Lymphedema Therapy Source was recently featured in an entrepreneurial article by Subkit, an organization that helps entrepreneurs and small business owners grow and learn. They cover a lot of industries (food, hospitality, health, cosmetics, home, etc.). You can read the article here: https://gosolo.subkit.com/rebecca-summers/.

What’s your business and who are your customers?

Lymphedema Therapy Source, PLLC provides complete decongestive therapy (CDT) for people with lymphedema (or aspects of CDT like manual lymph drainage for other conditions such as wound healing, post-op plastic surgery, etc.). And compression garments are also measured and provided.

Tell us about yourself

I was working as an O.T. and had a patient who had had a bilateral mastectomy (both breasts removed). She developed lymphedema in both arms as a result, and I couldn’t help her. My reason for becoming a therapist was to help people, so I decided to get special training to be able to help others in the future. Motivation comes from the desire to help improve the lives of others.

What’s your biggest accomplishment as a business owner?

Staying in business since becoming self-sustained as an entrepreneur in 2013.

What’s one of the hardest things that comes with being a business owner?

The hardest part is the many hats an entrepreneur must wear (without the training). You must learn as you go unless you find a successful mentor who has done exactly what you want to do.

What are the top tips you’d give to anyone looking to start, run and grow a business today?

1. Do your pre-launch homework. (Talk with others doing the kind of business you want to do. If you can find a mentor who is where you’d like to be, hire them and/or offer some type of service in return for their knowledge and time.)
2. Remember the golden rule (treat others as you wish to be treated). Whether integrity, kindness, patience, or service – put yourself in your customers’ shoes and most times, you’ll know what to do.

Is there anything else you’d like to share?

I’ve been an OT for over 20 years and self-employed half of that time. Being an entrepreneur is fulfilling. I like the challenge and being the one to make the decisions. But there’s certainly a lot of work and risk that goes with it. A key is enjoying what you do, finding success while doing it and never forgetting that life isn’t solely about money or accomplishment. No one reaches the end of their life regretting they didn’t make more money or spend more time at work. The regret is more often not reaching their potential and not having spent more time with those they love (or mended bad relationships they cared about).

Many of the posts on the Facebook page have been about famous people linked to lymphedema. You may know actress Kathy Bates (spokeswoman for the Lymphatic Education and Research Network – also known as LERN) has lymphedema. But I recently learned another entertainer joined forces with LERN: Actor Steve Guttenberg.

You may be familiar with Steve through movies like Three Men and a Baby, Police Academy and Short Circuit from the 1980s (I remember them all!).¹ He doesn’t have lymphedema, so how does lymphedema fit into the picture?

Steve became an ambassador for LERN because his mother developed lymphedema due to breast cancer. She had (presumably) axillary lymph nodes removed leading to arm lymphedema. You can read more about his story here (https://lymphaticnetwork.org/news-events/steve-guttenberg-nih).

¹ https://en.wikipedia.org/wiki/Steve_Guttenberg

The Lymphedema Treatment Act is an important piece of legislation that has been seeking passage for several years. You can find out more about it here.

The Act was initiated by Heather Ferguson who had twin boys. One of her sons was born with primary lymphedema. The lack of insurance funding to cover lymphedema compression supplies (i.e. compression garments) was recognized as a problem. Heather sought to create change.

Change began locally in her state of North Carolina. She has been seeking coverage nationally by changing Medicare law to require coverage of compression garments for lymphedema. Medicare will set a precedent for other insurance carriers to follow.

You can find out how to contact your Congressman or woman via the below video. I did. And I recently heard back from Representative Burgess in the attached letter. Take a moment – literally less than 5 minutes – to make your voice heard and create change.

Last month, I saw a news posting about Bachelorette’s Cam Ayala.

He had just had an amputation due to lymphedema. I hadn’t heard of Cam until learning of his experience with lymphedema. (I don’t watch the Bachelorette; I actually don’t even own a TV!) It’s unusual to hear of an amputation being needed as a result of lymphedema. Is it possible for lymphedema to require amputation?

I am not aware of any cases in which lymphedema alone was the cause of an amputation. I would be certain that there have been a few amputations when no other treatment option was available or when no one knew better. There are many cases (particularly in a leg) when other factors may be the root cause. For example, someone may have poor arterial blood flow (which carries oxygen & nutrients to tissues). Such a condition is peripheral artery disease; ischemia could cause tissue death & require amputation. Someone may have a severe accident, damaging a limb so extensively that salvaging the extremity isn’t possible due to blood vessel damage, lymphatic vessel damage, tissue damage, etc.

According to the government site (Medline.gov),¹ factors that may lead to amputation include:

• Infections
• Tumors
• Severe burns or frostbite
• Nonhealing wounds
• Loss of function or loss of sensation (increasing risk of injury).

Cam was diagnosed with primary lymphedema at age 11. I wondered about the possibility of lymphangiosarcoma. But apparently, he had had numerous knee surgeries (17 in total) on his primary lymphedema leg in the past. The 16^th was a knee replacement that wasn’t done correctly.² He also had a blood clot at some point & developed osteomyelitis – a serious bone infection. With lymphedema, the ability to fight infection is already reduced in the involved extremity.

The June 2022 surgery was an elective, above-knee amputation. He opted to have it done due to concern of a potential recurrence of osteomyelitis. If such infection did reoccur, a prosthetic device would likely be more difficult to fit, and a higher amputation would be needed. You can listen to an interview just before his hospital discharge here (skip to minute 7.45).

In another video,³ he talks about the challenge of people’s perception (skip to second 16). We live in a society that seems to demand perfection & social media exacerbates the illusion of perfection. He suggests what he’s learned is to “find your peace [with your own shortcomings], give yourself grace & surround yourself with people who are going to build you up, not just bring you down.” It reminded me of author Bronnie Ware’s findings.

Bronnie worked in palliative care. She summarized common themes in a book called Regrets of the Dying. They included:

1. Having courage to live a life true to yourself & to not live by what others expected of you.
2. Not working so hard you neglect relationships that matter to you.
3. Having the courage to express your true feelings.
4. Staying in touch with your [real] friends.
5. Not letting the fear of what others will think prevent you from enjoying life & laughing.⁴

They boil down to relationships (with others & yourself) & not letting fear limit your potential. Having spent some time with people who were facing their own mortality myself, it’s sobering to recognize how the anticipation of an impending death causes many unimportant things to fall away. Sometimes hitting rock bottom (as Cam talks about) can create a similar moment when things of lesser importance fade, & you take stock of life. You take time to reflect. You take time to listen. And your perspective shifts.

For anyone who may have an amputation & thinks their athletic life is over, check out these inspiring people: https://www.scheckandsiress.com/blog/10-famous-amputees/.  Visit the Amputee Coalition⁵ & the Challenged Athlete Foundation⁶ to find out about sports opportunities for people who have had amputations.

Rest assured, lymphedema alone is seldom a reason for amputation. Cam had several surgeries, a blood clot, osteomyelitis and a poor knee replacement that culminated in an elective amputation from which he is recovering. He anticipates getting his prosthesis around July/August. Best wishes to Cam in his recovery & going on to lead an amazing, happy, fulfilling life as I am certain he will. Heck – he’s already got tickets to see Lauren Daigle in September! Enjoy that mid-September concert, Cam!

References

1 Medline (https://medlineplus.gov/ency/article/007365.htm)
2 https://www.youtube.com/watch?v=Ia3XjXxRqE8
3 https://www.youtube.com/watch?v=sKG6OkIZNlg
4 https://bronnieware.com/blog/regrets-of-the-dying/
5 https://www.amputee-coalition.org/resources/adaptive-sports-programs/#:~:text=U.S.%20Paralympics&text=Sports%20available%20for%20individuals%20with,sled%20hockey%2C%20and%20many%20others
6 https://www.challengedathletes.org/athletes/

Turner Syndrome

This month, we’re featuring a cause of primary lymphedema called Turner syndrome. There will be a video series that follows with two interviews: one with a therapist (Jana) and one with a patient (Dorothy) who has Turner syndrome & lymphedema. Turner syndrome may be called:

• 45,X syndrome
• Bonnevie-Ullrich syndrome
• monosomy X
• Ullrich-Turner syndrome.¹

The condition affects 1 out of every 2,000-4,000 live births.² The condition is a genetic mutation of the X chromosome, a sex chromosome.

Biology Refresher

It may have been a while since biology class, so let’s refresh. A chromosome is a long chain of DNA that has part or all of the genes (genetic instructions) for an organism. People have 23 pair (or 46 chromosomes in all). Most are identical in men and women – except two:³ the X and Y sex chromosomes.  Sex chromosomes determine the sex of an individual. Men have both an X and a Y. Women have two X chromosomes.

In Turner syndrome, part or all of the X chromosome is missing in women. (This condition only affects women though there is a similar but different version called Noonan’s syndrome that affects men.⁵)  As a result, women do not develop normally.

The severity and extent of outcomes vary. Frequent symptoms include a short stature; short, webbed neck; low hairline; eyelids that turn downward; heart and kidney abnormalities; narrow palate; infertility; difficulty with spatial relationships; and, lymphedema (among others).

Primary vs Secondary Lymphedema

For our purposes, we’ll focus on lymphedema. The most common form of lymphedema is secondary. This means the lymphedema is due to a cause outside of itself such as: cancer, cancer treatment, venous insufficiency, obesity, infection, lipedema, surgeries, etc. The primary source results in structural damage to the lymphatic system. This structural damage causes impairment of fluid transportation back to the heart. Swelling results in the area damaged and may impact the entire extremity and trunk component that drains to that lymph node region. The swelling is chronic, inflammatory and will likely get worse over time.

In primary lymphedema, there is no cause outside of a congenital birth defect that results in abnormal lymphatic vessels and/or lymph node development. This abnormal development renders the lymphatic system in the affected area incompetent. As a result, swelling occurs which is chronic, inflammatory and often gets worse over time. Examples include Klippel-Trenaunay syndrome; Prader Willi; Milroy’s disease; Meige Disease; Noonan syndrome; Turner syndrome; Yellow Nail syndrome; Trisomy 13, 18, 21 and 22; and others.⁴

Because of the more severe complications of Turner syndrome that present as life-threatening, lymphedema is often an afterthought until a woman gets older and lymphedema progresses in severity (usually in the lower legs/feet). A common problem is finding shoes that fit as you’ll hear in the patient interview. Hands can be involved, too, but this may resolve as a child ages. Below are two images of Dorothy as a child.

References

1 Rare Diseases
https://rarediseases.org/rare-diseases/turner-syndrome/#:~:text=Consequently%2C%20in%20the%20past%2C%20Noonan,differences%20between%20the%20two%20disorders
2 Turner Syndrome Society
https://www.turnersyndrome.org/about-turnersyndrome
3 Medline
https://medlineplus.gov/genetics/understanding/basics/howmanychromosomes/#:~:text=In%20humans%2C%20each%20cell%20normally,in%20both%20males%20and%20females
4 Foldi, M, Foldi, E. (2006). Foldi’s Textbook of Lymphology (2^nd ed.), p. 509-510. Germany: Urban and Fisher.
5 https://rarediseases.org/rare-diseases/noonan-syndrome/

This is Part 2 of the first person I ever knew with lymphedema: my high school English teacher (Jackie Miller). Doctors weren’t sure why her leg was swelling. Was it cancer recurrence? A blood clot? An ovarian cyst? Scar tissue? Or possibly something else? (Jackie was referred to as Janie’s earlier in the blog for anonymity. With the family’s permission, her real name is being used.)