levels and try and get her back onto a regular feeding plan with all her medicine`s, became a real uphill struggle.

Harriet was not only struggling with her oxygen saturations, but also had problems feeding through her Gastrostomy tube, located in her stomach. We were initially feeding Harriet on a twenty four hour slow feed, but this was then changed to every two hours, with medicines, which simply became too much for us to cope with. A new feeding plan was put together only a few days ago, which Harriet, has thankfully started to cope with and gave us a bit of restbite from Harriet`s ongoing problems.

We made contact with the respiratory professor Harish Vyas, at the Queen`s Medical Centre in Nottingham and asked for an urgent appointment for Harriet, as were fearful Harriet would be admitted back into Intensive Care.

Within a week Harriet was seen by professor Vyas, he had looked at Harriet`s notes and the pattern surrounding her admissions, but also wanted to know what we thought, were the main causes of Harriet`s breathing problems.

We told professor Vyas, that Harriet would start to wheeze as though she had some form of asthma, over the course of a twenty four hour period, she would then develop flu like symptoms and would then start to suffer from extreme respiratory problems.

Professor Vyas wanted to have a good look at Harriet and started to listen to her chest, he seemed pleased that this was clear and that there was no wheeze or problems. He then went onto to say that Harriet`s overall breathing looked good and that she had a nice colour.

Professor Vyas then went through all Harriet`s current medicine`s and had to increase the volumes to some of these, but at the same time, he also wanted to introduce medicines that would help Harriet cope better. One of the medicines he wanted to introduce was called Singulair or Montelukast, the QMC were doing a study on how this medicine helped patients with asthma and it seemed a perfect time to introduce Harriet to the medicine.

We were then asked whether Harriet had been prescribed any form of steroids, which would help her air ways and chest areas. To our knowledge Harriet had not been on any form of steroids at any other hospital`s and was certainly not on any repeat prescription and therefore we both replied no.

Professor Vyas told us that he would need at least a week to sit down and work out a plan for Harriet, which could be combined with Harriet`s current feeding and medicine needs, but would contact us and the relevant medical people by letter to confirm this.

Leaving the office with Harriet, we felt as though someone was now listening to us and that Harriet would now have a fighting chance in the winter months ahead.

As the colder months and darker nights started to draw in, we knew that it was only a matter of time, before Harriet would be at risk from Viruses and infections that came with the colder winter months.

Harriet`s doctor came out to give Harriet and myself the Flu Jab on the 18<sup>th</sup> October, I asked him if there would be any side effects to the flu jab , I was told that it was very unlikely. I had my jab in the top of my arm and then it was Harriet`s turn, she became quite upset but calmed down within a few minutes.

We thought Harriet may become slightly poorly after the injection and kept a close eye on her, but nothing could have prepared us for the days ahead.

Within 48 hours of having the flu jab, Harriet became extremely poorly, she had a raised temperature and was starting to struggle to breathe.  One of our main concerns was that Harriet was breathing so fast and struggling to maintain her respiratory system.

After a constant night of nebulizers and suction to maintain Harriet`s saturation levels, we simply ran out of resources and had to call 999 for an ambulance as Harriet was now in full respiratory distress.

Within five minutes of making the phone call to the emergency services, the fast response ambulance arrived, he quickly assessed Harriet`s condition and helped us stabilize Harriet, just before the large ambulance arrived.

Harriet was then rushed to the Royal Derbyshire Hospital, where she was seen by the team of consultant doctors and nurses. It took more than two hours to get Harriet`s breathing under control, before the registrar inserted a line into Harriet`s hand for fluids and medicine.

It had now been just under four hours, Harriet seemed in a stable condition, but they wanted to observe Harriet for a few more hours before making a decision, as to whether she would be well enough to go home later in the day.

Harriet was admitted to the children`s Puffin ward, it was a ward that Harriet nearly died on a few years ago, when her tracheostomy blocked off, but knew that if we were with her, she would be alright. 

After only a few hours being on the Puffin ward, Harriet`s condition started to deteriorate, there simply were not the staff on this ward to give Harriet the care she needed.  The nurse looking after Harriet at the time seemed to think that she knew more about Harriet than we did and had to make several requests for the registrar to come and look at Harriet.

When the registrar finally came to see Harriet, I told him that Harriet`s condition was getting worse and that I did not feel that this was the right ward for Harriet. The consultant told me that he had already made plans for Harriet to be moved onto the Dolphin Intensive care unit, where there would be someone with Harriet at all times.

A few minutes later, Harriet was moved across onto the Dolphin intensive care unit, the nurse that had been looking after Harriet, signed everything over to the nurses and then went back to the puffin ward.

As I looked at Harriet, not only was she struggling to breathe, but she looked really uncomfortable and in pain and then I noticed her hand, it was almost twice the size as it was supposed to be and looked almost purple and blue. I asked the nurse to look at this, she seemed quite concerned and took the line out of Harriet`s hand. She then explained to me that this should have been picked up by the nurse looking after Harriet on the previous ward, but the main thing is that it was now out.

The registrar came a few minutes later to insert a new line into Harriet`s foot, as they needed to make sure fluids and antibiotics, could be administered quickly.

Harriet`s condition continued to deteriorate over the course of the next few days, the consultants were not sure why Harriet was struggling so much and kept running tests and trying different  medication.

One of our main concerns were that Harriet`s heart rate, was between 180 -190 beats per minute and that she was struggling so much that her heart may simply just give in. The medication that was meant to help Harriet`s airways and breathing, made her heart rate increase, but it was the only way the doctors and nurses could maintain her airway.

The nurses looking after Harriet on the Dolphin ward were very good and really helped us to understand why Harriet was breathing this way.

Professor Choonara and his team regularly came to see Harriet, in order to establish how she was doing and what the best course of action was. He decided that as Harriet had been on intensive care two days and was responding to certain antibiotics, that they now needed to replace the fluids with food, as Harriet needed to start taking on calories. A 24 hour feeding plan was established, which Harriet coped with along with oxygen and regular medication /nebulizers.

Harriet`s condition would seem to improve during the day and then at night her breathing would deteriorate to dangerous levels.

The nurses and doctors continued with their strategy and after four days of being admitted to intensive care, Harriet started to show significant signs of improvement.

The following day Professor Choonara said that Harriet could be discharged from the ward and come home, but only if we had the oxygen and all the other relevant equipment needed to care for Harriet.

Harriet was now home, she was on oxygen constantly throughout the day and night, in order to maintain a good oxygen saturation level. Harriet also required regular suction and nebulizers, just to stay on top of her condition.

The next few days were very testing, Harriet needed alot of care and attention and we were both exhausted, but had to push through the tiredness, just keep Harriet safe.

Today is the 27^th October, Harriet is nearly back to where she was before the Flu injection, but still requires oxygen during the night.

Doctors and consultants told us that it was very unlikely that the Flu jab caused Harriet`s problems, but could not categorically confirm this.

I firmly believe that the Flu jab caused Harriet`s respiratory problems and will think twice, before getting her Flu Jab.

There is also a very interesting piece on the NHS website about Flu Jab, at the bottom of the page, it has a section called “Who shouldn`t have the Flu Jab? “ – CLICK HERE to read the link.  Harriet not only had a bad reaction but also a high temperature, both of these are fundamentally listed on the NHS website.

We would like to say a Big Thank you – to the emergency ambulance crew and all those on the Dolphin ward that looked after Harriet.

Jason and Lesley