How a father copes with a rare disease
By Vimal Sumbly
Tribune News Service

LUDHIANA, Sept 28 — It was a sheer tragedy of fate for Pawan Kumar Gadi and his wife to learn that their two-and-half-year-old son Pranav, nicknamed Vicky, was suffering from primary immunodeficiency (PID).

Although there is no cure for the disease as yet, the treatment can certainly help the patient to live an almost normal life. But the treatment costs about Rs 6,000 every third week and gets on increasing with the weight of the person. And Pawan Kumar’s monthly salary is Rs 5,000.

This disease is quite rare and is said to prevail among one patient in every one lakh persons. It was quite after a long time that the doctors could diagnose Vicky’s diseases. Dr Surjit Singh, an Associate Professor in Paediatrics, Allergy and Immunology at the PGI Chandigarh was able to diagnose that Vicky was suffering from PID.

The disease is an inherited disorder, in which a part of the body’s immune system is missing or it does not function properly. Due to this condition such children become highly susceptible to all kinds of infections, usually within first few months of life. Many patients with PID disorders can be offered therapy which enables them to lead a normal life, if they continue with it as prescribed. The therapy is quite expensive and even not readily available in India.

Agammaglobulinemia is the most common of the PIDs and prevalent in males affecting one in one lakh. After maternal passive immunity is lapsed, which generally lasts for six months, the infants are susceptible to repeated bacterial infection. They suffer from gastrointestinal infections which limits the food absorption and assimilation. Even with the high doses of antibiotics, these patients do not get rid of infections. The only treatment in such cases is to infuse intravenous immunolglobulins (IVIG) along with antibiotics. Regular treatment with the infusion of IVIG to such patients, once in every 21 days, along with other appropriate treatment can give them a normal life on long-term basis.

Where there is a will there is a way. And Pawan Kumar found it. Initially a New Zealand-based International Patient Organisation for Primary Immuno-Deficiencies (IPOPI) came forward with help. It donated 10 IVIG vials for Vicky’s treatment. This was followed by a personal gift sent to him by the Red Cross Society of Finland, which sent him 1,000 IVIG vials worth Rs 12 lakhs. He retained 120 vials only for Vicky’s treatment and donated rest to the PGI, Chandigarh..

Pawan Kumar formed the National Patients Organisation for Primary Immunodeficiency (NPOPI) with the guidance of the IPOPI. The IPOPI also invited him to a conference in Greece in 1998 where he learned how parents of the patients coped up with the disease emotionally and financially.

So far Pawan Kumar has identified seven patients in India. They interact regularly. On October 1, this year the NPOPI is organising a conference of doctors who will deliberate on the disease and also help in creating awareness about it. The problem with all seven patients is that their families are not financially sound enough to meet the expenses.

The basic requirement of the dose is 400mg per kg body weight. Vicky’s weight is 19 kg and his cost per infusion comes to about Rs 5700. As the body weight increases the dosage will need to be increased, with an obvious increase in the expenditure. Pawan Kumar is left with one more dose only. However, he does not allow his worries to feel him let down. He is hopeful that he may manage further dose, as he has been doing for the past four and half years.

As an advertisement says, "Whenever you see colour, think of us." So also chorus all restaurants, bakeries, sweet shops, ice-cream parlours and even caterers. If there is any thing that sustains us apart from the air we breathe, it is the food. We are forever enticed by colourful gastronomic delights jalebis, ice-creams, ketchups, cakes and so on. Do we ever stop to ponder that some of these bright alluring hues are highly toxic? Perhaps a majority of us don’t even know that under the Prevention of Food Adulteration Act, no cooked food is permitted to have any colouring agent.

Broadly, colours are divided into two categories – natural and synthetic. Natural colours are permitted in any food, in any proportion. While the known natural colours are saffron, chlorophyll, caramel and turmeric, synthetic colours are represented by the four basic shades i.e. red, yellow blue and green. The Act states that synthetic colours are permitted in only certain food items within the limit specified for the particular item. For instance, in beverages, the maximum unit is 100 parts (gm) per million. In case of ice-creams, biscuits, cakes, pastries, jams and fruit syrups the limit is 200 gm per million. The use of colouring agent in cooked food is totally prohibited. It is mandatory for synthetic colours to conform to the ISI specifications.

It is appalling to see that general public is mostly unaware of the rules and regulations, which impose restrictions on the indiscriminate use of colours in the food items. Most of us equate the quality of the sauce with its redness. With the ignorance level being rather high, the most important question that arises is, "How safe are food colours?"

Leading chemical technologists are of the opinion that food additives are consumed in varying amounts and strictly speaking, enough of anything can be toxic. (Toxicity is the adverse effect of chemical and physical agents on living systems). A look at the various brands of food colours available in the market, showed that the common ingredient used by a majority of food parlours were lead chromate and metanil yellow. Other ingredients include propylene glycol and a certain other type of dye contents.

Propylene glycol is generally used to clean the colon before surgery. It acts as a laxative. Consumed via a coloured food item, it could act in a similar manner. Non-approved dyes pose more serious threat to health. Metanil yellow, the colour of choice of food adulterer, has been shown to cause testicular degeneration and also have carcinogenic effect in isolated cases. World over doctors see a large number of patients complaining of skin rashes, urticaria and respiratory allergies after eating food items which have been coloured. Animal toxicity experiments have revealed long term effects such as pathological lesions in vital organs like spleen, liver and kidney of the test animals with certain other commonly used dyes in popular food products.