Liam was discharged Friday April 27th from UCSF. The ONLY thing they did was send him home on oxygen. He was on half a litter when we left but since has been uped to 1 litter. Half a litter kept him from desatting but didn't help him much otherwise. 1 litter has taken away most of his "asthmatic episodes" of coughing and struggling. Monday he will be admitted to Valloey Childrens, again, where we will run several test:
CT scan of his lungs
Bronchioscope of his ariway
Sweat Test for Cystic Fibrosis
ECHO for pulmnary hypertention
On an xray that was taken of Liams lungs about 2 months ago or so, scar tissue was reportedly seen on the bottom of his right lung. Hopefully the CT will show more and be able to tell us more about this. Were praying that there is nothing wrong with his airway because if there is, he might need a trach. Liam has alot of symptoms of Pulmonary hypertention. We pray he does not have PH but if he does were praying they catch it before its critical and untreatable. Pulmonary Hypertention is something that kills alot of CDH babied before they even get a chance to get their repair. PH is also something that sneaks up on some CDH babies when we all think that theyre in the clear. We were forwarned to keep an eye out for PH. We are also testing for Cystic Fibrosis because our Pediatrician wants to cover all the basis. Even when Liams breathing is under control he is still very slow at putting on weight and CF is the last thing our Ped can think of that would cause slow weight gain.
What sucks is that Liams symptoms match both PH and CF. And patients with CF generally have PH as well. Liam does have alot of mucus in his lungs and airway (like CF) and when weve been qble to suction it out its been very thick (just like CF).
We, I, am asking that you keep our little Liam in your prayers. He's a fighter and has given us such hope that he will continue to fight. Things are hard on our family, being split up most of the time with Liam and I at the hospital and Justin and Lanie at home. It's hard for Lanie to understand and has told me during our recent stay at UCSF that she just wants me to leave him there and come home to be with her. Because of the strain on our family I am also asking that you keep us in your prayers and pray for strength and understanding as well as patience. God answers prayer and Liam is proof of that. Prayer has gotten us through the worst times of our lives and I have faith that prayers will continue to be answered. We sencerly thank you for your love and support of our little Liam.
floor at UCSF. She quickly became like
family.)
A glimpse into the future? Dr Liam has a nice ring to it
Playing with his food not actually eating it.
Sissy pretending to have a nasal canula like Liam and copying him.
More info on the conditions mentioned above:
Pulmonary Hyertention is rare and can be confused for asthma and COPD. (Those of you that have followed Liams story know that he has been diagnossed as asthmatic just because he has asthma like symptoms and asthma runs in the family. Asthma has since been basically cast aside)
In medicine, pulmonary hypertension (PH) is an increase in blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, together known as the lung vasculature, leading to shortness of breath, dizziness, fainting, and other symptoms, all of which are exacerbated by exertion. Pulmonary hypertension can be a severe disease with a markedly decreased exercise tolerance and heart failure. It was first identified by Dr. Ernst von Romberg in 1891.[1] According to the most recent classification, it can be one of five different types: arterial, venous, hypoxic, thromboembolic or miscellaneous.
Pulmonary venous hypertension typically presents with shortness of breath while lying flat or sleeping (orthopnea or paroxysmal nocturnal dyspnea), while pulmonary arterial hypertension (PAH) typically does not.
A detailed family history is established to determine whether the disease might be familial. A history of exposure to drugs such as cocaine, methamphetamine, alcohol leading to cirrhosis, and tobacco leading to emphysema are considered significant. A physical examination is performed to look for typical signs of pulmonary hypertension, including a loud S2 (pulmonic valve closure sound), (para)sternal heave, jugular venous distension, pedal edema, ascites, hepatojugular reflux, clubbing etc. Evidence of tricuspid insufficiency is also sought and, if present, is consistent with the presence of pulmonary hypertension.
All info listed was from Wikipedia. If you'd like more info just click here.
Cystic Fibrosis:
The name cystic fibrosis refers to the characteristic scarring (fibrosis) and cyst formation within the pancreas, first recognized in the 1930s.[2] Difficulty breathing is the most serious symptom and results from frequent lung infections that are treated with antibiotics and other medications. Other symptoms, including sinus infections, poor growth, and infertility affect other parts of the body.
CF is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR). This protein is required to regulate the components of sweat, digestive juices, and mucus. CFTR regulates the movement of chloride and sodium ions across epithelial membranes, such as the alveolar epithelia located in the lungs. Although most people without CF have two working copies of the CFTR gene, only one is needed to prevent cystic fibrosis due to the disorder's recessive nature. CF develops when neither gene works normally (as a result of mutation) and therefore has autosomal recessive inheritance.
CF is most common among Caucasians; one in 25 people of European descent carries one allele for CF.[3][4] The World Health Organization states that "In the European Union, 1 in 2000-3000 newborns is found to be affected by CF".[5] Individuals with cystic fibrosis can be diagnosed before birth by genetic testing, or by a sweat test in early childhood. Ultimately, lung transplantation is often necessary as CF worsens.
The hallmark symptoms of cystic fibrosis are salty tasting skin,[7] poor growth and poor weight gain despite a normal food intake,[8] accumulation of thick, sticky mucus,[9] frequent chest infections and coughing or shortness of breath.[10] Males can be infertile due to congenital absence of the vas deferens.[11] Symptoms often appear in infancy and childhood, such as bowel obstruction due to meconium ileus in newborn babies.[12] As the children grow, they must exercise to release mucus in the alveoli.[13] Ciliated epithelial cells in the patient have a mutated protein that leads to abnormally viscous mucus production.[9] The poor growth in children typically presents as an inability to gain weight or height at the same rate as their peers and is occasionally not diagnosed until investigation is initiated for poor growth. The causes of growth failure are multifactorial and include chronic lung infection, poor absorption of nutrients through the gastrointestinal tract, and increased metabolic demand due to chronic illness.[8]
In rare cases, cystic fibrosis can manifest itself as a coagulation disorder. A double recessive allele is needed for cystic fibrosis to be apparent. Young children are especially sensitive to vitamin K malabsorptive disorders because only a very small amount of vitamin K crosses the placenta, leaving the child with very low reserves. Because factors II, VII, IX, and X (clotting factors) are vitamin K–dependent, low levels of vitamin K can result in coagulation problems. Consequently, when a child presents with unexplained bruising, a coagulation evaluation may be warranted to determine whether there is an underlying disease.[14]
Lung transplantation often becomes necessary for individuals with cystic fibrosis as lung function and exercise tolerance declines. Although single lung transplantation is possible in other diseases, individuals with CF must have both lungs replaced because the remaining lung might contain bacteria that could infect the transplanted lung. A pancreatic or liver transplant may be performed at the same time in order to alleviate liver disease and/or diabetes.[76] Lung transplantation is considered when lung function declines to the point where assistance from mechanical devices is required or patient survival is threatened.[
Quality of life
Chronic illnesses are very difficult to manage. According to Schmitz and Goldbeck (2006), the fact that Cystic Fibrosis significantly increases emotional stress on both the individual and the family, "and the necessary time-consuming daily treatment routine may have further negative effects on quality of life (QOL)".[92] However, Havermans and colleagues (2006) have shown that young outpatients with CF that have participated in the CFQ-R (Cystic Fibrosis Questionnaire-Revised) "rated some QOL domains higher than did their parents".[93] Consequently, outpatients with CF have a more positive outlook for themselves. Furthermore, there are many ways to improve the QOL in CF patients. Exercise is promoted to increase lung function. The fact of integrating an exercise regimen into the CF patient’s daily routine can significantly improve the quality of life.[94] There is no definitive cure for Cystic Fibrosis. However, there are diverse medications used such as, mucolytics, bronchodilators, steroids and antibiotics that have the purpose of loosening mucus, expanding airways, decreasing inflammation and fighting lung infections.[95]
In addition CF patients cant be around others with CF because the bacteria in one patient can be spread to another causing death.
Info from Wikipedia. Click here for more info.
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