Health questions

Christelle Bergeron MD, FRCPC
Pulmonologist
Assistant professor

Director of the clinic of
adult cystic fibrosis
Co-director of the program
Of pneumology
CIUSSS de l'Estrie — CHUS

Faculty of Medicine and Health Sciences — University of Sherbrooke

Are there risks in consuming lactofermented products and/or bacteria-based products (e.g. kombucha) for CF, whether transplanted or not?

Answer:

Lacto-fermented foods are products for which a lactofermentation process (or lactic fermentation) has been used for their preservation. It is a process that consists in letting food macerate in the absence of oxygen, which encourages the proliferation of lactic acid bacteria (e.g.: Lactobacillus, Pediococcus, Bifidobacterium). Lactic acid bacteria survive in non-oxygenated or low-oxygen environments and produce lactic acid.

Lacto-fermented products have a high antioxidant content that would reduce oxidative stress and inflammation. In addition, the probiotics they contain could have a positive impact on the intestinal microbiota.

However, since these are foods that contain numerous bacteria and yeasts, their consumption is strongly discouraged for immunosuppressed people, including fibrocystic transplant patients, and also for pregnant women. Indeed, these people risk a gastrointestinal infection against which their body would have difficulty defending itself.

For non-transplanted fibrocystic patients, the risk of contamination is lower. Moreover, many consume probiotics when they are under antibiotic therapy for pulmonary superinfection. However, caution is required, especially with homemade products.

Do you have any tips for preventing or relieving kidney stones in CF?

Answer:

Nephrocalcinosis, i.e. the presence of calcium deposits in kidney tissue, is very prevalent in the fibrocystic population. Indeed, some studies have confirmed the presence of nephrocalcinosis in up to 92% of kidney samples taken from fibrocystic children. Nephrolithiasis (i.e. kidney stones or kidney stones) is also common and reported in 3 to 6% of fibrocystic patients. Most of the time, calcium oxalate stones are found in these individuals. However, few data are available in the literature on this subject and the mechanisms underlying these clinical manifestations are even more or less understood.

However, several hypotheses have been suggested over the years to explain this phenomenon. First, pancreatic failure leading to reduced fat absorption may cause fatty acids to bind more to calcium in the gut. In doing so, less calcium would be available to bind to oxalate and hyperoxaluria would result. The malabsorption of fats and the accompanying diarrhea would also lead to hypocitraturia, which would facilitate the formation of calcium oxalate and calcium phosphate crystals. Finally, frequent antibiotic treatments could eliminate the presence of the bacteria. Oxalobacter formigenes in the intestine, thus decreasing oxalate metabolism and promoting the formation of stones.

There are no guidelines for the management of nephrocalcinosis and nephrolithiasis in patients with fibrocystic fibrosis, but based on the various mechanisms described above, some recommendations can be made. It is essential to stay hydrated, that is, to drink enough to make the urine pale and odourless (usually 2-3 L/day, preferably water). In addition, it is important to take pancreatic enzymes well and to have the right dosage during meals and snacks in order to minimize malabsorption and its impact on crystal formation. It is also advisable to avoid foods rich in oxalate (e.g. spinach, beets, rhubarb, figs, nuts, soy, strawberries, strawberries, dark chocolate, etc.). Finally, it is necessary to prevent the deficiency of pyridoxine (vitamin B6) and avoid too much intake of ascorbic acid (vitamin C). It is also advisable to have blood and urine tests done once a year in order to detect potential kidney abnormalities.

How do you recognize thrush (fungal infection of the mouth)? What medications are most at risk? How to treat it?

Answer:

Oropharyngeal candidiasis, more commonly known as thrush, is an infection caused by Candida, a fungus found in normal gastrointestinal and genitourinary flora. Infection is detected by the presence of whitish plaques in the mouth, on the palate, on the tongue, or in the oropharynx. These plaques don't go away if they're brushed with a toothbrush or scraped with a tongue depressor. The diagnosis is usually clinical, but it can be confirmed by scraping the lesions and then making a KOH (potassium hydroxide) preparation in order to visualize the yeasts. Other symptoms are sometimes present such as the sensation of having a pasty mouth, loss of taste, and pain when swallowing.

Individuals at greater risk are elderly people wearing dentures, those receiving antibiotics or chemotherapy, and those with an immune deficiency (e.g. AIDS). Drugs that are more at risk are inhaled glucocorticoids (e.g. Flovent).^MD, Alvesco^MD, Pulmicort^MD, etc.). For this reason, it is important to use the inhalation chamber equipped with a metered-dose inhaler and to rinse your mouth after taking these inhalers.

Thrush is usually treated quite easily with an antifungal agent (nystatin) by gargling and swallowing it four times a day for about ten days. For more refractory cases, it is possible to prescribe an antifungal drug from the azole class in tablets, most often fluconazole. However, caution should be taken as there are drug interactions to consider with fluconazole, in particular with CFTR modulators (Kalydeco).^MD, Orkambi^MD, Symdeko^MD, Trikafta^MD).

Taking multiple medications often leads to bad breath. Do you have solutions?

Answer:

Before concluding that bad breath (halitosis) is caused by medication, you need to make sure that other potential problems have been ruled out. The best is to consult your dentist, who can do a complete examination of the oral cavity and ensure the absence of pathology (e.g., gingivitis, periodontitis, abscess, etc.).

If medication is really responsible for bad breath, it is most often because it causes dry mouth (xerostomia). Symptoms can be improved by being properly hydrated, by stimulating saliva production (for example by chewing gum), or by using artificial saliva.

Other interventions may be tried: reducing coffee and alcohol intake, ensuring exemplary dental hygiene (including the daily use of dental floss), cleaning the back portion of the tongue when brushing teeth, and using mouthwash to gargle at bedtime.

Are there resources available for the management of premenopause in cystic fibrosis?

Answer:

Unfortunately, there are no resources specific to people with cystic fibrosis for managing menopause and perimenopause.

In the general population, the usual age at which menopause occurs is between 45 and 55 years. Few data are available in the literature on the subject of menopause in the fibrocystic population. The exact age of its onset in these patients is unknown. However, a person who develops symptoms such as hot flashes, palpitations, chills, vaginal dryness, loss of libido, or sleep disorders before the age of 45 should discuss this with their primary physician.

If necessary, the family doctor or the doctor at the cystic fibrosis clinic may request a consultation with an endocrinologist or gynecologist-obstetrician in order to complete the evaluation, to do your hormonal assessment and to determine if hormone replacement therapy treatment would be necessary.

I would like to have breast surgery, what are the factors to consider before undergoing cosmetic surgery?

Answer:

Like any medical procedure, every surgery has risks. The risks involved in breast surgery should be discussed with the surgeon who will perform the procedure. Among other things, they include the risks of bleeding and infection as well as the risks associated with anesthesia.

Ideally, any surgery should be discussed with the doctor at the fibrocystic clinic to optimize the management of the disease and other comorbidities before the procedure. In particular, it must be ensured that there is no pulmonary superinfection in progress and that respiratory function is sufficiently preserved to be able to tolerate the intervention. In some cases, if lung function is too poor, local or regional anesthesia may be recommended rather than general anesthesia. Consideration should also be given to the management of other comorbidities such as diabetes associated with cystic fibrosis, for which a protocol and an adjustment of treatments to control blood sugar levels before, during and after surgery will be required. Finally, it is important to know the length of hospitalization and recovery in order to prevent foreseeable potential complications. For example, if opioid-type pain relievers (e.g. morphine, hydromorphone) are administered in the postoperative period, constipation/DIOS problems, which are common side effects, should be prevented. Also, if mobilization and respiratory physiotherapy are limited during convalescence, close follow-up by the physiotherapist team during hospitalization should be provided. It is sometimes even necessary to start prophylactic antibiotic therapy, that is to say to prevent pulmonary superinfection, since secretions will tend to accumulate in the bronchi and predispose to infections during the first few days following the intervention.

The main point to remember is that it is always best to discuss such an intervention with your surgeon and doctor at a cystic fibrosis clinic so that they can have a concerted and as safe approach as possible.

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