SPINRAZA FREQUENTLY ASKED QUESTIONS

Hi, everyone! For the past few weeks, I’ve been receiving so many awesome questions about Spinraza and my experience with it. So, I thought I’d take this opportunity to write a blog about your frequently asked questions in hopes of providing more insight into this treatment.

However, before I proceed, it’s important to understand that everyone is different and to consult a doctor before proceeding with anything. Whatever my personal experiences with Spinraza may be may very well differ from what you (your child) experience. Moreover, I’m not a doctor. 😉 The information I’ve provided below is simply based on what I’ve experienced firsthand, and it is my greatest hope that by sharing this journey with you all, I can, at least, provide some awareness and slightly ease the uncertainties that come along with this groundbreaking treatment. After all, you and I are in this together, my friend!
What type of SMA do you have?

Just before I reached 6 months old, my physician diagnosed me with SMA type I. It was 1991, so there was very little information readily available about this disease. However, I started showing signs at 3 months of age, so that was the reason for my type I diagnosis.

Today, I consider myself a strong type I. I’ve never really “followed the textbook” when it comes to the medical prognosis of this form of SMA. I don’t have a trach/vent and still completely eat by mouth but use a trilogy in BiPap mode when I sleep. Some of my medical records say Type I/II, so I usually joke and tell people I have type one and a half. (I may be the only person who thinks I’m funny.)

Do you have rods?

My parents and I considered the spinal fusion surgery, but with the high risks involved and after much deliberation from orthopedic specialists, we chose not to. I have a curve well over 100 degrees (I’m not even sure of the exact number- I stopped keeping track after the “it’s really bad” prognosis) and wear a body brace when I sit up for support.

Having severe scoliosis poses some difficulty for these procedures as access to the spinal canal is basically a tricky obstacle course. However, this isn’t to say that it ISN’T doable. It just takes a little more patience and navigational planning before the needle is inserted.

Do you have to prep for the procedure?

There really isn’t any major prep that comes before a lumbar puncture. However, I was forewarned by the surgeon to clean out my pipes the day before. Although the needle is going into your spine, you will feel a lot of abdominal pressure that will lead you to think you have to use the bathroom. So, I highly recommend investing in some Miralax and taking care of business the night before if you catch my drift.

I also recommend staying hydrated. Although you are unable to eat or drink a few hours prior to the procedure, be sure to drink plenty of fluids the day before you go in. Dehydration causes the extraction of the spinal fluid to slow down an already slow process. It’s also uncomfortable and somewhat painful, and unfortunately, I learned this the hard way.

What is the process of the procedure?

This will definitely be a little different for everyone. For me, I start off in Day Surgery Pre-Op where a nurse takes my vitals and the surgeon reiterates everything I need to know about the lumbar puncture before I sign off on my agreement to move forward. From there, I’m wheeled into an operating room and transferred onto the OR table. My parents follow me in so my father can set up my BiPap (just as a precaution) and my mother can work with the surgeon to get me in a good position while maintaining some level of comfort.

Once I’m situated, someone from radiology brings in this giant machine that rotates called a fluoroscopy. For a solid 15-20 minutes, the fluoroscopy manually rotates around me while taking pictures of my spine to help the surgeon map out a way to the spinal canal. This portion of the procedure takes place because of my severe scoliosis. Instead of jabbing me blindly, the surgeon can gain a clearer understanding of where the needle needs to be inserted into my “non-vanilla” spine.

After all this information is gathered, the actual lumbar puncture begins. The injection site is thoroughly sanitized, and then the local anesthetic is given by a shot. A few minutes later, the surgeon will slowly begin to push the needle into the spine while still administering the local to really numb the area. There is a lot of pressure and a moderate amount of pain for me at this point, but I do my best to take nice, deep breaths.

Then, a second needle is inserted through the first needle and is basically guided into the canal by that first, larger needle. Before injecting the drug, spinal fluid must be extracted. Like I said above, this is typically a slow process, but once it’s completed, the exciting part comes- Spinraza! Think happy thoughts and try your best to distract yourself from what’s going on behind you. It’ll be much easier to get through.

The time, from start to finish, will vary.

Do you receive sedation for it?

My body is too weak to receive anesthesia, so no. I have been offered something that will relax me, but I just choose not to take it.

Is it painful?

Plain and simple, the answer is yes. After all, there’s a foreign object going into your spine- what else do you expect?! Although my first lumbar puncture went so smoothly and nearly pain-free, I felt it was very misleading because something like this hurts. It’s uncomfortable, and it’s stressful not knowing how long it will take to find the spinal canal. I usually get heartburn which is probably just a mixture of not being able to eat, stress, and abdominal pressure. I also get slightly dizzy, but that just might be a “me” thing as I’m constantly having light bouts of dizziness throughout the day.

The pain is never long lasting for me. There will be moments of no pain, little pain, and extremely uncomfortable pain- fortunately, that only lasts a second or two! I will sometimes feel a shock or a little tingling in my foot which is due to all the nerves that surround the canal. The extraction of the spinal fluid and injection of the drug may hurt, but it’s nothing major.

I know this may seem like a lot to take in, but I promise that living with SMA, we’ve all been through MUCH worse. In due time, you will get used to these things.

What is the recovery process like?

To avoid a post-lumbar puncture headache, laying flat for an hour is required. I haven’t run into any issues thus far and have always been discharged after that hour. Following a day at the hospital, I usually like to take it easy for about 24 hours.

Do you experience any side effects?

The only side effects I’ve experienced have been a result of the lumbar puncture and not the actual drug. This includes headache, minor pain at the injection site, nausea, and light dizziness- nothing lasting more than a day.

What changes should you make to your lifestyle?

Although I wasn’t specifically advised to do so, I have made some changes or improvements to my life just for my own benefit. I’m eating more foods with protein, exercising via occupational therapy, and doing less. This has allowed me to truly focus on my body and what’s best for my health.

Do you feel any different?

Not yet, although I refuse to let myself feel discouraged. Doctors say IF this drug will be effective for me, it’ll take about 2-3 months for me to notice a difference. I’m about one month in and have received three injections to date. So, in the meantime, I just need to keep having faith.

How are you paying for this procedure?

Right now, I’m in the process of transitioning from the EAP to commercialization, so, unfortunately, this is something I cannot answer at the moment. My parents and I are currently exploring this matter and hope to receive answers soon.


*Disclaimer: While my goal is to remain as open and honest with you on this new journey, the opinions expressed here represent my own and not the SMA community as a whole. Should you seek medical advice regarding Spinraza, please consult with a medical professional. Thank you for following along!

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Chronicling my journey while teaching the world that I have SMA, but it will NEVER have me. Life's what you make it, dare to make it a remarkable one✨

29 thoughts on “SPINRAZA FREQUENTLY ASKED QUESTIONS

  1. I think you are very brave and hope that Spinraza brings some improvement for you and for all the others with SMA. Enjoy reading your blog so much. My granddaughter also has type 1 SMA, and will be 8 next Monday. Sending you love and healing.

  2. Hi Alyssa. Thank you for sharing this part of your journey. It’s very interesting. I think you are very brave & have a great upbeat attitude that inspires others. I pray that these treatments will help you. Hang in there!

  3. Great stuff Alyssa, Love the Q&A concept….. Great way to approach all the Q’s you must get and answer those who have thought but not asked…..Your mind to me seems always one step ahead (I love that kind of mind)…… God Bless and Rock on >>>>>>

  4. Thank you so much for sharing. Hoping to get my son (Type I, 14.5 years old) on the drug soon. In the beginning I was so worried about the actual lumbar puncture, but after reading yours and others’ experiences, my mind can rest a little easier. Good luck with your future injections!

  5. I am 18 I also am a strong type 1. How often do you have to get the Spinraz treatment? I did have a spinal fusion so that would probably make a difference for me.
    I’m also wondering if your parents are able to stay with you wall the procedure is going on or do they have to wait in the waiting room?

    1. Hey Katelyn! I’m currently receiving the loading doses which is 4 in 2 months. After that, I will go once every 4 months. To answer your other question, I think that will depend on your surgeon’s protocols. My parents were not with me for the first treatment but were with me for the other two.

  6. Thanks for your sharing the drug given experience. God bless you and hope you get recovery assoon as possible.
    I’m aChinese patient with type III

  7. Hi Alyssa,

    Congratulations on being the first adult patient to try Spinraza! My Dr. is currently trying to get it set up at UPenn. Just wondering, when you have the injection – are you on your side instead of your stomach? My Dr. was wondering if this was a possibility.

    Good luck!

  8. Thank you so much for sharing your story. My son has Type 3, was just diagnosed last week. I am praying he will have the opportunity for treatment as well. I can’t wait to hear your next update and will be praying for you every day.

  9. Hi Alyssa. Thank you for sharing this part of your journey. It’s very interesting.I have one question for you: can you or could you ever sit without support?
    I ask you this question because here in Europe it is considered that a SMA person who can or could ever sit without support can receive spinraza (nusinersen) in the EAP(expanded access program).My 9 year old daughter Maya is in the same situation as you:she have type one and a half(yes it is funny).
    Doctor neurologist who saw her since 6 months and under the guidance of whom she began physical therapy and other recovery procedures, even if the diagnosis not been established yet, says she is Type I.Dr. neurologist who diagnosed her hardly at 2.5 years old says she is a type II Werdnig-Hoffman.

      1. Hi Alyssa, My daughter Brooklyn was diagnosed with SMA type one at 11 months of age. We were told she would not live beyond two years of age. We have been through a lot of hospitalizations fighting pneumonia. Brook will be turning 29 this Aug. 29th. She has never walked, power wheel chair at the age of 4. She has a feeding tube at night and c-pap at nights. She is uncertain about taking the injections since she has been holding her own now for about 10 years. As her mother I would like her to at least go for a consult. She definitely will make her own decision. .Any words of advice?

  10. Hello Alyssa,
    Very informative blog. It is always interesting to get the perspective of others on things like this, even though they may not go the same for ones self. I’ve got SMA type 2 with a spinal rod and use a vent.
    I am wondering if by now you have seen any changes or improvement?
    Thank you for sharing!

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