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ANNOTATION
Year : 2015  |  Volume : 1  |  Issue : 2  |  Page : 65-69

My journey into the world of treating children with cerebral palsy


UCLA Department of Surgery, California, USA

Date of Web Publication7-Jan-2016

Correspondence Address:
Warwick J Peacock
UCLA Department of Surgery, 10833 LeConte Ave., Room 72-243 CHS, Los Angeles, California 90095-1749
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2395-4264.173428

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How to cite this article:
Peacock WJ. My journey into the world of treating children with cerebral palsy . Indian J Cereb Palsy 2015;1:65-9

How to cite this URL:
Peacock WJ. My journey into the world of treating children with cerebral palsy . Indian J Cereb Palsy [serial online] 2015 [cited 2017 Jul 27];1:65-9. Available from: http://www.ijcpjournal.org/text.asp?2015/1/2/65/173428

While I was a little boy growing up in Johannesburg, South Africa, I remember that high on the hill above our house, there was a tall stone building reaching up into the sky called The Hope Home. My parents told me that it was where little children with cerebral palsy were taken because their bodies were bent and doctors would operate on them to make them straight and they had to stay there until they were better. As a little boy, walking to school each day, I would look up at the forbidding hope home and feel fearful and sad for the children who were being kept away from their homes and families. Fearful, because of the treatment, they had to undergo and sad because there seemed to be nothing I could do for them.

Little did I know that 1 day, I would devote most of my working life trying to help these children and convince my colleagues that there were alternative ways of treating cerebral palsy, ways that might prevent the bones from becoming bent and make movement easier.

During my years at medical school, we learned very little about cerebral palsy other than its brief definition: Cerebral palsy is a motor disorder due to an insult to the developing brain. We also learned that because of some degree of brain injury at birth many of these children had stiff (spastic) muscles, which made movement patterns difficult and, secondary to the tight muscles, the bones and joints would become deformed. Sadly, there was very little in the way of preventative treatment at the time.

I graduated from the medical school at the University of Cape Town and spent time working with the great doctor, Professor Chris Barnard (who performed the first heart transplant) and after practicing as a family doctor in a small country town, I specialized as a pediatric neurosurgeon and, as the first one in Africa, I was kept very busy.

One day, I was approached by a local pediatric neurologist who said "You are now an expert in the treatment of nervous disorders in children, and we expect you to help us with the treatment of patients with cerebral palsy." My response to her was "But I know nothing about the condition except that it affects muscles, bones, and joints which make it difficult for the children to move. So, how can a neurosurgeon be of help?"

She educated me very quickly by explaining that the primary pathology in cerebral palsy is in the brain and that the muscles are secondarily affected making them stiff or spastic. The persistently spastic muscles then exert an abnormal effect on the bones and joints, pulling them into deformed positions.

She gave me some published articles to read which dealt with the cause of spasticity and how some European surgeons had used this understanding to develop surgical procedures to reduce spasticity.

The first article [1] was by Sir Charles Sherrington, a great British neurologist, in the 1890s. He had divided the brainstem in experimental animals and thereby inducing spasticity in all four limbs. He realized that in cutting across the brainstem, he had cut the nerves descending from the brain into the spinal cord that reduce the tone (degree of muscle tightness) in the limb muscles. He then opened up the spine to expose the spinal cord and the spinal nerves. He decided to cut the sensory nerves coming back from the muscles to the spinal cord and found to his amazement that the muscle tightness or spasticity had disappeared. His conclusions were that damage to the brain causes spasticity and cutting the sensory spinal nerve roots relieves spasticity.

Foerster, [2] an innovative German surgeon, then used this information to devise a surgical procedure to relieve spasticity in children with cerebral palsy. He opened the spine and cut completely the sensory roots entering the spinal cord from the lower limbs. The spasticity was reduced, and function was improved. However, despite his good results, others did not use his method, possibly due to the fact that in cutting each nerve root completely, he may have produced an unacceptable loss of sensation or interference with bladder function.

Fasano et al. [3] from Turin in Italy took the procedure a step further. It was well known at that time that the sensory nerve roots divide into a number of rootlets before entering the spinal cord, so instead of cutting the whole nerve root, Fasano separated each one's component rootlets and would only divide some of them. He used an electrical nerve stimulator and found that some of the nerve rootlets, when stimulated, produced a normal response in the muscles whereas certain other nerve rootlets produced an abnormal or spastic response. He cut the nerve rootlets associated with an abnormal response and left intact those with a normal response.

Armed with this knowledge, I asked Professor Barnard if I could test our nerve root monitoring system on one of the experimental animals in his laboratory. After some difficulties, we felt that we could reliably use the nerve stimulating technique. We now had to take the bold step and do the procedure on a child.

I remember the first case so well. He was a 5-year-old severely affected spastic quadriplegic child who had the very little functional motor ability. We performed a limited T12/L1 laminectomy and exposed the nerve roots over the lower end of the spinal cord and carefully stimulated each one in turn, dividing those associated with an abnormal response and sparing those with a normal response. The surgery took many hours but eventually we closed the wound, and he went back to the ward. He recovered well, and when assessed 6 months after the surgery, we found that the spasticity in his muscles had gone, and with support, he could now stand because his legs were no longer scissoring. Furthermore, with support, he could take a few steps, which had been impossible before the surgery. I was relieved that my patient had recovered well without any complications, but the physical therapists were ecstatic - and immediately presented me with a 4-year-old slightly less affected little girl and asked me to perform a rhizotomy on her.

We used the same technique but took less time and sent her back to the ward where she appeared to recover well. However, when her bladder catheter was removed, she was unable to initiate micturition. During the procedure, the nerves coming from her bladder must have been cut, and she lost her ability to control her bladder. I was appalled and refused to do another rhizotomy as I was not going to perform a procedure where there was a significant risk of damaging the nerves controlling the bladder.

The pediatric neurologists and the physiotherapists pleaded with me to try again. It was well known which nerve root supplied which muscle group in the legs and which nerve root supplied the bladder. I tried to think how I could identify the nerve root at each level. Using the limited exposure provided by a T12/L1 laminectomy accurate identification of levels was not possible. As I always did with a surgical procedure that seemed difficult or dangerous, I turned to the underlying anatomy.

I spent many long hours in our state mortuary looking at the anatomy of the nerve roots in the lumbosacral spinal canal. The conditions were awful. The mortuary was cold, damp, and smelly. The bodies were cold, and the instrumentation limited. Blood seeped down into my surgical field, and there was no suction so I used an old sponge. Initially, I felt most despondent. All I could see looked like strands of spaghetti in tomato sauce, but eventually the anatomy became clear. I could now see that if I extended the laminectomy lower, from L1 down to the L5, I could clearly see where each nerve root exited the canal so that each nerve root's level could reliably be seen. The anterior (motor) nerve roots could also be separated from the posterior (sensory) nerve roots, and the motor nerve roots kept out of the operative field (cutting anterior [motor] roots would cause permanent paralysis). I had not been able to do this using Fasano's technique (which I had used in my first two cases) but now could be certain, which nerve root I was stimulating. Most importantly, the second, third, and fourth sacral nerves which are involved in bladder function could be identified and preserved.

This technique worked extremely well and as our confidence grew, we began operating on less severely affected children. Each child was carefully assessed before and after surgery, and the information was gathered and published in the South African Medical Journal. [4] Strict selection criteria for the procedure were established based on our results. Having seen that the procedure did not help children with dystonic or mixed spastic/dystonic cerebral palsy, that group was soon excluded from the procedure. Children having cerebral palsy had to show clear clinical evidence of spasticity and had to display good antigravity strength in their spinal extensor muscles, knee extensors, and hip abductors. There also had to be certainty that the children would have postoperative physiotherapy as it was found that those who did not have frequent physiotherapy after rhizotomy did not improve nearly as well as those who did.

The people who had the most experience with children with cerebral palsy greeted rhizotomy with great enthusiasm. In South Africa, these were the pediatric neurologists and the physiotherapists, and many children having cerebral palsy were referred as candidates for rhizotomy. At that time, I was the only pediatric neurosurgeon in Cape Town being kept busy with general pediatric neurosurgery including hydrocephalus, brain tumors, congenital abnormalities, and surgical procedures for refractory epilepsy. I now included the spastic cerebral palsy cases as well.

In 1985, I was invited to join the faculty at the University of California at Los Angeles (UCLA) and moved to the USA with my family. After performing a few rhizotomies at UCLA, a journalist at the New York Times wrote an article entitled, "Bold New Surgery" which was all about how I had started doing the procedure and how promising the results were. My office was inundated with calls from parents all around the country and we soon had a waiting list for rhizotomy of 6 months and even longer, for a consultation.

All looked well, but disaster was just around the corner.

I had a paper accepted for the Academy of Cerebral Palsy and Developmental Medicine Annual Meeting in Boston in 1987. When I stood at the podium, the auditorium was surprisingly packed beyond capacity with the audience spilling out into the hallway. At the end of my brief presentation of the results of rhizotomy for the treatment of children with spastic cerebral palsy, the queues at the audience microphones ran to the back of the hall. I was attacked by many of the questioners with accusations that my work was unethical: "How dare you as a neurosurgeon operate on children with cerebral palsy?" "What do you as a neurosurgeon know about cerebral palsy?" Eventually, the moderator said the time for questions had expired, and my torment was over.

Although I ultimately received letters of apology from some of the questioners I was, at the time, deeply shaken by the degree of hostility directed toward me. I briefly felt like abandoning my whole involvement in cerebral palsy. However, I asked myself, why should I? The good results I had seen in my children having cerebral palsy were indisputable. More importantly to me, the physiotherapists who were most discerning and critical of how their children having cerebral palsy were treated - continued to refer children having cerebral palsy. I am still not entirely sure what prompted those attacks, but it may have been out of fear that this new approach might replace orthopedic procedures in the treatment of children having cerebral palsy, which of course it did not. Permanent structural abnormalities such as muscle contractures, bony, and joint deformities would still need expert pediatric orthopedic care.

This chastening experience did, however, prompt me to develop a more scientific approach to our patient assessment and result presentation, one that could not be accused of subjectivity. At the meeting, my paper had analyzed the results of a series of children having cerebral palsy undergoing rhizotomy, looking at sitting, crawling, standing, upper limb function, and speech using five grades of assessment for each category before and after rhizotomy. (Many of the orthopedic papers were definitely not as critically analytical). My group at UCLA reviewed with great care our more recent surgical patient group and also used gait analysis. This work was then published in the Journal of Neurosurgery [5] after thorough peer review.

A few years later when the American Academy of Cerebral Palsy and Developmental Medicine Meeting held in San Francisco, I was invited to give an overview of the role of rhizotomy in the treatment of cerebral palsy. I was rather nervous when I once again stood at the podium and faced a very large audience, but thankfully, the mood had changed. I received a warm ovation at the end, and the questions were all positive and polite. At last, I felt vindicated.

After the appearance of that article entitled, "Bold New Surgery" in the New York Times the article was reprinted in many other newspapers and the "leading medical journal" the Reader's Digest spread the word of rhizotomy around the world, so I started seeing children having cerebral palsy from many faraway places. Initially, I was invited to various medical centers within the USA and Canada to give lectures on rhizotomy, examine and select children having cerebral palsy for surgery and demonstrate the technique. Eventually, it became easier for the interested neurosurgeons to come to UCLA where they could attend the clinic and see children having cerebral palsy with me and then watch a number of operations before heading back to start a similar program.

My travels outside North America began because of a visit from India by a grandfather and his fairly severely affected grandson who both lived in Hyderabad. The child was a candidate for surgery but, because the costs in America for the grandfather would have been enormous, I said to him that I would go to Hyderabad and operate on his grandson there and show the neurosurgeons how to select children having cerebral palsy and how to do the procedure itself.

The visit to Hyderabad was a highlight in my life in cerebral palsy.

The hospitality was so warm and welcoming and the hospital where I would work, the Nizam's Institute of Medical Sciences (NIMS), was highly sophisticated with some of the latest neurosurgical tools available. The neurosurgeons were extremely well trained, being up to date with the latest research in the field and were excellent technical surgeons with extensive experience. Dr. Purohit was a talented young consultant at that time and showed great interest in treating children with cerebral palsy. I gave some lectures and the spent hours in the clinic seeing many children with cerebral palsy who had come to Hyderabad from all over India. We chose three children having cerebral palsy for surgery, one of them being the little boy whose grandfather had brought him to see me at UCLA. The postoperative care was excellent, and I was most impressed with the quality of the physiotherapists who handled the rehabilitation program and had such good results with the children who had undergone rhizotomy.

The following year, at Dr. Purohit's invitation, I was back at NIMS seeing the children having cerebral palsy that we had operated on the previous year and was most impressed with the results of the postoperative care and the rehabilitation program that had been set up. Under Dr. Purohit's leadership, the care for children has grown tremendously and has given rise to the "Indian Family of Cerebral Palsy."

After my last visit to Hyderabad, I spent some time in Bombay (Mumbai) and had the humbling experience of meeting Dr. Mullaforoze at her cerebral palsy center near the racetrack. This tall, elegant and quietly spoken lady was a huge inspiration to me. Despite the extreme lack of funds, the center was able to provide excellent care by making use of the most economical methods. I was amazed to see them make their own Plaster of Paris from rolls of gauze dipped in gypsum. This was then soaked in water and used to make casts hold limbs in place after corrective surgery or as splints and orthoses. Despite Dr. Mullaferoze's advanced age (she had been practicing as an orthopedic surgeon before I was born), she was energetic and enthusiastic and a master of her craft.

That day with Dr. Mullaferoze and her staff and children provided me with an important lesson that I have never forgotten-Things can get done even under the most trying circumstances, by making the most of what you have and being innovative.

My rhizotomy travels have taken me to medical centers caring for little children on all five continents where I feel that I have learned more than I was able to teach.

I believe that the negativity associated with the popularization of rhizotomy has spawned a new discipline for assessing the outcomes for the various treatment methods used for cerebral palsy. At the same time, various methods of classifying the type and severity of cerebral palsy found in each child have made treatment more specific. The selection criteria for rhizotomy have become more logical and will hopefully prevent this surgery being applied to children who will not benefit or possibly made worse.

Unfortunately, controversy still exists as to the value of the procedure. I venture that this might be because there are places around the world where vast numbers of children with cerebral palsy undergo rhizotomy based on questionable selection criteria and results. In the centers where thoughtful clinicians apply strict selection criteria and review results carefully, the procedure continues to be performed safely and effectively. I believe in the adage of quality, not quantity.

It is important to note that since I started performing rhizotomy, three alternative therapies have been introduced - botulinum toxin injection, peripheral neurectomy, and implantation of the Baclofen Pump. When used with each one's specific indications, they produce good results.

Working with children with cerebral palsy has taught me many lessons:

When we treat children with cerebral palsy, we treat a whole family and when the child's disability is reduced, we improve the life of all members of the family.

When treating a condition such as cerebral palsy, we should try to aim our treatment as close as we can to the primary pathology and move back from the secondary effects on other systems. As the primary pathology is within the central nervous system, the closer we can get to it the less we will have to do to innocent muscles, bones, and joints. A method for repairing the lesion within the brain is not yet within our repertoire, but a day will soon arrive when it will be.

The introduction of a new treatment method should come under close and scrutiny, but the criticism should be unprejudiced, kind, fair, and constructive.

Let us remain innovative and persistent in our attempt to treat cerebral palsy with the guidance of modern science.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Sherrington CS. Decerebrate rigidity, and reflex coordination of movements. J Physiol 1898;22:319-32.  Back to cited text no. 1
[PUBMED]    
2.
Foerster O. On the indications and results of the excision of posterior spinal nerve roots in men. Surg Gynecol Obstet 1913;16:463-74.  Back to cited text no. 2
    
3.
Fasano VA, Broggi G, Barolat-Romana G, Sguazzi A. Surgical treatment of spasticity in cerebral palsy. Childs Brain 1978;4:289-305.  Back to cited text no. 3
[PUBMED]    
4.
Peacock WJ, Arens LJ. Selective posterior rhizotomy for the relief of spasticity in cerebral palsy. S Afr Med J 1982;62:119-24.  Back to cited text no. 4
[PUBMED]    
5.
Peacock WJ, Staudt LA. Functional outcomes following selective posterior rhizotomy in children with cerebral palsy. J Neurosurg 1991;74:380-5.  Back to cited text no. 5
    




 

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