To Surgery or Not To Surgery (Long winded Question - Sorry)

My 3 1/2 year old boy has VWD Type 2B and has had frequent nose bleeds since he was 2yrs old (approximately every 4-10 days). During this time he’s been to the ER twice for the nose bleeds. We’ve done everything recommended; including humidifiers, saline spray, AYR or Aquaphor gel up the nose, keeping his nails cut short, etc. He takes Amicar or tranexamic acid to help stop the nose bleeds. He is also on regular liquid iron for 3 month intervals 2x a day. 95% of the time he gets the nose bleeds at night when he is sleeping. We recently met with a pediatric ENT to discuss a possible laparoscopic cauterization surgery. Even with his trips to the ER he has yet to be cauterized. The surgery would hopefully help with the frequent nosebleed problem BUT they say it’s not guaranteed to work and may do absolutely nothing to stop the bleeds. So the question is – do we take the chance and do it? Or continue life as we know it. To note: my fears for life as we know it are that: 1 - because he gets the nose bleeds at night and likes to sleep on his back – he will choke on his own blood or vomit (since swallowing the blood makes him throw up) 2 – How safe is it for him to be on a regular regimen of all those meds at the age of 3, what will that do to his liver or kidneys in the future 3- because of the night bleeds and lack of sleep, he is miserable from being tired and drained during nose bleed days and his temperament is terrible (even for a 3 yr old). HOWEVER – my fears of surgery on him and then it not working even out to my fears of no surgery. Any advice? Anyone been through this before? Anyone know of any statistics on the surgery working vs not? I, myself have VWD 2B – and didn’t get the nose bleeds – I had all the girlie issues instead, so I am at a loss and doctors don't want to give me a definitive yes or no to do it.

My daughter has type 2 VWD. She is 14 now and has had frequent nosebleeds since she was about 2 or 3 . We had had many emergency rooms visits so she could receive Humate to stop the bleeding. It was really awful. Like your son, bleeding often happens in the middle of the night. When she was about 3, she had her first cauterization surgery. It worked and was good for about 6 to 12 months or so. Ever since then, it’s on and off. We go back to ENT often and she has cauterization done regularly. It would work but bleeding would return at some point. We learned to do Humate P infusion at home and it’s much better so we could minimize her blood loss as much as possible. It’s an on- going issue for her. She recently had a terrible nosebleeding (chocking on blood clots) and we were very surprised because she has been receiving two Humate P infusions per week and was doing well.

I will say in her case, cauterization definitely helps. I would not hesitate to do that. Minimize blood loss is very crucial. Does Stimate or Humate P work for him?

It’s an on - going issue for my daughter and it’s not easy. We also have the period bleeding issue too. I guess we learn to take one day at a time. For us, cauterization and able to do Humate P infusion at home help a lot. We still struggle with it but we manage it.

Thanks May! This is helpful. We do have Humate P in our fridge. Insurance just approved us to get it and now we're waiting for the doctors to show us how to infuse it at home.

I'm sorry about your daughter, I can't imagine having both nosebleeds and menstrual issues as a teen. I also have Type 2 VWD, but never had problems with nosebleeds (knock on wood), but remember what it was like those first few years as a teenage girl. It wasn't until I was put on birth control that I got the bleeding under control (for the most part - with very infrequent issues), and they didn't even know I had VWD at that time. I thought I was just weird. My 1 year old daughter just tested for Type 2 as well, so fingers crossed she is more like me when it comes to bleeding (no nose bleeds).

I have noticed - through the years with myself and my son that Type 2 can be very unpredictable at times and we have many years left to go through this. However, a positive outlook for your daughter is that it does get better with age. Never perfect - but better.

May said:

My daughter has type 2 VWD. She is 14 now and has had frequent nosebleeds since she was about 2 or 3 . We had had many emergency rooms visits so she could receive Humate to stop the bleeding. It was really awful. Like your son, bleeding often happens in the middle of the night. When she was about 3, she had her first cauterization surgery. It worked and was good for about 6 to 12 months or so. Ever since then, it's on and off. We go back to ENT often and she has cauterization done regularly. It would work but bleeding would return at some point. We learned to do Humate P infusion at home and it's much better so we could minimize her blood loss as much as possible. It's an on- going issue for her. She recently had a terrible nosebleeding (chocking on blood clots) and we were very surprised because she has been receiving two Humate P infusions per week and was doing well.

I will say in her case, cauterization definitely helps. I would not hesitate to do that. Minimize blood loss is very crucial. Does Stimate or Humate P work for him?

It's an on - going issue for my daughter and it's not easy. We also have the period bleeding issue too. I guess we learn to take one day at a time. For us, cauterization and able to do Humate P infusion at home help a lot. We still struggle with it but we manage it.

Meredith:

I am a 60 year-old male with VWD Type III. I have suffered nosebleeds my entire life, have been in the health care system dealing with sometimes help and often lack of knowledge of how to treat VWD. I contracted HCV through cryoprecipitate in the 1980s and had chronic Hep C for 25 years until developed end stage liver disease, got a liver transplant in 2012 and cleared the virus with Sovaldi in April 2015. I can only give you my life experience, and I hope it is valuable.

Over the years, the HCV was scarring my liver, and my spleen began to destroy bleed cells. At the point of the transplant my platelet count was around 20-30k and I was having uncontrollable bleeds 4X week that required self infusion of Humate P. Nosebleeds, gum bleeds (from simply eating crusty french bread or tortilla chip), GI bleeds, etc. In the years leading up to the transplant my bleeding steadily got worse. So I've tried lots of things to stop bleeding and prevent it. I will give you my advice:

I have had my nose cauterized about 2-3 times. Each time, it stopped the acute bleeding, but the scarring from the procedure caused chronic nosebleeds after a couple of weeks. I personally avoided cauterization because of this. I would only let an ENT do it very very gently and just a tiny bit. And then keep my nose moistened and lubricated with vaseline or bacitracin with aloe. I think the key to stopping the bleeding is to heal the skin and not let it get irritated. Get a whole house humidifier that will maintain a particular humidity in the house, especially in the winter, or in California, when the Santa Ana winds blow off the desert. Also an ultrasonic humidifier (cold air) in the room where the person sleeps. And vaseline or Ocean spray regularly. No picking noses!

Secondly, when a nosebleed starts, NasalCease is a godsend. I have labels from nearly 50 boxes I used over the years (you can redeem them for product). It is a fiber that turns to gel and doesn't irritate the mucous membranes, and when you finally remove it, it doesn't tear off the scab or clot. This is key to a platelet disorder. You have to get it stopped up at the surface so the platelets can agglutinate. I used to joke that I was the poster child for nasalcease. Its cheaper to buy it bulk online and you can put some in the glovebox of the car, in your backpack or purse, etc. I kept it at work, everywhere. Also icepacks on the forehead helps, and pinching up as high on the nose as possible.

Finally, I used Humate-P as the last resort. It will stop it. Learn to self-infuse or infuse it yourself. I know it's difficult, especially with a child, but when its the weekend or you are somewhere far from an ER. And if there is a hemophilia center that is partnered with the Cancer infusion clinic, the nurse coordinator can get you in and have the infusion nurses give it.

So I would tend to stay away from surgery, cauterization, etc. As the docs say, there is no guarantee, and it may well make it worse after making the short-term acute bleeding stop. Like a lot of medical interventions, the intervention often causes a cascade of "side effects" and long-term issues. I'd try NasaCease first, with humidity, keeping the nose moistened, vaseline, etc. You want to heal those inflammed, tiny capillaries (with VWD often the vessel is so small they cannot find any clear vessel source. It can be a small area.

I hope this helps,

Joel

I would also recommend trying Ponaris nasal emolient on a daily/twice daily basis. I also agree that avoiding procedures is the way to go, generally. Most medical practitioners understimate the problems that their monkeying around can cause (except for the rare hematologist that truly understands vWD, most hematologists know more about oncology than bleeding disorders) Nasalcease sounds like a good thing too... Good luck to everyone with this. Almost 20 years old, this compilation of ER and ENT "pearls" of emergency nosebleed treatments is still worth a look, sometimes old school is the way to go. I found a cached version and made a tiny url for it, hope it works for every one: http://tinyurl.com/p6czzbw

Thank you everyone for your responses. We decided to be a lot more aggressive with our proactive approach in order to avoid surgery. We purchased a super duper humidifier and keep it on high right next to his bed, so that he’s almost soaked from the humidity every morning. My only concern now is that we’ll get mold in his room, ha ha. We also squirt his nose with saline every 2 hours instead 2-3 times a day. So far (knock on wood), it’s helping. His nose bleeds are much more infrequent and less severe. So we’re going to keep this up and see what happens. Thank you everyone for your suggestions.

On a side note: We have been incredibly fortunate with having our hospital/hematologists being out of Johns Hopkins Medical Center. The pediatric hematology department there focuses only on pediatric hematology and our doctor specializes in VWD. Same with the pediatric ENT. I think that if my son needs surgery in the future, I’m confident in my doctors – but I’d rather that we avoid surgery until he’s older if necessary (and hopefully not at all). If anyone is within a reasonable drive to Hopkins, I recommend and promote them. Aside from the amazing care, they have an advocacy group that is currently helping us with the issues surrounding children’s’ liquid Amicar. If you are not aware – the only company that makes the generic version of the liquid Amicar decided to discontinue it due to a business decision, so the only available liquid version is the named brand which costs $2100 for a 263ML bottle. What makes it even better is that my insurance doesn’t cover the liquid version, just the pill form – and it’s been impossible trying to get a 3yr old to swallow a pill. Hopkins has been appealing this decision on my behalf. Whether or not the appeal gets approved, I can’t thank them or promote then enough.

Hi my daughter also suffers and has done 4 about 5 yrs now, she will b 12 in a couple of wks. She has surgery on both sides of her nose which sorts it out 4 a couple of months then we r bac 2 square 1 again. They have now given her a cream called naspitene, think that’s how u spell it. Which again helps 4 a while. I think it’s something they just grow out off as my sister did and she has the blood condition aswel.

My nephew has type 2 vwd and suffers nose bleeds. He spend the main part of his life in hospital from nose bleeds that was so bad he even ended up in an induced coma. He went through caurtization and Id like to say it completely stopped his nose bleeds but unfortunately it lasted a month tops and he was back to square one. Ifind it highly important to mention when he was 2 and his bleeding got completely out of control doctors decided to insert a port a cath. Its hard to explain but it feels like a bottle lip cap above his chest which goes straight to the blood stream. His mum then gives him his factor twice a week and touch wood he hasn’t been in hospital from a bleed since. He has the odd bleeding but his life more free now his mum can just preform his factor rather than waiting for it to get worse and the whole hospital experiebcem. It’s taken her allot of emotional stress and training to do the home care but its defo something to consider especially to take the stress of nose bleeds and hospitals away. Hope this kind of helps.