Saturday, 18 March 2017

25 Year Male - Case History from 2010 till date (March 2017) - 1st Post










From childhood the care-seeker , a 26 years old young scientist from Kolkata , is suffering from a rare disease called HME (Hereditary multiple exostoses)/ Hereditary Multiple Osteochondromas. The disease was first diagnosed in CMC vellore in 2010 when care-seeker was in 1st year of his college. But no report is available from 2010. Due to certain logistical and financial reason only an operation took place in his leg but no long-term management plan was started or followed upon.

In 2015 when care-seeker started his PhD, the bony lump reappeared in his left neck/shoulder area causing complete paralysis. Again they traveled to Vellore but for an unfortunate event he never reached vellore ( His father expired on the way to Vellore in Train). Ultimately in 2015 Dec in Institute of Neurosciences in Kolkata this rare disease was confirmed and another operation happened in 2015 end - the neck dissection operation to remove a bony tumour was successful and he got back sense in his hand and leg.  A molecular genetic testing of the EXT1 and EXT2 genes is available to confirm the diagnosis but it's quite costly and from the observation of bony lumps , the disease diagnosis is almost a certainty.."Also known as diaphyseal aclasis - It is a rare medical condition in which multiple bony spurs or lumps (also known as exostoses, or osteochondromas) develop on the bones of a child. HME is synonymous with multiple hereditary exostoses and multiple osteochondromatosis, which is the

preferred term used by the World Health Organization." 


Ref: 
https://rarediseases.org/…/hereditary-multiple-osteochondr…/
https://rarediseases.info.nih.gov/…/hereditary-multiple-ost…
https://ghr.nlm.nih.gov/…/hereditary-multiple-osteochondrom…
https://www.ncbi.nlm.nih.gov/books/NBK1235/


Now cut to 2017 
==============
In Feb, 2017 the lump is again back on the left neck, with severe vengeance, causing a complete paralysis in the left side of this young guy requiring another costly operation. The incidents unfolded as below in the own word of care-seeker (I am doing a little edit for brevity): 

“On 2010, when I was in my college first year, my right side leg got extremely swollen up. Local doctors had done lots of check-ups and diagnosed it as CANCER. They mentioned that I have to get my leg amputated. So, me and my father left for CMC Vellore in 2011. DR. V.T.K TITUS did an operation and saved my leg and I survived. But we can’t pay for the whole treatment cost, and so the reports etc, remained with Vellore. I don’t have any record of it but, I can recall that he might have told that it is HME (some type of tumor in bone). After coming back from Vellore, though I had occasional swelling up in different parts of the body, I was neglecting it, as the pain was not unbearable. In 2015, I completed my M.Sc and started Phd. But in middle of 2015 (August) my left side neck got extremely swollen up and the pain became unbearable. So, we (me, my father and mother) again travelled to CMC vellore. But during the train journey, my father expired due to a massive heart attack and so we had to come back. My left side was starting to get paralyzed and so I visited Institute of Neuroscience kolkata and was treated by Dr Bose and DR. CHRISTOPHER GERBER. They did a neck dissection operation to remove a bony tumour – it helped, my pain got reduced and my paralysis was gone. But again this February 2017, my left hand got paralyzed. My left side neck, where operation had been done, is swollen up again. I visited INK and they mentioned that an urgent operation needs to be done (Attached) and that needs to be done by 24th March. The operation will cost 3.5 Lakhs which is difficult for me but my friends and families are trying best to arrange it. But also they mentioned that this operation is not a complete cure. This may come back. I am really, really confused? Please guide me



The latest reports and prescriptions from INK is attached here:






Also attaching all the slides soft-copy - as clear as possible -

















My observation :

Based on my discussion with the care-seeker and after going through the prescriptions and EBM based study from internet, it seems his treatment for HMO/HME has never been started back in 2011. This may be due to financial and logical constrains after the Vellore treatment. The current treatment and the operation seems to be symptomatic – the pain killers cannot help him and the nerves are being impacted causing paralysis. So the operation is being done. In my opinion, a holistic treatment plan should be done starting from the bone and treating the problem not the symptom.

But on the other hand the young guy is going through quite a bit of pain and left side is getting paralyzed – he is really concerned that he may lose his left side and obviously the stress and anxiety is causing the situation to deteriorate.

Under this circumstances, please suggest what will be the best way moving forward?

The minimal first thing that I am looking from the group is starting his long term treatment in Kolkata keeping in mind the paralysis issue of his left side. It will be ideal if it is in Govt Setup in Kolkata, due to the person's financial condition.

No comments:

Post a Comment