Wed | Oct 18, 2017

Uncovering sickle cell

Published:Tuesday | September 30, 2014 | 12:00 AM

SICKLE CELL disease (SCD) is an inherited disorder in which haemoglobin, the pigment that makes your blood red and transports oxygen, is abnormal. People with this condition have an atypical haemoglobin molecule called haemoglobins.

SCD affects millions of people worldwide and is most common among people whose recent ancestors originated from Africa itself, the African diaspora in the Caribbean, the US and Europe, as well as populations in the Mediterranean region, India, and South and Central America. About 10 per cent of Jamaicans have the gene that generates haemoglobins while in the United States of America, the condition is estimated to occur in one in 500 African Americans and up to one in 1,000 Hispanic Americans.

Healthy red blood cells, with normal haemoglobin, are disk-shaped and flexible, able to squeeze through the smallest blood vessels in the body. On the other hand, the red blood cells in SCD, because of their abnormal sickle or crescent shape, can block small blood vessels and obstruct the flow of blood. This reduces the supply of oxygen to the tissues, and may damage many organs and cause severe debilitating pain in what doctors call 'painful crises'. The bones, chest and abdomen are common sites for these painful episodes.

While normal red blood cells live for about 120 days, the abnormal sickle cells die after only 10 to 20 days, resulting in chronic anaemia. Because of anaemia, sicklers may experience fatigue, pallor, a rapid heart beat, and shortness of breath. A yellowing of the eyes and skin, known as jaundice, often prevails as a result of this abnormal breakdown of the red blood cells. Other common problems include lung and breathing difficulties, chronic leg ulcers, and an increased susceptibility to infections.

SCD includes sickle cell anaemia (SCA) and its many variants like the sickle-cell trait. These genetic disorders, referred to as haemoglobinopathies, cannot be 'cured' in the conventional sense. Recent developments in gene therapy show some promise of a breakthrough in the treatment of this condition. In the meanwhile, modern medical research clearly demonstrates that adopting healthy lifestyle practices can significantly improve some of the challenges of this disorder. This can mean a decrease in the severity of anaemia, less pain and infections, plus improvements in the healing of leg ulcers and better general health and well-being of those with this illness.

A new science called epigenetics has demonstrated that we are not helpless victims of our genes. Even if you are born with a particular genetic disorder, those genes may or may not express themselves, depending on many factors like nutrition, stress or even our thoughts and beliefs. In short, epigenetics explains that our environment, physical and mental, can influence the way our genes behave, even the sickle cell gene. For those interested in a deeper look at epigenetics, I strongly suggest reading The Biology of Belief by Dr Bruce Lipton.

The vitamin D connection

Several medical studies report that between 65 and 100 per cent of SCD patients are vitamin D deficient. There is a strong relationship between the severity of pain, particularly bone pain, and vitamin D status among sicklers. Here is a quote from recent medical research: Recent studies suggest that patients with SCD have profound vitamin D deficiency. - Journal National Medical Association, April 2010.

The lower the vitamin D level in a sickler, the more pain and problems they tend to experience. The pain seen in SCD with vitamin D deficiency is mostly in the lower spine, pelvis and extremities, and is described as a dull, aching pain made worse by weight bearing and movement. Despite the research reporting a high prevalence of vitamin D deficiency among patients with sickle cell disease, this problem remains both under-recognised and under-treated by their doctors.

I strongly recommend that individuals with SCD supplement with high doses of vitamin D (at least 10,000 IU daily), in addition to daily sunbaths, to optimise their vitamin D status. It would also be useful to have your doctor check your vitamin D blood levels. As SCA affects people with pigmented skin, it is important to remember that, the darker your skin, the more sun exposure you need to make vitamin D. While sunbathing, do some deep breathing as optimal oxygenation is very important to the sickler.

Diet and supplements

Although individuals with SCD are often led to believe that they can eat anything, I have found that a healthy diet emphasising lots of fresh fruit and vegetables, along with enough healthy protein, is most valuable for sicklers. I also suggest taking supplements, and I use a programme called Cellular Nutrition to provide further special benefits. Optimal hydration is essential: drink one half ounce of water per pound of your body weight daily and eat lots of water rich foods.

Additional useful supplements include the omega 3 fatty acids, the antioxidants vitamin C and E, the minerals zinc, copper and magnesium, the herbs green tea, garlic, ginkgo biloba and pycnogenol, and the amino acid L-arginine.

You may email Dr Vendryes at tonyvendryes@gmail.com or listen to An Ounce of Prevention on POWER106FM on Fridays at 8:15 p.m. His new book An Ounce of Prevention - Mainly for Men is available locally and on the Internet.