Cerebral palsy

A child’s brain development starts from the time his/her embryo is conceived, and goes on until a few years after birth of the baby. Any insult to the baby’s brain during this phase can cause irreversible deleterious effect on it and can cause damages which persist lifelong. The ‘insult’ on the developing brain could be an unfortunate event which happens before birth, during birth or after birth of the baby. As brain controls many other organs in the body, damage in the brain can result in problems with normal functioning of these organs.

This can be better explained with a classical example: When the baby is within mother’s uterus, the oxygen supply to baby’s organs comes from the mother. But, after the baby is born he/she has to breathe to maintain uninterrupted supply of oxygen to the body. Any delay or problem affecting this activity or supply chain can result in the baby being without oxygen for some time- this situation is called ‘Birth asphyxia’. The cause for birth asphyxia may be a delay in the first cry (which helps the baby’s lungs to expand and start working) or poorly developed lungs (as in a premature baby). As brain is the organ which gets affected first by shortage of oxygen, it soon sustains significant damages if birth asphyxia persists- the severity of which depends on the duration of asphyxia, maturity of the baby etc. Many times, those areas of brain which supply muscles of legs and arms are affected and this leads to weakness of these limbs and abnormal functioning of them. The damage occurring in the brain at a young age does not worsen further, but the resultant abnormalities within the limbs can become more and more severe with growth.

What is Cerebral palsy?

With the above background knowledge it is easy for you to understand the concept of CP: ‘Cerebral’ means related to brain, ‘Palsy’ means weakness. Cerebral palsy is a disorder of movement and posture resulting from a damage occurring in the developing brain.

Causes

Factors before birth:
* Infections affecting the mother
* Defects in the development of brain (absent areas of brain)
* Block of blood supply to an area of brain

Factors related to the birth process
* Prematurity (baby born before mature age/state)
* Low birth weight

Birth asphyxia

Factors occurring after birth:
* Infections
* Jaundice in new born period

Variability

The manifestations and severity of cerebral palsy differ considerably from patient to patient. The spectrum of CP extends from an almost normal child with very subtle limp to a totally bedridden child in vegetative state. So just when your doctor inform you that your baby may be having CP, do not panic because it may be a mild disease which gets better with regular treatment.

Doctors use different systems to classify CP. Depending on the type of movement disorder there are different names- if the muscle tone is increased (means muscles are ‘tight’) it is called spastic CP, if the tone is decreased it is called hypotonic type etc. Similarly depending on area of body involved, there are hemiplegic CP (either right or left half of body involved), diplegic CP (both legs involved), quadriplegic CP (all four limbs affected) etc.

Clinical features

Reasons for which parents bring their children with CP to doctors:

* Delayed milestones- There are some activities which every child should have done at a particular age (like head control- holding the head up independently- by 3 months, walking at least by one and half years etc). These are called ‘mile stones’. One important feature of CP is delay in achieving milestones.

* Tightness of limbs- Doctors call it ‘increased muscle tone’. Observant parents may note that it is difficult to stretch arms or legs of their baby.

* Abnormal walking- A child with CP usually starts walking at a delayed age compared to normal children. Once he/she starts walking there can be variations from normal pattern of walking like- tip toe walking, tip toeing with knees bent, flat feet with knees bent or many other vague patterns.

* Early achievement of handedness- usually we realize a child is a right hander or left hander by the age of two and half years. If he/she uses only one hand from an earlier age, it is most probably due to a problem with the other limb!

* Deformities- these usually develop at a later age if the child does not receive adequate or proper treatment.

* If your baby had any problem (like birth asphyxia, prematurity, low birth weight etc) during the delivery or new born period doctors would have warned you about the possibility of issues in future and advised you to watch for any warning signs like delayed milestones.

Doctor examination

* Once you seek medical help for a child with CP, doctors check for muscle tone, walking and various other clinical examinations.

* Various medical problems may be associated with CP which have to be found out and concerned specialists should be involved in the treatment. Eg- Seizures (‘fits’), problems with speech, learning disorders, dental problems, squint, bowel disorders, nutritional deficiency etc.

Investigations

* To make a clear diagnosis of CP doctors need to find the damage happened in the brain- for this an MRI scan of brain is the investigation of choice.

* An Orthopedic doctor may do x ray of hip joints because dislocation (‘slipping’) of hip joints can happen in a child with CP.

* Gait analysis: This can be done by recording video at the clinic or in sophisticated gait labs- this helps to find problems with walking of the child and to plan treatment accordingly.

* The child may have associated problems like seizures which needs further evaluation

Treatment

In India, the most common problems experienced by parents of CP children are lack of knowledge about the nature of the disease, unrealistic expectations about recovery of it and existence of experimental/ unscientific treatment methods. The confusion arising out of all these issues pushes these unfortunate parents into unsolved dilemma about which treatment method to follow! Naturally, they roam around different hospitals of different systems and often land up in the hands of a quack who claims he can cure it completely. By the time the poor parents realise that this person has looted all their hard earned money, lot of precious time also would have been wasted.

* The key problem in CP (The damage happened in the brain) is irreversible. So, anyone who claims that he can cure CP completely is wrong!

* But this does not mean that there is no treatment for CP. In Modern Medicine there are lots of effective treatment methods available, the aim of which is not to cure the disease completely but to improve the present condition of the patient.

* Doctors are not Gods but humans! Unfortunately if your child had suffered a severe damage to his/her brain at a tender age, the resulting CP will also be severe- the child may be bedridden or in vegetative state. In such a situation, our aim should not be to make the child walk or run- a more realistic expectation should be to make him/her sit up or train for wheel chair mobility.

* Out of exhaustion or desperation parents may stop the treatment in between or approach persons claiming to give ‘one shot treatment’ for the disease. But they should understand that the improvement in CP happens at a very slow pace and it needs persistent effort and tremendous determination on part of parents to get a reasonable result of treatment.

* Just as getting good improvement of CP needs determined effort of parents, maintaining the good result also needs persistent care.

* Treatment in CP aims not only to help with the problems or disabilities of the child alone, it should also consider present or future issues which parents face. For example a mother who carries her son with CP to the toilet now may not be able to do so once he grows up and she becomes old. So, it is better that he achieves independent mobility within the house (either by walking or in a wheel chair)

Treatment methods:

* Physiotherapy: Proper physiotherapy done by a therapist of the right attitude and good knowledge is the corner stone of CP treatment. It should be regular, uninterrupted and should continue till maturity (sometimes after that also).

* Occupational therapy: Children with more severe CP will need training to perform day to day activities like using hands for eating, writing etc.

* Supportive devices: These are called ‘orthotics’. It may be a splint which keeps the foot/hand in proper position, a walker frame on which the child supports while walking or a wheelchair.

* Medicines: (a) Medicines which reduce muscle stiffness (tone). (b) Medicines for seizures (c) Nutritional supplements

* Botulinum toxin (Botox) injection: The procedure is usually done in operation theatre, with or without anaesthesia. The drug is injected into the muscles to reduce their tightness. Botox is quite an expensive medicine. Parents should not misunderstand this as a one shot treatment or an alternative to physiotherapy- but since it reduces muscle tightness it facilitates physiotherapy. The effect of the drug lasts only for about 6 months within which regular physiotherapy reestablishes normal muscle tone and maintains it.

* Plaster casts: Plaster of Paris casts called ‘tone inhibiting casts’ are used to gradually correct deformities like toe walking.

* Intrathecal baclofen pump: Baclofen is a drug which decreases muscle tone. Usually it is given as tablets. But it can be given directly around the spinal cord to reduce muscle stiffness. Side effects of long term intake of baclofen tablets can be avoided with this. But it needs invasive procedure to implant the pump which delivers the drug.

* Surgery: Surgery also should not be considered as a miracle treatment which can cure every problem with CP children. It should be undertaken in a very selective manner. Unnecessary or poorly timed/performed surgery can worsen the condition of the patient. Well accepted indications for surgical treatment in CP are:
Severe stiffness of muscles (‘Contractures’) which cannot be relieved by physiotherapy or botox injections.
Hip joint which is about to slip (‘dislocate’) out of its socket.
Hip joint which has already slipped out and is severely painful.

There are many other situations in CP where doctors decide to do surgeries based on problems in individual patients. Presently, the most accepted trend worldwide is to do all necessary surgical procedures at a single time avoiding multiple visits of the patient to operation theatre (Single event multi level surgery- SEMLS)

Associated issues like eye problems, in a CP child need to be attended and treated by concerned specialists.

Multidisciplinary approach

An unfortunate trend seen in our country in the field of treatment of CP is lack of discussion and cooperation between different experts involved in its management. Sometimes this situation becomes worse to such an extent that one expert may advise against some mode of treatment without knowing much about it. Manifestations of CP involve various areas of the body. So its treatment needs the involvement of different specialists like Neurologist, Physiatrist, Orthopedic Surgeon, Pediatrician, Physiotherapist, Occupational therapist, Pediatric Surgeon, Ophthalmologist, and Dental Surgeon etc. Each specialist may be good in his field, but if he/she stays as an independent island and does not interact with other experts it is not ultimately good for the patient. Treatment of this disease needs combined evaluation, healthy discussions to assess various problems of the child and form a complete treatment plan for the best interest of the patient- called ‘Multidisciplinary approach’.

Importance of physiotherapy

Physiotherapists hold key role in the treatment of CP. This condition needs proper physiotherapy by a therapist of the right attitude and good knowledge, given on a regular uninterrupted way almost till the child is grown up. Fed up with the difficulty in following regular physiotherapy schedule many parents resort to treatment methods which appear to be shorter- but one thing they should understand is that no treatment (may be surgery, Botox etc) is an alternative to physiotherapy and therapy should continue as part of life for a child with CP till maturity and sometimes even beyond.

The goal of surgery is to correct the position of the bones within the foot. This will likely require lengthening tendons or ligaments to allow the bones to be moved. The bones are then held in place with pins and a cast. The pins can usually be removed in the office in 4 to 6 weeks. A special shoe or brace may be recommended to try to prevent recurrence of the deformity. Your doctor will likely recommend follow up visits for a few years to see make sure the foot grows well and doesn’t need additional treatment.

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