What is an autoimmune disease?

The human body's own defence system is called the Immune system. It comprises many specialised tissues and cells such as blood, the bone marrow, the thymus (a gland located in the neck), the lymph nodes, the lymphatic tissue in large organs of the body such as the liver, the spleen and the gut. In the blood, it is the white blood cells or leukocytes, esp. the lymphocytes, that regulate the immune system. Control of immunity is also through the formation and secretion of many important substances such as the "lymphokines".

A key element of our immune system is its ability to distinguish between the human body’s own cells (referred to as ‘self’) and foreign cells e.g. bacteria and viruses (‘non-self’).

Each cell carries protein markers called antigens that allow it to be identified as ‘self’ or ‘non-self’ by the immune system. Antibodies (also known as immunoglobulins) are important blood proteins in the immune system that are produced by a type of white blood cell called B cells. Antibodies naturally develop during exposure to infection and by binding to foreign antigens, help neutralise and eliminate the organisms that caused the infection. 

Antibodies are especially helpful when a particular type of bacteria or virus is encountered for a second time. In this situation antibodies already present in the blood against the particular bacteria or virus, prevent infection from developing. Antibodies, though, are just one aspect of immunity. 

Our body also has another kind of white blood cells called the T cells. These are responsible for cell-mediated immunity. T-cell surfaces also have what is called histocompatibility antigens, which help us to accept or reject transplants of organs done to us.

Autoimmunity is a failure of the immune system to always correctly recognise ‘self’. As a result, the body’s own immune system attacks part of the human body. This leads to the formation of antibodies directed against one's own tissues - the autoantibodies. Autoantibodies may directly cause harm, by causing the body to malfunction.

There are many conditions with an underlying basis of autoimmunity. They include lupus, thyroid problems and many more. 

(Ref: Mark McClure's article in www.vasculitis.org.uk)

What is a "Lumbar Puncture"?

This post pertains to a particularly important procedure that doctors need to perform on children (and adults) with certain specific kinds of illnesses. 

The procedure is known as a LUMBAR PUNCTURE. In this procedure, we insert a needle under sterile, aseptic conditions into the spine of a child to extract a few millilitres of CEREBROSPINAL FLUID - the watery fluid that bathes our brain and spinal cord - to analyse it to diagnose neurological conditions and nervous system infections. 

In children, we do not remove the CSF with a syringe; rather, we collect as it drops into a bottle

The most common diagnosis we are trying to corroborate is MENINGITIS. This means infection or inflammation of the meninges - the covering of the brain and the spinal cord. Children with suspected meningitis present with a short illness of high fever, inability to stare or to look at bright light, a stiffness of the neck and, in cases where the doctor is not consulted acutely, other significant neurological symptoms like headache, pain in the limbs, inability to flex one's legs on to the abdomen, and, in severe cases, loss of consciousness, or even fits.

A non-blanching rash may also be a harbinger of a particularly sinister kind of bug - the meningococcus. See my OTHER post on this kind of rash. 

While the exact procedure for a lumbar puncture varies from country to country or system to system the general steps are the same. Consent from a parent/parents is required. This may be verbal or written, depending on where this is being done. For example, in India, we take written consent, while in the U.K., it is all right to take verbal consent. The consent must include the fact that not only is the procedure explained, but also its side-effects. 

The child is laid on a flat-topped bed or procedure bed. He is turned on one side. The doctor aims to puncture in the centre of the back where the spine is located; he must be adequately immobilised by helper members of the medical crew. Usually, parents are advised NOT to stay beside the child but to wait outside the procedure room. A support worker may be allocated to stay with the parents for support. 

After cleaning the back, a needle is passed into the space between two of the back vertebrae till it is inside the cerebrospinal fluid. As the CSF drops, it is collected into bottles for examination and for organism culture studies. The needle is then withdrawn and disposed of safely. The puncture site is sealed with "OpSite" a kind of plastic dressing spray. This is then covered over with a small, circular bandage and the child is handed over to the anxious parents. 

The common side-effect of a lumbar puncture is a minor headache. Usually, there are no other harmful effects. Theoretically, there is a risk of introducing bugs through the puncture into the nervous system. This is very rare, however. 

Results of the tests are usually available within the hour. Based on the initial results, doctors will advise parents on which way the treatment is likely to go ahead. Usually, if an infection was suspected, doctors will have ALREADY started antibiotics or antiviral drugs, or both, even before a lumbar puncture is carried out. Usually, such antibiotic or antiviral therapy is then continued till the culture results from the fluid are made available - which could be up to 3-5 days later.

To summarise:

1. A lumbar puncture is putting a needle into the centre of the back to collect cerebrospinal fluid
2. Usually, this procedure is done to prove/disprove the presence of infection of the nervous system organs or tissues, usually, the meninges.
3. The procedure needs verbal consent in most countries. 
4. It is carried out with the strictest antisepsis. 
5. The procedure is generally safe and tolerated well by almost all children,
6. Theoretically, there is a rare risk of introducing infection. 
7. Results of the test will guide further therapy. 

Thank you.

Rights of a Patient - Right to be informed of harm occurring to them because of the medical system

I am writing this post to make patients all over the world aware that there is a significant risk of harm occurring to themselves or to their near and dear ones who are receiving medical care in a hospital. This is because human error is always possible - even in the best hands. Apart from human errors, there can be machine-led errors, systemic errors and other forms of misfortune that can befall users of medical systems.

As per prevailing ethics, patients or their wards have the right to be informed about any and every form of such errors that cause them any kind of misfortune. This is called the duty of candour or honesty all over the developed world. In the U.K., where I work, this duty of candour has been in place since the past few years. 

Doctors are expected to contact you in person, be empathetic and inform you in detail about what happened, why it happened and what is being done to mitigate the effects of this mistake. They are also expected to report the event on their medical system through what is known as the "Incident Report". They will have to inform their line managers as well. A detailed conversation must include the assurance that the incident will be investigated thoroughly, and responsibility will be fixed. The patient/caretaker must also be told that this investigation would guide the hospital to take steps that would prevent a repetition of the same error in the future.

So, there you have it. You have a right to know if you/the patient you are responsible for has been harmed in any way through a preventable error made by a faulty system.

Invited Post on Cleft Lip Surgery options in India

Cleft - A Smile Is What We Need

Cleft is an orofacial condition where the baby is born with a gap in the lip (cleft lip) or roof of the mouth (cleft palate) or both. It is a birth defect during pregnancy when different areas of the face that developed individually join together, but fail to join properly near the mouth and the lips leading to a split or a cleft. If the separation occurs near the lips then it is known as cleft lip, and if it is near the mouth then cleft palate.
It may or may not be detected during pregnancy by an ultrasound test. One in every 700 children is found to have this birth defect.
A definite reason behind it hasn't been detected yet, but several cases related to clefts are thought to be a result of genetic and environmental combinations. Vitamin deficiencies like lack of folic acid during pregnancy along with consumption of alcohol, anti-seizure medicines and steroid tablets, and smoking are the most common cases why this occurs.
If not treated on time, Cleft can have multiple adverse effects on the child. Apart from feeding problems, the child can become susceptible to infections related to ear, nose, teeth and speech, along with psychological and social problems at a later stage. Special advice from feeding or speech therapist must be sought while breastfeeding the baby affected with the cleft lip as it may find it difficult to form a grip around the breast, and it may choke due to cleft palate if not positioned carefully.
Luckily there are affordable surgery packages in India for treating cleft lip and cleft palate deformities in India. Plastic surgeons can close the cleft by connecting the muscles of the soft palate and rearranging the tissues to close the palate. The surgery takes 1 - 2 hours to complete and requires 4 - 5 days of stay in the hospital for recovery.
Surgery can be conducted once the child is two to three months old. It is important to monitor the child's growth to ensure that it is ready for the cleft lip surgery and cleft palate surgery.

• Care must be taken with the child's diet to ensure that it is healthy and gains weight before the surgery.
• Child's blood type and count must be checked.
• A thorough physical examination of your child must be done along with complete medical history examination.
• Share all the details regarding the medicines consumed by your child with the doctor.
• Get complete clarity regarding the medicines that can be consumed on the day of, and post-surgery.

A pink light scar can be visible after cleft lip surgery. Soreness and irritability are commonly experienced after cleft palate surgery. Chances of infection while in the hospital can be minimized by prescribing antibiotics. Clear instructions for general care and feeding must be sought for from the doctor right after the surgery. The stitches need not be cut open surgically as it usually dissolves during the recovery period.
The cost difference between cleft lip surgery and cleft palate surgery in India and in the West is huge, where India charges only one-tenth of what is charged across shores.

There are numerous success stories behind these surgeries. Each child is entitled to an uninhibited smile so let's make that happen by taking action!