Medical News Today helped fund treatment for Daniel Pretty, a young boy living with cerebral palsy. In this article, Daniel’s mom Catherine relates the story of his operation and recovery from the procedure:
My son Daniel is a wonderful, bright, cheerful 6-year-old boy who lives with cerebral palsy. To be specific, he has spastic quadriplegic cerebral palsy. The “spastic” part of that name means that his muscles are very tight, and the “quadriplegic” part means that all four limbs are affected, or more accurately, his whole body is affected by the condition.
He is unable to stand unaided, walk without a walking frame or even crawl along the floor. He depends on adult help to do almost everything that other 6-year-olds take for granted, including dressing or going to the toilet.
We are very lucky, in that Daniel can eat, drink and talk relatively easily, but he is very quietly spoken and cannot take deep breaths, as even these muscles are affected. His dexterity is limited, so handwriting is a real struggle, and there certainly were not any fiddly toys like Lego for Christmas!
Cerebral palsy (CP) is not a degenerative disease, so it does not get worse as the child grows. At least that’s the official line. In reality, the muscle strength that is able to carry the weight of a 3-year-old child often is not able to support a teenager, so many young children that can use a walking frame struggle to do so later in life as they grow older and get heavier.
The predictions for Daniel’s mobility later in life were not great; it was considered almost inevitable that he would end up using a powered wheelchair to get around.
An operation called Selective Dorsal Rhizotomy (SDR) gave us hope. This is a spinal operation that cuts the nerves carrying the most extremely spastic signals, leaving other nerves intact.
A drastic measure, it is one that permanently removes most of the child’s spasticity. A crude version of this operation has been used for many years and involved cutting into much of the spine, but a less invasive operation was pioneered by Dr. TS Park in St. Louis in the US 20 years ago. Dr. Park operates on a single lumbar vertebra, meaning that the risks and the side effects of the operation were minimized.
In 2013, Medical News Today wrote a feature outlining Daniel’s journey to this pioneering surgery.
We first heard about SDR when our son was a toddler, as family and friends sent us newspaper articles featuring a little girl who had gone to the US for the operation. Our local professionals did not have experience of any children having the operation, and they were not enthusiastic about the benefits that it might bring to Daniel, as he is at the more severely affected end of the scale of classic candidates for SDR.
At that time, the operation was not provided or supported by the National Health Service (NHS) here in the UK, so it was something we put to the back of our minds. Over the following couple of years, we were immersed in the day-to-day business of family life, bringing up a disabled child and his younger sibling.
It wasn’t until May 2013 when we were given a rare break and a day of childcare that we were able to sit back and look at the big picture, and think about the long-term implications of doing nothing. I remember walking on the South Downs of the UK in the spring sunshine and talking to my husband Simon – there and then we decided that we should investigate SDR more closely.
We joined two fantastic Facebook support groups, and through those, we met many UK families who had taken their children to the US for the operation. They were hugely enthusiastic about the changes they had seen, so we were encouraged to look further.
The St. Louis Children’s Hospital website is a fantastic resource for anyone considering SDR, and we pored over it daily. From there, we were able to find out more about the operation and precisely what it involved.
The website explains the procedure as follows:
“SDR involves sectioning (cutting) of some of the sensory nerve fibres that come from the muscles and enter the spinal cord. Two groups of nerve roots leave the spinal cord and lie in the spinal canal. The ventral spinal roots send information to the muscle; the dorsal spinal roots transmit sensation from the muscle to the spinal cord.
At the time of the operation, the neurosurgeon divides each of the dorsal roots into 3-5 rootlets and stimulates each rootlet electrically. By examining electromyographic (EMG) responses from muscles in the lower extremities, the surgical team identifies the rootlets that cause spasticity. The abnormal rootlets are selectively cut, leaving the normal rootlets intact. This reduces messages from the muscle, resulting in a better balance of activities of nerve cells in the spinal cord, and thus reduces spasticity.
SDR begins with a 1- to 2-inch incision along the centre of the lower back just above the waist. The spinous processes and a portion of the lamina are removed to expose the spinal cord and spinal nerves. Ultrasound and an x-ray locate the tip of the spinal cord, where there is a natural separation between sensory and motor nerves. A rubber pad is placed to separate the motor from the sensory nerves. The sensory nerve roots that will be tested and cut are placed on top of the pad and the motor nerves beneath the pad, away from the operative field.
After the sensory nerves are exposed, each sensory nerve root is divided into 3-5 rootlets. Each rootlet is tested with EMG, which records electrical patterns in muscles. Rootlets are ranked from 1 (mild) to 4 (severe) for spasticity. The severely abnormal rootlets are cut. This technique is repeated for rootlets between spinal nerves L1 and S1/S2.
When testing and cutting are complete, the dura mater is closed, and fentanyl is given to bathe the sensory nerves directly. The other layers of tissue, muscle, fascia, and subcutaneous tissue are sewn. The skin is closed with glue. There are no stitches to be removed from the back. Surgery takes approximately 4 hours. The patient goes to the recovery room for 1-2 hours before being transferred to the Neurology/Neurosurgery floor.”
As you can imagine, this operation blew our minds. The chance to permanently remove the spasticity that made each day a battle for our son? What an opportunity! Then, we considered the downsides, the risks and the potential worst-case scenarios of such major surgery, and it all seemed so daunting.
SDR is not a cure for cerebral palsy, and it does not make a child walk. All that SDR can do is to remove the spasticity in the muscles in the lower half of the body.
A child like Daniel will still have issues with balance, they will still have core weakness and they will still struggle with dexterity. But it is far easier to work on improving all of these issues if you do not have to battle spasticity at the same time.
Although SDR has been performed in St. Louis since the 1990s, it is still relatively unknown in the UK, so we did not know anyone with experience in the operation to turn to for advice and support. Luckily, the support online was fantastic and we tapped into the experience of a huge community of families and adult SDR patients.
In the summer, we started working with a private physio who specialized in children with cerebral palsy and who happened to have plenty of experience with SDR. She was confident that the operation would help our son.
With this advice behind us, we applied to the hospital in St. Louis and were accepted onto the program. It was a very smooth process to apply to the program in the US; they are clearly used to dealing with international applications, and everything could be done via email and YouTube videos. It was truly medicine in the modern age!
Next came the equally daunting task of fundraising. The operation costs around £30,000 ($50,302) but there are, of course, additional expenses like flights, accommodation and months (even years) of aftercare.
The SDR operation is worthless without a lot of hard work and therapy in the months after the operation, so you cannot risk running out of money during this critical time. The NHS do provide some level of care after the operation, but this varies from area to area and is unlikely to be enough to really maximize the results. Overall, we estimated our target to be £55,000 ($92,220), which would cover our trip to the US for surgery, some rehab equipment and about 1 year’s worth of physiotherapy afterward.
We were put in touch with a fabulous charity called Tree of Hope, who specialize in helping parents like us to raise money for medical care for their children.
They supported us in setting up our Just Giving page and with some ideas for raising such a fantastic sum of money. We set up a website www.onebigstepfordaniel.co.uk, which allowed us to keep in touch with people all around the world. It seemed impossible that we would raise the required total, but in fact, there was a fantastic response from friends, family and from the community. Local businesses, schools and even complete strangers helped out. Family and friends took on huge personal challenges. Within 4 months, the target was achieved and we knew that the operation was possible.
In the meantime, there were improvements to the provision of SDR in the UK. The NHS still do not fund this procedure, but there are a few centers that provide the operation to private patients. Great Ormond Street Hospital (GOSH) started providing the surgery in late 2013. We were keen to get on their list for assessment, and on November 7th, 2013 (by coincidence this was also the day of a huge fundraising gala night, and the day that we reached our fundraising target), we saw Mr. Aquilina and the multidisciplinary team for assessment at GOSH. To our delight, they considered Daniel a great candidate for surgery and we were accepted onto their self-pay program.
Daniel had his SDR surgery on Friday, February 21, 2014, in London, and we had 3 weeks in the area for intensive physiotherapy afterward. The day of surgery was nerve-wracking, to say the least. We had already seen the team the day prior to the operation and had all our questions answered, but nothing quite prepares you for kissing your child goodbye as they go under a general anaesthetic.
Daniel was in the operating room for about 5 hours, while his Dad and I paced the streets of north London nervously. We were delighted to receive the call from Mr. Aquilina, and we were reassured that the operation had gone smoothly.
We met Daniel back in recovery as he was groggily coming around. Straight away, we could see huge changes in his legs. Since birth they had always been stiff as a board, but now they could wiggle and wobble just like anyone else’s. We knew then that this operation was going to be life-changing for all of us.
Daniel moved up to the ward within an hour or so, and we were able to stay with him for his first evening on the High Dependency Unit (HDU). He was attended by two fabulous nurses, so even when we had to leave him to sleep overnight, we knew he was in good hands. The next day, he was gradually able to tolerate a little food and liquid without too much nausea, and by the end of the day, he was able to join the other children on the main ward.
The biggest challenge for a child recovering from SDR is that the clinicians want to keep their spine completely unmoved for several days. This means no rolling, no turning and certainly no sitting up. The angle of his bed could be gradually increased over the following few days, but for the first couple of days he was completely flat on his back.
He had to eat and drink in this position – which is hard enough even for the able-bodied – and there was no way to clean him or to change his clothes if he spilled food or even if he was sick. The anti-emetic medicine given to stop him vomiting was fairly helpful, but he did have a drastic response to one of them (becoming very agitated and angry for a few horrible minutes) so I’m glad that nausea wasn’t too much of an issue for Daniel.
We coped without too much frustration from either Daniel or ourselves. Luckily, our son simply loves listening to music, so he was happy with headphones and his MP3 player. How well a younger, perhaps more mobile child would cope, I don’t know.
The first few days passed without much sleep or peace. Daniel started in HDU and was attached to many monitors and with a catheter for urine, none of which were much fun. Each day, one wire or tube seemed to be removed, and he could sit up a little further, so that by day 3 post-op, Daniel was pretty much back to normal.
The physio team visited us on the Monday, and they very gently tried sitting him up fully. Some kids can find this process quite nauseating, but Daniel coped well, so they transferred him to a “tilting table” where they could gradually bring his body up to vertical without requiring him to bear any weight or make any effort. He coped with that – and enjoyed seeing the view out of the window for the first time – but was exhausted by the end of that day.
On day 4 post-op, the physio session took place in a gym away from the ward. He was finally allowed to be out of bed for 30 minutes at a time, and it was all very exciting for Daniel to finally be able to explore the hospital. Physio sessions took place each morning and afternoon for the next 2.5 weeks.
They worked him incredibly hard, pushing his limits in each session. You could tell they were experienced in getting the best out of the child, with plenty of rewards and encouragement along the way. Even so, it was an incredibly tough period.
We were staying nearby in a self-catering flat, and we tried to keep life for Daniel and his 3-year-old brother as normal as possible during that time. The biggest challenge was fitting in all the daily requirements.
Daniel needed to spend 2 hours wearing “gaiters” to stretch his legs, he needed to spend an hour each day in a standing frame, he attended his physio sessions twice daily (each lasting 60-90 minutes) and we had some active stretches to do as well each day. By the end of each week, we were all desperate for some rest and relaxation.
After 3 weeks, it was time to return home, and we settled back into working and family life again. Daniel was off school for 5 weeks after the operation and then returned on a gradual basis. We are now 3 months post-op, and he is still exhausted each day and has not yet coped with a full week of school.
Part of that is exhaustion after major surgery, but of course he is also learning how to use his “new legs” from scratch, which takes a huge amount of energy. A new born baby learns a map of their body and how their legs move over the first few years of life, but Daniel missed this entire step so he is now trying to work all this out for himself. It’s incredibly hard work, but of course, the rewards may be tremendous.
Daniel’s therapy timetable is absolutely phenomenal. For the next few months, he will be doing seven different weekly activities. There will be two sessions in the water (weekly hydrotherapy with trained physiotherapists, and one swimming lesson with his classmates at school). He will be able to ride twice a week (once with the Riding for the Disabled, and one session of hippotherapy with our private physio, Anna).
He will also see Anna for two 60-minute “dry-land” physiotherapy sessions each week, and finally, he will see our NHS physio Michelle once per week at the community health center for physiotherapy. Of course, there is also an hour in the standing frame and daily strengthening and stretching exercises that we do with him each evening.
But the big question is, what has all this effort achieved? Well, at only 3 months post-op, we are not yet seeing the full picture. We can hope for improvements to really start coming over the next few months and to last for a couple of years. As each new ability comes, it will now be permanent, as Daniel is no longer fighting the losing battle against spasticity.
Already we can see that the way he walks in his walking frame is hugely improved in comparison with what it was before the operation. He is learning to stand up using just quad sticks for balance, and we hope eventually that he may be able to walk using these. In the past month, he has learned far more independence and is delighted with his progress. We can’t wait to see what the next few months will bring.
Daniel calls it his “miracle operation.” And that’s good enough for me!