Guillain-Barre Syndome #TeamTrev

What is Guillain-Barré syndrome?

Guillain-Barre Syndrome (GBS) is an auto-immune syndrome where your body’s immune system attacks the myelin of the nerves in your peripheral nervous system (everything other than your brain and spinal cord). This creates a “short-circuit” in the nervous systems. Initial symptoms include weakness, tingling, and pain of the legs. In many instances, these symptoms also impact the arms and upper body. This syndrome becomes life threatening when your antibodies start to attack the nerves that control your lungs, heart, and organs, on top of your legs and arms. When GBS is this severe, the person is put on a ventilator to assist with breathing and is watched closely for problems such as an abnormal heart beat, infections, blood clots, and high or low blood pressure. Most individuals, however, have good recovery from even the most severe cases of Guillain-Barré syndrome, although some continue to have a certain degree of weakness.

Who susceptible to Guillain-Barre?

Guillain-Barré syndrome can affect anybody. It can strike at any age and both sexes are equally prone to the disorder. The syndrome is rare, however, afflicting only about one person in 100,000. Usually Guillain-Barré occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection. Occasionally surgery, pregnancy, or certain vaccines will trigger the syndrome. Recently, there has also been a link to the Zika virus and the GBS diagnosis. After the first clinical manifestations of the disease, the symptoms can progress over the course of hours, days, or weeks. Most people reach the stage of greatest weakness within the first 2 weeks after symptoms appear, and by the third week of the illness 90 percent of all patients are at their weakest.

How is Guillain-Barre Diagnosed?

For us, Trevor was diagnosed with an MRI and lumbar puncture. A lumbar puncture will present with higher amounts of protein when Guillain-Barre is present in the patients’ system.  All x-rays, ultrasounds, blood work, and physical observation came back normal.

How is Guillain-Barré treated?

IVIG, intravenous immunoglobulin therapy in our case! (Plasmapheresis and steroid treatment are also used for some patients in different hospitals or parts of the world.) Investigators have found that giving high doses of these immunoglobulins, derived from a pool of thousands of normal donors, to Guillain-Barré patients can lessen the immune attack on the nervous system. Researchers don’t know why or how this works, although several hypotheses have been proposed.

What is the long-term outlook for those with Guillain-Barré syndrome?

Guillain-Barré syndrome can be a devastating disorder because of its sudden and unexpected onset. In addition, recovery is not necessarily quick. As noted above, patients usually reach the point of greatest weakness or paralysis days or weeks after the first symptoms occur. Symptoms then stabilize at this level for a period of days, weeks, or, sometimes, months. The recovery period may be as little as a few weeks or as long as a few years. About 30 percent of those with Guillain-Barré still have a residual weakness after 3 years. About 3 percent may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack.

What research is being done?

The fact that so many cases of Guillain-Barré begin after a viral or bacterial infection suggests that certain characteristics of some viruses and bacteria may activate the immune system inappropriately. Researchers are searching for those characteristics. Certain proteins or peptides in viruses and bacteria may be the same as those found in myelin, and the generation of antibodies to neutralize the invading viruses or bacteria could trigger the attack on the myelin sheath.

Our Story

Timeline

1/7-1/10: Viral illness, runny nose, very high fever, but nothing out of the normal for a childhood illness

1/21: Severe leg pain, initial symptom

1/24: Urgent Care Visit, 2 x-rays, Mira lax prescription, constipation diagnosis

1/28: ER #1, constipation diagnosis, hip ultrasound, 1 x-ray, 1 full panel bloodwork, 1 enema

1/31: ER #2, Growing pains diagnosis, told to go home, the nurses thought I was crazy

2/1: Pediatrician appointment, sent immediately to ER

2/1: ER #3, 4 x-rays, full panel bloodwork #2, enzyme panel, electrolyte panel, everything came back normal

2/3: ER #4, Sent to main hospital ER for MRI

2/3: ER #5 MRI, Guillain-Barre Diagnosis

2/3-2/8: Admitted to Children’s Mercy Hospital

2/4-2/8: 5 rounds of IVIG

2/8: HOME

2/17: Post-hospitalization appointment

4/27: Return of reflexes

Initial Symptoms

Excruciating leg pain, especially the backs of his thighs.

Quit using the toilet (due to leg pain and pressure when sitting, was JUST potty trained)

Slight constipation (debatable)

Holding my hand frequently to walk (I thought it was a clingy stage)

Started crawling again (crawl to a tall object and use it to stand up)

Cruising with furniture to walk (loss of strength and balance)

No fever, no rash

Complained of leg, belly, and back pain (point and say “hurt”)

Difficulty sleeping (used to sleep a solid 11 hours)

Positive Kernig’s sign and Brudzinski’s sign (didn’t end up being meningitis, obviously)

Pain when lifting his legs in the air to do a diaper change (moving his legs in general caused him pain)

Ended with tremor-like weakness in his arms before we finally had a diagnosis

 

Explanation by Facebook Posts

2/3: Failed Legoland, MRI, diagnosis

“This morning I thought it would be fun to take the boys to the aquarium and legoland for the first time in a while. We have been in and out of doctors offices, ER’s, and waiting rooms for weeks now, and both boys have been so well behaved. We went to get our shoes on, and I notice Trevor couldn’t stand up. He got his shoes on, put his legs in place to stand, tried, and toppled over. He tried three times before he looked at me in utter confusion and panicked. For weeks he has been “cruising” holding onto couches, crawling from place to place, wanting to hold my hand to walk, and being very clinging. At first, the affection was thought to be his “constipation,” and general feeling of unwell, but it was actually his first symptoms of muscle weakness. It is nearly impossible for a 2 year old, who is already in speech therapy, to accurately describe the tingling, numbness, and pain associated with his diagnosis. “Leg hurt,” “belly hurt,” and “back hurt” was all we have gotten. It was immediately then in his inability to stand that I knew something was terribly wrong with him. The nurses a few nights ago might have belittled me, and until I saw his panic look, I felt broken. The doctors and nurses couldn’t ever see his symptoms because he was clinging to me at every appointment, so I immediately got out my phone to try to video record his failed attempts. I needed proof that he was having muscle weakness and failure, because up until today, no one believed me. When I tried to take the video, my phone died. Of course. So I get on my laptop and I called my mom via facebook, she got off of work to watch my oldest. I called my step-father-in-law and got my husband out of work to go with me to the ER, yet again. It was then that we were sent from our closest Children’s hospital to the main Children’s hospital 45 minutes away. The resident who saw us at first, just sat in the room not talking to Trevor. She knew, from our numerous current records that something was up. That or she was just observing me to see if I was really the problem, like the previous notes probably concluded. The hospitals records showed constipation and growing pains. Plus, tons of tests that I pushed for with all normal results. However, she saw something. He slightly lost balance. She gave him a toy, but intentionally dropped it, and in his attempt to catch it, he crumbled to the ground. She called the head neurologist and got us set up with the MRI. For her, I am eternally grateful. She likely saved him, and me, today. We are sitting in the hospital waiting room, eating soggy Subway, at 11pm. First time we have eaten today. I know what the diagnosis is going to be. Guillain-Barre Symdrome. We will find out the results here in an hour. Keep Trevor in your thoughts.”

2/4: Lumbar Puncture, IVIG #1

“Moms, all I can say is stick with your gut. Trevor woke with excruciating leg pain on 1/21. Three days later, and no pain relief or cause in mind, I took him to the urgent care. 2 x-rays later and he was “diagnosed” with constipation. He had been pooping, so I was skeptical, but started a laxative. Three days later I took him to the ER. Again diagnosed with constipation and received an ultrasound of his hips, large set of blood work, an x-ray, and an enema. Three days later I took him back to the ER and I was made to look like a fool. He was diagnosed with “growing pains” and I didn’t believe it, but they made me feel stupid and like I was nuts. The next morning we went to our pediatrician, she agreed something was wrong. Immediately went back to the ER where we got another round of blood work and 4 more x-rays. The next day our pediatrician called saying everything looked normal. Then yesterday, three days later, we went back to the ER and got a full brain and spine MRI done. Guillain-Barré. I went to the urgent care, pediatrician, and ER four times. 2 weeks. 6 doctor’s visits. 7 x-rays. 1 ultrasound. 2 full panel blood draws. 1 MRI. 3 misdiagnosis. Stick with your gut moms. Be a mama bear. Make them listen even when they made you feel small and stupid. No one knows your child better than you do.”

“Accomplishment #1: We have begun our first IVIG treatment! 4 more to go. Can’t keep the smiles away for long with Trev baby.”

2/5: IVIG #2

“Lumbar puncture results are in. Confirms what we already knew/assumed from his MRI. He has Guillain-Barré. The next 4 nights will conclude his initial treatment. Further action and treatment will be decided once this first treatment is complete and his symptoms are reevaluated. Our Neuro resident is amazing. She has been here day and night, I don’t think she ever leaves. This morning she asked if I was a chemist or biologist or had some kind of medical training. She said she had never seen a parent with so many amazingly challenging questions. When I told her I had a bachelors in human biology and have been spending my nights reading scientific articles on Guillain-Barré she joked, respectfully, calling me a nerd. Then she asked what I researched last night and I told her about how IVIG is made, she was very impressed as well. Pros and cons to being a researcher by nature. No sleep, but some peace of mind. Thank you Dr. Duong!”

“For anyone wanting to come visit, hours are 9-9, make sure you bring an ID or they won’t let you in the hospital, and no children under the age of 12 are allowed in our unit due to recent flu and RSV outbreaks in the area”

“I want to say thank you to all of those who donate blood and plasma regularly. It is because of you, Trevor gets to have the treatment he is getting to save his life. You may not realize how impactful a simple blood/plasma donation can be, but I hope this message can encourage continued and new donors. Also, a huge thanks to those who are scientists and engineers within the medical community. Without you, this treatment wouldn’t be readily available for him. I love you all.”

“Accomplishment #2: Trevor didn’t have an allergic reaction to his IVIG treatment, which they said is really the only main side effect they were concerned about.”

2/6 IVIG #3, Worst day for Trevor, lost complete ability to walk, Mysterious fever

“Waiting to get yet another IV line because this one is causing him constant pain. So, ultrasound will be here in thirty minutes to place a line into his forearm so we can avoid the wrist and elbow.

Today is poor Trev’s worst day yet. Legs are worse. IV is causing him constant pain. Hands and arms are very unsteady. His stomach is bothering him. He didn’t even want to go for a wagon ride. “Formers mama” aka I want to stay in bed and watch Transformers.”

“I am not looking forward to three more IVIG treatments. Trevor’s veins are very sensitive to the medicine. After running IVIG for three hours the catheter must be flushed. First with D5 (5% dextrose solution) to avoid sediment in the line, and then followed by NS (normal saline) to avoid clotting in the line. Last night while flushing with D5 he showed some serious pain, but it ended soon. Before starting his second treatment we found that he had phlebitis (inflamed, irritated, painful vein) where his original IV line was, so he got re-stuck in his other hand. He was a trooper! No tears, because I mean, let’s be honest… The poor kid has been stuck with an IV needle seven times in the past week. Sad to say he knows the drill. However, this gave me hope that tonight flushing the line would not lead to any pain. My thoughts were that the original little vein had just been overused and had gone bad. He had had 3 days, two rounds of sedation, and 6 hours of last night’s IVIG with the original IV. That’s a lot for a little boy vein.  Boy was I wrong to think it would help. Tonight, his pain level nearly led him to vomiting. Apparently, newly stuck veins can also be overly sensitive and the nurse tried to give me hope that tomorrow’s treatment might be better. I am losing faith that his procedures will be painless.  I also got ahold of his electrolyte blood panel from Friday night, and the numbers are all across the board. I will need to speak with his physician tomorrow about his kidney function. His blood carbon dioxide and creatinine are LOW, and his blood potassium and anion gap are HIGH. I am hoping it is just a minor, temporary side effect to the lack of nervous communication and that no real renal damage will be done.”

2/7: IVIG #4, Received TONS of cards from friends, neighbors, family, and strangers. Plus two amazing packages from great friends!

“TREVOR CAN STAND!  Obviously, I was close by for falls, and walking is still very unsteady, but he can STAND on his own very well!”

“UPDATE! Trevor just finished his 3/5 treatment, and unlike the last treatment, he was never in any pain and slept through the second half of it and the d5 and NS flushes. Last night he started to heave, shake, and nearly vomited from the pain of the d5, so this is such a welcomed surprise. It is all thanks to an AMAZING nurse who advocated on his behalf and got a vascular nurse to administer a new IV line in the forearm with ultrasound assistance instead of having it in his wrist where it was constantly rubbing on a nerve. You’re so strong Trevor. Mommy loves you!”

2/8: IVIG #5, Home!

2/12

“Update: Trevor is walking very well! Still unable to do stairs, up or down. Still no reflex in the arms or legs, but that may never return. I do believe he is starting to be able to feel when he needs to urinate again, which he had lost his ability to do. He can’t stand on his own from a sitting position, but those muscles are getting stronger. We have only seen progress, so that keeps me hopeful that we won’t end up back in the hospital. The only part that is still constant is his diaphragm/stomach pain, but from my research it seems like for those impacted by abdominal pain, it takes a few weeks post IVIG therapy to no longer be in pain. We love you all, thank you for your constant emotional support.”

Wes and Michelle Russel, Regional Sales Representative of Clopay, bring us an AMAZING dinner!

2/15

“This is why everyone needs health insurance… $73,860.60.

The billed amount (so far) for Trevor’s Guillain-Barré syndrome diagnosis, treatment, and hospital stay is $73,860.60. My healthy baby boy, who had never been to the doctor for anything except well visits, came down with an extremely rare and life threatening illness. All from a normal, fever causing virus. This amount doesn’t include his 5 IVIG therapies, last day of stay, or Thor only knows what other claims will be made over the next few months. What I do know is that I am so damn THANKFUL for health insurance. We have met our deductible. We won’t have to pay anywhere near the total amount of this. This is why we pay our premiums that seem outrageous. We are a healthy family. It HURTS every month to pay our insurance premiums, but you know what would hurt more? $73,860.60… if… Republicans take the ACA away without implementing something to make sure pre-existing conditions are covered, Trevor and so many others will be without coverage for the rest of their lives.”

2/17: First post-hospital visit

“Update: We had our first, post-hospitalization Neurology appointment today. Good news and bad news. First for the good… Trevor’s strength is great. The neurologist expects a full recovery in his physical abilities. The only reason I don’t feel he has made a full recovery is because Trevor was very advanced physically before Guillain-Barré decided to tear him apart. So now, he is slightly under, if not level to, that of a normal 2 year old. As most know, he used to be able to accomplish amazing physical feats, but I have hope he will be scaling my fireplace again in no time. Now for the bad news. His reflexes are toast. Eric tells me just to give him more time, but since he has bounced back so quickly with strength the neurologist expected some hint of reflex recovery and there is absolutely none. Even the neurologist said that he thought it “highly unlikely” the reflexes in his legs would return. Apparently, in the hospital I thought both arms and legs reflexes had been affected, but the neurologist said his arms have always fired during tests so I must have been looking for the wrong type of response. Oh well, arms are fine, which is great. Unfortunately, his patellar and Achilles reflex responses are non-existent now.

What this means for the future and long run? When you step on something sharp (or hot, etc), before you even physically feel pain, your body has natural reflexes to work quicker than your brain to pull your foot back to reduce harm. Well, Trevor won’t have this reflex. If he steps on something he won’t pull back before feeling pain, he will pull back due to pain. Not a HUGE difference, but reflexes are there for a reason. That might not make sense, but you can also imagine it with your hands. You touch something hot, and before you realize what has happened you have already pulled your hand back to reduce the burn. Then you feel the pain. The neurologist said it shouldn’t really harm him, but just to make sure he doesn’t become a hot-coal walker for the circus. I told him I would try my best. If you made it this far, thanks for reading and your continued support. We love you all.”

“Trevor can crawl stairs now. Still can’t walk them, with nerve damage it is very challenging to make your hip flex, thigh raise, knee flex, and foot flex all at the same time. Especially, if any muscle weakness and balance issues still exist. We have been home for 9 days, I am so very happy for how far Trevor has come. I hope it only gets better from here. Cross your fingers for no relapses, though it is even rarer, it is still a possibility with the disease.”

2/20

“First attempt at Zika virus test”

3/8

“One viral illness.
One urgent care visit.
One pediatrician visit.
FIVE ER visits.
Seven x-rays.
One hip ultrasound.
THREE misdiagnosis.
One enema.
Two full blood panels.
One enzyme panel.
One MRI.
One lumbar puncture.
Three IV lines.
Six days admitted at the hospital.
Five rounds of IVIG.
One day of complete inability to walk.
One strong little boy.
One persistent, furious mother.
One thankful family.
One disease.
One month since discharge.

It feels as if it were yesterday, almost impossible that it was one month ago, that we were finally coming home, but Trevor has made an amazing recovery. He continues to improve. Thank you all for your continued support. We hope to never see a relapse, but only time will tell.”

3/13

“CDC blocks our request for a Zika virus test”

3/22

“61 days! It has been 61 days since Trevor’s first symptoms of Guillain-Barré. Since getting home from the hospital, sleeping at night has been very difficult for him. It required melatonin, massages, and ibuprofen. Nerve damage and pain kept him up at night while tingling and healing nerves caused restless-legs, so even once he fell asleep he would wake up multiple times a night wanting to walk around, play or just get cuddles. Eric, Cooper, and Cali sleep like rocks, so it didn’t upset anyone, but I was waking up to an almost “newborn” like sleep schedule. However, finally, for the past three nights, Trevor has not needed anything besides his sound machine to fall asleep AND stay asleep at night. He occasionally will complain of his legs, stomach, or back hurting him during the day, and, unfortunately, that is to be expected still. But, it is becoming less and less frequent. Nerve damage causes a lot of pain, and nerves take much longer to heal/regenerate than other parts of our bodies. He still has not regained the reflex responses in his patella and Achilles tendons, but like I’ve said before, many people with Guillain-Barré lose them for good. He is getting stronger by the day, and the part that allows me to be able to sleep at night is that he doesn’t even realize he is, or ever was, sick. That his OWN body attacked him and tried to paralyze him. He and mommy had a weird vacation at a place with lots of needles. He has no fear of doctors or hospitals, thankfully, but the kid’s fear of needles now is very apparent. Getting vaccines or blood drawn before didn’t so much as bring a tear to his eye, but now he sees the syringe and it is game over. I’m hoping this will pass, but it isn’t the end of the world either. Trev-baby is alive, running, playing, and finally SLEEPING. Sorry it’s been so long since my last update, thanks for reading this far!”

4/3

“Update! Trevor had a doctor’s appointment this morning, and there is a SMALL hint of reflexes returning in his patellar tendons! We will go back to check on them again in a couple months, but this is amazing news. Today marks two months since his Guillain-Barré diagnosis! Today is also World Party Day! So party with us and celebrate Trevor’s amazing GBS recovery!”

4/27 Return of reflexes!

Strange Fact about GBS

FDR likely had Guillain-Barre Syndrome, not polio

More updates to come! Thank you all for your continued interest and support in Trevor’s Guillain-Barre diagnosis and recovery! On social media, follow #TeamTrev for pictures and videos!

Love,

Nakai

Source of information: NIH, NINDS, https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Guillain-Barr%C3%A9-Syndrome-Fact-Sheet