Neuromuscular and Skeletal diseases and conditions

MS – multiple sclerosis,

  • autoimmune, progressive, permanent myelin destroyed and nerve fiber breakdown, 20-40, environmental factors, relapsing/remitting most common type
  • FATIGUE, weakness, vision, spas. & trem., pain w/ parasthesia, no 2 patients alike
  • MRI & imunoglobin G bonded
  • Mobility, no heat, urinary and bowel – embarrassing young, eye dr. often, sex dysfunction
  • MEDS: Rebif, Betasteron SQqod / Avonex IMqwk / Copaxone SQqd / ßflulike symptoms / Methylprednisone for exacerbations, Mitoxarone IVq3mos (all to slow progression & reduce relapses

MG – Myasthenia Gravis

  • Autoimmune, myoneural junction, antibodies get in ACH receptors, 20-40W – 60-70M, face, throat (bulbar) swallow, voice, big thymus may hold antibodies
  • Tensilon test – bradycardia – Atropine
  • MG crisis ¯ or late meds (respir failure) , ACH crisis ­ meds (respir failure), always have suction available hospital and home, no sedative (hypoxia), conserve energy, energy for eating timed meds, sit ­ to eat
  • MEDS:  ACHsterase inhib – Mestinon / immuno mod (¯antibodies) Prednisone, Imuron, Cyclosporin, Cytoxan / Plasmaphoresis for exacerbations / Thymectomy

GBS – Guillain Barre Syndrome

  • Acute fixable myelin problem, after virus (CMV, pneum, flu) 16-25, 45-60, ASCENDING, fast
  • Respiratory failure, pulmonary embolis, , autonomic dysfunction, CV instability
  • In ICU, CSF will have ­ protein, Plasmaphoresis, IVIG
  • Have intubation close by, early detection, support limbs, temp pacemaker, TPN, communications before can’t talk, positive atmosphere, G-tube risk for aspiration

Parkinson

  • Progressive, basal ganglia, substantial nigra, dopamine ¯ – symptoms only after 80% decrease
  • ? cause – 2 out of 4 (trem, ridg, bradykensia, posture)
  • tremors, dementia, micrography, suffling gait
  • thalamotomy, Pallidotomy, stem cells
  • exercise, rest periods, assistive devices, no rugs, OT
  • MEDS: Antiparkinson –Levadopa, Carbidopa / AntiACH – Cogentin (Æ see pee spit shit) / Antiviral (¯ tremors) Symmetrel / Dopa-agonist – Parlodel, Permax, Requip, Mirapex / MAOI – Elderpryl / COMT inhibitors (­ dopa) – Comtan, Tasmar / Antidepressant – Wellbutrin, Prozac Elavil

Huntington

  • Chronic progressive, premature cell death basil ganglia & cortex / no treatment stops or ¯
  • Intellect, emotional and mobile decline
  • MEDS:  Haldol, Navane – reduces involuntary movement 

ALS – Lou Gerig’s

  • 2 yrs to life / loss of motor neurons in anterior horn and brain stem
  • Atrophy in extremities, trunk and bulbar
  • No cause, no cure / death by infection, aspiration, respiratory failure

Osteoporosis

  •  Loss of bone mass, altered bone turnover, in small white women
  • DEXA
  • Diet ­ in calcium and Vit D, walking, watch for fractures
  • MEDS: biphosphonates: Fosamax, Actonel, Boniva / Calcitonin / SERMS – Evista / Forteo / Reclasp IV 1 q yr

Osteomalacia

  • Deformity in long bones (bowing), low serium calcium and phosphate / due to GI, renal, ­thyroidism
  • Treat cause / fix with lots of calcium and Vit D / watch for hypercalcemia

Osteomyelitis

  • Infection of bone / due to soft tissue infection, direct bone contamination, spread from another site, mostly staphylococcus aureus
  • Bloodborne – signs of sepsis, tissue – no signs of sepsis – pulsing pain, swelling, tenderness
  • Round the clock IV antibiotics (lot of teaching), then oral 3 mos. / debridement
  • Diet protein & vit C, hydration

RA – Rheumatoid Arthritis

  • Autoimmune, synovial tissue, collagen breakdown, edema, joint pain
  • Stages:  Early – edu, balance exer & rest, NSAID, COX 2 – Viox, Celebrex, sm methotrexate / Moderate erosive – OT, PT, re-eval meds, cyclosporine (increases metho) / Persistent erosive – reconstructive surgery – synovectomy, arthroplasty, corticosteroids / Advanced unremitting – high methotrexate, cyclphosphamide, imuran

Osteoarthritis

  • Articular cartiledge, subcondral bone, synovium / pain in morning, relieved with moving around
  • Conservative ¯ weight, TENS/ surgical – osteotomy, arthroplasty, tidal irrigation
  • MEDS:  Tylenol, NSAID, COX 2, opiod, steroid, glucosamine and condroiton

Gout

  • ­uric acid  in blood/ cause by purines (shellfish, organ meats, asparagus, dried beans, oatmeal, alcohol), stress, illness /  or secondary can be genetic
  • Tophi – red and inflamed great toe, hands, ears / kidney stones
  • MEDS:  Colchicine, NSAID, corticosteroids, Benemid, Allopurinal – renal problems for kidney stones

Bone injuries

  • Contusion – soft tissue (fall) / strain – pulled muscle (over use) / sprain – ligaments, etc (twist) / dislocation – out of joint ¬ is a medical emergency (blood flow and nerve conduction – necrosis)
    • Treat with RICE – rest, ice, compression, elevation / dislocation – treatment,  immobilized, closed reduction
  • Fractures:  incomplete-greenstick – complete: comminuted (shattered), open (compound/complex), closed (simple)
    • Treat with:  internal fixators or external fixators
    • Pin care – don’t pick scabs, no weights on floor
    • Buck can unhook and move up in bed, Thomas (balanced) cannot unhook

Bone injury complications

  • Compartment syndrome
    • Increased pressure with in a fascia compartment, cuts of blood supply and nerve conduction, muscle death, happens under cast or dressing often
    • 5 p’s – pain, paralysis, parasthesia, pallor, pulselessness – relentless pain unrelieved by pain meds
    • Medical emergency
    • Remove cast, elevate, call dr, surgical decompression, stays open until swelling resolved
  • Shock – caused by blood loss, replace fluid
  • Fat embolism
    • Often from break in upper thigh, finger/ travels and clots major organ
    • S&S: hypoxic, tachycardia, Tachypnea, crackles, wheezes, fever, chest pain, coughing up yuck, ¯ BP, change in mental status
    • Prevent by immobilization at accident, give oxygen,

CHILDREN

CP – Cerebral Palsy

  • Most common permanent physical disability / non-progressive / extremely or very low birth weight – hypoxia during birth – kerniterous –
  • no sitting by 8-12 mos – may not be diagnosed until 2 yo
  • Can be: hypotonic, spastic/hypertonic, dyskinetic/athetoid, ataxic, mixed
  • Goal is optimum development / AFO, orthopedic surgery / align with bolsters, pillows
  • MEDS:  Baclofen, Diazepam, Dantrolene (for spas), Botox (prevent contractures), Baclofen  pump (intrathecal), Tegretol, Depokote (antiseizure)

MD – Muscular Distrophy

  • Largest group of muscle diseases / progressive / Duchenne most common / wkness, wasting, contractures / calf & thigh / loss of ambulation by 12 y/o / progress to resp. or card. failure
  • Gower sign, muscle biopsy shows fat replacing muscle fiber, ­ creatine kinase
  • Maintain indep. & function / nutrition & hydration / avoid obesity /  home s/b wheelchair accessible

Downs Syndrome

  • Most common chromosomal abnormality / extra chromosome, trisomy 21 / whites
  • Karyotyping / congenital heart – mostly septal / URI (due to depressed nasal bridge) / dys-thyroid / ­ risk leuk
  • Otitis media / atlantoaxinal instability (C1&2) / parents greive loss / ragdoll-like infant / swaddling / infection prone-handwashing

Fragile X

  • Most common cause of inherited mental impairment / males-all races / karyotyping-abnormality on x chrom / big ears, long face, prom. chin, big balls / aggressive / need routine / no cure / normal life span / MEDS:  Tegretol, Prozac (for aggressiveness), Catapres ( for hyperactivity)

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