I feel as if I should talk more about the treatment I am on. Especially since it hugely contributed to me living a better quality of life. As stated in previous blogs, I moved to California from my home Aotearoa (New Zealand) in 2019 to start Spinraza. I also won’t be able to return home anytime soon either because Pharmac continues to deny this lifesaving treatment, even still with their pay increase of 71 million for 2022/2023 and then an additional $120 million for 2023/2024. (Pharmac is the New Zealand Government agency that decides which medicines and related products are funded in New Zealand)
I was on death’s bed pretreatment, and many others with SMA are too. Yet Pharmac continues to do nothing, continues to overlook us knowing a lot of us are losing full physical capabilities and dying as a result of that. Or, dying due to the deep depression that hits from the grief of losing our complete independence. I’m actually so appalled by the greed and lack of any type of empathy Pharmac has. It’s time to step up and do better PHARMAC!
To explain in somewhat simple terms what Spinraza does - People with SMA can’t make enough SMN protein, this is the protein our motor neurons need to function because we have a mutated or deleted survival motor neuron 1 (SMN1) gene. The gene we do have, SMN2, does not produce enough protein for all of the body’s muscles - leading to atrophying of the body. That’s where Spinraza can help. By increasing the SMN protein production will slow/stop the progression of the disease and you can potentially gain strength back.
In most cases, Spinraza is administered through an intrathecal injection punctured into your lumbar. But since my spine is fully fused with titanium rods from my neck, to my tailbone it creates a challenge for doctors to administer it safely and effectively.
An alternative option they developed for administering Spinraza was an intrathecal injection through a suboccipital port (upper part of the neck) - I actually was part of the first 25 people with SMA to receive this surgery. I won’t lie and pretend everything went smoothly though because it didn’t. I mean the surgery itself went great, with no complications in the actual surgery room, but I ended up developing Meningitis a few weeks after surgery (this is rare) which gave me the worst spinal headaches I have ever experienced in my life. I ended up going to ER due to the extreme pain I was experiencing, I was literally screaming in the emergency room. It felt like my head was going to explode from the pressure I was feeling. It took a round of antibiotics to clear the infection and spinal fluid build-up. I just want to point out that I know this may sound scary for anyone considering this type of surgery, but this is extremely rare. I can also say that I don’t regret getting the suboccipital port at all, the good it has done has definitely outweighed the bad. So if I had to go through all of that pain again, to get where I am now I would absolutely do it again no doubt.
I had to wait 6 months until it was fully healed before beginning with 4 loading doses of Spinraza. The first 3 doses are administered at 14-day intervals. The fourth dose is administered 30 days after the third dose. And After the starting dose period, a dose is administered once every 4 months which is 3 times a year. I immediately felt a change after the first 4 loading doses, my energy levels increased immensely. I also had a lot of twitches in my calves and thigh muscles, it was like they were waking up for the first time since I was a little kid. I again want to note that not everyone will have this immediate result with Spinraza. But that’s like with most medicines you won’t have the exact same result with every person, it may take longer for Spinraza to kick in or it could potentially not work for you at all (Risdiplam is a great alternative) but I recommend giving Spinraza at least 2 years. I know a huge variety of people with SMA who experienced a change pretty quickly, and others who started feeling a change after the 1-year mark. So it’s just going to depend on how your body reacts to the treatment. But for now, I’m just going to speak of my own experience and the life-changing good it has done for me.
To do some comparisons of strength from pretreatment to now I’ll explain it through examples - Pre Spinraza I was sleeping 16-18 hours a day, and I would hardly leave my bed. I lost the ability to do any type of art that involved my hands. To put it into perspective how weak my hands were is that I didn’t have the strength to light a lighter or hold a pen properly. I struggled with losing the ability to do daily tasks like showering, and anything involving my arms like brushing my hair. I lost the ability to cook, and that’s something I loved to do. My head was floppy so my neck would give out constantly and I didn’t have the strength to pull my head back up, so I would be stuck like that without help. The mobility losses felt like it was happening daily, I was in a constant state of grief. Grieving for the abilities I once had before. It felt like dealing with a death of a loved one every single day for years. The gut-wrenching pain of losing yourself was unbearable. But I saw a glimpse of light at the end of the tunnel, and that was hope.
This hope manifested into me finally receiving treatment. I’ve now been on Spinraza for almost 3 years. I had a lot of abilities come back that I once lost. I could finally stay up during the day without feeling exhausted, I was having regular hours of sleep. I could brush my own hair, and doing daily tasks didn’t take all the energy out of me. I was able to do things I loved again like cooking as well as finding new passions like digital art and clay work. I can do art for hours without my hands getting sore - which has done wonders for my mental health. Art is a form of therapy for me, so gaining that back is truly an indescribable feeling. I’ve also developed new hobbies like becoming a plant mum, and up-cycling thrifted finds. I also take pride in keeping my space always aesthetically pleasing (photos below) especially since I now have the energy to do it mostly independently. The floppy neck is no more, and I actually gained the strength back in those muscles. I can now support my own head and lift it back up when tilted. Science man, where would we be without you?
As of right now, I feel as if I have leveled off, I’m not losing any more strength but I’m not making as many ability gains as I was within the first year. - I mean I am still having those strength gains, but not as frequently and consistently. Now it’s more about maintaining what I did gain back, I still get bursts of energy which allows me to get so much done. But then other days I’m a Lil tired, but nowhere near what I was - I’m so grateful for that too. I do want to point out one con that I have about Spinraza because it’s been a slight problem for me and I want to be transparent in my blogs, instead of pushing the idea of everything being sunshine and rainbows. As I said previously a dose of Spinraza is administered once every 4 months, so about 2 weeks before I’m due for Spinraza I’m extremely tired, and I’m back to sleeping a lot. It’s like a car running out of gas basically, gas being the Spinraza. I guess that’s how you know it definitely works though, but I rather not go through 2 weeks of not being able to do much. However, it is in the works to increase the Spinraza dosages to fit the patient's needs better so it lasts longer in your body. So that soon won’t be a con, but I thought I would mention it.
I guess the overall theme of this is to have hope. To carry hope during your darkest days, to know that where there is a will there's a way. To keep speaking up for our rights in hope that soon they will finally hear us. But for right now I hope one day Pharmac’s small heart grows three sizes bigger just like the Grinch Who Stole Christmas but in this case Pharmac Who Stole The Lives Of Those With Rare Diseases.
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