Please enable JavaScript.
Coggle requires JavaScript to display documents.
Learning issue 1: What is the nature of end-of-life planning for an ALS…
Learning issue 1: What is the nature of end-of-life planning for an ALS patient with a terminal illness that has a child under 18?
Advance Directives
make decisions early about artificial nutrition, hydration, tracheostomy and ventilation are essential since the disease usually progresses to difficulties breathing and eating
Ms. NR already expressed concerns about what kind of life it will be if she can't move and eat and can't breath on her own
Ms. NR should also rename her Health Care Representative, as it used to be her mother who passed away
End of life care
most people with ALS die peacefully in their sleep due to diaphragm weakness
morphine can be very effective in relieving most of the suffering
many patients choose to use hospice, which provides nurse case manager, home health aide and social worker
It is important for the parent to name a guardian in their will, otherwise a judge will appoint someone without knowing the parents wishes
Ms. NR should consider who she wants to name as the legal guardian, whether it be her aunt who has been helping take care of Gary, or Gary's father whom she hasn't spoken to for a while.
Also since Ms. NR does not have a spouse, Gary will inherit everything unless stated otherwise in her will.
Also important information to clarify is if Gary's father every had joint guardianship over him
In a joing gaurdian situation, if one parent dies or is removed as gaurdian, the other parent of the minor child becomes the sole gaurdian in the state of CT
standby guardian
Learning issue 2: What is the typical progression/timeline of ALS?
Most typical presentation is asymmetric limb weakness (80%)
can present as upper motor neuron or lower motor neuron issue in any body segment
UMN symptoms: slowness in movement, incoordination, stiffness, poor balance, spastic gait,
LMN symptoms: weakness, atrophy, fasciculations, muscle cramps. Difficulty manipulating small objects (buttons, zippers, coins). Proximal arm weakness, slurred speech.
20% of patients will have onset in the bulbar segment, which presents as dysarthria or dysphagia
declining frontotemporal behavioral and executive dysfunction may precede or follow the onset of UMN or LMN dysfunction
most patients do not have over dementia, but there is some degree of cognitive and behavioral dysfunction occurs in 1/3 of patients
relentless progressive disorder that is relative linear with a constant slope
the rate of progression between individual is variable, but usually gradual and progressive worsening over time (without any interventions)
on average, individuals live 3-5 years after the onset of symptoms
some may experience a more rapid progression of symptoms, while others may have more slowly progression
10-20% can live for 10 years or more after initial diagnosis
factors associated with longer survival: younger age at symptom onset, higher ALS functional rating scale and forced vital capacity at presentation, and limb rather than bulbar symptom onset.
Ms. NR had limb onset, young age at symptom onset and limb onset so she has good chance of longer life expectancy
spreads in a predictable pattern: in patients with unilateral leg onset, the most common pattern of spread is contralateral leg, then ipsilateral arm, then the contralateral arm, and then to the bulbar muscle.
this seems to be true for Ms. NR
the progressive course may eventually produce one or both of the life-threatening aspects of the disease: respiratory failure or dysphagia (risk for aspiration)
Sources:
https://www-uptodate-com.online.uchc.edu/contents/symptom-based-management-of-amyotrophic-lateral-sclerosis?search=life%20expectancy%20for%20ALS&source=search_result&selectedTitle=2~150&usage_type=default&display_rank=2
https://www-uptodate-com.online.uchc.edu/contents/clinical-features-of-amyotrophic-lateral-sclerosis-and-other-forms-of-motor-neuron-disease?search=ALS&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1
ALS Guide #11 Approaching End to Of Life in ALS
https://www.als.org/sites/default/files/2022-10/Resource-Guide-11.pdf
https://www.nolo.com/legal-encyclopedia/intestate-succession-connecticut.html
https://www.jud.ct.gov/lawlib/Notebooks/Pathfinders/GuardianshipinCT/Guardianship.pdf