Jordan Whitson, MD Duke University

1. LIFESTYLE APPROACHES IN
PULMONARY HYPERTENSION
Jordan Whitson, MD
Duke Pulmonary Vascular Disease Center
November 17, 2023

2. NON-MEDICAL THERAPIES FOR PH
• Oxygen Therapy
• Sleep Therapy
• CPAP/BIPAP/Trilogy/Oxygen
• Salt and Fluid Management
• Exercise and Activity
• Cardiopulmonary Rehab
• Diet and Nutrition
• Miscellaneous: Safety/immunizations/surgery or procedures
• Quality of Life, Support and Support Systems
• Social Support, Home Health, Palliative Care
• Transplant/ CTEPH/ other non-medical treatments

3. WHY OXYGEN?
LOW OXYGEN CAN DRIVE PRESSURE UP AND MAY STRESS
OTHER ORGANS
• Goal to try to keep O2 greater than 88-90% - for some this may not happen
• Congenital heart disease may not be possible
• At rest we may use one flow but often more with exertion.
• May have to adjust within reason for different types of activity.
• Shortness of breath does not always mean oxygen is low
• Not feeling badly does not always mean oxygen is good
• Portability (Continuous or pulse dose- make sure it works for you )
• IMPORTANT NOTE: 2 liters pulse ≠ 2 liters continuous

4. SLEEP
• Overnight oxygen check to see if you need oxygen or sleep test (can be done at
home)
• If you have sleep apnea and it’s not treated it can aggravate PAH and heart failure,
high blood pressure
• If mask or machine not working check with company to adjust or change
• Don’t just stop using it (may take getting use to or need adjustments)
• Some people need CPAP, others BiPap or Trilogy
• Good sleep habits are important. Sleep regular hours.

5. SALT AND VOLUME MANAGEMENT
• Limit intake of fluid to 1.5 to 2 liters as appropriate (not a standard value – depends
on height, weight, etc.)
• Limit salt intake and provide education about hidden salt in prepared foods
• 2 grams (more of a standard rec)
• Daily weights – first thing in AM, clothes off, stand on the scale, keep track on a
calendar
• Introduce concept of adjusting diuretic for rapid weight change
• Weight is not a perfect measurement and education about loss of lean mass and
gain of fluid and recognition of swelling is critical
• Under and over diuresis can negatively impact your heart failure, kidneys.
• Can be complicated but if you do the basics your team can help adjust

6. ACTIVITY AND EXERCISE
• Safety First (your doctor knows you best and there may be individual things about
you that may be important)
• If you are in advanced heart failure from your PAH you should not be exercising
without supervision
• Avoid or stop activity if making you lightheaded or dizzy, passing out
• Start slow and work up. If possible supervised first (plug for rehab)
• Use oxygen as indicated and instructed.
• We are all at different functional class
• Some of you will normalize your function and can do most anything
• Aerobic activity more useful than weight training or isometric activity
• Walking, exercise cycle, steppers, Nu – Step,

7. WHY DO I GET SO TIRED WITH ACTIVITY?
AM PIRFO, E CHEN “JUST DO IT” PRACTICAL ASPECTS OF PULM REHAB PROGRAMS. ADVANCES IN PH VOL
18 : 2 2019

8. CARDIOPULMONARY REHAB
• Can be either cardiac rehab or pulmonary rehab or combined
• Depends on what is available to you
• Depends on the “flavor” of your disease (other lung disease or congenital heart)
• Safe/careful monitoring
• Teach good habits
• Builds confidence
• Education
• Socialization

9. HOW DOES EXERCISE HELP
• Does not cure your PH but…
• Helps recondition your muscles to make it easier to do activity
• Makes us more efficient - heart/ lungs/ muscles
• All the way down to enzymes and chemicals in cells
• Improves endurance and tolerance of activity
• All these together improve Quality of Life
• Easier to do the same activity and then allows you to do more

10. EXERCISE RECOMMENDATIONS FROM PHA SCIENTIFIC
LEADERSHIP COUNCIL
• Speak with your individual doctor before exercise program
• Some form of exercise is not harmful and may be helpful
• Do not over exercise (symptoms of this include
lightheadedness, chest pain, or severe SOB)
• Recommend light to moderate aerobic activity, light resistance
of small muscle groups
• Class 4 symptoms (fainting in particular) should not exercise
• Safest to start in monitored setting if not well

11. NUTRITION
• Consider all of your health issues ( Diabetes, overweight, underweight)
• Weight loss/gain if need future transplant
• Is there underlying connective tissue disorder (scleroderma with dysmotility)
• Poor appetite or getting full fast may be due to your disease
• Swelling or edema, ascites, right heart failure
• Low output with renal dysfunction and potassium issues
• Poor control of diabetes etc will have negative impact on heart/ renal
• GERD (Reflux) issues in some patients or aspiration will also negatively impact
prognosis indirectly
• What foods, drinks, dietary supplements, how and when you eat

12. EMERGENCY PREPAREDNESS/SAFETY
• Backup power: oxygen or sleep machine
• Refrigeration of certain medications
• Plans for issues with IV access or pump malfunctions if on parenteral therapy. Loss
of access, leaking,
• Malfunction of pumps or nebulizers, batteries
• Many of you are on RARE medications. Have a list. May not be familiar to all
medical personnel
• (Bring MEDS/supplies with you) - not always available at local hospital
• Interruption of medication supply (even oral therapy)
• Plan for early supply if going away, storm coming ,etc.

13. (EMERGENCY) PREPAREDNESS
• Know who sends you which medications so you can contact if late or need extra or lost
• What do I do if my catheter falls out?
• What do I do if I am having a test or procedure and they tell me not to take medication.
• Can I get an MRI or CT scan or mammogram or colonoscopy
• If I am traveling how can I bring my medications and oxygen. Get permission up front from
airline etc. Get extra medications in advance. Ask if you can have walker or wheel chair at
destination.
• Speak with nurse, MD, specialty pharmacy, etc. about how to deal with these things
• DO NOT WAIT UNTIL LAST MINUTE

14. QUALITY OF LIFE
• When you are sick it can affect how you and your family live your life
• Depression / Fear/ Frustration/Anxiety can arise
• Financial issues or fear of them, Keeping Insurance
• Can I work? Can I adjust my job?
• Family Medical Leave
• Worry about our own future and our spouse/partner and children
• Can I go on vacation or do things I enjoy
• Plan well. Be creative about things
• The answers to all these things are different for all and can change over time
• May get better or worse

15. QUALITY OF LIFE –SUPPORT
SYSTEMS
• PH Center – MD, nurses, pharmacists, other patients, nutritionist, social worker
• Cardiopulmonary Rehab includes lots of education
• PHA local and national, groups and online sites. “Great resource to meet other folks
who are tackling same issues and might have good advice”
• Inpatient and outpatient teams
• Insurance or financial assistance programs
• Pharma companies assistance programs (yearly reauthorization)
• Nursing and Pharmacy companies that deliver special therapeutics

16. TRANSPLANT
• The timing of transplantation is critical and sometimes difficult
• Survival from severe IPAH refractory to medical therapy is poor and the availability of suitable
organs for transplantation is limited
• Bilateral lung or heart/lung are the options
• Guidelines for when to consider/refer for transplant evaluation are as follows :
• World Health Organization (WHO) functional class III or IV despite therapy
• Pressure in right heart and lungs remains high
• Cardiac index (output of your heart is low and not improving
• Failure to improve functionally despite medical therapy
• Rapidly progressive disease
• Age, additional medical issues, weight all play a role
• Transplant is a new chronic condition that requires medicines, etc.

17. HOME HEALTH/PALLIATIVE CARE/HOSPICE
• What if I am just getting worse and no more options or I have been in the hospital
and do not think I can go home?
• Short term rehab or placement can be problematic with oral, inhaled and especially
IV medication
• Cost or safety issues may preclude acceptance
• Can I get home health or palliation at home
• Palliative care can simply mean talking to someone to help out with all the non
medical stressors or the non-PH symptoms or
• It can be a step to transitioning to coming off aggressive care and opting to be
comfortable
• Hospice care is sometimes the last thing and can be the right thing. May sometimes
have to come off meds

18. THANK YOU
QUESTIONS?