- Pneumothorax (9/97)
- Meconium Ileus (MI) (5/97)
- Meconium Ileus Equivalent (MIE)
- Sinus/Nasal Problems
- Arthritis (joint pain)
- Hemoptysis (coughing up blood) (10/97)
- MESA – A Chance at Fatherhood
- Fertility and Prenatal Testing (8/97)
- Liver Dysfunction (9/97)
- Excess Stomach Acid
- Hypoglycemia (8/97)
- Can CFers Have High Cholesterol?
- Gallbladder (5/97)
- Kidney Stones
– denotes air in the pleural cavity due to disease or injury to the lung tissue or due to puncture of the chest wall.
Questions & Answers:
Q. Can the same lung collapse more than once?
A. I’ve had a few lung collapses. Well, it was the same lung four times, but in my case it never fully re-inflated until the second chest tube.
Q. Is there anything I can do in a preventative sense like deep breathing to keep expanding the lung?
A. As far as I understand it, there is nothing you can do. Lung collapses occur in the “normal” population as well. One of those flukey life things. In people with CF you just know what caused the hole:).
Q. I keep yawning-is that just a result of having all that air pumped inside?
A. Actually you had air sucked out of you. The tube creates negative pressure which is what re-inflates your lung. I found the chest tubes a really draining experience. It took me a couple of months to get my strength back. Maybe you’re tired;).
Q. Now that I’ve had one pnemo, does this mean I am risk for having more?
A. I don’t know what the statistics are. I think it depends on how big the original hole was and how well it healed. I had recurrant collapses because it never healed properly. If you do have recurrant collapses there is a procedure call pluerodesis (sp?). Essentially they glue your lung to the lung wall lining. I don’t say this to scare you, but to reassure you that there is something that will fix it permanently. The pleurodesis was just like a regular chest tube as far as discomfort goes-no big thing.
Q. I am also wanting to go on a vacation next week(driving, not flying). The docs are saying that I am not needing to limit my activities but I am curious what others think, especially any experienced collapsers.
A. I was pretty inactive for a couple of months, but that was because I didn’t have the strength to do much. I would say follow your gut instinct. Do what you think is reasonable. The only things MY doctor told ME not to do were activities that involved air pressure changes: diving, flying…
Funny that you should mention pneumothorax! Yesterday, I was discharged from the hospital after 6 days with one. It occurred spontaneously, as they usually do. What a pain. I was on self-adminstered demerol for a couple of days – just press a button and WHAMMO, the drug hits. Good stuff, I must say. Don’t know why it hurt so much this time. 20 yrs ago I had one on the other side, but don’t recall it being as much of a big deal. This was only deflated about 15%, but then my lungs are 20 yrs older and 20 yrs stiffer. The doctors elected to try to reinflate the lung via chest tube, which failed the first time, as after the suction was stopped it deflated again. The second time it stayed up and I was sent home after 2 more days. They could have used more drastic measures, to “glue” it as mentioned in another post, but they said that the resulting schlerosis at the point of attachment would make my impending lung transplant that much more difficult from a surgical point of view. Seems they would then have to physically scrape off that part of the pleura which had sclerosed – sounds painful and messy. By allowing the lung to reinflate under vacuum and reattach naturally, they avoided that, even if it did take a few more hospital days, (which in these days of (mis)managed care, was pretty good of them to think as doctors and not accountants!) They are a good bunch. Home now and on the mend. Found out I was #4 on the list, up from 95 less than a year ago. Still a category 4( stable). My only restrictions are no heavy lifting for a couple of weeks. Seems a sensible precaution.
Meconium Ileus (MI) (5/97)
– This condition is found only in the newborn, as opposed to MEI (see below) which is found in more mature persons. Says one medical professional: I am not surprised that you have not had a consistent answer concerning the cause of MI. The fact is that no one knows the reason why about 15% of PWCF are born with MI. I think it must be a combination of reasons – not just pancreatic insufficiency although that no doubt plays a part; I am sure that there are other factors such as water absorption across the intestine and bowel that are also important.
Another person adds: Meconium Ileus is not caused by pancreatic insufficiency as many are led to believe. It has to do with (as everything in CF) those nasty CFTR proteins in the distal small intestine. In the case of the intestine, Cloride can’t be secreted into the lumen of the intestine and water can’t follow the cloride to lubricate the intestine, thus causing build-up of muck (meconium). It seems to always occur at the ileum.
Meconium Ileus Equivalent (MIE)
– The distal intestinal obstruction syndrome (DIOS) is a ‘meconium ileus equivalent’ that occurs in adolescents and adults with CF. It is the consequence of the abnormally viscid mucofeculant material in the terminal ileum and right colon, where the fecal stream is normally liquid. Typical features are recurrent episodes of right lower quadrant pain with palpable mass in the right iliac fossa. Symptoms are exacerbated by eating. Prevention: One of the best ways to prevent a bout with meconium ileus equivalency is to take mineral oil about 2-4 tablespoons as needed to maintain regular bowel movements. Treatment: Golytely (an American brand-named drink) will sometimes work but tastes vile. A little sprinkling of Crystal Light powder (a drink mix) made it much more agreeable. And having it ice cold helps, too. It sorta kills that nasty fishy taste. If the bout has progressed to the point of vomiting taking anything by mouth will not work. By then a barium or other form of enema may be needed. NOTE: At hospitals where CF may not be that well known (indeed it happened to me at Children’s in Boston which should know better) a surgeon might be called in and surgery recommended. Surgery for a bowel impaction, MIE or DIOS is counter-indicated. The scars caused by surgery on the area will induce future episodes of impaction.
About 20% of PWCF develop nasal polyps (growth of extra tissue in the nares). Allergies, infection, and polluted air situations (where is it not?) cause the polyps. Sinus infections drain into the lungs and are difficult to treat successfully. Symptoms of nasal polyps may include: “difficulty breathing through my nose and sometimes when I blow it, it bleeds profusely”; “I had SEVERE headaches”; “often a doctor can see the polyps during a regular check up when he/she checks your nose”; inability to smell. Treatments may include non-surgical and surgical procedures; ESSAL combines both approaches. It’s important to go to an ear, nose, and throat specialist (ENT) who knows CF.
An adult CFer says: “If any children have sinus polyps keep in mine that with out surgery you can facially deform the child. As a child I had surgery every year until third grade. The Docs then figured “what’s the use, the polyps will come back anyway.” So from forth to eighth grade I didn’t have surgery. By ninth grade I looked as if my face was swollen across the bride of my nose all the time. After surgery the swelling never went away. I still look that way. It turns out that the bone structure in kids is very soft and can be moved slowly over a long period of time. To this day when I see a CF child with Sinus problems avoiding surgery and they have the same facial look I do I try to encourage the parents that the pro’s outweigh the cons”
Procedures include nasal saline irrigations (especially after surgery) to help moisten the nares and promote drainage (See Tricks of the Trade, for irrigation techniques). Vancenase or beclomethasone nasal sprays (steroid which is not absorbed systemically to any degree) may be advised, and this helps some people a lot. Antibiotics may also be used, because it is thought that post-nasal drip “seeds” lungs with infection. Sinus headaches may afflict some people from time to time (Tylenol or Motrin for those). Afrin Spray or Pediatric Nasal Drops may be advised when things are just too clogged up for breathing (limit the use of this to three days and infrequently or you will make things worse).
A first-person account of a non-surgical approach:
My (child), now 7 years old, has always had sinus problems which in turn were the cause of many upper respiratory infections. Up until (my child) was about 3 years old sinus infections would occur about every three to four months; we used low dose inhaled Tobramycin, and within 72 hours symptoms would improve. Then about 3-4 years ago we stated using high dose inhaled Tobra for 2 weeks at a time. We still got good results, sinus infections were about every 3-4 months and would go away quickly once we started the Tobra. Let me also add that (my child) inhaled the Tobra through a mask.
Then one year ago we started using Pulmozyme and started using a mouth piece. For the past year (my child) has had sinus infections almost every month. Once I sat down and went back over what was so different in this kid’s life, the only thing I could come up with was the change from the mask to the mouth piece.
Well two months ago when the sinus infection was not gone and (my child) was about to start Tobra again, I told the doctor that I was going to send the Tobra back up (my child’s) nose. Within 72 hours there was such a great improvement. The infection cleared up and has just now returned in a mild kind of way. This time of year here is very bad for him with the allergies and crazy weather changes day to day.
I sent a post to CYSTIC-L asking if anyone had any experience with inhaling Pulmozyme into the sinuses and I got some very positive responses. When this sinus infection started a few days ago I told (my child’s) doctor that I wanted to try an oral antibiotic and have him inhale Pulmozyme up into the sinus’. I was given the go ahead and we started him on Pediazole and four days later there was no more yellow mucus from (my child’s) nose and (my child) does not sound congested.
Most kids with CF sinus problems don’t need surgery and if they do, the question of when to do it can be difficult. You take your chances with sinus surgery in CF. Unfortunately, this is the nature of the beast … it comes back in some people sooner rather than later. When you see the word polyps, don’t think cancer … polyp is just a word to describe growths of extra tissue. Polyps are generally not a problem unless they block the nose or start to protrude out of the nose (a cosmetic problem) or start to widen the bridge of the nose (because of increasing number of polyps inside the nose). At this point, most will have the polyps removed. Nasal polyp removal can be done as an office procedure for some, others may need to be done in the hospital, some even requiring general anesthesia and sometimes an overnight stay in the hospital … depends on the ENT doing it, the age of the person, location of the polyps, etc. Some PWCF have just a few polyps and no more, some have many or recurring problems with polyps. Polyps can get larger or smaller without treatment. Treatment generally includes a steroid spray to help shrinkage of the tissue or surgery as mentioned above. For more detailed information on sinus surgery procedures, see the section of this document on Procedures.
Excerpts from a first-person account of a surgical approach:
My sinuses began to be a serious problem by the time I was 12. Since then, I have had over forty extensive polypoid tissue removals, and two major surgeries, …
… I have had the pain in my gums, and maxillary sinus areas for 52 years. I wouldn’t bore you any further with the incredible attempts by surgeons to alleviate my sinus problems, nor do I wish to frighten parents or PWCF about things to come.
… surgery leaves scars, and permanent pain …
ESSAL (Endoscopic Surgery w\ Serial Antimicrobial Lavage)
Patients attending Stanford CF center who were consecutively referred to ENT’s were evaluated before 1990 and treated conventionally and afterward by ESSAL. Conventionally refers to polypectomy, ethmoidectomy, antrostomy, or Caldwell-Luc operations.
The main outcome of this discovery was less frequent if ever needed sinus surgery. As of 1995 ESSAL is the MOST successful approach to treat CF sinus’s and reduces recurrence requiring further surgery for at least two years! All this knowledge was presented at the 1992 and 1993 Annual North American CF Conference in Washington DC October 15-18th(92) and in Dallas Texas(93). When Dr’ King left for Colorado Dr. Moss took over (his address is H306 Stanford Univ. Med. Center, Stanford, Ca. 94305-5519).
For further information see: “Management of Sinusitis in Cystic Fibrosis by Endoscopic Surgery and Serial Antimicrobial Lavage……………Reduction in Requiring Surgery” by, Victoria V. King M.D. and Richard B. Moss M.D., Archives of Otolaryngology Head and Neck Surgery Vol. 121, May 1995.
If necessary, you may request the protocol set up by Dr. King by contacting her staff. Ask for the CF Sinus Protocol, Endoscopic sinus surgery and Antibiotic Lavage. Dr. Victoria King1439 Main St.,Suite CCanon City, CO 81212
Phone – (719) 275-0008
A First-Person Account:
If anyone gets their sinus doc. to try this remember to be as informed as he his!!!!! I am a walking wealth of sinus info I’ve had 14+ surgeries the old ways and needed it again BAD. I had ESSAL in June by my peds. ENT and he changed the surgical approach during surgery. I awoke with tubes coming out my skull by my eyebrows and everything went downhill from there. You should have tubes coming out your nose holes. He screwed up the sinus flush orders, refused to come and see me and I taught the nurses how to flush the tubes. Needless to say I really didn’t know how either. I was on a regular talking basis to Dr. King so no one really knew what I knew and didn’t question me much. The after care of ESSAL is just as important as the surgery itself!! I never received the weekly aftercare to completely benefit from ESSAL so I am slowly headed back to where I started in June. My point to all this is educate yourself you need to rely on your own information, as well as the doctors. If I did not know what I did regarding ESSAL I would have thought everything went.
great. The really sad part in all this the ENT has done surgery on other CF’s using this approach and now thank goodness since I brought all this info to pulmonary they now are referring their patients to another doc. Our pulmonary Docs. (meaning yours too) are not familiar with the breakthroughs in this area because its not their field. Do your ENT’s go to the CF conferences for Doctors? The ones you see should, or consult with those that do. The Doc. I went to did but hey they can’t go to all the topics at one time and things are presented simultaneously.
Arthritis (joint pain)
Sometimes called “pneumopathic arthropathy” or “hypertrophic pulmonary osteoarthropathy”, it is a bout of arthritis that shows up when one’s lungs are in bad shape. Any pain killers treat the symptoms but do not resolve the underlying cause. The arthritic pain wanes away as the lungs get better.
Also, certain antibiotics can cause joint pain, making any recent changes in antibiotics the first thing to check if experiencing a sudden onset of this problem.
Hypertrophic Pulmonary Osteoarthropathy (HPO) refers to the situation where, on X-ray, the membrane covering the bone is elevated and there appears to be extra growth. The “Osteo” means bone, “arthro” means joints and “pathy” means something is wrong with. Clubbing of ones fingertips is considered a form of HPO.
In the book “Cystic Fibrosis”, Lynn M. Taussig, editor, Thieme-Stratton, Inc.(1984), it is indicated that severe HPO may occur in patients with stable lung disease, may occur within months of onset of lung disease or may be delayed for 20 yrs or more. The following is excerpted from Taussig’s book: “…HPO may increase with pulmonary exacerbations and tends to subside following treatment of the pulmonary disease; symptoms related to the bony abnormalities may occur independently of pulmonary symptoms. Signs and symptoms include pain, tenderness, swelling, and warmth of the involved areas….Often there is pain on walking…cold weather tends to exacerbate the pain, whereas warmth and rest tend to relieve the discomfort…..Painful, swollen joints without “x-ray evidence of” HPO are also seen in CF patients (episodic arthritis of CF), and it is unclear whether this is a variant of HPO, a serum sickness reaction to drugs, or a variant of juvenile rheumatoid arthritis” (excerpts from Taussig pgs.153-154). It goes on to say aggressive treatment of pulmonary symptoms tends to improve HPO “and joint discomfort” as well as rest, local heat, aspirin and various anti-inflammatory agents.
Another cause of severe joint pain, accompanied by red (chicken-pox like) lesions, is a rare virus called “Sweetz Syndrome”. The virus’s impact is exacerbated by respiratory infection.
The possibility of joint pain being due to rheumatoid arthritis can be checked out by a rheumatoid-factor test and a thorough joint exam.
One person notes: There IS a syndrome called “CF arthritis”, which a rheumatologist defined as similar to juvenile rheumatoid arthritis at a CFF Conference a year or two ago. That meant that 1) they don’t know why some people get it 2) there usually aren’t abnormal lab tests (such as a positive rheumatoid factor) 3) the treatment is symptomatic–lots of ibuprofen (as in 800 mg four times daily of ibuprofen for adults), and prednisone if the non-steroidal anti-inflammatories don’t work.
In my experience with adults with CF: * it’s more common the older you get * people who get it get it in certain joints; one woman always got it in her fingers, one in her hips, one guy in his knee… You NEED to take the meds to get ahead of the inflammation, so we tell people to take the drugs regularly for two to three days or until all symptoms disappear, then taper down and get rid of the drugs. One side effect of the ibuprofen study was a lesser incidence of arthritis and menstrual problems in the patients who were on the ibuprofen, according to Dr. Konstan.
Some people get the arthritis when their lungs are worse, and many women have more arthritis (and lung exacerbations) around their menstrual periods. This is probably more than you wanted to know–but I’ve found that many (PWCF and medical staff) don’t know of the association of CF with this type of arthritis, so have lots of unnecessary tests done and suffer needlessly.
For many people with CF, asthma is an added complication, bringing with it the need for additional medications and increasing the vulnerability of the lungs to infection. When asthma is first detected, it is very important to test carefully for allergies as they are often, though not always, the instigator of asthma. This is one of the areas in which parents sometimes need to reach beyond their usual CF caregivers and seek allergy expertise, testing, and nutritional guidance in reducing (sometimes even eliminating) the cause of the asthmatic reactions.
At present asthma is treated mainly with bronchodilators to relieve airway constriction, and — increasingly — with inhaled steroids which tackle the underlying inflammation of the bronchial tubes which causes asthma.
Hemoptysis (coughing up blood) (10/97)
Hemoptysis is not uncommon among people who have CF. Whereas there is no set medical explanation or treatment for it, experience has provided some possible causes as well as suggested ways to handle episodes of hemoptysis. Sometimes people have coughed up several cups of blood, so small amounts or streaking are considered mild amounts. Red (“frank”) blood indicates active bleeding; off-red or brown blood indicates old bleeding. While one drop of MY blood outside my skin is a major medical crisis for which the Marines should be called out, YOU should not be too upset about the appearance of mild amounts even if bright red. Call your doc, but no need to panic.
One way to look at coughing up specks of blood is as a warning – to slow down or temporarily suspend an activity which may be causing it. Drinking lots of liquids, resting, and not encouraging more coughing often brings the problem under control. IV meds are often prescribed next (though one person said the mucus acted as a pressure bandage and it’s reduction caused more bleeding).
Bronchial artery embolizations (a lengthy but practically painless procedure sealing off arteries) seem to be a last resort. First Person Account:” The procedure was painless (and very interesting as I could watch it on the monitors.) It turns out that my bronchial artery was over 10 times the diameter of a normal one. The procedure is simple enough once they find the offending artery. (They access the artery through the femoral artery in the groin in the same manner as they do for heart patients. The artery is blocked with a sponge like substance and it essentially dies as its only function is to supply blood to the Bronchial tubes and it seems veins supply enough blood to maintain the tubes. The only danger of this procedure is that they get the CORRECT artery as there is another artery in this area that feeds the Spinal Cord. My doctor said that the procedure is not very common as it usually is only needed by PWCF. What ever, it did the trick”.
Some take large amounts of vitamin K (5-15 mg per day — “normal” dose is 5 mg per week). Drinking ice water, sucking or eating ice, and placing a cold pack (water and alcohol in a zip-lock bag kept in the freezer makes slush) on the throat have been reported to be helpful in stopping the bleeding. A herbal remedy (Gotu Kola) is said to effectively treat fragile vessels and thus is effective in avoiding more drastic measures.
One person discovered chalk (used on a chalkboard) to be the likely cause of hemoptysis. Several women have found the problem linked to their cycles, raising questions about the effect of hormones on hemoptysis and/or the possibility of having endometriosis in the lung cavity (see “Discover” magazine, March ’95, pgs. 36-41). Other things that have been reported on CYSTIC-L to set off hemoptysis include: heparin (over 2000 units per day), aspirin, ibuprofen, hot and humid weather, caffeine-containing foods and drink (tea, coffee, colas, chocolate), cherry kool-aid, Septra, and even I.V. Tobra.
One person reported: “I was having problems with hemoptysis and high fevers (103) until I was treated for MAI, microbacterium avium intracellular complex (sp?). I was treated for this by a pulmonologist who consulted with an infectious disease specialist. My CF docs said MAI was common among people and did not want to treat me for it. I did not like this response so I went to a pulmonologist. Later the CF docs admitted that MAI does seem to cause problems for some CF people. MAI can be tested for in a sputum culture and it takes about six weeks to get the results. Before I was treated, I couldn’t bend over to tie my shoe laces without having a bleeding episode. I have only had one small episode since. I was also able to gain 10 lbs. after being treated. My over all health has greatly improved. I am a much happier person”.
One person reported: Taken from Consumers Reports Book “Complete Drug Reference”, 1995 Edition: Under subject title Penicillins and Other Medical Problems: “Bleeding problems, history of – Patients with a history of bleeding problems may be more likely to have bleeding when receiving carbenicillin, Piperacillin, or ticarcillin.” What it does is interfere with the platelets which in turn messes up the clotting and all…..even more so in people with renal insufficiencies (kidney problems make it even worse). In the mean time make sure your vitamin K levels are good…perhaps a vitamin K injection will help (used to do that for me when I was bleeding) and get off the piperacillin…and stay away from the other ones mentioned…that includes Timentin (derivative of Ticarcillin).
As the technology evolves which allows CF patients to live longer, new problems are cropping up. One of these is diabetes. About 30-50% of PWCF are reported to get glucose intolerance. This means that their sugars run higher than normal, and occasionally spill into the urine. It is not associated with diabetic problems such as increased fatigue, poor growth, excessive thirst or urination. It may not require insulin shots. True diabetes only develops in approximately 5-15% of all PWCF. Because diabetes tends to develop later on in PWCF, the prevalence goes up slightly, but is usually estimated up to 15% in adults with CF.
If there are any PWCFs out there who notice a change in their energy levels that doesn’t jive with their FEVs … and if you’ve been making more trips to the comfort station than usual … and if you’ve been somewhat off your feed … and if you’ve been losing weight at a fairly steady pace — get your glucose level checked! It’s a simple blood test, doesn’t take a lot of time, and may save your life!
The reason diabetes seems to be a problem in some people with CF has to do with the pancreas. The enzymes that are produced in the pancreas are not able to be used properly in people with CF. It is a theory that something similar happens with respect to insulin. Either the pancreas produces it, but the body can’t utilize the insulin because of too much mucus, or the pancreas is damaged in some way and stops producing insulin at all.
The development of diabetes in CF patients, and anyone else, for that matter, is not related to how much sugar you consume. In CF patients it’s caused by gradual scarring of the pancrease causing the destruction of beta cells which in turn lessens insulin production. Even when I was in the early stages of diabetes, my doctors told me that limiting my consumption of sugar would not slow the loss of insulin- producing sites. Also the diabetic with CF diet is a bit more liberal than the traditional diabetic diet because of the need to consume greater calories. Either way, the end result is the same – the person with CF and diabetes generally has to take insulin shots and monitor his/her diet. This may prove to be a troublesome burden, especially if the diabetes shows up around adolescence, when most people with CF are struggling with demands of both CF and adolescence at the same time.
It is important to try to find appropriate medical supervision for the person with CF and diabetes. Most regular endocrinologists may only have a sketchy idea at best about what CF is and how it relates to diabetes. But it is very important, because diabetes and CF/diabetes are really two separate diseases that must be treated as such.
Another important consideration is the person with CF/diabetes’ diet. If the person is underweight, restrictions on his/her diet may prove costly to overall nutrition. Again, this is where a well-informed physician can be a lifesaver.
One last interesting fact, that our diabetes docs let drop like it was common knowledge (which it WASN’T to any of us pulmonary people): If your blood sugars go over 200 (see Measurement of Blood Sugar below), your white blood cells go on holiday. CF bacteria grow really well in a high-sugar atmosphere, and then the WBCs shut down “your body’s main infection fighting mechanism , so that’s why PWCF get so sick so fast when they get diabetes.
Measurements of Blood Sugar (International System Units (SI) outside the USA; ‘units customarily reported’ inside the USA)
Normal (fasting) glucose:
US: 70 – 110 milligrams per decaliter (mg/dL) Everybody else: 3.85 – 6.05 millimoles per liter (mmol/L)
The conversion factor is 0.055; so…200 mg/dL = 11 mmol/L
“Episodic diabetes” is a name sometimes given to high blood sugars resulting from prednisone bursts prescribed to treat asthma or other conditions. A medical person reports “one of my patients had classic symptoms (weight loss of 10 lbs, fatigue, excessive thirst and urination) after 3 days on 40 mg. I think part is due to the dose, and part is due to any pre-existing tendencies/prior pancreatic damage” It’s been suggested that one way to detect/control this reaction would be to “have a fasting blood sugar drawn after the first couple doses of any prednisone bursts. If the blood sugar is high, one could use finger sticks and insulin just during prednisone bursts; this would need to be checked as often as four times daily, as the whole point is to keep those sugars under 200, and the patient eats regularly, right”
Glucose Tolerance Test (contributed by Dana Broehl)
Susanne Lanng Department of Pediatrics, Rigshospitalet, CF Centre Copenhagen, Copenhagen, Denmark
Reference: CYSTIC FIBROSIS RELATED DIABETES by Dr. Antoinette Moran, MD, assistant professor, Department of Pediatrics, University of Minnesota [published in the IACFA Newsletter]
Diabetes has evolved as a complication because of increased longevity of patients with cystic fibrosis. CF related diabetes (CFRD) is associated with increased morbidity and mortality, therefore, prompt diagnosis and aggressive management are important. The prevalence of CFRD increases with age with an age-dependent incidence rate of 5% per year; at 30 years 50% of patients are diabetic. CFRD develops insidiously. Screening by measurements of fasting, random plasma glucose or glycated haemoglobin A(1c), alone or in combination, do not reliably identify CFRD as compared with the 2-hour plasma glucose value measured during an oral glucose tolerance test. In addition, a high percentage of cystic fibrosis patients with diabetes do not have fasting hyperglycemia. Consequently, the best and most reliable way to screen CF patients for diabetes is to administer an oral glucose tolerance test.
An oral glucose tolerance test is one that can be performed in a doctor’s office or a lab. The person being tested starts the test in a fasting state (having no food or drink except water for at least 10 hours but not greater than 16 hours). An initial blood sugar is drawn and then the person is given a “glucola” bottle with a high amount of sugar in it (75 grams of glucose). The person will then have their blood tested again up to 4 times (30 minutes, 1 hour, 2 hours or 3 hours) after drinking the high glucose drink.
There are four categories of glucose tolerance in CF, based on the response to a standard oral glucose tolerance test. The first is normal, with normal fasting blood sugar and the challenged glucose level never above 200 mg/dL. The second is impaired glucose tolerance, in which the fasting blood sugar is normal, and the challenge glucose level exceeds 200 mg/dL but returns to normal after 2 hours. The third category is diabetic glucose tolerance, with normal fasting blood sugar and challenge glucose level that exceeds 200 mg/dL and stays elevated for at least 2 hours. The most severe type of abnormal glucose tolerance is overt diabetes mellitus, with fasting blood sugar above 140 mg/dL.
Also see Appendix L, Longer Articles, item 1, for an article on CF Diabetes. Also see item 7, for another perspective.
Summarized and quoted from: AN INTRODUCTION TO CYSTIC FIBROSIS FOR PATIENTS AND FAMILIES by James C. Cunningham, M.D. and Lynn M. Taussig, M.D., copyright 1989, 1991 Cystic Fibrosis Foundation, sponsored by McNeil Pharmaceutical, (pages 55-57).
“In most males with CF, the vas deferens may be blocked by thick mucus secretions, or it may be abnormally developed.
As a result sperm cannot pass. Even though the testes make sperm and intercourse occurs normally, the semen ejaculate contains no sperm and therefor cannot cause pregnancy. It is believed that blockage of the vas deferens often occurs even before birth, and there is no way to remove the blockage at the present time. Because of this problem, about 98 percent of males with CF are sterile.”
…Infertility should not be assumed. In a small percentage of men the vas deferens is not completely blocked enabling them to father children. This can be determined by a sperm count (Semen analysis). “The problem with the reproductive system in men with CF is strictly a mechanical problem) blockage of the vas deferens. The sexual hormones and glands are not involved in CF. Sexual growth and development proceed normally, although sometimes at a slightly slower rate because of lung disease and malabsorption of food…”
Fertility varies widely from one woman to another and may also vary over time depending upon the individual’s health and extent of her disease. Since physical limitations which accompany CF vary any woman (couple) considering pregnancy should evaluate the risks and consult with their partner and physician knowledgeable about CF and the person involved. Vaginal and cervical secretions (mucus) may be so thick that sperm cannot move through it in women with CF. Also women with CF may menstruate irregularly and ovulate less often, especially when experiencing lung problems. Consequently, women who have CF may be less fertile but conception is possible and “many hundreds of women with CF have had successful pregnancies.” Sex glands and hormones in women with CF are not affected though development may be slower due to malabsorption of food and lung disease.
MESA – A Chance at Fatherhood
The IACFA Newsletter (September, 1993, issue 34) featured an article on the Microscopic Epididymal Sperm Aspiration (MESA) technique written by Dr. Oates of Boston. The technique must be done in conjunction with in-vitro fertilization. Since it is known that at least one partner has CF, there will be the risk of any child conceived using this method of inheriting CF.
The technique has been in use primarily for those with CBAVD (Congenital Bilateral Absence of the Vas Deferens). Approximately 97-98% of men with CF are sterile due to an incompletely formed or blocked vas deferens (the tube which carries sperm from the testicles to the penis). The sperm cells made in the testes may be normal, but are blocked, similar to the effects of a vasectomy, and there is ejaculated fluid without sperm. MESA aspirates the viable sperm from behind the blockage. Once retrieved, the sperm may be used to promote in-vitro fertilization. Men with CF have fathered children using this method. There has been success using MESA in the UK, Sweden and Israel. There may be a complication, common to that found in vasectomized men, where the sperm upon aspiration is relatively low in count and/or motility. Apparently the Toronto team makes up a reservoir to heighten the sperm count, but their stats are not known.
It should be made abundantly clear that should two people with CF, one man, one woman, consider MESA, then an overall assessment of the woman’s health needs to be carefully considered since pregnancy is known to adversely affect the health of women with CF. In addition, as with any procedure, the probability of success and the risks (to the patient) should be thoughtfully discussed with a trusted physician.
Fertility and Prenatal Testing (8/97)
by Roe Ann Roberts
To fertilize an egg (ovum), your sperm must be able to perform these critical tasks: It must be able to swim to the egg It must be able to penetrate the egg.
To test sperm to assure it can perform these vital functions a semen analysis and a hamster test must be conducted. The semen analysis tells you if your sperm are adequate in number and form, and if the volume of your ejaculate are normal; these criteria are essential if the sperm is to be able to swim to the egg. Also, if the volume of the liquid which transports the sperm is too low, there may not be enough fluid to bring the sperm in contact with your mate’s cervix. The hamster or acrosin test can tell you if your sperm can penetrate the egg for fertilization.
Health fertile males produce sperm which has the following characteristics:
- 3 milliliters in volume
- It will contain 20 million or more sperm per milliliter
- After two hours about 50% of sperm will still be active
- 60% of the sperm will appear normal
- The ejaculate will liquefy within an hour
- ph: 7 to 8
Males with CF have a high incidence of congenital hypoplasia or absence of the major portion of the epididymis, vas deferens, and seminal vesicles. Studies indicate that 95-98% of males with CF are unable to produce sufficient live, healthy sperm due to blockage, or absence, of the ducts (vas deferens) leading from the testicles to the penis. However, many of them still have some sperm production in the epidemymis (testes) themselves. Thus, with help they may be able to have genetic children.
Advanced reproductive techniques such as epidymal sperm aspiration (removing sperm from the epididymis) MESA and ICSI (direct injection into eggs retrieved by IVF – see IVF) results in pregnancy rates between 25 and 30%.
MESA Microsurgical epididymal sperm aspiration (MESA), utilizes a microscope to get a specimen of sperm from proximal to the obstruction. It is done as an outpatient procedure, but is the most involved of the option. Testicular sperm aspiration or extraction (TESA) and percutaneous epididymal sperm aspiration (PESA) utilizes a small needle under local anesthetic to aspirate sperm from proximal to the obstruction.
Testicular Sperm Biopsy (TESE), taking a small biopsy of testicular tissue under local anesthesia. It is most often used when no sperm are obtained with MESA, TESA or PESA.
ICSI (intracytoplasmic sperm injection), has had success with men with no motile sperm; using a small amount of sperm, obtained from the epididymis via microsurgery.
ICSI involves taking a microneedle, puncturing the outside layers of the ovum and injecting a single sperm directly into the egg. It is an expensive procedure as the female must undergo in vitro fertilization (see IVF) in order to produce sufficient eggs for retrieval and fertilization.
After injection of the sperm, the eggs are incubated for 16-18 hours, then examined for possible damage and for evidence that fertilization has occurred. The resulting undamaged embryos can then either be transferred back to the woman’s uterus, using standard IVF techniques (or ZIFT), or can be frozen for transfer at a later time. (see female fertility/infertility)
Clinical indications for intra-uterine insemination.
Women with cystic fibrosis may have thick, scant cervical mucus. 1. Poor Quality Mucus does not store, mature, and transport sperm from the cervix into the uterus, so that the sperm can then enter the fallopian tubes where fertilization occurs. Abnormalities cervical mucus can sometimes be corrected with medical treatment. When medical treatment doesn’t intrauterine insemination can bypass the thick cervical mucus and place the sperm directly into the uterus.
Artificial Insemination Sperm can be placed directly into the uterus after it has been specially processed. First the semen is separated from the seminal fluid. Secondly, the semen is processed to remove abnormal sperm and thus to maximize the husband’s fertility.
IVF In vitro fertilization (IVF) was initially used for women with blocked fallopian tubes. In the case of a male with CF, IVF can be combined with MESA amd used to fertilize the female’s eggs. If two carriers desire to have a CF free child they may use IVF to fertilize a number of eggs which then become growing embryos. These can be tested for CF when they are eight cells in size. Using micromanipulation, a cell is removed from the embryo and subjected to genetic diagnosis. Two basic methods have been used. The first involved CNA amplification via the polymerase chain reaction (PCR); the second detection of one or both sex chromosomes in an intact interphase nucleus by in situ hybridization (FISH). The eggs which are not found to be positive for CF are then inserted into the mother’s uterus. This preimplantation genetic analysis approach may be preferable for many who are not comfortable with terminating an ongoing pregnancy. For those who would wish not to carry a CF fetus! to term, and who are not uncomfortable with termination of a pregnancy, amniocentesis and chorionic villus sampling are viable and much cheaper alternatives to IVF. Many IVF centers now use vitro fertilization for both infertility problems and as a genetic screening.
All IVF technologies involve a number of similar steps. First, the ovaries must be stimulated to produce multiple follicles (which each contain ova). A number of expensive medications are used to stimulate the ovaries to produce multiple follicles: Lupron and Metrodin or Pergonal. The lupron is used to ‘turn off’ the ovaries so that the doctor can control the cycle more effectively. The metrodin/pergonal is used to send the ovaries into overdrive. The mother may produce as few as three or as many as thirty plus follicles. One potential problem with IVF is if the woman is overstimulated she may become very ill. Therefore, she must be monitored closely while on these medications.
When the follicles are mature, you will given an HCG injection. This injection helps mature the ova. You are then scheduled for egg retrieval. You will be brought into the operating room and an anesthesiologist will give you a medication to make you as sleepy as you want. An ultrasound probe in the vagina will direct a needle located alongside of it, into the ovaries and the eggs will be suctioned from the follicles on your ovaries. This procedure lasts 20 to 30 minutes. The sperm will be inserted into the eggs via ICSI (if your spouse has CF) later on the day of the retrieval. Two days after the retrieval, if there are fertilized embryos, they will be replaced. In general, 3-5 embryos are transferred depending on the age of the mother.
IVF costs range from $8-15,000. A large part of these costs is due to the high price of the drugs.
Prenatal Genetic Testing
When two individuals, who both have the gene(s) which produces CF, have a child there is a 1 in 4 chance that the child will have CF and a 2 in 4 chance the child will be a carrier. One way to assess whether the fetus has CF is via amniocentesis. Of course, amniocentesis carries a risk of fetal loss (0.3-0.5%) as it is an invasive procedure. Miscarriage is uncommon, but remains the most serious risk stemming from amniocentesis. Amniocentesis, is performed by a physician who uses a thin needle to obtain a small amount of amniotic fluid from the uterus, amniotic fluid is the “water” which surrounds the fetus. Ultrasound is used to guide the needle so that the risk of the needle actually touching the baby is very low. The procedure may be done in the doctor’s office or at a hospital clinic, it can be performed as early as twelve weeks, but usually is done in the 16th week of pregnancy. Early amniocentesis may present an increase risk according to Cynthia G. Brumfield, MD.[1! ] Her study found that women undergoing amniocentesis between their 11th and 14th week of pregnancy are 11 times more likely to have a miscarriage, (2.2% vs 0.2%) than those who wait until at least the 16th week . Also early amniocentesis also presented an greater risk of immediate post-procedure amniotic fluid leakage (2.9% vs 0.2%) and vaginal bleeding (1.9% vs 0.2%), both of these complications can pose a serious risk to the mother and fetus.
The amniotic fluid that has been collected is then checked and a fetal chromosome analysis is performed. This test exams the cells contained in the amniotic fluid to identify whether the fetus has the CF genes. Only about 73 CF genes are currently being identified by testing centers, however these genes are the most commonly occurring. The cells are few in number and must be grown so the results are usually not available for about10 days. Early identification of a fetus with CF may serve one of several functions. It may allow the parents to consider termination of the pregnancy via saline induction; it may allow the doctor to more accurately monitor and maintain the in-utero health of the fetus; and it may enable the parents to better prepare themselves to undertake the challenge of caring for a child with CF. CF is no longer a death sentence, the median life expectancy of a child with CF is now 31 years. Parents and physicians who are prepared for the birth of a child w! ith CF may be able to better met their physical, emotional and social needs.
Chorionic Villus Sampling (CVS)
CVS can be conducted in pregnancies which are less than 12 weeks pregnant. This method can be conducted from the beginning of the 10th week through the 12th week of pregnancy, the risk is about 1% or less. One problem with CVS is the mother’s cells may get into the sample and thus give an inaccurate result. If a question arises as to the accuracy of the results, amniocentesis may be necessary to confirm them. The choronic villi are small finger like projections which contain the genes of the developing embryo, a small sample cam be removed by one of two ways: 1.Transcervical: Using ultrasound a thin tube is inserted through the vagina into the uterus 2.Transabdominal: This same approach is the same as is done for amniocentesis except the needle does not enter the amniotic sac. As with amniocentesis the results are usually available in about 10 days.
 Brumfield CG, Lin S, Conner W, et al. Pregnancy outcome following genetic amniocentesis at 11-14 vs 16-19 weeks’ gestation. Obstet Gynecol. 1996;88:114-118.
Liver Dysfunction (9/97)
Just like other organs in the body, the liver is affected by the production of mucus in those with CF. The ducts of the liver are infiltrated with mucus causing some dysfunction. At the extreme end, cirrhosis of the liver may occur. Usually liver dysfunction is related to the mucus build-up and fatty liver cells caused by malnutrition and for the most part is not serious. One CF professional reports “Considerably more than 3% of the CF patients in our clinic experience elevated liver enzymes, and are on Actigall (URSO deoxycholic acid). Some CF doctors have discussed the idea of using URSO in a large percentage of CF patients as a prophylactic measure”.
CF Liver Problems Q & A
Q. My CWCF has a problem with the liver. They make a echo every year but she doesn’t get any medication for it.
A. I don’t know what an “echo” is – do you mean an ultrasound? I have these done regularly, and it’s mostly because I have cirrhosis of the liver (sometimes just referred to as hepatitis, as that just means “illness of the liver” or something), although also because this has meant I have developed “portal hypertension”, which is where the pressure of the blood unable to get through the liver fast enough is diverted through the spleen and makes it bigger than it should be, resulting in a “beer gut” like look to the abdomen (although this is common to some extent – the look, not the syndrome – in many PWCF) and making the lower left side of the abdomen more tender than the rest. Do you know if your CWCF has this, or maybe if your docs think she might develop it sometime and that’s why they keep doing the echoes?
Q. Can you do anything about the condition of the liver??
A. Not as such, but if it becomes a problem there are many ways round it – I have a TIPPS shunt to deal with the portal hypertension (but don’t worry about what it is unless it’s actually an issue), regular checkups to make sure nothing goes wrong, and am currently half awaiting a transplant – of course normally this isn’t needed just because there’s some liver problem, and if your CWCF isn’t on any medication it probably doesn’t matter at the moment.
Q. I wonder what others are taking for medication.
A. Ursodeoxycholic acid (urso, or brandname ursofalk) is often used as it seems to improve liver function generally over time, although I don’t think they know quite why. I’m on urso and have been for years, and it doesn’t make any noticeable difference, but then I’ve nothing to compare it with; it just seems like a good idea to do whatever can be done to help it. The biggest problem with CF liver problems is prognosis can be very inacurate, or impossible – it deteriorates, or not, at a completely random, irregular, unstable rate.
Excess Stomach Acid
Some CFers suffer from excess stomach acid which may digest pancreatic enzyme capsules too soon, or result in frequent gastric reflux (stomach acid comes up esophagus). Acid blockers including Tums, Tagamet, Zantac, etc. may be used to combat these effects. Prilosec has been reported to succeed when others have failed. It’s rather expensive (~ $4.00/capsule in L.A.) and you need to take it daily. Prilosec works differently from the acid blockers: the acid blockers block the chemical production of acid in the stomach; Prilosec turns off the acid pumps in the stomach. DO NOT take Prilosec with any of the acid blockers (Tagamet, Zantac, Pepcid, etc.) They counteract one another and basically result in no effect at all. Transitioning from one to the other can take a day or two to flush the residual discontinued med from your system. During that time you can experience some real reflux. First-person Account: “Finally, the circular also talks about some ** Suspected ** carcinogenicity. I asked my Gastroenterologist about that and he said that it was only a mild suspicion and that the doses given to the rats that developed cancer from its use were so large that an adult human would have to take several pounds of the med per day (as opposed to one capsule) for a couple of years to get the equivalent dangerous dosage. I have been on it for three or four years at least and my doc just keeps renewing my script every month. So far as I know I have no problems”.
Tremors break down into 2 categories: essential and medicine induced. With essential tremors there is usually a family history of tremors or Parkinsons Disease. Theodure, Seldane, and Sudafed are the biggest shake causers for the medicine induced. Medicines are available for both types (just what you needed: more pills!). The exam was a pleasant surprise. A quick test of reflexes and a quick physical. No blood or other testing was required. It was a nice change!
In CF adults HYPOglycemia–too little blood sugar–is much more prevalent than HYPERglycemia. That cold, clammy, weak feeling is not just you — it’s your CF. Classic symptoms include weight loss of 10 lbs, fatigue, and excessive thirst and urination. Many people with CF do make insulin — they just produce it more slowly than those without CF, so the insulin is secreted into the blood stream AFTER the peak blood sugar rather than during, so your blood sugar drops and you feel weird. Luckily, the cure is simple; keep simple sugars on hand for emergencies, and eat lots of complex sugars (rather than simple sugars) to avoid that high high sugar peak and maintain a more constant blood sugar level.
CF related hypoglycemia was explained to me by the medicos as follows: PWCF’s with pancreatic insufficiency produce pancreatic enzymes, but the pancreas does not release them due to blocked exocrine glands. The pancreatic enzymes remain in the pancreas and start to digest (ughhh) the pancreas, causing the pancreas to scar. This scarring inhibits the pancreas from releasing glucagon, the hormone that increases blood glucose levels. I overheard a doc that is not my own, tell another PWCF that individuals with hypoglycemia may be more apt to develop diabetes at some point since the hypoglycemia indicates the pancreas has substantial scarring. Diabetes in CF occurs when the islet cells in the pancreas are damaged by scarring.
Measurements of Blood Sugar (International System Units (SI) outside the USA; “units customarily reported” inside the USA) for normal (fasting) glucose:
USA: 70 – 110 milligrams per decaliter (mg/dL) Everybody else: 3.85 – 6.05 millimoles per liter (mmol/L)
(The conversion factor is 0.055; so…200 mg/dL = 11 mmol/L)
First Person Accounts of Coping Techniques:
Glad to hear the info on AWCF hypoglycemia. I’ve been walking around telling people I had it for years. For me, it comes with headache and nausea. It’s also easy for me to manage – I learned a long time ago that orange juice and pretzels (or crackers) do the trick if I’m “crashing”. I know the orange juice provides the sugar, my only guess is the crackers give me some salt and something for the acid in my stomach to work on. BTW, *high* sugar foods – like chocolate don’t help me, ’cause 10-15 minutes later I’m going through the exact same thing.
The sugar in orange juice is fructose rather than glucose, but I can’t remember why this might be significant. The difference between, say, chocolate and this is not to do with the amount of sugar as such, but the type of carbohydrate. Fast-acting carbohydrates like simple sugar is used up quickly, while slower-acting ones like starch take time to be used as they have first to be broken down into simpler sugars. So the best way to treat a hypo’ is to give enough fast-acting carbohydrate (sugar, chocolate, honey) to bring your blood-levels up to normal, and then some slower-acting stuff (bread, potato, or in your case pretzels or crackers) to keep them there. I find a honey sandwich is perfect.
Can CFers Have High Cholesterol?
A study reported in the Journal of the American Dietetic Association concluded that a high-energy, high-fat diet does not raise serum lipid levels in CF patients with pancreatic insufficiency. However, those CF patients with normal pancreatic function may be at the same risk as the general population for developing high serum lipid levels. The researchers recommended that these patients have their serum lipid levels monitored and be given appropriate dietary recommendations.
Reference: Slesinski, Mary Jane, et al. “Lipid levels in adults with cystic fibrosis.”, Journal of the American Dietetic Association 94.4 (1994): 402-408.
One person reports: My gallbladder problems we now know started when I was about 14 or 15 (I’m 25 now). I’d get these kind of intense pains in the middle of the night up high, sort of just below my sternum. At that time, if I had a drink of cold milk – and for some reason it *had* to be really cold – it would go almost straight away. Occasionally it would get really bad, then of course it was like – is it gallstones? is it a blockage? Treat the chest infection while we’re at it and the pains must be all in the mind.
About two years ago it suddenly got really bad, to the point where I couldn’t breathe for the pain and we’d rush into hospital just to get so pethidine…you know it’s hurting when you’re actually glad to see a big needle heading for your behind!!!
Anyhow they finally decided it had to be my gallbladder, I was a bit jaundiced by then too. So I went down to Perth and had ultrasound etc (finally!) and they took it out. The thing was such a mess that they couldn’t do it via laparoscope as planned, so now I have another *zip* added to my collection. 🙂
I don’t know how common it is in PWCF, but there was another guy in the ward afterwards; I did put on a few kilos, but definitely not fat!!! Anyhow, nobody seemed surprised at all, not like when they found out my appendix had ruptured at least three times before it was taken out!!!
Another says: My Dad had a bit of trouble with his gallbladder a while back, I told him to drink cold milk for the pain and he thought I was nuts, but he did, and it worked.
Another says: I used to have problem with my gallbladder also every 4-6 months. Another remedy you could try when your gallbladder is acting up, at bedtime take about 4 oz. of Olive Oil and wash it down with lemon juice or hot apple juice. Sleep on your right side as this is the side your gallbladder is on. This remedy helps dissolve the build up of bile in the gallbladder with the acid of the apple juice and the Olive Oil moves the residue. I haven’t had a problem in over two years now.
Another says: I have had a very unhappy gall blatter for some years now and these are some things thay really get it hurting : beans (chili, burritos, etc.), nuts, popcorn, chicken (I know sounds wrong but especially white meat chicken), high fat foods, and large quantities of any food (especially in the evenings). There is a book called “7 Days to a Settled Stomach” by Dr. Ronald Hoffman…you can get this on the internet from his site:http://www.wor710.com:80/index.htm
There is a section on gall blatters that may be helpful, it’s a paperback and not too expensive. I have had all kinds of tests for stones or inflamation and this has never been found. The pain which is acute, comes from spasms…….so far I can usually avoid severe attacts but occasionally I need a 600mg dose of Advil :-/. Remember each situation is different keep in touch with your Dr……gall blatters can cause lots of trouble, stones, infections, etc.
Kidney stones are a side affect from too many enzymes, malnourishment and just our basic body chemistry. I found this out one Saturday suddenly when my urine turned, basically, to straight blood. It turned out that my kidney stones were moving around and scraping causing the bleeding. It just so happened I was on home care and the doctors thought I was having kidney failure from too high of a dose of medication. I was quite happy to find out it was just stones. I’ve heard that kidney stones are pretty rare but I just wanted to let all of you guys know that this is just another complication to store in the back of your minds so that you aren’t as panicked as I was when this happened.
On March 16, while installing a new PICC line (under flouroscopy with die injection), they found a blood clot in the sub-clavical (sp?) vien under his left collarbone. They figure this occurred in conjunction with the PICC line inserted on March 9 which we pulled out around April 4. Apparently once a clot is more than 1 or 2 weeks old it is harder to dissolve. Since he also needed sinus surgery the decision was made to proceed with the surgery and let the clot wait. They did not want to try any clot dissolving or blood thinning protocols near the time of surgery for fear of creating bleeding problems where the surgery is healing. Right now we are in a holding pattern and may just end up leaving it there and see if the body breaks it down naturally.
… I had to have my port-a-cath removed as I developed a
right internal jugular clot which was deemed too risky to dissolve with Streptokinase. The vascular surgeon talked it over with me and opted to remove the whole port.
… You want clot experiance, I’m your man, unfortunately. I had about six medi-ports and or Hickman catheter lines. All in various places, both of my suppieor veinacava (big veins in your neck), both major veins in my groin and a few other places are totally blocked. One time, they tried urocinase to disolve a clot, but it caused a piece to break off and send me to ICU on a 100% o2 face mask. So, I’d be carful as to disolving clots cause thing can go wrong. Anyway, most of the blood has found collaterial circulation around the clots.