New diagnosis

My sister was just diagnosed with ALS (bulbar, I believe), and she is already having difficulty speaking and swallowing so it can be hard to understand her, and she runs the risk of aspiration when she eats or drinks. She is also tripping and falling frequently, and she doesn't seem to be able to even brace herself before landing, so she lands hard and has sustained cuts and bruises when she falls. She is attempting to maintain her strength by riding her bicycle 16 miles a day, but she has fallen on several occasions, so I don't know if this is advisable or not. What troubles me the most is that she seems to be one of the relatively few people who is also experiencing cognitive decline, and the change over a short period of time is really marked and just heartbreaking for such a vital, intelligent woman. She is her family's sole wage earner and is panicky about losing her job, and worry about losing her job is causing severe insomnia, so she has started to drink to excess at night in an effort to fall sleep and get some rest (a very counterproductive strategy, obviously). I'm just in a panic for her, because her family is less than supportive, and I live a very long way away. I've suggested that she consider selling her house and moving to a more accessible home before the need arises, but she won't leave the security of the 3-story Victorian home she has lived in for many years (I'm not sure she grasps what may lie ahead). Does the ALS have any advocates for patients who might be able to step in and help someone like my sister? She is such a wonderful person, and she has been a tower of strength to her family over the years, so this is just breaking my heart.

Welcome and very sorry.
Resources? Her ALSA chapter, the local MDA chapter and her clinic social worker. The VA if she is a vet. The strength of these organizations varies a lot with location. Where is she located?

Exercise, as discussed here often,is controversial. She will not be able rebuild muscle or gain strength. What is gone is gone. Most think some activity is good but not to the point of exhaustion. My clinic said if it took more than an hour to recover FULLY it was too much.

Cognitive issues are actually sadly common ranging from mild issues to severe dementia. FTD can really affect insight and executive function. It may take a while to separate denial and fear from other issues. Of course a three story Victorian is not the optimum place but if the rooms are large and a stairlift possible it might be workable.

You are a good sibling and it is great you want to help. Distance is hard but there are things you can do from a distance as well as during visits

Oh, Nikki, I am so very sorry your family has been so affected. My family has been severely affected by one chronic neurological disease already, so this latest diagnosis is an especially tough blow. I know that the most important thing is for me to be positive and supportive of both my sister and her young children, but it's good to be able to let my hair down (so to speak) here and express my fears for her. I will pass on your suggestion that she seek out advice from the local ALSA chapter (I'm sure there is one in Boston). Thanks very much!

She is in Boston? So am I. Does she go to MGH? They are just getting / just got a social worker at clinic. There is also a group called compassionate care that works out of the Cape not sure how far they cover. They do cover at least a good part of the South shore.

Sorry to hear your family has another neurological issue. Do not feel you have to share here and if she is in a good clinic like MGH they will be looking at this if it is a possibility. C9 orf72 my genetic defect has some neurological manifestations other than ALS

Hewitt,
So very sorry. You are right in that your sister is making some uninformed choices at this time. It's a hard diagnosis to accept and the local Associations and a true ALS Clinic now is so important.

You're a great brother. I noticed some cognitive decline in my PALS in early stages and then severely near the end. It's not uncommon as Nikki said. Might she get on this forum and might she find an ALS support group to attend as well?

Sherry

Sorry to welcome you Hewitt.

Cognitive decline is reported now in around 50% of PALS and is very common with bulbar onset. The need for her to be supported by a clinic is just huge. As you have noted, she falls and when she fall she can't break the fall and so the risk of serious injury is increasing daily.

I hope she does attend the clinic Nikki goes to as she speaks so highly of it.

I Believe she's at Beth Israel Deaconness, Nikki. I gather that her neuromuscular specialist is well-regarded, but it doesn't seem as if there was much follow-up post-diagnosis. I asked her when her next appointment was, and she had no idea, so perhaps none has been scheduled. Don't they usually follow you at regular intervals?

I did a little research and found that cognitive problems occurred more frequently than I thought, but I had no idea that the problem is so common. Her husband and I wrote off the cognitive problems we saw as early as a year ago because we thought they stemmed from side effects of another drug she was taking, and it was only when the neuromuscular problems appeared in September that her neurologist referred her for further evaluation with a neuromuscular specialist. I was totally unprepared for the regression I saw when she visited over the holidays, and we all were privately crushed, because it seems that we are losing her already. However, it may be a blessing that she is not as acutely aware of the changes happening to her because of her cognitive issues. Her closest friend has counseled me to focus on gratitude for the good times we have had together and try to be positive, and I know her arguments have some merit, but I am still grappling with my personal loss right now, and it's very hard to be upbeat. Her bravery astounds me, though. I guess we really don't know what inner resources we have until we are tested.

I just looked up her specialist, a neurologist specializing in movement disorders, and ALS was not among the clinical interests noted as being the focus of his practice. I know that it is not a common disease so that a neurologist specializing in movement disorders would probably be qualified to handle such a case, but are there clinics devoted particularly or largely to ALS? Would it be worth looking into finding such a clinic or practice for the most comprehensive care?

3 months seems standard. I am told at the end of my appointment the interval I should return and except when my visit is late and the appointments secretary gone when I am leaving I have an appointment made then and there. BIDH is a great hospital. It is not THE place for ALS in Boston though. I had a look just now at their website. Compare and contrast that with MGH. The BI team leads you to the doctor list. The MGH has a page for doctors but also nurses, pt, st ,rt and ot. It is unclear from their website how comprehensive the BI services are. In ALS the doctor is only part of what you need you need the whole team. I can't speak more about BI I don't know anyone who goes there. Everyone I know in the area goes to MGH or UMass

Cross posted. YES! Get her to an ALS clinic. A movement disorder specialist is not what she needs. Strongly recommend MGH. I do not know a bad doctor in the ALS clinic but I am going to pm you

Nikki is so right. I've dealt with both the movement disorders clinic and the ALS clinic at Duke (mom and husband). They are both fabulous, but there is a definite difference. Your sister needs people who specialize in ALS, and the team is a large one. You don't want her traveling all over town visiting specialists who will all see her on the same day in an ALS clinic.

I've seen the look on the faces of friends and family when they haven't seen my husband in awhile. I'm used to the progression, but they are always shocked. It's heartbreaking, but your sister is fortunate to have you. Not all family members are as involved.

Welcome. You'll find a great deal of information here, as well as people who truely understand what your family is going through.
Becky