ALS is like Niagara Falls, in that they are both relentless in their assault as they erode the foundation of that which supports them.
Patients with bulbar onset ALS make up an unusual group because of the progressive and multi-system nature of their illness. Bulbar disease accounts for the majority of the worst ALS symptoms. The neuromuscular disabilities associated with bulbar ALS cause a myriad of related symptoms associated with swallowing, speech, and respiration. Although the rate of progression cannot be predicted, a pattern of progression is noted. Loosing the ability to swallow changes eating from a pleasurable task to a burden of survival. Loss of communication effectively imprisons the patient in a state of isolation.
The progressive weakness of respiration is the cause of death for nearly all ALS patients.
Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Patients experience increasing difficulty moving, swallowing (dysphagia), and speaking or forming words (dysarthria).
Symptoms of upper motor neuron involvement includes tight and stiff muscles (spasticity) and exaggerated reflexes (hyperreflexia) including an overactive gag reflex. An abnormal reflex commonly called Babinski’s sign (the big toe extends upward and other toes spread out) also indicates upper motor neuron damage.
Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps, and fleeting twitches of muscles that can be seen under the skin (fasciculations). Around 15–45% of patients experience pseudobulbar affect, also known as “emotional lability”, which consists of uncontrollable laughter, crying or smiling, attributable to degeneration of bulbar upper motor neurons resulting in exaggeration of motor expressions of emotion.
To be diagnosed with ALS, patients must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.
ALS Fighter, Brian Dillon 
my mom has bulbar onset also , the first sign was 10/2009 and then the disease start to be progressive , up to now after three years she is ok and she just lost her voice and ability to eat , she is using gastrostomy tube for feeding , and she is ok , the only big problem is the severe saliva that she cant holding it and she cant swalling it , her hands are more weak than three years before , but comparing to other cases her situation is perfect , something else that my mom was 55 years , and her grandmother also was 55 when she had als !!
i think we have a familial form
can u help me ?
My father was diagnosed was bulbar Als in January. Based on the doctors reports, the signs presented themselves clearly from Oct last year. He is on rilutek, a gluten free diet, therapy. He has been maintaining hasnt gotten worse. His speech is slurred, he can still eat, he has restrictions like putting his hands over his head ( right one more than the left), combing and blow dryinh hair, putting on his shirt, buttoning pants etc. he only complains about weakness in the arms. He can still walk strong, is still cautious about stumbling on things.In addition he is forgetting stuff but his cognitive function is still there.
My Sister was diagnosed with ALS March 9, 2012. Since that time she has lost all power of speech, has had to have to have a PEG tube placed to get nutrition, and has experienced respiratory failure twice but was able to be revived on both occassions. She is now still hospitalized and has been given the choice of getting a Tracheotomy and going on a ventilator to prolong her life, or going off the bi-pap and dying within a few hours with comfort care measures (hospice) in place because she can’t breathe without using the bi-pap. My sister is still young very early 60’s has two kids and 5 grandkids ages 16 and younger. This is the worst situation I have ever experienced in my entire life. I used to think when our dad died that was the most difficult thing I had ever had to endure, this is far worse and honestly I don’t know what else to say or do. She says she is not ready to go, but I know she suffered this last episode when she was unable to breathe and went into the respiratory distress ( I had to give her CPR until the paramedics arrived). I pray no one else who gets this awful, awful disease will be faced with such a grim decision. Choosing to live or die is not a decision anyone should have to make.
RM, i am moved by your story. My heart goes out to you and beloved family. My father was diagnosed with ALS in February, 2005. On December 7, 1012 he stopped breathing; the ventilar started to beep. My brother n’ law was there at the time and did CPR and revived him. In the ambulance he coded again. And 3 more times in ER. And one more time after that when he was placed in ICU. It turned out to be a mucous “plug” that blocked his airway. He later got what they said is aspiration due to the all the compressions from CPR. Two days in ICU he got HAP (hospital aquired pneumonia). This took him to ARDS (Acute Respiratory Distress Syndrome). He was in such distress and extremely critical that he was sedated with propofol for almost 12 days. We were in so much anguish as a family. We were told my father would not survive and so on a Friday, we made a family decision to withdraw lif support and provide comfort care. However, Saturday morning, the new doctor on shift said he noticed my father starting to trend SLIGHTLY better. What a relief! We gave him a few more days and his trend continued on the positivie side. He is now in a LTAC (Long Term Acute Care) hospital. He is awake and is SLOWLY recovering. However, we must also face the challenges of his weakened state. We have just discovered since he has been awake that there are personal things he does not remember, like my mother’s name, etc. We know eventually ALS will take his life, and from the looks of it, it can happen at any moment. He is a fighter, but is also tired of being this way for 8 years now. We love him dearly, and it is so sad to see him in this condition. Our thoughts and prayers are with you and your family and all the ALS patients and their families who care for them.
I have Bulbar onset too. Just finshed reading through Chaper 12, I didn’t see quite so many doctors as you did, but with the same results. I have a feeding tube, I can no longer eat,or speak.
Barbara I was diagnosed with bulbar onset two months ago and the doctors, nurses have not given me one thing I could do. I feel like a statistic . Carol
Barbara… when were you diagnosed with ALS Bulbar onset…. and what have you been doing to stop it or slow it down. Carol Lee Bellam