50/50 chance of PBP

[rocmg]

dear all. i met with my mum's neuro today who said she had a 50/50 chance of having PBP -- or some bulbar ALS, because of her clinical presentation. he said her particular speech presentation was most often associated with ALS and he said there is no other possible differential diagnoses apart from maybe a stroke that went undetected by the MRI. he said other neurologies do not have this particular bulbar presentation when i asked about other neuromuscular disorders -- hoping for something treatable.

i couldn't bring myself to tell her all of this when i came home today. she refused to come with me to speak with him -- to ask the questions that should have been asked back in October, when she was similarly too scared. she is petrified and crying all the time. i couldn't bare to look at her little face and tell her that the only thing giving us hope that this is not ALS is the fact her speech is getting better -- but to me, i see no change in her speech. i see it getting no better and no worse. i dread the day if it gets worse. i really think she will try to kill herself if this is diagnosed -- my dad has the same fear. she is a very nervous, flighty person and prone to depression. and i love her so much because that's who she is.

i just told her that the neuro was helpful -- he didn't tell us she didn't have it but was optimistic that it could be something other than ALS.

i read often read BethU and rose's posts on here and know that you both have this particular variety of ALS.

i'm really devastated. i just need advice on where to go from here? do we go for a second opinion? should i give up my life in america and come home to be with her? i know no one can answer these questions definitely, but any advice is appreciated.

 

[rose]

My heart really goes out to you and your family. Like you said, there really are no easy answers to come from anyone here. We can't know what is the "right" thing.

Most importantly, its got to be a change from the inside of your dear mum herself, as to how she will cope with upcoming changes.

If it is possible for her to take the new drug they've been using for emotional lability, that might possibly help her. I don't know if it is being used yet in the UK, and don't have the name of it (but BethU will). Or of course an antidepressant, but if she can get the new meds, I think they would do more for her than just zoloft or the likes.

For you to be willing to give up a life that you worked for, is a testament to your love for her, but even if she does have the PBP, which it sounds more and more like she does, how is one to know the rate she will progress at, at least until she has more history with it.

I can say that I will always wish I'd spent more time with my dear daddy before he passed. (he did not have MND). I never knew until after he was gone how much I would miss him. ~ and how much I could use to have him around now, but me having what I have would have devastated him, so its best this way.

Maybe you can just sit tight and give it all some time, and see how things go. No major changes for a bit. If she's not having huge issues with eating, or other signs that the progression is aggressive, some time may be just the answer to put thing into perspective.

She may claim she doesn't want to know, but perhaps you can gently remind her that the fear of anything is usually worse than what the fear is of. Knowing the enemy is much better than not.

good luck to you!

 

[BethU]

Hi, rocmg ... I am so very sorry for what your mom is going through ... and what you and your dad are dealing with, too. It is at least as hard on the caregivers as it is for the patient.

The medication I am familiar with for emotional lability, which is often a problem with bulbar involvedment, is ZENVIA. It is already on the market for treatment of diabetic neuropathy, and any doctor can prescribe it. My neuro told me it affects the whole bulbar area, which is where emotions originate, as well as speech and other activities. I have been taking it in a clinical trial since last fall, and have had very beneficial effects.

My experience is that if you can help control even one symptom of ALS, that is a battle won. Ask her neuro about it, or about other means of helping her emotionally. When she is less distressed, it will help your dad, too. It is SO hard to see a spouse or parent/child suffering.

Even if it is ALS, your mother can continue to live a full and vital life. I'm almost at my third anniversary with bulbar onset. There are physical restrictions to be managed, but I'm still here and having a pretty good time.

All that said ... I would get a second opinion. "50/50" chance is not good enough when you're talking about such a serious condition. With something like ALS, you need certainty. I'm sure your mom's neuro would understand that it is essential to have a second opinion. Where do your folks live? A university clinic is the best bet. For your own situation, I wouldn't make any major life-altering plans until you have a DEFINITE diagnosis. I'm really puzzled by your neuro's statements that there are no other diseases that present with these bulbar symptoms ... My impression is that a variety of diseases can cause "disarthria," which is what ALS speech impairment is called technically.

Let us know what is happening. I will be praying for you all. Keep your hopes up, and try to help your mom and dad see that this is not "the end." There is much good living ahead of them still.

Take care.

 

[rocmg]

thank you both so much for your replies. i really do marvel at your strength. i have been playing with the idea of taking my mum to America to see someone in New York about her symptoms. This waiting game is horrible, but I know there is very little else we can do. I will continue to pester the thousands of secretaries to get my mum an appointment with the top neurologist in Ireland. How many distraught daughters they must speak to every day.

I hope you both have a wonderful, healthy day. They say it is minus 12 in New York today! I hope it is not that cold in New Jersey -- and I'm sure California will be a little warmer. :smile:

god bless.

 

[rose]

rocmg,

If you're seriously considering bringing her here to the states, even though you live in NYC, you might think about a referral to Hopkins in Baltimore. There are lots of flights into there, and the drive from your home would not be bad either. We drive from here and its only about 3 hours. Johns Hopkins is tops.

 

[rocmg]

rose! you saved me from spamming the forum again! i was going to ask you (since I know you are from NJ) if you would recommend your hospital. Thanks so much for your advice. I will look into a referral. in the mean time i'm working on keeping mum's spirits up and giving her little things to look forward to. again, thank you so much for replying.

god bless.

 

[rose]

You're more than welcome. I just think if she were to fly 6 hours to get to the states for the purpose of getting a diagnosis, you might as well take her to the top. I did start out with NYC doctors, and they are excellent, but there is no comparison. Hopkins is like an entirely different world. Plus, they treat me very kindly. It seems like having at least a little compassion must be a requirement to work in their neuro-muscular department.

Your mum is blessed to have you for her daughter!

 

[mndireland]

Hi Rocmq
I cant believe that a neurologist is telling you 50/50, might be this or that? That is not an answer! I would have been a little more than annoyed with that to be honest, so I would bring her somewhere else for a definate diagnosis.
My mom was the same in the beginning and still is today, 15months on. She got the diagnosed and at the time we never thought it was als, when the doctors told her the diagnosed, she just said you die from that, as the doctors started to explain she said she didnt want to know anymore, and she didnt want to know what was coming next. This was her way of dealing with this and to this day when we go the neuro my mam sometimes says can I wait outside and you can talk to my daughter, I dont want to know. This hasnt been a huge problem to be honest, I get the info and sort the medication etc. When things started changing, like choking etc she would say to me, is this part of my complaint, and then I could re-assure her and maybe tell her what might happen also. So dont worry if she doesnt want to know whats coming next, its her perogotive.

As for moving, I agree with the others my sister still lives in Spain, whenever I think my mam is bad or needs others i call her and she comes home for a few days or weeks etc.. Maybe now might be a little early to make those big changes.

Praying for you and yours

 

[CindyM]

I am sorry for what is happening to you and your family, Rocmq. I wish you all the best as you make some of these difficult decisions. Cordially, Cindy

 

[rocmg]

dear MNDIreland... thank you for your reply. The letter from Dr. Hardiman actually came today. So we have an appointment with her on Feb. 12th. I was exploring the idea of taking mammy to America since I hadn't heard anything from Dr. Hardiman's secretaries -- one of whom was lovely, the other a little bit abrupt. I sent a request to John Hopkins in Baltimore for an appointment also. But I'm happy enough at the moment to see Dr. Hardiman and she what her appraisal of mammy might be. I was thinking about it last night and maybe it would not be fair to take her away from Ireland to hear such news -- since my whole family could hardly afford to fly out for the appointment. So I guess I will just wait and see how this all pans out. The unfortunate thing is I have to go back to America on January 29th due to visa issues, so I won't be able to go with her to Dr. Hardiman's appointment. I'm still trying to figure out of I could make it back to Ireland two weeks after leaving -- we're not millionaires and I'm only a freelance writer so my income isn't exactly luxurious. I really wish I had a sister right now. I am an only girl and I am very close to mammy -- we wear the same clothes. I guess she is the closest thing to a sister I have. It makes me so sad because she is only 50 and she is so beautiful. She doesn't even look her age. I always thought she would be around to see me married (not that there's any danger of that happening soon!) -- that she'd be there to look after my kids when my husband and I went on holidays. The stuff that mammies do when their daughters are grown up. I guess that will never happen now.

Today when the letter came it was very sad. She just saw it and her heart sank. My dad is trying his best to stay positive -- he keeps saying "give me a year, and she'll be the woman she was before -- just give me time." Yet time is the thing that scares me the most - not knowing how much time we have and what we will endure between now and then.

Thank you to both rose and Cindy for your kind words and thanks to anyone who read this for allowing me to vent.

god bless.

roisin (rosheen)

 

[CindyM]

Vent anytime Roisin. I like your name, BTW. Is is Gaelic in origin?

 

[rocmg]

it is! irish for rose, actually. my middle name is mary, which is also my mums name

thanks cindy.

 

[mndireland]

Hi Roisin, I am so glad that you got an appointment with Dr Hardiman, one of her secretaries has a real attitude problem and my sister had a run in with her before over waiting times, but I found then it got worse, so now I am overly nice to her! Dr Hardiman however is lovely, she is so chatty and asks a lot of backround info, sometimes you are thinking what has this got to do with anything, but in time I noticed that my mum loves to have her little chats with her now, so I dont mind. On her last notes she had noted that I had lost weight although 4 months pregnant at the time, and looked tired, she figured i needed more help and sure enough the nurses were around a lot, she doesnt only look after your mum but the whole family, this is very comforting for you when you are away.
She will probably admit your mom for a week to run tests, this is what happened with us, so she wont give a diagnosed on the day. So if you can return a week or so later I think thats when you might want to be here, even if the diagnosed is a good one, you might want to celebrate, keep positive! There is also a small waiting list, so expect your mum to go in on early feb for the week..... It gives you a little more time.

I have sisters but to be honest, I carry the can mainly myself, I have always had a much closer relationship with my mam that any of the others, and still do. It doesnt matter if you had 30siblings your relationship would still be the same, so all you can be is her daughter and support her at this time. I will be away next week until the 31st of Jan, but will be watching out for your posts, so be sure to let us know how you get on. Feel free to send me messages anytime.

 

[rocmg]

mndireland -- thank you so much for your message. it's comforting to know there are other people out there going through or who have went through this.

it's the waiting game that is driving me crazy. i'm not sure what to do for the best to be honest. i still have commitments that i have to fulfil in america but it feels bad of me to head back to head back and act like there's nothing wrong, and i know i will be thinking about it all the time. so right now i'm just not sure.

should i be congratulating you on a new baby!? or is it not quite time yet? either way i hope the both of you are happy and healthy.

you're so right about the whole sibling-parent relationship. mum and i have always been very close, more like sisters and we did our fair share of bickering when i was growing up. i just look at her now and what i wouldn't give for her to give me a good old fashioned scolding. but she can't because her voice has changed and she can't make those shrill sounds she used to. :sad:

I just have a couple of questions about preparing for Dr. Hardiman's appointment. I have made an appointment with our GP to request copies of all of mammy's medical records, since i know medical history is very important in the diagnosis of MND-ALS. Should I also request copies of the raw data of the EMG/Nerve conduction studies? Or would Dr. Hardiman want to do her own EMG? Also, we have the images of the MRI she got done so I will have her bring those along as well.


Should I tell mammy to pack a case for the week? Or should I get in touch with the hospital to see how we should prepare for the appointment?

thanks for your help. i truly appreciate it.

 

[mndireland]

Hi Roisin, sorry for the late reply, my computer is playing games lately. When you first see Dr Hardiman she will just discuss with you what she plans to do, and what will happen next. I would imagine after your appointment she will put your mam down for a bed which usually takes a few days, so she wont need a bag right away. I am just going on past experience, but Dr Hardiman will do the emg and all tests, she has a full team up there incl occupational therapists, speech therapists and physio's etc so they will examine her thoroughly before a diagnosed is made. On our first appointment she said nothing about what it can be and she wont until she runs tests as she will not speculate at all. But once a diagnosed is made things will happen a little quicker, so your frustration will calm down. These next few weeks is when nothing much will happen so you should think about heading back to New York and coming back mid or late feb, just a suggestion?

Thank you for the congratulations on the baby, I am almost 6months pregnant and really excited about it. Its getting more difficult to help my mam but she is really excited about the new arrival also, and just keeps saying she hopes shes here to see it. Fingers crossed. We've had a bad 2 weeks so just hoping things get a little better this week.

Speak soon