Things you should know about this autoimmune disorder with an ‘unknown cause’

Creative lettering of awareness by Sini


The first step towards curing any disease/disorder is to identify the cause and accordingly go with diagnosis. But what if a disorder is diagnosed as “Idiopathic (unknown cause) ” on the first hand?

How does one go about the treatment or even try to understand the problem? How does a battle with an unknown enemy look like? What if I say the enemy has turned your own body against yourself?

Well, this post is written in order to bring out awareness on a rare auto immune disorder, ITP (Idiopathic Thrombocytopenia Purpura) in layman terms, through personal experience.

Disclaimer

This is just about getting to know the basics of the autoimmune disorder written as part of global ITP awareness week and certainly not a complete guide. This is to help you understand ITP in layman terms.


Your questions and reflections are welcome.


What does ITP mean


It’s an autoimmune disorder, where the counts of circulating platelets are low causing excessive bleeding (externally and internally) and easy bruising.

What does the abbreviation ITP stand for


ITP stands for Idiopathic Thrombocytopenia Purpura.

The word Idiopathic is the more pathetic thing about ITP; because idiopathic means “for reasons/cause unknown”, thereby making this disorder complicated for diagnosis and treatments.

Thrombocytopenia means low platelet counts. By the way, platelets are the tiny cells present in blood that helps in clotting. So, low platelet levels mean less/no clotting and so excessive/unstoppable bleeding.

Purpura means red or purple coloured spots found on the skin caused due to bleeding underneath the skin.

Why do we call ITP an autoimmune disorder and not a blood disorder


Well, though the level of platelets gets low here, the underlying issue is mainly about one’s own immune system attacking the healthy organs or cells of the body (in this case, the target organ being the platelet cells).

How is it diagnosed


It can be initially diagnosed through bruising, excessive or uncontrollable bleeding, nose bleeds or appearance of purpura (purple spots on skin) or petechiae (tiny round purple or reddish to brown spots or bumps on skin).

It can also be revealed through routine blood tests with significantly lower levels of platelets. My initial diagnosis started with the routine blood tests during my pregnancy.

Normal platelet count ranges between 1,50,000 to 4,50,000 platelets per microliter of blood.
Any count of platelet below 100000 per microliter of blood will be considered as thrombocytopenia.

Does a lower platelet count always mean ITP


Certainly NOT. ITP is diagnosed through elimination of other causes for lower platelets. A decrease in platelet counts can happen because of an infection, or viral fevers, or leukaemia or bone marrow abnormalities.

How to conclude that low platelet in a particular case is because of ITP


It’s mainly and only through elimination of causes due to other diseases that one can conclude as ITP. There’s no direct reasoning or diagnosis for ITP.

Treatment for ITP


The sad part of ITP is that there’s no cure. The treatment is generally available for the symptoms and not for the cure of the disorder itself.

Since the reason behind this attack of one’s own immune system is weird and unknown, cure for the disorder is a question mark.

What does it mean to treat the symptoms and not the disorder


Well, there is no proven direct method of increasing platelets through medication or diet. What the doctors do is start to treat the symptom like spontaneous bleeding only when the platelet count drops down below 20000/mL.

How do they treat the symptom


Won’t treatment mean increasing the platelet counts? Doesn’t it then prove that platelets can be increased?


Well, here is the catch. To understand how the treatment works and what’s done to increase platelets, let us first understand what happens in the case of an ITP patient.

To be diagnosed as ITP at first means that other “causes” for low platelets is eliminated which certainly means everything necessary for a proper production of platelets is fine.

It is our body’s immune system that fights against the infections and other foreign bodies and helps us stay healthy. Now what happens with an ITP patient is that his own immune system considers platelets as foreign bodies and attacks it.

Now you can picturise platelets being produced in the body which is simultaneously destroyed inside the body. So, there’s no problem with production.

All that needs to be done is to stop the immune system from destroying the platelets. And this is done by suppressing the immune system.

Now you can imagine what will happen when the immune system that which fights from infections, is being supressed. Certainly, by suppressing the immune system, the platelet levels stay intact as they are protected from destruction.

But then, the supressed immune system gives way for other foreign bodies and infections to get into the body easily thereby causing severe side effects.

Given the complications, what exactly is the solution for ITP


Let me remind you, there is no solution at all. Yes, you hear it right (have hopes, researches are on to find a cure). What can be done till then is that ITP can be “managed”.

Unless there is an episode of spontaneous and critical bleeding, one can actually “wait and watch” for severe symptoms as they move on with their everyday life.

Treatments start only under a situation when the counts are so low that it can be life threating and when the doctors feel side effects of treatments are worth risking.

How critical or life-threatening is ITP


Relax, it’s not fatal in most cases. But it’s complicated. There can be unstoppable external bleeding and unreasonable internal bleeds which can get severe and prove fatal if not attended on time.

What are the complications with ITP


To make things even more complicated, not every ITP patient have same complication. Even the treatments are unique to each. To list down few, here are few complications faced.

a. Excessive bleeding is a visible complication. There can be nose bleeds, gum bleeds and easy bruising.

b. The menstrual bleeding can be heavier.

c. Internal bleeding and clotting inside vital organs leading to haemorrhage (rare occurrence though).

d. Weakened immune system prone to various infections.

e. Loss of bone density.

f. Undergoing surgeries and other procedures gets more complicated with risks of high bleeding and infections.

g. Chronic fatigue, as the body has to work overtime to make up for the loss of platelets due to self-destruction and meanwhile flush out the toxin build up because of destroyed platelets. It’s like thrice the work done by a normal human body.

Can platelets be increased through medication or diet


Let me put this clear. Platelet production is good and just that it’s getting destroyed. To stop that from happening, immunosuppressants are given most times which helps in controlling the destruction and thereby keeping the counts stable but all at the cost of weakening the immune system.

There is no proven medication of increasing the counts without side effects or simply through diet.

Trust me, recently famous papaya leaf extract mostly works in case of lowered platelets where the “cause” is a viral fever and not ITP.

But otherwise, a healthier diet certainly helps in keeping the other vitals in tact and keep other infections at bay even while we are battling with lowered platelets.

What does the life of an ITP patient look like


Every ITP patient is different. There are commonly two broad groups; bleeders and non-bleeders.

Bleeders are those with severely low counts and bleeding episodes which needs frequent treatments including platelet transfusions and hospitalisations.

It’s highly important to avoid contact sports and be very cautious as to not get hurt in such cases.

With the non-bleeders, the counts are managed mostly above 50000 per mL of blood though there can be high fluctuations.

There needs to be a regular observation for visible symptoms. But that’s not all.

They can be looking perfectly normal even while they can’t escape from the chronic fatigue caused due to excess work done in the production of additional platelets to keep up with the destruction rate.

Bleeding under the skin with appearances of purpura (purple spots on skin) is a common sight which keeps them in a constant fear anticipating internal bleeds too.

Managing the side effects of treatments is even more complicated and it’s a different story in itself. (It needs a separate post on treatments and its side effects.)

Overall, it’s an everyday battle over reasons unknown and making choices and priorities to save on energy in every possible way.

What’s the necessity or importance of this post


The ITP awareness scenario is pathetic in India and even worldwide to an extent that google will not even list ITP among the rare autoimmune disorders.

Low platelets being associated with other viral infections too, it’s been misunderstood and it takes much longer time to even diagnose it as ITP.

In some cases, if not approached the haematologist on the first hand, the patients are started with unnecessary medications leading to lots of side effects which can be avoided if there is an awareness.

Most importantly, fighting ITP is as much as a mental battle as is a physical one. Awareness on ITP helps the autoimmune warriors and their support system take informed decisions.

It also eases the battle when the autoimmune warrior feels their invisible struggle heard & validated. Hence this post.

This post is part of Blogchatter’s CauseAChatter.