What is Kidney Transplant for Fabry Disease and its Cost?

Managing Fabry Disease: Understanding Kidney Transplant and Its Cost Across Indian Cities

Fabry disease is a type of genetic disorder characterised by the accumulation of a fatty substance called Globotriaosylceramide (GL3). An accumulation of GL3 in the kidneys leads to irreversible damage to the organs and impairs their function.

Chronic Kidney Disease (CKD) is a common complication of Fabry disease, resulting from progressive damage caused by GL3 accumulation. In this situation, a kidney transplant can improve survival and quality of life in Fabry patients with end-stage renal disease, but it does not treat the underlying enzyme deficiency. Enzyme replacement therapy remains essential post-transplant.

How is Kidney Transplant Beneficial for Fabry Disease?

In Fabry disease, an accumulation of GL3 in the kidney cells causes damage and, as a result, manifests in symptoms like the presence of blood in urine, swelling of lower limbs, and changes in urine patterns. The first symptoms of kidney damage can arise in childhood and may appear mild at first.

However, most patients see a progression in kidney failure between 30 and 50 years of age. A kidney transplant is useful in this situation.

What are the Costs of a Kidney Transplant in India?

The estimated cost of kidney transplantation in India typically ranges from ₹5.5 lakhs to ₹14 lakhs, depending on the hospital, city, and patient condition. It includes costs of pre-transplant evaluation, surgery and other related fees. However, other factors can also contribute to the overall costs of kidney transplant procedures in India. Some of them are:

• Location of the medical facility
• Expertise of the surgeon
• Total duration of hospital stay
• Severity of condition

Costs of Kidney Transplant for Fabry Disease in Different Cities of India

Here is an overview of some of the estimated costs of kidney transplant treatment in some cities of India:

Who Has a Higher Chance of Getting Fabry Disease?

People affected by Fabry disease inherit a mutated gene from a biological parent on their X chromosome. Men inherit X chromosomes from their mother, and women who have two X chromosomes inherit one from each parent.

A parent can pass the faulty gene which causes Fabry disease to a child in the following ways:

• Men with Fabry disease pass their affected X chromosome to all daughters, making them carriers or affected. Sons inherit the Y chromosome and do not receive the faulty gene from their father, though they can inherit it from their mother.
• Female parents who have Fabry disease have a 50% chance of passing on the affected X chromosome to each of their children, whether it be a son or a daughter. Hence, some children in the family get the gene change, while others do not.

Other Treatment Options for Fabry Disease Beyond Kidney Transplant

Here are two of the most popular treatment options for Fabry disease:

• Enzyme Replacement Therapy (ERT) : ERT is considered to be one of the primary treatments for Fabry disease. It involves giving patients regular intravenous (IV) infusions of a synthetic version of the enzyme (alpha-galactosidase A) that the body lacks. It helps to reduce a buildup of GL3 in the heart, kidneys and other organs.
• Chaperone Therapy : It is suitable for patients with particular types of mutations in the GLA gene. It uses a small-molecule drug which helps to stabilise the body’s faulty enzyme, enabling it to function more effectively.

While the cost of a kidney transplant in India varies depending on the hospital, geographical location of the medical facility and surgeon’s expertise, understanding the medical expenses can help to make informed decisions.