Friday, October 10, 2014

LIVING with CF--updates from NACFC 2014

The NACFC discussions that receive the most attention are typically geared toward the scientific developments.  Obviously, I care deeply about progress toward a cure, but I try to balance those technical talks with some information on ways that we--as a community of patients and families, might be able to have a real positive impact on health through the informed choices we make today.  

The Relationship Between Exercise Capacity and Glucose Tolerance in a Pediatric CF Population Not Diagnosed with Cystic Fibrosis Related Diabetes.
Foster, K.E. Cincinnati Childrens Hospital

Hopefully, most people recognize that regular exercise can provide numerous health benefits to ANYONE.  Regular exercise is especially important for those with CF.  CF related diabetes (CFRD) is a frequently occurring complication of cystic fibrosis, and is associated with greater decline in lung function, poorer nutritional status, and lower life expectancy.  In this discussion, Foster examined the connection between exercise capacity, and the ability to successfully regulate blood sugars.  

Foster measured both exercise capacity, and glucose tolerance (via oral glucose tolerance testing) in 50 patients, and discovered a correlation between low exercise capacity, and increased 2 hour blood glucose values.  This suggests that patients with low exercise capacity have poorer glucose tolerance. Foster speculates that working to improve exercise capacity may help delay the onset of CFRD via improved blood sugar control.  

As you can see in this slide, there isn't hard data on this correlation, but rather, a strong suspicion that blood sugar control may be improved with regular exercise--leading to a delay in the onset of CFRD.  

Since I personally believe strongly in the value of exercise in health maintenance, I want to hammer the point home with one more discussion:

Yoga Improves Posture and Muscular Performance in Adult Persons with Cystic Fibrosis
Scott Russell, University of Southern California

"Persons with cystic fibrosis are prone to posture dysfunction, in part due to pulmonary hyperinflation and chronic coughing which can lead to injury, chronic pain disorders, vertigo, headaches, balance dysfunction, increased risk for vertebral fractures, and loss of physical functioning.  The purpose of this study is to investigate the effects of Hatha yoga on posture, neuromuscular performance, and self-reported psychometric measures."

In this small study, patients agreed to participate in twice weekly, 90 minute, outdoor yoga sessions over the duration of 8 weeks.  A couple of important points worth noting:

1) Strict infection control guidelines were observed--persons with CF were each provided with their own (vs. shared) yoga props, and were equipped with hand sanitizer gel, tissues, etc...  Yoga was also practiced in an open, outdoor area and the "6 ft. rule" was observed due to multiple CF participants in the same class.  Of course, if you decide to take up yoga, it is unlikely that you would be surrounded by other CF patients, and such strict precautions may not be required.  

2) Russell made sure to note that practice of yoga for CF patients often requires modification of poses to avoid the "head down" position, or "inversions," which can aggravate acid reflux/GERD for many patients.  It is very important to educate the instructor about the need for these modifications, since an increase in acid reflux symptoms would be an undesired effect, and could lead to a worsening, rather than an improvement in lung and postural health.

This is hard to read, but it says that the primary aims of this study are to discover 1) the effects of yoga on pulmonary functional, and chest wall mobility, 2) the effects on physical function and posture, and 3) effects on scapulothoracic posture.   Self-reported psychometric measures regarding quality of life were also recorded.  Ultimately, Russell wanted to determine the SAFETY and POTENTIAL BENEFITS of yoga for persons with CF.

These are examples of the "head down" positions that should be AVOIDED if you suffer from acid reflux/GERD.

Here we see an example of a modified downward-facing dog position.  Note that the trunk and head stay parallel to the ground, rather than the typical head down positioning.  A few extra props may be helpful for the appropriate modifications

Russell provided an example of the poses used by the instructor in the intervention.  

The results showed an improvement in chest wall excursion after 8 weeks of the yoga intervention.

Scapular positioning was also improved--positive effects on posture. 

Patients showed improvements in the "Sit to Stand Test" and several other fitness measures.

Patients had improved self-reported attitudes toward their weight/body image.

As I mentioned earlier, this was a small study, but I appreciate that we are discussing topics like this AT ALL, so I wanted to report on it anyway!  It is my blog, and I do what I want! :)

You might even consider doing your own "future investigation" on whether yoga is something that might be beneficial for you, remembering to follow the appropriate precautions.

In conclusion, Russell reported that in this phase 1 feasibility study, yoga improved posture, chest wall excursion, lower extremity muscle performance, and the self-reported body/image perceptions.  

In my opinion, exercise is an extremely underutilized tool to improve health outcomes for CF patients...and basically everyone else (in the U.S., at least!).  While we don't have a ton of evidence from a large scale study on the benefits of yoga specifically, there is PLENTY of evidence regarding the benefits of regular exercise.  Maybe yoga doesn't sound so great to you...and that is OK!  The best type of exercise will be something you enjoy, so that it will be done consistently.   Since no new breakthrough therapies are hitting the market today...why not try EXERCISE as a natural, effective way to provide multiple health benefits?  I know that when I recently attended a "restorative yoga" class with my friend Savannah, I left the class feeling awesome--both physically and mentally.  I think that the attention paid to breath control in yoga makes it a particularly attractive option for patients with pulmonary dysfunction.  

The next talk I want to summarize deals with a topic that some people wouldn't consider CF related at all, but I will argue that mental health issues can have deep and long lasting effects on health for both CF patients themselves, as well as their family members.  As a CF mom, and a member of the Patient Engagement Advisory Committee for the CF Foundation, I've come to realize that mental health simply cannot be segregated from our physical health.  Babies and young children are absolutely dependent on their parents for everything from proper nutrition to adherence to their prescribed CF treatment regimen.  If a caregiver is struggling, negative effects can ripple throughout the family, and ultimately affect the child.  When I was a new mother struggling with Brady's CF diagnosis, I would have never even considered bringing up my own mental health struggles with Brady's CF clinic team...even though I realize now that I probably should have.  It didn't seem like the time or place for me to air my own concerns about how out of control I felt over my emotions, and life in general.  Parents of children with special needs often put their own problems on the back burner, and find the topic to be too personal and painful to discuss.  I struggled with depression, crippling anxiety in regard to clinic visits, and persistent sleep problems during Brady's infancy.  I didn't want to admit that this time in my beautiful son's life was a really dark and scary period for me. I just figured that because CF sucks so much, I would probably never feel really good again, and that the depression and anxiety were par for the course.  I'm sure that my struggle is not unique, and that many of you may potentially relate to the helpless state I found myself in.  

What Healthcare Providers Need to Know About Postpartum Depression (Associated with Newborn Screening), But Were Afraid to Ask.
Audrey Tluczek

This discussion dealt particularly with postpartum depression associated with a CF diagnosis following newborn screening.  The speaker explained that she uses the term "postpartum depression" simply because it is defined by the onset of depression symptoms coinciding with the birth of a child. Unfortunately, for families with a newly diagnosed infant, that early period can be so overwhelming and scary.  Many would probably consider postpartum depression/anxiety a problem exclusive to mothers--but when management of a chronic disease in a child is required, it can be just as pronounced in FATHERS, GRANDPARENTS, or anyone that is functioning as a care provider for the child.  Tluczek discussed the need for more formal depression screening as a part of the regular clinic visit for families with newly diagnosed infants, and training of the clinic care team to provide useful interventions.

Clinic teams are becoming aware that mental health assessments NEED to become an integrated part of CF care.  Unfortunately, many members of the care team may not be formally trained to recognize the symptoms.

For family members exhibiting signs of depression in association with care of the infant, treatment options can include psychotherapy, support groups, medication, and hospitalization for the most severe cases where the health of the individual or child is believed to be endangered.

This is important, because depression negatively impacts parent/child interaction.

I didn't realize how much my own mental health might be negatively affecting the precious child I wanted so desperately to protect.

Having a good relationship with a stable partner, family support, and the ability to provide for the basic social and economic needs of the family were found to be important factors in lessening the negative effects of post diagnosis depression. 

The CF Foundation recommends that mental health screenings should be a part of the regular clinic experience for both CF patients, and their families.    
In this day and age of comprehensive care, I think it is so important that we feel that we can discuss depression, anxiety, OCD symptoms, or any mental health concerns with the CF care team.  Looking back, I certainly wish I hadn't suffered in silence for so long.  It is equally important that care teams are appropriately trained to recognize the symptoms of mental health issues, and provide options for treatment that may include referral to an outside professional.  This topic is simply too important to remain taboo.  It should be noted that while this talk focused specifically on postpartum depression, mental health concerns can come up at any time, especially with the demands of chronic disease management.  These issues are important to address at any stage of life.

I know many of my readers tune in to the blog for science updates.  I hope I haven't tarnished my rep by devoting this entire entry to the fine art of living and thriving with CF.  Tomorrow, I plan to return to the cutting edge...and honestly, I need a night to sleep on the information I heard this morning in Symposium 8: Gene Editing Strategies for Therapy and Research.  I believe that the future cure for EVERYONE with CF--regardless of their mutation, may become reality through one of techniques discussed in that room.   Until tomorrow...


  1. Thank you so much for taking the time to keep us updated and for sharing such diverse information! I look forward to your updates so much! I wish the mental health discussion wasn't so taboo, either. After my son's diagnosis I would have never thought that I was suffering from depression and anxiety. However, three and half years later we welcomed another baby and both my husband and I were sunk. I began to see a counselor and eventually got medication from my doctor. Oh, how I wish I had done that years sooner! I am so grateful that I was able to discuss things with our clinic's social worker and I'm grateful for the support of friends who continued to encourage me to seek help. I'm grateful that there IS help out there and I'm so grateful that I found the right help for me. I didn't know how bad my anxiety was until I was freed from it. It is amazing and I finally feel like I can be the mother I always wanted to be. I hope our teams will be able to help other families find those same resources! Thank you for sharing that they are talking about it.

  2. hi!
    my name is miriam and I am a uni student of physical therapy.
    i came across your post and tried tofind the articleof Scott Russell about yoge and it's benifits for cf. and i just can't find it!!!
    it is very importent tomy so iwill manege to pass it to my patient.
    thank you for your blog!