Radiation-Induced Fibrosis Risks: Connective Tissue and Autoimmune Disorders
Hello,
I am 53yrs and was diagnosed with Invasive Ductal Carcinoma (IDC) in May 25. I had a lumpectomy and SLNB in June. Histopathology: ER+ (95%); PR+ (2%); HER2 neg; Ki67 10%; N0 (node neg); I had two further surgeries. One for a bleeding complication and the other for a re-excision of the anterior margin. Histopathology: Clear margins.
I recently attended consultations with two radiation oncologists (RO). I was advised that I was at higher risk of developing radiation-induced fibrosis (RIF) as I have a connective tissue disorder - Ehlers Danlos Syndrome (hypermobile subtype hEDS). I also have exaggerated inflammatory responses and multiple allergies - suspected autoimmune issues.
I assumed that the term fibrosis was referring to tissue fibrosis but after some research have come to understand that this also includes fibrotic conditions of the lungs in varying degrees.
Thank you to everyone who has posted regarding their experiences with RIF on this forum to date. Given I am at an increased risk I have asked to delay the RT commencement date while I weigh-up the risk of local recurrence against the risks associated with the potential late effects associated with RIF.
It is already a daily struggle living with hEDS so am taking some time to carefully consider my options. I have completed the CT planning scan for the treatment and have an appointment with the RO on Monday. At present I understand the prescription is 40gys (dose) in 15 fractions (sessions).
I would like to invite forum members with/without connective tissue and/or autoimmune disorders to share their RT outcomes. It would helpful to include if possible:
1.Type of RT e.g. Intensity Modulated Raditation Therapy (IMRT); Volumetric Modulated Arc Therapy (VMAT) with/without deep inspiration breath hold (DIBH). I think this is the standard treatment.
2.Total dose if known and number of fractions (sessions)
3.Immediate side effects e.g. pneumonitis; nausea; swallowing pain; burns; blistering; none etc.
4.Late-onset effects e.g. pulmonary fibrosis; tissue fibrosis etc.
5.Treatments (prednisone etc) and outcomes
6.Right, left or both breasts
7.Prone (lying stomach) or supine (lying back) position
It would also be helpful to know if anyone chose to omit the RT and why.
Thank you all in advance. I am finding the decision-making process difficult and any information you can share would be very welcome.
Healing wishes
🌷
Klio
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Hi @klio44,
According to research reports radiation reduces the chance of local recurrence from 15 -25% to 5-8% over 10 years. It does not affect distant recurrence.
Currently the only disease where radiation is not recommnded is Sjogrens. However, according to my ILD pulmonologist, breast cancer RT is not a good idea for anyone with an AD which could affect the lungs. Radiation triggers transforming growth factor beta 1 which heals damaged tissue. In those with an AD TGFB1 does not turn off when it should and continues to create scar tissue as it spreads. It can shrink and harden breast tissue, scar interstitial lung tissue preventing O2/CO exchange, and after nodal treatment cause brachial plexopathy leading to arm pain and dysfunction.
Usually if the lungs are affected you develop a severe cough caused by pneumonitis about 3 months after RT which a short couse of steroids will cure. In my case this never happened and when symptoms showed up six months later a chest CT showed organizing pneumonia (a process not a disease) and scarring throughout my right lung, prompting a stat referral to an ILD pulmo. I figured I still had one working lung but my pulmo warned it can spread to the other lung with the obvious consequence. I was on long term oral steroids and O2. Fortunately I made it through with my left lung still intact. RIPF is rare (.03%), can occur anywhere from six months to two years after rads, and can resume in the future because of a lung infection, drug reaction or intubation. I live like it's still covid lockdown.
My pulmo thought a lung cancer RO would have advised skipping RT since my upper GI bleed, silent GERD and Barret's esophagus were red flags. I apparently inhaled stomach acid into my lungs while sleeping causing subclinical ILD. Radiation scatter is mild but it caused rapid regrowth of my excised thyroid goiter, esophageal dysplasia and minor brachial fibrosis. After a recent bout of drug induced liver injury I now have brachial neuritis causing a numb right hand and arm from my biceps to finger tips. A neurosurgion thought the radiation plexopathy made that location vulnerable.
Needless to say radiation has greatly affected my life but I'm still here hoping PT can re-enervate my right hand. I believe that radiation is a good idea for most people but if I knew that I were at risk there is no way I would go anywhere near a LINAC machine. I would suggest consulting your rheumatologist or whoever manages your hEDS and is much more familiar with your condition than a RO. Good luck with your decision and further treatment.
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Klio -
I'm presuming you had a mastectomy? Radiation is almost always recommended after lumpectomy. Mastectomy is more of a murky area, depending on age, grade, size of tumor, nodal involvement, etc.
It's good that two RO's are warning you about EDS., and it sounds as though they would plan carefully to keep it as safe as possible for you. I would be cautious, though, about relying too much on anecdotal evidence from members regarding protocols and side effects. First, many treatment decisions are standardized - for instance DIBH is standard on left-sided tumors. Second, each individual reacts differently to treatment and one person's experience won't necessarily be yours. Your providers can give you estimated risks of side effects, but you might be the one in twenty who does (or does not have) that side effect.
I would ask, if you haven't already, what your estimated risk of recurrence is with and without radiation. Maggie's info about risk reduction is good, there is also some evidence that for every four local recurrences prevented, one distant recurrence is. But those are pretty small numbers.
I would advise asking if they plan on treating your chest wall only, or also three sets of nodes: axilla (the ones under your arms); supraclavicular, above your collar bone; and internal mammary nodes (IMNs) , under your sternum. Each additional group of nodes increases the radiation dose. The internal mammary nodes, in particular, increase heart and lung dose. Only 9% of local recurrences occur there, but if your tumor was toward the sternum (as opposed to the side of your chest) or you were node positive, they might recommend covering them. In general, you want to try and keep the heart dose at under 2.5Gy.
Usually, if pneumonitis occurs and is treated early it is fine, but clearly Maggie's experience was different. I would also add that I had substantial chest wall fibrosis despite no known pre-existing conditions. This prevents my lung from fully expanding. I am just starting hyperbaric oxygen therapy, which both my pulmonologist and physiatrist believe will help. Studies are very limited, but you can look up last year's HONEY trial. Not certain it would be compatible with EDS, though.
I remember what a difficult decision this was. Hoping others chime in with wise words, and that you can come to a decision you are at peace with.
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Hi @maggie15 ,
Thank you for sharing your experiences . Articulating the details so thoroughly is very helpful. It highlights the limitations of oncologists in terms of understanding how pre-exisiting conditions in patients interact with RT in the immediate and longterm.
Grateful to you for listing the other medical conditions that may have been contraindicated for RT. I have a hiatus hernia, mild mitral valve prolapse , GERD and a multi-nodular thyroid goitre. I also develped a chronic cough last year. Due to hEDS and suspected autoimmune issues I can relate to your living like it's 2020.
I appreciate your informative, wise and kind words. I hope that you have success with the re-enervation of your right hand.
Sending you postive healing energy Maggie.
Klio 🌷
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Hi @ratherbesailing ,
Appreciate your comment and opening question. I have edited my original post accordingly - thank you.
Grateful to you for suggesting questions to raise with RO. I have a meeting with them next week to discuss the outcome of the measurement plan and scan. Being as prepared for this meeting as possible will be helpful.
Thank you for sharing your experience regarding chest wall fibrosis despite not having any known pre-exisiting conditions. I will look up the HONEY trial - all information is useful.
The information, suggestions and kind words you wrote are sincerely valued. Thank you for acknowledging what a difficult decision this is. I hope the hyperbaric oxygen therapy makes a positive difference and I wish you all the very best.
Klio 🌷
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It's been a year and a half since my RT and I don't remember many details other than being on my back, and having about five weeks of radiation for left breast and a positive auxiliary node
I found this post because I have a worsening cough that I am trying to make sense of.
I was diagnosed with sarcoidosis of the lung at least 25 years ago, with a presenting symptom of coughing that recurred once or twice a year, usually when I had a cold.
Long story short in addition to the radiation I am also on Anastrazole and Verzenio, both of which I think list cough as a side effect, and the cough has gotten a lot worse; usually 300 coughs a day and sometimes six hundred or more according to an app I'm using.
I have a pulmonologist who tells me I have asthma, and my cancer team has attributed changes on CT (Mild reticulation and consolidation, trace traction bronchiectasis, is most likely radiation fibrosis. Minor peripheral chronic atelectasis or scarring in the right lower lobe posterior/posterior medial aspect) as due to radiation and insignificant.
In addition to the history of sarcoidosis I also have vitiligo, both of which are sometimes considered autoimmune, I have some type of joint ailment that seems to be inflammatory.
I am about to have a follow up CT with contrast to see if things are stable.
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Hi @shrinkrap59, Based on your radiology report your cancer team seems to have correctly diagnosed you with radiation fibrosis. Since you have lung sarcoidosis, a type of ILD, you were predisposed to getting pulmonary fibrosis, another type of ILD, from radiation. The fibrosis causes nerves in the lung which are normally covered to be exposed to air, hence the cough. An inhaled corticosteroid can reduce the inflammation and lessen the annoying cough. My cough got so bad that it would cause me to pass out and I had to stop driving for a while. This cough syncope happened during a pulmonary function test and my oxygen level briefly went down to 40%. Increasing the dose of my inhaler stopped it.
The best way of imaging this is a HRCT (high resolution ct) without contrast. Many pulmonologists are not familiar with interstitial lung disease. The Pulmonary Fibrosis Foundation has a list of hospitals that have ILD specialists. The one nearest to you is UCSF. UC Davis in Sacramento is another location. Both are a trek from your home but that seems to be the price you pay when you live in a scenic rural area (I drive 3 hours each way.) Maybe your current pulmo would be willing to do some research or arrange a virtual consult for you. Stopping the cough really improves your QOL. All the best.
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Thank you! I guess that the radiation fibrosis looks different from the interstitial lung disease said to be a side effect of CDK 4/6 inhibitor meds like Verzenio? I believe I have heard that referred to as "pneumonitis" as well, but I understand the radiation one is more localized, while the drug related one is more systemic. That sounds worse in terms of prognosis than radiation fibrosis! I've read that a history of sarcoidosis suggests a tendency for inflammation and might mean a greater risk for an inflammatory response. I just found a recent paper describing a potential additive effect. Not sure I should read it! 🤔
My last CT (regular and without contrast) before cancer treatment showed "Mild septal reticulation and ground glass at the lung bases …can be from atelectasis or vascular congestion."
I was switched from Advair to Wixela for "asthma" about a year ago, and Spireva was added in April because the "asthma" wasn't responding, but I felt the Spireva was making things worse.
My medical oncologist mentioned a referral to UCSF but it is a bear to get to. Stanford was mentioned too, but UC Davis (I assume in Sacramento) would be better. I go there for the majority of my treatment. It usually doesn't take more than an hour.
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They are often able to tell the cause of pulmonary fibrosis by the scarring pattern and other visual characteristics. Reticulation is net-like scarring. When it does not start at the base of the lungs (like in your pre-cancer treatment CT probably from the sarcoidosis) it is generally caused by radiation. Idiopathic PF (most common but unknown cause) has a honeycomb pattern of scarring which starts at the base of the lungs. Drug induced PF has a mosaic pattern of scarring which is spread throughout the lungs in patches. Fibrosis can't be cured but there are drugs that may slow progression if it continues to spread.
Pneumonitis is inflammation of the lung tissue not caused by bacteria/virus. It can often (but not always) be cured with oral steroids. It's better in that if cured it leaves little damage but worse in that if it can't be stopped it wipes you out quicker than fibrosis which destroys your lungs more slowly.
Consolidation is fluid/pus/cells that fills up your lung and looks solid on CT scans. Ground glass opacities are hazy, slightly cloudy exudates in the lung. ILD pulmonologists are pretty good at figuring out what is going on by looking at CTs or doing a bronchoscopy biopsy.
My PF has been pretty stable since oral steroids stopped the progression 5 months after it was diagnosed (7 months after finishing rads.) I did have some recurrence in October so am waiting for a CT at the end of January to see if that stopped. I'm sure glad we have two lungs. However, my pulmo did inform me it can move to the other lung. Having ILD makes you much more likely to develop lung cancer so that's something you need to be aware of. Your PF seems to be pretty mild and it often remains that way.
Wixela contains fluticasone propionate which is a corticosteroid. There were two clinical trials proposed in 2015 to see if fluticasone propionate HFA would prevent the spread of RIPF. They never ran due to lack of participants but the 880 mg I take over the course of a day helps my cough and may be doing more. A higher dose of Wixela might improve your cough. There are other meds out there, too.
Since you have sarcoidosis as well as RIPF and are taking Verzenio which can cause pneumonitis your lungs are complicated. UCSF has one of the best ILD programs in the country and 15 specialists so the bad drive might be worth it (my sister lives in SF and my brother in Santa Rosa so I get what you mean.) There is just one ILD specialist at Davis. I hope you get an appointment with a doctor who can help.
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Thank you!
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CT with contrast improved, but cough is not! Happy there is no evidence of interstitial disease and will be putting the ball back in the pulmonologist' court.
Coincidentally I am reducing Verzenio from 100/150 to 100/100 because of GI concerns.
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Good news on the CT improvement. I hope the Verzenio dose reduction improves your GI issues and they can find the cause of / treat your chronic cough.
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