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Hemangioblastoma nightmare

Jankster
  • By Jankster
  • Posted October 19, 2008 at 5:49 pm · 30 replies
  • Shared with the public
3 Recommendations

Earlier this year my husband was diagnosed with a benign brain tumor located on the brain stem about the size of a half dollar, which was removed. The neurologist told us all the risk, but said they were "rare" HA HA. Everything that could of happened, happened to my husband. He had almost no symptoms before the diagnosis and now has all the issues he should of had before. i.e. ataxia, double vision, swallowing issues, gastrointestinal problems. He currently has a tracheostomy in place, on oxygen all the time, very ataxic, numbness, tingling in all extremities, low blood pressure, central sleep apnea, and the list goes on. He was told he had VHL, which wasn't diagnosed from blood work, it was diagnosed from the location of the tumor and biopsy. We spent 4 months at the University of Colorado and have been home almost 4 months now. Everyday is scary for us, especially me. My main concern at this time is the sleep apnea. He's on a Bipap machine and still cont not to breath at times. Anyone who has experienced these symptoms or anything similar please write. Thank you

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Explore topics in this journal entry and replies:

Exercise Cancer Surgery Pheochromocytoma Ataxia Pneumonia Sarcoidosis Stress Brain tumors Sleep apnea CPAP Kidney cancer Hemangioblastoma Pain Weakness Edema Speech therapy Depression Paralysis

30 replies

beccaloo

First let me say I really feel for you and your husband. My husband and I went through about 2-3 years of just pure misery (not to the extent your family is going through though) when I was diagnosed w/VHL. By the time all was said and done I'd had 1 spinal cord surgery, 3 surgeries on my brain stem, 6 weeks of radiation, and part of my right kidney removed b/c of cancer. Stay strong. You and your husband will get through this! I can't imagine what you're going through b/c like your husband I was the patient. Like I've told my husband David and others, it's so much easier for me to be the patient than the caregiver. I'm pretty strong when I get sick but let David get the sniffles and I'm a wreck! When someone I love is sick it's a thousand times harder b/c I feel so helpless. There is something special about folks like him and you. I truly don't know how you do it. So even though we've never met, thank you for all you do b/c without you and others like you, folks like me would really be in trouble!

Anyway, I had a walnut sized hemangio removed from my brain stem when I was 18 and the pathology lab missed the VHL diagnosis. Then 8 years later I had another hemangio removed from the same spot: my brain stem, and the path lab got it right and diagnosed the VHL.

I had no symptoms w/hemangio #1. With #2 I started getting dizzy and around Oct. By the beginning of Jan. when I had the surgery I couldn't walk (too dizzy), had uncontrollable hiccups to the point that i could barely talk, and couldn't swallow. I was basically on a pudding, jello, mashed potato and otherwise liquid diet over new year's b/c we had to wait until after the holiday to have surgery. It sounds kind of similar to some of the stuff your husband's going through. I know that for me, the reason I was having trouble swallowing and hiccupping was b/c the brain stem controls parts of the autonomic nervous system. Basically it controls the stuff you don't even think about like: digestion, breathing, blinking, and swallowing. I'm not trying to talk down to you so please don't take anything medical I say here that way. I'm not a doc, but I've always been kind of a biology/anatomy person and so when I was diagnosed I hit the books!

I agree that PT, OT, and CT (physical, occupational, and communication therapies) would probably help A LOT! It's also going to take time. After my last surgery I could walk maybe around inside my apartment. That was it. Now it's been about 6 years and I function almost normally. I still get the hiccups. Even in my sleep from what David says! And I wouldn't pass a roadside sobriety test: I wobble. I can't lift heavy things, over 5lbs is really tough and 10's impossible. I also just plain hurt some days and I stay either on the couch or in bed. I'm on enough pain meds to kill a herd of elephants but w/o them I'd never get out of bed. I also started going to the local YMCA and walking on the treadmill. When I started I could do maybe 15 min. at 2.2 mph and now I'm up to 30 min. at an avg. of 2.7 mph which is almost a 20 minute mile. Sure I have to hold onto the bars on the treadmill for dear life b/c of the wobbles, but I've noticed I'm getting a little stronger, a little bit more stable, and a whole lot more confident.

Whew. Another long post. So don't loose hope and try to just stay the course. It'll get better but it may take some time. You're on the right path in that educating yourself about VHL. Every case is different, but that's the best reason for us to share our experiences. I'm sorry to have talked your ear off but I hope I've helped a little. Oh, and one last thing. I'm not really a religious person, but sometimes when I'm having an off day or a bad day I try to keep telling myself that God never gives us more than we can handle. I also hear my mom saying to me, "You can do it Becky. You're made of stronger stuff." and I think that's true. We ARE made of stronger stuff! Good luck and good health to you!

Jankster

Thanks for you thoughts Gale. The neurosurgeon has told my husband everything should come back, just don't know how long. As far as the breathing issues it's a combo of everything. He went into respiratory distress twice while in the hospital, then they finally got smart after me pushing the issue and put him on a bipap machine. Back at home after the hospital stay we had a sleep study and discovered he has central sleep apnea, which I knew all along. I kept telling them in Denver he stopped breathing at times. He also had pneumonia in the hospital and his vocal cords are fixed together, which wont allow him to fully get the oxygen he needs. Any activity makes him short of breath. We see the pulmonologist in a couple weeks and hopefully he can shed some light on all of this. Thanks again. Are you or someone you know going through this?

gale

Jankster,

I was diagnosed 32 years ago. I am the Florida chapter chair for the VHL Family Alliance and I oversee the Southern Region, so I have talked to a number of people, besides family members.

In 1997, I finally received my degree in Natural Healing and I have been searching for information and receiving training since.

I consider myself a bean counter so I tend to look for patterns in many of life's issues.

I also requested my husband (non VHL) to have a sleep study test. He actually went to his doctor and told him "my wife said to get a prescription for a sleep study, she believes I have sleep apnea." Of course the doctor asked a number of questions including if I was a nurse. My husband explained that I am a "professional patient". After explaining himself, he received the script for the sleep study. They found that he stopped breathing 37 times an hour. He is now on a CPAP as well.

I can actually pinpoint some things that make his breathing worse. Of course he doesn't want to believe me, but he has made changes with his behavior, so he is coming around...slowly, but he is doing great.

Why are your husband's vocal cords fixed together?

Gale

Jankster

Most likely due to the number of incubations and exubations. (4) hopefully that is all. the brainstem has a nerve that controls that area, so hopefully it's not damaged because of that. Thanks for your reply.

Sha

Kberri310

My 12 yr old son had a brain stem hemangioblastoma removed last year. The tumor was 3cm, wrapped high around the medulla. It caused cranial nerve damage removing it. We spent 3 months in the hospital, 1 month inpatient rehab, and about 4 months outpatient rehab. He still requires a lot of care. He lost his ability to swallow for 10 months. He has a PEG feeding tube. He is taking solid food by mouth now, just no liquids or meds. We have been doing a therapy called Vital-Stim to help strengthen his swallowing muscles by stimulating the nerves. He also has other cranial nerve damage like your husband. He gets double vision, which is starting to get better after 1 yr. He also has CENTRAL sleep apnea(not obstructive)due to cranial nerve damage. He uses oxygen via a nasal canula whenever sleeping. He still stops breathing at night. The oxygen is enough to keep his pulse ox in the 90's. The doctor told us never to use CPAP or BiPAP on him because he doesn't have the phryngeal sensation to feel the secretions that would be blown into his vocal cords under the high pressure. I think this might be different for your husband with a trach to protect his airway. I don't know, just something to think about. My son has frequent silent aspirations. They think most are from reflux. He gets very sick fast with a high temp, high heart rate, and requires oxygen to breath until the temp is in control. In the begining his B/P was very low, especially standing up. This went on for months. He couldn't do much of anything without getting so dizzy, couldn't see, and would fall down. Dr's finally started him on medication. It really didn't help. It took about 7 months to get better on his own. Now he has high B/P most of the time. Sometimes it will still go very low. When he's tired or sick his gate is ataxic. He can't tolerate running, standing long, or warm temperatures. He is getting better, it's just so slow. I'm so grateful to still have him in our life. God bless you and your family. Please know your not alone. It helps talking with others in similiar situations.

Jankster

Wow. Someone who has most of the issues that my husband has...I am so sorry that it is your little boy, it makes it so much more difficult when a child is going through it rather then an adult. Was your son diagnosed with VHL? Anyone else in the family have it if so? In the pic of me that is my little boy on the left (with the blonde hair) he is 10 and we are so afraid this could happen to him. My husband keeps saying we need to have him checked but I am afraid of the outcome. If he does have a tumor what if he ends up like my husband. Im not sure I could handle two of them:) What kind of symptoms did your son have?

That vital stimulation was a god send for my husband. While he was in inpatient rehab thats when they did it. By the time we left he was on nectar thick liquids, he could drink water though. They said that wasn't as dangerous if aspirated. He could eat almost normal stuff. At this time he is on a regular diet, whatever regular means, I guess stuff like you and I eat and he can drink anything. Unfortunately when we left the hospital our community doesn't have a licensed vital stem person so he couldn't get anymore treatment. He did con't with speech therapy exercises and they helped. He only had to do that about a month if that. We are so thankful that he was able to eat again, since they told us it was a possibility he wouldn't. Food is a big part of our lives, enjoying cooking together (my husband is a great chef) he can't really do much anymore, although he thinks he can. OT is working with him on that so maybe he will be back in the kitchen before we know it!

I have read some articles about controversy with the bipap and cpap machines being used on central sleep apnea, but it is our safety blanket. In Denver before he was diagnosed he went into respiratory distress twice. I actually found him dead and they called a code. The second time we were on the rehab floor and he was acting out a dream, when i flipped the lights on he was blue and foaming at the mouth. Once again a code was called. They said it happened from a mucous plug in the trach but that was a lie. When the respiratory therapist suctioned him there was NOTHING there. What happened was his CO2 levels got too high again. Since he stops breathing he don't blow it off like we do. There is a lot of factors with this but please if you dont already...keep up on your sons lab work, especially the co2 levels. My husbands are still above the normal range, but I think since he cont to stop breathing it is going to be like that. We are seeing a pulmonologist next month so hopefully he can shed some light on this and help. There aren't many treatments for CSA but we never know. My husbands apneas are also very postional. When he lays on his back they happen more. No one is sure why, but they suggest he lays on his side. As far as the secretions...I hear them and get up to suction him, sometime they wake him up, we are more concered with them getting thick and clogging the trach, that is our worry.

I have gone on and on haven't I? I will write more later, please let me know if you have any questions. It is so nice to hear other stories to let us know that we are not alone. GOD BLESS you and your family.

Sha

lifeandhope

My Dad had brain stem surgery five months ago and has been in the hospital ever since. He gets to go home tomorrow (yeah!) but he's still got a very long way to go. He has a trac, oxygen, difficulty walking/coordination, and he cannot swallow, so he has to do suction constantly. We are also in Colorado. Have you tried Craig Rehab Hospital? They are one of the top ten in the nation and they have helped Dad a lot. He'll continue his therapies on an outpatient basis there.

Jankster

Thanks for you comments. I wish your dad the best. Why was his hospital stay so long if you don't mind me asking. Where did he have the surgery done? Yes we tried getting into Craig when we were told that we were being kicked out and they told us a brain tumor removal was not considered traumatic brain injury. My husband is doing much better now, almost able to walk completly on his own and we are still doing outpatient therapy here in Nebraska. Good luck

Kberri310

Hi Sha,
Your son is beautiful. I have VHL and so do both my kids. My son James' only symptoms were nausa. He was being worked up by GI and they discovered he was lactose intolerent. Dr's were happy to leave it at that. I knew something was wrong and he was loosing weight. I asked for an MRI of his head to check for a brain tumor given my history of VHL. They talked me out of it at first, saying I was overeacting. I took him to another Dr. who did more GI tests. This Dr. also said we didn't need an MRI. I couldn't understand why this was such a big deal. I wasn't asking for surgery to be performed! I ended up saying to the Dr. that if he didn't order an MRI of his brain, I was taking James to the ER with complaints of the worst headache of his life. So he ordered it! Thank God someone finally listened. His tumor was 3cm on an 11 yr old brainstem. It turns out James had another symptom of double vision. He never told anybody because he thought this was normal.
I found out that I had VHL a few yrs ago. I have been dealing with my own issues; brain tumors, eye, kidney cancer, and most recently a pancreatic tumor. Because of this stress and recovery from operations I choose not to do the DNA testing for VHL on my kids. This was a huge mistake for me. I live with guilt and regret every day thinking things would have turned out differently if we caught the brain tumor sooner. I was so afraid to find out if my kids had this too. The Dr's told me to just have their eyes screened for hemangioblastomas because of their age. That's what we did. There was nothing at the time so I felt relieved. DNA testing has been done on both kids now. My daughter is 15 yrs. The only things she has right now are small kidney and pancreatic cysts that we will watch with MRI's.
I was wondering if you had a pulse ox. at home for monitoring the oxygen saturation in your husbands blood while he sleeps. The alarms can be set if it goes too low and he needs suctioning or has trouble breathing. This has helped us so much. Good luck.

Jankster

Berri- thank you for the nice comment about our son. Yes I do have pulse ox. I spot check him at times during the night and he stays above 90, but he also has oxygen that is hooked up to the bipap machine. When we had our sleep study done they tried to turn the O2 down and his levels dropped. 3 liters is what he needs at night. I think the fact that he had bacterial pneumonia in the hospital, his lung function isn't the greatest. Also the fact when he breaths the vocal cords don't open so he can't take a "normal" breath. That part is getting better though. Before, he would cap his trach and try to suck in air and you would hear what they call a stridor. It was bad. Now there is hardly any stridor at all. We seen the voice box lady next month and hopefully she will be able to remove the trach soon. They will have to release the scar tissue on the cords and probably some other minor stuff. She said his chances were about 50/50 whether it would work or not.
Don't beat yourself up over not having you kids checked sooner. You were listening to someone u trusted. A doctor. Most of us would of done the same thing. I know it's a crappy saying, especially in times like these, but everything does happen for a reason although only the lord knows what it is right now. This experience has definately brought my husband and I closer together. He has been able to spend more time with our son. We have to find the positives in this mess somewhere. Right? I am sorry that your health isn't the best, I too suffer from a disease (called sarcoidosis) that makes it difficult at times to care for Jim (my hubby) so ironic we both have James's in our family. My husband wanted me to ask you if you don't mind telling us where you live. We are in Nebraska. He told me to also tell your son to hang in there, he is scared at times also.
Well I have taken up enough of your time...gotta go get the little one up for school. Take care. O' you ever heard of the serinity prayer? No, im not a really religious person but it has helped me on those days that I want to pull my hair out. If you want it let me know, I will write it down for u.

Kberri310

Hi, My family and I live in Rhode Island. We receive medical care for VHL in both RI and Boston, MA. Massuchusetts General Hospital has a VHL clinic which is about 1 hr away. I find it very scary how many Dr's are unfamiliar with VHL disease.
I would love to read the serenity prayer. I think we can use all the help we can get. My son James has been very interested in hearing about someone else who is fighting a similiar battle. He wants me to say "Hi" for him and to hang in there too. Thanks for listening to me and sharing your story. -Kerri

lifeandhope

Dad had his surgery done at NIH in Maryland; they didn't even expect him to survive the surgery, so the fact he's even here is miraculous. They transferred him to Craig so he could be closer to home. I'm not sure how he got in; the folks at NIH took care of the transfer and the transport. Dad is still in the hospital because his recovery is so slow; actually he's going home today.

joyceg98

For the serenity prayer, see
http://www.cptryon.org/prayer/special/serenity.html

I should say for most of the folks reading this discussion that these are UNUSUALLY DIFFICULT cases. This is NOT the normal course of this kind of treatment. So don't read this and think this is what will happen to you. It MIGHT happen, IF the set of circumstances you were facing is just like these very difficult cases, but hopefully it will not.

If you are facing a difficult brain surgery, here are a few pointers:

(1) are you comfortable that you have the right surgical team on the job? If not, never hesitate to get a "second opinion" from a doctor with greater experience in VHL, anywhere in the country. You want the best thinking on your case. A second opinion from an expert can serve as coaching for your local doctor.

(2) microsurgical techniques are not just a matter of skill and experience, they are also to some extent dependent upon technology. Are you in a place where your doctor has the tools available to do the best possible job for you? Have this conversation with them quite frankly. They might frankly tell you that they don't have the tools available locally, and that you would be better off going to a large teaching hospital, either in a nearby city or a longer trip away.

(3) I know it is inconvenient and somewhat frightening to have surgery far from home, far from your primary support group. However, this is not about the convenience of your family, it's about the best possible outcome for the rest of your life. It's an investment in making sure you will be well and having fun with them 20 years from now. What is the best way to achieve a good outcome for your long-term health?

Hemangioblastomas of the brain and spinal cord represent TWO (2) percent of all the brain tumors there are. For that reason, every surgeon has limited experience with them, except doctors at centers where they do a lot of VHL research. You need the best VASCULAR NEUROSURGEON you can find, someone with experience dealing with the blood vessel structures of these delicate areas. Even if the doctor does not know VHL per se, this expertise will help you find the right level of training and skill for the job.

Feel free to ask other questions.

Best wishes,
Joyce

Jankster

Thanks for the advice Joyce, but its a little too late. Maybe in the journal I didn't say surgery...thought most would of guessed from all the issues, of course I guess people could have these issues before surgery too. Anyways its too late now. Damage is done. Now we are faced with another tumor on the inferior vena cava, although no one really wants to address it at this time. They say if it's not causing problems to leave it there. Who really knows that it isn't already causing issues. Anyways thanks for taking the time to write.

joyceg98

Yes, we can't go back. I felt it important, though, to speak to other people who may still have time to get some better advice before their surgeries.

As for what to do about the current quandary, please feel free to write directly to me at director@vhl.org and we can try to get you connected with an expert team who can give you some good advice. It's always a good idea to get another opinion since the situation you describe is so uncommon. Let's see if we can find you someone who has dealt with this situation before, and successfully.

We can't re-play the previous surgery, we can only move forward -- hopefully with better success than before.

All the best,
Joyce

diaryofageneticdefec

my father has had very similar issues, he is slowly crumbling away and is not the man he was 3 years ago. Our Prof still says there is hope of a spontaneous recovery. interestingly the problems are most likely caused by a complication of surgery and not VHL.
The sooner they find the pill that will shrink tumours the better.

Jankster

I am so sorry to hear about your father. How old is he? Hopefully the doctors are right about the recovery. I wish for the same with my husband. All of the docs say everything should come back with time, although how much time is unknown. We are almost a year out now and he still struggles with everyday things. Best of luck to your father and your family.

Sha

diaryofageneticdefec

hi again
dad is 59, the issue with him is that he doesn't have as much fight in him as he once had. We keep finding things for him to focus on, he just did a 'trip of a life time' being in a wheel chair didn't make it quite as memorable as he and my mum had planned but they did it anyway. Next thing on the list of things you have to keep going for is my wedding. So we will do it sooner than perhaps we thought but I've always expected him to walk me down the aisle and do a funny speech, he'll try both, i hope, we hope. All we can do is hope.
My thoughts with you guys

raekel

i have vhl and have had a similar experience. in 1997 i had conventional surgery on a brainstem hemangioblastoma. part of it could not be removed and in 2006 scans revealed that it was rapidly growing, but i had no symptoms. i was told i was a good candidate for cyberknife surgery so i underwent cyberknife surgery for the brainstem tumor in august of 2006. all looked well for the first 5 months and i returned to work. unfortunately, by the end of december, i began to experience bizarre sympioms--numbess and tingling in both legs and feet, with increasing weakness and eventually an inability to walk and inability to swallow. my blood pressure was so lowi i was unable to sit or stand and i eventually experienced respiratory failurenow i have a tracheostomy and gi feedeing tube w3hich i have3 haqd for a year. i have learned that all of this was caused by late effect radiation edema which causes damage to heaqlthy tisswue around the tumor. symptoms can persist for up to two years and if they persist longer, the damage can be permanent. after having waitied 2 years, my doctors are reviewing ny latest scans to see if open surgery can help. i think its interesting to learn that your husband experieced similar problems from open surgery and not from the cyberknife. i really want this trach and gi tube removed. anybody else have a similar experience?

raekel

hi jankster,

how is your husband doing and how many months post op is he now? you did say that his doctors told him everything will come back but did they say when? i've had a trach for two years now but have been unable to eat. anybody had their trach removed yet? thanks, raeanne

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