August 10, 2020 at 9:05 pm #15430
I frequently hear CF referred to as a lung disease. While I understand that for those who aren’t informed about CF, calling it this is a quick and easy way to describe it. But also, calling it this can be incredibly misleading. As we know, CF is a whole-body disease!
I have friends within the community who have never been hospitalized due to lung issues, but regularly have GI-related hospitalizations. Personally, my sinuses have been far more problematic in recent years than my lungs have been. CF is such an individualized disease and the ways in which it manifests itself are as unique as each of us living with it.
In what ways does CF affect your body? Do you have other organs that are affected more than your lungs? What symptoms do you have that differ from the “classic” explanation of CF?
August 12, 2020 at 8:19 pm #15454Tim BlowfieldParticipant
My wife Reva is one such ‘Atypical” CF’er. Diagnosed at age 67 just 11 years ago she had 20 issues of a checklist of the 25 most common issues of CF. She had lost her large intestine some 10 years earlier due to severe and painful hirschsprungs-like bowel disease, (a later review of the pathology agreed it was consistent with CF), had Atrial Fibrillation diagnosed in 1996 with Cardiomyopathy evident in 2005 (not recognised as being CF caused till very recently). After having 4 children (1972 -1986) she had a hysterectomy as she was having painful dysmenorrhoea (again probably exacerbated by the undiagnosed CF). In the 1960’s and ’70’s she had issues coping with altitude first in southern India and later in Kenya at 7,500feet. In 2007 she was diagnosed with a Parathyroid Adenoma and again 2 more in 2014 (caused severe deep leg pain ‘in bones’).
Since diagnosis in 2009 issues with hypokalaemia (low blood Potassium) have been common. Throughout these later years her lung function has maintained a relatively good 60-80%. Dry eyes and issues with saliva and tears are ongoing issues.
We have struggled to make sense of it all but as we understand more we realise that many, even most, of her issues are CF related. That CF is not just a disease of the mucous secreting globoid cells but it is the faulty Chloride transport that not only affects the mucous but also affects the electrolytes within the cells. Those faulty electrolyte levels affects the function of so many organs eg: the Adrenal Glands (regulation of corticosteroids and mineralosteroids), muscle function (hypokalalaemia) including Heart Muscle (cardiomyopathy), Parathyroid Glands (reacting to increased intracellular Calcium).
CF is indeed a whole body disease or rather I prefer to use the term disability.
To view it just as a ‘lung’ disease or even a ‘lung and bowel’ disease is to short change our people living with it.
In Research; monitoring electrolytes (eg serum Potassium) may be a more rapid method of seeng whether a drug is beneficial.
August 13, 2020 at 6:07 pm #15462
Tim, what a thorough and thoughtful response! As of late, there’s been quite a bit of talk about this on the forums, and what you’ve shared here about Reva is the perfect example of why it can be detrimental to have a narrow view of CF. As you said, even describing CF as a “lung and bowel” disease is “short changing” the people living with it. (I particularly love how you worded that.)
I am always fascinated by late diagnosis stories. If I’ve read your response correctly, Reva is now in her 70’s? Incredible. Thank you, Tim, for sharing your thoughts with us here. I have been truly fascinated and enlightened by your words.
August 14, 2020 at 12:52 am #15466Tim BlowfieldParticipant
78 last week. She tells her CF Dr Prof John Wilson that she will live till 103. There was a lady in California who was diagnosed at 91 after her son was diagnosed at 57.
We understand that Reva is probably third oldest in Australia.
August 14, 2020 at 11:39 am #15469
Tim, this is absolutely amazing!
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