Sickle cell disease: silent suffering

Stabroek News
June 24, 2001

"When you talk about the disease from a patient's perspective it's nothing but pain, pain and more pain. There is nothing else I can talk about as a child and a young adult growing up." - Sickle cell disease sufferer.

When Stabroek News first called Eton London, who suffers from sickle cell disease, and explained the reason for contacting him, he calmly replied, "I'm in crisis right now, perhaps this could wait until tomorrow."

A meeting was subsequently arranged, and he then elaborated on how this most debilitating of anaemic conditions had affected his life.

"Last week when you called, I was having my worst crisis since 1988," he said. "The nurse was called in and what she was seeing was a person in such a state of stress that she considered it to be life-threatening.

"I can remember my brother when he was sick," he continued. "I would look at him and think he is going to die. He was a person of great fortitude with a high pain threshold and when he recovered he would just laugh it off, and I guess I do the same."

Sickle cell anaemia is a group of inherited (genetic) red blood cell disorders which affects many nationalities including Afro-Caribbeans, Afro-Americans, Arabs, Greeks, Indo-Caribbeans, Indians, Italians, Latin and Native Americans.

Normal blood cells are shaped like doughnuts and move easily through the body's circulatory system to deliver oxygen. However, 'sickle' red blood cells become hard, sticky and shaped like sickles used to cut wheat. Sickle blood cells do not flow easily through the circulatory system, in fact they clog the flow and break apart.

Crises occur when heavy clogging takes place, causing pain and often tissue damage which increase the risk of infections.

The disease is a lifelong one, incurable without perfect-match bone marrow transplant, and treated purely with lifestyle changes such as drinking plenty of fluids and taking supplements. Child sufferers are required to take daily supplements of penicillin and all sufferers are advised to avoid extremes of temperature.

Crises are usually treated in hospital with the administering of analgesics to numb the pain.

For the average sickle cell disease sufferer, according to Eton, "the disease defines who you are."

"I will not swim, I can't waste energy on those kinds of recreation, I don't think about running, cycling, exertion, drinking - things that others may enjoy in life."

For the sickler, excessive exercise, swimming (due to cold water), dehydration, alcohol and stress are all potential triggers of a life-threatening crisis.

"You have to learn to manage the condition to the best of your ability to prevent sickness," Eton explained. "I remember as a youngster I would go out with the guys and have a few beers, and I realised there was a coincidence. If I went out drinking one night, I would be sick for the next day and the whole of the next week."

In the end it was his friends that acted. "We would go out and I'd say six beers to the waiter. One night, my friends turned round and ordered five beers and a Malta. For nine years that's the way it has been."

For thousands of sicklers across the globe, these challenges are just the beginning of a struggle that is exacerbated by public and medical ignorance, a life expectancy of 38, the prospect of leg ulcers, late puberty, priapism (painful, prolonged erection), bone damage and a high risk of stroke.

Nine-year-old Guyanese, Tanesha Lindie, suffered a stroke at the tender age of six. As a sickler, she must deal with the stresses of growing up in a largely ignorant world and learning to manage a condition that has left her left side close to paralysis.

"She can move her foot and hand but can't grip," her mother, Roxanne, explained. "Other than that, she copes very well. At least, at her age she couldn't care less. It is not worrying her right now, but she would occasionally ask the question 'when am I going to work good again?'

"She don't sit down and mope over it, we don't have that problem with her.

"She is very normal and very active. She does everything, except she walks with a little limp. When she is cold we put on extra clothes and she is fine."

Careful parental guidance has helped to save Tanesha from experiencing the excrutiating pain that many sufferers seem doomed to face.

"I don't expose her to cold places and don't send her swimming because I have this knowledge. She does nothing excessive."

As for school, "the other children have been very co-operative. She does not have much of a problem in school.

"They would assist her opening her lunch kit and the teacher would tell them to always help Tanesha,"

According to her mother, before her stroke Tanesha would experience only mild crises with pains in her stomach. Since the stroke, she has suffered no crises.

"Tanesha is very jolly," she said. "When she gets older we will have to tell her more about it in terms of having a partner. She has to take someboidy who is absolutely negative."

Education in coping with and restricting the occurance of the disease is vital. The disease is caused by genetic mutation, if two individuals with this mutation decide to have a child, there is a risk that their child will be a sickler.

Tanesha, as a sickler, will have to choose a partner who has no sign of the mutation.

"We could significantly reduce the occurance of sickle cell through education," London remarked. "It is frustrating that most people either don't want to know or don't care sufficiently. Most people in the Third World are unaware they have sickle cell trait [the genetic mutation] until they have a child with the disease."

Reproductive education is not the only problem, however. Across the world, sicklers have to tolerate ignorance even when they arrive in the emergency ward to receive treatment.

London described an occasion when his brother was taken to hopsital in Georgetown during a bad crisis. He was given a placebo by doctors who suspected that the patient was just after a fix of the analgesic (which is believed to produce a rush). He returned home and was forced to see the crisis out without aid.

Similar problems have arisen with the belief that sickle cell disease only affects those of African descent. At a Sickle Cell Association seminar at Georgetown City Hall earlier this year, an Indo-Guyanese man retold tale after tale of woe in trying to peesuade many in the Guyanese medical profession to take his wife, who has severe crises, seriously.

"There is a lack of awareness among health professionals across the world," explained Dr Swann, Co-ordinator of the Guyana Sickle Cell Association. "I would like to see a greater commitment among doctors to learn and understand more about the condition.

"At present patients have to convince doctors they are ill which wastes time and resources," he continued. "Patients do have some legitimate concerns."

The Guyana Sickle Cell Association has been active for the past four years, recently receiving funding from CIDA for valuable office equipment and having just completed an awareness week across the country.

Based presently at the Jesus Rescue Mission Children's Hospital, Georgetown, the group faces a constant uphill struggle to sensitize the populace and assist sufferers.

"There is a great volume of things to be done," Dr Swann remarked. "We need to define the number of sufferers here in Guyana, we need to build an effective patient support group, similar to the one in Jamaica, and there is a definite need for more co-operation among all health workers."

In the meantime, many sicklers will continue to suffer in silence and go about their business trying not to let the disease interfere with their professional and private lives.

Guyana too will continue to lose valuable individuals such as Eton London's brother who died after complications, brought on by sickling, following a road accident. He died shortly before completing his medical studies.