Comprehensive Guide to Cystinosis Treatment and Costs in India

Understanding Cystinosis: Treatment Options, Costs, and Challenges in India

Cystinosis treatment in India primarily involves oral cysteamine to reduce cystine buildup in the body, and in severe cases kidney transplant. Cystinosis is a rare genetic disorder, and the cost of medication for a month can be around Rs. 15,000. However,  it is not widely available in India and often needs to be imported, making access and affordability a challenge.

What are the Treatment Options of Cystinosis and its Cost in India?

Treatment for cystinosis mainly focuses on reducing cystine buildup, particularly in the early stages. Here are the treatment options available in India to resolve this condition:

● Cysteamine therapy

Cysteamine therapy is a treatment for nephropathic cystinosis, which reduces the levels of cystine within the cells, slowing the progression of kidney damage and other complications. Oral cysteamine capsules can range from Rs. 2,000 to Rs. 5,000 per box, while cysteamine creams can cost around Rs. 400 and Rs. 1,000 per tube, cysteamine creams available in India are primarily used for dermatological purposes like pigmentation and are not part of standard cystinosis treatment..  Capsules such as Cystagon are imported and can cost between Rs. 25,000 to Rs. 50,000 per 100-capsule bottle, depending on dosage and supplier.

● Fluid and Electrolyte Management

This procedure primarily focuses on addressing the consequences of renal Fanconi syndrome, a condition where your kidneys lose essential electrolytes and minerals into the urine. Dialysis is one of the most common procedures to refill the electrolytes, which costs Rs. 3,000 per session (2-3 times a week).

● Kidney Transplantation  

In rare or severe cases, nephrologists recommend kidney transplantation to treat cystinosis permanently. The cost of a kidney transplant in India generally ranges from Rs. 5,00,000 to Rs. 15,00,000. This procedure aims to replace the function of the recipient’s failed kidneys, offering a better quality of life and longer survival compared to dialysis.  

Please note that Cysteamine is not widely available in India. Thus, it needs to be imported from another country, which will cost somewhere around Rs. 4,00,000.

What is the Prevalence of Cystinosis in India?

In India, specific prevalence data are limited, but around 19 cases have been registered with the Cystinosis Society of India in 2022. While numerous healthcare providers consider this a rare condition, it is one of the most common causes of inherited Fanconi syndrome in India.

What are the Benefits of Cystinosis Treatment?

Cystinosis treatment, primarily with the drug cysteamine, is highly helpful in managing the disease and improving the quality of life for individuals with cystinosis. While it is not a permanent cure, cysteamine effectively lowers cystine levels in the body.

This treatment procedure slows down the progression of kidney damage and its complications. Furthermore, cysteamine eye drops can help dissolve cystine crystals in the cornea, relieving light sensitivity and improving vision.  

What are the 5 Complications Associated with Cystinosis Treatment?

Treatments for cystinosis are crucial for managing the symptoms, but they can lead to several complications. Here, we will highlight some of the complications of cystinosis treatments. These include:

1. Liver Problems: Although it is less common, sometimes cystinosis treatment can affect the liver, potentially leading to an enlarged spleen or portal hypertension.
2. Kidney Function: Even with cysteamine treatment, some patients may experience progressive kidney damage, leading to end-stage renal disease (ESRD).
3. Ocular Complications: Cystine crystals can accumulate in the cornea, leading to photophobia, eye pain, and vision problems.
4. Endocrine Issues: Cysteamine can affect the endocrine system, leading to hypothyroidism, diabetes, and other hormonal imbalances.
5. Neurological Complications: Some patients experience neurological complications, like cognitive and motor deficits (difficulty walking and seizures), when undergoing cystinosis treatment.   

It is crucial to consult with specialists from a multidisciplinary team, such as nephrology, ophthalmology, neurology, and endocrinology, to treat cystinosis. They will help you get proper guidance regarding the treatment and prevent complications.