Today I came across this video on Youtube. I wanted to share it because it’s a great overview and explanation of HSP, what causes it and it’s treatments.
Say what?! This article certainly is an eye catcher. According to this article from medicalnewstoday.com, the scientists from University College London have discovered why tetanus is so toxic, but could also be used as a therapy for motor neuron related disorders.
Tetanus is delivered via a group of proteins called nidogens that coat the surfaces of the cells are directly involved. Tetanus neurotoxin sticks to these proteins and is transported inside the cells. Once inside, the neurotoxin then begins to spread and cause damage throughout the nervous system.
For the study, researchers inhibited the interaction between tetanus neurotoxin and nidogens in mice. They found that in doing so, the neurotoxin was prevented from binding with neurons, and the mice were protected from spastic paralysis caused by tetanus.
Through exploiting nidogens in the same way that the bacteria do, the researchers hope that new methods can be developed with which to deliver drugs directly into the nervous system.
For senior author Prof. Giampietro Schiavo, successfully finding a way to transport treatment to motor neurons solves half the problem of treating neurological disorders. “Now that we understand how tetanus gets in, we hope to mimic the mechanism to deliver advanced therapies,” he says.
Today I saw a flyer for the 2015 Spastic Paraplegia Foundation’s annual conference which I would like to share. Next year on June 26th- June 28th, the conference will take place at Seattle Airport Marriott hotel in Seattle, WA. Their theme will be “Singing in the Rain in Seattle”. The conference will include food and other events, as well as special speakers including Dr Fink (one of or the leading doctor/specialist in spastic paraplegia) and Dr. Catherine Lomen-Hoerth, Director of the ALS Center at UCSF. This conference is a great way to meet other people living with HSP as well as discuss the latest research in HSP.
I really like this flyer that was put together. The theme is very relative too…because if there’s any message to give about HSP it’s that you have to ‘sing in the rain’. Even when you feel like giving up, always have hope, faith, and excitement.
This week, like many new and exciting things I come across on the Spastic Paraplegia Facebook page, I discovered a Kickstarter for project for a revolutionary wheelchair concept. This wheelchair, called the Freedom Chair, will give people with walking disabilities much greater freedom in how they use their wheelchair.
This new project, the Freedom Chair, will allow people formally bound to the confines of paved grounds to explore trails, parks, fields, beaches, and even gardens.
The Freedom Chair will feature a lever drive for easier maneuvering over obstacles with 50% more torque then pushrims. It also has standard bike parts for easy repairs and customization, three big wheels, and boasts in portability and being able to fit in the trunk of a small sedan.
The idea of the Freedom Chair was started by a group of students at MIT. They have already sent over 1000 of their first model Freedom Chairs to people in need in over a dozen countries, and with their new Kickstarter program they hope to make the Freedom Chair to people with disabilities the US.
Although this chair seems to be pretty pricey (looks like retail price is in the $3000 range), I think it is an amazing idea. The Freedom Chair will allow people with physical impairments to go places they haven’t been able to before, such as on hikes and to the beach.
So go check out this awesome Kickstarter.Here
For this weeks blog I want to share a list of websites that provide great information and news on HSP.
If you are looking for some more information on HSP, here are some great websites to check out.
Another interesting article I found on the Spastic Paraplegia Foundation Facebook page. A paralyzed man from Poland is able to walk again after a new method of therapy involving transplanting cells from his nasal cavity into his spinal cord. He didn’t suffer HSP, but was paralyzed from the waist down after a knife attack in 2010. This therapy, the first time ever attempted in the world, was preformed by surgeons in Poland in collaboration with scientists in London. The treatment used olfactory unsheathing cells (OECs) which act as pathway cells that enable nerve fibers in the olfactory system to be continually renewed. The first operation involved removing one of the patient’s olfactory bulbs and growing (multiplying) the cells. Then two weeks later they transplanted the OEC’s into his spinal cord, The scientists believe the OECs provided a pathway to enable fibers above and below the injury to reconnect, using the nerve grafts to bridge the gap in the cord.
The man was paralyzed for 2 years before the transplant therapy. After surgery and physical therapy, he regained his movement and was eventually able to walk with a frame.
Hearing about this new transplant therapy is great news, especially because it opens up possibilities for HSP therapy as well as for other people with spinal injures.
This week I decided to do some research on the baclofen pump therapy. I’ve been staying up to date with the Spastic Paraplegia Foundation Facebook page and from what I’ve been seeing, the bacolofen Pump is the most used treatment for HSP.
What is a bacolofen pump?
Baclofen is a medication used to decrease spasticity (muscle spasms) related to multiple sclerosis, spinal cord injuries, or other neurological diseases. Baclofen is a derivative of gamma-aminobutyric acid (GABA). GABA, as some people might know, is also used for ADHD, ADD and relaxation.
Baclofen can be administered topically or intrathecally (directly into the cerebral spinal fluid) using a pump implanted under the skin.
Intrathecal pumps offer much lower doses of baclofen because they are designed to deliver the medication directly to the spinal fluid rather than going through the digestive and blood system first. They are often preferred in spasticity patients such as those with spastic diplegia, as very little of the oral dose actually reaches the spinal fluid. Besides those with spasticity, intrathecal administration is also used in patients with multiple sclerosis who have severe painful spasms which are not controllable by oral baclofen.
Here is a youtube video of a man describing the baclofen pump and his experience with it.
When I first started this blog, I had no idea what the difference between HSP, PLS, FSP and ALS were. I knew HSP and PLS were vary similar in it’s symptoms, but I didn’t realize FSP (Familial Spastic Paraplegia) was also a branch of HSP.
Until lately when the big ‘ice challenge’ began on facebook I had no idea what ALS (or Lou gehrig’s disease) was. I was surprised to find that ALS is actually very similar. I came across a blog recently on the Hereditary Spastic Paraplegia HSP Primary Lateral Sclerosis PLS facebook page written by Adam Lawrence, a young man who suffers from HSP in which he has a great post about the differences between them. His post The ALS Ice Bucket Challenge defines each term and gives a great overview of their differences. They are are similar in that the early symptoms for all three are similar. Also all three cause upper motor neurons to degenerate laterally from the brain down to the spine. The differences are ALS and PLS progess to more parts of the body, while HSP and FSP primary effects lower extremities and lower muscles. Of HSP/FSP, PLS and ALS, ALS is the only one which life expectancy is reduced due to the disorder.
Reading Adam’s blog was very insightful, because like many people, I had no idea ALS was a related to HSP. It’s kind of ironic that everyone knows what ALS is, but if you ask them what HSP or FSP is, they probably wouldn’t know.
There is no cure for Hereditary Spastic Paraplegia, only treatments which minimally help the symptoms. There are medications that are used to limit pain, but then there is a risk of addiction. There is physical therapy, such as stretching and water therapy which help to n extinct on the short term scale, but nothing close to a cure or way to slow the progressive disorder. But it seems researchers are getting closer to an answer.
Today I decided to look up news articles on Google about Hereditary Spastic Paraplegia. Last week I did the same google search and was returned with zero articles mentioning HSP. Today I found this article posted by the The Scripps Research Institute. The insitute has discovered that a gene mutation found in HSP patients interferes with the normal breakdown of triglyceride fat molecules in the brain.
After my search last week I was confused, not sure if they were even doing any research into HSP and it’s causes/treatments. But this article confirms that scientists are getting closer to finding a treatment for HSP. To me, it sounds like they are getting pretty close to narrowing down the cause to the DDHD2 protein. Not having much biology background, this article was a little intense and over my head, but the fact that their study is getting closer to finding an inhibitor for this protein is amazing news for people with HSP.
What is HSP? Many people have never heard of the term HSP, and even fewer have ever met a person with HSP. HSP is short for Hereditary Spastic Paraplegia. HSP and FSP (familial spastic paraparesis), together refer to a group of inherited disorders. HSP is caused by defects in the way that proteins and other substances are transported through the cell. Long nerves (nerves in the legs) are affected because they have to transport cell information through long distances, and are particularly sensitive to defects of the cell’s transportation system. Although symptoms vary, the main characteristics of HSP are
- progressive stiffness and contraction (spasticity) of the legs
- weakness in the lower limbs caused by neurological dysfunction.
- Gaited/unbalanced walk
Worldwide, the occurrence of all HSP is rare, with related diseases combined estimated to be 2 to 6 in 100,000 people. In the posts to follow I hope to give people a better understanding of a very unknown about disease. I will give people a better understanding of HSP, share news and studies related to the disease, talk about different medical journals about HSP, as well as share an interview with a first person experience of the disease.