Care of Patients with Intellectual and Developmental Disorders (French, 8/11/2020)

Many thanks to Dr. Anne French, who gave us a jam packed 45-minute mini-fellowship in the Care of Patients with Intellectual and Developmental Disorders (IDD) this week. I learned so much! But also am left feeling like I have so much more to learn. Her 11 years of experience as a family doctor at the Sonoma Developmental Center (SDC) and her sharing about her personal care of own brother with Down Syndrome make her an invaluable community resource. If you or someone you know is caring for someone with IDD and needs consultation, keep Dr. French in mind!

Risks: patients with IDD are at higher risk for neglect, abuse, poverty, substandard medical and dental care

• ~46% of patients with IDD are on psychotropic medications 
• 3/4 haven't seen a psychiatrist, 1/3 don't have a psychiatric diagnosis
• Many folks with IDD have untreated hearing and/or vision deficits
• High rates of obesity (sedentary lifestyle, poor diet)
• Despite high rates of diabetes and hypertension, most are under-treated and under-screened
• Very high rates of dental caries



(Dr. French recommends 3 sided toothbrush to prevent caries)

Cultural competency: caring for patients with IDD requires a special cultural competency

• learn their language
• "listen with the ears of your heart"
• how do you get a patient to relax in the clinical environment?
• how does illness present differently in this population?
• see them as a fully aware human being
• develop trust
• be willing to abandon your own agenda
• use "people first" language (eg. person with spastic quadriplegia, person with autism)
• understand disability etiquette

Consent and Conservatorship: who speaks for the patient? (remember to document this in the chart)

• Conservator: the legally appointed person to manage financial, legal, and medical affairs of an adults who is unable to do so (requires $$ and court)
• Consenter:  typically family member but also can be a friend (without papers) who demonstrates consistent involvement, recognized in California as valid
• North Bay Regional Center: if there is no conservator and no family involvement, NBRC signs and acts on behalf of their clients if they are clients of the NBRC
• Some patients with mild IDD consent for themselves

Vital signs: interpretation of vital signs can be challenging

• Temp: use infrared thermometer when possible
• BP: consider wrist BP cuffs (more comfortable)
• don't assume an elevated BP is correct in setting of agitation/anxiety
• home BP checks may be more accurate
• Weights: getting accurate weights (especially for wheelchair bound) can be challenging, use table weights if available (they have at Dutton)

***

Dr. French spent some time talking about the Fatal Four: SBO, Aspiration, Sepsis and Seizures. These are the Top 4 Preventable Causes of Death in patients with IDD. This section was particularly compelling to me because it brought forth many memories of previous patients I have cared for (in clinic and the hospital) presenting with one or more of the following challenges:

SBO

• Pts with IDD tend to be on multiple meds with constipating side effects (antipsychotics, seizure meds). They also may have poor PO intake, low fiber diet, sedentary lifestyle
• Underrecognized and undertreated chronic constipation 
• Some people with IDD have neuromuscular dysfunction due to their underlying condition (e.g. CP)
• What should you do about it?
• Ask patients and caregivers about stooling at every visit
• Insist upon daily bowel care (not PRN only)
• If unclear, get KUB to assess stool burden

Aspiration

• pts with IDD often have dysphagia secondary to neurological impairment (e.g. CP) or medications (depakote, antipsychotics)
• neuromusclar disorders also include motility disorders, leading to more GERD/reflux
• some people with IDD have postural issues
• recurrent aspiration PNA causes scarring with reduce lung volumes, actually leading to a special type of COPD
• feeding tubes do NOT prevent all aspiration
• What should you do about it?
• instruct patients and caregivers to keep people sitting up after eating
• care with volume/quantity of feeding tube feeds
• hold formula, use pedialyte instead when sick with URI Sx (x48 of hte worst symptoms)
• use beta agonists nebs aggressively after aspiration sx
• consider ABI vests (if can tolerate)
• can use guaifenisin to thin secretions if aspiration event

Sepsis

• early warning signs are key: know the patient (e.g. "quieter than usual", decreased appetite, "just not themselves", weakness may show up as exaggerated baseline neuro issues
• understand patient hx and risk factors (e.g. recurrent aspiration, UTIs, gallstones, SBO)

Seizures

• uncontrolled seizures can lead to sudden epilepsy death, aspiration PNA, other injury
• epilepsy: there is in epilepsy specialist (for IDD and not) at Dutton
• many new seizure meds that have less monitoring and less toxicity (e.g. keppra, pharmaceutical grade CBD=Epidolex)
• Vagal Nerve Stimulators (VNS) can improve qol, especially after 2-3 unsuccessful seizure meds

Additional pearls:

• Slow medicine: use serial exams, don't try to get it all done at once
• Train caregivers to look out for signs of illness
• Agitation may be a sign of communication or physical distress (e.g. pain)
• don't forget neurogenic bladder, BPH in older men!
• consider emotional sources: frustration, inability to communicate, abuse
• Dual diagnosis is common (depression, anxiety, psychosis, ADHD)
• Dementia (particularly in DS) very high incidence
• Always send dislodged J tube to ER
• Health passport and pre-appointment checklist available on IDD orderset in ECW at SRCH (see images below)

Special Services available at Dutton Clinic (SRCH)

• primary care IDD co-management (Dr. French, Dr. Camarata, Brittney English FNP)
• dental care (including sedation)
• podiatry
• neurology/epilepsy (Dr. Rowena Korobkin)
• general surgery (Dr. Michael Bozuk)
• psych, behavioral health, including psych tech (Dr, Tract Jones, Mark Hecker PT)

Resources recommended by Dr. French:

American Academy of Developmental Medicine and Dentistry (https://www.aadmd.org/)

IDD order set (ECW)

IDD tab on SRCH intranet

Knee and Hip Osteoarthritis (Smith, 8/5/2020)

I am always grateful to Sutter Medical Group's Dr. Briant Smith for his orthopedics teaching, and this week's Grand Rounds was no exception. Dr. Smith reviewed the basics of non-surgical evaluation, diagnosis and treatment of osteoarthritis of the hip and knee, definitely very common conditions seen in primary care! 

Here are Dr. Smith's tips:

Examination of the patient with knee pain 

• Gait: ask patient to walk across the room and back (look for flat feet, among other things)
• Alignment: look at legs standing (varus=bow-legged, valgus= knock-kneed)
• Range of motion: looking for difference in joint ROM compared to the OTHER knee
• Joint line tenderness
• Effusion?
• Check hips (seated internal rotation-- this is to be sure you are not missing a primary hip problem)

• 
• 
  

Imaging for a patient with knee pain

• X-ray is the imaging modality of choice for osteoarthritis 
• For knees, always get standing AP views of BOTH knees, lateral view of BOTH knees
• For hips, always get images of BOTH hips
• Remember, sometimes the xray may be incongruent with the patient's symptoms (e.g. both hips look severe, but patient only has pain on one side)
• 
• If you look at the x-ray (ideally look at the image WITH the patient), you are looking to characterize mild-moderate arthritis (some joint space narrowing, small spurs) vs moderate-severe changes (bone on bone, bone sclerosis, sub-chondral cysts)

• Do NOT order an MRI before an xray because. . .guess what?
• If there is evidence of DJD on the xrays, you don't actually NEED an MRI! 
• You have made the diagnosis and can start the treatment.

What about arthritis and meniscus tears?

• All knees with arthritis have meniscus tears
• The pain is almost always due to the arthritis, and arthroscopy (meniscectomy) doesn't help, with very rare exception

Treatment of knee and hip arthritis

• Don't forget to explain the diagnosis: "you have arthritis in the right knee""
• Make the distinction between mild-moderate and mod-severe "You have severe arthritis in your right knee"
• Dr. Smith recommends this analogy: The protective coating on the ends of the bones has worn away, like the tread on a tire. You are now on the steel belt (mild-mod arthritis) or the rim (mod-severe arthritis)
• 

What can patients do?

• Relative rest/modified activity: many patients love to walk and run, but probably best to switch to swimming and/or stationary cycling (then they can keep up their aerobic exercise)
• OTC meds: NSAIDs (if not contraindicated, be sure to check labs, particularly for SCr), acetaminophen, glucosamine, fish oil, CBD. Avoid opiates.
• Physical therapy,  (it's good idea to do some PT before knee or hip replacement, but remember Medicare only covers 18 visits/year, don't use them all up if a patient is going to get surgery)
• Steroid injection: if the injection works and lasts a few months, okay to repeat (up to 4 times/year) for severe pain, but if only helps for a week, then probably not worth it
• hips done by radiology at the hospital 
• Not great evidence for hyaluronic acide injections (costs $500-1000, usually cash)
• Knee bracing not indicated, some patients feel more comfortable/supported in a neoprene knee sleeve. This isn't helpful but if it makes them feel better is fine

When is a patient ready for surgery ?

• When they are ready.
• When you might consider them ready?
• If they are having sleep issues due to pain
• If they have significantly modified their activity and are missing out on things they would normally enjoy because of their pain
• There is no lower or upper limit of age for hip or knee replacements. It's really the severity of the arthritis and their degree of illness/wellness.

Surgical Preoperative Optimization

• Anemia, goal Hb>12 reduces transfusion risk (perioperative transfusions actually increase the risk of infection and LOS)
• Smoking, goal quit at least 6 weeks before surgery, including ALL nicotine products (nicotine affects wound healing and infection risk)
• Diabetes, goal <7.5 (infection risk)
• Obesity, goal BMI <30, most surgeons won't operate if BMI>40
• Malnutrition (albumin >3,5)
• And more! 
• immune status, hypothyroid, low vitamin D, CV disease, ASA class, opiates, CKD, sleep apnea, psychiatric illness, social factors, deconditioning

Rheumatoid Arthritis Part 1 (Kremer, 2/26/2020)

Dr. Lisa Kremer gave a wonderful Grand Rounds this week on Rheumatoid Arthritis. To be clear, RA is not a topic that normally gets me out of bed in the morning. But Dr. Kremer's presentation was so good that I found myself wishing for it not to end. Or for Part 2 to follow asap.  And even several hours later, in the chaos of a busy day in the hospital, I found myself considering this strange disease-- rarely seen before the 1600s, now quite common, terribly disabling, and brought about by a "perfect storm" of genetics, environment, and stress.


RA is characterized by symmetrical polyarticular swelling of the small and medium joints on more than one occasion, over more than six weeks, supported by lab and/or xray and absence of other diagnosis. Exact causes are unknown. Multiple triggers.

Dr. Kremer described RA as an autoimmune condition, with some genetic susceptibilities (e.g. HLA DR4), for which smoking doubles the risk. RA can be precipitated by infections, environmental toxins, social and physical stresses, and hormonal triggers.

• 1% of the the adult world has RA (1.5 million people in the US)-- the most common chronic inflammatory arthritis
• 4:1 female to male
• Peak age onset 40-60 years (but anytime after puberty is possible)
• All races and geographic areas are affected
• Specific populations with higher incidence (Native Americans, particularly: up to 10% of Sioux, Algonquian, Pima, Yakima, and Inuit peoples)

And while Dr. Kremer presented us with these data and more, she also presented the case of Pierre Aguste-Renoir (1841-1919), a French artist and a leading painter in the impressionist movement. She described him as a joyous and radical young man, struck by RA around age 50. His RA seems to have been precipitated by a fall from a bicycle and a resulting arm fracture. From which he never really recovered. And yet Renoir continued to paint long into his illness-- even designing his own wheelchair and equipment to be able to reach up to his large canvass painting surface. 

Dr. Kremer espouses that Renoir is a particularly excellent painter of hands. Perhaps he spent a lot of time thinking about hands. And looking at them. . .

Laboratory testing in RA is helpful but pretest probability determines the benefit of the test. 

• Rheumatoid factor (RF) is not specific
• Anti-CCP is more specific (can actually be positive a few years prior to onset of symptoms, but not always)
• ANA can be positive
• ESR and CRP really convey inflammatory cascade

RA vs. OA (from PPM here): 

Extra-articular complications of RA only occur only in seropositive patients (i.e. +RF ):

• fever and weight loss (can look like cancer)
• nodules (can be anywhere: eyes, heart, etc)
• interstitial lung disease
• pleuro-pericarditis
• CAD
• malignancy (specifically mymphoma)
• infections (like pneumonia)
• a variety of hematologic abnormalities (anemia, thrombocytopenia)
• osteoporosis

Prognosis:
Untreated RA shortens life by 5-10 years. Aggressive RA therapy decreases mortality risk due to CV disease, lung disease, and more (more on this next time). Treatment reduces need for joint replacement byup to 50%.

Disability:
Over 33% of RA patients working at the time of diagnosis will leave workforce within 5 years
Fatigue and unpredictable joint symptoms are frequently the most disabling issues

And finally, here are Dr. Kremer's pearls of wisdom:

• Deformity does not equal disability
• RA does not cause back pain
• Never order tests if you don't know what you are looking for
• Low SES is associated with onset and severity of RA
• Smoking DOUBLES the risk and worsens the progression
• RA is "soft and spongy" (not hard and bony like osteoarthritis)
• A positive RF is not diagnostic, it should prompt you to keep looking for a diagnosis
• DIP joints are almost always spared
• If after careful exam and lab testing, you suspect RA, refer early to rheum!

Stay tuned: Dr. Kremer will present Part 2 (Rheumatoid Arthritis: Treatment) in July or August of 2020. Keep your eyes out! And don't miss it.